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SYMPOSIUM: PSYCHIATRY
Dealing with acutely disruptive children in hospital

Anthony Crabb
Difcult behaviour in the classroom or at home is one thing, but when it presents itself on a paediatric ward it brings with it some unique challenges. In this article I hope to address some of the issues surrounding disruptive behaviours and briey describe a range of strategies used to manage them, as well as some of the National guidance and legal frameworks.
Understanding the impact of Systemic Anxiety in behavioural disturbance Abstract

Disruptive children in a paediatric setting can be difcult to manage, can make treatment decisions more complex and put the young person, parents and staff at risk. Risks include delayed or incomplete treatment, and physical harm to the patient, parent or staff members. Anxiety generated around a young person acting in a disruptive way can further escalate the problem. Being aware of the systemic impact of disruptive behaviour is an important rst step in managing it. Whilst there are legal frameworks to help manage the acutely disruptive patient, each case needs to be considered on its merits. The Mental Health Act is not always the most relevant legal framework and its implementation does not normally enable the responsible clinician to treat a physical illness. Maximizing use of play specialists and creating a suitable environment can reduce the need for restraint and medication, which should be used with caution and only with adequate training.
Keywords ADHD; autism; behaviour; disturbed; mental health; Mental Health Act; paediatrics; restraint; schizophrenia; sedation
Introduction
When children come to hospital they should do as they are told, take their medications and get better. Unfortunately children are mostly unaware of Parsons seminal 1952 paper on the Sick Role that society (and most importantly the medical profession), say they must adopt. Even before Hippocrates time children were violating societal norms and generally making life difcult for those caring for them. Plato writes of Socrates observations; The children now love luxury. They have bad manners, contempt for authority; they show disrespect to their elders and love to chatter in places of exercise. They no longer rise when the elders enter the room. They contradict parents, chatter before company, gobble up dainties at the table, cross their legs and are tyrants over their teachers. And that was in the 5th Century BC.
Anthony Crabb BHB MBChB MRCPsych is a Consultant Child and Adolescent Psychiatrist who is the Clinical Lead for the Paediatric Liaison (CAMHS e PL) service at University Hospital Southampton, UK. His specialist interests include the assessment and management of mental health conditions as they present in, or result from, physical illness. The acute management of behavioural disturbance in the paediatric setting is one of his key roles within the Paediatric Department and he has given presentations at National Conferences on this topic.
The spectrum of difcult or disruptive behaviours that challenge both staff and parents on a paediatric ward ranges from lack of communication, through to deance and outright aggression, sometimes targeted at particular objects or people. Disruptive behaviours can include the following: Poor or unreliable history Non-compliance with treatment Increased symptom reporting Refusal of treatment or absconding Over activity, (running around the ward) Aggression and violence In this article I will focus more on aggressive and disturbed behaviour rather than non-compliance or medically unexplained symptoms that are comprehensively covered elsewhere in the literature. Behaviourists argue that all behaviours are goal directed. Having a better understanding of the goal of a particular behaviour is the key to managing it. On one level any behaviour is driven by the need to reduce anxiety (a homeostasis of calm). If we are anxious about heights, we will climb out of the tree for example. If we are anxious about paying bills we will seek work (or if in the banking industry lie, cheat and steal!) Children are aware of their anxiety, but less able to understand how to reduce it, so can adopt very primitive strategies to do so. They are prone to adopt maladaptive behavioural strategies to reduce anxiety e such as avoidance, regression to younger emotional states, and aggression. If children cannot internally reduce their anxiety they will seek external measures to do so. For some children, disruptive behaviour has the goal of reducing anxiety, and mobilizing those around them to provide for their needs, both emotional and practical. The hospital setting can be an overwhelming place for young people when they are feeling at their most vulnerable. Pain will exacerbate anxiety and diminish resilience, so it is not surprising that those that rely on aggression as a coping strategy will act out whilst in hospital. Anxiety is also generated in parents, who under normal circumstances should be the primary container for their childs anxiety, modelling coping behaviours. However in a hospital setting they may have their plate full containing their own anxiety about the health of their child, so may be expecting medical and healthcare staff to take on this role. James Robertsons 1952 video A Two Year Old Goes to Hospital is harrowing watching. Filmed at a time when parents werent allowed on to paediatric wards, it powerfully demonstrates the distress in a child in a hospital setting without parental support. Supporting parents and acknowledging their own anxiety can be a simple but effective measure to reduce acutely disturbed behaviour. Parents are an important part of managing difcult behaviour, even if they are seen as part of the cause by staff!
PAEDIATRICS AND CHILD HEALTH 23:1
2012 Elsevier Ltd. All rights reserved.
Anxiety in the child can become systemic very quickly. Behaviourally challenging or aggressive paediatric patients generate anxiety within the hospital system. Anxiety is generated about treatment, or lack thereof, and safety, the clinicians own and that of others, particularly other vulnerable patients. Children can have little concept of their potential impact on others, (for example displacing a catheter by knocking into a neighbouring patient). Highlighting this to the young person and parents may serve only to increase their anxiety and worsen the behaviour. Parents can then feel both blamed and guilty, therefore limiting their capacity to be the container for their childs anxiety-driven disruptive behaviour. In staff, anxiety manifests as certainty (being certain reduces anxiety) so if anxiety is generated in a system then carers get more certain of what should happen e this can lead to internal team conict (nursing/medical staff) and between staff and parents. What started with an anxious child is now a system of anxiety including parents, nursing and medical staff. The disturbed behaviour has now escalated........ For staff it is important to remember Samuel Shems Third Rule of the House (in his novel the House of God). At a cardiac arrest, the rst procedure is to take your own pulse. By this I mean that acutely disruptive behaviour raises anxiety in staff and it is important to acknowledge it specically. It is something that we are not taught to manage, but will be expected to deal with. By being aware of our own emotional response to the situation, unhelpful or escalating strategies can be avoided, for example seeking a dened cause for the behavioural disturbance, when the cause of the behavioural disturbance is likely to be multifactorial. He is behaving like this because of X (insert the drugs, the parents, pain, etc) therefore reduces ones own anxiety about the situation by being certain of the causal factor, but greatly limits the thinking required to effectively manage the situation.
tends to evoke ambivalent responses in carers. ... adoption of the sick role is permissible only when the disability is considered genuine and the patient cooperates in efforts towards a return to health.
Environment
The environment can play a big part in whether behaviour is manageable or not. Side rooms are not always available but can make a big difference in maintaining the safety of both the disturbed child and other patients. Removal of throwable objects is a priority. Giving toys to children to distract them is ne, but perhaps the large wooden or metal trucks could be left at home! At times it may be necessary to remove a bed, leaving just a mattress. It can impact on some aspects of care but may just make the environment safe enough so that required treatment/medication can be given. Medical treatment is always a balance of risks e better to be able to contain and treat in a soft room than put both patient and staff in danger in a normal clinical space.
Distraction techniques
A powerful yet simple way to divert disturbed behaviour is to engage the young person in activities that distract them from their concerns/anxiety. Most paediatric departments employ play specialists who are brilliant at this e make good use of them! Recently I was involved in the care of an 8-year-old boy with a learning disability who was acutely distressed on admission to a paediatric ward. He was requiring restraint by two burly psychiatric nurses. In walked the play specialist who started a game of Connect Four by herself. Within minutes the boy had joined her and asked to play. A much safer intervention than sedative medication or physical restraint!
Restraint
Some children will require restraint. Most parents will be familiar with having to hold their child and if appropriate they should be the rst port of call in restraining their children. If this is not possible then it should only be done by trained staff as injuries to both patient and staff can result from restraint by those that are not trained. At University Hospital Southampton we are introducing a rolling program of training so that there is a pool of staff that have had training in the restraint of children. In terms of staff planning, prolonged restraint should be avoided due to impact on staff and the young person and other means of containing behaviour (for example sedation) should be considered.
Hidden risks of disturbed behaviour

Obviously disturbed behaviour can put the safety of the children themselves, staff and other patients at risk. The behaviours can put the young persons safety at risk in obvious ways. However more subtlety, it can even put their own treatment and survival at risk. Gethin Morgan coined the term malignant alienation e a concept originally applied to psychiatric patients that are so hard to like that the resulting patient/carer dynamic leads to rejection and ultimately suicide. Whilst clearly the dynamic between paediatric patients and staff is not exactly comparable, it can be much more difcult to persevere with treatment in very difcult or disturbed children and clinicians should be explicitly aware of feelings stirred up in themselves by difcult patients and how it might impact on decision making. The desire to discharge or transfer difcult patients can be overwhelming and ultimately detrimental to their care. Darryl Watts described those most at risk of malignant alienation as follows: patients who do not improve with treatment, those who present challenging behavioural problems, and those where a clear diagnosis and/or treatment plan is lacking. Sound familiar? Watts observed that there is the risk of exceeding the limits of tolerance of the carers. In addition, abnormal care-eliciting
Medication
Medication for sedation of the aggressive, uncooperative child should always be the last resort and used only when other strategies have failed and there is an immediate risk of harm to either to the young person or others on the ward. Remember that the only immediate, completely sedative medications are anaesthetic agents which will not be discussed here. All other sedatives, even if given I.M., take at least 15e20 minutes to work. Thats a long time to hold someone down, so stafng levels and environment remain important. The aim of sedation is just that e sedation, not unconsciousness (see anaesthesia, above!). There should be local
hospital guidelines on acute sedation/rapid tranquilization of children on the ward. Generally there is a choice between benzodiazepines and anti-psychotics which are briey described below. There is also the choice between oral and I.M. injections. IV diazepam is sometimes used, but carries signicant risk of respiratory depression, and is usually practically unfeasible in a struggling child. I.M. medication will act more quickly, but will be more painful when administered. Oral medication should generally be offered rst, and if possible consent should be sought from parents or patient. If there is not time for consent to be sought the Children Act has provision for giving treatment in the childs best interests. The choice of anti-psychotic versus benzodiazepine will depend partly on medical factors of the patient and desired sedation. All those that administer rapid tranquilization should be trained in Immediate Life Support and resuscitation equipment should be available (Nice Guidelines). Short-acting benzodiazepines such as lorazepam or clonazepam can give quick and relatively safe sedation with less potential for movement side effects (EPSE) common with anti-psychotics. 1 mg of lorazepam is broadly equivalent to 10 mg of diazepam and its peak plasma concentration occurs 60e90 minutes postadministration I.M., and 2 hours given orally, so be cautious about repeat dosing. Anti-psychotics generally act more quickly but have more adverse effects, particularly EPSEs (oculogyric crisis and other acute dystonias) and the choice is generally between olanzapine, risperidone or haloperidol. All anti-psychotics can cause hypotension. Olanzapine 2.5e10 mg can be given orally or by I.M. injection. The peak plasma concentration being 15e45 minutes postadministration. Note that benzodiazepines cannot be given within an hour of I.M. olanzapine. Both haloperidol and risperidone have higher incidence of EPSE. Peak plasma for haloperidol I.M. is 20e30 minutes and orally 4 hours. So again be cautious about repeat dosing. Haloperidol carries increased risk of acute dystonias and an anti-muscirinic such as procyclidine (2.5e5 mg) can be used to mitigate this. Again, for immediate and complete sedation, ask a friendly anaesthetist about propofol.
The law
I frequently get asked if we can section a patient because they are being disruptive or aggressive. The answer is yes and no!. In the UK (except Scotland), the Mental Health Act 1983 enables clinical staff to detain and treat somebody who is suffering from a mental disorder and poses a risk either to themselves or others. It is not the intention of this article to delve into the ner points of the Mental Health Act, but here are two key points to keep in mind. The rst is that the Act requires that the young person is deemed to be suffering from a mental disorder e a refusal of treatment or aggressive behaviour alone does not constitute a mental disorder. Secondly it gives the power to detain/restrain a patient, but only to treat a mental disorder, not a physical illness. You cannot force a child to have dialysis, for instance, using the Mental Health Act. The only exception to this in terms of physical treatment is the Nasogastic feeding of anorexic patients, as the feeding is considered part of the treatment for their mental disorder. The most common use of the Mental Health Act on paediatric wards is Section 5(2) of the Act. This gives the Responsible
Medical Ofcer (RMO, in the case of a paediatric ward this would be the duty consultant paediatrician) the legal right to detain a patient in hospital for up to 72 hours. It does not confer the right to treat the patient without valid consent. The person who has to sign the form (hospital site managers will have copies of MHA documents) is either the RMO (i.e. the paediatrician) or their nominated deputy e in practice the paediatric registrar on call. The Child Psychiatrist can advise and consult, but cannot do the form for a 5(2) unless they are the patients responsible clinician. The Children Act 1989 makes clear that you can act in best interests of the child with or without consent. You can rely on parental consent if within the Zone of parental control e meaning that if it is a decision that most parents would make in the day to day care of their child. The UK has signed up to the UNCRC, the United Nations Convention of the Rights of a Child. Two core principles of the UNCRC are that the best interests of the child are a primary consideration in all actions concerning children (Article 3), and ensuring respect for the views of the child (Article 12). Keep in mind that human rights law (Human Rights Act 1988, European Court of Human Rights) now gives more weight to the views of children and young people as they develop their understanding and ability to make decisions for themselves. Therefore if you are having to restrain and medicate a child without consent, ensure that documentation is clear and that other measures have been attempted prior to this. Recent guidance on Gillick competence suggests the following: Although case-law suggests that the refusal of a Gillick competent child can be overridden by the courts or a person with parental responsibility, the recent trend in other cases relating to children has been to give greater emphasis to the autonomy of a competent child. The childs refusal is an important consideration in deciding whether the intervention should be authorized and its importance increases with the age and maturity of the child. Where the child is considered to be Gillick competent, it may be unwise to rely on the consent of a person with parental responsibility (The Legal Aspects of the Care and Treatment of Children and Young People with Mental Disorder: A Guide for Professionals, NIMHE 2009). Under the Mental Capacity Act 2005 all children under 16 are assumed to lack capacity to consent and therefore the clinician has to demonstrate that they are capacitous (Gilllick competent) to consent or refuse treatment. Those over 16 are assumed to have capacity unless demonstrated otherwise. Making a poor decision in the clinicians view does not mean they lack capacity according to the Act.
Advice on specic mental health conditions

ADHD Do not stop ADHD medication unless clinically necessary. Although this sounds obvious, this is sometimes forgotten, especially as the child or parents may not tell the admitting doctor about being on the medication unless specically asked. Impulsive, overactive and distractible children can be a real handful on paediatric wards and for a lot of children these symptoms are well controlled on methylphenidate, so try to keep them on it if possible.
Schizophrenia Young people suffering from psychosis can be very frightened by a hospital environment. They can be unpredictable and (less often) aggressive. They may develop unusual beliefs around medication and treatment. Organic psychosis such as anti-NMDA encephalitis can require intensive treatment and some patients will need restraint as well as sedation/anti-psychotics e seek advice early. Autism Young people with Autism can nd the hospital environment overwhelming and give them sensory overload. Try to reduce the level of stimulus e if you can turn down the volume of buzzers and alarms, this will be helpful. Remember the core decits of autism. Eye contact and social interaction is a struggle, they are not just being rude. Be careful about language used, they can be very literal Just pop off your shoes will be met with a black stare or look of horror! They can be sensitive to minor routine change. If the doctor has told them they will get a medication at 8 am make sure they get it at 8 am or do not specify a time, as they can be inexible and rigid and will nd it difcult to understand why they have been misinformed. A
settings and emergency departments CG 25. London: National Institute for Health and Clinical Excellence. Paediatric Formulary Committee. BNF for children 2011e2012. London: BMJ Publishing Group, RPS Publishing, and RCPCH Publications, 2011. Roberston James. Film e a two year old goes to hospital. National Archives, 1952. Strous Rael D, Ulman Anne-Marie, Kotler Moshe. The hateful patient revisited: relevance for 21st century medicine. Eur J Intern Med 2006; 17: 387e93. The legal aspects of the care and treatment of children and young people with mental disorder: a guide for professionals. NIMHE, 2009. Watts D, Morgan G. Malignant alienation. Dangers for patients who are hard to like. Br J Psychiatry 1994; 164: 11e5.
Practice points
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FURTHER READING Bingley L, Leonard J, Hensman S, Lask B, Wolff O. Comprehensive management of children on a paediatric ward: a family approach. Arch Dis Child 1980; 55: 555e61. National Institute for Clinical Excellence (2005). Violence e the short term management of disturbed/violent behaviour in psychiatric in-patient
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Be aware of systemic impact of disturbed behaviour on parents and staff exacerbating the problem Maximize the environment and use of available staff skills, especially play specialists Address specic mental health difculties Plan ahead e restraint train some staff Sedation e it is a last resort, use with caution Understand legal frameworks Advocate for on-site paediatric CAMHS liaison/paediatric psychology teams
Diagnosing autism
Kirsty Yates Ann Le Couteur
Whats new?
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Abstract
Awareness of autism within public and medical domains has continued to increase. Children and young people are being presented for earlier advice, assessment and diagnosis resulting in increasing demand on services. Recently published standards in the recognition, referral and diagnosis of autism mean it remains essential for paediatricians, child and adolescent psychiatrists, general practitioners and allied professionals with a role within developmental child health and child and adolescent mental health to have sound knowledge of the presentation and assessment of autism spectrum disorders (ASDs). Whilst routes of entry for referrals can vary due to diversity of presentation and local service provision, access to a local multidisciplinary team specialized in ASD assessment is recommended. Early identication of deviation away from the typical developmental trajectory in this group of patients is advantageous in order to maximize their potential, provide targeted intervention and minimize co-morbidities. This review addresses the diagnosis of ASD and provides an assessment framework for professionals who encounter a child with suspected autism.
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NICE guidance on the recognition, referral and diagnosis of children and young people on the autism spectrum was published in September 2011. Assessment of children and young people with suspected ASD should be a timely multidisciplinary process with local pathways providing access to a specialist ASD team. New evidence on the recurrence rate for ASD in siblings is higher than previously reported with rates of 18.7%. Changes in the DSM International diagnostic criteria are expected in 2013 with publication of the DSM 5. ICD-11 is likely to be 2015. For DSM the only diagnostic category will be ASD. There is a new emphasis on transition planning to young adulthood, supported by the SEN Green Paper, Autism Act 2009 and Statutory Guidance 2010. Therapies for children with ASD 2012 Effective Health Care Programme.
Keywords assessment; autism; autism spectrum disorder; diagnosis;

pervasive developmental disorder
qualitative behavioural abnormalities in communication, reciprocal social interaction together with interests and activities that are repetitive, restricted and stereotyped. The new DSM classication will have two symptom domains; Social Communication and Restrictive, repetitive behaviours including sensory behaviours. It will also consider the severity of symptoms and level of impairment. These abnormalities are pervasive, usually present in early childhood and likely to be seen across different settings.
Epidemiology What is autism?

Autism is a lifelong neurodevelopmental disorder, currently classied under the umbrella of conditions known as pervasive developmental disorders. The term autism refers to the prototypical condition described in 1943 by Leo Kanner, also known as core autism. However, it is well recognized that there is a spectrum of the condition and a broader autism phenotype with less severe and more subtle behavioural features that may only manifest after a change in environmental demand. The term autism spectrum disorder (ASD) is currently used synonymously both in lay and medical terms with autism, Asperger syndrome, and pervasive developmental disorders not otherwise specied (PDD-NOS). However, new revisions in international diagnostic criteria in DSM V (and ICD-11), expected in 2013 (2015), will have ASD as the only diagnostic category. ASD is characterized by ASD is not rare. The National Institute of Health and Clinical Excellence (NICE) states the diagnosis is queried in approximately 3% of the population and epidemiological studies now suggest minimum prevalence rates of one in 100 for ASD. This is likely at least in part to the broadening of diagnostic criteria and inclusion of cases previously classied differently, along with improved case recognition. There are no high quality robust studies conrming a rise in the true prevalence of ASD. The condition is three to four times more common in boys, with a male preponderance rising in the high functioning group. Recent NICE guidelines recognize that in clinical practice girls may well be under diagnosed.
What causes autism spectrum disorders?

