HO/01(P) THE ASSOCIATION BETWEEN IRON DEFICIENCY ANEMIA AND
FEBRILE CONVULSION
Kunwar Bharat, B.Mukherjee , Khalid Md. Saiffulah, Rajiv Sharan, S.Nath .
Department of Paediatrics, Tata motors hospital, Jamshedpur 831008
bibek737@gmail.com
Introduction: Febrile seizures[FS] are the most common seizures in children. Their incidence
ranged between 2-5 %. The pathophysiology of febrile seizures is unknown. However, there
are some studies which are giving conflicting results about association of FS with iron deficiency
anemia, which is a very common pediatric problem. Aim and Objectives: To study the
association between febrile seizures and iron deficiency anemia. Material and methods:
prospective case control study done in Tata Motors Hospital from August 2009 to July 2010. All
patients with history suggestive of febrile seizure between age of 6 months to 5 years and
controls matched for age and sex were included in the study. Exclusion criteria: Any metabolic
disturbances, developmental, structural or neurological abnormalities or infective CNS pathology
.Patients were divided in two groups as per history and examination. Group 1 Cases (50): with
history of febrile seizures. Group 2 Control (50): with febrile illness of any origin without
seizures. A standard protocol for doing Hgb, HCT, MCV, MCH, Serum Ferritin estimation in
both groups were drawn. Analysis: Final evaluation done in respect of certain fixed parameters
and total data collected were analyzed to arrive at a conclusion. Statistical data – t test applied. T
value was chosen as the data was continuous and we wanted to find out significant differences
between the two groups. Age: Case 18.16+/-8.09 months, Control 20.64+/- 5.16 months (t value
1.80) Sex Case Male=35 female=15, control Male =39 Female=11 (t value .83)Hgb (11-13.6
gm/dl) Case 10.86+1.23 Control12.72+/- 1.33 (t value-.97) MCV(75-87 fl) Case72.37+5.77
Control 74.07+5.34(t vaue-1.52) ,MCH (25.4-29.6pg) case 23.02+2.88 Control 23.72+3.85 (t
value 1.03) ,MCHC (32.9-35.7) cases 31.76+1.79 Control 32.09+1.94(t value 0.89) Sr Ferritin
(10-300 ng/ml) Case 42.17+17.15 Control 50.40+30.12 (t value-1.66) Conclusion: There was no
statistical difference between the groups(case Vs control) ,in variables like Hemoglobin, MCV,
MCH, MCHC and Sr. Ferritin. (T value suggesting no significance either at 0.01 or at 0.05 level
of significance i.e. >2.5) .Hence there is no association between iron deficiency anemia and
febrile seizure.
HO/02(P) CORRELATION OF SERUM FERRITIN WITH
CONCENTRATION BY MRI IN THALASSEMIA MAJOR
Maj Ritesh, Wg Cdr V Venkateshwar, Col S Chatterjee, Col R Ghuliani
Department of Pediatrics, AFMC & CH(SC) Pune
ritesh2000sriji@yahoo.com
LIVER
IRON
Introduction: Assessment of iron overload in multi-transfused thalassemia major is routinely
done by serum ferritin levels. Magnetic Resonance Imaging is a non-invasive method of
determining liver iron overload. There are scanty Indian studies correlating these two parameters.
Aims and objectives: To study the correlation of serum ferritin level with liver iron concentration
measured by MRI in multi-transfused children with thalassemia major Material & Methods: This
cross sectional study was conducted at a tertiary care teaching hospital. The study group
consisted of children with thalassemia major below the age of 14 years, receiving regular blood
transfusions, with or without chelation therapy. Serum ferritin level was estimated on the date of
MRI study. MRI T1, T2 weighted imaging of the liver was done and liver iron concentration
was quantitatively estimated using the Univ of Rennes version 2003 software for 1.5 T scanner.
Spearman’s correlation method was used. Results: 30 patients were included in the study, with
age group ranging from 6 -14 yr. The total number of transfusions ranged from 54 to 330. 18
children were on chelation therapy. Average duration of chelation was 6 years. Serum ferritin
ranged from 100 to 34,200 mcg/ml, with a mean of 5419.7 mcg/ml . The liver iron
concentration ranged from 160 micromol/g to more than 370 micromol/g (normal: < 36
micromol/g). The Spearman’s coefficient of correlation was 0.656. Conclusion: Serum ferritin
correlates moderately with liver iron concentration quantified by MRI. MRI is recommended to
non-invasively detect early liver iron overload in multi-transfused thalassemia major. - This the
largest study done in India on thalassemic patients to find the correlation between serum ferritin
and Liver iron concentration measured by MRI Liver. MRI is a fast, non-invasive and relatively
accurate diagnostic tool for assessing liver iron concentration (LIC). LIC never changes with
inflammatory condition of the body. Monitoring the Iron overload of the body is essential in
cases of thalassemia. Total body iron determines iron chelation therapy. Serum ferritin level
correlates poorly with hepatic iron concentration. Serum ferritin level is influenced by vitamin C
deficiency, hepatitis and many conditions where serum ferritin is elevated, as it is also a marker
of inflammation, all of which are seen in thalassemics(1). Liver iron concentrSation is not
affected by these conditions, is presently taken as a most accurate method to predict total body
iron stores (2). LIC can be measured by liver biopsy (invasive method and can not be repeated
many times for monitoring the treatment response), SQUID (available only at four places in
world) and MRI liver. Total body iron stores in mg/kg= 10.6*the LIC (in mg/g dry weight). The
only non invasive and widely available method of measurement of LIC is MRI Liver (3). A total
of 30 children of thalassemia major were studied for correlation between liver iron and serum
ferritin. Our study reveals that there is no relation between these two. Serum ferritin may be used
to monitor the therapeutic response, but it does not reflect the true burden of total iron of the
body. High levels of serum ferritin showed linearity with liver iron content (r = p = ). Serrum
ferritin levels has a tendency to be significantly correlated with the true status of iron load in
thalassemic patients; however, the discrepancies recorded in several patients and the scarce or
total lack of correlation with MRI(4). The study concludes that magnetic resonance imaging has
good potential to quantify the liver iron deposition non-invasively and may denote the efficacy of
iron-chelation therapy which is used to reduce the body iron burden in these patients.
HO/03(O) FINANCIAL BURDEN ON THE FAMILIES
DEPENDENT THALASSEMIC CHILDREN
Karanjit Singh, Karnail Singh, Ravikaran Singh, Dilnoor Kaur
D-124, Ranjit Avenue, Amritsar-143001
kjspatti@yahoo.co.in
OF
TRANSFUSION
Introduction: The thalassemias are a heterogeneous group of inherited hypochronic anaemias of
varying severity. The mainstay of the supportive treatment is regular blood transfusion
accompanied by iron chelating therapy. The lifetime cost of treating a patient with thalassemia
major is estimated at > US $ 1,10,000 in UK and US $ 2,84,154 in Israel. No definite data on per
capita expenditure on thalassemic children in India per year is available. The cost of supportive
treatment only of thalassemic children, makes a huge financial burden on the families of
thalassemic children, a cause of concern for the poor families. Aims and Objectives: Present
study has been taken to estimate the monthly expenditure; a financial burden on the families of
regularly transfused thalassemic children. Material and Methods: The parents of 59 patients of
transfusion dependent thalassemic children transfused regularly at the department of pediatrics,
Govt. Medical College, Amritsar, were interviewed, as per the structured proforma. Results /
Observations: Out of 59 transfusion dependent thalassemic patients, 42 (79.66%) were male and
17 were female (28.85%). 9 patients out of 59 (15.25%) were less than 4 years of age, 25
(38.98%) were between 4-8 years, 13 (22.03%) were of 8-12 years and rest 12 (20.33%) were in
the age group of 12-18 years. Monthly income in majority of the cases i.e. 27 out of 59 (45.76%)
was between Rs. 3500-7000/- except 4 out of 59 (6.77%) where it was even less than Rs. 3500/( a family of 5), 19 (32.20%) were in the income slab of 7000-12500/- and only 9 i.e. 15.25%
were earning more than 12500/- per month. No amount was spent on the investigations for rest
of the family members to detect the carrier stage of thalassemia in 34 out of 59 (57.62%). The
total expenditure incurred on the investigations for detecting the thalassemia major and the
carrier stage on whole of the family, in majority of the cases, i.e. 23 families out of 59 (38.98%)
was less than Rs. 5000/-, while 12 (20.33%), 3 (5.08%) and 21 (35.59%) have spent in the range
of Rs. 5000-10000, Rs. 10000-15000 and Rs. 15000 and above respectively. As for as total
expenditure on the transportation and purchase of logistics, 39 out of 59 (66.10%) have spent Rs.
200-400 while 8 (13.55%), 4 (6.77%) and 8 (13.55%) spent less than Rs. 200, Rs. 400-600 and
Rs. 600 and above respectively. Considerable number of patients i.e. 48 out of 59 (81.35%)
received more than 50 units of blood transfusions, thus requiring iron chelation therapy but only
21 patients (35.60%) have taken it that too for a variable period and irratically. The cost factor
prevailed in discontinuation of iron chelation therapy in majority of the cases. If we go by the
income group slab adopted in this study, all the families 4 out of 4 in the income group of less
then Rs.3500/- (100%) could spend only Rs. 700/- per month. In the income group of Rs. 700012500/- per month, 10 out of 19 (52.63%) have afforded only Rs. 1400/- or less. In the income
slab of Rs. 12500/- per month, only one (11.11%) could afford beyond Rs. 4200/- per month.
