Professional Documents
Culture Documents
PAKISTAN
PAEDIATRIC
JOURNAL
www.ppa.org.pk
http://www.pakmedinet.com/PPJ
ISSN 0304-4904
Founder
Prof. SMK Wasti (Late)
PAKISTAN PAEDIATRIC
JOURNAL
Patron
Prof. SM Haneef
Chief Editor
Prof. Said ul Haque
Managing Editor
CONTENTS
Editors
Prof. AG Nagi
Prof. A Hameed
Prof. Akmal Laeeq
Prof. Ayesha Arif
Prof. DS Akram
Prof. Humyaun Iqbal Khan
Prof. Iqbal Memon
Prof. Shakila Zaman
Prof. Tahir Msood
Prof. Nadeem Khawar
Prof. ZA Bhutta
Dr. Abdul Rehman
Article
Editorial
Review article
Editorial Board
1.
2.
3.
International
David Southall (UK)
Mobeen Rathore (USA)
Prof. Peter Raine (UK)
Prof. Charlotte M Wright (UK)
Statistical Adviser
Prof. Amanullah
128-34
BUSHRA
FATIMA,
MUHAMMAD
FAHEEM
MUHAMMAD ASHRAF SULTAN, Asif Hanif
Developmental Assessment
Intervention
in
Children
Developmental Delay
135-38
AFZAL,
and Effect of
with
Global
139-44
4.
National
Prof. Afroz Ramzan
Prof. A H Haquani
Prof. Asma Fouzia Qureshi
Prof. Atta Ullah Mazhar
Prof. Ayesha Mehnaz
Prof. Pervaz Akbar
Prof. Qamar ud din Nizami
Prof. Salman Ali (Brig)
Prof. Tariq Bhutta
Dr. Asad Hafeez
Dr. Liaquat Ali
Dr. Tayyaba Khawar Butt
118-27
Original article
Editorial Executive
Committee
Prof. Agha Shabbir Ali
Prof. Abdul Waheed
Prof. Javaria Mannan
Prof. Muhammad Ali Khan
Prof. Sajid Maqbool
Page
115-17
5.
145-48
149-54
155-61
162-68
Case report
1.
2.
3.
ABDULMONEIM AL-AGHA
Abstract service
News and views
169-70
171-74
175-77
178-79
180-81
II
5. Prof.
Bashir
Ahmed
Kakar,
President
Balochistan Branch, House # 10, 9/450-A Opp.
GOR Colony, Main Shahboo Road, Quetta
E-mail: nil
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III
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IV
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V
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Are welcome from readers.
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Sequence of title page, abstract and keywords, introduction, materials and methods,
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EDITORIAL
www.ppa.org.pk
Editorial
116
form if crystalloids do not improve the
hemodynamic status. It should however be kept
in mind that most of the patients of dengue
infection have dengue fever. They neither require
hospitalization nor intensive monitoring.
The governments all-out efforts this year were on
improvement of treating facilities for dengue
patients. All teaching hospitals of the province
were converted into dengue hospitals. Though
this approach may have saved many lives but
cannot be recommended as on ongoing policy
keeping in view the misery of a huge number of
non-dengue patients who did not receive
appropriate and on time management. In
addition fumigation drive to exterminate adult
mosquito is not very helpful. Poor hygienic
conditions that lead to stagnant water provide
excellent breathing sits for Aedes. To avoid a
dengue-related catastrophe in coming years not
only government but organizations like Pakistan
Paediatric Association must launch a well
designed and motivated campaign to increase
public awareness about the hazards of poor
hygiene and how people can contribute in
improving these conditions. Surroundings must be
kept lean, water drained from empty cans, trapholes, tires, etc, and potted plants must also be
well drained. Health managers should also keep
an eye on emergence of other arboviral diseases
like chikungunya and yellow fever. Larvacidal
drugs should also be sprayed at appropriate
time. Remedial and preventive measures are the
key to control the menace of these infections.
PAEDIATRIC DENTISTRY
Paediatric Dentistry is a sub-specialty of Dental
Surgery, also called oral medicine. The Paediatric
Dentistry has got further sub-specialties. There are
many oral diseases, which may which include
genetic and developmental, acquired and may
effect not only the health of a child but also
leads to speech, esthetic and psychological
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117
Editorial
www.ppa.org.pk
REVIEW ARTICLE
-----------------------------------------------------------------ABSTRACT
Authors affiliations
-----------------------------------------Correspondence to:Khalid N. Haque*
276, Club Drive, San Carlos,
California 94070, USA
E-mail:
Khalidnh99@yahoo.com
INTRODUCTION:
Apnea of prematurity (AOP) is diagnosed in more
than 50% of premature infants and in nearly all
the infants who weigh less than1000 g at birth1.
Whilst literature clearly defines clinically significant
apnea in infants as pauses in breathing that last
for >20 seconds or for >10 seconds if associated
with bradycardia or oxygen desaturation (SpO2 <
80% for > 4 seconds and heart rate < 2/3rd of base
line for > 4 seconds)2. There is no consensus about
the frequency or duration of apnea required to
diagnose AOP. Nor is there consensus on the
degree of change in oxygen saturation, or
severity of bradycardia required to diagnose
significant apnea that requires therapy to be
initiated. Bradycardia is reported to occur in 10%
of infants when apnea lasts between 10 and 14
seconds, around 34% with apnea lasting 15 to 20
seconds and in 75% of infants who have apnea
lasting for more than 20 seconds1.
Whilst it would be logical to assume that apnea
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associated
with
either
bradycardia
or
desaturation may affect neurodevelopmental
outcome due to disturbance in cerebral blood
flow. No direct link has been demonstrated
between
AOP
and
neurodevelopmental
outcome. This may be due to various
confounding comorbidities that influence the
neurodevelopmental outcome of premature
infants. Thus, it is also not clear whether AOP has
clinically important long-term effects. Never-theless when faced with a premature infant with
AOP there is an immediate and irresistible urge
amongst clinicians to respond to apnea.
Clinicians are able to respond successfully to
apnea events with drugs or physical and or
mechanical interventions, but it remains
unproven which of these interventions are
beneficial and/or have any long-term effects.
This review will therefore discuss the pathogenesis,
diagnosis, causes and risk factors, associated
conditions, management modalities, prognosis
and conclude by suggesting a pragmatic
119
Central Apnea
At least 20
seconds
Absent
Absent
May Occur
Obstructive Apnea
At least 20 seconds
Mixed-Type Apnea
At least 20 seconds
Periodic Breathing
5 10 seconds
Present
Reduced/Absent
May Occur
Absent/Present
Reduced/Absent
May Occur
Absent
Reduced/Absent
No
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120
a)
b)
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121
Emergency Treatment
Positioning
Physical or Kinesthetic stimulation
Respiratory support
Medication
Carnatine supplementation
Emergency Treatment
1.
3.
4.
5.
6.
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122
bronchopulmonarydysplasia
and necrotizing
enterocolitis25,26. Based on these data, there is
insufficient evidence to recommend routine red
cell transfusion to treat AOP.
Respiratory Support: Respiratory support to reduced
work of breathing is thought to be beneficial in
the management of AOP. This can be achieved
by providing oxygen and distending pressure via
either synchronized NIPPV or variable flow nasal
continuous positive airways pressure (nCPAP)
devices. In an earlier publication we have shown
how to make and deliver bubble nCPAP cheaply
using
commonly
available
equipment27.
Cochrane systematic review of randomized or
quasi-randomized
trials
comparing
nasal
intermittent positive pressure ventilation (NIPPV)
vs. nCPAP for apnea showed that NIPPV may
reduce apnea frequency more than nCPAP but
that there was insufficient evidence to
recommend NIPPV as standard therapy for
AOP28. There was some suggestion that NIPPV or
nCPAP using variable flow ventilator was more
effective than conventional ventilator, but a
recent study29 found no significant difference in
event rate between the NIPPV/conventional
ventilator and bubble nCPAP. Therefore if
required bubble nCPAP should be the preferred
respiratory support. However, if ventilation is
required it should be as gentle as possible e.g.
peak pressures of 10-12 cm of water, end
expiratory pressure of 3-5 cm of water with a rate
of 20-25/minute with an inspiratory time of 0.350.40 seconds and inspired oxygen between 30 to
50%.
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123
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124
Diagnosis: AOP
Position: Head elevated tilt position and three-stair-position
ApneaResponds
No Response
Start Theophylline/Aminophylline:
Loading dose 5 -6 mg/kg
Maintenance: 2 4 mg/kg/day in 2 or 3
divided doses
No Response
Apnea Responds:
Stop medication after 10-12 days of continuous
apnea free period or at corrected gestational
age of 35 weeks.