ASD is accepted to be a neurodevelopmental condition with a biological basis. The heterogeneity of affected individuals and genetic complexity has undoubtedly contributed to the daunting task of identifying the cause(s) of ASD. Continuing research has not identied a clear aetiology but evidence suggests complex genetic basis with strong heritability. Taking into account twin studies and the population base rate for ASD, genetic heritability of core autism is approximately 90%. Recurrence rates of up to 18.7% for ASD have been recently reported in a prospective study with a relative risk of 2.8 if the sibling is male. This is much higher than the 3e10% estimates previously reported. Advances in molecular genetics have
Kirsty Yates MBBS MRCPCH is a Consultant Paediatrician, Chester Le Street Community Hospital, Durham, UK. Ann Le Couteur BSc MBBS MRCPsych FRCPsych FRCPCH is Professor of Child and Adolescent Psychiatry, Institute of Health and Society, Newcastle University, Sir James Spence Institute, Royal Victoria Inrmary, Queen Victoria Road, Newcastle upon Tyne, UK. Conict of interest: ALC is one of the authors of the ADI-R and a member of the Clinical Guideline Group for the ASD NICE guidelines.
identied genetic variations e.g. copy number variants and single gene polymorphisms in up to 10% of people diagnosed with ASD. De-Novo events maybe implicated in simplex families whereas multiplex families (when more than one family member is affected by ASD) may pass a specic genetic variation through the generations which increases the risk of ASD. It is possible that several genes of small effect may act through an epigenetic mechanism and environmental factors inuence phenotypic expression. In less than 10% of cases ASD is associated with a known medical condition, but mechanisms relating to causality are not well understood. Consistently recognized genetic conditions include tuberous sclerosis (TS) and fragile X. Studies have shown that between 1% and 3% of children with autism have TS and similar percentages have fragile X. Other associations and a list of additional medical risk factors are shown in Table 1. Clinical research has demonstrated differences in trajectories of head growth in children with ASD. Macrocephaly is a recognized feature of ASD in 20e30% of cases. Studies have shown that as a group, head circumference accelerates during the rst 2 years of life, with deceleration possibly occurring in later childhood since average head circumference has been reported in adolescence and adulthood. Although there have been conicting views around the relevance and cause of these changes, they are reported to happen prior to the onset of clinical symptoms and therefore may be a useful clinical indicator. Research continues to study neurobiological differences in ASD considering variation in neurotransmitters, volumetric and functioning differences of various regions within the brain, but further studies are needed to ascertain relevance for clinical practice. Various environmental factors have been reported in the literature. Prenatal inuences, such as maternal valproate use and congenital rubella are accepted risk factors. There was much controversy following links between the MMR vaccine and a so-
called regressive type of ASD. Evidence has consistently stated there is no causal link between the two, and that the hypothesis of the vaccine causing a gut disorder leading to ASD is unfounded.
Making a diagnosis
ASD is a heterogeneous condition with no single pathognomonic feature or specic diagnostic test. Diagnosis can be challenging as affected individuals can not only display variation in the degree of behavioural severity, language and intellectual abilities across the diagnostic domains, but their behavioural proles can change with age. Nevertheless, for a diagnosis of autism abnormal or impaired development should be present by the age of 3 years using existing published international guidelines (DSMIV-TR; ICD-10). Whilst many parents express concerns as early as 15e18 months of age, average age at diagnosis is 4e5 years, possibly due to a combination of factors that include lack of recognition of subtle difculties at a young age, variability of assessment pathways and broadening of the spectrum to include higher functioning individuals. Studies have shown that diagnosis of ASD at 2 years of age is possible and stable over time, although it is less reliable for the broader spectrum.
Symptoms and signs of autism spectrum disorders

Reciprocal social interaction Difculties and delay in social interaction are often the earliest features in ASDs, but they can be subtle and easily missed. Absence of joint attention (i.e. failure to show interest, share a focus of attention and follow gaze) is highly suggestive of ASD. Carers may describe that the child fails to respond to their name when called repeatedly, raising the possibility of a hearing impairment. Inadequate facial expressions, including lack of social smiling and limited use of gestures, e.g. shaking head, nodding, waving, clapping, are also features. Individuals with ASD lack awareness of others feelings and the impact of their behaviour on others. Sometimes this manifests as inappropriate behaviour in a specic social context or inappropriate response to others emotions. There can be misinterpretation of tone of voice and facial expressions of others, leading to difculties with peers, often combined with the failure to develop mutual sharing of interests, activities and emotions. Younger children may not seek to share enjoyment, e.g. showing a toy to a parent or pointing out objects of interest to others. Conversely, higher functioning individuals often seek interaction with others and make attempts to socialize but come across as socially odd. Often, social play is limited and in isolation to their peers. Communication Concerns may be raised when a child has failed to acquire language as expected. Some children with ASD may develop no useful communicative speech or sounds. In contrast to those with specic language disorders, children with ASD often fail to use gestures or mime to compensate. Instead, parents may describe the child either obtaining a required object themselves or taking another persons hand to the object as if to use them as a tool. Language is often atypical with idiosyncratic use of words or phrases, e.g. nonsense or jargon words, or referral to self as you (pronominal reversal). Other features include delayed echolalia and abnormal delivery of speech (prosody), i.e.
Risk factors for autism spectrum disorder

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Sibling with ASD Parental schizophrenia-like-psychosis or affective disorder Maternal sodium valproate use during pregnancy Gestational age less than 35 weeks Intellectual disability Birth defects associated with central nervous system including cerebral palsy Down syndrome Fragile X Tuberous sclerosis
Other medical conditions associated with ASD C Neurobromatosis C Phenylketonuria (untreated) C Fetal alcohol syndrome C SmitheLemlieOpitz syndrome C CHARGE syndrome C Duchenne muscular dystrophy C Congenital rubella C Iron-deciency anaemia Table 1
unusual pitch, speed, volume or tone. For example, children may speak in a monotonous or sing-song voice or use stereotyped phrases (with constant form or pattern). Delayed echolalia is the term applied to copied or directly imitated speech, e.g. from an adult (such as relative or teacher), television or radio, that is repeated some time after it is originally heard. Whatever the language skills present, two-way reciprocal conversational interchanges tend to be difcult, particularly if the topic of conversation is restricted to the narrowed/circumscribed and repetitive interest of the affected individual. An individual with ASD often struggles to engage in social chat and build on conversation about someone elses hobby or interest. Interests and activities In addition to limitations in make believe and pretend play, interests and activities in individuals with ASD are often restrictive and repetitive. A child may have a preoccupation with an interest that is abnormal in intensity, content or both, e.g. shiny objects, trafc lights or a non-functional aspect of a toy such as the noise or vibration it generates. For many individuals, play may lack creativity and imagination, but isolated examples of pretend play and imitative behaviour do not exclude a diagnosis of ASD. Change in routines or environment are often resisted, not uncommonly resulting in distress and/or temper tantrums. Repetitive Behaviours are common in young children and are part of normal development. However for individuals with ASD excessive rates of repetitive behaviours can cause signicant social impairment, interfere with learning new skills and contribute to levels of parental stress. Stereotypies or mannerisms are commonly seen and include hand apping, nger icking, rocking, head banging and twirling. Sensorimotor interests may also feature in the presentation. Hypo- or hyper-sensitivity to environmental stimuli is often recognized, e.g. loud noises, insensitivity to pain, or fascination with smells, textures or colours of food or fabrics. Some individuals with ASD have superior or special splinter skills/abilities in one or more areas of functioning e.g. calculations, memory, music, artistic endeavours. Regression Regression or a period of stasis occurs in 20e30% of cases. Regression most commonly affects language, usually at the less than 10 word stage, therefore often reported from 18 to 24 months of age. Motor development is preserved but other skills can be affected and parents may concurrently report a change in sleeping or eating habits, loss of eye contact and development of a specic interest. Signs and symptoms of ASD with regression in social communication skills in a child under 3 years is strongly associated with a diagnosis of ASD although the aetiology is not understood. Regression can occur in children with ASD above 24 months, but pre-existing development is usually atypical. Autistic regression in children over 3 years or regression in motor domains warrant careful assessment by a paediatrician or paediatric neurologist to consider neurodegenerative conditions, such as Rett syndrome and Landau Kleffner. Learning disabilities Historically, autism was mainly recognized in individuals with severe impairment and learning disabilities. Estimates suggested learning disabilities (IQ less than 70) affected 70e80% of individuals with autism, although with widening of the spectrum to
include higher functioning individuals, gures of approximately 50% have been quoted. Many individuals may show an unusual cognitive prole with signicant discrepancies between verbal and non-verbal scores (in either direction). However, it is important to note that for individuals with higher scores in either verbal or non-verbal abilities this may not reect their social skills which may be signicantly impaired. Epilepsy The risk of epilepsy in ASD is increased compared with the general population and linked to lower IQ and regression, with peaks of incidence occurring at pre-school age and adolescence. Between 18% and 29% of children with ASD are affected and any seizure type can occur. Epileptiform EEGs are common in ASD, and studies have shown that 10% of children with ASD have an epileptiform EEG without any clinical evidence of seizures. There is no evidence that these discharges have a causal relationship to ASD or that routine EEGs should be performed. Any investigation and treatment should be guided by the clinical presentation of the individual.
Psychiatric, neurodevelopmental and behavioural co-morbidities

Disturbances of behaviour, attention, activity, thought, mood and emotion are common in children with ASD. Children with ASD can have any developmental, medical and mental health condition experienced by children without ASD. Disordered sleep and food selectivity are well recognized. Problematical emotional reactions and behaviours can occur and may include selfinjurious behaviour, aggressiveness, temper tantrums and emotional lability. Co-morbidities within ASD are well recognized. Approximately 70% of individuals meet the diagnostic criteria for at least one other disorder, highlighting the importance of identifying co-morbid mental health and behavioural problems, particularly in those who are higher functioning. A list of psychiatric and neurodevelopment disorders associated with ASD is given in Table 2.
Assessment
The purpose of assessment in ASD is to make a diagnosis where applicable and guide interventions and treatment based on
Psychiatric, behavioural and neurodevelopmental co-morbidities associated with autism spectrum disorder
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Attention-decit hyperactivity disorder (ADHD) Tourette syndrome/tic disorder Dyspraxia/developmental coordination disorder (DCD) Dyslexia Obsessiveecompulsive disorder (OCD) Specic phobias Anxiety Depression/mood disorder Sleeping difculties Feeding difculties Oppositional deant disorder and conduct disorder Self-injurious behaviour
Table 2
a prole of strengths, impairments, skills and needs of the child and family. This includes identication of co-morbidities and associated developmental problems which can have signicant impact on a child with ASD and their family. Diagnosis may be difcult due to communication impairment and possible associated cognitive problems, making it hard to determine whether the features are the result of ASD, due to co-morbid conditions, environmental factors, or a combination of all three. The NICE guideline on assessment and diagnosis of ASD recommends that children and young people with suspected ASD have access to a local community multidisciplinary autism team who can provide advice and expertise in assessing and formulating an individuals prole.
Differential diagnosis of autism spectrum disorder (ASD). A differential diagnosis may also be a coexisting condition
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Global developmental delay Learning difculties Hearing problems Visual impairment Specic language disorders Selective mutism Reactive attachment disorder/maltreatment Lack of opportunity for interaction Rett syndrome (if features of regression) Epileptic encephalopathy
History
The possibility of ASD should be considered if there are concerns about the development or behaviour of a child or young person. Reference tables of symptoms and signs are given within the NICE guidance. Deviation away from the typical developmental trajectory warrants further assessment. Key features indicating that further evaluation is essential are absence of babble, gesture or pointing by 12 months, no single words by 18 months, no twoword spontaneous phrase (non-echoed) by 24 months and any loss of language or social skills at any age. Developmental concerns raised in a pre-school child will tend to generate initial referral through child health e.g. presentation with developmental delay or speech and language difculties. The entry point is more diverse in school-age children as they can present with a broader range of symptoms, e.g. behavioural difculties or co-morbidities. The diagnosis of ASD is based on developmental history and direct observation looking for behavioural features as described in NICE Clinical Guideline 128 (2011). Information about symptoms and signs and direct observations should be gathered from different settings, including home, education and social services (if applicable), noting the duration, severity and impact. The features should be pervasive but may appear different across the various settings. Interpretation should take into account the childs overall development, medical history and presence of risk factors. Absence of certain symptoms and signs does not exclude the diagnosis e.g. poor eye contact, social smiling. Likewise presence of symptoms and signs consistent with ASD may be accounted for by other factors. It is important to note that a lack of concern from the parents about early development does not imply a normal developmental history. Even though there is little published rigorous data, feeding difculties, gastrointestinal symptomatology, sleeping problems and sensory sensitivities (both increased and decreased) are recognized in children with ASD and should be sought in the history. Standardized instruments can be used to provide a useful structure but mostly they have not been designed specically for use in community assessments and are not essential for every assessment. They may enhance or facilitate diagnosis in that they can bring a broader understanding to the strengths and difculties experienced by the patient and family, but should not be used in isolation and are less reliable in the younger age group (less than 2 years). A range of standardized tools is reviewed in recently published NICE guidelines. The differential diagnosis for ASD is listed in Table 3, and should help guide further assessment.
Table 3
Examination
General examination should be performed as part of the assessment and include a full neurological examination, looking for dysmorphisms, neurocutaneous stigmata and include Woods light examination. Signs of injury, self harm and maltreatment should be recognized and appropriate action taken according to local safeguarding procedures. Observation of behaviour in different settings, e.g. home and school/nursery/play group, will allow assessment of the child in environments with varied social structures and give an impression of how the child interacts with peers and adults and how they adapt to predictable and less predictable routines. An Autism specic observational assessment such as the Autism Diagnostic Observational Schedule (ADOS) can provide useful information about social communication, repetitive behaviours and play skills during a set of standardized social situations with a professional trained to administer the tasks.
Investigations
Investigations should aim to exclude or identify medical conditions associated with ASD, but should only be performed where there are clinical indications and/or there are specic management, treatment or genetic implications. Paediatricians and Child and Adolescent Psychiatrists should be aware of the evidence base as to which investigations are appropriate, and ensure parents receive peer-reviewed and consensus driven information as to why a particular test is or is not indicated for their child. Investigative yield for most biomedical testing is generally low, quoted as between 8% and 37% depending on the population studied. Karyotype and fragile X testing should be targeted to those where there is higher yield of positive results e.g. if there is lower IQ or dysmorphic features, with any other investigations guided by clinical presentation and family history. Neuroimaging and EEGs for example should only be performed if there is a clinical indication. Similarly, evaluation of the gastrointestinal tract should be guided by clinical presentation and should follow standard evaluation as for any child. Hearing and visual impairments should be ruled out by performing appropriate assessments.
Multiagency assessment
Referral to the ASD team should be made if ASD is suspected. Children younger than 3 years with possible regression in language or social skills should be referred immediately for an ASD assessment. Entry to the local ASD pathway should be via a single point. The team should also be available to discuss any queries potential referrers may have. Assessment by the team should identify ASD, learning disabilities and co-morbidities, but also needs to be skills based, aiming to determine the patients individual and family prole of strengths and needs. Previously obtained information should be collated to prevent unnecessary referrals, reduce waiting times and speed up decision making. Diagnosis should be based on all available information to inform clinical judgement and the expertise of professionals knowledgeable about ASD. Membership of the core autism team should include a paediatrician and/or child and adolescent psychiatrist, speech and language therapist and a clinical or educational psychologist. The team should have access to allied professionals including occupational therapy, physiotherapy and paediatric neurology. Such multiagency work requires careful coordination and will undoubtedly impact on assessment timescales. NICE recommends that assessment by the ASD team is started within 3 months from initial referral. Case coordination, identication of a key worker and regular feedback to the parents is an essential part of the process at all levels of assessment.
monitored closely in this neurodevelopmentally vulnerable group. Follow-up of developmental progress, changing needs of the patient and identication of any additional medical problems are required. Although anecdotal evidence exists, current levels of scientic evidence are insufcient to support the use of biomedical interventions in the management of ASD, e.g. dietary supplementation and gluten- and casein-free diets.
Prognosis
ASDs are lifelong neurodevelopmental conditions. Behaviours and presentation vary over time with a tendency for progress in all domains, although there is huge individual variation. Determinants of outcome include severity of behaviours, cognitive abilities and useful speech. Many individuals require specic supports; some adults with higher functioning ASD may be able to live independently and obtain employment but at the present time few adults appear to achieve their full potential. This may be a reection of lack of appropriate current social support or inadequate targeted early intervention in this group. Recent studies have reported some encouraging ndings for individuals receiving early intervention focussing on skills development. It is perhaps too soon to know how these early interventions will impact on the longer term outcomes for individuals with ASD.A
Management
Management should be multidisciplinary and take in the rst instance a behavioural, developmental and educational approach to focus on social e communication, repetitive behaviours and any other needs of the child and family. The interventions planning also need to include signposting the family to appropriate local services and support. Identication of strengths and difculties should generate access to targeted skills-based interventions for the family and child, even if there is diagnostic uncertainty. Early social and communication based intervention programmes can be effective, e.g. UK EarlyBird and EarlyBird Plus programmes. Educational needs should be met with appropriate support and additional specialist resources such as speech and language therapy. Use of visual augmentation for communication such as the picture exchange system (PECS) might be appropriate for use in home and nursery/school settings. Under the Special Educational Needs green paper, joint education, health and social care plans should be in place by 2014 and extend to 25 years of age. Such new arrangements may address some of the issues faced by young people who require specic support as they transition to young adulthood. New legislation (Autism Act 2009 and Statutory Guidance 2010) should also support this process. Referral for genetic advice may be necessary to discuss implications for future or current siblings. Identication of an associated medical disorder or co-morbidity will require management for that condition by the appropriate professionals. Use of medication should not be withheld because of an underlying ASD but be considered within the intervention/management plan. This may include stimulant medication for ADHD, melatonin for sleep disorders, selective serotonin reuptake inhibitors for obsessiveecompulsive disorder or ritualistic behaviours, and risperidone for aggression and irritability. Careful monitoring is necessary and the rates of side/adverse effects needs to be
FURTHER READING 1 National Institute of Health and Clinical Excellence. Autism: recognition, referral and diagnosis of children and young people on the autism spectrum. Clinical Guideline CG 128. Available from: www.nice. org.uk; September 2011. 2 American Psychiatric Association. Diagnostic and statistical manual of mental disorders. Text revision. 4th edn. Washington, DC: American Psychiatric Association, 2000. 3 World Health Organisation. The ICD-10 classication of mental and behavioural disorders. 10th revision. Geneva: World Health Organisation, 1993. 4 American Psychiatric Association DSM-5 Development. www.dsm5.org. 5 Scottish Intercollegiate Guidelines Network. Assessment, diagnosis and clinical interventions for children and young people with autism spectrum disorders. A National Clinical Guideline. Available from: www.sign.ac.uk; July 2007. 6 Ozonoff S, Young GS, Carter A, et al. Recurrence risk for autism spectrum disorders. A baby siblings research consortium study. Pediatrics 2011; 128: a1e7, www.pediatrics.org/cgi/doi/10.1542/peds. 2010-2825. 7 Attwood T. The complete guide to Asperger syndrome. London: Jessica Kingsley, 2007. 8 Lord C, Rutter M, DiLavore PC, Rise S, Gotham K, Bishop SL. Autism diagnostic observation schedule. 2nd edn. Los Angeles, CA: Western Psychological Services, 2012. 9 Wing L. The autistic spectrum. A guide for parents and professionals. Constable & Robinson, 2003. USEFUL RESOURCES www.cafamily.org.uk, Contact a family. www.nas.org.uk, National Autistic Society. www.researchautism.net, Research autism. www.autismgenome.org, Autism Genome Project.
Practice points
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ASD is a heterogeneous lifelong neurodevelopmental condition with behavioural difculties affecting social communication and restricted/stereotyped patterns of behaviour, activities and interests Aetiology of ASD is unknown, although it is highly heritable with environmental factors possibly playing a role in phenotypic presentation. Diagnosis can be made accurately in the pre-school period Early recognition of the condition is likely to have a positive impact on outcome Co-morbid conditions are common and should be recognized and managed appropriately Yield from medical investigations is low and should be guided by clinical presentation and features of the individual patient
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Behavioural eating disorders

Dasha Nicholls Caro Grindrod
Abstract
Eating disorders are serious mental health disorders characterized by morbid preoccupation with weight and shape, manifest through distorted or chaotic eating. Planned changes to diagnostic criteria will broaden the denition of feeding and eating disorders to include presentations characterized by restricted food intake not associated with weight and shape concerns. Anorexia nervosa (AN), bulimia nervosa (BN) and partial syndromes are relatively common, and early intervention is advisable. Aetiology is multifactorial, with high heritability. Prognosis overall is good but treatment can be long and intensive, signicantly impacting families. Essential aspects of management are an integrated multidisciplinary approach, working collaboratively with families and young people when possible. Psychological interventions focus on the eating disorder, supported by medical monitoring and dietetic guidance. Although working with families is the backbone of treatment for AN, young people also need opportunities for condential discussion. The role of inpatient treatment is evolving. For BN, family or individual approaches may be equally effective. Paediatric expertise is of particular value in the assessment and management of acute malnutrition and complications secondary to disordered eating behaviours, in the early stages of refeeding, and in the monitoring and management of long-term complications such as growth retardation, pubertal delay and osteopenia.
Keywords adolescent; anorexia nervosa; bulimia nervosa; child; eating

disorders
psychological development, and need specic treatment. Feeding disorders are more common in children with medical or developmental problems, such as signicant prematurity, but can occur separately. Problems currently classied as feeding disorders are seen across the age range, although the term is usually used to describe eating problems in younger children or where feeding skills have never developed. Debate about the classication of feeding and eating disorders has intensied in the process of revising the two major classication systems for mental disorders, the Diagnostic and Statistical Manual for Mental (DSM) Disorder and the International Classication of Diseases (ICD). The main challenge for the DSM 5 revisions have been addressing the increasing evidence that the majority of those presenting with clinically signicant eating disorders do not full diagnostic criteria for AN or BN, and are therefore classied as have an eating disorder not otherwise specied (EDNOS). Proposed changes to the diagnostic criteria therefore aim to broaden the denition of AN and BN, and to identify Binge Eating Disorder as a separate diagnosis (it is currently subsumed with EDNOS). Additional changes in DSM 5 particularly relevant to the child and adolescent age group is the proposal to group feeding and eating disorders together and to reframe feeding problems as food intake disorders, thus removing age-related criteria (currently feeding disorder requires onset before age 6 years). The new diagnosis of Avoidant Restrictive Food Intake Disorder will apply to patients currently diagnosed with feeding disorder of infancy and early childhood. Proposed criteria are in Table 3. In addition to recognition and diagnosis, the main areas where paediatric expertize is vital are in the management of malnutrition and other acute medical complications, and of long-term complications such as the impact on growth, development and bone density. For a review see Nicholls et al. (2011).
Epidemiology Introduction
Anorexia nervosa (AN) and bulimia nervosa (BN) are characterized by morbid preoccupation with weight and shape, manifest through distorted or chaotic eating (Tables 1 and 2). This behaviour differentiates these disorders from other psychological problems associated with abnormal eating, including feeding disorders, although consensus on terms for these presentations is lacking. Feeding problems are reported by a signicant proportion of parents at some point during the early years, and many resolve with no effects on development. When severe, feeding disorders can affect nutrition and growth, as well as social and AN occurs in children as young as 7 years, while BN tends to occur later, from around 12 years of age, and rarely before puberty. The prevalence of AN is around 0.3e0.5%, with a peak age of onset between 15 and 18 years of age, although cases are seen to steadily increase from 10 years of age. Higher prevalence rates are found in specic high-risk populations, such as athletes, models and ballet dancers. Within the Western world, eating disorders are seen regardless of class, culture and ethnic group. The prevalence of BN is slightly higher than AN, at just under 1%, with a slightly later mean age of onset, but BN is much less likely to come to clinical attention. Although these rates may seem low in terms of those presenting with full syndrome disorders, the prevalence of EDNOS is over three times that of full syndrome AN or BN combined and up to 12% of adolescents show clinically signicant disordered eating and are at risk of developing full syndrome eating disorders. The prevalence of AN rose up to the 1970s, possibly due to increased recognition, but since then has remained relatively stable, while the prevalence of BN has been more subject to uctuation. It is a matter of debate whether eating disorders are becoming more common in children and adolescents, although there is some suggestion of this. More cases may be presenting for treatment, due to public and professional awareness and
Dasha Nicholls MBBS MRCPsych MD is a Consultant in Child and Adolescent Psychiatry and Honorary Senior Lecturer, Feeding and Eating Disorders Service, Department of Child and Adolescent Mental Health, Great Ormond Street Hospital for Children NHS Trust, London, UK. Caro Grindrod BSc (Hons) RN (Child) PG Dip (Systemic Theory) is a Clinical Nurse Specialist, Feeding and Eating Disorders Service, Department of Child and Adolescent Mental Health, Great Ormond Street Hospital for Children NHS Trust, London, UK.
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Diagnostic features of anorexia nervosa (adapted from DSM-IV and ICD-10 criteria)
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Proposed criteria for avoidant/restrictive food intake disorder (ARFID) in DSM 5

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Weight lost or maintained at less than 85% of expected weight for height and age, or failure to make weight gain during a growth period Fear of gaining weight or becoming fat, even though underweight Disturbance in the way ones body weight and shape is experienced (body image distortion), undue inuence of body weight or shape on self-evaluation, or denial of the seriousness of low body weight Amenorrhoea, i.e. absence of at least three consecutive cycles or delayed pubertal developmenta Weight loss is achieved by restriction of food intake and specic avoidance of fattening foods and one or more of the following: self-induced vomiting, self-induced purging, excessive exercise, use of appetite suppressants and/or diuretics If bingeing or purging behaviours are absent, this is known as restrictive anorexia nervosa; if present, as binge purge anorexia
DSM 5 revisions propose removal of the amenorrhoea criterion.
Table 1
better service provision, leading to increased healthcare expenditure. Even so, there is often a delay in seeking help and many do not reach mental health services at all, particularly those with partial syndromes where weight loss is not evident. This may account for some of the discrepancy between clinical and epidemiological rates, and has led to recent drives to focus on recognition and early intervention. Once an eating disorder is established, longer treatment may be needed and the illness risks becoming entrenched. There is a marked increase in female-to-male ratio following puberty, leading to an overall ratio of around 11:1. For BN the
Eating or feeding disturbance (including but not limited to apparent lack of interest in eating or food; avoidance based on the sensory characteristics of food; or concern about aversive consequences of eating) as manifested by persistent failure to meet appropriate nutritional and/or energy needs associated with one or more of the following: Signicant weight loss (or failure to gain weight or faltering growth in children) Signicant nutritional deciency Dependence on enteral feeding or nutritional supplements Marked interference with psychosocial functioning There is no evidence that lack of available food or an associated culturally sanctioned practice is sufcient to account for the disorder The eating disturbance does not occur exclusively during the course of Anorexia Nervosa or Bulimia Nervosa, and there is no evidence of a disturbance in the way in which ones body weight or shape is experienced The eating disturbance is not better accounted for by a concurrent medical condition or another mental disorder. When occurring in the context of another condition or disorder, the severity of the eating disturbance exceeds that routinely associated with the condition or disorder and warrants additional clinical attention
Table 3
ratio is around 30:1. Female gender is the strongest risk factor for eating disorders, but this bias can lead to under recognition in boys. Presentation in boys is clinically similar to that of girls, excepting a tendency to be concerned about shape rather than weight. What is, and what is not, classied an eating disorder may account for some of cultural and gender variation seen in the prevalence of eating disorders. Clinician vigilance is needed to ensure atypical cases are not missed.
Diagnostic features of bulimia nervosa (adapted from DSM-IV and ICD-10 criteria)
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Pathology and pathogenesis

