Unassigned

The following would be expected at a one year surveillance review: A Walks with one hand held B Good pincer grip C Drinks from a cup D Happy to go to strangers E Understands simple commands Comments: At the one year review, expected findings are: (True) (True) (True) (False) (True)

GROSS MOTOR: Walks with furniture (9 months), and walks with one hand at 12 months, taking a few steps unassisted. FINE MOTOR/VISION: Good pincer grip from 10.5 months with no squint on observation or parental report. LANGUAGE/HEARING: Understands simple commands, says "mama" or "dada", and one or two appropriate words by 13 months. No parental concerns about hearing. SOCIAL: Drinks from a cup, feeds using a spoon or fingers. Wary of strangers, but socially responsive. Waves "bye bye" at 8 months.

Copyright 2002 Dr Colin Melville

Parental smoking increases the risk of: A Acute otitis media B Chronic secretary otitis media C Sudden infant death syndrome D Admission with bronchiolitis E Low birth weight Comments: A reduction in smoking during pregnancy is a Health of the Nation Target. Passive smoking increases the risk of SIDS, secretary otitis media, admission with respiratory illness, and increases the risk of fires in the home. It also increases the risk of children taking up smoking themselves (they are role models). Unfortunately, smoking cessation is difficult as nicotine is highly addictive. It is best pursued in a wideranging effort at health promotion within particular families, using a good doctor/parent relationship to promote parental self-confidence. Support groups, telephone helplines, counselling, and medication may all contribute to success. Copyright 2002 Dr Colin Melville (False) (True) (True) (True) (True)

The following are at increased risk of paralytic poliomyelitis from oral polio vaccination: A A 4 year old on chemotherapy for recently diagnosed ALL. B A 2 year old child who received a bone marrow transplant for X-linked Severe Combined Immune Deficiency (SCID), 1 year ago. C A 3 year old boy with Di George Syndrome. D An 18 month old girl with IgG2 sub-class deficiency. E A 7 year old boy with tuberculosis. Comments: Children with a defective immune response to live vaccines can suffer from severe manifestations, such as BCG-osis, or paralytic polio from vaccine virus. At risk individuals include: (True) (False) (True) (False) (False)

Patients on chemotherapy, or receiving generalised radiotherapy, or following organ or bone marrow transplant, those receiving immunosuppression. These are at an increased risk for the first 6 months following these procedures. Those on Prednisolone >2mg/kg/day for >7 days, or >1mg/kg/day for one month. Occasional children on lower doses of steroids may have significant immunosuppression, and in cases of doubt, expert advice should be sought. Patients with defective cell mediated immunity, including HIV infection, severe combined immune deficiency, Di George Syndrome. Patients with minor antibody immunodeficiency are not at risk.

For all these individuals, an impaired response to inactivated vaccines may be expected. Copyright 2002 Dr Colin Melville

The following suggests a diagnosis of Hurler's Syndrome rather than Hunter's Syndrome: A X-linked inheritance B Mental retardation C Gibbus D Cloudy cornea E Cardiomyopathy Comments: Hunter's Syndrome (MPS-2) is of X-linked inheritance. The cornea are clear. The skeletal involvement tends to be mild with no gibbus present, though scoliosis is often found. Mental retardation and heart involvement are less severe than in Hurler's Syndrome. Hurler's Syndrome (MPS0) is autosomal recessive in inheritance and is associated with cloudy cornea. (False) (False) (True) (True) (False)

There is severe mental retardation, and gibbus deformation of the spine is characteristic. There is the characteristic coarse facies with hepatosplenomegaly. Copyright 2002 Dr Colin Melville