The expenditure per blood transfusion, in majority of families i.e. 32 out of 59% (54.73%) was
less than Rs. 700/- per blood transfusion. Only 3 patients (5%) were given blood transfusion with
leucocyte filtration set, costing Rs. 650-700/- per set. The total ideal expenditure which includes
leucocyte filtration set proper investigations and iron chelation therapy comes out to be Rs.
2500/- per month, which only a few have afforded in this study. Conclusion: All the transfusion
dependent thalassemic children have got the fundamental right of supportive as well as curative
treatment, which cannot be provided by the family alone. The huge financial burden for
treatment on these patients is to be shared by NGOs, Thalassemic Societies and the Govt. of
India through school health programme.
HO/04(P) HEMOLYTIC UREMIC SYNDROME: A DIAGNOSIS TENDS TO BE
MISSED IN CHILDREN
Payal Shah, Manish K. Arya, A. D. Rathod, Pallavi Saple.
Dept. of Paediatrics, Grant Medical College & Sir J.J. Group Of Hospitals, Mumbai 8.
manjioo7@yahoo.co.in
Introduction : HUS is primarily a disease of infancy and early childhood characterized by the
triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Case
Report: 1yr old girl presents with loose motion, vomiting and fever since 7 days. She had
decreased frequency of urine since 4 days and altered sensorium since morning. Birth and
developemental history were normal. On examination child was drowsy with pulse rate of
135/min with APPWF. Respiratory rate was 36/min with mild retraction. BP was normal with no
sign suggestive of dehydration. Pallor was present alongwith periorbital edema. No Cyanosis,
Clubbing, Icterus or petechial rash was present. CNS examination showed GCS:7/15
(E1M4V2). Cranial nerves and fundus were normal. Tone was normal with power more >3/5
with normal reflexes. No evidence of meningeal irritation. On P/A there was
hepatosplenomegaly. Other systemic examination was normal. CBC showed normochromic
normocytic anaemia and thrombocytopaenia with Hb- 8.7, TLC-30000 and Platelet-22000.
Peripheral Smear showed Schistocytes with fragmented RBC’s suggestive of microangiopathic
haemolytic anaemia. PT and PTT were normal. There was electrolyte imbalance with S.Na 121
and S.K 2.9 meq/L. RFT was dearranged with BUN 208 and S.Creatinine 2.9. CRP and Coombs
test were negative. Occult blood test was positive in urine and stool. Blood c/s isolate no
organism. Patient was managed with restricted fluid, diuretics and peritoneal dialysis.
HO/05(P) CORRELATION OF BLOOD GROUP INCOMPATIBILITIES WITH
NORMOBLASTEMIA AND DIRECT COOMBS TEST.
K.K.Locham, Manpreet Sodhi, Ashwani Kumar
Deptt. Of Pediatrics & Biochemistry, Govt. Medical College/ Rajindra Hospital,Patiala-147001
kklocham@hotmail.com
Blood group incompatibilities are an important cause of neonatal jaundice. Normoblastemia and
direct combs test is done in such cases to predict the occurrence of clinical jaundice. Aims &
Objective:To find out correlation of blood group incompatibilities with normaoblastemia and
direct coomb’s test. Material & Methods:The study was conducted on 20 babies with blood
group incompatibilities (15 ABO & 5 Rh) admitted to Neonatology Section of Department of
Pediatrics, Govt. Medical College, Patiala. Blood group and parity of mother along with
gestation,sex,apgar score and weight of baby were recorded.Cord blood was evaluated for blood
group of baby,and direct coomb’s test.Peripheral blood film was examined for normoblasts.
Result:Out of 20 babies,13 were preterm and 7 were term.There were 11 male and 9 female
babies ,70% mother were nulliparous whereas 15% each were Para1 and Para 2 respectively.5
mothers were given anti-RhD in previous pregnancy.15% babies had birth asphysia,only one
baby with Rh incompatibility had normblasts in PBF upto 24/100 WBC,rest did not have
significant normoblasts.None of the baby had positive direct coombs test. Conclusion:Only one
baby (5%) with Rh incompatibilities showed normblastemia.Though direct coombs test was
negative in all.
HO/06(P) TO STUDY VARIOUS HORMONAL ASSAY IN SICKLE CELL ANEMIA IN
ADOLESCENT
Sharja Phuljhele, Virendra Kumar Kurrey, D.K Patra, Kodiyar
A-15 Parshuram Nagar, Near Mining Office , Ring Road No.-1,Raipur
sharjaphuljhele@yahoo.com
Introduction: Sickle cell Disease is hereditary chronic hemolytic anemia with word wide
prevalent .present study is an effort to various hormone in sickle cell anemia. Aims &
Objectives: To assess impact of Sickle cell Disease on hormonal level in Adolescent. Material
& Method: Study conducted in department of pediatrics and Department and Bio Chemistry Pt.
JNM Medical College Raipur. Total 40 patients were taken into study, Out Of 40, 20 are
homozygous & 20 were trait were, (60% Female & 40% Male). All Patients are undergone
estimation of thyroid hormone (T3, T4, TSH), RBS, LH, FSH, prolactine , testosterone ,estradiol
. The Result Were Calculated & mean 2SD Value were Compared with those obtained In Age &
Sex Matched Normal Control. Observation: Hormonal assay were carried out in all forty patient
considering their sex. Finding indicate decrease level of gonadal hormone in 20% Sickle cell
homozygous shows significant Lower level of Hormone i.e. .LH, FSH , Testosterone . Only
4% Cases of Sickle cell trait show significant impact on gonadal hormone .24% Patient show
boarder line low hormonal level of both Sickle cell disease & trait.55% patient with sickle cell
homozygous show lower T3,T4, & high TSH level , rest show normal level in both sex .Only
2% SCD show low level of RBS. Conclusion: The Finding indicates that in Sickle cell
homozygous has significant impact on thyroid and gonadal hormone as compared to trait and
healthy children.
HO/07(O) PSYCHIATRIC DISORDERS AND
PSYCHOSOCIAL ASPECTS IN
THALASSEMIC CHILDREN AND THEIR CARE GIVERS
Tonke S, Shrivastava J, Sahu RN, Dwivedi R
Department of pediatrics, Gandhi Medical College, Bhopal
mydrsudhir@gmail.com
Introduction – Beta thalassemia major, is a chronic, genetically determined hematological
disorder poses severe psychosocial stress to the patient and considerable burden to their care
givers. Aims & Objectives – To study psychiatric disorders and social morbidities among
thalassemic children and their care givers. Material & Methods –A cross sectional study
conducted over a period of one year in a tertiary care medical college hospital. Forty Six
thalassemic children ( 5 – 16 years) and their care givers were included in the study after written
informed consent. Details regarding demography, clinical and various psychosocial life aspects
were collected using a pre-tested proforma and finally the study group underwent a clinical
psychiatric interview and a diagnosis was ascribed as per DSM – IV TR . Results – Sixty five
percent (n=30/46) of thalassemic children had psychiatric problems with anxiety disorder in
19.5% (9/46) and elimination disorder (nocturnal enuresis) 19.5% (9/46) were most common
disorders followed by depression in 15.2% (7/46), ADHD in 8.7% (4/46) and phobic neurosis in
2 % (1/46). Adverse impact of disease was also perceived in the domains of education (86%),
sports (65%), physical growth (under nutrition in 69% and stunting in 58%) and self image
(8.6%). Among care givers 50% (23/46) had psychiatric problems of which depressive disorders
(major depression 6.5%,3/46, dysthymia 26%, 12/46) is the most common followed by addiction
and substance abuse 10.8%, (5/46) and anxiety disorder 4.5% (2/46). Conclusion – Results of the
study showed that majority of the thalassemic children and their caregivers had psychiatric and
severe psychosocial problems emphasizing the need of psychological aid and psychiatric
treatment apart from medical management of disease.
HO/08(P) ECG AND ECHO CHANGES IN SICKLE CELL DISEASE
Sham B. Lohiya, N.L. Phuljhele, Suryavanshi, S. Phuljhele
sham.lohiya19@gmail.com
Introduction :- Cardiovascular changes are comman in SCD. In previous studies, Akinola and
balgoun et al found abnormal resting ECG abnormlities included LVH in 14 (63.8%), sinus
arrhythmia in 6 prolonged PR interval in (9.1%). LA Lester et at studied 64 children. Left atrial,
left ventricular and aortic root dimensions increased in over 60% of the children.
