Wilson’s disease in children. Experience from a tertiary care centre

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Sujay Chaudhuri, B.R.Thapa,

Division of Pediatric Gastroenterology PGIMER-Chandigarh
Objective of the study- To detect incidence of Wilson’s disease in children the following study was conducted. Methods used- The children (age<10 years) with Wilson’s disease who are admitted in Pediatric Gastroenterology ward of PGIMER-Chandigarh from July 1993 to June 2003 were enrolled for the study. Clinical examination, investigation (Ultrasonography , serum cerulospasmin , 24 hour urinary copper,KF ring detection , LFT,Liver biopsy , Liver Copper estimation etc.) were done. Results- 50 cases of Wilson’s disease were admitted in 10 years. Mean age was 7.5 years (range 5-10 years).Male was 30 and female was 20 in numbers. In 35 out of 50 cases(70%) hepatosplenomegaly with transaminitis was presenting symptoms.Cirrlosis was present in 15 cases(30%),fulminant Wilson’s disease in 5 cases(10%),KF ring in 35 cases ( 705),neuropsychiatric symptom in 2 cases(4%),renal tubular acidosis in 5 cases(10%) .Liver copper was increased in all cases. Conclusion- Wilson’s disease is an important cause of chronic liver disease in Northern India.Early detection is mandatory for better management of l the cases.


Trupti G Velapure, Santosh Valvi, Sanjeev Dodke, Sachin Chitalkar, Pushkar Ahire, Meena Malkani

Department of Paediatrics, Grant Medical College, Sir J.J, Group of Hospitals, Mumbai .

CASE REPORT- A two and a half year old female child born of 3rd degree consanguinity presented with recurrent episodes of loose motions which were watery, foul smelling, voluminous and associated with dehydration. The symptoms started at the age of one year. At the same time she developed skin lesions which were vesiculobullous and eczematous over the perioral, perianal areas, over the knees and elbows. These lesions would heal with some treatment leaving behind dark and dry skin. These episodes of diarrhoea and skin lesions recurred at an interval of 4-5 months with a waxing-waning course. Gradually she developed loss of scalp hair and eyelashes and eyebrows leading to Alopecia Totalis. The child had progressive loss of weight and was malnourished (grade III). She also had intermittent episodes of ear discharge which was mucopurulent. She has an elder brother of 4 years who has similar skin lesions with intermittent exacerbations. Both the children were weaned from breast milk at around 9 months of age. Her investigations revealed low serum Zinc levels (<50 mcg/dl) and low Alkaline Phosphatase levels. Skin biopsy revealed parakeratosis and other features suggestive of Acrodermatitis enteropathica. Kwashiorkor was ruled out as there was no edema and had normal albumin levels. Sweat chloride test was normal for Cystic fibrosis, Anti-Gliadin antibodies were not present for malabsorption, and her Urinary Aminoacidogram was normal. She was started on therapeutic doses of zinc (2mg/kg/day) and a dramatic response was seen – diarrhoea stopped, skin lesions dried up and appetite improved and she gained weight. Hence the diagnosis of AUTOSOMAL RECESSIVE ACRODERMATITIS ENTEROPATHICA, also known as BRANDT’S DISEASE.


Ranjan Kumar Pejaver, Nisarga R

KR Hospital , Bangalore, KIMS , Bangalore

Cow’s milk protein or lactose intolerance has been suggested to play a role in infantile colic. Probiotic bacteria have been used to treat milk and lactose intolerance. Aim:We therefore undertook a pilot study to determine whether oral pre&probiotic would ameliorate the symptoms of colic.Methods:We accepted infants for treatment if they were screaming for at least 3 hours a day for 3 or more days a week for at least 3 weeks We advised breast-feeding mothers of infants with colic to take Pre&probiotic (Pro-welTM - Alkem Laboratories Ltd) one sachet with each main meal. Half a sachet of (Pro-welTM ) was added to each feed of formula fed infants with colic, immediately prior to ingestion. Each gram of (Pro-welTM – Alkem Laboratories Ltd) contains 250 mg of L. acidophilus, B longum, B.bifidum & B. infantis and 25mg of Fructo-oligosaccharide. Results: The results of the intervention were assessed by questionnaire. Forty six of fifty ( 92%) questionnaires were returned. Twenty six of them were male and 20 were female infants. 40 of the infants were born at term and 6 were preterm infants. 40 of the 46 infants were first born child to their mothers. 9 mothers had used some sort of remedy or the other for their baby’s colic, varying from ‘colic drops’ to herbal preparations. 22 infants were breast fed exclusively, and 18 were on breast and infant formula and 6 were formula fed . Possible side effects were reported by 3 mothers; loose stools in 1 infant and vomiting in 2 infants. Thirty-two infants stopped screaming between 1 and 7 days after introduction of (Pro-welTM ) . Seven had diminished duration and intensity of screaming. Thus 83% of infants improved and in 70% the colic resolved within 7 days. Conclusion:This favourable result of the uncontrolled study demonstrates the need for a larger, placebo controlled study on the role of pre&probiotics in the therapy of infantile colic. 