A child presents at 10 months of age with scissoring of the legs. The following pointers may indicate a specific diagnosis: A An apgar of 5 at 5 minutes B A blood glucose of 2.2mmol/L in the neonatal period C A history of ABO incompatibility D History of neonatal septicaemia E Midline cleft palate Comments: 80% of cerebral palsy is thought to result from antenatal insults including cerebral dysgenesis, cerebral malformations or congenital infection. Rubella may be indicated by antenatal rash, toxoplasma by glandular fever-like illness following exposure to raw meat or cat faeces, and cytomegalovirus by contact with children attending nurseries. Intrapartum insults are thought to account for 10% of cases and are usually related to birth asphyxia or trauma. A 5 minute apgar score is not a good prognostic indicator, but the apgar score at 10 minutes may be much more informative. If this is <5 then significant birth asphyxia may well have occurred. Postnatal insults account for about 10% of cases including IVH or ischaemia, meningitis, encephalitis, encephalopathy, head trauma or NAI, symptomatic hypoglycaemia, hydrocephalus, or hyperbilirubinaemia. In the neonatal period, hypoglycaemia may be significant with levels lower than 2.5mmol/L, particularly if symptomatic or prolonged. Copyright 2002 Dr Colin Melville (False) (True) (True) (True) (True)

A newborn child should be given hepatitis B immunisation and specific immunoglobulin if: A Mother is Chinese. B Mother is HBSAg positive. C Mother is HBSAg positive and HBe antibody positive. D Mother is HBC antibody positive. E Mother is HIV positive. Comments: Immunisation is recommended for the following groups: (False) (True) (True) (False) (False)

Babies born to mothers who are chronic carriers of hepatitis B or to mothers who have had acute hepatitis B during pregnancy. Parental drug misusers.

Individuals who change sexual partners frequently. Close family contacts of a case or carrier. Families adopting children from countries with a high prevalence of hepatitis B (particularly Eastern Europe, South East Asia and South America). Haemophiliacs. Patients with chronic renal failure. Health Care Workers including students and trainees. Staff and residents of residential accommodation for those with severe learning difficulties. Certain occupational groups: morticians, embalmers. Inmates of custodial institutions. Those travelling to areas of high prevalence.

Active (hepatitis B vaccine) and passive (HBIG) should be given if:

Mother is HBsAg positive and HBeAg antigen positive. Mother is positive without E markers (or where they have not been determined). Mother has had acute hepatitis B during pregnancy.

If mother is HBsAg positive and anti-HBe antibody positive then immunoglobulin can be omitted. The vaccine is currently prepared from used cells using recombinant DNA technology, and HBsAg is absorbed on to aluminium hydroxide. The vaccine has an 85% protectivity, with an antibody level of below 10miu/ml classified as non-response. Immunisation may take 6 months to confer adequate protection. Copyright 2002 Dr Colin Melville

Regarding a lumbar puncture: A It can show a CSF pressure of >200mmH2O in normal obese post-pubertal patients. B The pressure is unrelated to the presence of retinal vein pulsation. C Abducent nerve palsy is a complication. D The test is safe with a platelet count of 40 x 109/L. E The severity of headache is related to the size of the needle. Comments: Contraindications to lumbar puncture include:

(False) (False) (False) (False) (False)

Raised intracranial pressure (e.g. 3rd or 6th nerve palsies, decreased level of consciousness, bradycardia and raised blood pressure, abnormal respiratory pattern) Shock Skin infection over the LP site Low platelets or DIC (until coagulopathy treated) CT scan should not delay therapy.

Copyright 2002 Dr Colin Melville

Cataracts:

A When inherited, are rarely bilateral. B Can be seen in infants of diabetic mothers. C Can be seen in patients with rubella. D Can be seen in pre-term babies exposed to high oxygen concentration. E Are found in patients on long-term steroids. Comments: Inherited conditions will usually cause bilateral disease. Causes include:

(False) (True) (True) (True) (True)

Developmental disorders. Prematurity. Inherited AD or Lowes (XR), or chromosomal defects. Congenital infection. Metabolic disease e.g. GIPUT, IDDM (rare), infants of diabetic mothers. Drugs: steroids. Trauma.