No response:
Some clinicians give prophylactic Caffeine or Methyxanthine to babies born at or before 29 weeks of
gestation (level of evidence is poor).
24 hours prior to extubation from a ventilator caffeine or methylxanthine should be started to prevent postventilation spells of apnea (level of evidence is good)
www.ppa.org.pk
125
7.
8.
9.
10.
11.
12.
13.
--------------------------------------------------------------Authors affiliations
Prof. Khalid N. Haque
Department of Neonatal Medicine, 276, Club Drive,
San Carlos, California 94070, USA
Ahmed Quddusi
Department of Neonatology, Childrens Hospital &
Institute of Child Health, Abdali Road, Multan, Pakistan
REFERENCES
1.
2.
Michigan
Association
of
Apnea
Professionals. Consensus Statement on
Infantile Apnea and Home Monitoring. 3rd
ed. Lansing, MI: Michigan Association of
Apnea Professionals; 1994
14.
3.
15.
4.
16.
17.
5.
6.
www.ppa.org.pk
126
19.
20.
21.
29.
30.
31.
22.
32.
23.
33.
Schmidt B, Roberts RS, Davis P et al. Longterm effects of caffeine therapy for apnea
of prematurity. N Engl J Med 2007; 357 (19):
18931902.
34.
35.
36.
37.
24.
25.
26.
27.
www.ppa.org.pk
127
38.
39.
40.
48.
49.
50.
51.
52.
53.
54.
55.
56.
41.
42.
43.
44.
45.
46.
47.
12(34): 23649.
www.ppa.org.pk
ORIGINAL ARTICLE
ABSTRACT
-------------------------------------------
Subjects, Materials and Methods: The data was collected through self
responding questionnaires sent to the parents of primary school
children from urban Karachi. We received 610 appropriately filled
forms. Inclusion criteria were student age and parental consent to
provide information. There were no exclusion criteria, except to
exclude grossly incomplete forms.
INTRODUCTION
The role of poor dietary, hygienic, and social
habits is known and established for their
individual and cumulative effects on caries
predisposition and etiology1. Based on this
ground, we set out to learn the prevalence of
these habits in primary school children.
There is ample of research evidence that favors
the correlation between the triad of chronic
dental problems (like caries), oral hygiene
habits and dietary practices2. They have also
favored mass awareness through health
services professionals3.
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Oral Hygiene and Dietary Habits among Primary School Children, in Urban Karachi
RESULTS
The study was conducted in Karachi, the
economic hub of the country, and it is therefore
not representative of the complete national
status (rural and urban). We believe however,
that it may well represent the larger cities of
Pakistan. This may also represent a tilt towards
the higher socio-economic stratum of the
society.
Demographic Profile
Age: The mean age of our studied group was
6.79 years, with a SD of 1.61 years (n=596; 14
missing)
129
Gender
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130
everyday; 71.6% (n=437) marked for sometimes
or rarely; and 5.9% (n=36) did not answer. 56.4%
(n=344) of our studied children eating
chocolates and sweets after meals; 29% (n=181)
during or ahead of meals; and 10.7% (n=65) did
not answer.
Sticky foods: When inquired, the response
towards their consumption was: once or less
than once a day 80.7% (n=492); twice or more
daily 17.9% (n=109); and no answers by 1.5%
(n=09).
Cheese: We found that cheese consumption
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Percentage
Number
45.2
03.1
09.3
02.6
00.3
276
19
57
16
2
33.8
206
03.0
18
00.7
00.3
98.4
01.6
600
10
100.0
610
Soft
Drinks
6.1%
(n=37)
Packed
Juice
13.9%
(n=85)
Fresh
Juice
11.5%
(n=70)
91.1%
(n=556)
84.6%
(n=516)
86.2%
(n=526)
No Answer
2.8%
(n=17)
1.5%
(n=09)
2.3%
(n=14)
Almost
once
everyday
or
more
DISCUSSION
Prof. WD Millers published scientific work in 1891
provided scientific grounds to the theories of
Milles proposed in 1881, which state the role of
acids in decaying and thereby weakening the
protective structure of the teeth. The more
contemporary versions of these theories is
based not only on the diet, the tooth
susceptibility, and time, but also upon
frequency and duration of exposure to
cariogenic nutrients, the individual immunity,
and the use of fluorides. Factors like socioeconomic stratum of the individual, the level of
education and lifestyle behaviors are also
indicted1,2. However, it can perhaps be argued
equally strongly that most of them are
eventually secondary contributors to the
original sinners, the sugar and the microbes.
Oral Hygiene and Dietary Habits among Primary School Children, in Urban Karachi
131
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132
with three monthly professional flossing for 3
years, and self performed flossing for two years,
did not reduce the risk of caries. However, the
former study was conducted on primary
dentition, while the latter were conducted on
teeth of young adolescents15. Most of our
respondents were not flossing 73.8% (n=450);
leaving only those flossing 13.8% (n=84); and
12.5% (n=76) not answering. This underscores
the need for education in dental practices.
Use of Tooth paste: Researchers have found a
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like
saliva27
and
bovine
milk
is
non-
Oral Hygiene and Dietary Habits among Primary School Children, in Urban Karachi
CONCLUSION
Measurement and healthy change in childs
practices can contribute to limit the
occurrence of caries, particularly at this crucial
stage of life when habits are being formed and
ingrained.
REFERENCES
1.
2.
3.
4.
5.
6.
7.
8.
ACKNOWLEDGEMENT:
The researchers gratefully acknowledge the
efforts of the final year student volunteers of
133
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134
20.
9.
21.
10.
22.
11.
12.
13.
14.
15.
16.
23.
24.
25.
26.
27.
28.
29.
30.
17.
31.
18.
32.
19.
www.ppa.org.pk
33.
ORIGINAL ARTICLE
ABSTRACT
Background: Acute watery diarrhea (AWD) is defined as passage of 3 or
more loose stools/day or more frequent for the individual with duration of
less than 14 days. Dehydration is biochemically categorized according
to serum sodium concentration as isonatremic (135-145mEq/L),
hyponatremic(<135mEq/L) and hypernatremic dehydration(>145mEq/L).
Objective: To determine the biochemical types of dehydration and
determine the risk factors for sodium imbalance in children with AWD.
Authors affiliations
---------------------------------------------
Correspondence to:
Bushra Fatima,
Department of Paediatrics
Unit-I, King Edward Medical
University/ Mayo Hospital,
Lahore.
E-mail:
fatimaalvi2000@yahoo.com
Results: Mean age was 2.53 years. Clinically severe dehydration was
found in most children (51%). Highest and lowest serum sodium level was
166 and 117 mEq/L respectively with mean of 1346 mEq/L. Isonatremic
dehydration was found in 51% children followed by hyponatremic
dehydration (46%). There was positive association of nutritional status and
degree of dehydration. (p value 0.05 each) More malnourished and
more severely dehydrated the child was more was the chance of having
sodium imbalance.
Conclusion: Isonatremic dehydration is most common type of
dehydration. Malnutrition and clinically severe dehydration are
significantly associated with biochemical type of dehydration.
Key words: isonatremic, hyponatremic, hypernatremic dehydration,
acute watery diarrhea.
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136
INTRODUCTION
Acute watery diarrhea (AWD) is defined as
passage of 3 or more loose stools/day or more
frequent for the individual with duration of less
than 14 days. Diarrheal disease is the second
leading cause of death in children under five
years of age. Globally, it is causes about two
billion cases and 1.5 million deaths every year1.
Dehydration is a state of negative fluid balance
that leads to depletion of extracellular fluid and
electrolytes resulting in reduction of plasma
volume
with
consequent
disturbance of
physiologic functions2. Serum sodium is the
dominant
cation
and
determinant
of
extracellular osmolality. Dehydration can also be
categorized according to serum sodium
concentration as isonatremic (serum sodium level
135-145
mEq/L),
hyponatremic
(serum
sodium<135
mEq/L),
or
hypernatremic
dehydration
(serum
sodium>145
mEq/L).
Isonatremic dehydration is the most common (7080%) type of dehydration. Hypernatremic and
hyponatremic types of dehydration each
comprise 10-15% of cases. Both types can lead to
neurological symptoms3.
Based on this review, this study was conducted to
help determine the biochemical types of
dehydration and the risk factors for sodium
imbalance in children with AWD, so that patients
at risk can be identified earlier and special
treatment strategies can be instituted regarding
rehydration of these patients so that mortality
associated with this preventable disease may be
reduced.