Rajwanti. K. Vaswani , Dhananjay A . Sangle

Deptt of Pediatrics,Seth G.S. Medical College /KEM Hospital, Mumbai-400012
Hepatic encephalopathy is one of the leading causes of childhood mortality. Widespread use of vaccines and success of liver transplantation has made the etiology and outcome pertinent. Aim : To study the etiology, clinical features, laboratory parameters of hepatic encephalopathy in children with special emphasis on outcome. Design: A prospective hospital based study. Methods: Children between 1 month to 12 years and presenting with hepatic encephalopathy were studied over one year. Detailed history and clinical examination were recorded . Liver function tests , serum electrolytes and blood sugar were carried out in all children. Viral markers ( Hepatitis’ A, E ,B ,C) , diagnostic tests for Wilson disease and metabolic disorders were done as per requisite . Results: The records of 35 children ( 16 boys , 19 girls) were analysed using Pearson’s Chi square and unpaired T tests The main cause of hepatic encephalopathy was viral in 19 children (54.4%) . Hepatitis A (48.6%) , Hepatitis B ( 2.9% ) and Hepatitis C ( 2.9%) were observed.. Two children had Wilson disease and one had drug- induced hepatitis . Etiology was undetermined in 25.7 % cases. Nineteen children (54.3 %.) died . Amongst these , on admission 15.2 % , 37.1% and 47.7 % had Grades II , III and IV encephalopathy, respectively Advanced stage of encephalopathy was associated with poor outcome (p<0.001) . The mean Prothombin time and activated partial thromboplastin time (38.7 ±18.4 and 92.4 ±33.3 seconds) were significantly high in non-survivors (p<0.001). Conclusion: Viral hepatitis is the commonest etiology of hepatic encephalopathy . Advanced stage of hepatic encephalopathy on admission , raised prothombin time and raised activated partial thromboplastin time are associated with poor outcome. Liver transaminases, serum bilirubin and ammonia have no prognostic significance. KEY WORDS: Hepatic encephalopathy, Viral hepatitis.


KK Locham, Manpreet Sodhi, Kamaljeet Kaur, Ravneet Kaur

Deptt. Of Pediatrics, Govt. Medical College / Rajindra Hospital. Patiala.147001

Objective : To study the pattern of peristaltic movements in normal children. Setting and Methods : 50 children with different disease pattern were the subjects of the study. The children with gastrointestinal disease and system disease having effect on intestinal tract were excluded. Age, sex, place of residence, chief complaints, general physical and systemic examination were recorded on a predesigned profroma. The frequency of bowel sounds was auscultated per minute. An average of 3 readings taken consecutively was recorded. The data so obtained was analysed statistically. Results : There were 30 male and 20 female children in the study. 20 children had respiratory illness, 10 children had haemetological sickness. 6 each had CNS and renal disease. 8 Children had hepatic illness. The frequency of bowel sounds/mt was <5, 5-10, 11-15 and >15 in 14, 12, 16 and 8 children respectively. The mean bowel sounds were 7.50±5.25; 14.0±0; 10.33±2.56 in age group of 1-6 months, 6 months to 1 year and 1-3 year respectively, whereas mean sounds were 6.33±3.33, 4.71±1.25 and 2.28±0.45 in 3-6 years, 6-9 years and 9-12 years respectively. The difference in mean bowel sounds between 1-6 months and 9-12 years group was statistically significant. The difference in mean bowel sounds of 6 months - 1 year and 1-3 years group with 3-6 years, 6-9 years and 9-12 years group respectively was statistically highly significant. The difference in mean bowel sounds between 3-6 years and 6-9 years respectively with 9-12 years was statistically highly significant (p<0.05) the rest of differences between groups were insignificant. Conclusion : The lowest frequency of bowel sounds 2.28±0.45 was observed in 9-12 years group and highest frequency of 14.0±0 was observed in 6 months to 1 year.


KK Locham, Manpreet Sodhi, Kamaljeet Kaur, Ravneet Kaur

Deptt. Of Pediatrics, Govt. Medical College / Rajindra Hospital. Patiala.147001

Objective : To study the peristaltic movement pattern in normal neonates. Setting and Methods : The study included 50 healthy exclusively breastfed babies admitted to Neonatology section of Department of Pediatrics, Govt. Medical College, Patiala. Gestation, sex, birth weight, Apgar score, mode of delivery and antenatal risk factors were recorded on a pretested proforma. Bowel sounds were auscultated for one minute before and after breast feeding. Results : The study included 28 (56%) preterm and 22 (44%) term babies. The study included 7 SGA babies. Rest of babies were AGA. In preterm group, before feeding the bowel sounds were <10/min., 10-20/min., 21-30/min. and 31-40/min. in 3, 15, 2 and 8 babies respectively. After feeding in same group, the bowel sounds were in range of 10-20/min. 21-30/min. and 31-40/min. in 7, 7 and 14 babies respectively. In term group, before feeding 9, 3 and 10 babies had bowel sounds in range of 10-20/min, 21-30/min. and 31-40/min. respectively. In same group, after feeding bowel sounds were in range of 10-20/min, 21-30/min. and 31-40/min, in 1, 4 and 17 babies respectively. In preterm group, mean bowel sounds were 20.6413.27 and 30.4610.43 before and after breast feeding respectively (p<0.01). In term group, mean bowel sounds were 28.8110.66 and 37.185.04 before and after feeding respectively (p<0.01). The difference in bowel sounds between preterm and term group before feeding and after feeding was statistically significant (p<0.05) respectively. Conclusion : Mean bowel sounds in preterm babies were 20.6413.27 and 30.4610.43 before and after feeding respectively. Whereas mean bowel sounds were 28.8110.66 and 37.185.04 before and after feeding respectively in term babies.

Gaurav Jain,Nagraj Singh,Ashok Kumar Singh,S.P.Goel

Department of Paediatrics, L.L.R.M.Medical college /S.V.B.P.Hospital,Meerut.
Objectives: To study serum Zn levels in children aged 6-60 months and its role in acute diarrhea. Design: prospective hospital based study Settings and methods: 88 children of diarrhea aged 6-60 months seen in wards of department of pediatrics , L.L.R.M Medical college, Meerut were studied . Out of total children 44 were taken as cases and other 44 as controls Children included in acute diarrhea group had >=5 stools /day and stools for >3 days .Dehydration status was classified as no, some and severe dehydration according to WHO classification .Plasma samples for Zn were analysed by ZINCKIT using calorimetric method .antibiotic therapy for concomitant infections was also started ,Oral Zn preparation was given to test group ,double the R.D.A that is 10 mg for<1 year,20mg for>1 year while placebo was given to control group .Serum Zn concentration was evaluated at the time of admission and on 3rd day of stay in hospital. Result:.It was seen that cases on Zn supplementation required less Intravenous fluid therapy in terms of no. of days than control group .On serial measurements on day 1 and day 3 of admission serum Zn conc. was significantly high on day 3 in cases than control group .Duration of diarrhea and average frequency of stools was lower in cases than in controls on day 3.Average amount of stools /day was much lower along with early normalization in case group than in controls. Conclusions: Our study reveals benefits of Zn supplementation in acute diarrhea which resulted in shorter diarrhoeal episodes.