Biological, psychological and sociocultural factors all have a role in the aetiology of eating disorders, which are often thought of in terms of predisposing factors (risks), precipitating factors (triggers) and perpetuating (maintaining) factors. Such a formulation is useful as a working hypothesis for treatment interventions; the results of treatment will prove or disprove the hypothesis. Having some understanding of how the eating disorder has come about in the young persons life will likely be helpful to them and their parents. However, focussing on maintaining factors and ways of overcoming these may be most useful in treatment and recovery. For example, if an episode of weight-related teasing is identied as a trigger, addressing the bullying will not in itself address the eating disorder. By contrast, if perfectionism or athleticism are predisposing and maintaining risk factors, these may need to be addressed during the recovery process. The importance of heritance in the development of eating disorders comes from family studies, twin studies and adoption
Persistent preoccupation with eating and recurrent episodes (over a period of months) of binge eating, which are characterized by: eating a large amount of food in a short period of time, a sense of lack of control while eating Attempts to counteract the fattening effects of food by use of compensatory behaviours such as: self-induced vomiting, purgative abuse, alternating periods of starvation or excessive exercise, use of drugs such as appetite suppressants, diuretics, thyroid preparations or, in diabetics, misuse of insulin Psychopathology consisting of a morbid dread of fatness and setting of a target weight way below what might be considered healthy Bulimia nervosa may follow on from a period of anorexia nervosa, but would only be diagnosed if the patient is no longer signicantly underweight
Table 2
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studies, with heritability estimates around 60e75% for AN and 30e80% for BN. There is also considerable evidence for genetic contributions to individual symptoms, attitudes and behaviours, such as self-induced vomiting, or perfectionism traits, which increase risk within individuals. Puberty may also activate some aspects of genetic heritability. Understanding of the aetiology of eating disorders has been subject to denite fashions. Recently, there has been increased interest in the neurobiological aspects, thanks largely to advances in neuroimaging, and molecular genetics. This is not to devalue sociocultural theories, but rather to understand better why, within one family, one member but not another is affected. Over 30 risk factors have been identied, suggesting that none is either necessary or sufcient on its own. Conceptualizing eating disorders as brain disorders can be helpful in reducing the stigma and trivialization associated with eating disorders. However it is also clear that social and cultural factors play a role in the development and maintenance of these disorders.
Outcome and prognosis

Overall, outcome studies reveal good outcome in adolescents with AN of between 49% and 75% after 10 or more years followup, although even with intensive treatment recovery can be slow. In adolescents in treatment for AN, Gowers et al. (2007) found that fewer than one in ve had fully recovered by 1 year after presentation, and only one-third recovered by 2 years. For patients with established illness, time to recovery is estimated at around 5 years Around 10% will go to have a severe and enduring eating disorder, for which outcome is poor, with the highest mortality and morbidity of any psychiatric disorder. There are fewer studies on the outcome of adolescent BN, although full recovery is expected in over 50% of patients. EDNOS can be just as clinically severe as AN and BN and is more likely to present with co-morbid conditions. AN precedes BN or EDNOS more often than BN precedes AN. Fluctuation in clinical features developmentally and over time has led some to question the validity of distinguishing AN from BN, but rather to take a transdiagnostic approach. Some genetic and outcome ndings, however, would suggest the distinction between restrictive and binge purge pathology is valid. Once a young person has recovered from the eating disorder, secondary psychopathology may remain, most commonly depression or anxiety disorders.
Course of the disease(s)

Eating disorders are often thought to be rooted in difculties in emotional regulation and expression, the route to recovery being the acquisition of age-appropriate ways to identify and express difcult negative emotions, especially anxiety and anger or protest. Zucker further suggests that it is the way that young people with AN attempt to regulate emotion that has a detrimental effect on their self-awareness and their abilities to relate inter-personally. Eating disorders in children and adolescents present with varying levels of severity, from mild with short illness duration to long-standing chronic conditions that have detrimental effects on many aspects of a young persons life and health, continuing into adulthood. At present, a clear approach to interventions based on severity at presentation is lacking, since it can be quite difcult to predict prognosis at the point of presentation. Maguire et al. (2012) have been developing tools to stage anorexia at presentation with a view to better predicting the course of illness for an individual. As yet this has not been applied to children and adolescents, where systemic factors may prove as important to outcome as the characteristics of the presentation itself. Lask describes three stages of recovery during the course of early-onset AN (Lask, 2000). In Stage 1 the eating disorder is the predominant feature, characterized by preoccupation with food, and weight and shape concerns. At this stage denial is common. Stage 2 brings increased assertiveness, the young person expressing powerful, negative feelings, often directed towards parents or professionals. It is important to warn parents to expect this stage, which can last around 6 months, and to herald it as part of recovery. Thereafter, more age-appropriate expression of feelings becomes apparent (Stage 3). This description of the recovery process ts neatly with the three stages of treatment described in the empirically supported family based treatment that would usually be the rst line treatment. BN tends to have a more chronic and uctuating course. Identication is often delayed, the nature of the disorder being easier to conceal than AN. Often conceptualized as a coping strategy at difcult times, and highly mood related, it is often likened to addictive disorders.
Diagnosis
Diagnosis relies on good clinical interviewing skills, the presence of eating disorder psychopathology being ultimately identied through subjective report by the young person, interviewed alone. However, in young people, parental account may provide important information about disordered eating behaviours that may not be disclosed in interview with the young person. Ageappropriate semi-structured interviews are the gold standard for eliciting eating disorder psychopathology, although interview by an experienced clinician is adequate. When there is determined food avoidance in the absence of specic eating disorder or other psychopathology, the terms food avoidance emotional disorder or non-fat phobic anorexia has been used, although these are not formal diagnostic terms. If the diagnosis remains uncertain following a thorough clinical assessment by an appropriately trained clinician, starting to increase food intake often claries the diagnosis. If fear of weight gain is present, active weight loss behaviours will ensue. Similarly, a fear of swallowing will become more obvious if certain foods are avoided or only liquids can be managed. Some simple questions are useful to ask as an initial screen for an eating disorder, for example e do you think you have an eating problem? or do you worry excessively about your weight?. The history is best taken with the whole family together, informing them at the start of the need for individual time with the young person, and a physical examination. Young people should be given a choice about how and when the physical assessment is undertaken. Assessment should be regarded as the rst step in treatment and an important opportunity to engage and motivate the young person and family. This translates to avoidance of challenge and confrontation, but to listening and the provision of clear information and guidance. Young people
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repeatedly say that being listened to and talked to with respect is the most important aspect of the assessment. Listening to their parents account of the eating difculties and the context in which they arose can be helpful to young people, even if they cannot directly contribute to the story e it can help them understand the factors that may have been beyond their control. Asking about current eating patterns and a typical days intake, as well as specic questions about compensatory behaviours is necessary for assessment of risk. Current intake gives important information regarding risk of nutritional deciencies, and is important in establishing how re-feeding, when needed, should be tailored safely. Onset is often slow and insidious, with acute deterioration once it comes to light. Children often stop drinking as well as eating. A full history also addresses family risk and protective factors, such as history of mental disorder, and the role of the extended family. Marital relationships are only of relevance in relation to parents capacity to work together in the interests of their child. Family attitudes and beliefs about food, weight and shape inuence the way that eating disorders are addressed within the family context and may therefore affect prognosis. Social context (housing, employment and nancial situation) and practical considerations are important for treatment planning. A developmental history should include feeding and early attachment, and premorbid personality including perfectionism, peer relations, obsessional traits, separation anxiety, autism spectrum disorder traits and self-esteem. Eating disorder psychopathology, mood and psychological risk (e.g. suicidal ideas or self-harm) are best assessed individually. Ask about eating patterns and current intake, dietary restrictions and rules (such as set calorie limits, eating at certain times), compensatory behaviours (purging, laxatives, exercise) and binge eating. Beliefs about weight and shape, preoccupation with weight and shape, concerns about eating, fear of weight gain, self-evaluation with respect to weight shape or eating and motivation to change are all key to making a diagnosis and treatment plan.
Adequate time should be allowed for feedback and decisionmaking at the end of the rst assessment, given the, often appropriate, anxiety about the need for change.
Physical assessment (Table 4)

AN carries considerable serious physical risks and needs careful monitoring. Early, robust intervention is vital, in order to prevent or reverse signicant physical complications. In BN, physical problems are caused by frequent vomiting and potential excessive use of laxatives. Some of the complications of AN and BN are due to lack of energy, some to metabolic disturbance and some to endocrine disturbance (of hypothalamic origin) (Table 5). Some are potentially life-threatening, whilst others are associated with long-term compromise of health. The Junior MARISPAN guidelines (Management of really Sick Patients under 18 with Anorexia Nervosa: http://www.rcpsych.ac.uk/les/pdfversion/ CR168.pdf) provide a risk assessment framework as a basis for determining medical risk and immediate management. Complications that are unique to younger patients, due to the dynamic nature of growth and development, are growth retardation, pubertal delay or arrest, and reduction of peak bone mass. An atypical picture needs an open mind and a thorough medical review. Common differential diagnoses include gastrointestinal disease such as Crohns disease, chronic disease affecting appetite and growth such as renal failure, endocrine disorders, intracranial pathology and other psychiatric disorders such as obsessiveecompulsive disorder and depression. Nutritional assessment should consider the past, the present and the future: duration of low weight, rapidity of weight loss, menarcheal status, body mass index (BMI) centile (or % median BMI), haemodynamic stability and future predicted intake (more commonly over- than under-estimated). Fluid intake may be restricted (to lose weight) or excessive (to increase weight temporarily). Rapid weight loss (more than 1 kg/week) can cause medical instability even if the child is not underweight. Muscle weakness and peripheral neuropathy are signs of serious
Physical assessment
What to look for on physical examination
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When to worry
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Weight, height and BMI centiles (or % median BMI if below 2nd centile) falling or below 9th BMI centile Bradycardia and orthostatic changes in pulse or blood pressure, based upon age-appropriate norms Hypothermia Dull, thinning hair Sunken cheeks, sallow skin/skin integrity Lanugo hair Delayed pubertal development for age/atrophic breasts Pitting oedema in peripheries Cold extremities/acrocyanosis/weak peripheral pulses Dehydration (skin turgor, mouth, tongue) Muscle wasting Signs of bingeing/purging e.g. dental erosion, callouses on ngers Signs of vitamin deciency
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<85% BMI for age (between 2nd and 9th centile) is underweight. <70% high risk Pulse <50 (45 at night); BP <80/50; orthostatic changes in pulse (>20 bpm) or blood pressure (>10 mmHg) <35 C No signs of puberty at 13; premenarcheal at 15 Normal capillary rell <1 second If visible in older child, suggests >5% Difculty sitting up from supine, and rising from squat to standing (SUSS test) without use of hands
Table 4
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Physical complications of eating disorders

Medical complications of calorie restriction C Cardiovascular C ECG abnormalities e bradycardia; T wave inversion; ST segment depression; prolonged QT interval; dysrhythmias (SVT, VT); pericardial infusions C Gastrointestinal system C Delayed gastric emptying; slowed GI motility; constipation; bloating; fullness; hypercholesterolaemia; abnormal liver function (carotenemia) C Renal C Increased blood urea (from dehydration and reduced GFR) with increased risk of renal stones; polyuria (from abnormal ADH secretion); depletion of Na and K stores; peripheral oedema with re-feeding due to increased renal sensitivity to aldosterone C Haematology C Leucopenia; anaemia; iron deciency; thrombocytopenia C Endocrine C Sick thyroid syndrome (low T3); amenorrhoea; growth failure; osteopenia C Neurological C Cortical atrophy; seizures Medical complications of purging Fluid and electrolyte imbalance Low K; low Na; low Cl Chronic vomiting Oesophagitis; dental erosions; oesophageal tears; rarely rupture and pneumonia Use of ipecac/laxatives Myocardial damage; renal stones; low Ca; low Mg; low KCO3 Amenorrhoea
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Table 5
nutritional decit. Local protocols agreeing thresholds for paediatric admission can be helpful. Growth slows down and even stops during a period of starvation. After starvation is over, catch-up growth can occur but it is still unclear to what extent. Our best guess for the dose of starvation needed to have a permanent effect on height is 4 years before completion of growth. There are case reports of people going through puberty in their mid to late 20s, and anecdotal accounts of menarche at nearly 50. Between 25% and 40% of young people with AN will have osteopenia on bone density scan. The long-term fracture risk is around three times that of the general population. Interpretation of reduced bone density in AN in young people should consider the impact of pubertal delay and growth failure on bone size.
Management
The treatment of eating disorders in children and adolescents presents many challenges to the clinician. Young people with AN are terried at the thought of eating and weight gain, and at best ambivalent about receiving help. They may be suffering physical effects from their eating behaviours, impairing their capacity to think. Many patients do not accept that they are unwell, and are often brought to treatment by family members.
Parents often experience rst-line healthcare professionals as minimizing eating difculties on initial presentation. It is crucial that weight is monitored in a standardized way as soon as concerns arise. Eating disorders are unlikely to resolve on their own once they have reached the stage of clinical presentation, so a wait and see approach is contraindicated. Ongoing weight loss should alert concern, but parents often report changes in behaviour, such as social withdrawal, altered eating behaviours, secretiveness, and ritualized and restricted activities, long before low weight is apparent. Written information given early in treatment is important, followed by the time to answer questions and to discuss areas of concern. Professionals should recommend resources for parents and children on eating disorders in the younger population (see below). Parents and children need a clear statement about diagnosis, the likely course and possible complications of the illness, and proposed treatment. The aims of treatment are to reduce risk, encourage weight gain and healthy eating, reduce other related symptoms, and facilitate psychological and physical recovery. The evidence base for effective treatments is limited, and existing guidelines, such as the NICE guidelines (National Collaborating Centre for Mental Health, 2004), are largely based on consensus expert views. Treatment must address both physical and psychological aspects of the condition. Early intervention in a developmentally appropriate and specialized treatment setting is likely to produce the best outcome. A combination of integrated interventions offered by a multidisciplinary team is needed. In most situations, parents should be involved in treatment. Currently, the effective evidence base for the use of medication in eating disorders is weak, although studies in this patient group are difcult to do. In AN, depression often lifts with improved nutritional state. If not, antidepressants, usually selective serotonin re-uptake inhibitors (SSRIs) such a Fluoxetine, may have value. Medication should be used with caution due to the increased risk in malnutrition, for medications with metabolic or cardiovascular side effects, which must be clearly explained to the patient and family. Evidence for the effectiveness of SSRIs in BN is stronger, with high-dose Fluoxetine being the treatment of choice, although there are no studies exclusively in adolescents. Occasionally nutritional supplements and hormonal support are indicated, most often as damage limitation in chronic illness or during acute re-feeding. These should only be considered by specialists and in conjunction with the rest of the treating team. In particular, the use of hormone replacement for the treatment of impaired bone density is rarely indicated. The mainstay of treatment remains weight gain and nutritional rehabilitation. In consultation with a specialist multidisciplinary team, the use of transdermal oestrogen may be considered by way of damage limitation for severe bone depletion. Improvement in bone density is not usually seen in the rst year of treatment, but after 1e2 years. Re-feeding The aim of re-feeding is healthy weight restoration in the least invasive way. Wherever possible, re-feeding is done orally and in the home if safe. The child needs clear expectations about what they need to manage, and dietetic input can be very helpful with this. The aim for weight gain is 0.5 kg for outpatients and between 0.5 and 1 kg for inpatients. This generally requires
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between 3500 and 7000 extra calories a week. Starting with low intake but building up to full requirements within a week to 10 days is safest in order to avoid further weight loss. It is useful to acknowledge the cognitive effects of starvation, which often intensify similar cognitive aspects of AN. Starvation can increase obsessionality, perfectionism, low self-esteem; and can lead to delayed gastric emptying which may increase the desire to food restrict. Evidence suggests the most objective way to assess healthy weight for female young people, is to use baseline and follow-up pelvic ultrasound scanning. Early weight gain in treatment has been linked with better long-term outcomes.
reviews are important and all professionals involved should have clear, documented roles and responsibilities. Increasingly, more intensive methods of outpatient treatment are being sought, because of questions about the costeffectiveness of inpatient treatment. Outreach services and home based treatment teams can contribute to decreased need for inpatient admission, while intensive family approaches such as multifamily therapy are undergoing trials. In hospital The decision to admit a young person with an eating disorder is made for one of four reasons: A rapid deterioration in medical state Marked depression, suicidal ideation or intent Other major psychiatric disturbance Care may be on a paediatric ward or a child or adolescent psychiatric inpatient ward. Paediatric admission works best if close links remain with other elements of treatment and if locally agreed protocols are in place between paediatric and mental health services. It is helpful to distinguish the need for medical stabilization from re-feeding. Young people may nd it easier to eat at home, if the risks can be managed. Psychiatric admission is an altogether more serious decision, admissions usually being for 4e6 months or more. The following need to be considered prior to psychiatric admission for an eating disorder: Care should be provided in an age-appropriate setting Treatment should be provided as close to home as is possible The potential side effects of inpatient admission need to be considered, including isolation from family and increased resistance, and needs to be balanced with the educational and social needs of children and adolescents The long-term effects on the family, in terms of time commitment and emotional effects of admission A plan needs to be in place to aid transition to local outpatient services on discharge. There is a limited role for inpatient services in the care of patients with BN, except in the management of suicide risk and self-harm. Psychiatric admission usually follows failure of outpatient treatment. However, a recent randomized controlled trial found no advantage in outcome of inpatient over outpatient psychiatric care, and as such challenge the intuitive clinical belief that a step up from outpatient to inpatient psychiatric care is indicated for those who fail to make progress. Once in a healthy weight range, meal plans will need to be adapted to allow for continuing growth and the nutritional demands of puberty. Although weight gain is ultimately associated with improvement in all aspects of functioning, initially it may increase eating disordered behaviour (in an attempt to eliminate the extra weight), anxiety and distress. Adequate weight gain may reduce the risk of readmission, and the speed of re-feeding should be fast enough to avoid negative institutionalization effects but not so fast as to cause overwhelming anxiety or risk of the re-feeding syndrome. Nasogastric feeding is considered only when patients are medically compromised or are unable to gain weight with supported meals and behavioural re-feeding regimens. Consent to treatment is needed and treatment against the patients or parents wishes is always a last resort. Treatment
Psychological interventions
In the community Ideally, young people with eating disorders should be treated as outpatients, incorporating psychological and physical treatment, by healthcare professionals competent to give this treatment and in assessing physical risk. Although working with families is the backbone of child and adolescent mental health, children and adolescents should also be offered individual appointments separate from their family or carers. Psychological interventions need to focus on both the eating behaviours and the young persons thoughts about their weight and shape, alongside clear expectations for weight gain in the case of AN. Effective treatment requires a skilled multidisciplinary team who are able to work collaboratively, in a good alliance with the family. For AN, the rst-line treatment is family based treatment, weekly or more frequently at rst, supported by regular medical monitoring and dietetic input. The model for which there is the greatest evidence supports parents being in charge of their childs eating until the young person is well enough to share responsibility. Family interventions both address and focus directly on the eating disorder, with behaviour change focussed on the maintaining behaviours of AN. The ethos of this style of family therapy, is that parents are seen as a resource to the resolution of the problem, and not as a causative factor. This work can be undertaken with the family all together (conjoint family therapy) or with parents separately (parental counselling); the latter has been found to be useful when there is high expressed emotion within the family. Individual therapy has a role, especially for older adolescents who might be expected to take more responsibility, or where the young persons family is not willing or able to participate in family therapy. The evidence base for individual therapy is weaker. If obsessive ecompulsive features are marked, progress in treatment may be slower. Among treatment trials for adolescents with BN two studies merit mention. In the rst, Schmidt et al. found that guided selfcare, using a cognitive behavioural model, was equally and more rapidly effective at reducing eating disordered behaviour than family therapy, and was more cost-effective and acceptable to the young people. By contrast, Le Grange et al. found that family therapy was both more effective and more rapid than an individualbased treatment, in this case supportive psychotherapy. In practice therefore, it seems reasonable to offer choice and assess the individual circumstances of the young person and their willingness to involve, and likely support of, their parents. Alongside outpatient treatment, monitoring of growth is required, especially for premenarcheal children. Regular progress
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against consent is a highly specialized procedure requiring expertise in the care of patients with severe eating disorders. It can be done in the context of the Mental Health Act 1984 or Childrens Act 1989, which allow a young persons refusal of treatment to be over-ridden. See Tan et al. (2007) for a detailed discussion of these issues. Parental consent should not be relied on indenitely, and clinicians should ensure the legal basis for clinical action is clear. The NICE guidelines for eating disorders recommend seeking a second opinion when consent issues are highlighted. Adolescents views of treatment Young people with eating disorders say that without the motivation to get well, they struggle to make use of treatment. Young people with eating disorders value being listened to and having their views respected, even when treatment decisions need to be made against their wishes. When treatment is experienced as disempowering or punitive, they tend to reject and ght against it. Parents views What parents nd helpful includes being rm and presenting a united front, support and understanding, connecting with other parents in similar situations. Parent peer support groups are very powerful in this respect. Structured interventions and a skills manual for carers of people with an eating disorder have been developed. Parents want clinicians to include them in treatment, support and guide them in their childs care and demonstrate positive attitudes towards them. The implications for clinicians include the need for sensitivity to parents vulnerability, ensuring congruence between clinicians and parents expectations about treatment, and strengthening formal channels of communication. Around 50% of carers suffer anxiety and around 13% will have signicant depression.
Prevention approaches that target high-risk female adolescents over the age of 15 are more effective than those aimed at all children. They work best if they are interactive rather than didactic (impact that didactic (psychoeducational)). Prevention approaches for younger children may need to target parents and other systemic factors rather than the young person themselves. For example, there are concerns about the impact of antiobesity messages such as fat is bad for children with a perfectionist and literal mind. No prevention programme specically designed for parents has been reported in the literature to date to our knowledge. A
Follow-up
In determining length of follow-up, it is important not to focus solely on whether eating disorder symptoms are present, but to think more broadly about the extent to which coping strategies for the future stresses have been developed, ongoing risk and impairment, and whether the young person is equipped for developmental tasks appropriate to their age. In practice, young people usually want to leave outpatient treatment before clinicians and their parents want to discharge them. Regular reviews of progress and treatment are needed to inform changes in treatment intensity. In general, outpatient treatment for a minimum of a year after an inpatient admission is advisable. Monitoring of physical outcomes, e.g. bone density and menstruation, may need to continue beyond psychological intervention. Careful transition to adult services is needed for chronic cases.
FURTHER READING Allan R, Sharma R, Sangani B, et al. Predicting the weight gain required for recovery from anorexia nervosa with pelvic ultrasonography: an evidence-based approach. Eur Eat Disord Rev 2010; 18: 43e8. Bryant-Waugh R, Lask B. Eating disorders: a parents guide. Routledge, 2004. Bryant-Waugh R, Markham L, Kreipe RE, Walsh BT. Feeding and eating disorders in childhood. Int J Eat Disord 2010; 43: 98e111. Hudson LD, Nicholls DE, Lynn RM, Viner RM. Medical instability and growth of children and adolescents with early onset eating disorders. Arch Dis Child 2012 Sep; 97: 779e84. Epub 2012 Jun 19. Junior MARSIPAN: management of really sick patients under 18 with anorexia nervosa. "http://www.rcpsych.ac.uk/publications/ collegereports/cr/cr168.aspx". Keel PK, Brown TA. Update on course and outcome in eating disorders. Int J Eat Disord 2010; 43: 195e204. Lask B, Bryant-Waugh R, eds. Eating disorders in childhood and adolescence. Routledge, 2012. Misra M, Katzman D, Miller KK, et al. Physiologic estrogen replacement increases bone density in adolescent girls with anorexia nervosa. J Bone Miner RES 2011; 26: 2430e8. Nicholls D, Hudson L, Mahomed F. Managing anorexia nervosa. Arch Dis Child 2011; 96: 977e82. Treasure J, Russell G. The case for early intervention in anorexia nervosa: theoretical exploration of maintaining factors. Br J Psychiatry 2011; 199: 5e7. Treasure J, Smith G, Crane A. Skills-based learning for caring with a loved one with an eating disorder: the new Maudsley method. Routledge, 2007.
Practice points
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Prevention
The efcacy of prevention is not yet established, although there have been some promising inroads. Targeting mental health and self-esteem, and focussing on development of dissonance between lived experience and cultural messages seem to be more effective than targeting weight and eating behaviours.
Diagnosis should ideally not be based on either a parents or young persons report alone Eating disorders are rarely self-limiting and, when suspected, should be monitored carefully The most important skill is engagement, for which a collaborative and information sharing stance is most helpful Most patients should be treated on an outpatient basis, provided risks can be managed safely, but both physical and psychological risks can increase at rst with intervention The burden of caring for a young person with an eating disorder is enormous and adequate support for parents and siblings is essential
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Obsessiveecompulsive disorder in children and adolescents