Farrana D. Palhankar et. al studied 62 patients with homozygous SS/SB0 by echo . 30% had
increased pulmonary artery systolic pressure Aims:-To study cardiovascular changes in children
with sickle cell disease at Dr.B.RA.M.H.RAIPUR Inclusion Criteria :Children with sickle
cell disease with SS pattern on Hb electrophoresis of age 2-14 yrs. METHOD:23 Children
studied in relation to cardiovascular system by ECG and out of these 23,15 were studied by 2-d
ECHO M –mode. Observation : In ECG, Sinus tachycardia observed in 7 [30%], LVH changes
in 6[26%]. T wave inversion in 3 patientsand RVH in 1.On echocardiography,aortic root
dilatation was observed in 5[33%] ,LV dialatation in 3 and LA dilatation in 2. Mean aortic root
value was18 mm and mean LV dimension was 37mm. Discussion :- Our study showed that
in ECG,sinus tachycardia and LVH are the commonest findings. On 2D – Echo, aortic root
dialatation is most comman finding and second is left ventricular dilatation. There is no evidense
of increased PASP in our study. Our study will be completed till the time of presentation in jan
2011.
HO/09(P) MATERNAL CARRIER SCREENING AND PRENATAL DIAGNOSIS OF
BETA-THALASSEMIA
Mehndiratta S, Mehndiratta A, Garg S, Vaid NB
B- 246, Yojna Vihar, Delhi- 110092
drsmehndiratta@gmail.com; drsmehndiratta@rediffmail.com
Introduction: Thalassemia is one of the most common inherited disorders of haemoglobin
synthesis. A large number of thalassemia children are born every year in our country. The only
definitive treatment is by a bone marrow transplant which is unaffordable for a majority of
patients. Regular blood transfusions and chelation therapy forms the mainstay of management.
Prenatal diagnosis is possible by the screening of the parents for carrier status and further
evaluation of the foetus. Materials and Methods: The antenatal thalassemia screening program is
operational in the Department of Gynaecology and Obstetrics of GTB Hospital. Screening for
carrier status is done in early pregnancy using NESTROFT and RBC indices. Confirmation of
the carrier status is done by HPLC. If the mother has the thalassemia trait, the husband is also
screened. If both the parents are positive for the thalassemia trait, they are counselled and the
foetus is then tested by DNA mutation analysis. Results: The data of 2 years (April 2008-March
2010) was analyzed. A total of 7307 pregnant women were screened. Both partners were found
to have the thalassemia trait in 12 cases. Further analysis revealed 4 foetuses to be thalassemia
major and in these cases the pregnancy was terminated. 5 foetuses were diagnosed to have
thalassemia trait and the mother continued with the pregnancy. 3 cases were lost to follow up.
Conclusion: A program for routine screening for thalassemia in the early antenatal period and the
determination of carrier status of thalassemia is a socially acceptable and feasible method to
avert the birth of a child with thalassemia major. The screening program is a cost effective
strategy to reduce the immense financial as well as social burden on the society due to this
disease.
HO/10(P) INFANTILE TREMOR SYNDROME ASSOCIATED WITH VITAMIN B12
DEFICIENCY- A CLINICAL PROFILE WITH HAEMATOLOGICAL CORRELATION
Vinay kumar, Sanjay.K.S, Rajashekar murthy, Shivananda
Indiragandhi Institute Of Child Health, Bangalore, Karnataka.
vinay_iv@yahoo.co.in
Introduction: Infantile tremor syndrome is characterized by anemia, hyperpigmentation of the
skin, delayed developmental mile stones and tremors. It is seen more commonly in the Indian
subcontinent.Objectives: To study the clinical profile of children with infantile tremor syndrome
and its association with vitamin B12 deficiency with haematological correlation. Design: A
retrospective study involving 16 children done over a period of 2years i,e between august2008
and august2010 at Indiragandhi institute of child health, Bangalore. Subjects And Methods: All
children presenting with the typical features of ITS i,e anemia, hyperpigmentation of the skin,
delayed milestones and tremors, admitted between aug2008 and aug2010 were analysed after
excluding other causes of anemia and neurological symptoms. A detailed clinical history &
physical examination was carried out. Investigations including complete hemogram, peripheral
smear , vitamin B12 and folic acid assay, bone marrow examination serum LDH level and
serum ferritin level were done. Results: Age and sex distribution- All children belonged to age
group between 6mts and 18mts with a male predominance(M:F – 11:5) Presenting symptoms Pallor(100%), Lethargy(87%), Delayed developmental milestones(68.7%), Tremors(68.7%),
Neuroregression(6.2%), failure to gain weight(81.25%), vomiting(18.7%),cough(37.5%),
diarrhea(18.75%) fever(68.75%) , CCF(25%). Feeding history- 62.5% children were purely
breastfed.Delayed weaning was present in all the cases with mean age of weaning being 8.8mts.
All children had a low calories intake(mean-690 kcal/day) and low protein intake(mean-5.6
g/day). Though 62.5% of the mothers were pure vegetarians,importance was not given for
vitamin rich foods like milk,pulses,etc. All children belonged to low socioeconomic status.
General physical and systemic examination revealed -Pallor(100%), undernutrition(100%), all
children looked chubby with dull expressionless face(100%), all had sparse hypopigmented
hairs(100%),
hyperpigmentation
of
the
skin(100%),
hepatomegaly(43%),
hepatospleenomegaly(25%), generalised hypotonia(18.7%) Investigations- All children had
anemia with Hb levels ranging from 1g/dl to 9.8g/dl(100%), about 75% had severe
anemia(Hb<5g/dl), leucopenia with neutropenia was seen in 37.5%, thrombocytopenia in 87.5%.
Pancytopenia in 87.5%, peripheral smear showed Macrocytic anemia in 62.5%, dimorphic in
37.5%. Serum ferritin was done in children with dimorphic anemia and those with iron
deficiency were excluded, Hypersegmented neutrophils in 100% children. Bone marrow was
hypercellular with erythroid hyperplasia and severe megaloblastic changes in 62.5%,
megaloblastoid changes in 37.5%, reversal of M:E ratio was seen in all cases. Vitamin B12
levels were low in all cases, folic acid levels were normal in all cases, LDH levels were elevated
in all cases. Mother’s vitamin B12 levels were low in 70% cases who were purely breastfed.
Treatment-All children were given vitamin B12(1mg/day) and folic acid(5mg/day) IM daily for
2weeks followed by same dose once in a month, proper dietary advice was given. Four patients
who were severly anemic in CCF received blood transfusion. Follow-up- Showed improvement
in all cases. Mean duration of tremor phase was 14.6days(range- 5to 28 days) anemia was
corrected in 3-6wks in all cases. Conclusion: Infantile tremor syndrome is invariably associated
with vitamin B12 deficiency. Early diagnosis and treatment promptly improves the neurological
and haematological symptoms and signs and prevents longterm sequelae.
HO/11(P) BONE MARROW FAILURE SYNDROMES: THEIR SIGNIFICANCE IN
INDIAN CONTEXT
Kameshore N, Tulika Seth, Nitin Gupta, Sanjeev Sharma, Narendra Agrawal, Tulika Seth,
P. Mishra, M Mahapatra, HP Pati, R Saxena.
Dept of Hematology, AIIMS, New Delhi
tuliseth@yahoo.com
Introduction-Inherited bone marrow failure syndromes (IBMFS) can present as anemia,
thrombocytopenia, pancytopenia and lead to bone marrow aplasia, myelodysplasia and
malignancies. However, the diverse clinical picture of these syndromes and their rarity result in
diagnostic difficulties. Aim- To study the incidence of IBMFS, and their outcome in an Indian
referral hospital. Methods- retrospective analysis of files of children with IBMFS, evaluated for
initial hemogram, clinical manifestations, associated features, treatment and outcomes. ResultsThe number of Fanconi anemia cases identified= 57, which are 12% of all cases of Aplastic
anemia, median age 10+2, Male : Female 4:1, without Fanconi phenotype-43%, only 3 from
consanguineous family. Age of onset of symptoms or presence of phenotypic features did not
predict survival. Response to androgen therapy significantly influenced survival responders 23+6
months versus non responders 14+2 months (p=o.oo1). Acute myeloid leukemia=3, MDS =1. A
total of 14 Diamond-Blackfan anemia, Male : female, 11:3, median age of presentation 18
months (range 2 months to 7 years). Sustained steroid response in only 28%. One patient
received a matched sibling transplant 4 years ago the rest are receiving supportive care. Single
case of Amegakaryocytic thrombocytopenia. Conclusion- The limitations of this study are that
children with severe manifestations or poor response to treatment are referred to our center.
These diseases may be much more common. Several cases were initially misdiagnosed as ITP,
and thalassemia outside. Family counseling, evaluation of other siblings and appropriate care are
needed to prevent adverse outcome.
HO/12(P) GROWTH ASSESSMENT IN THALASSEMIA MAJOR PATIENTS ON
DEFERIPRONE THERAPY
Sunil N. Jondhale, Anil K. Bhalla, Inusha Panigrahi, Ram K. Marwaha
Department of Pediatrics Advanced Pediatric Centre, Postgraduate Institute of Medical
and Education and Research, Chandigarh 160012
seeena_pgi@yahoo.co.in
Introduction: Thalassemia patients require regular transfusionfor survival.Growth retardation
may be due to multiple factors. Effect of deferiprone has not been well studied till date. Aims-.