Sharma D, Nag N, Chowdhary S K, Sibal A

Indraprastha Apollo Hospitals
Background – Choledochal cyst is a congenital dilatation of extrahepatic or intrahepatic biliary radicles or both. Early diagnosis and treatment is mandatory to prevent the possible development of choledocolithiasis, biliary cirrhosis and cholangiocarcinoma. Aims and Objectives – To retrospectively study the clinical presentation, investigative methods and management of choledochal cyst. Material and Methods – This analysis was carried out at Indraprastha Apollo Hospital, New Delhi on 13 patients with choledochal cyst over a period of 3 years (Jan ’03 to Feb ’06). The average age of presentation was 4.56 years. The youngest patient was a premature baby and the oldest was 11 years of age. Male: Female ratio was 2:11. The average duration of hospital stay was 15.6 days. The most common symptom at presentation was abdominal pain (61.5%) followed by jaundice, vomiting and fever (30.7%) each. One patient had history of recurrent pancreatitis. The most common finding on examination was abdominal tenderness (46.1%) followed by jaundice (38.4%) and hepatomegaly (23%). The diagnosis was established by ultrasound in 12 (92%) patients out of which 2 were of the forme fruste variety. Ten patients were subjected to surgical intervention (complete excision of cyst and hepaticojejunostomy) after confirming the diagnosis with a per operative cholangiogram. Two patients had to be reopened for bile leak. The remaining 3 patients refused surgery. All the 13 patients were discharged in good health and there was no mortality. Conclusions – The classic triad of abdominal pain, jaundice and abdominal mass was not observed in any of the patients. Ultrasound abdomen is an effective investigative tool for diagnosing choledochal cyst and per operative cholangiogram confirms the diagnosis. Surgical management involves excision of cyst and hepaticojejunostomy.

Bhattacharya M, Dubey AP, Mathur NB, Malhotra V.

Deptt of Pediatrics and Pathology, Maulana Azad Medical College and Associated Hospitals, New Delhi-110002
Introduction: Celiac disease is a permanent intolerance to gluten present in some cereals, particularly in wheat. With the introduction and widespread use of serological markers of celiac disease it is becoming increasingly clear that celiac disease may have been underdiagnosed. An increasing number of experts are in favor of early, mass screening of celiac disease. Objectives: To determine the prevalence of celiac disease in north Indian children using anti-tissue transglutaminase (tTG) assay. Subjects: 400 children between 6 months to 12 years of age attending the pediatrics department at Lok Nayak Hospital, New Delhi. Method: All study subjects underwent history-taking, physical examination and blood sampling for a hemogram, investigations relevant for their presenting illness and anti-tTG antibody levels. An upper gastrointestinal endoscopy and duodenal biopsy were done in those subjects who tested positive for anti-human tTG antibodies after written informed consent. The biopsy specimens were evaluated as per the modified Marsh’s classification. The diagnosis of celiac disease was established as per the modified ESPAGHAN criteria. Results: Of the 400 subjects 5 tested positive for anti-tTG antibodies. All five positive subjects underwent upper gastrointestinal endoscopy and duodenal biopsy. Four of the biopsy specimens were suggestive of celiac disease and one showed features of duodenitis. Conclusions: The prevalence of celiac disease as determined by this study is 1%. This is in concordance with population screening studies using serological markers conducted in the west.

Praveen Kumar, V K Anand, Akshay Kapoor , B Rath & R Gondal

Division of Pediatric Gastroenterology, Hepatology & Nutrition, Lady Hardinge Medical College and Kalawati Saran Children’s Hospital and Department of Pathology, GB Pant Hospital, New Delhi
INTRODUCTION:-Endoscopic grasp biopsy is a widely used diagnostic tool during Upper GI Endoscopy for detecting pathogens like Giardia lambia and H. pylori. However due to its precise nature Endoscopic biopsy might miss areas where the aforementioned organisms harbour. Present study was conducted to evaluate the diagnostic role of Endoscopic brush cytology in children who were subjected to Upper GI Endoscopy for various gastrointestinal symptoms.Aim: To study the diagnostic utility of brush cytology in childrenMaterial & Methods: Records of all children in whom brush cytology was taken in addition to grasp biopsy during June 2002-July 2005 were analyzed. Endoscopic brush cytology was performed with a reusable sheathed cytology brush in the antrum, body of the stomach and second / third part of duodenum in addition to grasp biopsy. The brush was retracted under the sheath, taken out and vigorous to and fro brushing was performed on glass slides, placed in 95 % ethyl alcohol and were examined for pathogens like H. pylori and G. lambia. Endoscopic grasp forcep biopsies were subjected to routine histological examination.Results: 175 children between 1-14 years (median age-4.5 yrs) underwent UGI endoscopy for chronic diarrhea, recurrent abdominal pain, failure to thrive or recurrent vomiting. 25 cases (14.28%) were found to be colonized with H.pylori, 28 cases (16%) by G. lambia and 5 cases (2.8%) by both H.pylori and G. lambia.4 cases of H.pylori ( 16%) missed by grasp biopsy were detected by brush cytology while 11 cases ( 39%) of G.lambia missed by grasp biopsy were detected by brush cytology.Discussion& Conclusion: Diarrhea, abdominal pain, malabsorption and malnutrition are common sequale of Giardiasis. H.pylori has been associated with recurrent abdominal pain, duodenal ulcer and nonulcer dyspepsia .Grasp biopsy may miss to detect giardia and H.pylori due to uneven distribution. EBC provides a larger area of gastric & duodenal mucosa compared to EGB & thus enhances recovery of H.pylori & giardia. Our result suggests that EBC is a useful diagnostic modality for giardia and H.pylori which enhances detection of these organisms and this may be used as an adjunct to EGB.