No Malnutrition
n (%)
Grade I
n (%)
Grade II
n (%)
Grade III
n (%)
Grade IV
n (%)
Total
Isonatremic
46 (9.2)
49 (9.8)
51 (10.2)
67 (13.4)
43 (8.6)
256
Hyponatremic
51 (10.2)
63 (12.6)
55 (11)
35 (7)
25 (5)
229
Hypernatremic
02 (0.4)
03 (0.6)
05 (1)
04 (0.8)
01 (0.2)
15
Total
99 (19.8)
115 (23)
111(22.2)
106(21.2)
69(13.8)
500
(Association of nutritional status with biochemical type of dehydration was statistically significant; p value was 0.05
by Chi square)
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137
Issonatremic, hy
yponatremic,, hypernatrem
mic dehydratio
on, acute watery diarrhea
T
TABLE
2: Deg
gree of dehyd
dration (n=500)
A
Age
Issonatremic
H
Hyponatremic
c
H
Hypernatremic
c
T
Total
Some
n (%)
142 (28.4)
96 (19.2)
6 (1.2)
244 (48.8)
Severre
n (%
%)
114 (22.8)
133 (26.6)
9 (1.8)
256 (51.2)
Total
256
229
15
500
cal dehyd
dration with
(Association of clinic
b
biochemical
l type of dehydration was
w statistically
s
significant;
p value was 0.05
0
by Chi square)
s
16%
32%
%
15%
16%
21%
1<6months
6<12m
months
2<5years
512ye
ears
12
2<24months
D
DISCUSSION
N
Diarrhea is most
D
m
common in childrren especially
t
those
betwe
een 6monthss to 2 years of
o age. Major
c
complication
ns of diarrhea include dehydration,
abnormalitties and malnutrition.
e
electrolyte
P
Present
study
y had age range 1 mo
onth up to 12
1
y
years
with a mean ag
ge of 2.53
3 years. Mo
ost
c
common
ag
ge group was
w
infancy
y [268 (53%
%)]
T
These
results are co
onsistent with a stud
dy
c
conducted
on children
n with diarrrhea in 200
05
m
mentioning
that most of
o the child
dren sufferin
ng
from AWD were
w
betwee
en 6 months up to 4 yea
ars
o age4. The prevalence of diarrhea is also high in
of
c
children
be
elow 5 years in western world
d5.
A
Although
A
AWD
is not sex speciific howeve
er,
p
present
stud
dy showed preponderan
p
nce of male
es
(57%). This coincides witth a local sttudy6 showin
ng
6
60%
male ca
ases in AWD
D. This may be
b due to th
he
fact that ma
ales with severe infectio
ons are more
frequently brought
b
to the hospita
al than the
eir
female coun
nter parts in our
o culture.
Present stud
P
dy had pre
edominance
e of clinica
ally
s
severe
dehydration (52%
%), while 51%
% children ha
ad
ydration. Wa
athen7 repo
orted 96%
isonattremic dehy
childrren of AWD
D with isona
atremic deh
hydration.
Local study co
onducted in Lahore reported
ydration as 81%8. This difference
d
isonattremic dehy
may be
b due to th
he fact that we
w included
d children
with clinically
c
som
me and sev
vere dehydration. In
this studied population, 3% children had
d
hyperrnatremic dehydration
. These results are
comp
patible with other
o
studiess7,8 in which 3.3% and
7% patients
p
had hypernatremic deh
hydration.
Present study had
d 46% hypon
natremic deh
hydration
which
h is compa
atible with internationa
al report
(56%)9. However, it was 12% in a local sttudy from
s
had statistically
s
significant
Lahorre.8 Present study
assoc
ciation of biochemica
al dehydrattion with
nutritional status which is comparable with
w
study
by Caksen
C
et al10. Prese
ent study had no
statisttically signific
cant associa
ation of bioc
chemical
dehyd
dration with
h type of feeding. However,
H
hyperrnatremic dehydration
d
is reported
d to be
more commonly
y associate
ed with hy
ypertonic
ng preparatiions11.
feedin
Present study ha
as certain limitations. This was
ed out from March to August
A
(peak season
carrie
of AW
WD) which did not in
nclude all the four
seaso
ons. If the study
s
had included
i
th
he whole
calen
ndar year, the result might hav
ve been
differe
ent. Factors contributing
g to all three
e types of
dehyd
dration were
e not studied and we could
c
not
get in
nformation on
o consump
ption of OR
RS before
presenting to ho
ospital. Elec
ctrolytes oth
her than
sodium
m were not included in data.
d
CONCLUSION
We co
onclude from
m present sttudy that isonatremic
dehyd
dration is mo
ost common
n type of deh
hydration
follow
wed by hyp
ponatremic and hypernatremic
dehyd
dration wh
hose timely
y identification is
necesssary for furrther manag
gement. Ma
alnutrition
and
clinically
severe
dehydration
n
were
al type of
significantly associated with biochemica
dehyd
dration.
--------------------------------------------------------------------------------Authorrs affiliations
a Fatima, Muhammad
M
Faheem Affzal, Prof.
Bushra
Muham
mmad Ashraff Sultan
Deparrtment of Pae
ediatrics Unit-I, King Edward
d Medical
Univerrsity/ Mayo Ho
ospital, Lahore
e.
anif
Asif Ha
Biostattistician, Annals
Univerrsity, Lahore.
of
Kin
ng
Edward
Medical
www.p
ppa.org.pk
138
REFERENCE
1.
2.
3.
4.
5.
6.
www.ppa.org.pk
7.
8.
9.
10.
11.
Developmental Assessment and Effect of Intervention in Children with Global Developmental Delay
139
ORIGINAL ARTICLE
-----------------------------------------------------------------ABSTRACT
Authors affiliations
--------------------------------------------Correspondence to:
Shazia Maqbool
Department of Developmental
Paediatrics, The Childrens
Hospital & Institute of Child
Health, Ferozepur Road, Lahore
Email: drshazimaq@yahoo.com
Delay,
Early
Intervention,
www.ppa.org.pk
140
INTRODUCTION
Child development refers to the ordered
emergence of interdependent skills of sensorimotor, cognitive, language, and socialemotional functioning1. Many children in
developing countries are exposed to multiple risks
for poor development including poverty, poor
health and nutrition. According to a recent series
of reviews it is estimated that more than 200
million children under 5 years of age in
developing countries do not reach their full
potential. Sub-Saharan African countries have
the highest percentage of disadvantaged
children but the largest number live in South
Asia2.
Developmental disorder" and "developmental
disability" refers to a childhood mental or physical
impairment or combination of mental, physical,
behavioural and emotional impairments that
result in substantial functional limitations in major
Global Developmental Delay
life activities3.
(GDD) is a subset of developmental disabilities
defined as a significant delay in two or more of
the following developmental domains: gross/fine
motor,
speech/language,
cognition,
social/personal and activities of daily living4.
Developmental delay occurs in up to 15% of
children under 5 years of age5 while in the total
paediatric
population
childhood
disability
prevalence is up to 10%6. The prevalence of
disability in selected developing countries is as
follows:
Jamaica-15%,
Pakistan-15%
and
Bangladesh-8%7 with GDD as one of the major
contributors. The causes of GDD are many and
include genetic conditions like Downs syndrome,
Fragile X syndrome, Phenylketonuria, prenatal
problems like TORCH infections and maternal
malnutrition, perinatal problems like birth trauma,
hypoxia,
prematurity,
cerebral
ischemia,
hypothyroidism, and childhood problems like
intracranial infections, failure to thrive and lead
poisoning8. There is no universally accepted
approach to the etiological assessment of
developmental delay. Recommended tests used
to detect aetiology are cytogenetic studies and
molecular testing even in the absence of
dysmorphic features along with neuroimaging
preferably MRI. EEG and metabolic screening
should be done as and when indicated by history
and physical examination4. Besides biological
www.ppa.org.pk
141
Developmental Assessment and Effect of Intervention in Children with Global Developmental Delay
RESULTS
26 children with Global Developmental Delay
who completed one year of study were
reassessed after intervention.