Reenee Barton Isobel Heyman
Whats new?
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Neuroimaging studies support fronto-striatal-thalamic model Further evidence of the need for CBT to treat partial responders of OCD on medication OCD to be classied as a separate disorder from anxiety as part of the obsessiveecompulsive spectrum disorders in DSM V Trialling glutamate modulating drugs in the treatment of OCD
Abstract
Despite obsessiveecompulsive disorder (OCD) being one of the more common serious mental illnesses, it continues to be shrouded in shame and secrecy. The shame surrounding the condition, in combination with a lack of recognition of its dening symptoms, can lead to delays of several years before it is diagnosed, yet it is highly responsive to treatment once recognized. Children with OCD frequently present to nonpsychiatrists. It is important that paediatricians familiarize themselves with the characteristic symptoms. This review summarizes current research regarding the epidemiology and aetiology of OCD, the assessment of childhood-onset OCD, as well as its psychological and pharmacological management.
Keywords adolescents; assessment; children; cognitive behaviour

therapy (CBT); obsessiveecompulsive disorder (OCD); selective serotonin reuptake inhibitors (SSRI); treatment
Neuroimaging studies have identied alterations in brain fronto-striatal-thalamic circuitry and in the basal ganglia in patients with OCD. One paediatric study demonstrated a smaller globus pallidus volume in unmedicated patients with OCD compared to healthy controls. Recent research in adults suggests that the dorsal prefrontal cortical and bilateral midbrain grey matter abnormalities in OCD appear to be primarily driven by the effects of OCD symptom severity. Other studies suggest that there may be brain-markers for OCD even in the absence of symptoms; for example, a recent study demonstrates orbitofrontal brain dysfunction not only in individuals with OCD, but also in their unaffected close relatives. Furthermore, treatment with medication and/or CBT is associated with reversal of functional neuroimaging ndings. Although there is no evidence for life events as a cause of OCD in susceptible individuals, these may trigger relapse or exacerbate symptoms. This could be at the time of school transitions, exams, or relationship difculties with peers or family.
Introduction
The lifetime prevalence of OCD is 2%. It may occur in children as young as 6 or 7 years old, and a third to a half of newly diagnosed adults report onset of symptoms in childhood or adolescence. It has an equal incidence in both sexes.
Denitions
In the WHO ICD-10 Classication for Mental and Behavioural Disorders, the following must be present for the full clinical diagnosis for OCD to be made. Either obsessions or compulsions or both present on most days for a period of 2 weeks. Obsessions (unwanted ideas, images or impulses that repeatedly enter a persons mind) and compulsions (repetitive stereotyped behaviours or mental acts driven by rules that must be applied rigidly) share the following features: The child is aware that these originate from their own mind; Repetitive unpleasant and distressing to the child. At least one is perceived as excessive or unreasonable (egodystonic); At least one is resisted unsuccessfully, even though others may be present which the sufferer no longer resists; Thought of carrying out the obsession or compulsion is not intrinsically pleasurable (simple relief of tension momentarily on completion of the thought/act is not regarded as pleasure in this sense); Obsessions may not be present; a child may describe that things are just not right if they do not do the ritual;
Aetiology
The aetiology of OCD is not well understood, but there has been extensive research on environmental and genetic risk factors. Twin, family, segregation and linkage studies strongly support a genetic component for OCD. Most studies looking at candidate genes have focused on genes in the serotoninergic and dopaminergic pathways, but none has reached genome-wide signicance.
Reenee Barton BSc MBChB (Hons) MRCPCH MRCPsych is a Consultant Child and Adolescent Psychiatrist in Learning Disability, SCAN, CAMHS, St Anns Hospital, Barnet Eneld and Haringey Mental Health Trust, London, UK. Conicts of interest: none. Isobel Heyman MBBS PhD FRCPsych is a Consultant in National and Specialist Service for Tourette Syndrome, Great Ormond Street Hospital NHS Foundation Trust, London, UK; and Honorary Reader, Institute of Psychiatry, Kings College London, UK. Conicts of interest: none.
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2012 Published by Elsevier Ltd.
Younger children may not recognize obsessions as their own thoughts and may refer to them as voices. Hence differentiating this from psychosis is important; The symptoms must be disabling. Even young children will have some insight into the senselessness of the thoughts and behaviours. Typically, the child experiences an escalating vicious cycle of obsessions and compulsions. The obsessions generate signicant anxiety, and rituals develop as a way of temporarily reducing anxiety levels. After ritual completion, anxiety levels soon rise again, further fuelling the obsessions, and so the cycle continues (Figure 1). The temporary relief of anxiety with the compulsions reinforces the behaviours and prevents habituation to the anxiety. Eventually, the rituals become more automatic and increase, rather than reduce, the anxiety, so that no temporary relief is experienced. These symptoms are phenomenologically distinct from the ordinary childhood rituals that are common in early childhood. For example, bedtime rituals with a parent or routines about food, toys or clothes, but in the absence of other problems are non-distressing, do not take up excessive time or stop the child from doing other things, and are self-limiting.
Symptoms and risk factors that may alert you to obsessiveecompulsive disorder (OCD)
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Anxiety Depression Tics Sydenham chorea and rheumatic fever Recent streptococcal infection (ENT) Chapped hands, eczema from handwashing Trichotillomania Hypochondriasis: fear of cancer, fear of dying, fear of HIV Concerns about body appearance (body dysmorphic disorder) Strong family history of OCD, anxiety, tics, depression
Table 1
Assessment and diagnosis

There can be a long delay in the diagnosis of OCD due to lack of recognition or, often, because sufferers are embarrassed by their symptoms and may be unaware that they can be helped. The presence of certain symptoms (Table 1) and a simple sixquestion screen may alert the clinician to the possibility of a diagnosis of OCD (Table 2). A short screening instrument suitable for use in children is also available: the short OCD screener (SOCS). This has a sensitivity of 0.97 (95% CI 0.91e0.98) to detect OCD cases, although further assessment is required to conrm the diagnosis. If a diagnosis is suspected, then a more thorough assessment can be completed. For more complex cases it is advisable to refer the child to the local Child and Adolescent Mental Health Service (CAMHS). The child and carer(s) are invited to the assessment; it may be helpful to
Obsessions
involve siblings. Most CAMHS will typically carry out the following assessment. A detailed history of obsessions and compulsions is taken: This in part should be done with the parents giving a history, and perhaps other family members such as siblings. Engagement with the family is essential, as they are likely to be central to successful treatment. The whole family needs to understand the diagnosis and techniques for its management. It is also important to involve the family in the discussion as they can often give information about how the child involves other family members in their compulsions, and how the condition impacts on everyday life, e.g. making everyone late for school because of morning rituals, or limiting activities because of fear of contamination. A helpful technique, referred to as externalizing the problem, is to acknowledge how the condition makes the child do things, rather than saying that the child makes everyone late for school. This can enable everyone to talk about the symptoms without feeling that the child is being blamed. Quite often, the child may even have a name for their OCD. Younger children may nd it difcult to identify the underlying obsession driving a compulsion.
Temporary relief
Anxiety
Screening questions for obsessiveecompulsive disorder recommended by NICE

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Compulsions
Figure 1 The vicious cycle of OCD. Compulsions provide temporary relief from anxiety thereby reinforcing the behaviour to continue them. Cognitive behavioural therapy targets reduction of compulsions which in turn leads to reduction in obsessions.
Do you wash or clean a lot? Do you check things a lot? Is there any thought that keeps bothering you that you would like to get rid of but cannot? Do your daily activities take a long time to nish? (e.g. getting ready for school) Are you concerned about putting things in a special order or are you very upset by mess? Do these problems trouble you?
Table 2
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Conditions that commonly co-occur with obsessivee compulsive disorder

C C C C C C C
Depression Specic phobia Social phobia Eating disorder Alcohol dependence Panic disorder Tourette syndrome
During the assessment it is important to identify co-morbidity although a focus on the treatment of OCD may lead to resolution of other problems. OCD co-occurs (Table 3) and overlaps with many disorders, which are sometimes referred to as the obsessiveecompulsive spectrum disorders (Table 4). The most likely differential diagnoses are other conditions with repetitive behaviours such as autism spectrum disorders and Tourette syndrome, although both of these groups may co-occur with OCD. A subgroup of children with OCD may fall into the PANDAS category. PANDAS/PANS Patients with Sydenham chorea frequently have emotional and/ or behavioural symptoms, particularly OCD. Sydenham chorea is associated with group B haemolytic streptococcus infection. In the late 1990s, Swedos group described 50 patients, who did not have Sydenham chorea, but did develop OCD and/or tics in response to a post-streptococcal autoimmune response. This type of OCD is referred to as PANDAS (paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections). More recently the concept has been broadened to PANS (Paediatric Acute-onset Neuropsychiatric Syndrome). The criteria for PANDAS are presence of OCD or tic disorder onset between 3 years and beginning of puberty abrupt onset of symptoms and a uctuating course characterized by dramatic exacerbations of symptoms onset or exacerbations of symptoms is temporally related to infection with Group A beta haemolytic streptococcus abnormal neurological examination (hyperactivity, choreiform movements and/or tics) during an exacerbation. It was proposed that, through a process of molecular mimicry, autoantibodies were generated against the basal ganglia, leading to behavioural disorder. There is a body of evidence to support this hypothesis and there is now also evidence to suggest that some children with typical OCD may have antibasal ganglia antibodies. Central nervous system autoimmunity may therefore have a role in a subgroup of cases of OCD. There has been research and interest in whether immunomodulatory treatments are indicated in this group, [but current evidence suggests that, outside of research studies, children whose OCD is thought to be triggered by Streptococcus should have a course of antibiotics if they still have an active infection, but otherwise receive the ordinary evidence-based treatments for OCD (see below)]. Autism spectrum disorders/pervasive developmental disorders The repetitive and stereotyped patterns of behaviour in autism can resemble some of the compulsive symptoms of OCD. A major difference between the two disorders is the underlying emotion that is associated with the rituals. A diagnosis of OCD, by denition, requires an element of internal anxiety and distress experienced if the obsession/compulsion is interfered with, and the child with OCD has some insight into the unnecessary, timeconsuming and unwanted nature of the rituals. In contrast, the child with autism enjoys the rituals and sees no reason to try to limit or stop them. The rituals typically seen in autism are part of the third symptom domain of autism: restricted, stereotypical, repetitive
Table 3
A detailed early developmental history, family history, and past medical and surgical history (and brief physical examination) should be undertaken to identify the risk factors outlined in Table 1. Children with early onset OCD (less than 10 years old) are often characterized by a longer duration of illness, higher rates of co-morbid tics, or frequent ordering and repeating compulsions and greater reported psychological difculties. Due to the sensitive nature of the symptoms, the child should also be seen on their own for a mental state examination to assess for OCD and related co-morbidities such as depression. The use of the validated CYBOCS (Childrens YaleeBrown Obsessive Compulsive Scale), a semi-structured checklist, is a useful adjunct to generate a list of current and historical symptoms and to gauge severity. Factor and Cluster analysis has demonstrated OCD as a heterogeneous condition with symptoms falling into ve independent symptom dimensions. contamination and cleaning. harm (aggressive), obsessions and checking. symmetry, ordering, repeating and counting. hoarding. sexual and religious obsessions. Enquire about symptoms that may identify co-morbid disorders and related OCD spectrum disorders (Table 4).
Co-morbidity, related disorders and differential diagnoses

80% of young people with OCD have a co-morbid psychiatric disorder such as ADHD, depression, tics, specic, developmental disabilities, oppositional deant disorder and anxiety disorders.
Conditions related to obsessiveecompulsive disorder (OCD spectrum disorders)

C C C C C
Body dysmorphic disorder Trichotillomania Hypochondriasis Tourette syndrome Compulsive hoarding
Table 4
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repertoire of interests and behaviours, and are in the context of autistic rigidity. The autistic child may insist on the performance of particular routines and rituals and have stereotyped, often unusual, preoccupations. Quite often, disruption of these routines can lead to a signicant behavioural disturbance. Children with autism are at increased risk of psychiatric comorbidity (this risk is further increased in the presence of learning disability). Children with autism may therefore have comorbid OCD, but frequently clinicians attribute all repetitive phenomenology to the autism, often because it is difcult to differentiate the two syndromes. Where appropriate, OCD should be detected and treated if present. Co-morbidity of OCD in children with autism can be difcult to demonstrate for a number of reasons. It is often difcult to assess the mental state of children on the autism spectrum. Many children with autism have associated severe learning disability and are non-verbal; hence it is difcult to infer an internal state. This can only be done through behavioural measures (reduced sleep, reduced appetite, pulse rate increase, sweating, pacing, agitation, papillary dilatation, cold and clammy). The anxiety caused by stopping a compulsion could easily be confused with the distress caused by the stopping an autistic ritual (because of the cognitive rigidity of the autistic child). Autism/autistic features should also be looked for in those diagnosed with OCD, especially in those who are treatment resistant. A diagnosis of OCD is perhaps more easily made in high functioning autistic children where they are able to verbalize their internal distress regarding their obsessions and compulsions, and are able to convey that they have some notion of the senselessness or the feeling that things are just not right if they do not complete the ritual. Rituals and mealtimes A child who has a fear of contamination may as a consequence restrict their diet. A child may present with compulsions related to mealtimes but in the case of an eating disorder have a fear of fatness. A child with autistic rigidity may present with rituals related to how the meal is presented, the texture, the consistency, food stuffs, and who feeds them. There may be challenging behaviours linked to a change in food related rituals. Hoarding 70% of children are thought to own collections at some point. Hoarding symptoms which impacts on social functioning and is disabling is seen in 5e10% of people with OCD. However increasingly it is being recognized as a separate disorder and this is reected in the new DMS V classication. OCD and its classication In earlier versions of DSM, OCD was classied as an anxiety disorder. In ICD-10, OCD is classied separately from anxiety disorders but is included as part of a wider group of disorders neurotic, stress related and somatoform disorders. There is a drive to move OCD to a separate category from anxiety as part of the obsessive compulsive spectrum disorders.
Treatment
In 2004, a multicentre randomized controlled trial compared cognitive behaviour therapy (CBT), sertraline [a selective serotonin reuptake inhibitor (SSRI)] and a combination of these in children and adolescents, and concluded that children with OCD should begin treatment with CBT alone, or CBT plus an SSRI. In 2006, UK government guidelines from the National Institute of Health and Clinical Excellence (NICE) summarized the evidencebased treatment options for OCD (and body dysmorphic disorder) for young people and adults, following a stepped care treatment model (Figure 2). Psychoeducation and CBT were
Step 1 Individuals, public, organisations, NHS Provide, seek, and share information about OCD
Step 2 GP, practice nurse, school health, health visitors, general health settings Recognition and Assessment of OCD
Step 3 GP, primary care team, primary mental health worker, family support team CAMHS Tier 1/ 2 Management and initial treatment; guided self help, CBT (and ERP), involve family, carers, consider school
Step 4 Multidisciplinary care in primary or secondary care CAMHS Tier 2/3 OCD with co-morbidity or poor response: CBT (and ERP), then consider combined treatments of CBT (+ERP) with SSRI, alternative SSRI or clomipramine
Step 5 Multidisciplinary care with expertise in OCD CAMHS Tier 3/4 CBT (+ERP),then consider combined treatments of CBT (+ERP) with SSRI, alternative SSRI or clomipramine. Consider referral to specialist centre
Step 6 Inpatient care or intensive treatment programmes CAMHS Tier 4 OCD with risk to life, severe self-neglect or disability, reassess care coordination, augment and reassess treatment options
Figure 2 Stepped care model for treatment of obsessiveecompulsive disorder (OCD) in children.
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recommended as rst-line treatment, and then CBT combined with an SSRI for more severe cases. The POTS II randomized control trial published in 2011 emphasized the importance of having access to Cognitive Behavioural Therapy incorporating Exposure Response Prevention (ERP). Among 124 children aged 7e17 years with OCD and who had had a partial response to serotonin reuptake inhibitors, the addition of CBT to medication compared with medication alone resulted in a signicantly greater response rate. They also had a third treatment strand where the clinician gave advice on how to use CBT but did not administer CBT directly. This treatment strand did not show a demonstratable difference from having medication alone. This also suggests the need for delivering full rather than truncated CBT protocols. Children with early onset OCD (less than 10 years old) are equally responsive to developmentally appropriate CBT or CBT and medication as older children. Psychological treatment Mild functional impairment: very early onset or mild OCD should be managed with psychoeducation and guided self-help, but with early referral for professional intervention if there is poor recovery. A list of additional resources is given at the end of this review. Psychoeducation should include an explanation of what obsessions and compulsions are, and the mechanisms of anxiety, as well as the principle of learning to face the fear and cut back on rituals gradually. Moderate or severe functional impairment: this should be managed with CBT with exposure response prevention (ERP) and it is usually helpful to involve the family. By involving parents as co-therapists, they can play a key role in shaping treatment, and encourage and reward the child for progress. It also shows parents how they may be inadvertently reinforcing OCD behaviours, accommodation and helps them learn to tolerate their own distress when aiding their children in ERP tasks. In CBT (usually a brief therapy of 12e14 sessions), patients work collaboratively with the therapist to learn gradually to confront their feared stimuli (e.g. perceived contamination); this is exposure. In conjunction with this, they learn to understand and tolerate graded amounts of anxiety while resisting carrying out compulsions; this is response prevention. The patient works through a hierarchy of feared stimuli, starting with the least feared of the stimuli, and practices facing the situation whilst monitoring their anxiety levels, observing that the anxiety levels subside eventually without the need for a ritual. These experiments, exposure response prevention, need to be practised several times a day, as over time a patient will have avoided many feared stimuli or developed rituals. CBT with ERP has response rates of 40e85% for those who complete treatment. There is no evidence to support the efcacy of psychodynamic psychotherapy in OCD and NICE does not recommend its use. Medication For children with more severe OCD or those or who have shown limited response to CBT incorporating ERP, there is evidence supporting their treatment with SSRIs for symptom remission
and improvements in global functioning. These can be used in combination with CBT. At the time the NICE guidelines were published, 18 published trials were identied, of which four did not meet the inclusion criteria. The 14 included studies provided efcacy data from 1034 participants and tolerability data from 1068 participants. Sertraline and uvoxamine are licenced for use in children in the UK, but other SSRIs may be chosen for clinical reasons. Sertraline is licenced from the age of 6 and uvoxamine from the age of 8 years. In practice many clinicians nd that uvoxamine produces unacceptable gastrointestinal side effects, including nausea and vomiting. SSRI numbers needed to treat range from 2 to 10. Fluoxetine is the only recommended SSRI for use in the treatment of depression in children under 18 years old. For children with OCD co-morbid with depression, this is the preferred rst-line medication. Patients with OCD require a trial of an SSRI for at least 12 weeks at the maximum tolerated therapeutic dose. The dose should be increased gradually, frequently with no therapeutic response noticeable on a low dose or for 4e6 weeks. It may take several weeks to build up to the therapeutic dose, and then the full effects of this may not be evident for several more weeks. If a trial of one SSRI fails, the patient should be reassessed, clarifying compliance and ensuring that co-morbidity has not been missed. A trial of a different SSRI or clomipramine, a serotonin reuptake inhibitor tricyclic antidepressant, should be considered. Nausea and appetite loss in the rst few weeks, before there is a therapeutic response, may be seen, but is usually tolerated and resolves. SSRIs are also associated with behavioural activation syndromes, which have been linked to a small increase in the relative risk of self-harm and suicidal thoughts (but not completed suicide). Monitoring is especially important in the early stages of treatment and at times of change in dosage. SSRIs should not be used if a child has renal or hepatic problems, and occasionally they have been associated with increased seizure frequency. As for all medications in children, the potential risks of untreated OCD, including a potentially life-long impact on emotional wellbeing, social and educational development, need to be considered against the risks of medication side effects, both acute and long term. Medication may also be indicated in those whose capacity to access CBT is limited by learning disabilities, although every attempt should be made to modify CBT protocols for the less able child. Smaller doses and doses increasing over a longer period of time are recommended for children with learning disabilities, as they have a tendency to develop side effects more quickly. Medication has occasionally been used where there is no availability of CBT. It is not acceptable to use medication instead of CBT unless the child is unable or unwilling to use psychological treatment. Whilst medication should not be withheld from the child who needs treatment for their OCD, parents and clinicians should be aware of the NICE recommendations that all young people with OCD should be offered CBT; if necessary, the child should be referred to a specialist who can provide CBT or the primary care trust childrens commissioner should be asked to change local provision.
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Patients refractory to treatment/partial response to treatment Long-term follow up studies have shown 40% of children with OCD have persistent symptoms: some children with OCD may fail to respond to an initial SSRI administered for at least 12 weeks at the maximum tolerated dose, in combination with an effective trial of CBT incorporating ERP. These children should usually have additional trials of at least one other SSRI. Following this, if response is still limited, the child should be referred to a specialist centre. Often children with more severe or chronic OCD have co-morbidities that can affect the initial response to treatment and long-term prognosis. The POTS II trial outcomes emphasize the need to ensure adequate CBT provision in the community in addition to medication. Further developments Glutamate may also be implicated in OCD which has led to research into glutamate modulating agents in its treatment.
Conclusions
Specialist child and adolescent psychiatry provision is still very variable throughout the UK, although there is now a nationally commissioned service for the most severe, treatment-refractory OCD. Given the hidden nature of OCD, it can take many years for children to be seen in specialist child mental health services. However this is a condition where 80% of children can experience clinically useful responses to the correct treatments, skilfully administered, so it is essential to make the diagnosis as early as possible. Paediatricians as part of their routine history taking should enquire about a childs worries and mood, and where possible have the opportunity to talk to children without their family present. Asking an anxious or worried child a few specic questions about OCD symptoms may allow them to reveal their symptoms for the rst time, and lead to diagnosis and effective treatment. At service level, primary care trusts should ensure specialist CBT is available as it is the treatment of choice for a number of mental health issues in children, including OCD. A
Child psychology and psychiatry: frameworks for practice. 1st edn. WileyeBlackwell, 2011. Derisley J, Heyman I, Robinson S, Turner C. Breaking free from OCD: a CBT guide for young people and their families 2008. Franklin ME, Sapyta J, Freeman JB, et al. Cognitive behaviour therapy augmentation of pharmacotherapy in pediatric obsessiveecompulsive disorder. The Pediatric OCD Treatment Study (POTS II) RCT. JAMA 2011; 306: 1224e32. Heyman I, Matais-Cols D, Fineberg NA. Obsessiveecompulsive disorder. BMJ 2006; 333: 424e9. National institute for Health and Clinical excellence; NICE guideline on OCD for patients. www.nice.org.uk. National UK Charity OCD Action. www.ocdaction.org.uk. South London and Maudsley NHS Trust Information on OCD and how to recover. www.ocdyouth.info. Veale D, Wilson R. Overcoming obsessiveecompulsive disorder. London: Constable and Robinson, 2005. Wagner A. Up and down the worry hill: a childrens book about obsessivee compulsive disorder. New York: Lighthouse Press, 2002. Wever C, Phillips N. The secret problem. ShrinkeRap Press, 1996.
Practice points
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FURTHER READING Chung I, Heyman I. Challenges in child and adolescent obsessivee compulsive disorder. In: Skuse D, Bruce H, Dowdney L, Mrazek D, eds.
Obsessiveecompulsive disorder (OCD) often presents for the rst time in childhood or adolescence A clinician should ask specic screening questions if OCD is suspected, as diagnosis is often delayed because the child is embarrassed to mention their rituals or worries In the best outcome studies 80% of young people with OCD will respond well to the evidence-based treatments, so detection and referral for treatment is important There are NICE Guidelines for the assessment and treatment of OCD; it is recommended that people with OCD should be offered cognitive behaviour therapy incorporating exposure response prevention Some children and adolescents with OCD will benet from treatment with serotonin reuptake inhibiting medication, which should be initiated by a specialist OCD can be relapsing or chronic; people with obsessivee compulsive disorder who relapse should have rapid access to services
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Conduct disorders in children and adolescents