Upasana Kapoor, Praveen Kumar, AK Patwari, V K Anand, B Rath, R Gondal

Division of Pediatric Gastroenterology, Hepatology & Nutrition, Lady Hardinge Medical College and Kalawati Saran Children’s Hospital and Department of Pathology, GB Pant Hospital, New Delhi
INTRODUCTION:-Chronic diarrhea is a heterogeneous group of disorders affecting gastrointestinal tract which constitutes 3-20% of all diarrheal disorders. Etiology is different in developing Countries if we compare with Developed Countries. There is scanty data regarding etiological spectrum of chronic diarrhea from India. Aim: To study the etiological spectrum of chronic diarrhea in children & correlation with their clinical presentation Material & Methods: Case records of all consecutive children attending Pediatric Gastroenterology Clinic with chronic diarrhea during September 2004 -August 2006 were analyzed. All patients underwent 1st line investigations –Haemogram, stool examination for ova/cyst (3 days), work-up for tuberculosis and HIV. If 1st line investigation were non conclusive then patients were subjected to Upper GI endoscopy. Duodenal biopsy from distal duodenum & brush cytology were taken from these patients. Further investigations (celiac serology, Sweat chloride, CT abdomen, Barium meal FT etc) were done as required. All these Pts were followed up in PGN clinic. Results: 109 children between 1-14 years were investigated for chronic diarrhea during the period.9 patients had diagnosis with 1st line investigations (3 HIV positive, 4 Giardiasis & 2 Tuberculosis). 100 patients who were further worked-up 50 were diagnosed Coeliac Disease(CD),18 Giardiasis, 2 IBD, 2 Koch’s, 12 tropical sprue,1 cystic fibrosis, 1 suspected immunodef with fungal hyphae ,and14 remain undiagnosed or lost follow-up before reaching a diagnosis. When we analyzed their clinical presentation in addition to diarrhea Abominal distension was present in 67 patients out of which 44 were diagnosed as CD,Pain abd in 48 ( 27 CD) , Growth failure in 74 ( 39 CD) and Pallor in 78( 45 CD). Discussion& Conclusion: Celiac disease has emerged as major cause of chronic diarrhea at our centre. Presence of other features like abdominal distension, pain abdomen, Growth failure and pallor increases possibility of Coeliac disease in these patients.

Gitanjali P.Mansukhani, Shilpa Aroskar, A.K.Singal, N.N. Kadam

Department of Pediatrics, MGM Medical College & Hospital, Navi Mumbai, Maharashtra.
Aims: Small bowel intussusception comprises only 5% of all intussusceptions in children. We present a child who presented with classical features of small bowel intussusception secondary to ileal juvenile polyps. Less than 20 cases have been reported so far. Methods: A 10 months old child presented with pain abdomen, vomiting and bloody diarrhea. Clinically, a mobile mass was palpable in the left lumbar region. A intussusception was suspected but the site of the palpable mass was unusual. Results: Ultrasound confirmed an intussusception and an attempt at USG guided hydrostatic reduction showed the colon to be free. So, the intussusception was small bowel in nature. At laparotomy, there was a bunch of 3 polypoidal masses acting as a lead point of an ileo-ileal intussusception. The rest of the intestine was free of polyps on palpation. A 10 cm segment of the bowel was resected and histopathology showed juvenile polyps. The child recovered uneventfully. Conclusions: Our report confirms the literature results – small bowel intussusceptions often have a lead point and require surgery. It is difficult to differentiate between classical ileocolic and small bowel intussusception clinically and a USG guided reduction will often fail.

Dewangan M, Gopalan S, Sibal A ,Chowdhary Sk

Apollo Centre for Advanced Pediatrics , Indraprastha Apollo Hospitals, New Delhi
Recurrent pancreatitis in childhood is uncommon .In the last one year , three children have been managed by us for recurrent pancreatitis.Two of them had minimally dilated common bile duct (5-8mm) with gall stones, which were diagnosed to be choledochal cyst forme fruste type. Less than 30 cases of choledochal cyst forme fruste have been reported in English literature. Patients & Methods A seven year old girl was transferred with severe acute pancreatitis precipated by endoscopic retrograde cholagiopancreaticography at a regional hospital.She was being investigated for recurrent right upper abdominal pain,and minimal dilatation of common bile duct(8mm) with gall stone.On admission to our intensive care unit, she progressed to develop respiratory distress syndrome with respiratory failure. After recovery , a surgical consultation was sought.A MRCP revealed similar dilatation of CBD, gall stones with hydrops and no significant dilatation of intrahepatic biliary ducts. A clinical diagnosis of choledochal cyst forme fruste was made and elective surgery was planned 6 weeks later.She was taken up for per operative cholangiogram and excision of bile duct with Roux en y hepaticojejunostomy. One year after surgery, she remains asymptomatic. A twelve year old girl was admitted with two previous attacks acute pancreatitis.On investigation, she was found to have gall stones , minimal dilatation of common bile duct (8mm) and no signs of resolving pancreatitis. She was taken up for cholangiogram and excision of bile duct and hepaticojejunostomy. Post operatively, she developed bile leak( 250ml/day) without signs of peritonitis. She underwent reexploration and repair of the posterior layer of hepaticojejunostomy. Seven months after surgery, she remains symptom free. Conclusion Minimal dilatation of CBD (5-10mm) must raise the diagnosis of choledochal cyst forme fruste in children .Excisiion of bile duct, Pancreaticobiliary disconnection and hepaticojejunostomy is curative.     

