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[This corrects the article DOI: 10.1016/j.jceh.2018.08.009.].
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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 role of serum copper and penicillamine challenge test in the diagnostic algorithm. A new scoring system - Modified Leipzig score has been suggested with extra points being added for family history and serum ceruloplasmin lower than 5 mg/dl. Liver dry copper estimation and penicillamine challenge test have been removed from the scoring system. Differences in pharmacological approach to neurological and hepatic disease and global monitoring scales have been included. Rising bilirubin and worsening encephalopathy are suggested as indicators predicting need for liver transplant but need to be validated. The clinical practice guidelines provide recommendations for a comprehensive management of WD which will be of value to all specialties.
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OBJECTIVE: Little is known about cow's milk protein intolerance (CMPI) in India. This study was aimed at finding CMPI cases and determining the role of IgG anti-beta-lactoglobulin antibody in the diagnosis of this condition in India. METHODS: From June 2004 to December 2005, 30 children with presumptive diagnosis of CMPI, based on endoscopic rectal or duodenal biopsy showing excess eosinophils and response to milk withdrawal, were enrolled and studied prospectively. Definite diagnosis was made in 20 children on the basis of positive milk challenge. IgG anti-beta-lactoglobulin antibodies were tested in children with CMPI before and after stopping milk and after milk challenge. Antibody levels were also studied in 27 age-matched disease controls and 50 healthy adults. RESULTS: The median age of 20 children (16 boys) with CMPI was 16.5 (6-36) months. Of them, 18 presented with diarrhea (12 bloody) and 2 had rectal bleeding. The presumptive diagnosis was most often (85%) based on colonic or rectal biopsy findings. Rectal biopsy was diagnostic in all 20 cases irrespective of the mode of presentation compared with duodenal biopsy which was diagnostic in 3 cases (p< 0.0001). There was no difference in antibody levels between cases and controls; the antibody level decreased significantly after milk withdrawal (p< 0.005), but did not rise significantly after milk re-challenge. CONCLUSIONS: CMPI is a common cause of chronic diarrhea in children in northern India. Sigmoidoscopy and rectal biopsy help in establishing the diagnosis in most cases. IgG anti-lactoglobulin antibody test is not useful in diagnosing CMPI in the Indian setting.