Copyright 2002 Dr Colin Melville

By the age of 2 years, a normal child should be able to build the following structures using 1 inch cubes: A A train B A bridge C Steps (after demonstration) D A tower of 6 cubes E A pyramid Comments: By the age of 18 months, a child can build a tower of 3, a tower of 6 by 2 years, and a tower of 8 by 2.5 years. By this stage, simple conceptual objects can be built, such as a train, but a bridge (with one cube balancing on 2 others) requires the co-ordination of a 3 year old. Steps or pyramids are more complex structures requiring demonstration before accomplishment at about 4 years. Copyright 2002 Dr Colin Melville (False) (False) (False) (True) (False)

Concerning juvenile idopathic arthritis: A It may be associated with HLA DR4. B Complement factor 3 is elevated in large joint effusions. C It is almost always associated with Sicca Syndrome. (True) (False) (False)

D The pauciarticular form is associated with anterior uveitis. E The systemic form is usually associated with splenomegaly.

(True) (True)

Comments: Pauciarticular arthritis type 1 is associated with HLA DR8, 5, and 6, and with chronic iridocyclitis. Pauciarticular arthritis type 2 is associated with HLA B27 and the spondyloarthopathies. Rheumatoid factor + arthritis is associated with HLA DR4. Systemic onset disease is associated with fever which is high and intermittent, and rash, which is red/pink, evanescent and macular, and hepatosplenomegaly and lymphadenopathy. The latter tend to get bigger with active disease. Sicca Syndrome is rare: It is associated with Sjogren's Syndrome, chronic graft vs host disease and scurvy. Copyright 2002 Dr Colin Melville

A normal healthy three year old is able to: A Ride a tricycle B Speak in sentences C Demonstrate a positive Gower's sign D Write his full name E Recognise and name three primary colours Comments: At 3 years a normal child: (True) (True) (False) (False) (True)

GROSS MOTOR: Is able to hop on one foot (42 months). FINE MOTOR/VISION: Manipulates small objects well, and by 4 years can draw a man of 3 parts. By 4 years he can copy a cross. LANGUAGE/HEARING: Able to give his first and last name and to recognise colours. SOCIAL: Can name a friend, can wash his hands and brush his teeth with help, can eat with a knife and fork, has vivid make-believe play, and will play independently with other children present, and may show sympathy to an injured child. Likes hearing and telling stories.

A Gower's sign is positive in Duchenne muscular dystrophy. Any children who cannot write their names when they begin school at the age of 5. Copyright 2002 Dr Colin Melville

The following are examples of primary prevention: A Fluoridation of the water supply. B The Ortolani manoeuvre. C Auscultation of the chest for heart murmurs in the neonate. D Vitamin K prophylaxis in the newborn. E Guthrie card screening for PKU. (True) (False) (False) (True) (False)

Comments: Primary prevention aims to stop the disease from happening at all. Examples include addition of iron in the diet of preterm babies, fluoridation of the water supply to prevent dental caries, or Vitamin K administration to prevent late haemorrhagic disease of the newborn. Secondary prevention aims to identify the disease at an early stage, hopefully before symptoms become irreversible, so that early intervention can improve the outcome. Examples include screening for CDH, and congenital heart disease, palpation for the testes, screening for congenital hyperthyroidism and PKU on Guthrie card testing, haemoglobinopathy screening and screening for cystic fibrosis. Copyright 2002 Dr Colin Melville

An 11 year old boy with renal failure is on haemodialysis: A Hepatitis A titres should be checked. B Hepatitis B titres should be checked. C Protection against hepatitis B is marked by anti-HBsAb >10/miu/ml. D He should be immunised annually for influenza. E He should be immunised against Hib. Comments: Haemodialysis patients are at increased risk of virus infection transmitted by blood products. Serology for hepatitis B should be done, and antibody negative individuals immunised with 3 doses of hepatitis B vaccine, preferably prior to dialysis. Titres >10miu/ml are protective. Levels below this should prompt reimmunisation. Renal transplant patients and those with chronic renal failure are at increased risk of infection, and should receive influenza vaccination annually, in addition to Hib and pneumococcal immunisations. Copyright 2002 Dr Colin Melville (False) (True) (True) (True) (True)