M Dewangan, A Sibal, S Gopalan, Jain Sk,Chowdhary Sk

Apollo Centre for Advanced Pediatrics , Indraprastha Apollo Hospitals, New Delhi
Introduction Gall stones disease in children is rare and is reported to have a prevelance of 0.5% .Gall stone may be seen in an asymptomatic child or may present with complications.Controversy exists regarding indications to operate, ERCP/cholangiogram, technique of surgery in children. In the last years, we have managed 10 children who have been managed on a fixed clinical protocol and follow up data is presented. Patients and methods Ten consecutive children varying in age from 2yrs-13 yrs (mean 5.5yrs) have been managed .All children with gall stone were investigated for hemolytic disease or any other known predisposing factor.Symptomatic gall stones underwent detailed US evaluation localising any dilatation of bile duct or hepatic duct.ERCP/ cholangiogram was reserved only for those with dilated CBD.Asymp-tomatic single gall stones were observed.Symptomatic children and those with multiple stones were taken to surgery. Results Six children presented with abdominal colics and following attacks of acute cholecystitis.None of these had dilatation of CBD.No ERCP or cholangiogram was done.All underwent laparoscopic cholecystectomy.They remain asymptomatic for varying periods of follow up( 3mo- 8yrs). Three children were found to have mildly dilated CBD,one of them packed with stones.One had failed attempt at ERCP .All of them were diagnosed to be variants of choledochal cyst. Each had cholangiogram,excision of extrahepatic biliary duct and hepatoicojejunostomy. They remain asymptomatic with follow up periods of 6mo-7yrs. One child had asymptomatic single gall stone,left to follow up without surgery. Two years later the stone has remained without symptoms and now the parents have requested for surgery.None in this study had any suggestion of hematological disorder. Conclusion In the investigative protocol for gall stone in children, hemolytic disorder should be ruled out with basic tests as the overall incidence does not seem to be high. Unless the bile duct is dilated there is no role for preoperative ERCP/MRCP or per operative cholangiogram. Laparoscopic cholecystectomy is safe and can be offered to every child with gall stone disease.Gall stone disease associated with choledocholithiasis or even mildly dilated CBD must raise the question of choledochal cyst pathology

V Taneja, A Sibal, Sachdev MS, Joshi Rk, V Kohli, R Joshi , Chowdhary Sk

KApollo Centre for Advanced Pediatrics , Indraprastha Apollo Hospitals, New Delhi
Aim: Retrospective review of all pediatric cardiac surgery patients over a twelve month period (2005-2006) and evaluation of GI symptoms following neonatal cardiac surgery. Patients & Methods Case records and radiological films of 86 consecutive pediatric patients who underwent cardiac surgery during the period under review were examined.The neonates who underwent cardiac surgery and developed gastrointestinal symptom to seek a GI consultation were separately evaluated for diagnosis, investigations and management. Results Out of 95 cardiac surgeries performed over the twelve month period under review, 17 were neonates. 6 of these 17 underwent open heart surgery and 11 were closed heart surgery. All neonates who underwent surgery survived. In 2 babies, out of the six , who underwent open heart surgery and 1 out of 11 babies with closed heart surgery, postoperative gastrointestinal symptoms were serious enough to seek GI consultation and intervention.The first postoperative baby continued to have large gastric aspirates, and features of gastric outlet obstruction. An US & contrast study were done to rule out a known pathology. A provisional diagnosis of gastroparesis was considered, and nasojejunal tube was inserted for feeding. Gastric paresis persisted for 27 days after surgery. The second post operative neonate developed cervical achalasia and required several days of pharyngeal suction and remains on gastrostomy feed. The third baby developed severe upper gastrointestinal bleeding. Neonates(term + 4weeks) were divided into two groups . Group A with GI symptoms and Group B without GI symptoms. Group A had a mean age of 31 days and mean weight of 2.5Kgs with mean hospital stay was significantly( p <.05) longer in group A (37+/- 4 days) versus Group B (20+/- 5days) . All the babies who developed serious GI problems had undergone open heart surgery for transposition of great vessels and coarctation of aorta with patents ductus arteriosus. All 18 neonates who underwent cardiac surgery were discharged. Conclusions Open heart surgery in newborns is associated with significant gastrointestinal symptoms in the post operative period. Majority of them are transient and may need temporary intervention.      


Dewangan M, Kasana S , Mahajan A, S Kaul, Sibal A, Chowdhary Sk     

Apollo Centre for Advanced Pediatrics , Indraprastha Apollo Hospitals, New Delhi

Introduction Laparoscopic resection and anastomosis of small bowel bearing pathology in children is a new technical advancement in laparoscopic surgery .In the last year, we managed two children with this technique.The purpose of this poster is to present these two cases and discuss the feasibility of the this technique for small bowel pathology hitherto not reported in English literature. Patients and methods A 7 y boy was managed as abdominal tuberculosis on the basis of repeated abdominal colic , dilated small bowel and associated lymphadenopathy. Despite two months of drug therapy,symptoms persisted..A plan for laparoscopic exploration was offered.This revealed a segment of small bowel was thickened ,inflamed ,oedematous with proximal dilatation.This segment of bowel was exteriorized through the umbilical port and palpation confirmed it to bea mass in the small bowel suggestive of tumour.This segment was resected with 5 cm margin on either side and end toendanastomosis was done .The bowel was dropped back into the abdomen and he made uneventful recovery.Histopathology confirmed Non Hodgkins lymphoma, chemotherapy schedule has been completed and he remains asymptomatic six months after surgery . A 3 y boy was transferred from a neighbouring country with massive lower GI bleed .He had been stabilized with 3 units of blood transfusion and hemoglobin on transfer was 5g/dl. Clinical examination was unremarkable. An US was done to exclude duplication cyst, Te scan was negative for meckels diverticulum, colonoscopy did not reveal any pathology.He was taken up for a laparoscopic exploration and a meckels diverticulum was found at 25 cm from ileocaecal junction with broad base and inflammation.This segment of the bowel was delivered through the umbilical port site.The adjacent bowel had thickening suggestive of heterotropic mucosa and hence extracorporeal resection and anastomosis was done . Small bowel was dropped back in the abdominal cavity.Recovery was uneventful and he remains asymptomatic one year after follow up. Conclusion In a symptomatic child where small bowel pathology is suspected , laparoscopic assessment may allow an early correct diagnosis with a chance for cure.