To assess growth parameters (height, weight, sitting height, & BMI) in deferiprone-treated
thalassemia major and to determine if there is any correlation with serum ferritin (SF), pretransfusion Hb, dose and duration of deferiprone. Methods: The anthropometric parameters of
thalassemia major of age 2-20 years were recorded every 6 ±1 months and Hb, SF were recorded
at each visit. Results- Mean age of subjects was 13.5±4years. Mean Hb was 7.5 gm%. The mean
duration of deferiprone therapy was 6.6 years. The SF at starting of chelation therapy was 3665±
2865(mean±SD) and during study was 2501±1633ng/ml(p=0.007). Means± SD of initial height
was 115.43±19.1. Highest growth velocity was in age 2-6 years i.e 2.35cm/6 month, while
lowest in 14-18 years age group i.e 1.4cm/6 months. Mean±SD height of patients receiving
deferiprone >10years was 77.06±4.9cm compared to 68.80±8.9cm those receiving
<5years(p=0.003). Mean weight velocity /6 month was highest in 6-10 years group i.e 1.65
followed by 10-14 years group i.e,1.44kg/6month. Mean±SD weight of patients receiving
therapy >10 years was 41.52±8.3kg compared to 29.69±11.2kg for those receiving < 5 years
(p=0.026). Mean±SD BMI of patients was 17.24±2.1kg/m2; for girls & boys were 17.23±1.8 &
17.24±2.2kg/m2 respectively. Mean±SD sitting height of thalassemia major was 72.31±7kg/m2.
Conclusions- 94% of thalassemia major were below standard mean height and weight of NCHS
standards & 92% of WHO standards suggesting overall growth retardation. There was no
statistically significant effect of Hb, SF & dose of deferiprone. Those who were chelated for
longer time with deferiprone had better growth.
HO/13(P) BOMBAY BLOOD GROUP IN A NEONATE- A DIAGNOSTIC CHALLENGE
Manish Chokhandre, Sushma Malik, Charusheela Warke, Manish Ramteke, Harshal Lahoti.
C/o. Dr Manish Chokhandre , Pediatric Resident, Department of Pediatrics, 1st Floor, College
Building, TNMC & BYL Nair Hospital, Mumbai Central, Mumbai-400008
drmanish2310@yahoo.co.in
Introduction:-Bombay blood group‘Oh’ is one of the rare blood type wherein H-antigen (antigen
present in blood group O) is not expressed leading to absence of A and B antigens. It was first
discovered in Bombay by Dr.Y M Bhende in 1952, occuring in 0.0004% of human population.
Incidence of ‘Oh’ is 1 in 2,50,000 worldwide & 1 in 7600 in India. Case history:- G2P2L1
mother with hepatitis E, delivered a female preterm(32wks) with 1.4 kg. Baby developed
septicemia with depression in all hematopoietic cell line requiring transfusion of packed red
cells. Mother’s blood group was AB+ve, father was AB+ve and baby was O+ve .This was not
explained on mendelian inheritance of the ABO & Rh blood group classification. On further
investigation, it was found that baby as well as the other sibling had ‘Oh’. Baby recovered
completely and was enrolled in Institute of Immunohaematology (IIH) as a case of Bombay
blood group. Discussion:- Mutations in FUT1(H) gene, present over RBCs, which is responsible
for activation of enzymes and expression of A & B epitope, appears to be responsible for this
non expression of ‘H’ antigen. As both parents must carry this recessive allele to transmit this
blood type to their children, this condition mainly occurs in small closed-off communities where
there is a good chance of both parents of a child either being of ‘Oh’type. Other examples may
include noble families, which are inbred due to custom rather than local genetic variety. Reverse
grouping or Serum grouping has to be performed to detect ‘Oh’ if incompatibility is seen during
cross matching between two ‘O’ blood group types. Conclusion:- High index of suspicion,
timely diagnosis and knowledge of Bombay blood group plays an important role in preventing
fatal blood transfusion reactions. It is essential to develop cryo-preservative facilities for rare
donors to facilitate availability of these rare blood groups in emergency situations.
HO/14(P) CASTLEMAN’S DISEASE: CASE REPORT
Divya Nagabushana, Girish G., Asha Benakappa
Department of Pediatrics, Bangalore Medical College and Research Institute, Bangalore
Divya.nagabushana@gmail.com
Introduction: Castleman’s disease is an atypical lymphoproliferative disorder, histologically
classified as the hyaline-vascular type and the plasma cell type. Clinically, there are two forms:
the localized and the multi-centric forms. Patients with plasma cell type of Castleman’s disease
present with systemic manifestations such as fever, rash, frequent infections, weight loss,
arthralgia, bleeding, generalized lymphadenopathy and hepatosplenomegaly. It is more common
in adolescents and adults, and has an aggressive clinical course. So far, about 20 cases have been
reported. Case Report: A 12 year old female child presented with a history of eczematous skin
lesions since 2 years of age. She had suffered recurrent episodes of respiratory and
gastrointestinal infections. She was hearing impaired since birth in the right ear. She was
admitted with a lower respiratory tract infection. On examination: the child was febrile with
signs of respiratory distress. She had hyperpigmented scars in the flexural areas with seborrheic
dermatitis, pallor and bilateral cervical lymphadenopathy. She had right-sided lower lobe
pneumonia and pleural effusion. Perabdominal examination revealed hepatosplenomegaly.
Investigations: CXR showed right middle and lower zone consolidation with pleural effusion.
CBC showed neutrophilic leucocytosis. Mantoux, HIV and ANA were negative. ESR was
53mm/hr. Serum immunoglobulin levels were normal. Histopathological examination of lymph
node biopsy revealed prominent lymphoid follicles with distinct germinal centers and
eosinophilic amorphous material with a diffuse sheet of mature plasma cells in the para cortex,
consistent with the plasma cell type of Castleman’s disease. She is being treated for the
pneumonia, and further management with oral steroids is planned. Conclusion: a differential
diagnosis of Castleman’s disease must be considered when a child presents with a constellation
of symptoms such as recurrent infections, skin rash, generalized lymphadenopathy and
organomegaly.
HO/15(O) PUBERTY AND BMD IN THALASSEMIC ADOLESCENTS: ARE WE
DOING THEM JUSTICE?
Gupta Anshul, Virmani Anju, Kalra Manas, Ravishankar Uma, Mahajan Amita
Apollo Center for Advanced Pediatrics (ACAP) & Dept of Nuclear Medicine, Indraprastha
Apollo Hospital, New Delhi.
manaskalra_27@yahoo.co.in
Introduction: Adolescents with thalassemia frequently have short stature, delayed/ failed puberty,
and poor bone density, inter-related issues which are unfortunately often neglected. We are
looking after 181 thalassemics in 2 settings: Group 1: Apollo Hospital (n=50; managed
optimally: regular transfusions and chelation), and Group 2: peripheral monthly free clinic
(n=131; transfused in various centres, suboptimal care). Methods: We compared puberty, peripubertal growth and bone density (Z-score for lumbar spine) between Group 1: n >14y= 16;
Group 2: n >14y= 45; data available: 27. Statistics: two tail T-test. Results: Spontaneous puberty
occurred in 56% group 1 vs. only 26% group 2; mean age at menarche was 15.4y vs. 16.5y. Final
height SDS (FHSDS) was lower in group 2: -0.71 vs. -1.91 in group 1 (p=0.007). So was
pubertal change in height SDS 0.02 vs. 0.66 (p=0.02). FHSDS was similar in girls vs. boys: -1.49
vs. -1.56 (p=0.9). Initially, we found hypogonad thalassemics even >18y had not been advised
gonadal replacement. Overall bone mineral density (BMD) Z-scores were low, with group 2
being worse: -2.20 vs. -1.69 in group 1 (p=0.3); females vs. males -1.97 vs. -1.95. BMD was
significantly higher in those with spontaneous puberty: -1.2 vs. -2.4 (p=0.05). Conclusions:
Thalassemics continue to be short, hypogonad and with poor bone density; monitoring and
therapy are neglected. Delayed gonadal replacement appears to worsen height prognosis and
BMD. Pediatricians must focus on improving BMD (exercise, adequate dietary calcium, high
dose Vitamin D supplements), and start sex steroid replacement at the appropriate age.
HO/16(P) ESSENTIAL THROMBOCYTOSIS AND ANTIPHOSPHOLIPID ANTIBODY
SYNDROME CAUSING BUDD-CHIARI SYNDROME- CASE REPORT
Dinesh Yadav, Jagdish Chandra, Sunita Sharma, Varinder Singh
Division of Pediatric Hematology, Lady Hardinge Medical College & Kalawati Saran Children’s
Hospital, New Delhi
dineshmamc@gmail.com
In most pediatric cases, thrombocytosis is secondary to some stimulus to thrombopoiesis eg.