Regarding human varicella zoster immunoglobulin (VZIG): A It comes as a freeze-dried powder, which is reconstituted before administration. B It is recommended for all patients with eczema exposed to chickenpox. C It should be given to neonates if the onset of maternal chickenpox is more than 7 days after the child's date of birth. D It is invariably protective against severe of fatal varicella. E Should be routinely given to children with IgA deficiency who have varicella contact. Comments: Varicella has a secondary infection rate in household contacts of 90%. It is commonest in spring time, and the incubation period is 14-21 days. It shares the herpes virus family properties of latency and (False) (False) (False) (False) (False)

reactivation (zoster). Risks to the fetus and neonate relate to the time of infection:

Less than 20 weeks pregnancy: congenital varicella (limb hypoplasia, microcephaly, cataracts, growth retardation, skin scarring). High mortality. Second to third trimester: herpes zoster in an otherwise healthy infant. Minus 7 days to plus 7 days after delivery: severe and even fatal disease (30% mortality). Although a live attenuated vaccine is available, it is not licensed for use in the UK.

Varicella zoster immunoglobulin is prepared from pooled plasma of UK blood donors with a history of recent chickenpox or herpes zoster. Being an immunoglobulin, it is a protein concentrate, and should be stored between 2 and 8oC. Donors are screened for HIV, hepatitis B and hepatitis C. VZIG prophylaxis is recommended for patients who fulfil all the following criteria:

A clinical condition that increases the risk of severe varicella, (e.g. immunosuppression, neonates, pregnant women). No antibodies to varicella zoster. Significant exposure to chickenpox or herpes.

Severe or fatal varicella can occur despite VZIG prophylaxis. Active immunisation should therefore be used for susceptible immunosuppressed patients at long term risk. Clinical chickenpox occurs in 50% of those who receive VZIG prophylaxis, and 10% more will be affected sub-clinically. Copyright 2002 Dr Colin Melville

Regarding Guthrie card testing: A It is done at 7 days in the breast fed infant. B It should be delayed till 14d if the child is exclusively on TPN. C It may be affected by concurrent antibiotic administration. D The card contains information about the patient's post code. E Hypothyroidism can be screened for by measuring TSH alone. Comments: The Guthrie card was originally introduced for PKU screening using a specific strain of bacillus subtilis. For this reason, results of PKU screening are affected by concurrent antibiotic administration. The thresholds have been set for fully fed infants, so children should be on a full enteral intake. Those on TPN should have card collection delayed until fully orally fed. In most European programmes, TSH is measured by radioimmunoassay. This fails to detect the extremely rare secondary hypothyroidism due to hypothalamopituitary disease (1:100,000). However, it is extremely effective at picking up primary hypothyroidism (1:4000 births), mainly due to dysgenetic or ectopic thyroid tissue. The card does contain information about the patient's postcode. In one study this was ingeniously used to map breast feeding rates at a week of age to individual postcode sectors. Copyright 2002 Dr Colin Melville (True) (False) (True) (True) (True)

The following conditions may give a false/positive sweat test:

A Congenital adrenal hyperplasia B Hyperthyroidism C Hyperparathyroidism D Obesity E Glucose-6-phosphatase deficiency Comments: Non-CF conditions associated with elevated concentrations of sweat electrolytes include:

(False) (False) (False) (False) (True)

Endocrine: Untreated adrenal insufficiency, hereditary nephrogenic diabetes insipidus, hypothyroidism, hypoparathyroidism. Metabolic: Glucose-6-phosphatase deficiency, mucopolysaccharidoses, fucosidosis. Other: Ectodermal dysplasia, familial cholestasis, pancreatitis, malnutrition.

Copyright 2002 Dr Colin Melville