Deepti Chaturvedi, Rajesh Khadgawat, Govind Makharia, A.C.Ammini

Department of Endocrinology and Metabolism and Gastroenterology & Human Nutrition, All India Institute of Medical Sciences, Delhi

BACKGROUND & AIMS: Osteopaenia(OP) and osteoporosis(OPO) are very common in Indian population in comparison to western countries.  Celiac disease(CD) is considered to be an important risk factor for OP and OPO. No data is available on bone density of CD children in Indian population. This study was planned to evaluate BM in CD patient before & one year after treatment. This study is continuing and we are presenting preliminary data. DESIGN: Prospective study. METHODS: We measured BMD in all CD cases before starting treatment. Diagnosis of CD was suspected on the basis of clinical features, screened with estimation of serum tissue Transglutaminase (tTG). CD was confirmed by duodenal biopsy in patients with elevated tTG. Serum IgA estimation was done were clinical suspected CD but tTG negative to rule out IgA deficiency. BMD was measured by dual X-ray absorptiometry (DXA, Hologic QDR 4500) at three different sites i.e. spine, hip and forearm. These scores were compared with age and sex matched available standards. RESULTS: The mean age of children was 15.7 years (Range 13-18yrs). 5 were male while 10 were female. BMD was found to be markedly lower in untreated CD patients. The average BMD at spine was 0.427 gm/sq cms, which was significantly low in comparison to age-matched controls (Z-score spine -5.6). BMD at hip& forearm was 0.313g/sqcms  & 0.244 gm/sq cms respectively. CONCLUSIONS: Untreated CD in Indian children is associated with significantly low BMD. We are following these children for effect of treatment on BM.


Rajiv Kumar, Nomeeta Gupta, Shalini

Department of Pediatrics, Batra Hospital & Medical Research Centre, New Delhi
INTRODUCTION: Preterm infants during their hospitalization in tertiary care neonatal intensive care units (NICU) develop an abnormal pattern of microbial colonization, which may contribute to diseases such as septicemia and necrotizing enterocolitis (NEC). Enteric feeding of probiotics may provide benefit to such infants. Lactobacillus acidophilus, Lactobacillus rhamnosus, Bifidobacterium infantis, Bifidobacterium longum and Saccharomyces boulardii have been used as probiotics to reduce the incidence of NEC, but the efficacy of probiotics remain controversial. OBJECTIVE: To evaluate the beneficial effects of probiotics in reducing the incidence and severity of NEC in preterm infants. MATERIAL AND METHODS: A prospective, randomized, double-blind, placebo-controlled clinical study was conducted on 92 preterm infants admitted in NICU of our hospital from September 2004 to August 2006. Newborn infants with gestational age <33 weeks or birthweight <1500 gm were randomized into the study or placebo groups after written informed parental consents. The study group was fed with DAROLAC (L. acidophilus, L. rhamnosus, B. longum and S. boulardii) half sachet twice daily with expressed breast milk (EBM) until discharge, starting with the first feed. The placebo group was fed with EBM without probiotics. NEC was diagnosed using Bell’s criteria. To exclude mild and doubtful cases, only cases of NEC Bell’s stage 2 or higher were considered. RESULTS: 92 infants were eligible for enrollment in the study. The probiotics group (n=45) and the placebo group (n=47) were similar in the demographic and clinical variables. The incidence of death or NEC was significantly lower in the study group (5%) than in the placebo group (12.8%). The incidence of NEC (stage 2) was also significantly lower in the probiotics group (n=2) than in the placebo group (n=10). There were 2 cases of severe NEC (Bell stage 3) in the placebo group and none in the study group. None of the positive blood culture grew Lactobacillus or Bifidobacterium species. CONCLUSION: An early supplementation of oral probiotics with breast milk in preterm infants reduces the incidence and severity of NEC. L. acidophilus, L. rhamnosus, B. longum and S. boulardii as probiotics is protective of NEC in preterm infants. Further studies are required to confirm our results.


Choudhary S, Sibal A, Sachdev MS, Joshi RK, Kohli V, Joshi R

Apollo Centre for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi.

Gastrointestinal problems (GIP) requiring hospitalization occur in x/100 term newborns while they are significantly higher after neonatal heart surgery (NHS). These problems may arise from congenital malformations or may be co morbidity of cardiopulmonary bypass. We report three such newborns requiring surgical intervention following NHS. 20 NHS were performed over 12 months in the pediatric cardiology and cardiac surgery unit. 6/20 were open heart surgeries and 14/20 were closed heart surgeries. These patients were divided into Group A (with GIP) and Group B (without GIP). Mean hospital stay was significantly longer in group A (xyz+/-ab days) than in Group B (abc+/- xy days), p < 0.05. The cardiac diagnoses in Group A were transposition of great arteries (2/3) and coarctation of aorta with PDA (1/3). The GIP noted in Group A (3/20) included prolonged gastroparesis, acute GI hemorrhage and feeding inability requiring gastrostomy. 1/3 in Group A required surgery (gastrostomy) while 1/14 in Group B required non-cardiac surgery (tracheostomy). Comorbidities in Group A included renal tubular acidosis, sepsis related hepatitis and dysmorphism.. Gastroparesis and GI hemorrhage were medically managed while feeding inability required a gastrostomy. The medically managed patients improved and the one with gastrostomy continues to show good weight gain with gastrostomy feeding. In conclusion a small percentage of newborns undergoing cardiac surgery may have significant GIP requiring prolonged medical management and occasionally surgery.