There was a statistically significant increase in the
mental ages of these children (p=0.000, t=22.74)
(Fig 1). There was significant improvement in the
developmental skill areas including cognition
(p=0.000, t=-24.48), socialisation (p=0.000, t=18.20), language (p=0.000, t=-17.72), self-help
(p=0.000, t=-9.56) and motor (p=0.000, t= -9.04)
(Table 1). The change in mental age correlated
with improvements in cognition (p=0.000,
r=0.780), language (p=0.000, r=0.642) and self
help (p=0.026, r= 0.435) while the improvement in
motor (p=0.465, r=0.150) and socialisation
(p=0.115, r=0.317) had no influence on the
mental age (Table 2). The Developmental
Skill Areas
Pre Mean
(In
Months )
Cognition
Socialization
37.60
Post
Mean
(In
Months )
61.40
-24.48
0.000
39.30
57.60
-18.20
0.000
Self Help
46.00
61.20
-9.56
0.000
Motor
36.60
61.90
-9.04
0.000
Language
36.62
61.92
-17.72
0.000
www.ppa.org.pk
142
Skill Area
Cognition
Socialization
Self Help
Motor
Language
r
0.780
0.317
0.435
0.150
0.642
p
0.000
0.12
0.026
0.47
0.000
r
0.310
0.024
0.021
0.034
0.228
p
0.123
0.907
0.921
0.867
0.262
DISCUSSION
Awareness of developmental delay and possible
interventions is very low in our part of the world.
An Indian study found that the mean age of
children referred for an early intervention
program was 4 years18 while in the west the mean
age of referral was 1.2 years19. The mean age of
referral in our study was 5.6 years with a mental
age of 3.5 years showing that the situation is
worse in our country. There is a dearth of research
on
early
intervention
studies
in
global
developmental delay and early intervention can
be better planned if developmental screening
and surveillance are done20. Portage was first
devised as a home based program to facilitate
early education in normal children and it soon
became a popular tool for developmental
assessment and therapy in children with special
needs. We compared the DQ of Portage with the
mental ages in addition to the IQ of SIT. In our
study DQ and mental age scores were
comparable both before and after intervention.
Developmental profile of DQ forms the basis of a
therapeutic plan and it gives an extra benefit to
use Portage both as an assessment and
therapeutic tool especially for age range less
than 6-7 years. Furthermore IQ at this age is not a
reliable index for measurement in children.
Early Intervention has been defined as a broad
array of activities designed to enhance a young
childs development21. These interventions are
intended to affect children directly via structured
www.ppa.org.pk
CONCLUSIONS
Early intervention to improve skill areas is effective
and should be planned according to the need
within the resources available. DQ based on
Portage is a useful index of developmental
assessment as it is comparable to mental age. IQ
may not be considered a useful indicator of
improvement in skill areas in young children.
ACKNOWLEDGEMENTS
We are grateful to Mr. Muhammad Shakeel Azam
for typing of the manuscript and to all the staff of
Developmental Assessment and Effect of Intervention in Children with Global Developmental Delay
9.
10.
11.
12.
13.
14.
15.
16.
17.
of
Mian M Shariff
Currently working as Consultant Haematologist,
Broomfield Hospital, Chelmsford, United Kingdom
REFERENCES
1.
2.
3.
4.
143
5.
18.
6.
19.
7.
www.ppa.org.pk
144
20.
21.
22.
Home
Based
Intervention Trail (Brain-HIT). Revista Romana
De Pediatrie 2010, 59(2): 137-145.
23.
24.
25.
www.ppa.org.pk
27.
28.
29.
30.
141
ORIGINAL ARTICLE
-----------------------------------------------------------------ABSTRACT
Authors affiliations
--------------------------------------------Correspondence to:
Muhammad Saeed,
Department of Pediatric
Neurologist, P.O.Box 6741.
Childrens Hospital Taif,
Kingdom of Saudi Arabia.
E mail:
muhammad964@hotmail.com
Objective: The objective of this study was to find out the frequency of
meningitis among children who present with first febrile seizure.
Study design: A retrospective record review of the patients from 6
months to 5 years of age who presented with first febrile seizure and
evaluated for CSF at Childrens hospital Taif, KSA.
Methodology: All patients who presented to emergency department
with first febrile seizure, from January 2008 to May 2011, from 6 months
to 5 years age were studied. Lumbar puncture was performed in all
cases.
Results: A total of 272 patients were studied. 165 cases (61%) were male
and 107(39%) were female. All patients underwent lumbar puncture, of
whom 6(2%) had abnormal CSF findings suggestive of meningitis. Thus
meningitis was diagnosed in 2% of children with first febrile seizure.
Conclusions: Our results indicate that proportion of meningitis was very
low in children presenting with first febrile seizure. We suggest that by
practicing meticulous clinical examination and close monitoring, it is
possible to avoid unnecessary lumbar puncture in these patients.
Key words: Febrile seizures, lumbar puncture, clinical examination,
meningitis
INTRODUCTION
Febrile seizures (FS) are the most common
problem in pediatric neurology. The relation
between fever and seizures had been
documented by Hippocrates in the 5th century
B.C1.
Febrile seizures occur in 2- 5% of all children and,
thus make up the most common convulsive
event in children younger than 60 months. The
American Academy of Pediatrics (AAP) issued
practice
parameters
regarding
the
neurodiagnostic evaluation of children with first
simple febrile seizures in 19962. The term febrile
seizure is not intended for use among children
www.ppa.org.pk
146
TThe study wa
as conducte
ed with the objectives to
t
find out the value of CS
SF analysis with
w
presume
ed
febrile seizures who ro
outinely und
dergo lumba
ar
p
puncture,
to
o find out the predictiv
ve factors for
m
meningitis
a
and
whethe
er lumbar puncture
p
wa
as
n
necessary
in
n children presenting with first febriile
s
seizure.
M
METHODOLO
OGY
TThis study was
w
hospita
al based, retrospectiv
ve
a
analysis,
co
onducted in
i
the de
epartment of
p
pediatric
neurology, Childrens Hosp
pital Taif, KSA
A,
from January
y 2008 to Ma
ay 2011.
A total of 27
72 children aged
a
from 6 months to 5
y
years,
who presented
p
wiith first febrile
e seizure were
in
ncluded in the study. These cases were
w
identifie
ed
b reviewing
by
g the admiission registe
ers and datta
w
was
collecte
ed from med
dical record
ds. The deta
ails
o
of
current illness an
nd investig
gations were
r
recorded.
Children witth other ne
C
eurological diseases lik
ke
c
cerebral
palsy, mental re
etardation, past
p
history of
m
meningitis
with sequel, other neurological
d
diseases,
and fever afte
er occurrenc
ce of seizure
es
o antibioticss for more th
or
han forty-eig
ght hours were
e
excluded
fro
om the study
y. Children having
h
axilla
ary
t
temperature
e 38oC (recorded in eme
ergency, OP
PD
o in the warrd), were considered as febrile. All th
or
he
p
patients
we
ere examin
ned in de
etail by th
he
p
pediatricians
s of the hospital. Lumb
bar puncture
es
w
were
perform
med after ta
aking written consent from
t
the
parents and
a
CSF was collected in three steriile
v
vials.
One viial was sent for total an
nd differentiial
c
cell
count, one
o
vial for sugar and protein, an
nd
t
third
one forr culture. Ab
bnormal CSF results were
la
abeled in a child with fever and seizure if CSSF
h
had
a comb
bination of tw
wo of the folllowings:
A Cells gre
A.
eater than 5//mm3, prote
ein more tha
an
40mg% and sugar less than 2/3
2
of bloo
od
sugar.
B Positive smear
B.
s
on Grrams stain and
a
growth of
bacteria in CSF.
R
RESULTS
TThere were a total of 272
2
cases th
hat presente
ed
w
with
first feb
brile episode of seizure
e between 6
m
months
to 5 years of age
e. One hund
dred and sixxty
five cases (6
61%) out of 272 were male
m
and 10
07
p
patients
(39
9%) were fe
emale. The duration of
s
seizures
rang
ged from 0.2
20 minutes to
t 40 minute
es
w
www.ppa.org
.pk
Saee
ed M, Nadeem T, Samir M. Al-Nufiee
o five minu
utes. The ma
ajority of
with a median of
patients (96%) ha
ad seizures lasting less than or
equal to 15 minutes; 12(4%) patients
p
had
d seizures
lasting
g greater th
han 30 min
nutes. There were a
total of
o 89 patien
nts in 6 -12 months
m
age group,
g
95
in 12-18 months and
a
88 abov
ve 18 month
hs age. 6
m
childrren (2%) werre diagnosed to have meningitis
and other cause
es of seizure
es associate
ed fever
mentiioned in tab
ble 1.
