Clinical practice guidelines for Wilson's disease (WD) have been published by the American Association for the Study of Liver Diseases and European Association for the Study of the Liver in 2008 and 2012, respectively. Their focus was on the hepatic aspects of the disease. Recently, a position paper on pediatric WD was published by the European Society of Pediatric Gastroenterology Hepatology and Nutrition. A need was felt to harmonize guidelines for the hepatic, pediatric, and neurological aspects of the disease and contextualize them to the resource-constrained settings. Therefore, experts from national societies from India representing 3 disciplines, hepatology (Indian National Association for Study of the Liver), pediatric hepatology (Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition), and neurology (Movement Disorders Society of India) got together to evolve fresh guidelines. A literature search on retrospective and prospective studies of WD using MEDLINE (PubMed) was performed. Members voted on each recommendation, using the nominal voting technique. The Grades of Recommendation, Assessment, Development and Evaluation system was used to determine the quality of evidence. Questions related to diagnostic tests, scoring system, and its modification to a version suitable for resource-constrained settings were posed. While ceruloplasmin and 24-h urine copper continue to be important, there is little
A retrospective analysis of the clinical profile, endoscopic features and management of 22 children (age 18 months-18 years) diagnosed as solitary rectal ulcer syndrome is presented. The majority (81.8%) were ≥8 years of age. Rectal bleeding was the presenting feature in all the children. Mucorrhea, constipation, tenesmus and rectal prolapse were observed in 77.3%, 63.6%, 59% and 13.6% children, respectively. Colonoscopy showed classical single rectal ulcer in 68.2% and multiple ulcers in 22.7%. Polypoidal and erosive lesions were documented in 4.5% each. The medical management comprised of bowel training and high fibre diet for all children. The other modalities included oral 5-amino salicylate (59%), sucralfate enema (4.5%) and rectal mesalamine in 9%. 64% children recovered and 13.6% had recurrence of symptoms.
To our knowledge, this is the largest reported series of children with PIBD. PIBD is a safe, well-tolerated and effective alternative to liver transplant in children with PFIC and AS who have intractable pruritus in the absence of synthetic liver failure.
Pediatric inflammatory bowel disease (P-IBD) in India shares similarities with adult-onset IBD. Distinctive features were growth failure and more severe forms of the disease necessitating immunomodulators.
Pancreatic disease in children has a wide clinical spectrum and may present as Acute pancreatitis (AP), Acute recurrent pancreatitis (ARP), Chronic pancreatitis (CP) and Pancreatic disease without pancreatitis. This article highlights the etiopathogenesis and management of pancreatitis in children along with clinical data from five tertiary care hospitals in south India [Chennai (3), Cochin and Pune].
Crohn's disease (CD) in children younger than 5 years of age is termed as early onset inflammatory bowel disease (EO-IBD). We report a 4 yr 6 mo-old child with EO-IBD, who presented with palatal ulcer, an extra intestinal manifestation of Crohn's disease as the dominant feature.
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