Karen Baker
Whats new?
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Abstract
Conduct disorders in children and adolescents are the most common mental health difculty presenting to child health professionals. This review describes their prevalence and aetiology, as well as relevant evidenced-based interventions crucial to successful management.
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Adolescents with less severe forms of conduct disorder, as well as those with more serious problems, experience poor outcomes as adults. Effective early interventions to treat conduct disorder in children will have overall cost benets for society both nancially and in terms of emotional well being. Treating children and young people is more effective than punishing them.
Keywords conduct disorders; review
Introduction
It is important for professionals working with children and young people to understand conduct disorders or related challenging behaviours. The concern they can produce was highlighted by the panic following the 2011 riots in England over the behaviour of feral or criminal children and teenagers. The riots were one of the most serious instances of civil unrest in the mainland UK for a generation. From the second night signicant numbers of young people took to the street in various cities, include London, Birmingham and Nottingham. In the analysis of arrests and prosecutions more than 90% were male and 17% were aged between 11 and 17 years. However the reason for understanding and treating conduct disorders in children is more than to avoid such serious but rare episodes. The excessive presence of non-compliance in a child, with or without aggression, inevitably produces enormous challenges to parents and teachers. Also there is a growing awareness of the nancial and social costs to the wider community. Children with conduct problems often falter in their interpersonal relationships and struggle to function in a socially effective way. Of course children are born with a remarkable potential for emotional and social development. It takes time for them to learn to behave properly, i.e. to the relevant socially and culturally dened norms. Aggressive or oppositional responses are not always in themselves inappropriate behaviour. The key is in recognizing how, when and where this kind of response may be socially acceptable. One way to understand violent or antisocial behaviour in children and adolescents is as a failure to acquire the most appropriate social response alongside the development of undesirable or ineffective behaviours.
Denitions
Establishing when antisocial behaviours necessitate professional intervention is a challenge. Conduct difculties have needed to
be distinguished from the normal emergence of oppositional behaviours as part of a growing sense of individualization and autonomy in an age-appropriate way. The terrible twos or teenage tearaway developmental phases seen in many children, while challenging to parents and other adults caring for children, are to some degree normal, important stages in the childs psychosocial development. However, some childrens oppositional or antisocial behaviours are in excess of the ordinary. Both of the recognized psychiatric classication systems (ICD10 and DSM-IV) dene conduct disorder as a repetitive and persistent pattern of aggressive, deant or antisocial behaviour. DSM-IV further delineates two categories of antisocial disorder in childhood: oppositional deant disorder (Table 1) and conduct disorder (Table 2). These categories are recognized as heterogeneous and overlap each other. The oppositional young child may develop more severe conduct problems or delinquent behaviour as time goes on. To qualify as oppositional deant disorder, the behaviours must occur more frequently than is typically observed in children of comparable age and at a similar developmental level. Furthermore, they must lead to signicant impairments in social, academic or occupational functioning. Although oppositional deant disorder and conduct disorder overlap in denition and presentation, they can be differentiated. Conduct disorder entails the violation of the basic rights of others, societal norms or rules (Table 2). DSM-IV distinguishes two subtypes of conduct disorder on the basis of age at onset. This follows extensive evidence that early childhood conduct problems often persist and show a distinct risk prole. They have a markedly poorer long-term outcome than those that develop for the rst time in the teens. In childhood-onset type, at least one of the 15 behaviours (Table 2) must appear before the age of 10. In adolescent-onset type, none appears before the age of 10 years. The DSM classication is currently being revised. There are no proposed revisions for the main criteria of Conduct Disorders in DSM eV. However there is a recommendation being considered for the groups to add an additional specier for callous and unemotional traits in an individual.
Karen Baker BMedSci BM BS MRCPsych MA is a Consultant Child and Adolescent Psychiatrist, Nottinghamshire Healthcare NHS Trust, Mandala Centre, Gregory Boulevard, Nottingham, UK. Conict of interest: none.
Prevalence
Community studies have consistently shown that the prevalence of conduct disorders is high. A recent UK study based on a national sample of 10 000 children aged 5e15 years, found that
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Oppositional deant disorder

DSM-IV states that it is characterized by the frequent occurrence of at least four of the following, that persist for at least 6 months:
C C C
C C C C C
Losing ones temper with adults Arguing with adults Actively defying or refusing to comply with the request or rules of adults Deliberately doing things that will annoy others Blaming others for their own mistakes or misbehaviour Being touchy or easily annoyed by others Being angry and resentful Being spiteful or vindictive
Conduct problems are the most common reason for referring young children to mental health services, accounting for 30e40% of referrals to Child and Adolescent Mental Health Service (CAMHS). In early childhood boys are far more likely than girls to manifest disturbance of conduct. During adolescences the proportion of girls increases markedly. There are indications that gender differences in delinquency have narrowed in recent years. Symptoms of conduct disorder may differ between females and males. Some studies suggest that girls who meet the criteria for conduct disorder have a preponderance of non-aggressive or covertly aggressive symptoms.
Aetiology
A wide variety of biological, psychological and social factors are now thought to contribute to the development and maintenance of conduct disorders. These factors overlap and, when problems are longstanding, simple unidirectional models of causation will fail to capture the complex, cumulative nature of the processes involved. Many of the features of oppositional behaviour involve interactions of reciprocal compounding factors, e.g. a temperamentally difcult child may evoke negative responses from parents, which reinforce or exacerbate aggressive, non-compliant responses from the child. Gerald Pattersons seminal research described cycles of escalating coercive childeparent interactions in the homes of aggressive children. He felt harsh, abrasive and inconsistent parenting practices showed strong correlation with antisocial behaviour in children. Twin studies suggest that shared environmental effects account for approximately 30% of the variance in childhood behavioural problems. Though less heritable than other childhood disorders, conduct problems probably do have an inherited component, which is most important for the early-onset difculties co-morbid with hyperactivity. Neuropsychological decits in children, such as low IQ, poor verbal skills and impairment in executive functioning, have been proposed as key vulnerability factors for early-onset conduct problems. Characteristic patterns of social cognition, whereby some young people over-attribute hostile intent to others, are among individual correlates of disruptiveness and aggression, though their causal role remains less certain. Some researchers have become interested in differentiating the children who develop or are born with difculties in emotional empathy i.e. children with high callous and unemotional traits. Alongside these individual factors, much research has shown that antisocial behaviour is more likely to develop in adverse family and social circumstances. Poverty, disorganized neighbourhoods, poor schools, family breakdown, parental psychopathology, harsh and ineffective parenting and inadequate supervision are all strong correlates of conduct disorder. Thus, many apparently environmental effects may reect geneenvironment interplay, whereby heritable child characteristics evoke particular responses from parents, or heritable parent characteristics inuence the styles of parenting and models of behaviour the parent provides. At present, the research is only at the very earliest stages of teasing out how these complex processes operate.
Table 1
5e10% met DSM diagnostic criteria for oppositional deant disorder. The more serious problems included in the diagnosis of conduct disorder have a comparable prevalence of 2e9%. Prevalence is higher in low socioeconomic status groups.
Conduct disorder
DSM-IV lists the following 15 behaviours categorized under four headings. At least three must be overtly present in the previous year to meet the criteria, with one present in the last 6 months: Aggressiveness to people and animals C Bullying C Fighting C Using a weapon C Physical cruelty to people C Physical cruelty to animals C Stealing with confrontation of the victim C Forced sexual activity Property destruction C Fire setting C Other destruction of property Deceptiveness or theft C Breaking or entering C Lying for personal gain C Stealing without confronting the victim Serious rule violation C Staying out at night or being truant before the age of 13 years C Has run away from home overnight at least twice C Is often truant from school, beginning before the age of 13 years Table 2
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Co-morbid difculties
Many pre-pubertal children diagnosed with oppositional deant disorder and conduct disorder show predictable co-morbidity (Table 3). Decits in verbal skills have long been recognized among children with conduct disorder. These can commonly manifest as specic reading difculty or as problems in language development. As the child progresses through the educational curriculum, the demands for tasks that require verbal skills escalate. This may contribute to a breakdown of the relationship between the child and the school. Specic language impairment may be associated with impairments in social cognitions. These include the inability to understand humour or sarcasm, and anothers mental state or intentions. Clinical observations of children with these social difculties suggest they often use inappropriate aggressive responses to perceptions of victimization. These are particularly common during unstructured school breaks or poorly supervised peer interactions. This can lead to rejection by peers and teachers so children with conduct disorder face misunderstanding, punishment and alienation from school or other social environments. Parents and schools can resort to avoiding any situation which will result in a public conict scenario. This in turn produces feelings of rejection, sadness and isolation in the child when they are not allowed or invited on ordinary social events such as school trips, peer birthday parties or even family gatherings. Attention-decit hyperactivity disorder (ADHD) is a key differential diagnosis of conduct disorder and may reect abnormalities or immaturity of prefrontal cortical functioning. It is because of this decit that the child appears, for instance, distractible and impulsive. The life course persistent conduct disorder of early-onset and sustained course may be characteristically linked to ADHD. There can be a failure to develop an anticipation of consequences of actions, so serious risk-taking behaviour may result if the decit persists.
employment, difculties in interpersonal relationships, both social and intimate, high rates of alcohol problems and substance use, increased risk of mental health difculties and compromised physical health. Though children with oppositional deant disorder and conduct disorder frequently come from disadvantaged backgrounds, these poor outcomes are not simply a function of social adversity. Criminology statistics reect the long-term consequences of conduct problems. UK analyses of Home Ofce statistics show that in 1994 boys and young men between the ages of 10 and 20 committed 42% of all indictable offences. These gures probably underestimate the prevalence of youth criminal activity as a large number of offences are not reported or recorded by the police. Adult development is compromised for boys and girls indifferent ways. Outcomes for boys tend to be characterized by continuing antisocial behaviour, risk of substance misuse and problems in employment, while for girls the central features may be early pregnancy, unsupportive relationships, depression and parenting difculties. All too often young people with conduct problems seem attracted to one another as partners in early adulthood and form volatile relationships. This way, intergenerational continuities in antisocial behaviour are likely to be high. Conduct problems in children increase demands on multiple services, including social services, education, health and juvenile justice services. There are signicant extra costs for the families with a child with a conduct disorder because of their destructiveness, and loss of employment opportunities for parents. Preliminary ndings from a study of the lifetime costs found that an individual who has a conduct disorder at age 10 costs over 100 000 more in services, up to age 28 years, than one who does not.
Assessment
Antisocial behaviour affects many aspects of a childs and young persons development. Therefore, an assessment needs to be multifaceted, involving information gathering from school and other agencies, as well as the carers and child themselves. Of all the parties involved, the child may be the least concerned about their difculties and may resent any attempts to help. The childs perspective is crucial, both for a complete account of the behaviour and also for an understanding of family or other problems that may exacerbate their behaviour. Drawing on a range of sources can establish the characteristics of the behavioural problems, including impulsive or risk-taking behaviours. A picture is gained of any possible environmental variation, such as the behaviours being worse at home or school, and how much other aspects of functioning are impaired. Because co-morbid conditions can affect the course of conduct problems and may require specic interventions, it is important to enquire about symptoms of ADHD, anxiety and depression, as well as antisocial behaviour. It is essential to ascertain an understanding of the childs interpersonal skills and approaches to conict negotiation. The latter can be obtained by parental descriptions of examples of oppositional behaviour. A detailed blow by blow account of a recent episode of difcult behaviour is a useful tool in gaining an understanding of how behaviours can arise and attempts to resolve them. Observations in the clinical setting when parent
Outcomes
There is now little doubt that the long-term outlook for many children with conduct problems is poor. Longitudinal studies in a number of countries, and in different historical eras, have documented a litany of adverse adult outcomes. These include persistent antisocial behaviours and offending, problems with
Co-morbidity with conduct disorders

C C C C C C C C
A DHD Depression/anxiety Self-harm Substance misuse Post-traumatic stress disorder (PTSD) Learning disability Tourette syndrome Autistic spectrum disorder
Table 3
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and child are engaged in a joint task, e.g. tidying up toys, can be a helpful addition to the assessment. The quality of peer relationships, as well as the types of activities undertaken with peers, is important in formulating treatment plans. Direct observations of peer interactions, e.g. in the playground, can aid the understanding of distorted social skills. Several checklists can be used for rating symptoms in children. One of the most commonly used is the child behaviour checklist, which has between 100 and 113 items that describe specic behavioural and emotional problems. Ideally diagnostic interviews should be accompanied by a full psychometric assessment. Reports from school need to cover not only the impact of the behaviour in the classroom but also academic strengths and weaknesses, and the presence or suspicions of specic learning difculties. Assessment of family relationships and parenting styles is crucial. Parental discord, especially around how to respond to the childs behaviour, is important to illicit and may not be readily offered if only one parent attends. It is therefore important to ascertain if both parents and other carers, such as grandparents and child minders, have a consistent approach to the challenging behaviour of the child. It is also essential to gain a picture of the affective tone of the family interactions. Unfortunately, by the time carers present to services they may view the child in a predominantly negative light, because of the challenging behaviours. However, it is important to look for evidence of warmth, approval and sensitivity to the childs needs. Professionals assessing a child or adolescent for a conduct disorder need to be vigilant, as they would with any child, to indicators of neglect and maltreatment.
management training refers to the procedures in which parents are trained to interact differently with their children. It involves the teaching of parents, individually and in groups, to pay attention to and reinforce desirable behaviours, and to practise effective strategies for dealing with unwanted responses. Role play and video feedback are used to facilitate new styles of parental responses. Accumulating evidence shows that parent training programmes may be applied to a wide range of conduct problems and effectively delivered in various settings. NICE guidance was published in 2006 on parent training/education programmes in the management of children under 12 years with conduct disorders. It identied 16 reviews that assessed the effectiveness of one or more parent training programmes. From this analysis, the recommendations listed in Table 4 were offered. Failure to benet from parent training/education programmes is associated with parental disadvantage, lack of parental perception of a need for an intervention, parental mental health problems, especially alcohol and drug problems, personality difculties and depression. Co-morbidity in the child, such as untreated ADHD, language disorders and learning difculties, may also reduce the efcacy of this kind of programme. The increasing recognition of social skills decits has generated a range of treatments that focus on the distorted approaches to social events by young children with conduct problems. Cognitive problem-solving skills training which is designed to improve the childs understanding of interpersonal situations and extend their repertoire of effective responses has also been shown to have some positive effects, if offered alongside parent training programmes. Interventions effective for adolescents Meta-analysis of treatment approaches to conduct disorder in adolescents has produced different ndings in relation to efcacy. Multisystemic therapy (MST) is the most promising intervention for adolescents with serious conduct disorders. This approach recognizes the multidimensional nature of serious antisocial behaviour. It draws on a broad spectrum of techniques to address individual, parental, family and peer relationship problems. It uses multiple interventions in combinations directed by the clinical picture. The main treatment interventions include systemic and structural family therapy, parent training, marital therapy and supportive psychotherapy related to interpersonal problems and social skills components, as well as case management where the therapist acts as an advocate to outside agencies, for the young person and family. The main goal of MST is to give parents the skills and resources needed independently to address the difculty of raising adolescents, while also empowering the young person to cope with familial and extra familial problems. There have been a number of trials of MST which have shown improvement of family functioning and reduction in the rate of recidivism. A functional family therapy (FFT) approach assumes that an adolescents problem behaviour is serving a necessary function in the family. Such functions include regulation of support and intimacy or regulation of distance between family members. The treatment focuses on the interactional aspect of the family
Interventions
Conduct disturbances are difcult to treat, especially if longstanding. To be effective, any intervention needs to address the full range of the childs difculties, at home, school and the wider community, in a developmentally appropriate way. Because conduct problems often arise in disadvantaged families, broader family problems may also need to be targeted. Engaging carers is crucial but is at times challenging. Conduct disorders often take a chronic course and rarely respond to short-term interventions. There is now a strong argument that interventions should be informed by the increasing understanding of the psychopathology underlying conduct problems. Therefore, for early-onset conduct disorder or oppositional deant disorder, interventions should focus on psycho-education and support for parents and school, to avoid reinforcing undesirable behaviours. Problems with language, literacy and the ability to cope with peers should also be identied and addressed. Treating co-morbid psychiatric conditions such as ADHD or depression is crucial. Parent management training For young children (under 8 years), parent management training has the strongest support from evaluative research. Although there are many types of parent management training, the underlying rationale for all is based on the general view that conduct disorders are inadvertently developed and sustained in the home by maladaptive parentechild interactions. Parent
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NICE guidance on parent training/education programmes in the management of children under 12 years with conduct disorder23
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Group-based parent training/education programmes are recommended in the management of children with conduct disorders. Individual-based parent training/education programmes are recommended in the management of children with conduct disorders only in situations where there are particular difculties in engaging with the parents or a familys needs are too complex to be met by group-based parent training/education programmes. It is recommended that all parent training/education programmes, whether group or individual based, should: Be structured and a curriculum informed by principles of social learning theory. Include relationship-enhancing strategies. Offer a sufcient number of sessions, with an optimum of 8e12, to maximize the possible benets for participants. Enable parents to identify their own parenting objectives. Incorporate role play during sessions, as well as homework to be undertaken between sessions, to achieve generalization of newly rehearsed behaviours to the home situation. Be delivered by appropriately trained and skilled facilitators who are supervised, have access to necessary ongoing professional development and are able to engage in a productive therapeutic alliance with parents. Adhere to the programme developers manual and employ all of the necessary materials to ensure consistent implementation of the programme. Programmes should demonstrate proven effectiveness. This should be based on evidence from randomized controlled trials or other suitable rigorous evaluation methods undertaken independently. Programme providers should also ensure that support is available to enable the participation of parents who might otherwise nd it difcult to access these programmes.
a solution by parents and schools. However, they have not been demonstrated to be effective in any age group as a stand alone intervention. Trials of social skills training in adolescents with antisocial behaviour reveal there is no evidence for the skills that can be generalized beyond the treatment setting, and therefore these have limited impact on the delinquent behaviour. Some evidence has emerged that placing aggressive or antisocial children and adolescents in group therapy may be less than useful or even exacerbate the unwanted behaviours. When youngsters with conduct disorders are placed together, in the absence of children with pro-social behaviour, i.e. without conduct disorder, peer bonding to deviant group members may occur and reinforce antisocial attitudes, values and behaviours. Ironically, the current management of children and adolescents in schools and youth offending facilities often emphasizes group interventions for conduct disorder. Another clear nding is that for adolescent offenders, therapeutic programmes are much more effective than punitive interventions such as boot camps or intensive parole and probation supervision. Indeed punitive measures have been shown to usually have negative returns with programme costs exceeding benets.
Concluding remarks
Conduct disturbances in children and adolescents have signicant effects on an individuals mental and physical health and are an enormous strain on the resources of child health and welfare services. Advances in the understanding of the pathways leading to conduct disorder and delinquency have increased in recent years. More elaborate and effective interventions have been developed as a result. They have also given a sense of optimism to a previously heart sink aspect of childhood behaviour difculties. If relevant interventions are available to children, parents and schools early enough, the developmental pathways for these children can be more positive. Early intervention programmes offered to at-risk families when the children are under 5 years may even prevent youngsters developing conduct problems and promote the healthy emotional wellbeing that accompanies the development of pro-social behaviour. The recently published NICE guidelines are an attempt to clarify what kind of parent management works. If applied in a strategically targeted approach, with adequate resources, this might not only reduce rates, but also improve the quality of life and opportunities for a signicant number of young people. Further positive effects could be on rates of substance misuse, teenage pregnancies, suicide and crime. A
Table 4
processes as well as behavioural and cognitive dysfunctions. The goal of treatment is the achievement of a change in patterns of interactions and communications, in order to promote adaptive family functioning. Highly conictual family interactions are a primary target of the intervention. FFT is unusual among psychosocial therapies for children and adolescents in having a respected body of research to support its efcacy. However, effective delivery of this treatment requires considerable training and supervision. This may explain why, despite the relatively well-established manner of this treatment approach, its use is limited. Interventions for conduct disorders that seem less effective Social skills training and programmes to improve problem-solving skills, often referred to generically as anger management training, seem to have high face validity and are often pursued as
FURTHER READING Audit Commission. Children in mind e report on child and adolescent mental health services. London: Audit Commission, 1999. Audit Commission. Mis-spent youth e young people and crime. London: Audit Commission, 1996. Berelowitz S. I think I must have been born bad. Emotional well being and mental health of children and young people in the youth justice system, www.childrenscommisioner.gov.uk; 2011. Colman I, Murray J, Abbott RA, et al. Outcome of conduct problems in adolescence:40 years follow up of national cohort. BMJ 2009; 337: 2981.
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Farmer M. Language and social cognition in children with specic language impairment. J Child Psychol Psychiatry 2000; 41: 627e36. Fonagy P, Target M, Cottrell D, Phillips J, Kurtz Z. What works for whom? A critical review of treatments for children and adolescents. New York: Guilford Press, 2002. Hill J. Biological, psychological and social processes in the conduct disorders. J Child Psychol Psychiatry 2002; 43: 133e64. Kazdin AE. Treatment of conduct disorders. In: Hill J, Maughan B, eds. Conduct disorders in childhood and adolescence. Child and adolescent psychiatry. Cambridge: Cambridge University Press, 2001; 408e448. Knapp MRJ, Scott S, Davies J. The cost of antisocial behaviour in younger children; A pilot study of economics and family impact. Clin Child Psychol Psychiatry 1999; 4: 457e73. Maughan B, Rutter M. Antisocial children grown up. In: Hill J, Maughan B, eds. Conduct disorders in childhood and adolescence. Child and adolescent psychiatry. Cambridge: Cambridge University Press, 2001; 507e552. National Institute for Health and Clinical Excellence. Conduct disorder in childreneparent-training/education programmes. National Institute for Health and Clinical Excellence, 2006. Patterson GR. Coercive family process. Eugene, OR: CastiliaPublishing Company, 1982. Sainsbury Centre For Mental Health. The chance of a life time; preventing early conduct problems and reducing crime 2009. Simonoff E. Genetic inuences on conduct disorder. In: Hill J, Maughan B, eds. Conduct disorders in childhood and adolescence. Cambridge: Cambridge University Press, 2001; 202e234.
Webster-Stratton C, Hancock L. Training for parents of young children with conduct problems: content, methods and therapeutic processes. In: Briesmester JM, Shaefer CE, eds. Handbook of parent training. 2nd edn. New York: Wiley, 1998; 98e152.
Practice points
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Professionals working in childrens services need to know how conduct disorders present and the range of interventions that are available to support children and families Community studies have shown the prevalence of conduct disorders to be as high as 10% up to the age of 15 years. They represent the most common reason for referring young children to Child and Adolescent Mental Health Service Conduct disorders in children and adolescents produce enormous challenges to parents and teachers, and represent a signicant burden to wider society, both in social and nancial terms. The long-term outcome for conduct disorder, in the absence of effective interventions, is poor Parent management training has the strongest evidence base, as an effective intervention for conduct disorder, in children under the age of 12 years In adolescents with conduct disorder, multisystemic therapy and functional family therapy have been shown to be effective
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Stress and post-traumatic stress disorder