Rajiv Kumar, Nomeeta Gupta, Arvind Sabharwal

Department of Pediatrics & Pediatric Surgery, Batra Hospital & Medical Research Centre, New Delhi-110062.

INTRODUCTION: Abdominal pain in childhood is a common cause of pediatric emergency, evaluation of which is at times difficult, its assessment and diagnosis continues to be a clinical challenge. OBJECTIVE: To study the clinical profile of recurrent abdominal pain (RAP) in children and determine the value of investigative procedures in finding out the etiology of RAP. MATERIALS & METHODS: 166 children with recurrent abdominal pain satisfying Apley's criteria in the age group 5-12 years attending Pediatric OPD of our hospital during June 2004 and July 2006 were prospectively evaluated. A detailed history, complete physical examination and detailed investigations of all children were done. RESULTS: The mean age of the patients was 6 years. Male to female ratio was 1:2. Stool microscopy was positive for ascariasis in 6, giardiasis in 4 and amoebiasis in 2 children. Urine culture was positive in 8 children. USG abdomen showed renal calculus in 2 children. Barium study showed malabsorption pattern in 44, malrotation in 2, ascaris worm in 4 and features suggestive of tuberculosis in 2. Upper GI endoscopy showed esophagitis in 14, esophagogastritis in 2, gastritis in 2, gastritis and duodenitis in 4, duodenitis in 8 and deformed duodenal bulb and gastric mucosal prolapse in 2 each. Mantoux, Chest and abdominal X-ray, urine routine were not contributory, 10 children were diagnosed as pancreatitis with high serum amylase. 6 children fitted with the diagnosis of somatisation disorder. 92 children were found to have dysfunctional pain. CONCLUSION: RAP is one of the commonest childhood problems, which cannot be ignored in view of high incidence of organic cause. The efficacy of certain investigations like barium meal and UGI endoscopy was high (31.2 & 22.9 respectively). Gastro-esophageal reflux (GER) was found to be a major cause, which is often missed unless looked for. Psychogenic pain constitutes a very small percentage.

Smriti Nath, Rajendra Pancholi, Somnath Sinha Roy

Dept. of Pediatrics, Tata Motors Hospital, Jamshedpur, Jharkhand
Objectives-To evaluate the efficacy of Racecadotril along with Oral Rehydration Solution(ORS) in comparison to ORS alone in acute watery diarrheoa in children . Design- Randomized double blind placebo controlled prospective study. Setting- Pediatric ward. Subjects- Total 80 children <5 years admitted with watery diarrhea were studied. Methods- Children were randomly divided into study group and control group. They were treated with Racecadotril (1.5 mg/kg every 8 hourly) or placebo respectively along with ORS. Cases were evaluated on the basis of Frequency of stool, Stool output, Total intake of ORS and Duration of diarrhoeal illness. Results- The mean duration of diarrhoeal episode was 2.93 +/- 1.06 days in Racecadotril (study) group and 3.38 +/- 1.32 days in placebo (control) group (p>0.05). Total intake of ORS in study group was 134.1 +/- 97.4 ml/kg and 167.9 +/- 113.5 ml/kg in control group (p>0.05). Stool output was found to be 223.3 +/- 81.5 gm/kg in study group and 252.5 +/-58.7 gm/kg in control group (p>0.05). Frequency of stool in first 24 hours was decreased in 42.5% of study group compared to 27.5% in control group. But it was increased in first 24 hours in 37.5% of control group in comparison to only 17.5% of study group. Only 1 child among the 40 receiving Racecadotril showed abdominal distention and constipation. Conclusion- In our study addition of Racecadotril with ORS did not show any statistically significant improvement in comparison to ORS alone. Therefore, Racecadotril therapy though found to be safe but it does not provide any additional benefit in acute watery diarrhea in children.

Jacob M Puliyel, Nalini Abraham, Ashish Batham, Dherian Narula, Tanmay Toteja, V Sreenivas, 

Department of Pediatrics and Neonatalogy, Otorhynolaryngology, St. Stephens Hospital, Tis Hazari, Delhi-54
Objective To evaluate the point prevalence of Hepatitis B in India Design Meta-analysis of data on point prevalence from different parts of the country. Data sources Searches were made in Medline, Cochrane Library and Best bets and previous reviews. A limited hand search of cross references was also done. Finally a consultation with experts was held to enlarge the references base.  Review methods Studies reporting prevalence of HBsAg were selected. Data from high risk groups were excluded.  Main Results: 53 papers reporting data on 63 populations were identified. The true prevalence for each studywas calculated from the reported prevalence using the specificity and sensitivity of the test employed. The true prevalence in non-tribal populations is 2.1% (95% CI: 1.8% - 2.4%). True prevalence among tribal populations is 14.9 % (CI: 10.8% -19.5%). Conclusion: This is arguably the first meta analysis of the data on point prevalence of Hepatitis B in India These figures may be useful in estimation of the burden of the disease in the country and for projecting the cost-benefits of immunization.

N. Mohan, A Sehgal, A. S. Soin, R. Kakodkar, S.Nundy

Department of Pediatrics, & Liver Transplantation, Sir Ganga Ram Hospital, New Delhi
Living donor liver transplantation (LDLT) is especially suited to children since pediatric cadaveric whole grafts are scarce, partial adult livers are sufficient for their metabolic needs and one of the parents is usually a suitable donor. 84 LDLT procedures were performed between Jan 02 and Sept. 06. Of these, 8 were performed in pediatric recipients. Mean age 6.7 yrs; 4M:4F. All expect one child received segments 2,3,4 with middle hepatic vein. Indications for LDLT were Wilson’s disease (3), PFIC type III (1), tyrosinaemia (1) and biliary atresia (2) and giant cavernous hemangioma (1). The donors were mother (4), father (1), grandmother (1) uncle (1) and aunt (1).