Two hundred an
nd sixty six cases (98%
%) of our
nd only six (2%)
(
had
patients had norrmal CSF an
raised
d CSF protein and pleoc
cytosis. 4 ca
ases were
male and 2 were
e female. None
N
of the patients
had cutaneous
c
signs, neck sttiffness or otther signs
of me
eningeal irritation. CSF culture
c
was negative
in all cases. Am
mong casess of mening
gitis, the
duration between
n the onset of fever and
d seizures
was more than 24 hours and seizurres were
recurrrent, but in remaining cases, seizures
occurrred within th
he first 24 ho
ours of fever. No case
of me
eningitis cam
me with status epilepticuss.
All pa
atients who presented with fever,, seizures
and abnormal
a
CSSF appeared
d extremely ill or had
febrile
e seizures witth complex features.
f
300
250
200
150
100
50
0
Total Patie
ents
Male
e
Fem
male
Number of
ents
patie
214
4
30
0
20
0
6
2
Perc
centage
79.0
00
11.0
00
7.0
00
2.0
00
0.7
73
USSION
DISCU
Febrile
e seizures are
a
the mosst common form of
seizure
es seen in children. The prevalence of
www.ppa.org.pk
147
CONCLUSION
To conclude, considering the complications of
lumbar puncture in children, if the children do not
have any signs indicating meningitis (decrease in
consciousness
level,
headache,
irritability,
nausea, vomiting), pediatricians could possibly
avoid unnecessary lumbar punctures from being
performed, by observing and monitoring these
patients and regularly examining them in the
early stages, except when other signs of
meningitis are seen.
---------------------------------------------------------------------------Muhammad Saeed, Tahir Nadeem, Samir M. Al-Nufiee
Department of Pediatric Neurologist, P.O.Box 6741.
Childrens Hospital Taif, Kingdom of Saudi Arabia.
REFERENCES
1.
148
2.
9.
3.
10.
4.
11.
5.
12.
6.
13.
14.
15.
7.
8.
www.ppa.org.pk
149
ORIGINAL ARTICLE
ABSTRACT
Objective: To document the number, disease pattern and outcome of
patients admitted to Neonatal Unit.
Study Design: Descriptive study.
Place and Duration of Study: The study was conducted in the Neonatal
Unit of Department of Child Health, Peshawar, Pakistan from 1st January
2009 to 31st December 2009.
Material and Methods: The data of all Neonatal admission was
recorded and analyzed for age, weight, sex, duration of stay, cause of
admission and outcome.
Authors affiliations
-------------------------------------------Correspondence to:
Qamar Ali Khan,
Department of Child Health,
Khyber Teaching Hospital
Peshawar.
E-mail: ppa.nwfp@hotmail.com
www.ppa.org.pk
150
INTRODUCTION
Neonatology is a part of pediatrics which is
coming up now as a sub-specialty and in near
future will expand more and more as a result of
neonatal screening programs, the availability of
resources for diagnosing different metabolic as
well as congenital problems. Neonatal period
(0 to 28 days of life) is the most dangerous period
of life because of various problems it has to come
across. Most of them may not have any serious
problem or difficulty and will need only minimal
care, which can be provided by the mother if
properly counseled by a health worker. Preterm /
low birth weight babies born to high risk mother
are more prone to have problems1. Most of the
causes of neonatal morbidity in our country are
preventable2. Some of the newborns in
developing countries have an impaired growth
during their intrauterine life, reflecting the
nutritional status of the mother3. Almost half of the
infant deaths in our country occur within first 28
days of life4. Prematurity accounts for majority of
high risk newborns as they face a large number
of problems.5 The prognosis of these neonates
depends upon their underlying condition and its
severity, management and their outcome. For
this purpose neonatal audits are carried out in
Pakistan from time to time in order to create an
awareness regarding the preterm babies and
other neonatal problems which they face and
are managed in an effective way6-8. Neonatal
morbidity and mortality has not decreased due
to the lack of the available resources in
developing countries. This can be decreased by
proper and timely intervention9. For better
neonatal
care
and
prevention
of
the
preventable diseases leading to high neonatal
morbidity and mortality, there should be a proper
and continuous system for audit of neonatal
admission all over the country. The purpose
behind such types of audits in neonatal units
should be the identification of various
deficiencies in the management of these
neonates and also to assist the health workers
especially those at the community level for the
better
understanding
and
effective
management of various neonatal problems in
Pakistan. Inequalities in the provision of health
care are one of the reasons for differences in
neonatal morbidity and mortality. These can be
reduced by proper intervention9. The neonatal
disease pattern changes between different
www.ppa.org.pk
c)
d)
f)
g)
h)
i)
RESULTS
A total of 3203 neonates were admitted
from 01-01-2009 to 31-12-2009. Out of these 2142
Pattern of Neonatal Diseases in Special Care Baby Unit, Khyber Teaching Hospital, Peshawar
151
Number
Percentage
2142
1061
66.87
33.10
Male
Female
Number
2416
787
Percentage
75.43
24.57
Number
15
228
567
2100
193
Percentage
1.56
10.2
17.70
65.56
6.02
Number of patients
893
1183
387
195
26
Number
799
790
787
676
14
25
12
18
10
02
02
50
Percentage
24.94
24.66
24.57
21.10
0.43
0.78
0.37
0.56
0.31
0.06
0.06
1.56
www.ppa.org.pk
152
TABLE 7:- Out come of the admitted cases (n=3203)
Disease
Neonatal Jaundice
Sepsis
Premature/low birth weight
Birth Asphyxia
Surgical
Congenital heart Disease
IDM
Meningitis
Pneumonia
Dysmorphism
Hypothyroids
Criglar najar
Total Number
799
790
787
676
25
12
14
18
50
10
02
02
DISCUSSION
In our study most of the babies were admitted
with in 1st 24 hours of life (44.1 %). The other study
conducted at different cities of the country shows
that 33.61% admitted during first 24 hour at NIH
Karachi10, 44.7% in Chandka Medical College
Larkana8 and 75% at Childrens Hospital Lahore11.
These figures show the majority of neonatal
problems occur in the 1st 24 hours of life, the male
predominance at admission is consistent with
other studies from different cities of the country.
Low birth wt and very low birth wt are the major
contributor to neonatal mortality and morbidity.
In our study 24.57 % were low birth wt/preterm
which seems to be high when compared to other
studies, Chandka Medical College Larkana8 36%,
children hospital Lahore11 39% and NIH Karachi10
55.4% while much less were reported in
Bangladesh14 13.2%, Ethiopia 13.25%15 and India16
20%.. LBW admission may possibly be due to poor
maternal nutrition and poor access to antenatal
care because majority of the mother are severely
anemia, short stature and malnourished10. These
factors were also reported in Ethiopia15. In our
study 84.82% neonates were discharged
satisfactorily after receiving the necessary
treatment. This high percentage could be due to
increase awareness among heath workers and
provision of all possible management in spite of
limited resources. 0.96% neonates were sent
whom on request of their attendants/parents
while 0.49% left against medical advice (LAMA).
This low percentage shows increase awareness
among parents to aware the opportunities of
hospital stay for the better management of their
babies.5 9.6% were refer from hospitals,15.8% in
www.ppa.org.pk
Discharge
Expired
Percentage
752 (94.11%
47
5.88
664 (84.05%)
126
15.94
646(82.08%)
141
17.91
528(78.10%)
148
21.89
All were return to surgical and could not follow
10 (83.3%)
02
16.60
11(78.78%)
03
21.00
16 (88.8%)
02
11.11
46 (92%)
04
8.00
9 (90%)
01
10.00
02 (100%)
0
0.00
02 (100%)
0
0.00
Pattern of Neonatal Diseases in Special Care Baby Unit, Khyber Teaching Hospital, Peshawar
CONCLUSION
Neonatal jaundice, low birth weight, asphyxia
and sepsis were the major causes of admission in
our study. Therefore it should be a part of overall
preventive strategy towards reducing neonatal
morbidity and mortality. This goal can be
achieved by proper and affective intervention by
community mobilization and participation.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
REFERENCES
1
153
www.ppa.org.pk
154
http://www.orion-group.net/journals/
Journals/Vol6_May2000/start.htm
15.
18.
16.
19.
20.
17.
www.ppa.org.pk
-----------------------------------------------------------------Authors affiliations
-------------------------------------------Correspondence to:
Abdul Rehman,
Department of Paediatrics,
Quaid-E-Azam Medical,
College, Bahawalpur.
E-mail:
drarehman100@yahoo.com
ABSTRACT
INTRODUCTION
Pakistan, like other countries especially of South
East Asia, is confronting increasing incidence of
dengue infection. Dengue virus is now endemic
in Pakistan, circulating throughout the year with a
peak incidence in the post-monsoon period.