Margaret DeJong Sin ead Marriott
Diagnostic criteria
Symptoms of PTSD cluster into three broad categories: reexperiencing, avoidance and hyperarousal (See Table 1). These criteria are the same for adults and children, although need some modication for very young children. Developmentally appropriate modications have been proposed for pre-school children and will be included in DSM-V. These rely less on the childs verbal descriptions and more on what can be observed. Re-experiencing in young children often takes the form of posttraumatic play, characteristically displaying a re-enactment of part of the trauma. Play may have a compulsive, rather stereotypic quality, and is less elaborated and imaginative than usual play. Verbally competent young children may recall fragments of the experience not necessarily showing any associated distress. There may be nightmares without an obvious trauma-specic content. Emotional numbing can take the form of constricted play patterns, social and emotional withdrawal. Regression, in the form of a loss of previously acquired skills (e.g. language, toilet training) or more immature behaviour can occur. Night terrors, sleep difculties, general fearfulness and aggression are common. The cognitive model of trauma places increasing emphasis on the role of negative thoughts and beliefs in maintaining trauma symptoms. This will be incorporated in DSM-V as an additional criterion. Symptoms of hyperarousal will include reckless behaviour, which can be seen in adolescents. Many clinicians expected DSM-V to include a diagnosis of Developmental Trauma Disorder. This is a proposed diagnosis for individuals presenting with persistent dysregulation across several areas (including affect, physiology, behaviour, attention, self and relationships) as a result of exposure to chronic or severe interpersonal trauma such as abuse. There is ongoing controversy about its diagnostic validity and to date it has not been included in the DSM-V proposed criteria. Paul had just turned 3 years old when he witnessed his mothers partner fatally stab her. Paul continued to present as a happy little boy who was compliant and well engaged at nursery. However he became increasingly aggressive with his siblings and his play and drawings usually involved killings and being dead forever. He also repeated the details of the killing to other family members who found this difcult and tried to change the subject. In therapy sessions he displayed high levels of controlling behaviour and a somewhat manic, overly busy quality to his play. Alex was knocked down on a zebra crossing outside school when he was 12 years old. He suffered a broken arm. When Alex was referred for therapy at the age of 16 he had not travelled anywhere outside of the home without his parents, not slept in a room without his mother, or played football since the accident 4 years earlier. Alex was experiencing a repetitive nightmare of the event several times a week.
Abstract
There is growing awareness and concern about the high prevalence of traumatic experience and its long-term impact on the physical and mental health of children. Professionals working in the eld of child health have a crucial role in identifying children at risk and in providing support for resilience and recovery. This review provides a further update to an earlier article on current research and evidence-based treatment.
Keywords acute stress disorder; disaster; post-traumatic stress disorder (PTSD); stress; trauma
Introduction
Paediatricians have an important role in the early recognition of stress symptoms, which may develop into post-traumatic stress disorder (PTSD). Some of their patients will be victims of stressful events such as life-threatening illnesses, medical interventions, road trafc accidents, natural disasters or terrorist attacks. Others will develop symptoms as a result of chronic trauma resulting from abuse, neglect and witnessing domestic violence. Paediatricians who are alert to the possibility of trauma symptoms can do a great deal in a preventative capacity to ensure that appropriate supportive care is put into place. If unrecognized and untreated, trauma symptoms can persist for many months or years, impairing quality of life and development.
Denition
Following stressful events it is not unusual for survivors, including children, to experience transient symptoms such as difculty sleeping, troubling memories and thoughts, and some disturbance in everyday functioning. An acute stress disorder may be present if the stressor was highly threatening and the reaction continues for days or weeks. When the disturbance lasts longer than 1 month and causes signicant distress or impairment, it may meet criteria for PTSD.
Margaret DeJong MDCM FRCPsych(Can) FRCPsych(UK) is a Consultant Child and Adolescent Psychiatrist in the Department of Child and Adolescent Mental Health, Great Ormond Street Hospital, London, UK and Honorary Senior Lecturer, Institute of Child Health/UCL. Conicts of interest: none declared. Sin ead Marriott BSc (Hons) DClin Psych C Psychol is a Consultant Clinical Psychologist in the Department of Child and Adolescent Mental Health, Great Ormond Street Hospital, London, UK. Conicts of interest: none declared.
Epidemiology
Community samples suggest a lifetime prevalence of PTSD of 4 e12% and a point prevalence of 1%. Refugees and children exposed to the trauma and losses of war will have much higher rates of PTSD, around 30 e40%. It is estimated that one-third of children will develop PTSD after road trafc accidents. 69% of children diagnosed with PTSD after an RTA still met criteria 6 months later. After a natural disaster very
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Screening for PTSD

Exposure and response Has the individual witnessed or experience or heard of someone close to them experiencing an event involving actual or threatened death, serious injury or sexual violation Re-experiencing Has the traumatic event been persistently re-experienced in one (or more) of the following ways: C Intrusive thoughts or memories or repetitive play C Recurrent distressing dreams of the event. Note: in children, there may be frightening dreams without recognizable content C Dissociative reactions (ashbacks) a sense of re-living the experience C Intense psychological distress at exposure to internal or external cues that symbolize or resemble an aspect of the traumatic event C Physiological reactivity on exposure to internal or external cues that symbolize or resemble an aspect of the traumatic event Avoidance Is there persistent avoidance of stimuli associated with the trauma including: C Efforts to avoid thoughts, feelings or conversations associated with the trauma C Efforts to avoid activities, places or people that arouse recollections of the trauma Negative Alterations in Cognitions and Mood associated with the traumatic event as indicated by two or more of the following: C Inability to recall an important aspect of the trauma C Markedly diminished interest or participation in signicant activities C Feeling of detachment or estrangement from others C Restricted range of affect, e.g. unable to have loving feelings C Sense of a foreshortened future, e.g. does not expect to have a career, marriage, children or a normal lifespan C Persistent and exaggerated negative views about self and the world C Persistent, distorted blame of self or others about the cause of the event C Persistent negative emotional state C Persistent inability to experience positive emotions Hyperarousal Persistent symptoms of increased arousal (not present before the trauma), as indicated by two or more of the following: C Difculty falling or staying asleep C Irritability or outbursts of anger C Difculty concentrating C Hypervigilance C Exaggerated startle response C Recklessness Duration of symptoms is more than 1 month, causes signicant distress or impairment. Table 1
high rates (40 e50%) of PTSD can be expected initially. Approximately one-third if untreated will continue to exhibit PTSD at 1 year, and as many as one-third will still have PTSD 5 e8 years later. A particularly important vulnerable group for paediatricians to be aware of is children who have had traumatic medical experiences. High rates of PTSD (up to 21%) have been found in children who have been in a paediatric intensive care unit (PICU), as well as in their parents. Children who are subthreshold for a diagnosis of PTSD (34% post-PICU in one study) may nevertheless have signicant impairment.
Pathogenesis
In situations of acute stress and danger there is an automatic psychophysiological response mediated by the autonomic nervous system, which enables us to respond (ght, ight or freeze). As part of this response, the hypothalamicepituitaryeadrenal (HPA) axis releases noradrenaline (norepinephrine) and cortisol into the bloodstream, prolonging the bodys capacity to cope with stress. Symptoms of post-traumatic stress occur when the emergency response system has been conditioned, as a result of the experience, to respond to stimuli which trigger memories of the event. However, the trauma memories that are recalled are very different from normal memory. Normal event memory is stored in the brain as information which is experienced as having occurred in the past; it includes contextual information and can be retrieved at will, in narrative form, e.g. I remember going to my sisters wedding last week. Memories of this kind have been termed verbally accessible memory (VAM). In contrast, trauma memories are stored in the form of the original sensations, are experienced as it is happening again, now.. (i.e. re-living the event) and lack a narrative form. They are retrieved involuntarily, being triggered by environmental cues, and have therefore been termed situationally accessible memories (SAM). Normally, event information entering the amygdala and the thalamus is converted to VAMs by means of processing in the hippocampus. However, in conditions of stress, cortisol and noradrenaline (norepinephrine) inhibit the hippocampus from processing the event information in the normal way, so that storage occurs without the addition of the contextual information characteristic of VAMs. Chronic or repeated traumatization can lead to ongoing HPA axis dysregulation, contributing to long-term adverse effects on mental and physical health. In chronic early trauma such as severe abuse there can be structural changes to the brain including smaller brain size and corpus callosum.
Natural history
Not all acute stress reactions will develop into PTSD and, of those that do, 10e15% will be of delayed onset, developing 6 months or more after the event. However, quite often symptoms will resolve after a few days or weeks. Clinical and empirical studies have identied a number of factors that are associated with a heightened risk of developing PTSD after a traumatic event including: previous mental health difculties in the child or parent, poor family functioning and inadequate social support, perceived life threat and the degree of fear that was associated with the event, active avoidance of any thought about the event and being female.
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If untreated, PTSD symptoms can persist for years. Follow-up of survivors of the Aberfan disaster after 33 years found many to be suffering from PTSD. Traumatic experience is often accompanied by bereavement and other signicant losses which may involve major disruptions in a childs living situation. The traumatic loss of a loved one, especially if unexpected, can lead to emotional numbing or intrusive thoughts and images, which can make it difcult for normal grieving to take place.
Assessment
It is useful to start with children and parents together. The interviewer should begin with current circumstances, general development and functioning and then inquire directly about symptoms listed in the current DSM criteria or in a standardized trauma questionnaire. Children will not necessarily volunteer information and may be reluctant to disclose information in front of parents for fear of distressing them. Children who have experienced maltreatment may not show an emotional response because they have become desensitized to the psychological impact upon them. Parental accounts may not be completely reliable; they may themselves be traumatized and may underestimate the degree to which their child has been affected. Interviewing the child individually and collecting information from other settings is therefore also important. Withdrawn, avoidant or dissociative behaviour can make it difcult to communicate with traumatized children. Clinical observations are important: increased startle reex or other hypervigilant responses, lapses in concentration or dissociative spells may be observed during the interview. With younger children, play or drawing may be their preferred means of expressing themselves. At the end of any interview about a traumatic experience, a child should be helped to wind down by summarizing and reviewing what was said and praising them for their courage in discussing such painful events. Parents will often be able to provide useful background history. If they have shared in the traumatic experience they should be screened for PTSD. Their mental health and coping skills will have a signicant impact on their ability to contain their childs anxiety and provide the support that the child needs. The use of a screening instrument, such as the child version of the Impact of Events Scale (CR-IES), is a useful adjunct to a clinical assessment involving single traumatic events. It is also recommended for routine use by paediatricians after a known traumatic experience, such as organ transplant, PICU or a road trafc accident. Self-report questionnaires are only considered valid in children over the age of 7 years. Complex trauma symptoms stemming from interpersonal trauma such as abuse are harder to access and usually require specialist mental health expertise.
hyperactivity disorder (ADHD) in a child with hyperarousal and poor concentration, missing the connection with traumatic experience. The increased arousal and mood swings can be confused with the manic symptoms associated with bipolar disorder. Dissociative episodes can be mistaken initially for petit mal epilepsy. Children with a background of abuse and neglect can present with a wide range of psychological problems, including learning difculties, self-harm, substance abuse, conduct problems and aggression, often leading to the development of personality disorder as an adult. Exploring the possibility of traumatic experiences is vital, as it has important implications for intervention. David was 6 when he was referred for assessment of behaviour problems that were causing major problems in the classroom. He had been diagnosed as having ADHD and was taking Ritalin twice daily, but the effect of this was wearing off very rapidly. A detailed psychosocial history interview revealed that he had also witnessed signicant domestic violence between his parents. Davids behaviour improved with incremental adjustments to his dose of Ritalin and support for his mother in understanding and managing the effects of past trauma.
Role of the Paediatrician

NICE guidelines (2005) advise that healthcare professionals should inform parents about the risk and common symptoms of PTSD after a child has been treated in A&E and that they should contact their GP if the symptoms continue after a month. Paediatricians have a crucial role in enabling children and their parents to recover. Simple reassurance, information and advice are needed: helping to restore a sense of safety providing information about help available normalizing trauma symptoms and reassuring about recovery advising on the need to restore basic routine and consistency suggesting practical strategies for dealing with troubling symptoms such as hyperarousal and sleep disturbance encouraging family to identify and access sources of support within family and community promoting self help by providing access to useful resources such as leaets and websites (see below). This approach, described as psychological rst aid, may be sufcient to enable recovery to occur. It is, however, important that the paediatrician remains involved in a monitoring role. The child or adolescent can be asked at follow-up appointments whether they continue to be preoccupied by the traumatic event(s) or troubled by symptoms. If symptoms are very troubling and persist for more than 1 month, referral to a specialist will need to be considered. Families and young people can be reluctant to access Child and Adolescent Mental Health Services (CAMHS), and a supportive relationship with a paediatrician can greatly assist the referral and engagement process.
Differential diagnosis
Reactions to stress can take many forms, PTSD being one of them. There is a spectrum of responses, ranging from a normal stress response to a self-limited adjustment disorder, to a range of psychiatric disorders including but not limited to: anxiety, depression, dissociative states or somatization disorder. Co-morbidity with PTSD is very common, particularly anxiety and depression, which should be looked for specically. Trauma symptoms may also mimic other conditions. Busy clinicians who are not alert to this may be tempted to diagnose attention-decit
Management and prevention

Establishing safety and security for the child and their parents is of immediate importance. This principle is equally applicable after a natural disaster or in circumstances where a child has been witnessing severe domestic violence. The importance of each hospital and region having an up-todate disaster plan, which makes provision for the mental health
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needs of all those affected, was underlined by recent experience following the London bombings in 2005. It is important that the particular needs of children are not overlooked by planners. Psychological rst aid (see above) carried out by a paediatrician or other professional is an important preventive measure, and may be all that is required. Debrieng, a technique developed in the Vietnam War, is now considered controversial and is not routinely recommended.
New developments in the eld

Group CBT interventions in schools in areas where there are high levels of community violence or after large scale disasters such as Hurricane Katrina. Primary prevention of PTSD in medical settings such as PICU (COPE study, use of diaries). Childeparent psychotherapy and adolescent individual psychotherapy.
Psychological treatment
Specialist psychological treatment may be required in a small number of cases. It is now established that brief trauma-focussed psychological therapy is as effective in children as it is in adults, even in very young children. Treatment of trauma may involve an initial stabilization phase focussing on enhancing coping mechanisms, and ensuring a stable and effective support system is in place before proceeding to trauma-focussed desensitization work. In complex cases, such as in developmental trauma, a great deal of stabilization work is often required and treatment is longer. Cognitive behavioural therapy (CBT) is a well-established trauma-focussed treatment with a solid evidence base in children and young people. This approach involves helping the child to recall the distressing event in a safe way and reducing unhelpful avoidant coping strategies (exposure & desensitization), accessing and modifying unhelpful thoughts (cognitive restructuring). The sense that children make of their traumatic experiences (cognitive appraisal) and the beliefs about themselves and the world is a key focus of treatment. Children from around 7 years of age can usually engage in modied CBT. Very young children can be helped to establish a narrative of the events and make sense of their difcult thoughts and feelings using play, drawing and story telling. Eye movement desensitization and reprocessing (EMDR) was developed in the 1990s and is included in the NICE guidelines for adults with PTSD. The traumatic memory is desensitized via short bursts of imaginal exposure whilst the therapist provides simultaneous bilateral stimulation such as tapping the patients hands or asking them to track the horizontal movement of the therapists ngers. EMDR uses many of the same elements as CBT but requires less talking, which makes it particularly helpful with avoidant or very young children. A recent metaeanalysis found that children with PTSD benetted from EMDR and it was found to add a small but signicant incremental value when compared with CBT.
A
FURTHER READING AACAP practice parameter for the assessment and treatment of children and adolescents with posttraumatic disorder. J Am Acad Child Adolesc Psychiatry 2010; 49: 414e30. Brewin CR. A cognitive neuroscience account of posttraumatic stress disorder and its treatment. Behav Res Ther 2001; 39: 373e93. Carrion V, Weems C, Ray R, Reiss A. Toward an empirical denition of pediatric PTSD: the phenomenology of PTSD in youth. J Am Acad Child Adolesc Psychiatry 2002; 41: 166e73. Cohen JA, Kelleher KJ, Mannarino AP. Identifying, treating, and referring traumatized children: the role of pediatric providers. Arch Pediatr Adolesc Med 2008; 162: 447e52. Cohen JA, Mannarino AP. Psychotherapeutic options for traumatized children. Curr Opin Pediatr 2010; 22: 605e9. DSM-V development. American Psychiatric Association (updated May 2012) www.dsm5.org/ProposedRevisions/Pages/proposedrevision. aspx?rid165 (accessed 10 January 2013). Harmon RJ, Riggs PD. Clonidine for posttraumatic stress disorder in preschool children. J Am Acad Child Adolesc Psychiatry 1996; 35: 1247e9. McCrory E, De Brito S, Viding E. Research review: the neurobiology and genetics of maltreatment and adversity. J Child Psychol Psychiatry 2010; 51: 1079e95. National Institute for Clinical Excellence. Post-traumatic stress disorder: the management of PTSD in adults and children in primary and secondary care. London: NICE, 2005. Rees G, Gledhill J, Garralda ME, Nadel S. Psychiatric outcome following paediatric intensive care unit (PICU) admission: a cohort study. Intensive Care Med 2004; 30: 1607e14. Robb AS, Cueva JE, Sporn J, Yang R, Vanderburg DG. Sertraline treatment of children and adolescents with posttraumatic stress disorder: a double-blind, placebo controlled trial. J Child Adolesc Psychopharmacol 2010; 20: 463e71. Sheeringa M, Zeanah C, Cohen J. PTSD in children and adolescents: toward an empirically based algorithm. Depress Anxiety 2011; 28: 770e82. Stamakatos M, Campo JV. Psychopharmacologic treatment of traumatized youth. Curr Opin Pediatr 2010; 22: 599e644. The National Child Trauma Stress Network is a major resource for parents and professionals. http://www.nctsnet.org/ (accessed 10 January 2013). The Royal College of Psychiatrists has a useful series of downloadable factsheets for parents and young people. http://www.rcpsych.ac.uk/ expertadvice/youthinfo.aspx (accessed 10 January 2013). van der Kolk BA. Developmental trauma disorder: toward a rational diagnosis for children with complex trauma histories. Psychiatr Ann 2005; 35: 401e8. Vasterling JJ, Brewin CR, eds. The neuropsychology of PTSD: biological, cognitive and clinical perspectives. New York: Guilford Press, 2005.
Pharmacological intervention
Although medication (SSRI antidepressants) has proved useful in treating adults, there is insufcient research evidence to support this in children. One small RCT of sertraline was unable to demonstrate a signicant benet and other more favourable reports of successful pharmacological treatment come from case studies and small open label trials. Clonidine, an alpha agonist, has been used to treat trauma symptoms including hyperarousal. Although some form of psychotherapy remains the treatment of choice, medication may be indicated as an adjunct to ease acute symptoms such as sleeplessness, to assist engagement in trauma work or to enhance response. It also has an important role in treating co-morbid disorders such as depression or anxiety, for which there is an established evidence base using SSRI medication.
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Practice points
C
Screening for trauma symptoms should be routine after traumatic medical experiences, stressful events and where there are concerns about possible abuse and domestic violence A detailed psychosocial and trauma history should be taken in conditions presenting with hyperarousal and affect dysregulation, e.g. ADHD Early interventions to support resilience and enhance coping can be highly effective in preventing long-term morbidity Referral to specialist services should be made when PTSD symptoms persist for longer than 1 month
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Chronic Fatigue Syndrome in children and young people