There was no donor or recipient mortality. Intraop. blood transfusion was not required in any of the donors and four of the recipients. Among recipients, venous outflow was reconstructed by anastomosing the graft outflow vein(s) to the common stump of left and middle hepatic veins extended on to the recipient vena cava. The median hepatic arterial diameter was 1.5mm (1-2.2mm) and all arterial anastomoses were performed without microscope under 3x loupe magnification one patient had 2 hepatic artery reconstructed . One patient with extensive porto-mesenteric vein thrombosis underwent cavoportal transposition while in the rest, direct anastomosis between graft and recipient portal veins was performed. For biliary reconstruction, duct-duct anastomosis without stent was performed in 4 patients and Roux en Y left hepatico-jejunostomy with stent in 4. One patient had an episode of luminal bleed which was managed by embolisation of vessel (br of SMA) There were no vascular complications. One patient underwent re-exploration and conversion to Roux en Y reconstruction on second post-op day for biliary leak. One child developed chylous ascites that subsided in 3 wks with a conservative regime of nil per os, parenteral nutrition and octreotide followed by oral MCT diet. All patients and donors are well post-operatively with median follow up of 10.8 months (1- 25 months). With meticulous technique, living donor liver transplantation can be performed in children with a high success rate and low incidence of complications, often without the need for blood transfusion. Though the incidence of biliary atresia in India is as common as in West, our indication of liver transplantation was predominantly for metabolic liver disease.


Neelam Mohan, A Sehgal, R Sud

Division of Pediatric Gastroenterology & Hepatology, Sir Ganga Ram Hospital, New Delhi – 110060
Objective: Role of ERCP as a diagnostic and therapeutic modality in pancreatitis is gaining wider application. Our aim was to determine the role of ERCP in the management of pancreatitis in children (age < 16 yrs) presenting to a tertiary health care centre in North India. Material & Methods: 65 children (<16 yrs) presenting to the pediatric medical services of the hospital with pancreatitis were recruited in the study from Oct. 1998 to Nov. 2003. Out of 65 patients, 24 were diagnosed as chronic pancreatitis, 27 as acute pancreatitis and 14 as recurrent pancreatitis. ERCP was done in 26(40%) patients, 17 cases were of chronic pancreatitis (70.8%), 5 (18.5%) & 4 (28.5%) cases of acute & recurrent pancreatitis respectively. The mean age of ERCP patients was 11 yrs (range 4yrs – 15 yrs) with male: female ratio of 14:12. In all cases indications, ERCP findings, complications, patients course and therapeutic intervention (if any) were recorded. The procedure was carried under midazolam and ketamine sedation. Results: Successful cannulation was possible in all cases. Of the 17 patients with chronic pancreatitis ERCP revealed abnormal pancreatic duct in all in the form of duct irregularity/ blunting of secondary branches/ dilatation/ tortousity with stricture in 6 cases (25%). Pancreatic stents were placed in 12 (50%), 6 in stricture cases & 6 in cases with multiple pancreatic duct calculi till the stone clearance was done. Associated CBD stones were seen in 2 cases, which were removed. There was one case each of pancreatic divisum and communicating pseudocyst (stent placed). In the 5 cases with acute pancreatitis ERCP revealed bulge of pseudocyst in stomach in 4 cases, two of which had associated pancreatic duct cut off in body region and CBD stone in one which was removed. Successful endoscopic cystogastrostomy was done in all 4 cases of pseudocyst with double pigtail stent. This procedure was preceded by a CT scan or Endo ultrasound to assess the suitability of the case. Of the 4 patients with recurrent pancreatitis ERCP revealed normal pancreatic duct in 3 and one had anomalous pancreatico biliary junction (APBJ) type III C3 showing dilated common channel with multiple stones, dilated CBD & dilated but not tortuous pancreatic duct. After papillotomy stones were removed & stenting done. Thus overall ERCP was used as a therapeutic modality in 17 cases (65.3%). Conclusion: ERCP is a useful modality in the management of both chronic and acute pancreatitis in children. It served as a therapeutic modality in 65% of our patients. Like in adults, endoscopic cystogastrostomy can be safely and effectively performed in children.

Manoj Gupta, A. Batham, Dhirian Narula, V Sreenivas, J Puliyel

St Stephens Hospital, Tis Hazari, Delhi 110054
Systematic reviews sometimes show different trials coming to diametrically opposite conclusions. One reason is that the samples for the trials are dawn from different populations and each trial reflects the truth in that population. This matter is often overlooked when meta-analysis is done. We have previously suggested that when results of different studies are aggregated in a meta-analysis, the studies must be given weights according to the size of the population they represent rather than the sample size. It was suggested that large study samples from small populations would get undue weightage otherwise (Puliyel JM, Sreenivas V. Meta-analysis can be statistically misleading. EBM 2005.10;130.)). A systematic search and conventional meta-analysis of studies on point-prevalence of Hepatitis B in India has been presented at this meeting. In this communication we revisit that data and perform a new meta-analysis using population weights, to see how this approach alters the inferences drawn Meterial Methods The systematic search was performed looking for published papers on the point prevalence of Hepatitis B in India. Studies from high risk populations were excluded. Briefly, 53 papers reporting data on 63 populations from15 States qualified for inclusion in the meta-analysis. Results Point prevalence among non tibal populations was 2.1% (CI 1.8 – 2.5) and among tribals it was 19.4% (CI 15.3-23.5). Using these weights it is estimated that the true prevalence among non tribal populations is 2.7% (CI 2.1 -3.4). Prevalence among tribal populations was 10.8% (CI 9.8 – 11.9). Overall the prevalence was 3.2% (CI 2.6 -3.8) Discussion Although the use of population weights allows such synthesis of disparate data sets, it has the disadvantage that the tribal group’s distinctly higher prevalence, gets submerged in the data of the majority and thus the group does not get the attention they deserve. The data in the two groups is therefore best analyzed separately as done in the traditional meta-analysis.

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