Vidyut Bhatia

Management of neonatal cholestasis: Consensus statement of the pediatric gastroenterology chapter of Indian academy of pediatrics

JustificationNeonatal cholestasis is an important cause of chronic liver disease in young children. Late referral and lack of precise etiological diagnosis are reasons for poor outcome in substantial number of cases in India. There is a need to create better awareness among the pediatricians, obstetricians and primary care physicians on early recognition, prompt evaluation and referral to regional centers.ProcessEminent national faculty members were invited to participate in the process of forming a consensus statement. Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. A round table conference was organized; presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft.ObjectivesTo review available published data on the subject from India and the West, to discuss current diagnostic and management practices in major centers in India, and to identify various problems in effective diagnosis and ways to improve the overall outcome. Current problems faced in different areas were discussed and possible remedial measures were identified. The ultimate aim would be to achieve results comparable to the West.RecommendationsEarly recognition, prompt evaluation and algorithm-based management will improve outcome in neonatal cholestasis. Inclusion of stool/urine color charts in well baby cards and sensitizing pediatricians about differentiating conjugated from the more common unconjugated hyperbilirubinemia are possible effective steps. Considering the need for specific expertise and the poor outcome in suboptimally managed cases, referral to regional centers is warranted.

Management of Acute Liver Failure in Infants and Children: Consensus Statement of the Pediatric Gastroenterology Chapter, Indian Academy of Pediatrics

ProcessSelected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. On 17th December 2011, Kunwar Viren Oswal round table conference was organized by the Apollo Center for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi and the Sub-specialty Chapter of Pediatric Gastroenterology, Indian Academy of Pediatrics. Presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft.ObjectivesTo formulate comprehensive evidence based guidelines for management of acute liver failure in IndiaRecommendationsViral hepatitis is the leading cause of acute liver failure (ALF) in India. Search for metabolic etiology, particularly in infants and neonates, and in apparently idiopathic cases needs to be done. Planning for early transfer is important as the risks involved with patient transport may increase or even preclude transfer at later stages. Management should be in an intensive care setting in select situations. There is currently insufficient evidence to routinely prescribe branched-chain amino acids, non-absorbable antibiotics or lactulose. Group recommends use of N-acetyl cysteine routinely in patients with ALF. Administration of antibiotics is recommended where infection is present or the likelihood of impending sepsis is high. Enteral nutrition is preferred to parenteral nutrition. Protein restriction is not recommended. An international normalized ratio >4 or Factor V concentration of <25% are the best available criteria for listing for liver transplantation. Overall 40–50% of ALF patients survive without transplantation. Survival in patients fulfilling criteria for liver transplantation and not transplanted is 10–20%. Liver transplantation is a definite treatment for ALF with high one-and five-year survival rates.

Experience of 100 solid organ transplants over a five-yr period from the first successful pediatric multi-organ transplant program in India

To analyze the clinical profile and outcome of pediatric patients who had undergone a liver and/or RT at our center over a five yr period, case records of all the patients who had undergone a liver or RT were analyzed retrospectively. One hundred solid organ transplants were performed at our center between January 2007 and January 2012. These included 50 liver, 44 renal, one sequential liver and renal, and two CLKT. BA was the most common indication for an LT (38%). At a median follow‐up of two yr three months, the patient survival was 88%. The most common indication for an RT was chronic glomerulonephritis (54.5%). At a median follow‐up of three yr, the survival was 91%. The CLKT were performed for hyperoxaluria. Two yr post LT, a sequential RT was performed for ESRD resulting from transplant associated microangiopathy. All patients received a living related graft. The common post‐operative complications were infections, vascular complications, and graft dysfunction. Survival rates for liver and RT at our center are comparable to those in the established centers in the West.

Are fathers catching up with mothers in liver donation

abnormalities [1-3]. Fluorescence in situ hybridization (FISH) analysis was carried out using TUPLE region probe (from Kreatech Diagnostics, Netherland) on metaphase and interphase cells. Presence of two intact signals on chromosome 22 ruled out 22q11.2 deletion. Thus, chromosomal analysis was carried out using the GTG-banding technique and the patient was found to be tetrasomy for sex chromosome-X i.e. 48,XXXX.