Carrie Mackenzie Alison Wray
Abstract
Chronic Fatigue Syndrome/myalgic encephalomyopathy (CFS/ME) is a relatively common and serious condition in children and young people, having a signicant impact on their physical, emotional and cognitive well being. Although some progress has been made in understanding this perplexing condition there is still a long way to go and controversies persist about terminology, aetiology and treatment. However, there is a general agreement that CFS is a heterogeneous condition and good evidence regarding management. This review explores the best practice approach to assessment, diagnosis and management of CFS/ME in children and young people. Early diagnosis and appropriate multidisciplinary intervention facilitate recovery. There remains a pressing need for research to improve understanding of the condition and thereby ensure all children and young people with CFS/ME are understood and receive effective treatment.
This in turn frequently contributes to a poor patient/carer experience with delay in diagnosis and not infrequent hostility. This is unfortunate as CFS is a serious illness causing significant school absence and has long-term consequences for the educational, social and psychological development of the child or young person if there is no appropriate intervention. It also has an impact on family function and in some instances may result in nancial hardship. As specialist provision is patchy it is important that any professional working with children and young people with CFS has a thorough understanding of the condition and its management.
Denition
The fact that the terminology associated with this condition has created such controversy over a number of years indicates the difculties inherent in labelling such a heterogeneous group of individuals who present with a wide range of physical and emotional symptoms. However the persisting and disabling fatigue universal in this group of children and young people has lead to our team favouring the use of the term Chronic Fatigue Syndrome (CFS). We believe this reduces confusion and assists in acceptance of the diagnosis when used in conjunction with a sympathetic explanation of the very variable nature of the condition which includes both physical and psychological symptoms. The term favoured and adopted by the Royal College of Paediatrics and Child Health (RCPCH) is CFS/ME but in our experience the term ME carries with it a much more physical or medical basis which patients and carers often confuse with conditions such as Multiple Sclerosis (MS). Moreover there are still those who believe that CFS and ME are two separate and distinct entities which share features with other conditions such as Medically Unexplained Symptoms (MUS) or other functional somatic disorders and overlap with chronic pain syndromes. In making the diagnosis, it is crucial to also demedicalize the condition, drawing a line under all that has gone before in terms of investigating the constellation of presenting symptoms and reaching agreement with the affected child or young person and their carers that the way forward is by means of rehabilitation from the point they now nd themselves at. Indeed if this does not happen it may prove difcult to engage the child and family fully in a treatment programme (Table 1).
Keywords Chronic Fatigue Syndrome; myalgic encphalopathy
Introduction
Chronic Fatigue Syndrome (CFS) or Myalgic Encephalomyopathy (ME) is a relatively common and serious condition affecting between 0.1% and 2% of children and young people aged under 18. It received public recognition as a specic clinical condition in 2002 and understanding of the condition is slowly improving. However, it is a heterogeneous condition with no diagnostic tests so diagnosis can be a complex task, particularly as the child or adolescent can present with a range of signs and symptoms. The presentation and course of the condition is often seen to ebb and ow further exacerbating the difculty in predicting, understanding and managing symptoms. Although NICE guidance recommends referral to a paediatrician after 6 weeks of symptoms, the time from onset of symptoms to initial consultation with an appropriate professional is extremely variable and for some children and young people never takes place. Inevitably the lack of positive investigative ndings coupled with the on-going debate about the pathophysiological basis of the condition leads to considerable and confusing debate amongst professionals and lay groups alike.
Epidemiology
Prevalence data for CFS are heterogeneous and confusing as a result of different study methodologies (e.g. settings and diagnostic criteria) and different age limits. A number of studies report prevalence rates from 0.1% to 2% of children under 18. Most studies focus on the condition in adolescents: those reporting prevalence rates in younger children show it to be markedly lower. There remains a distinct need for further research to truly understand the prevalence of CFS in the child and adolescent population. The evidence on gender remains inconclusive. Some studies report no signicant gender difference whereas others report a female excess of 2:1. Our own gures based on 176 children and young people under 16 show a ratio of two boys to every three girls.
Carrie Mackenzie MBchB FRCPCH DCCH MD is Consultant Paediatrician in the Childrens Hospital, Shefeld, UK. Conicts of interest: none declared. Alison Wray BA MPhil CPsychol is Principal Clinical Psychologist in the Dept of Clinical Psychology at the Childrens Hospital, Shefeld, UK. Conicts of interest: none declared.
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Denition of CFS in children and young people

C
history and often normal examination ndings are key in making the diagnosis (see Table 2).
NICE guidance e CFS/ME involves a complex range of symptoms that includes fatigue, malaise, headaches, sleep disturbance, difculties with concentration and muscle pain. The pattern and intensity of symptoms vary between people and during the course of each persons illness. RCPCH e generalized fatigue persisting after routine tests and investigations have failed to identify an obvious underlying cause. The fatigue is likely to be associated with other classical symptoms such as difculty in concentrating and disturbed sleep patterns and is classically exacerbated by effort (both mental and physical).
Investigations
As a minimum we require that the child or young person will have undergone the screening investigations set out in the NICE guidance and that the results of these tests are normal when seen for initial assessment by the specialist CFS team. Diagnosis marks the start of a long and challenging journey for the child or young person and their carers. It is important to demedicalize and demystify the condition and positively and condently recognize the symptom clusters shared by CFS sufferers. A further key feature of the initial consultation is to explain the nature of the condition, acknowledge the difculties they have encountered on their journey thus far and reassure the patients and their carers that the expectation is that their condition will improve and that in our experience the vast majority of children and young people with CFS recover and go on to achieve academic qualications. 99% of young people aged 16 seen by our specialist team achieved some academic qualications and were able to progress to the next stage of their education or training. The vast majority of children and young people will not require transition to adult CFS services. About 5% continue to have a persisting and disabling illness. I visited the clinic and was diagnosed with CFS. It was a relief to know that my debilitating symptoms had a name. After more than 9 months of feeling so terrible someone could nally tell me what was wrong. It helped when I knew I had CFS. It was far better knowing than being left worrying or trying to guess. The fact that CFS is a heterogeneous group is now generally agreed and evidence is growing to support this. One study has identied three phenotypes in CFS in children that are differentially associated with severity; the musculoskeletal phenotype is
Table 1
Pathology and pathogenesis

There has been a plethora of research into CFS but the very wide and varied nature of the hypotheses being pursued indicates the clear lack of understanding of the pathophysiological basis for this condition. Many of our children and young people can describe an intercurrent illness immediately prior to the onset of their fatigue but the exact nature of this usually relatively minor illness is often unclear and it is by no means a universal precursor. It may well be that some external trigger provokes an autoimmune process in genetically predisposed individuals but the evidence for this does not yet exist and it seems likely that the causation is multi-factorial. Various aetiological factors such as level of exercise taken, personal and maternal psychological well being, gender, socioeconomic status, birth weight, birth order, coexistence of atopic conditions, exposure to certain viruses, school attendance and achievement have all been considered but as yet their contribution, if any, remains unclear. Any hope of intervention to prevent CFS in future generations will demand a better understanding of the aetiological factors at play. However the once popular stereotypical picture of the affected individual being female, highly intelligent and high achieving is not borne out by our experience.
Diagnosis and differential diagnosis

The diagnosis of CFS relies not only on the exclusion of other pathology by means of appropriately directed investigations but also on the presentation of symptom clusters shared by other children and young people with the condition in addition to persistent and disabling fatigue. These symptoms may classically include: malaise, sleep disorder, headache, dizziness, poor temperature control, abdominal pain, nausea, anorexia, sore throat, tender lymphadenopathy, arthralgia, myalgia and cognitive difculties such as poor short-term memory, impaired concentration, anxiety and low mood. Since many of these symptoms overlap with diagnoses relevant to many disciplines such as: Gastroenterology, Rheumatology, Endocrinology, Neurology, Infectious Diseases and Immunology, Oncology and Haematology as well as Childhood and Adolescent Mental Health it is beholden upon us to ensure that appropriate paediatric subspecialists have been consulted and the necessary investigations undertaken. However in the absence of any other disease, the
Investigations
These tests should usually be done: C Urinalysis for protein, blood and glucose C Full blood count C Urea and electrolytes C Liver function C Thyroid function C Erythrocyte sedimentation rate or plasma viscosity C C-reactive protein C Random blood glucose C Serum creatinine C Screening blood tests for gluten sensitivity C Serum calcium C Creatine kinase C Serum ferritin Table 2
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associated with muscle and joint pain and these children have worse fatigue. The migraine phenotype which is associated with headache, abdominal pain, nausea and dizziness and noise sensitivity is associated with a lower level of physical function, worse pain and lower school attendance. The sore throat phenotype is associated with sore throat and tender lymph nodes and is the least severe.
Impact of the condition

It is one of the loneliest illnesses in the world because we dont have anything to show for it. Everyone involved with CFS agree that the condition has a profound impact, not only on the child or young person, but also on their families. It is associated with considerable school absence, social isolation, loss, changes in family relationships and parental time off work. My symptoms meant that I started to miss a lot of school e I just didnt have the energy to make it through the day. My daughter lost all her spark.
learn to self manage the condition. At the outset of treatment this commonly involves three aspects: Sleep hygiene Activity/energy management School liaison In the absence of a clear understanding of the cause of CFS we make use of a number of analogies which t well with patients experience of the condition. We explain to the child that their body is like a battery that has lost its charge and now requires recharging. The ways to do this are good sleep hygiene and activity management or GET. We also nd that the idea of vicious cycles arising as a consequence of the condition is a useful concept with which families can identify (Figure 1).
Sleep hygiene
Disordered sleep patterns are commonly found in CFS including day/night reversal, interrupted sleep, insomnia or hypersomnia. It is therefore important to establish effective and positive sleep routines which include: Establishing a regular waking time Avoiding prolonged sleep and day time sleeps Always sleep at night in own bedroom and avoid use of computer/TV prior to bed Relaxation to aid falling asleep and consistent bedtime routine Melatonin and/or amitriptyline are used in some case to support good sleep hygiene and improve the quality of sleep.
Management
Once children and young people and their families have gained an understanding of the condition, the basis of treatment and an optimistically realistic expectation of what the future might hold, a co-ordinated multi-disciplinary approach to management is required in the majority of cases. Commonly, occupational therapist, physiotherapist, nurse specialist and clinical psychologist may need to be involved. Evidence based guidelines recommend the following: Activity management: this is a person centred, goal directed approach to managing a child or young persons symptoms. It uses analysis of activity and graded activity through learning the skills of pacing to improve physical and cognitive function. Cognitive behaviour therapy: this is an individualized psychological therapy which incorporates two major components: the cognitive element which focuses on the identication and modication of thoughts, beliefs and assumptions which may shape the child or young persons understanding of their condition, and the behavioural element which aims to gradually and consistently introduce a change in behaviour e.g. an increase in activity or return to school. A CBT model can include treatment of accompanying anxiety or depression and can be tailored to include involvement of the family. Graded exercise therapy (GET): is a structured and supervised programme of exercise agreed between doctor, therapist (usually a physiotherapist) and the child or young person. It is based on their current level of ability. Intensity and duration of exercise begin at a very low level and are increased very slowly depending on progress. The aim of GET is to increase tness and stamina. The primary aim of management is to re-establish the child or young person in a sustainable routine and then to help them
Activity/energy management
The young person needs to keep a diary to establish their baseline of activity. Activity meaning everything that they do from having a shower being on the computer to going to school. Realistic goals then need to be agreed with the young person so
Figure 1 Vicious cycle.
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they avoid the boom and bust pattern of activity which hinders recovery. It can be useful to think in terms of the different energy demands of situations e.g. high or low. Increase in activity levels needs to take place gradually and be carefully monitored. We would recommend a 15% increase in activity e.g. if able to read for 30 minutes a day increase to 35 minutes.
Top tips for management from our team

These patients take time: C in clinic so it is useful to book appointments at the end of clinic C to recover Stay condent when the inevitable relapse occurs Remember progress is always slower than you expect There is no quick x Involve parents Table 3
School liaison
CFS has a negative impact on education and many children and young people are unable to attend full time school. A crucial element of any successful management plan, therefore, is the establishment of a sustainable educational routine. This may involve the home tuition services initially, followed by a carefully managed plan for school re-integration. It is very likely to involve part-time school attendance. A recent study suggests that the factor most strongly associated with reduced school attendance is poor physical function. It is therefore important to ensure that the child or young person is fully engaged with treatment to improve physical function. However it is also necessary to be aware of the cognitive impact of CFS and to ensure teachers are informed of the effects of CFS on memory, information processing and concentration. Children with CFS are entitled to exam concessions when taking public exams if requested by a doctor. The key professional needs to be prepared to put considerable time into liaizing with educational services. In our experience this patient group needs more time for liaison than other children with a chronic illness.
Provision of services
The provision of services for children and young people with CFS remains patchy. Despite NICE guidance which recommends referral to specialist services immediately if severely affected, within 3 months if moderately affected and within 6 months if mildly affected, there are still only 13 specialist teams in Britain and these are often small with limited capacity. The burden of treating this condition for the majority therefore lies with paediatricians and local therapy services. However, the specialist teams now have considerable experience of this perplexing, enigmatic condition. We would encourage professionals working with CFS to consult with a specialist team. In recognition of the scarcity of resources we have been instrumental in setting up a clinical network in North and Central England for those working with CFS in order to improve knowledge and services.
Management of specic physical symptoms

We nd that as the child or young person learns to manage their fatigue effectively and their well being begins to improve so other troubling symptoms such as nausea, abdominal pain, myalgia and joint pain all decrease. We therefore tend to avoid specic pharmacological agents. Gastrointestinal symptoms can be managed through diet and ensuring healthy eating and adequate uid intake avoiding caffeine and sugary drinks. Small frequent meals are often better than three heavy meals. Pain is managed with simple analgesics like paracetamol and/or ibuprofen. In addition, relaxation and cognitive behaviour techniques may be considered in parallel to improve pain.
Prevention of the primary disease and disease complications and disabilities

In the absence of a recognized aetiology the search for a method of primary prevention of the condition seems futile. However prompt recognition of the condition and onward referral to a specialist service where available, with expertise in managing the condition seems likely to be in the best interest of the affected individuals and their families. Since CFS affects not only the child or young person but also those around them, the sooner appropriate education and support is put in place the more effective rehabilitation can be and the less likely physical and psychological co-morbidities are to become entrenched. The absence from school and lack of socialization with peers leave these children and young people at very real risk of long-term psychological and emotional difculties which early recognition and intervention might go a long way to ameliorate or even prevent.
Role of the family

The role of the family is a crucial in helping a child or young person learn to manage this condition. Family life is affected and families have to adjust their expectations of what they can do together. Some parents have to stop work in order to care for their child. Support and encouragement from family members is valued by children and young people; it is therefore important that parents understand the condition fully and are involved in treatment. The package of care we have developed involves an initial period of treatment of six sessions every 3e4 weeks. At the end of this we hold an MDT review and a further six sessions can be offered to consolidate activity management and continue with school liaison. Other contributing factors may have emerged by this stage such as anxiety, low mood or family factors. It is then appropriate to arrange CBT or family therapy (Table 3).
Conclusion
CFS is a disabling and serious condition affecting physical and mental function and compounded by the uncertain and unpredictable course of the illness. There is still much to understand about this enigmatic condition and both clinicians and researchers have a duty to develop understanding of the causes and course of the condition. However, it is clear that early diagnosis and appropriate management greatly facilitate recovery, and the majority of children and young people make good recoveries.
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OCCASIONAL REVIEW
To me keeping positive and determination I think were really important in my recovery. I think it was really important to keep trying e you go through such bad times and good times with this condition it is important to know you will get there eventually.
Practice points
C
A
FURTHER READING Chalder T, Goodman R, Wessely S, Meltzer R. The epidemiology of fatigue in children. Br Med J 2003; 327: 654e5. Chalder T, Hussain K. Self-help for chronic fatigue syndrome. Blue Stallion Publications, 2002. Collingridge E. Severe ME/CFS: a guide to living association of young people with ME 2010. Crawley E, Sterne JAC. Association between school absence and physical function in paediatric chronic fatigue syndrome/myalgic encephalopathy. Arch Dis Child 2009; 94: 752e6. Davies S, Crawley E. Chronic fatigue syndrome in children aged 11 years old and younger. Arch Dis Child 2008; 93: 419e21. Garralda M, Chalder T. Practioner review: chronic fatigue syndrome in childhood. J Child Psychol Psychiatry 2005; 46: 1143e51. Haig-Ferguson A, Tucker P, Eaton N, Hunt L, Crawley E. Memory and attention problems in children with chronic fatigue syndrome or myalgic encephalopathy. Arch Dis Child 2009; 94: 757e62. Jelbert R, Stedmon J, Stephens A. A qualitative exploration of adolescents experience of chronic fatigue syndrome. Clin Child Psychol Psychiatry 2010; 15: 267e83. National Institute for Health and Clinical Excellence. Chronic fatigue syndrome/myalgic encephalomyelitis (or encephalopathy): diagnosis and management of CFS/ME in adults and children. Clinical guideline no 53. London: NICE, 2007. Royal College of Paediatrics and Child Health. Evidence based guidelines for the management of CFS/ME in children and young people. London: RCPCH, 2004. Van de Putte EM, Engelbert R, Kuis W, Sinnema G, Kimpen J, Uiterwaal C. Chronic fatigue and health control in adolescents and parents. Arch Dis Child 2005; 90: 1020e4. Viner R, Hotopf M. Childhood predictors of self reported chronic fatigue syndrome in adults: national birth cohort study. Br Med J 2004; 329: 941e3.
Children and young people with CFS do recover with good outcomes CFS is a clinically heterogeneous syndrome characterized by persisting and disabling fatigue. It is usually associated with a cluster of other troubling symptoms Diagnosis is made by a paediatrician once other potential disorders have been excluded CFS is a genuine physical illness and this needs to be communicated to child and family CFS is increasingly being recognized as a cause of signicant school absence/poor school attendance in children and young people Demedicalization of symptomatology is essential and benecial and in particular the need to draw a line under previous investigations and management in order to move forward is crucial The absence of a magic wand/pharmacological cure requires to be explained from the outset Management requires an MDT approach and both CBT, GET and activity management are recommended treatments Commitment to the acceptance of the need for rehabilitation by means of energy management and the adoption of good routines on the part of patients and carers is very important Professionals need patience and condence and to be prepared for relapses It is important to engage family members as well as the child or young person
Acknowledgements
We are grateful to the young people and their parents for permission to use their comments in this review.
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PERSONAL PRACTICE
Attention decit hyperactivity disorder

Paul McArdle Denitions
Attention decit hyperactivity disorder (ADHD) or hyperkinetic disorder is a neurodevelopmental disorder related to immature or different functioning of the brain compared to non-affected controls. It is characterized by well-known symptoms encompassing inattention, impulsivity and hyperactivity and is sometimes explained as an undue aversion to delay. In both ICD and DSM systems, impulsivity includes behaviours such as outing of social rules putting social impairment at the heart of the DSM system. The WHO adds, hyperkinetic children are often reckless and impulsive, prone to accidents, and nd themselves in disciplinary trouble because of unthinking (rather than deliberately deant) breaches of rules. Especially when compounded with substance misuse, impulsivity in these terms contributes to injury and mortality.
compensatory but inefcient developments in other brain regions. Recent studies of brain metabolism also emphasize the role of the subcortical reward pathways perhaps explaining preference for immediate reward and the common complication of substance misuse.
Comorbidity
Perhaps because brain areas implicated by imaging point to diffuse effects, ADHD is often associated with other developmental anomalies such as learning and language including reading delays, decits in social interaction that may range from inability to abstract [hampering adolescents in grasping peer banter or the increasing abstractions of secondary school curricula], and which if severe can resemble autism; conduct problems, substance misuse and aggression as well as, consequent upon multiple adversities and disappointments, anxiety and depression.
Genes and environment