Infections, surgery, iron deficiency anemia (reactive thrombocytosis). Primary or essential
thrombocytosis is rare in children; annual incidence reported is approximately 1 per 10 million in
literature. We hereby report a child with Budd-Chiari syndrome resulting from essential
thrombocytosis and associated antiphospholipid antibody syndrome. A thirteen year old male
child presented with progressively increasing anemia and hepatosplenomegaly. Blood
investigations revealed mild thrombocytosis and microcytic hypochromic anemia. Bone marrow
examination showed hypercellular marrow with increased megakaryocytes and occasional giant
forms. CT scan abdomen and echocardiography demonstrated calcified thrombus in inferior
vena cava. Work up for prothrombotic state revealed lupus anticoagulant antibodies. Diagnosis
of essential thrombocytosis was considered in view of persistent thrombocytosis, anemia,
associated antiphospholipid antibodies, bone marrow showing increased number, clusters and
giant forms of Megakaryocytes and thrombotic complication,. He was started on moderate dose
warfarin prophylaxis and did not have thrombotic recurrence on follow up. Presence of antiphospholipid antibodies supports the diagnosis of primary thrombocytosis in present case as
increased prevalence of anti- phospholipid antibodies has been reported in patients with essential
thrombocytosis. Thromboembolic or hemorrhagic complications are described in 30% children
with essential thrombocytosis at the time of diagnosis or later. Presence of antiphospholipid
antibodies may be further contributory to development of thrombotic complications in these
children. Early diagnosis and timely institution of antithrombotic prophylaxis prevents
thrombotic recurrences.
HO/17(P) DEFERASIROX IN THALASSEMIA: TWO YEAR EXPERIENCE
Mayank Dhamija, L. S. Arya, Manas Kalra, Amita Mahajan
Apollo Centre for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi
manaskalra_27@yahoo.co.in
Background: Deferasirox, a new once-daily oral chelator has offered an innovative approach to
iron chelation in patients with thalassemia major. There is no published experience from India
especially with regards to its efficacy and safety in patients with iron overload and existing
transaminitis. Aims: To document our experience with this drug with regards to safety and
efficacy. Methods: Deferasirox was offered to new patients starting chelation and to those who
were suboptimally chelated on desferoxamine and deferiprone. The dosages recommended (20–
40mg/kg/day) were dependent on their initial ferritin levels and ferritin trends thereafter.
Monthly SGOT, SGPT, S. creatinine and urine albumin were done for the first 3 months
followed by 3-monthly evaluation. The results of the first 50 patients (mean age 9.6 years),
observed over a period of two years, are reported Results: Rash was noted in three patients.
Nausea and diarrhoea were reported by two patients. Fourteen patients developed transaminitis
which eventually settled in all but one patient, who required temporary discontinuation of drug.
Transient elevation in serum creatinine was observed in eleven patients, of which, three patients
required discontinuation temporarily. Albuminuria was not seen. Forty eight (96%) patients
experienced a fall in their ferritin levels. Median ferritin levels at start, 3, 6, 9, 12, 15 and 24
months were 3555, 3300, 2944, 2997, 2810, 2700 and 2079 respectively. Six patients achieved a
ferritin of less than 1000. Conclusions: Deferasirox represents a novel, effective approach to the
management of iron overload. According to this early data, it appears to be efficacious and well
tolerated.
HO/18(P) CLINICO-HEMATOLOGICAL PROFILE OF SEVERE ANAEMIA AMONG
CHILDREN IN 1MONTH TO 14YRS AGE-ADMITTED TO A TERTIARY CARE
HOSPITAL
Satish, P.Nagasree, K.Umamaheswar Rao, Abani kanta Sahu, P.Sudarsini
ASRAM Medical college & Hospitals, Eluru, West Godavari, AP
Satish.ddr@rediffmail.com
Background: mild to moderate anemia constitute major degree of anemia in children <14 years
old and iron deficiency is the leading cause. Despite of anemia screening and control programme
a good no. of cases in this age group sleeps into severe anemia. This study was undertaken to
ascertain the factors underlying severe anemia ,so as to suggest various remedial measures.
Objectives: to assess the Clinico-Hematological profile of severe anemia among children of 1
month -14 years age group admitted to ASRAM HOSPITAL -a tertiary care health set up .in
ELURU Study design: a cross sectional study SettingASRAM medical college Hospital indoor-a
tertiary care health set up in ELURU,ANDHRAPRADESH Material & methods: The study
included 50 indoor children with severe anemia as defined by W.H.O as <6 gm% of Hb .Age,
sex, place, chief complaints, detailed general physical and systemic examination were recorded.
A detail hemogram including Hb,MCV,MCH,MCHC,RDW,Peripheral smear study,serum
ferritin ,Hb electrophoresis and other relevant investigations wre performed to establish the cause
of anemia.Proper treatment including blood transfusion was given on case to case basis. Data
pertening to each case was entered into designed proferma sheet and analysed.
Results:23(46%)Male,27(56%)Female.In1mo-5yrs,6-10yrs
and
11-14yrs
there
were
22(44%),15(30%)and 13(26%) children respectively distributed.Mean Hb level was 4.56g%.
Fever was the most common presenting symptoms in 64% cases.Other presenting symptoms
were jaundice 8(16%),h/o blood transfusion7(14%),breathing difficulty/easy fatigability 5(10%)
sibling with similar complaint3(6%),poor appétit/lethergy4(8%) cases. In 20cases(40%)the
underlying cause of severe anemia was Iron deficiency.Other causes were sickle cell anemia 8
case, malaria 6,beta thalassemia 5 cases,malignancy 4,anemia of chronic diseas 4,dimorphic
anemia 1 and in one case the causu could not be ascertained and referred to higer centre .
Conclusion: Iron deficiency is the most common cause of anemia in young children. Clinical
anemia mostly mild –moderate develops when iron deficiency is severe and prolonged. In this
study the highest number of case with severe anemia reflects missing the opportunity of early
diagnosis of mild cases needing Iron supplementation. This also emphasizes the missing to
identify subtle clinical fatures of anemia in both clinicians and care givers who should be made
more aware to prevent the child into severe degree and its complication.
HO/19(P) CHRONIC MYELOID LEUKEMIA IN LYMPHOID BLAST CRISIS IN A
TWO YEAR OLD CHILD: SUCCESSFULLY TREATED BY ALLOGENEIC BONE
MARROW TRANSPLANTATION
Narendra Agrawal, Nitin Gupta, Sanjeev K Sharma, Avinash K Singh, Manoranjan Mahapatra,
Tulika Seth, Pravas Mishra, Renu Saxena, H.P.Pati
Department of Hematology, All India Institute of Medical Sciences, New Delhi
narendra_ag1@rediffmail.com
Introduction: Chronic myeloid leukemia (CML) is rare in children and accounts for only 1-3% of
pediatric leukemias. With advent of imatinib therapy, stem cell transplant has taken a backseat in
management of CML in chronic phase. However, in blastic phase CML carries poor prognosis
with median survival of 6 months. Case: A 4 year old female child, found to have persistent
leucocytosis (30000-60000/µl) since 1 ½ year of age. Peripheral smear showed leucocytosis with
shift to left and no blasts. She was investigated outside and diagnosed as CML based upon
presence of Philadelphia chromosome and BCR-ABL positivity by RT PCR. Subsequently, she
was treated with imatinib (100mg/d) and achieved complete hematologic response. After 1 year
of imatinib therapy she developed progressive anemia, hepatosplenomegaly and
lymphadenopathy. At this time she was referred to our center. Investigations revealed Hb:
10.2gm/Dl, TLC: 34000/μl, platelets: 36000/ μl PS: Showed blasts. Bone marrow morphology
and flow cytometry was suggestive of B-lymphoid blast crisis. Patient was treated with
chemotherapy (Augmented BFM protocol for childhood ALL) along with imatinib 340 mg/m 2.
She went into remission after that she was taken up for allogeneic bone marrow transplantation
with myelo-ablative conditioning (Busulphan and Cylophosphamide) from HLA identical sibling
(2 ½ yrs old sister). The course of transplantation was uneventful. At present patient is 6 months
post transplant and in complete molecular remission (quantitative PCR for bcr-abl- 0) with no
evidence of graft v/s host disease or infections. Conclusion: Our patient represents a case of
CML which occurred at unusually early age. She showed initial response to imatinib but then
progressed to B-lymphoid blastic phase. She was successfully treated with chemotherapy
followed by allogeneic bone marrow transplantation.
HO/20(P) PEDIATRIC PERSISTENT /CHRONIC ITP: A SINGLE CENTRE
EXPERIENCE
Dilraj Kahlon, Veronique Dinand, Satya P. Yadav, Anand Prakash, Gaurav Kharya, Mohammed
Ramzan, Vikas Dua, Himani Manchanda, Anupam Sachdeva
Pediatric Hematology Oncology & BMT Unit, Department of Pediatrics, Sir Ganga Ram
Hospital, New Delhi
dilraj2009@gmail.com
Introduction : Idiopathic thrombocytopenic purpura (ITP) is an immunoregulatory disorder of
antibody-mediated damage of platelets. ITP in children is usually short-lived with spontaneous
recovery. Materials And Methods: We conducted a retrospective study by reviewing charts of
children with ITP from 2003 to 2010. ITP was defined as acute (from diagnosis-3months),
persistent (3-12 months) and chronic (>12months). Persistent/chronic ITP work-up included
bone marrow examination; HIV, HCV, HBsAg; ANA, dsDNA, C3/C4, APLA, Anticardiolipin,
Lupus anticolagulant; immunoglobulin profile. Results: Out of 108 ITP patients, 56 had acute, 15
persistent and 37 chronic ITP, due to referral bias. Persistent/chronic ITP patients included 37
males and 15 females. Median age was 8 years (range 1-16). Median duration of
persistent/chronic ITP was 3 years (0.6-7 years). Lowest platelet count ranged from 1,000 to
28,000/cmm. Site of bleeding was skin in 52, epistaxis-7, gastro-intestinal-6, oral-1 and retina-1.