Median age of dengue patients has decreased
and younger patients may have become more
susceptible1. The Government of the Punjab
(Pakistan) in collaboration with the Sri Lanka
College of Pediatricians published guidelines for
the management of dengue fever in September
20112 which are nearly the same as those
published in Sri Lanka in December 20103 and
those published by World Health Organization,
Regional Office for South-East Asia in 20114. These
are not the same as those recommended by
World Health Organization, Geneva 20095. The
reasons of these controversies include; lack of
animal model for research, most of the studies
are conducted in low resource countries, and
there is very limited knowledge available which is
based on personal experience and is not
evidence based. In addition there are ethical
issues due to research on humans. The purpose of
this review is to discuss the scientific justifications
CLASSIFICATION
Many dengue cases are self-limiting but
complications such as hemorrhage and shock
can be life-threatening. If untreated, mortality
from the complications of dengue is as high as
20%, whereas if recognized early and managed
properly, mortality is less than 1%6; hence, it will
be useful if certain symptoms, signs, and
laboratory parameters associated with the
development of complications are identified so
that such cases may receive more attention.
The World Health Organization (WHO) case
classification of dengue into dengue fever
(DF)/dengue haemorrhagic fever (DHF)/dengue
shock syndrome (DSS) was formulated by the
Technical Advisory Committee at its meeting in
Manila, Philippines in 1974 and updated in 1997
(shown in fig 1). Several criticisms have been
raised against the case definition of DHF. It is
argued that the case definition of DHF is too rigid
and too difficult to apply in primary care or
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156
Rehman A, Khan HI
Asymptomatic
Dengue
Dengue
Infection
Symptomatic
Dengue Infection
Undifferentiated
Fever
Dengue Fever
Without
Hemorrhage
With
hemorrhage
Without
Shock
Dengue
Hemorrhage Fever
Dengue
Shock
Syndrome
4 criteria for
DHF
Evidence of
circulatory
failure
Rapid and
weak pulse
Narrow pulse
pressure (<20
mm/Hg) or
hypotension for
age
Cold clamy skin
www.ppa.org.pk
SEVERE DENGUE
With warning
Without
1.
2.
sings
3.
Nausea, vomiting
Rash
Leukopenia
Warning signs*
Abdominal pain or tenderness
Persistent vomiting
Clinical fluid accumulation
Mucosal bleed
Lethargy, restlessness
Liver enlargement >2 cm
Laboratory: increase in HCT
concurrent with rapid decrease
in platelet count
Shock (DSS)
Fluid
accumulation
with
respiratory distress
Severe bleeding
As evaluated by clinician
Severe organ involvement
Liver: AST or ALT>=1000
CVS: Impaired consciousness
Heart and other organs
DENGUE
FEVER
VERSUS
HAEMORRHAGIC FEVER
DENGUE
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158
the virus is present in the blood and its clearance
from this compartment generally coincides with
defervescence. Humoral and cellular immune
responses are considered to contribute to virus
clearance via the generation of neutralizing
antibodies and the activation of CD4+ and CD8+
T lymphocytes. In addition, innate host defence
may limit infection by the virus. After infection,
serotype specific and cross-reactive antibodies
and CD4+ and CD8+ T cells remain measurable
for years5,15. The hypothesis of antibody-mediated
enhancement is not sufficient to explain the
development of severe disease (DHF). The
pathogenesis of severe dengue is not well
understood and more than one mechanism may
be involved. It is the interplay between virus
factors and genetically determined host factors
that determine the disease outcome in the
individual patient14. It has been observed that the
risk of severe disease is increased at least 15-fold
during secondary
compared to primary
infections. Various mechanisms have been
suggested,
including
antibody-dependent
enhancement, complement activation by virusantibody complexes and T-cell mediated
immunopathology. Differences in virulence of
viral genotypes have also been suggested to
explain the pathogenesis of severe dengue5,16-17.
It has been extremely difficult to measure the
contribution of viral genetics to dengue disease
presentation in humans mainly because there
have been no adequate models of disease. It is
very difficult to say that DF and DHF are two
different clinical conditions2 or the same clinical
condition with varying severity5,18. The endothelial
cell activation may mediate plasma leakage
which seems to be functional rather than
destructive
effects
on
endothelial
cells.
Thrombocytopenia may be associated with
alterations in megakaryocytopoieses by the
infection and increased peripheral consumption.
Hemorrhage
may
be
due
to
the
thrombocytopenia and associated platelet
dysfunction
or
disseminated
intravascular
coagulation. These changes are mostly transient
and reversible and occur in severe dengue4,5.
www.ppa.org.pk
Rehman A, Khan HI
FLUIDS
Fluid management in dengue is difficultthe
balance between too much and too little is
critical and getting it wrong can be life-
159
www.ppa.org.pk
160
Rehman A, Khan HI
2.
3.
4.
5.
6.
7.
8.
9.
50 ml/kg upto 50 kg
REFERENCES
1.
www.ppa.org.pk
161
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
22.
23.
24.
25.
26.
Premaratna
R,
Liyanaarachchi
E,
Weerasinghe M, et al. Should colloid
boluses be prioritized over crystalloid
boluses for the management of dengue
shock syndrome in the presence of ascites
and pleural effusions? BMC Infect Dis
[Internet]. 2011 Feb 28 [cited 2011 Aug 25];
11: 52. Available from: http://www.
biomedcentral.com/content/pdf/14712334-11-52. pdf
27.
28.
29.
www.ppa.org.pk
INTRODUCTION
Rheumatic fever (RF) is an inflammatory disease
mediated by humoral and cellular auto-immune
response that occurs as delayed sequelae of
group A beta-hemolytic streptococcal infection
of the throat1. It affects the connective tissue
particularly in the heart, joints, skin and nervous
system2. Rheumatic Heart Disease (RHD) is a
crippling disease and is the most common
cardiovascular disease in children and young
adults and remains a major public problem in
developing countries3. Poor housing conditions
and over-crowding remain major contributing
factors in facilitating the spread of group A betahemolytic streptococci4.
RF and RHD cannot be separated from each
other from epidemiological point of view. Recent
data from Pakistan has shown a very high
prevalence of RHD in both urban and rural
population. In a large cross-sectional survey,
conducted on more than 25000 urban school
going children from inner Lahore using
echocardiography to confirm the cardiac lesion,
has estimated a prevalence of 22/10005. A study
from Rahim Yar Khan from a rural area showed a
prevalence rate of 5.7/1000. Less than 20% of the
patients were aware of their diagnosis and only
8% were taking rheumatic fever prophylaxis6.
Programs ensuring intense secondary prophylaxis
and primary prevention, have attained relevant
decrease in mortality, prevalence, incidence,
hospital admission and severity of RF/RHD in the
developing world7.
The Scientific Council of Pakistan cardiac Society
has extensively reviewed the current evidence
based data on Rheumatic Fever to update the
guidelines for Pakistan. The main objective of
www.ppa.org.pk
Pakistan Cardiac Society Guidelines for Prevention of Rheumatic Fever and Rheumatic Heart Disease
Arthritis:
163
www.ppa.org.pk
164
PRIMARY PREVENTION
Primary prevention of acute rheumatic fever is
accomplished by proper identification and
adequate antibiotic treatment of group A
B-hemolytic
streptococcal
(GAS)
tonsillopharyngitis. The aim of the primary
prevention is to promptly recognize and treat all
the patients with group A beta-hemolytic
streptococcal tonsillopharyngitis. While this
approach is therapeutically simple, in practice, it
is difficult to achieve. In order to prevent a single
case of RHD, several thousand cases of
streptococcal throat infection must be identified
and treated17.
At least, one third of acute RF results from inapparent streptococcal throat infection17-18. In
addition, not all symptomatic patients seek
medical care. Therefore, in these instances, RF is
not preventable. Primary prevention is difficult
even if strict management of sore-throat is
followed on scientific lines18. Benzathine Penicillin
G is the anti-microbial agent of choice for the
treatment of GAS, except in patients with history
of penicillin (PCN) allergy. There has been
concern raised that recurrent Group A betahemolytic (GABH) infections occur more
frequently after PCN treatment than with other
anti-microbial therapy, indicating emergence of
resistant strains against penicillin PCN19. However,
Benzathine PCN still is the drug of choice as it has
narrow spectrum of activity and long standing
proven efficacy and is the least expensive
regimen. PCN may be administered IntraMuscularly (IM) or orally, depending on the
patient`s likely adherence to an oral regimen20.