Incidentally Detected Hepatocellular Carcinoma in Cirrhotic Children

To the Editor: Unlike hepatoblastoma, hepatocellular carcino-ma (HCC) in children is rare and generally develops in thepresence of some underlying liver disease and/or cirrhosis. Inchildrenitisusuallyassociatedw ithviralhepatitis,tyrosinemia,progressive familial intrahepatic cholestasis (PFIC) or rarely inbiliary atresia [1]. Although, regular surveillance done by ul-trasoundexaminationandalpha-fetoprotein(AFP)testingleadsto early detection of HCC, it can be missed and only detectedincidentally during the histopathological analysis of explantedliver tissue [2–4].We decided to look at the explanted liver histopathologyof the last 35 children, without increased AFP or nodules onimaging, who had undergone a liver transplant at our center.The underlying etiology was biliary atresia (n=16), PFIC(n=8), Wilson disease (n=4), cryptogenic cirrhosis (n=6)and tyrosinemia (n=1). All patients had been screened forbiochemical markers like alpha-fetoprotein, viral markers(hepatitis B and C) and radiological imaging including ul-trasonography and CT abdomen. The explanted livers wereexamined at intervals of 0.5 cm. Two specimens (5.7 %)were detected to have an underlying HCC. Both the caseshadsinglefocusofthelesion.ThediagnosiswasPFICinonecase and cryptogenic cirrhosis in the other. Both the caseshad no clinical features suggestive of HCC either during thesurgery or on follow-up. The AFP levels were 6.7 and8.5 mg/dL respectively. Both the children have remainedwell and have not shown any sign/symptom of HCC.The rate of transition from established cirrhosis to HCCoccurs at a rate of 1–4 % per year [5]. The proportion ofincidentally detected HCC in our series is 5.7 % whereas inother studies it ranges from 9.74 % to 63.3 % [2–4].Both ourcases were unifocal whereas in reports from adults the inci-dence of multinodular HCC varies from 23.3 to 60 %. Thiscouldpossiblybeduetothelesserdurationforwhichtheliverremains cirrhotic in children. Incidental finding of hepatocel-lular carcinoma on explanted liver pathology is uncommon,but not rare [2–4]. Our study shows that biochemical andradiologicalscreeningmaynotalwaysbehelpfulfordetectionof the malignant change in spite of extensive pre-transplantwork up in cirrhotic children. Serum alpha-fetoprotein levelsand ultrasound screening though helpful but are not absolutemarkers of the malignant change. If HCC is detected inexplanted liver histopathology, the patient needs to be evalu-ated for the recurrence of HCC in follow-up.References

Antituberculosis Therapy–Associated Cutaneous Leukocytoclastic Vasculitis

Antituberculosis therapy-associated cutaneous leukocytoclastic vasculitis (CLV) has been rarely reported. We describe a case of CLV induced by rifampicin and pyrazinamide. A 14-year-old male diagnosed with disseminated tuberculosis developed purpuric lesions after 1.5 months of treatment. Histopathology was consistent with leukocytoclastic vasculitis. Skin lesion improved after cessation of the two drugs and treatment with corticosteroids.

Pediatric inflammatory bowel disease

BackgroundThe incidence of inflammatory bowel disease is increasing in the pediatric population worldwide.Need and purpose of reviewThere is paucity of high quality scientific data regarding pediatric inflammatory bowel disease. Most of the guidelines are offshoots of work done in adults, which have been adapted over time to diagnose and treat pediatric patients. This is in part related to the small numbers in pediatric inflammatory bowel disease and less extensive collaboration for multicentric trials both nationally and internationally.MethodsA literature search was performed using electronic databases i.e. Pubmed and OVID, using keywords: pediatric, inflammatory bowel disease, Crohn’s disease, Ulcerative colitis, epidemiology and guidelines. This article amalgamates the broad principles of diagnosing and managing a child with suspected inflammatory bowel disease.Main conclusions25% of the patients with inflammatory bowel disease are children and and young adolescents. The primary concern is its impact on growth velocity, puberty and quality of life, including psychosocial issues. Treatment guidelines are being re-defined as the drug armamentarium is increasing. The emphasis will be to achieve mucosal healing and normal growth velocity with minimal drug toxicity.