Underlying diagnosis was SLE in 5, juvenile idiopathic arthritis in 2. One patient had low IgG.
One patient evolved into Evan’s syndrome and arthritis 5 years later. Steroids were given to all
patients, IVIG to 19, Anti-D to 10 and Rituximab to 3 patients with refractory thrombocytopenia.
Overall, platelets rose to a median of 50,000/cmm (range 11,000-2,50,000) within a median of 3
days (range 1-20). Response persisted for a median of 2 months (range 0.5-12 months).
Splenectomy was required in one patient. There was no patient with CNS bleed or death due to
bleeding. Conclusion : Persistent/chronic ITP has a variable clinical course. Adequate workup
must be done to identify the underlying cause.
HO/21(O) SPLENECTOMY IN CHILDREN: INDICATIONS AND CLINICAL PROFILE
FROM A TERTIARY CARE CENTRE IN NORTHERN INDIA
Bhavna Dhingra, Tulika Seth, Manoranjan Mahapatra, Pravas Mishra.
87-S, Sector 7, Jasola Vihar, New Delhi- 110025.
drbdhingra@gmail.com, drbdhingra@yahoo.com
Aim: To study the indications and clinical profile of patients upto 18 years of age, who
underwent splenectomy, presenting at the department of Hematology, All India Institute of
Medical Sciences (AIIMS), New Delhi. Material and Methods: Retrospective review of the
medical records of all the patients who underwent splenectomy from June 1998 to June 2009.
Data was entered in a pre-designed proforma and analysed using SPSS version 16.0. Results:
Total 60 patients underwent splenectomy. The median age at presentation was 9 years (0.5-18
years). 37 and 5 patients underwent open and laparoscopic splenectomy respectively, data for 18
patients pertaining to the method was not available. Cholecystectomy was performed in 5
patients. The presenting diagnosis were: Chronic ITP (23), hereditary spherocytosis (13),
thalasemia intermedia(12), thalasemia major(7) and autoimmune haemolytic anemia (5). 29
patients had received pneumococcal, meningococcal and influenza vaccines before splenectomy.
Complications occurred in 6 patients (septic shock 1, sepsis 1, fever requiring hospitalisation 1,
deep vein thrombosis in 3). All the patients received penicillin prophylaxis postoperatively.
Accessory spleen was observed in 2 patients. The histopathology details of the spleen were
available in 11 patients.2 patients had a complete response to splenectomy, 28 patients had a
partial response, 4 patients each had no response and relapsed, 2 patients were lost to follow-up.
Conclusions: Splenectomy is a second line treatment for various chronic haematological
disorders, especially in resource-poor settings where the other expensive options cannot be tried.
Limitations: Retrospective review with limitation of availability of all the details.
HO/22(P) CLINICAL AND DIAGNOSTIC EVALUATION OF PANCYTOPENIA IN
PEDIATRIC PATIENTS
Charu Kalra, G.C.Bothra, Neha, Bhanu Bansal , Ajit
Santokba Durlabhji Memorial Hospital cum Medical Research Centre, Jaipur.
charu_kals@yahoo.co.in
Introduction: Pancytopenia is defined by reduction of all the three cell lines of blood below the
normal reference range resulting in anemia, leukopenia and thrombocytopenia. Pancytopenia is a
common clinical problem in pediatric age group and varieties of hematopoietic and nonhematopoietic conditions manifest with features of pancytopenia. While bone marrow failure
syndromes and malignancies are important causes, non-malignant conditions such as infection
and nutritional anaemia are common causes. Aim And Objectives: To determine the incidence of
pancytopenia in relation to sex and age in Pediatric patients, to know various clinical
manifestations and causes of pancytopenia. Material And Methods: Retrospective study done
over a period of 1 year in department of Pediatrics Results: Out of 36 pancytopenia patients 24
(66.6%) were male. Most common age group was between 10-15 years (38.5%).fever was the
commonest presenting symptom 24(66.6%) followed by generalised weakness
(33.3%).Commonest presenting sign hepatomegaly 20(55.5%) followed by pallor (47.2%),
splenomegaly (38.8%). Most common cause of pancytopenia was megaloblastic anemia
14(38.8%) followed by acute lymphoblastic leukemia (16.6%), malaria (13.8%), enteric fever
(11.1%), lymphoma (8.3%), severe iron deficiency anemia (5.5%) and acute myelocytic
leukemia (5.5%). Conclusion: Common causes of pancytopenia are easily treatable such as
megaloblastic anemia and infections such as enteric fever and malaria. These conditions though
look ominous but respond rapidly to effective therapy.
HO/23(O) CLINICOHEMATOLOGICAL COMPARISON OF THE EFFECT OF EARLY
VERSUS LATE ENTERAL IRON SUPPLIMENTATION IN PRE-TERM INFANTS
Guruprasad.C.S, Santhosh Kumar A, Geetha S, Aneesh T.S, Lalitha Kailas
Dept. of Paediatrics, SAT Hospital, Govt. Medical College, Thiruvananthapuram.
drasanthoshkumar@gmail.com
Objectives: To compare the effect of early versus late enteral iron supplementation in pre-term
infants (<34 weeks) on their anthropometric (Weight, Length), hematologic (Haemoglobin) and
iron status (Serum Iron, Sreum Ferritin) on follow up at four months of age. Settings: Tertiary
care teaching hospital. Design: Descriptive study. Methods: Pre-term infants less than 34 weeks
gestational age by Modified New Ballard score, admitted in the In Born Nursery of SAT
Hospital, Thiruvananthapuram for preterm care was selected and was randomly assigned as early
(starting enteral iron at 2 weeks of age, n=25) and late (starting enteral iron at 6 weeks of age,
n=25) enteral iron group. The anthropometric, hematologic and iron status was determined at
birth. Then they where started on enteral iron in colloidal form at 3 mg/Kg/day introduced at
above described time in both group. Both groups were followed up at four months of age (1
infant from early group was lost to follow up and another expired during study period) and their
anthropometric, hematologic and iron status was assessed. The improvement in the status of
these parameters in the two group was compared statistically using SPSS version 17.0. Results:
There was a statistically significant gain in Weight, Length, Haemoglobin value, Serum Iron and
Sreum Ferritin on follow up at four months in the early enteral iron supplementation group
compared to the late supplemented group. Conclusion: It will be advisable to introduce enteral
iron in pre-terms at 2 weeks of post natal age.
HO/24(P) MANAGEMENT OF TUMOR LYSIS AND HYPERPHOSPHATEMIA IN
PAEDIATRIC HEMATOLOGICAL MALIGNANCIES
Dilraj Kahlon, Veronique Dinand, Anand Prakash, Gaurav kharya, Nita Radhakrishnan, Manas
Kalra, Satya P Yadav, Anupam Sachdeva
Paediatric Hemato-Oncology, Sir Ganga Ram Hospital, New Delhi,
dilraj2009@gmail.com
Purpose: Tumor lysis syndrome (TLS) is an important cause of morbididty and mortality in
paediatric malignancies.It may occur spontaneously or in fast proliferating tumors with large
tumor burden or high sensitivity to chemotherapy. We describe the management and outcome of
TLS
in
pediatric
malignancies
in
our
Centre.
Method: Charts of all newly diagnosed children with hematological malignancies developing
metabolic complications following induction chemotherapy were reviewed from Oct 2007 to
March 2010. TLS was defined as per Cairo-Bishop definition. All received standard treatment
with Allopurinol and hyperhydration. Outcome was assessed by decrease in laboratory and
clinical
TLS
score.
Results:Twenty children were included in the study. Two patients who developed
hyperphosphatemia without fulfilling TLS criteria were excluded. The remaining 18 patients had
T-cell ALL/NHL (n=6), pre-B ALL (n=4), Burkitt lymphoma/leukemia (n=4), AML (n=3) and
DLBCL (n=1). Laboratory TLS was present in all patients and clinical TLS in 8 after starting
chemotherapy. Median phosphatemia was 7.5 mg/dl (range 6.2-18.5). Median calcium-phosphate
product was 60.1 mg/dl (range 41-97.8). Sevelamer was used in all patients. Median uric acid
was 7.9 mg/dl (range 4.1-23.9). Rasburicase was used in 2 patients. Four patients required
hemodialysis. TLS was corrected within 24 hours in 8 patients, within 48 hours in 6, within 72
hours in 2 and in 2 after 72 hours (1 dialysed patient in each subgroup). One patient with AML
died of TLS-induced renal
failure
on day 15 of
induction therapy.
