Ujjal Poddar

Etiological spectrum of esophageal varices due to portal hypertension in Indian children: is it different from the West?

Background and Aim:  Information about portal hypertension (PHT) in children is meagre. We therefore studied the spectrum and outcome of PHT in children (≤14 years of age) over a period of 9 years.

Endoscopic retrograde cholangiopancreatography in the management of pancreaticobiliary disorders in children

Background and Aim: The role of endoscopic retrograde cholangiopancreatography (ERCP) is not yet fully established in children. The purpose of this study was to assess the use of ERCP in the diagnosis and management of various pancreaticobiliary disorders in children.

Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries

Background and Aim:  A major challenge in neonatal cholestasis (NC) is to differentiate biliary atresia (BA) from other non‐atretic causes. In developing countries there are considerable problems of late referral of NC cases and performing surgery without prelaparotomy liver biopsy that contributes to a high proportion of negative laparotomy and increased morbidity. We evaluated the hepatic histopathology for presence of features that correlate best with the diagnosis of BA and assessed the accuracy of percutaneous liver biopsy.

Natural history and risk factors in fulminant hepatic failure

Background: The natural history of fulminant hepatic failure (FHF) without liver transplantation is not well known. Aims: To study the natural history and prognostic factors, especially the presence of ascites and spontaneous bacterial peritonitis (SBP), in children with FHF. Methods: FHF was defined by the onset of encephalopathy within 12 weeks of onset of jaundice. From August 1997 to December 2000, 67 children (≤12 years) were diagnosed with FHF. Their clinical features, investigations and outcome were noted. Viral markers A to E (IgM, anti-HAV; IgM, anti-HEV, HBsAg, and anti-HCV) were determined by ELISA. SBP was defined by the presence of ≥250 neutrophils with or without a positive culture in ascitic fluid. Results: Mean age of the children was 5.8 years with an almost equal sex distribution. Viral markers were positive in 63 (94%) cases: hepatitis A in 34 (54%), E in 17 (27%), A+E in seven (11%), and B in five (8%). Thirty one children presented with grade I or II encephalopathy and all recovered, whereas 17 of 36 children who had grade III or IV encephalopathy died. Ascites was detected (both clinically and ultrasonically) in 34 (51%) cases, nine (26%) of which had SBP. Overall mortality was 25%. Mortality was higher in those who had ascites than in those who did not (32% v 18%); among those with ascites it was maximum in those who had SBP (78% v 16%). Total serum bilirubin and grade of encephalopathy were significantly higher, serum albumin was significantly lower, and prothrombin time was significantly prolonged in those who died than in those who recovered. Conclusion: The natural history of FHF in Indian children depends on age, grade of encephalopathy, ascites, and SBP. SBP depicts worse outcome. In all cases of FHF with ascites, the presence of SBP should be investigated.

Celiac disease in India: Are they true cases of celiac disease?

Background In a developing country, many conditions other then celiac disease (CD) can give rise to villous atrophy. We therefore assessed the role of immunoglobulin A (IgA)–antigliadin antibody (AGA) in addition to the ESPGHAN criteria in the diagnosis of CD in 104 Indian children. Methods Consecutive children with suspected CD were evaluated over 3 years with an intention to diagnose CD. Complete hemogram, d-xylose absorption test, endoscopic duodenal biopsy, and IgA–AGA titers were performed in all. CD was diagnosed on the basis of modified ESPGHAN criteria irrespective of IgA–AGA positivity (>5 U/mL), and those diagnosed were put on gluten-free diet and were monitored regularly. Children with suspected CD who had a normal duodenal biopsy result were taken as controls. Results The mean age of 50 children with CD was 6.3 ± 2.6 years, with a male to female ratio of 3:2. The mean duration of symptoms was 3.4 ± 2.2 years. Predominant symptoms were pallor in 96%, failure to thrive in 92%, and diarrhea in 80%. On follow-up (19.6 ± 8 months), symptoms subsided within 16 ± 9.8 days, and patients showed significant weight gain (mean weight at diagnoses and at last follow-up visit were 66% and 86% of expected, respectively;P < 0.001) and height gain (mean height at diagnoses and at last follow-up visit were 88% and 94% of expected, respectively;P = nonsignificant). The control group comprised 47 children with a mean age of 6.9 ± 3 years. On comparing CD with controls, diarrhea, anemia, low weight, and stunting were significantly (P < 0.001) more frequent in patients with CD. Sensitivity and specificity of AGA at a cutoff value of 5 U/mL were 94% and 91.5% and at 10 U/mL 88% and 100%, respectively. Follow-up AGA test was performed in 42 of 47 positive cases. All showed significant decrease in AGA titer, and 29 (70%) had a negative test result. Conclusions Indian children with CD are true cases of CD. They present late, diarrhea is absent in 20% of cases, and AGA test results show 88% of children without false-positive results at a cutoff value of 10 U/mL. However, AGA test with 94% sensitivity at a cutoff value of 5 U/mL can be used as screening test to select suspected cases for further workup.

Changing spectrum of sporadic acute viral hepatitis in Indian children.