ADHD travels in families, and appears to be substantially genetically determined. Although a multi-gene disorder, alterations in dopamine connectivity inuenced by genetic variations such as the 7-repeat allele of the dopamine receptor gene are associated with increased risk of ADHD. Gene-environment correlations, genetically inuenced individual variations in exposure to risky or protective environments may explain some of the association of ADHD with difcult environments e it may not always or often be the parental disharmony or inconsistent discipline that causes the ADHD or linked conduct disorder, but at least in part genetically inuenced behaviour shaping risk environments. This is a clinically relevant insight that should preclude exclusive focus on altering family dynamics and, at the extreme, the parent blaming of the past. Nevertheless, as in the case of in-utero alcohol, later lead exposure and severe early deprivation, environment may act to impair brain development. Late adopted children who had experienced early adversity may present with an ADHD-like syndrome that is particularly refractory to intervention. More speculatively, the disappearance of simple, supervised manual tasks from post-globalization Western economies, and the increased requirement for educational success (to compete in a knowledge economy), planfulness (to make choices as part of a business or professional project) or social skills (for customers relations, teamwork) may specically disadvantage people with ADHD. This may explain what sometimes appear to be a modern epidemic and a new adult syndrome. Working in highly structured, supervised concrete occupations as in traditional assembly line manufacturing, mining or the military, with the emphasis on physical strength and sometimes courage, ADHD appears to be no handicap.
Presentation
ADHD is typically evident in different settings, at home and in school and, later, in the workplace. It is often observable in the clinic but the signs can be subtle and their absence does not rule out the diagnosis. The attention decit is often most obvious during sustained effortful concentration; it may not be prominent when particularly engaging activities such as novel toys, computer games, cognitive assessments with positive immediate feedback or sympathetic supervision are available. It is possible to imagine the amelioration of symptoms under these favourable conditions as similar to the normal responses of very young children.
Age of onset
The early onset (before the age of 7 years) criterion has been removed from DSM V in order not to exclude adults who may not recall their early years. Nevertheless, early onset remains a clinical reality. It is suggested that, rather than causing abnormal brain development, even perinatal difculties might be the earliest manifestation of abnormal brain development or preexisting genetic risk. Among those who present later, some able young people have successfully drifted through primary and early secondary school only to founder when effort is demanded.
Neurodevelopment
The syndrome is associated with delayed or impaired brain myelination affecting frontal areas especially and perhaps
Understanding children
Clinically, the predicament of children with ADHD can often be grasped as the experience of being developmentally disadvantaged, failing many of the social and intellectual challenges of childhood and adolescence, in effect being taught failure in school. Harking back to the moral disorder described by Still of Stills disease fame, adults may judge apparent deliberate
Paul McArdle MB MRCPI MRCPsych DCH is Honorary Senior Lecturer at the Institute of Health and Society, Newcastle University, and Consultant Child and Adolescent Psychiatrist at Ferndene Inpatient Young Peoples Inpatient Service, Northumberland, UK.
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PERSONAL PRACTICE
outing of rules or underachievement as deliberate willfulness or laziness, and respond with serial reprimand. Sadness, personal disappointment and ultimately disaffection with all its consequences may ensue. Healthcare staff need to be mindful of such a previously misunderstood life story, should attempt to grasp how ADHD and comorbidity shaped a series of adverse life experiences with which the young person, and often their family, has struggled unsupported, to cope. Elucidate too the educational failure often missed by authorities preoccupied by matters of discipline. This can lead to effective advocacy in terms of helping parents and schools see more clearly the childs predicament, generating additional educational support or constructing more fruitful encounters with teachers.
Diagnosis
Diagnosis depends on experienced clinical evaluation of symptoms rather than signs so that history is more important than direct observation. As they can judge behaviours against their knowledge of age appropriate norms, the observations of teachers are particularly useful corroboration. Even for adult patients, corroboration by a family member, especially to identify early symptoms, is helpful. Teacher questionnaires can usefully quantify symptoms, their change over time and differentiate from oppositional behaviour presenting largely in the home. Some children with learning disability and autism present in a supercially similar way so assess overall development.
Stimulants are associated with pressor and chronotropic cardiac effects but the risk of sudden death is not elevated among those receiving treatment compared to the general child population. However, among those receiving stimulants or atomoxetine a small minority may become hypertensive. Hence it is crucial to assess underlying cardiac health by history and examination including BP. There appears to be no effect on QT interval and no indication for ECG monitoring. A large US trial was unable to detect long-term positive effects of methylphenidate; it is important to attempt to withdraw medication from time to time to assess continued efcacy. Some adolescents misuse illicit substances and alcohol and a small illicit market for methylphenidate exists. Apart from cannabis and alcohol, which do not appear to interact, many illicit substances are stimulants and in principle could add to cardiac risk in combination with prescribed agents. This underlines the importance of BP monitoring and appropriate questioning. In these cases, a judgment whether the agent is still contributing to positive adjustment, or merely helping a young person be a more effective dealer, or in the case of a child out of school, merely helping him/her concentrate on day time TV or computer games, and crucially whether someone responsible is monitoring the dispensing of the prescribed agent, is required. If in doubt, it is best to stop the prescription because it is failing in its purpose. A
Intervention
NICE recommends parenting programmes as a rst stage intervention but these appear to have little inuence on school behaviour. Although proof is lacking, drawing on the wider literature on educational success and experience, it is likely that teachers who can relate to the child despite his/her difculties, a predictable routine, and a curriculum somewhat geared to the childs strengths, including a bias towards the technical (hands on, real not abstract concepts) are important. In some respects, the importance of structure and the teachers relationship with the young person is also reminiscent of the needs of younger children. It is possible that the willingness of schools to listen to clinicians and to adapt to the needs of this substantial minority of children is the key to a public health approach to ADHD. It is likely too that some types of within-school programmes are potentially helpful, although at present evidence is lacking. Methylphenidate increases the intrasynaptic levels of dopamine and norepinephrine and the efciency of the brain when faced with a cognitive task. Its utility is not to sedate or chemically cosh but to help children concentrate on the tasks set for them. Among school age children, a slow release preparation can be used to enable attention during the school day, sometimes supplemented by an immediate release dose in the evening for homework. Among older adolescents an immediate release preparation can be timed to focus on assignments or other tasks requiring discrete periods of sustained effortful concentration.
FURTHER READING Breslin C, Pole J. Work injury risk among young people with learning disabilities and attention-decit/hyperactivity disorder in Canada. Am J Public Health 2009; 99: 1423e30. Castellanos X, Rapoport J. Longitudinal mapping of cortical thickness and clinical outcome in children and adolescents with attentiondecit/hyperactivity disorder. Archiv Gen Psychiatry 2006; 63: 540e9. Li D, Sham PC, Owen MJ, He L. Meta-analysis shows signicant association between dopamine system genes and attention decit hyperactivity disorder. Hum Mol Genet 2006; 15: 2276e84. Graham J, Banaschewski T, Buitelaar J, et al. European guidelines on managing adverse effects of medication for ADHD. Eur Child Adolesc Psychiatry 2011; 20: 17e37. Taylor E, Rodgers J. Practitioner review: early adversity and developmental disorders. J Child Psychol Psychiatry 2005; 46: 451e67. Taylor E, Dopfner M, Sargeant J, et al. European clinical guidelines for hyperkinetic disorder, rst upgrade. Eur Child Adolesc Psychiatry 2004; 13(suppl 1): 1e29. Taylor E, Sandberg S, Thorley G, Giles S. The epidemiology of childhood hyperactivity. Oxford University Press, 1991. Thapar A, Langley K, Owen M, ODonovan M. Advances in genetic studies on attention decit hyperactivity disorder. Psychol Med 2007; 37: 1681e92. Volkow ND, Fowler JS, Wang G-J, et al. Methylphenidate decreased the amount of glucose needed by the brain to perform a cognitive task. PLoS ONE 2008; 3: e2017. http://dx.doi.org/10.1371/journal.pone. 0002017.
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SELF-ASSESSMENT
Self-assessment
Questions
Case 1 A term female infant is born by normal vaginal delivery to a 28-year-old mother. At 30 minutes of age she is noted to have a distended abdomen and you are asked to review her on labour ward. She is the second baby to parents whose previous child is 3 years old, alive and well. The mother has no signicant past medical history and smoked 5e10 cigarettes a day during pregnancy. The pregnancy was unremarkable with normal antenatal foetal anomaly scans. Labour was spontaneous, membranes were articially ruptured approximately one hour before delivery in hospital. On examination the baby is pink in air, but appears to be in discomfort. Cardiovascular, respiratory and neurological examination is unremarkable. Abdominal examination reveals a grossly distended abdomen that is tense and tender e this makes it difcult to determine whether there are any masses or organomegaly. Few bowels sounds are present. The anus appears patent and normally positioned. Question 1 Which of the following would you include in your differential diagnosis? a. Hirschsprung disease b. Pyloric stenosis c. Meconium plug syndrome d. Midgut malrotation with volvulus e. Ano-rectal malformation f. Meconium ileus g. Oesophageal atresia with proximal tracheooesophageal stula Your initial management plan includes insertion of a nasogastric tube, intravenous line and intravenous uids, bloods including group and save, a chest X-ray and abdominal X-ray and a surgical review. The abdominal X-ray (Figure 1) shows multiple gas lled distended loops of bowel. There are no visible air-uid levels and no gas in the rectum. The baby is reviewed by the surgical team and an abdominal ultrasound scan is obtained. This shows normal liver, kidneys and spleen with large loops of meconium lled bowel and visible peristalsis. A contrast enema (Figure 2) demonstrates a microcolon with evidence of meconium in the distal small bowel resulting in a double contrast outline. A diagnosis of meconium ileus is made and the baby is prepared for laparotomy. At laparotomy there is volvulus of the mid small bowel with gangrenous areas. Lumps of meconium are impacted in the distal small bowel and the colon is small. The dead bowel is removed and a double stoma formed as the surgeon is unable to decompress the distal colon. The baby returns to the neonatal unit with a plan to wean off ventilation overnight and to remain nil by mouth until the stoma is working. Washouts of the distal loop are started at 5e7 days and the plan is to re-join the stoma after 2e3 weeks. In addition the baby is treated with 10 days of intravenous antibiotics. Question 2 At this stage what further investigations are appropriate to explore the diagnosis of Cystic Fibrosis? a. Faecal elastase b. Sweat test c. Lung function tests d. Blood Immunoreactive trypsinogen (IRT) levels e. Full CF genotype f. Chest X-ray Case 2 A 9-year-old boy with CF is admitted with a history of haemoptysis. He was diagnosed with CF at 10 months due
Katrina Cathie BM (Hons) MRCPCH is a Paediatric Clinical Research Fellow at University Hospital Southampton NHS Foundation Trust, UK. Conict of interest: none. Gary Connett MB ChB MD FRCPCH is a Consultant Respiratory Paediatrician at University Hospital Southampton NHS Foundation Trust, UK. Conict of interest: none. Julian Legg MA MB BChir MD FRCPCH is a Consultant Respiratory Paediatrician at University Hospital Southampton NHS Foundation Trust, UK. Conict of interest: none.
Figure 1
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SELF-ASSESSMENT
cm length), ante grade blood ow in the portal vein and no varices. He has a gastroscopy a week later, which shows no evidence of oesophageal varices. He is discharged with no on going evidence of haemoptysis. Question 2 What treatment would you consider using in a patient with CF to treat their liver disease? a. Vitamin K b. Vitamin E c. Vitamin A D d. Ursodeoxycholic acid e. Creon capsules f. DNase g. Insulin h. Advice to avoid alcohol i. Immunization against hepatitis B and C Question 3 What are the potential complications of CF related liver disease? a. Splenomegaly b. Ascites c. Oesophageal varices d. Jaundice e. Coagulation problems f. Hypersplenism g. Encephalopathy This patient continues to suffer with frequent respiratory exacerbations requiring admission to hospital for IV antibiotics. His lung function is declining and there is evidence of progressive liver disease. At the age of 16 he has an MRI of his abdomen (Figure 3) which shows a large spleen of 26 cm with a leash of blood vessels behind it and no varices.
Figure 2
to respiratory symptoms. He grows pseudomonas intermittently, treated with IV antibiotics. At the age of 6 he was found to have radiological and biochemical evidence of liver disease with portal hypertension and gallstones and has been on the appropriate medication. In the past 24 hours he has been coughing up blood, approximately 1 teaspoon initially, then sputum containing small clots and fresh blood. He has no history of recent nose bleeds, vomiting or melaena. On examination he is cardiovascularly stable with no respiratory signs. His abdomen is soft and non-tender with a liver edge palpable 4 cm below the costal margin and a 4 cm spleen. Sputum is green and contains blood clots. A full blood count shows Haemoglobin (Hb) 112 g/L, White Cell Count 3.1 109/L, neutrophils 0.7 109/L and platelets 116 109/L. Question 1 Your immediate management plan should include a. Bloods including G S b. Discussion with senior colleague c. Urgent gastroscopy d. Abdominal USS e. Abdominal X-ray (AXR) f. Sputum MC and S g. IV antibiotics h. Oral antibiotics i. Nil By Mouth (NBM), Nasogastric tube (NGT) and intravenous (IV) uids j. Blood transfusion The patient is kept on the ward for on going assessment and treatment. He undergoes an abdominal USS, which shows liver cirrhosis, hepatomegaly and splenomegaly (at least 18
Figure 3
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SELF-ASSESSMENT
The liver shows marked surface nodularity consistent with cirrhosis. At clinic he and his parents want to discuss whether he might be eligible for lung transplantation. Question 4 Which of these is NOT a criterion for lung transplant referral in CF? a. Signicantly reduced lung function, usually with FEV1 less than 30% predicted. May include rapidly declining FEV1 if still more than 30% predicted. b. Severely impaired quality of life c. Oxygen dependant (resting SpO2 less than 90%) d. Need for non-invasive ventilation as an adjunct for physiotherapy e. Recurrent pneumothorax in advanced disease f. Severe haemoptysis not controlled by embolization Question 5 Which of these is NOT an absolute exclusion criterion for lung transplant in CF? a. Untreatable advanced dysfunction of another organ (excluding hepatic when combined liver/lung transplant could be considered) b. CF related diabetes mellitus requiring insulin therapy c. Untreated mycobacterium tuberculosis d. Invasive pulmonary aspergillosis e. Malignancy in the last 2 years f. Colonization with burkholderia cenocepacia g. Child does not want procedure despite receiving the information
Answers
Case 1 1. a, c, d, f Pyloric stenosis presents later (usually around 6 weeks), Ano-rectal malformation with a patent, normally sited anus would not result in abdominal distension and in cases of oesophageal atresia, abdominal distension only occurs if the stula is distally sited. 2. e Faecal elastase is not routinely used for CF diagnosis and is only of value when the infant is established on full feeds, sweat test is the gold standard for diagnosis over 2 weeks of age (see below for more details), standard lung function tests are not performed in newborn babies, there is no value in an IRT test in babies with meconium ileus as the levels are articially low and a chest X-ray may be useful if there are respiratory problems, but will not diagnose CF. Explanation Meconium ileus: meconium is a combination of bile acids, salts and debris shed from the mucosa of the intestine while the baby is in utero. Most babies pass meconium within 6 hours and 99% of term infants have passed meconium within 24 hours of birth. In meconium ileus there is
impaction of thick meconium in the distal ileum resulting in intestinal obstruction and abdominal distension. Abdominal X-ray shows numerous dilated air-lled loops of small bowel suggesting distal small bowel obstruction. Contrast enemas are required to determine the diagnosis of meconium ileus. This typically shows a microcolon representing an underused bowel. Contrast is reuxed into the dilated terminal ileum revealing pellets of meconium thus establishing the diagnosis. Approximately 15% of CF patients present with meconium ileus. Prior to the introduction of newborn screening, it was hypothesized that those with meconium ileus were conferred potential long term benets as a result of early diagnosis and the prompt initiation of appropriate CF therapy. Recent studies have demonstrated that children presenting with meconium ileus have similar long term outcomes to those presenting later in childhood by different means, suggesting that early diagnosis counterbalanced the deleterious effects of MI. Since the introduction of newborn screening, however, it has been suggested that babies with meconium ileus are likely to fall into a poorer prognostic group and have a CF phenotype with more severe respiratory dysfunction. Newborn screening: in the UK approximately 1 in every 2500 newborn babies have CF. Early diagnosis through screening enables the timely institution of appropriate therapies including pancreatic enzyme supplements, antibiotics and physiotherapy. Such early interventions have been shown to result in improved growth, better lung function and ultimately culminate in longer survival. A blood spot is collected between days 5 and 8 of life and analyzed for immunoreactive trypsinogen (IRT). If the IRT level is raised then the blood spot is tested for the UKs 4 most common CF genes followed by further analysis depending on results (http://newbornbloodspot.screening.nhs.uk/cf). Sweat test: the sweat test remains the gold standard for the diagnosis of CF. Topical pilocarpine is used to induce sweat production which is then analyzed for chloride concentration (raised in CF). A variety of techniques are available with recent evidence suggesting that the Macroduct sweat collection system is most reliable in the neonatal period. Indications for having a sweat test are: 1. A positive newborn screening test (an independent diagnostic test is essential in all screened positive infants) 2. Presenting features (phenotype) suggestive of CF (whether or not newborn screening has been carried out): a. Babies with CF may present with meconium ileus in the newborn period. They may have frequent chest infections, and often fail to thrive and have bulky, pale stools. b. In older children, the major manifestations include chronic sino-pulmonary disease with infection and malabsorption due to pancreatic insufciency. Finger clubbing is often present and Staphylococcus aureus or Pseudomonas aeruginosa is frequently cultured from the sputum.
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SELF-ASSESSMENT
Sweat tests can be performed from 2 weeks of age in babies over 3 kg and who are well. Earlier testing can lead to misleading results, because sweat chloride concentration in healthy newborns gradually decreases over the rst weeks of life. If necessary, it can be carried out from 7 days of age, but may need repeating if an insufcient quantity of sweat is obtained.
REFERENCES
transplant assessment, although the child may not necessarily be listed. 5. b CF related diabetes mellitus requiring insulin therapy is not a contraindication to lung transplant. Explanation Haemoptysis in CF: initial assessment should carefully distinguish between haemoptysis and haematemesis, particularly in patients with a history of liver disease and who are at risk of bleeding varices. In patients with chronic pseudomonas infection, blood streaked sputum is common as a result of surface bleeding from areas of chronic airways inammation. Bleeding might be indicative of an acute respiratory exacerbation, so sputum should be cultured and antibiotics considered. Large haemoptyses typically occur as a result of erosion into hypertrophied bronchial arteries. If a patient has a totally implantable venous access device in-situ then the possibility of pulmonary embolism should be considered (although sudden chest pain and dyspnoea are more typical). Initial management of a patient coughing up frank blood should include appropriate resuscitation, sputum culture, chest X-ray and taking blood for full blood count, clotting studies, and group and save or cross match. Signicant anaemia should be corrected by transfusion and the patient commenced on broad spectrum IV antibiotics. If the bleeding doesnt settle or if there has been a large haemoptysis bronchial artery embolization should be considered to prevent further life threatening episodes. Tranexamic acid is an acceptable adjuvant treatment. Other treatment options are largely experimental and include intravenous vasopressin, bronchoscopy for clot removal, tamponade of bleeding sites and attempted haemostasis using thrombin glue or vasoconstrictor lavage. CF related liver disease: mild CF related liver disease occurs in up to a third of CF patients as a result of absent or dysfunctional Cystic Fibrosis Transmembrane Regulator (CFTR) protein in the biliary epithelium. Thickened secretions plug the intrahepatic bile ducts resulting in focal biliary cirrhosis. 1e2% of patients progress to severe complications for which transplantation might be considered. Mutations in other genes such as the alpha-1antitrypsin AT-Z mutation are associated with a greater risk of developing liver disease. Liver disease is independently associated with a history of meconium ileus and pancreatic insufciency. Most patients with CF related liver disease are identied in the rst decade of life. The usual presentation is with an enlarged liver on clinical examination or routine abdominal ultrasound scanning, with or without associated abnormalities in liver function tests (LFTs). Patients present less frequently with complications of liver disease such as splenomegaly with or without hypersplenism, portal hypertension and upper gastrointestinal variceal bleeding. Patients should be examined for an enlarged liver and spleen at routine clinic visits. Often the left lobe is more
Dijk FN and Fitzgerald DA. The impact of newborn screening and earlier intervention on the clinical course of cystic brosis. Paediatr Respir Rev 2012;13:220e5. Eng W, LeGrys VA, Schechter MS, Laughon MM and Barker PM. Sweat-testing in preterm and full-term infants less than 6 weeks of age. Pediatr Pulmonol 2005;40:64e7. Johnson JA, Bush A and Buchdahl R. Does presenting with meconium ileus affect the prognosis of children with cystic brosis? Pediatr Pulmonol 2010; 45:951e8. Kappler M, Feilcke M, Schroter C, Kraxner A and Griese M. Long-term pulmonary outcome after meconium ileus in cystic brosis. Pediatr Pulmonol 2009; 44:1201e6. Laguna TA, Lin N, Wang Q, Holme B, McNamara J and Regelmann WE. Comparison of quantitative sweat chloride methods after positive newborn screen for cystic brosis. Pediatr Pulmonol 2012; 47:736e42. Li Z, Lai HJ, Kosorok MR, Laxova A, Rock MJ, Splaingard ML et al. Longitudinal pulmonary status of cystic brosis children with meconium ileus. Pediatr Pulmonol 2004; 38:277e84. http://newbornbloodspot.screening.nhs.uk/cf
Case 2 1. a, b, f, g Urgent gastroscopy is not indicated as the patient is stable. An AXR will not provide further additional diagnostic information that will help identify the cause of the bleeding. The patient needs IV antibiotics, not oral, in view of the likelihood that haemoptysis is caused by a severe pulmonary exacerbation. NBM, NGT and IV uids are not indicated at this stage as the history would suggest haemoptysis rather than haematemesis. A blood transfusion is not indicated at this point as the patient is stable and the Hb is 112 g/L. 2. a, d, h, i Vitamins A, D & E are supplements used for CF patients with pancreatic insufciency who cannot absorb these fatsoluble vitamins. Creon capsules contain pancreatin granules consisting of porcine-derived pancreatic amylase, lipase and protease to facilitate digestion for patients with pancreatic insufciency. DNase is a nebulized treatment used for mucolysis and to aid expectoration of sputum. Insulin is used for treating CF related diabetes. 3. aeg 4. d The need for non-invasive ventilation as an adjunct to physiotherapy is not a criteria for referral for transplant assessment. If the need for NIV is type 2 respiratory failure with CO2 retention then it would be appropriate to refer for
45
SELF-ASSESSMENT
affected resulting in abnormal liver palpation over the epigastrium. Patients should be screened for CF related liver disease with annual LFTs and regular liver ultrasound scans. Treatment with ursodeoxycholic acid aims to improve biliary excretion and the composition of circulating enterohepatic bile acids. Its use typically normalizes raised liver transaminase enzymes and might help prevent progression to liver failure. Patients should be advised to avoid alcohol. Practice differs between centres, but can include Vitamin K supplements and immunization against Hepatitis B and C. Liver transplantation in CF: liver transplant is a consideration for patients who have relatively mild pulmonary disease but progressive liver failure and/or recurrent variceal bleeding despite treatment. Combined liver and lung transplantation is possible, but rarely carried out in the UK. Lung transplantation in CF: lung transplantation is a treatment option for patients with advanced CF and is currently performed in two paediatric centres; Great Ormond Street Hospital, London and The Freeman Hospital, Newcastle. The rst successful heartelung transplant in CF was performed in the UK in 1985. Bilateral sequential lung transplantation is now the procedure of choice in CF. Transplantation is not a cure, but can result in improved quality of life and prolonged survival. It involves major surgery and carries considerable risks including peri-operative mortality. Currently about 50% of paediatric transplant recipients survive for more than 7 years. The timing of referral for transplant assessment can be difcult but should be considered when respiratory complications are such that survival without a transplant is unlikely to be longer than 2 years. Contra-indications vary between transplant centres and should be discussed directly with the relevant centre. Absolute contra-indications to transplantation are outlined in Question 5. Relative contra-indications include: Long term corticosteroids more than 20 mg/day Non-pulmonary infections e.g. hepatitis B or C, Human Immunodeciency Virus (HIV) Previous thoracic surgery e pleurodesis will make the procedure more difcult and should be discussed with the surgical team Multi-resistant organisms e.g. NTM, MRSA, pan resistant Pseudomonas aeruginosa Severe osteoporosis Psychosocial issues/lack of family support Refractory non-adherence to current treatment Most people are now familiar with the concept of transplantation as a result of media coverage. However, reports tend to be biased towards successful outcomes.
Patients and their families must be fully aware of both positive and negative issues. These include the fact that acceptance onto a waiting list does not guarantee a transplant. Unfortunately up to one third of patients die whilst waiting for organ availability. It can be extremely stressful for patients on a waiting list carrying a pager and waiting for a call. Sometimes patients are called into hospital only to be sent home after tests reveal an unsuitable organ match. False alarms such as these can be upsetting. Whilst the epithelial defects that characterize CF do not recur in the transplanted lung, there is still potential for infection from the upper airway and sinuses requiring on going use of antibiotics and physiotherapy. One of the main transplant related complications is organ rejection. Chronic rejection can result in obliterative bronchiolitis causing breathlessness and worsening obstructive lung function. Non-pulmonary aspects of CF are not improved by lung transplantation and so treatment of pancreatic insufciency and CF related diabetes, for example, need to continue post transplant. For some patients transplantation is life transforming and results in sustained benets that are extremely worthwhile for the individuals concerned.
REFERENCES
Colombo C, Russo MC, Zazzeron L and Romano G. Liver disease in cystic brosis. J Pediatr Gastroenterol Nutr 2006; 43 Suppl 1:S49e55. Debray D, Kelly D, Houwen R, Strandvik B and Colombo C. Best practice guidance for the diagnosis and management of cystic brosis-associated liver disease. J Cyst Fibros 2011; 10 Suppl 2:S29e36. Flume PA, Mogayzel PJ, Jr, Robinson KA, Rosenblatt RL, Quittell L, Marshall BC, Clinical Practice Guidelines for Pulmonary Therapies and Cystic Fibrosis Foundation Pulmonary Therapies. Cystic brosis pulmonary guidelines: pulmonary complications: haemoptysis and pneumothorax. Am J Respir Crit Care Med 2010; 182:298e306. Lamireau T, Monnereau S, Martin S, Marcotte JE, Winnock M and Alvarez F. Epidemiology of liver disease in cystic brosis: a longitudinal study. J Hepatol 2004; 41:920e5. Orens JB, Estenne M, Arcasoy S, Conte JV, Corris P, Egan JJ, et al., Pulmonary Scientic Council of the International Society for Heart and Lung Transplantation. International guidelines for the selection of lung transplant candidates: 2006 updatee a consensus report from the Pulmonary Scientic Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006; 25:745e55. http://www.rbht.nhs.uk/healthprofessionals/clinicaldepartments/paediatrics/childrencf/transplant-assessment/. www.cftrust.org.uk.
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