Conclusion: Most children with TLS can be managed conservatively without dialysis and
Rasburicase.
HO/25(O) ALLOGENIC HEMATOPOIETIC STEM CELL TRANSPLANTATION IN
CHILDREN WITH BLOOD DISORDERS- EXPERIENCE OF A TERTIARY CARE
HEMATOLOGY CENTER FROM INDIA.
Nitin Gupta, Suman Kumar, Manoranjan Mahapatra, Tulika Seth, Pravas Mishra, Renu Saxena,
Hara Prasad Pati, Rajan Kapoor, Narendra Agarwal, Sanjeev K Sharma.
Department of Hematology, All India Institute of Medical Sciences, New Delhi
docnitingupta@gmail.com
Introduction: Allogeneic stem cell transplantation is curative for aplastic anemia, acute leukemia
and genetic disorders like thalassaemia. We report our experience of allogeneic stem cell
transplantation in pediatric patients. Objective: To determine engraftment rate, infectious
complications, graft versus host disease, overall survival. Methods: Retrospective review from
July 2004 to September 2010 of patients less than 18 years of age. Results: A total of 30 patients
transplanted, median age 10 years (2.2-18). Indications were; aplastic anemia- 13, thalassaemia11, leukemia- 6 (2 CML, 2 ALL, 2 AML). Stem cell source; bone marrow- 14 peripheral blood15, both- 1 with median total nucleated cell count- 4.4 x 108 cells/kg (1.66-22.66 x 108) and
median CD34 cell count- 4.49 x 106 cells/kg (0.7-10.2 x 106). Infection focus was found in 33%
patients (pneumonia- 4, UTI- 1, Hickman line infection- 5) though neutropenic fever was
universal. Median time to engraftment- 11 days (8-19) and median time to platelet engraftment16.5 days (11-42). Acute graft versus host disease (aGVHD) occurred in 12 (40%) with grade ¾
aGVHD in 4 (13%) patients. Six patients developed chronic GVHD (cGVHD), one died after 8
months. Two patients had secondary graft failure. After median follow up of 19 months, 23
(76.6%) patients are alive, 7 (23.3%) died (aplastic anemia-3, thalassaemia-1, leukemia-3).
Cause of death aGVHD-2, cGVHD-1, fungal pneumonia-1, tubercular meningitis-1 and
leukemia relapse- 2 patients. Conclusion: Allogeneic matched sibling transplantation is curative
in high proportion of patients, still the graft versus host disease and disease recurrence remains
challenging problems.
HO/26(P) ROLE OF PARENTAL COUNCELLING FOR THE MANAGEMENT OF
THALASSEMIC CHILD
Manish K Arya, Payal Shah, A D Rathod, S K Valinjkar
Dept. Of Paediatrics, Grant Medical College And Sir J.J. Group Of Hospitals, Mumbai 8.
manjioo7@yahoo.co.in
Introduction: Role of parental councelling and proper understanding of parents about the disease
has utmost importance in the management of thallasemic child. Half education may become a
curse for the child. Case: A 3 year old child admitted with complaint of progressive palor since
6 months of age, history of easy fatigability since 5 to 6 months and history of not gaining
weight and height adequately. Child had cough , cold fever and increased respiratory since 3
days. There was no history of any blood transfusion. On examination child was afebrile with
tachycardia( PR- 186/MIN) and tachypnoea( RR- 46/ MIN). Severe pallor was present. Child
was wasted and stunted with feature suggestive of haemolytic facies. On CVS exanination there
was tachycardia with haemic murmur with feature suggestive of cardiomegaly. So clinical
impression was haemolytic anaemia with congestive cardiac failure. On enquiry father told us
that child was fully investigated for the disease at 1 year of age and diagnosed as a case of
thalassemia major and advised for blood transfusion and given some medication. As he was
educated so he read about the disease and complication of transfusion and decided against it as
according to him it may have harmful effect on child. Bone marrow transplant was not affordable
to him. So kept the child on aurvedic preparation. On investigation CBC showed Hb of 2.1 with
microcytic hypochromic picture and Hb electrophoresis was s/o thalassemia major. So poor
councelling and half knowledge of parent was responsible for the child condition.
HO/27(O) PREVALENCE OF ANEMIA IN URBAN MIDDLE-INCOME ADOLESCENT
SCHOOL GIRLS IN SOUTH INDIA
Sridevi A Naaraayan, Lakshmi, Sabapathy raj, V.Seetha
Assistant Professor of Paediatrics, Institute of Child Health and Hospital for Children, Halls
road, Egmore, Chennai – 600 008.
childdoctorsri@yahoo.co.in
Introduction: Adolescence is a vulnerable period in human life cycle for the development of
nutritional anemia, especially in girls due to medical and social reasons. This study was planned
to highlight the magnitude of anemia in adolescent girls belonging to the middle-income group,
which will enable planning of nutritional schemes by the Government. Aim: Aim of the study
was to determine the prevalence of anemia and it’s severity in urban adolescent school girls
belonging to the middle-income group. Materials And Methods: This cross-sectional study was
done in a private girl’s higher secondary school catering to middle-income group in Chennai
from June to August 2010. All students of age 11 to 18 years, who consented, were included.
After noting the age, 2 milliliter of venous blood was drawn from the antecubital vein and
hemoglobin estimation was done using automated analyzer. Hemoglobin level of 12 gram% and
above was considered normal. Hemoglobin level of 10 – 11.9, 7 - 9.9 and < 7 gm% were
categorized as mild, moderate and severe anemia respectively. Prevalence of anemia was
expressed in proportion with 95% confidence interval. Results: Out of 535 children, hemoglobin
estimation was performed in 306, who consented. The mean hemoglobin level was 11.6 gm%
(95% CI = 8.4 – 14.9 gm %). Prevalence of anemia was 27.5% (95% CI= 22.6% - 32.9%).
22.5%, 3.6% and 1.3% had mild, moderate and severe anemia respectively. Prevalence of
anemia was found to be maximum in the age 15 (39.8%) and minimum in the age 12 (6.7%).
Conclusion: One in four urban adolescent school girls belonging to the middle-income group in
South India is anemic, most of which is of mild category.
HO/28(P) CLINICAL, DEMOGRAPHIC & SOCIAL PROFILE OF SICKLE CELL
DISEASE AT A TERTIARY CARE HOSPITAL.
U.J.Anekar, Meenakshi Girish, V.P.Dandge, Nilofer Mujawar
Department of Pediatrics, Lata Mangeshkar, Medical College& Hospiral,Digdoh Hills ,
Hingna, Nagpur. Pin 440019.
–ujanekar@yahoo.co.in,mobile94
Aim—To study the burden of sickle cell disease at a tertiary care hospital.Study design –This is
a restrospective indoor case record study of patients from july 2007 to june 2010. Mater ial &
methods – The case records of all patients suffering from Sickle cell disease and admitted in a
tertiary care hospital between july 2007 and june 2010 were examined and data collected as per
our proforma. Results –There were 96– patients in the study. Most ( 56%) of the admitted
patients were under 5 yrs of age , ( 89.58%)admitted in vasoocclusive crisis, hemoglobin range
varied from 5.2 to 9.4gms/dl, with an average hemoglobin7. 2gms/ dl. 28 patients received
transfusion during hospital stay & 33 received transfusion in past. All pts had we ight. below 3 &
ht. between 3 & 5 percentile. 9 Fathers were Hb SS& no Hb SS mother was seen. All pts
belonged to schedule caste community in Maharashtra & to tribal community in Madhya Pradesh
& Chattisgarh.6 patients died during hospital. stay, 2 due to malaria, 2 due to septicemia & 1
each due to inf.hepatitis & sequestral crisis.All pts.given advise per standard protocol regarding
vaccination& hydroxyl urea, but only 5 patients were able to afford it.( 23.95%) Had multiple
siblings with SS, suggesting poor genetic counselling. Conclusion ---Study reveled poor
compliance due to unaffordability , inaccessibility to the only 2 regional centers Govt. Medical
college & Indira Gandhi Medical college Nagpur. In view of the heavy burden of sickle cell
disease in this region , it is suggested that at every district head quarter a sickle cell center be set
up , if the government is serious about controlling the burden of the disease.
HO/29(O) NEWBORN SCREENING IN SICKLE CELL ANEMIA IN HIGH RISK
POPULATION
N.L. Phuljhele, Sachin S. Pitlawar, B.N.Rao.