In many countries resistance to macrolide
antibiotics has reached more than 15%. This must
be taken into account when considering a
macrolide therapy for Group A streptococcal URT
infection21. There have been no controlled studies
showing that tonsillectomy is effective in reducing
the incidence of RF and it is not recommended
for the primary prevention of RF22. Treatment of
streptococcal
tonsillopharyngitis.
The
oral
antibiotics of choice are penicillin and amoxicilli .
The recommended dosage of intramuscular
www.ppa.org.pk
SECONDARY PROPHYLAXIS
Due to impossible logistics and financial
constraints associated with primary prevention,
focus has been to prevent the recurrence of
Rheumatic Fever (secondary prevention). This
approach is more practical and has shown to be
effective in developing countries24. An individual
with previous attack of RF, in whom
streptococcal pharyngitis develops, is at higher
risk for developing recurrent attacks of RF. A
group A beta hemolytic streptococcal (GAS)
infection need not to be symptomatic and can
trigger recurrence, even if optimally treated.
Further, with each recurrence of RF, the chances
of acquiring carditis increase exponentially.
Duration of Prophylaxis: Risk of recurrence depends
165
Pakistan Cardiac Society Guidelines for Prevention of Rheumatic Fever and Rheumatic Heart Disease
TABLE 1: Criteria for the Diagnosis of Rheumatic Fever and Rheumatic Heart Disease (Pakistan Cardiac Society 2010)
Diagnosis Categories
Criteria
Primary Episode of RF
Rheumatic Chorea
Minor Manifestations**
Fever
Raised ESR or CRP
Polyarthralgia (When arthritis is present as major criteria,
arthralgia can not be considered as minor)
Dose
Mode
Benzathine penicillin G
Intramuscular
Penicillin V
Oral
Sulfadiazine
0.5 g once daily for patients <27 kg (60 lb), 1.0 g once daily
for patients
Oral
Macrolide or Azalide
Oral
Duration of Prophylaxis
Minimum of 10 years after most recent episode ARF or until age 21 years
(whichever is longer)
Minimum of 10 years after most recent episode ARF or until age 30 years
and then specialist review for consideration of the need for continuation of
prophylaxis.
www.ppa.org.pk
166
Distraction
techniques
conversation)
Prevention
of
ACKNOWLEDGMENTS
We would like to thank the following for their
support and encouragement in formulating the
guidelines on prevention of rheumatic fever and
rheumatic heart disease.
www.ppa.org.pk
during
injection
(e.g.
with
-------------------------------------------------------------------------------Authors affiliations
Naveed Akhtar,
Chairperson Scientific Council of Pakistan Cardiac
Society on Valvular Heart Diseases, Associate Professor
of Cardiology, Shifa International Hospital, Islamabad.
Prof. Masood Sadiq,
Department of Paediatrics Cardiology, The Children`s
Hospital/The Institute of Child Health & Punjab Institute
of Cardiology, Lahore. drmasoodsadiq@hotmail.com
Faiz ul Hassan Rizvi,
Department of Paediatrics Cardiology, Sheikh Zayed
Medical College, Rahim Yar Khan.
Haseena Chagani,
Department of Pediatrics Cardiology, Dow University of
Health Sciences Karachi.
Asad Hafeez,
Health Services Academy of Islamabad.
Adnan Gul
Department of Paediatrics Cardiology, Lady Reading
Hospital, Peshawar.
Madeeha Hina,
Penicillin Clinic Pakistan Cardiac Society Islamabad.
REFERENCES:
1.
Pakistan Cardiac Society Guidelines for Prevention of Rheumatic Fever and Rheumatic Heart Disease
2.
3.
4.
5.
12.
167
EdwardsWDetal.Activevalvulitisassociatedwith
chronicrheumaticvalvulardiseaseandactive
myocarditis.Circulation.1978;57:181185.
13.
14.
15.
6.
16.
7.
17.
18.
19.
20.
21.
22.
8.
9.
10.
11.
www.ppa.org.pk
168
www.ppa.org.pk
24.
25.
26.
27.
CASE REPORT
-----------------------------------------------------------------Authors affiliations
------------------------------------------Correspondence to:Shaafia Fatima
Department of Paediatrics, Sir
Ganga Ram Hospital, Lahore.
E-mail:
shaafia1986@hotmail.com
ABSTRACT
INTRODUCTION
Nephrogenic Diabetes Insipidus is a rare disorder
of water metabolism characterized by an inability
to concentrate urine even in presence of
Antidiuretic hormone1. Mostly congenital but can
be acquired (secondary) due to disorders
affecting renal tubular functions1,2. Incidence of
Diabetes Insipidus in general population is 3 in
100,000 with a slightly higher incidence among
males (60%)2. We report here a case because of
its rarity3.
CASE REPORT
A male baby weighting 2.6 kg, who was born
following a normal pregnancy and delivery. He
could not gain height and weight according to
his age. He was second issue of a cousin
marriage, was breast fed and appeared healthy
otherwise. Weaning started at 5 months of age
but was not tolerated by him; he began to vomit
several times a day, cried unless given milk and
soaked 10-12 diapers daily. He also had
constipation passing hard pebble like stools every
5th day and sometimes after giving suppository.
History of repeated hospital admissions due to
dehydration was also there. Now at 11 months of
age he presented to our emergency with
dehydration and irritability. He had dry skin,
sunken eyes, slow skin pinch and slightly moist
www.ppa.org.pk
170
DISCUSSION
Congenital Nephrogenic Diabetes Insipidus (NDI)
is a rare disease the most common pattern of
inheritance is an X-linked recessive disorder which
occurs from mutation in antidiuretic arginin
vasopressin V2 receptor gene (AVPR 2).
Autosomal dominant or recessive pattern is due
to mutation in gene designated AQP21,4.
Hypercalcaemia and hypokalemia can also
cause partial Nephrogenic Diabetes Insipidus.
Diagnosis is suspected in a male infant with
polyuria, hypernatremia and dilute urine. Serum
osmolality >290mOsm/kg and urine osmolality
<290mOsm/kg is suggestive of Nephrogenic
Diabetes Insipidus1
CONCLUSION
Main differential diagnosis is central diabetes
insipidus which can be excluded by doing water
deprivation test and administration of vasopressin
intranasally. Prevention of recurrent dehydration
and hypernatremia in patients with congenital
Nephrogenic Diabetes Insipidus has significantly
improved the neurodevelopmental outcome.
-------------------------------------------------------------------------------Authors affiliations
Shaafia Fatima, Hafsa Muhammad, Prof. Ayesha Arif
Department of Paediatric, Sir Ganga Ram Hospital,
Lahore.
REFERENCES
www.ppa.org.pk
1.
2.
3.
4.
5.
6.
CASE REPORT
-----------------------------------------------------------------Authors affiliations
---------------------------------------------Correspondence to
Muhammad Naveed
Department of Pediatrics
Medicine Fatima Memorial
Hospital Lahore.
Email:
naveed_khaleeque@hotmail.
Com
ABSTRACT
This is the case report of three babies age 4 months, 21/2 months and
2 months who presented to us. Case 1 and 3 as sepsis, whereas case 2 as
anemia without visceromegaly. Appropriate treatment failed and bone
marrow biopsy revealed malignant histiocytosis in case 1 and
hemophagocytosis in case 2 and 3.
Keyword: histiocytosis, Hemophagocytic Lymphohistiocytosis, Langerhans
cell.
INTRODUCTION
These disorders are grouped together because
they have in common a prominent proliferation
or accumulation of cell of the monocytemacrophages and dendritic cell in affected
tissues. These disorders sometime are difficult to
distinguish clinically and usually treated as sepsis.
Hence Histiocytosis syndrome should be kept in
mind with differential of sepsis
Case1: A 3rd issue of consanguineous marriage,
3 months old baby boy presented with history of
fever for 1 week, which was low grade to start
with became high grade intermittent without any
constitutional symptoms. Two days later he
developed cough and breathing difficulty
followed by loose motion, 6-7 episode/day,
grade 4 without blood and mucous .Child was
breast and formula fed, immunized according to
EPI, programme. He had one healthy sister 3
years old, where as one brother died at age of 30
day due to prematurity and its complications.
On examination baby had no dysmorphism,
growth parameter were at 50th percentile, looked
sick was tacypneic, dyspneic maintained O2>
90% at Fio2 of 0.40. There was good bilateral air
entry without added sound. Abdomen was
distended, with liver 5 cm, spleen 4 cm palpable
below costal margin. Rest of the examination was
normal.
