Pediatric Inflammatory Bowel Disease.

Background: The incidence of inflammatory bowel disease is increasing in the pediatric population worldwide. Need and purpose of review: There 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. Methods: A 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 conclusions: 25% 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.

Management of Neonatal Cholestasis: Consensus Statement of the Pediatric Gastroenterology Chapter of Indian Academy of Pediatrics.

Justification: Neonatal 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. Process: Eminent 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. Objectives: To 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. Recommendations: Early 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 sub- optimally managed cases, referral to regional centers is warranted.

Outcome of Live Donor Liver Transplantation in Indian Children with Bodyweight <7.5 kg.

This case-series analyzed the outcome of live donor liver transplantation (LT) performed in children <7.5kg from January 2008 to June 2009 at our center. Five patients (3 males, 2 females, mean age, 8.2 ± .4 months; mean weight 6.8 ± 0.4 kg) underwent LT. The indications of LT included biliary atresia (3) and idiopathic neonatal hepatitis (2). Postoperative complications included acute rejection (1), portal venous thrombosis (1), bile leak (1), severe hypertension (1) and bacterial sepsis (4). There were no donor related complications. The median follow-up duration is 11 months with patient and graft survival rates of 100% each, respectively.

Cholecystoappendicostomy for Progressive Familial Intrahepatic Cholestasis.

We report a rare case of progressive familial intrahepatic cholestasis type 2 from India. The diagnosis was confirmed on the basis of gene mutation analysis. The child had intense pruritus refractory to conventional medical management. As liver biopsy did not reveal any cirrhosis, partial external biliary diversion was considered as an alternative to liver transplant. We performed cholecystoappendicostomy rather than the conventional method of using an ileal loop as a conduit between the gall bladder and abdominal wall. Child recovered completely.

GastrointentinaI bleed with clopidogrel and aspirin.

Anti-platelet drugs have been used to prevent thrombosis of systemic to pulmonary artery shunts. Aspirin has traditionally been used. Clopidogrel is being studied as an alternative and in combination with aspirin for shunt patients. We report a near fatal gastro-intestinal bleed in a patient with shunt and on aspirin and clopidogrel. This combination has been known to produce similar bleeds. The authors recommend caution in combining them. Prospective studies currently underway should evaluate this aspect of the antiplatelet drugs.

Current status of liver transplantation.

Liver transplantation is accepted therapy for acute or chronic liver failure. Survival after LT has improved significantly in developed countries and this has increased the awareness of this treatment modality in the developing world. Successful LT in both children and adults have now been reported from India. Chronic liver failure secondary to cholestatic liver disease in the most frequent indication for LT, with biliary with atresia as the single commonest cause. Innovative techniques such as reduced size, splint, and living donor liver transplantation are being applied more often to decrease long waiting times and reduce associated morbidity and mortality. Early postoperative complications include primary graft failure, venous thrombosis, rejection, biliary complications and infections. Late complication includes CMV or EBV infections, side effects of immunosuppression, post transplantation lymphoproliferative disease and late biliary strictures. Most children achieve good quality of life. There are still many lessons to learn and there are future challenges such as the ever increasing problems of donor scarcity and the search for potent but less toxic immunosuppressive agents.