Department Of Paediatrics Administrative Block PT.J.N.M.M.C. Dr.Bramh Raipur(CG).
drsachinpitlawar@gmail.com
Introduction: The prevalence (%) of SCD(Sickle Cell Disease) in Orissa is 1-44.4% ,and in
Madhya Pradesh(Chhattisgarh) 1-40.0% .with such high prevalence there is need to identify
new born at risk at earliest . Aims & Objectives: To identify newborn with SCA(Sickle Cell
Anemia) in high prevalence communities immediately after birth. To start prophylaxis and
immunisation at earliest. Material & Methods: Study was conducted in the Department of
paediatrics DR.B.R.A.M. Hospital,Raipur(CG).during Jan2009 to Aug. 2010. Mothers belonging
to communities having high prevalence(Sahu,Kurmi,Satnami&Tribes) of SCD were identified
.From birth to first 3 days of the newborn period , heel prick blood was collected in an EDTA
for Hb electrophoresis. In positive test Newborns, mother was given a follow-up card and
advised for repeat testing after 3 months. Result: Out of 487 newborns of high risk mothers
tested 451 (92.6% )cases were FA, 35(7.2%) cases were FAS, 1 case was of FS (0.2%). For
retesting at 3 months only 397(81.71%)followed-up in which after repeating Hb electrophoresis
364 cases (91.8%) are AA, & 32 cases (8 %) turned out to be AS,1 case is SS (0.2%). This
showed false negativity in 0.75%. In infants with sickle cell trait mean gestational age ,mean
weight
,mean
Hb%
&
PCV
were
38.57weeks,2.33kg,15gm/dl,and53.47%respectively.Conclusion: Therefore in high risk
population routine neonatal screening for SCD is recommended.Hb electrophoresis is easily
available,and reliable method.
HO/30(P) T2* M.R.I. FOR IRON LOVERLOAD IN MULTIPLY TRANSFUSED
THALASSEMICS ON CHELATION
Pooja Agrawal, Rajiv Kumar Bansal, G.C. Bothra, Sunita Ojha, Neha Rastogi, Bhanu Kumar
Bansal
Santokba Durlabhji Memorial Hospital cum Medical Research Institute, Jaipur
godbless.pooja@gmail.com
Introduction: Thalassemia represents the most common single gene disorder causing a major
public health problem. Regular transfusion therapy and iron chelation has improved the quality
of life but complications of inadequate transfusions, transfusion transmitted diseases,
allosensitisations, iron overload disease and toxicities of iron chelators are there. Aims and
Objectives: To study organ iron overloading by T2* M.R.I., in multiply transfused thalassemic
patients on chelation therapy. Materials and Methods: Total 40 thalassemic patients were studied
who undewent history, examination, mean pretransfusional Hb, mean S.G.P.T. and mean serum
ferritin levels of all months for last 4 years followed by T2* MRI Scan. Design: Combined
Retrospective and Prospective Analytical Study. Results: In this study, 65% patients (N = 26)
wee detected to have iron overload (by T2* MRI) of which, 37.5 % had only hepatic iron
overload, 17.5% had only cardiac iron overload and 10% patients had iron deposition in both
vital organs. Positive predictive value of serum ferritin was 63.63 % with sensitivity of 53.84 %
and specificity of 42.85 %.. Also there were 12 patients with serum ferritin < 2000 ngm/ml but
significant iron overload as detected by T2* M.R.I. This suggests that serum ferritin might not be
ideal test for detection of iron overload in thalassemics. Conclusion: Though serum ferritin is
used to identify iron overload in transfusion dependent thalassemics, if facility of T2* M.R.I. is
available, it might emerge as a better modality to find iron deposition in vital organs.
HO/31(P) CHROMOSOMAL BREAKAGE ANALYSIS IN APLASTIC ANEMIA: A
NECESSITY IN THE DIAGNOSTIC WORKUP
Jain Dharmendra, Raina V, Malik BK, Kalra Manas, Mahajan Amita
Apollo Centre for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi
manaskalra_27@yahoo.co.in
Background: Optimal management and prognostication in aplastic anemia depends on etiology
i.e. constitutional vs. acquired. Mitomycin-C induced chromosomal breakage study distinguishes
between the two. There is scant data on the prevalence of constitutional aplasia in this cohort.
To date, this is the largest study from India analyzing chromosomal breakages in aplastic anemia
patients. Aim: To find out the frequency of constitutional aplastic anemia in patients presenting
with bone marrow aplasia. Methods: Peripheral blood samples from patients were processed for
chromosomal breakage study. Patients were examined for the presence of phenotypic features of
constitutional aplastic anemia. Hemogram and bone-marrow biopsy results were documented to
classify the severity. Results: 178 patients, 2-18 years of age (<10 years: n=55, >10 years:
n=123) were enrolled. Moderate, severe and very-severe aplastic anemia was seen in 27.6%,
69.8% and 2.6% patients respectively. Male: Female ratio was 3.3:1 (p<0.01). Chromosomal
breakages were seen in 18 patients (10.1%) with only 5 of them having phenotypic
abnormalities. There was no difference in the incidence of chromosome breaks in the children
above or below 10 years of age (9.75% and 10.9%, p=ns). Constitutional and acquired variants
did not differ in their patterns of severity of marrow aplasia. Conclusions: In this cohort, 10.11%
had evidence of constitutional aplastic anemia. The skewed sex ratio in our study probably
reflects the gender bias in our society. In view, of the therapeutic implications, it should be
mandatory for all patients with aplastic anemia to undergo this investigation prior to initiation of
therapy.
HO/32(P) GROWTH PATTERN OF SUB-CUTANEOUS FAT IN CHILDREN WITH
TRANSFUSION DEPENDENT Β-THALASSEMIA
A. K. Bhalla, R. K. Marwaha, Harvinder Kaur
Department of Pediatrics, Postgraduate Institute of Medical Education & Research, Chandigarh
anilbhalla@sify.com
The growth pattern of sub-cutaneous fat evaluated in terms of skinfold thicknesses amongst 192
boys and 60 girls between 1 to 10 years of age, diagnosed as cases of β-thalassemia was studied.
These children were enrolled from ‘Thalassemia Blood Transfusion Unit’ of the department and
represented mixed socio-economic strata. Every patient was measured for triceps, biceps, subscapular mid-axillary skinfold thicknesses at half yearly age intervals in Growth Laboratory of
the Department of Pediatrics using standardized techniques and instruments following a mixedlongitudinal growth research design. The average pre-transfusion haemoglobin level of the
patients was 9.6 g % in boys and 9.7 g% in girls. The pattern of growth for all the skinfold
thicknesses in general, exhibited an inconsistent trend. The maximum deposition of subcutaneous fat was noticed at the level of triceps and minimum at the level of mid-axillary. While,
sub-scapular and biceps skinfold thicknesses retained intermediate position. Thalassemia girls
generally, measured fatter than the boys in respect of all skinfold thicknesses throughout the age
range considered. The skinfold thicknesses measured amongst thalassemia boys and girls
measured thinner than their normal Indian and Western counterparts. As compared to normal
children, the lesser amount of sub-cutaneous fat noticed amongst thalassemia patients speaks of
the adverse influence of the disease.
HO/33(P) INVESTIGATIONAL SUPPORT TO DIAGNOSE VARIOUS SICKLE CELL
CRISIS
N.L. Phuljhele, Tarachand Patel, S. Panigrahi
tarachand_patel@yahoo.com
Aims and objective – Investigational support to diagnose various sickle cell crisis. Inclusion
criteria – Age group 6 month- 14 year patient with sickle cell disease confirmed by Hb
electrophoresis on crisis admitted in Dr. B.R.A.M.H. Raipur. Material and method – Complete
clinical record of patient of patient was maintained. Blood sample were taken to measure
hemoglobin, PS, Bone marrow, ESR, LFT, KFT, CRP, CK, LDH. CBC, LFT and KFT by
autoanalyser, SR by westerngreen method, method, CRP by Nephelometry. CK and LDH by
Kinetic method. Result and discussion: Total of 33 cases with 26 (male 17 and female 9) VOC
and 5 cases (male 3 and female 2) hemolytic crisis and 2 cases of aplastic crisis (1 male and 1
female), Sickle cell Hb SS25 cases ( male 16 and female 9) and Hb AS 8 (male 5 and female 3)
case. Mean age of detection is 4.21 yrs and age of 1st blood transfusion is 5.04yrs. In study the
mean value of ESR is 21.9 (increased), Mean Billirubin total 2.0mg/dl,direct 0.4 mg/dl, indirect
1.57 mg/dl . mean CRP 6.2 (normal value 0.08-1.12mg/dl) studied in 22 patient in 18 patient 13
male and 5 female with max. value 71IU/L. In sickle cell crisis mean LD 1692.9U/L (normal
150-580U/L) studiend in 15 patient with max. value 4242U/L.
HO/34(P) RARE CASE OF ACUTE LYMPHOBLASTIC LEUKEMIA (ALL) WITH
INITIAL PRESENTATION AS RENAL MASS WITH RENAL FAILURE.
Pushpalatha, C.N. Reddy, Reshma A. Nadaf
C/o. DR. Reshma A. Nadaf, Flat no 13, Homely Homes Apartment, Near Deva Mata School,
Horamavu, Bangalore 43
reshma_nadaf@yahoo.com
Acute Lymphoblastic leukemia (ALL) accounts for 75% of all leukemias in children, and 67% of
cases have renal infiltration which is predominantly microscopic. Only 3 to 5 % will show
clinically enlarged kidneys. Most of the cases have been reported at later stages of the disease,
only very few cases have been reported where palpable renal mass was the initial presentation.
We report a case of ALL in a 12 yr old girl, who clinically presented as pyelonephritis and later,
on investigating proved to be Acute Lymphoblastic Leukemia grade L1/L2 with bilateral
enlarged kidney and cortical infiltration.