Initial CBC showed Hb 9.9 gm/dl, WBC count
6.0x103, neutrophils. 30%, lymphocytes 60%,
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172
exposure. She was born normally at home, no
history of maternal fever, rash and jaundice.
Father and mother were not cousins. She was
active, pale baby with no dysmorphic features.
All growth parameter at 50th percentile. Pallor was
positive rest of examination was unremarkable.
On basis of history and examination provisional
diagnosis was Blackfan Diamond anemia so
bone marrow biopsy requested. On bone
marrow
biopsy
impression
was
Hemophagocytosis with megaloblastic bone
marrow. Serum folic acid, Vit B12, fibrinogen and
serum cholesterol were normal. Antimalararial,
antiviral started and blood transfused. This patient
refused chemotherapy and is still on follow up.
On follow up at 2 month of age she developed
hepatomegaly. At 3 month of age she reported
with chest infection and thrombocytopenia.
Currently she requires packed cell and platelets
transfusions repeatly.
Case 3: A male baby, 2 month old, presented
with history of high grade intermittent fever for 15
days along with respiratory difficulty and
reluctance to feed. Antibiotics and antimalarial
were given at local health facility but fever did
not settle and child was referred to Fatima
Memorial Hospital. At presentation fever was
persistent but respiratory difficulty and feeding
problems were improved.
On examination he was irritable baby with no
dysmorphic feature, growth parameter at 50th
percentile, maintaining O2 saturation at room air.
He had hepatomegaly and splenomegaly; 8cm
and 6 cm respectively. CVS, respiratory and CNS
examination were normal.
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173
Syndromes
LCH
Eosinophilic Granuloma
Congenital
self-healing
reticulohistiocytosis
II
Histiocytosis
of
mononuclear
phagocytes other than Langerhans
cells
Familial
and
reactive
hemophagocytic lymphohistiocytosis
(HLH)
Sinus histiocytosis with massive
lymphadenopathy (SHML, RosaiDorfman disease)
Reticulohistiocytoma
Juvenile xanthogranuloma (JXG)
III
TABLE 2: Diagnostic
Lymphohistiocytosis
criteria
for
Hemophagocytic
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174
The diagnostic distinction between FHLH and
secondary HLH sometimes can be based on the
acute onset of secondary HLH in the presence of
a documented infection. In this case, treatment
of the underlying infection, coupled with
supportive care, is critical. If the diagnosis is made
in a setting of iatrogenic immunodeficiency,
immunosuppressive
treatment
should
be
withdrawn and supportive care should be
instituted along with specific therapy for
underlying infection. When FHLH (gene mutations
in perforin or Munc 134 proteins) is diagnosed or
suspected and when an infection cannot be
documented,
therapy
currently
includes
etoposide, corticosteroids, and intrathecal
methotrexate. Some recommend antithymocyte
globulin and cyclosporine for maintenance
therapy. Nevertheless, even with chemotherapy,
FHLH remains ultimately fatal, often after a
relapse of the disease2. Allogeneic stem cell
transplantation
is
effective
in
curing
approximately 60% of patients with FHLH. In
contrast, in secondary HLH, when an infection
can be documented and effectively treated, the
prognosis is good without any other specific
treatment. When a treatable infection cannot be
documented, which the case in most patients is
presumed to have secondary HLH, the prognosis
may be as poor as that of FHLH, and an identical
chemotherapeutic
approach,
including
etoposide, is recommended2.
Acute monocytic leukemia and true malignant
histiocytosis are included among the class III
histiocytoses, because they are unequivocal
malignancies of the monocyte-macrophage
lineage. These patients present with sudden onset
of generlized symptoms that include fever,
sweating, weight loss, enlarged lymph nodes,
heptospleenomegaly,
anemia,
leukopenia,
www.ppa.org.pk
REFERENCES
1.
2.
Ladish
S.
Histiocytosis
Syndrome
of
Childhood. In: Kleigman RM, Behrman RE,
Jenson HB, Stanton BF, editors. Nelson
textbook of pediatrics. 18th edition.
Philadelphia: Elsevier; 2007: 2159-61.
3.
4.
5.
CASE REPORT
Pseudohypoaldosteronism,
Hypothyroidism
Hypoparathyroidism in a Two-Year-Old Boy
and
ABDULMONEIM AL-AGHA
-----------------------------------------------------------------ABSTRACT
Authors affiliatios
-------------------------------------------Correspondence to:Abdulmoein Al-Agha
Department of Pediatrics, King
Abdulaziz University Hospital,
P.O.Box 80215, Jeddah 21589,
Saudi Arabia
E-mail: aagha@kau.edu.sa
INTRODUCTION
Pseudohypoaldosteronism (PHA) is a rare
condition of salt wasting in infancy with renal
tubular resistance to the actions of aldosterone.
The disease is divided into 2 forms: primary and
secondary (transient) PHA. The primary form is
further divided into type 1 and type 2. Type 1 is
subdivided into renal type 1 (also known as
Cheek and Perry syndrome), Multiple Target
Organ Defect type 1 (MTOD) and Early
Childhood Hyperkalemia. Type 2 is subdivided
into Gordon Syndrome (also known as Chloride
Shunt syndrome), and adolescent hyperkalemic
syndrome. The underlying pathology differs
according to the types and subtypes of PHA,
which is due either to a defect in the quantity
and functions of aldosterone receptors, the
epithelial sodium channels or the sodium-chloride
co-transporters.
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176
and renal function should be normal. Renal
ultrasound is usually indicated to rule out any
urinary tract malformations, urinalysis and urine
culture should be done to rule out urinary tract
infection. Management includes rehydration,
correction of acidosis and hyperkalemia with
alkalinizing
agents
(sodium
bicarbonate).
Potassium binding resins (sodium resonium) are
usually prescribed to control hyperkalemia. High
sodium and low potassium diets are advised, in
addition to the supplementation of sodium
(sodium chloride). The use of exogenous
mineralocorticoids has no role in correcting the
abnormalities of this disorder. During outpatient
care serum electrolytes, height, weight and
blood pressure should be closely monitored. We
present a case illustrating the unusual association
of pseudohypoaldosteronism, hypothyroidism
and hypoparathyroidism in a two-year-old boy.
CASE REPORT
A two-year-old male patient, product of 34
weeks of gestation, with a birth weight of 1.8 kg
presented to the emergency room of King AbdulAziz University Hospital, Jeddah, Saudi Arabia, a
few days after birth. He had a two-day history of
frequent attacks of apnea, associated with bluish
discolouration of his lips (each lasted for a few
seconds), poor activity, reduced oral intake and
poor weight gain since birth. His vital signs were
stable except for an oxygen saturation of 88% on
room air. He was lethargic and irritable, in
addition to being underweight for his age but
with no dysmorphic features.
Regarding family history; his parents were first
degree cousins, and his brother died during
infancy at the age of 3 months in another
hospital secondary to hypocalcemic convulsions
and electrolyte imbalance. There was no
medical record of the brother's case. During his
first admission, the laboratory investigations
revealed severe hyponatraemia of 125 mmol/L
(136-145), hyperkalemia of 6.4 mmol/L (3.5-5.1)
and moderate metabolic acidosis pH: 7.22 (7.377.45), a serum bicarbonate level of 18.5 mmol/L
(21-26). A hormonal study was done showing a
normal serum cortisol level of 170.6 nmol/L (138636), normal levels of 17- hydroxyprogesterone of
3.2 ng/ml (0.6-7), normal ACTH level of 18.76
www.ppa.org.pk
Al-Agha A
DISCUSSION
A thorough literature review regarding the topic
at
hand
(pseudohypoaldosteronism,
hypothyroidism and hypoparathyroidism) that
extended to the past decade revealed no
reported cases with similar associations. The
cases
we
found
were
of
transient
pseudohypoaldosteronism with urinary tract
malformations,
i.e.
vesicoureteral
reflux2
4.
5.
6.
7.
8.
9.
10.
ACKNOWLEDGMENT
I would like to thank Mrs. Joy Almeda De Silva for
her secretarial assistance.
REFERENCES
1.
2.
177
www.ppa.org.pk
ABSTRACT
Abstract Service
-----------------------------------------------------------------Pak Paed J 2011 35(3): 178-79
by
www.ppa.org.pk
by
179
Abstract Service
A,
Wagdy
M,
Alali
M,
by
www.ppa.org.pk
www.ppa.org.pk
181
Society. Their policy statement will be published in
Pediatrics, September 2011 issue.
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1.
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2.
Pamper
3.
Nutri Mama
4.
5.
M/s GlaxoSmithKline
ii)http://www.pakmedinet.com/PPJ
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