From August 1997 to January 2000, 172 children (< or = 14 years) with acute viral hepatitis were studied. Their clinical features, investigations and outcome were noted. Viral markers (IgM anti-HAV, IgM anti-HEV, HBsAg and anti-HCV) were measured by ELISA using commercial kits. The mean age of these children was 5.6 +/- 2.9 (range, 4 months to 14 years) with a male to female ratio of 120:52. Prodromal symptoms were present in 161 (94 per cent) and icteric hepatitis was diagnosed in 168 (98 per cent) cases. Splenomegaly was noted in 53 (31 per cent), ascites in 52 (30 per cent) and encephalopathy (ALF) in 56 (32.6 per cent) cases. Sixteen (31 per cent) children with ascites had spontaneous bacterial peritonitis (SBP). Fifteen (27 per cent) children with encephalopathy died. Viral markers were positive in 166 (96.5 per cent) and they were: A in 111 (64.5 per cent), E in 28 (16.3 per cent), B in 13 (7.6 per cent), A + E in 12 (7 per cent), A + E + C and A + C in one each. Mortality in acute liver failure was more when associated with SBP (100 per cent) than without (20 per cent) (p < 0.001). We conclude that HEV is the second most common cause of sporadic acute viral hepatitis in children. Atypical presentations, such as splenomegaly, ascites, and SBP were present in virtually one-third of cases. In cases of ALF, the presence of ascites and SBP depicts a worse outcome.

Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country.

Aim:  To study the accuracy of various clinical and investigational parameters to differentiate biliary atresia (BA) from neonatal hepatitis (NH).

Clinical features of celiac disease in Indian children: are they different from the West?

Objective: This study was designed to prospectively evaluate the clinical features of celiac disease (CD) in a large group of Indian children and to compare them with those from the West. Patients and Methods: Over a period of 5 years, a total of 549 children (≤14 years) with a clinical suspicion of CD were evaluated. Their detailed clinical features, investigations, and follow-up data were recorded. Complete hemogram, endoscopic duodenal biopsy, and celiac serology were done in all of the cases. Celiac disease was diagnosed on the basis of modified European Society of Paediatric Gastroenterology, Hepatology and Nutrition criteria. Results: Celiac disease was diagnosed in 300 children; 39 were excluded because of lack of follow-up or poor response to gluten-free diet. The remaining 210 had normal villous architecture and served as controls. The mean (± standard deviation) age of children with CD was 6.7 ± 3 years, and the mean duration of symptoms was 3.5 ± 2.5 years. The majority (84%) presented with diarrhea; other features were failure to thrive in 91%, anemia in 84%, wasting in 87%, and stunting in 60% of cases. Among the serological tests, the best results were obtained with tissue transglutaminase. On follow-up (19.4 ± 15.5 months), symptoms subsided in all cases of CD with a significant weight and height gain. Conclusions: Indian children with CD present late, with a significant delay in diagnosis. The majority presents with classic symptoms of diarrhea, failure to thrive, and anemia. There is a need for increasing awareness to pick up the atypical forms of the disease.

Endoscopic transpapillary nasopancreatic drainage alone to treat pancreatic ascites and pleural effusion

Background:  Pancreatic ascites and pleural effusion are uncommon sequelae of pancreatitis and are associated with significant morbidity and mortality. Endoscopic decompression of the pancreatic duct through transpapillary stent or nasopancreatic drain (NPD) has shown encouraging results but the experience is limited. The aim of the present study was to evaluate the efficacy of endoscopic transpapillary nasopancreatic drainage in patients with pancreatic ascites and pleural effusion.

Acute on chronic liver disease in children from the developing world: recognition and prognosis.

Objectives: A subset of children with chronic liver disease (CLD) decompensate following an acute insult; however, data for children are not readily available. The present study aims to characterize the clinical presentation, etiology, outcome, and determinants of short-term mortality in children with an acute hepatic insult superimposed over CLD. Patients and Methods: Children of acute on chronic liver disease (ACLD) were grouped as acute on chronic liver failure (ACLF) and non-ACLF. ACLF was defined as per the definition proposed by Asian Pacific Association for the Study of Liver. The acute insult, etiology of CLD, and clinical and laboratory parameters at admission along with 3-month outcome were assessed. Receiver operating curve (ROC) was plotted to measure the performance of pediatric end-stage liver disease (PELD) score in predicting the 3-month mortality. Results: Of the 36 children with ACLD (median age 9.5; range 3–15 years), 17 fulfilled ACLF criteria and 19 non-ACLF. CLD was diagnosed for the first time in 86% children during their presentation with a superimposed acute insult. Wilson disease and autoimmune liver disease were the most common underlying etiology. Acute insult was caused by hepatitis E virus (HEV) in 27 (75%) children and hepatitis A virus (HAV) in 10 (28%). The 3-month mortality of ACLF group was significantly higher than that of non-ACLF group (59% vs 11%, P = 0.001). PELD score of >25.5 predicted death, with a sensitivity of 100% and specificity of 83.3%. Conclusions: Superinfection with hepatotropic viruses on CLD in children manifests as ACLD: ACLF and non-ACLF. Hepatitis E virus is the most common superinfection in the population studied. The mortality in ACLF is 5 times higher than that in the non-ACLF group. PELD score is useful in differentiating likely survivors and nonsurvivors.