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
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.
Micronutrient supplementation, algorithm based diet regimens, and good supportive care are sufficient in most children above 6 months of age with persistent diarrhea. Paucity of diagnostic facilities limits evaluation of chronic diarrhea in most parts of the country and regional laboratories need to be set up urgently. Lack of awareness regarding cows milk protein allergy, celiac disease and immunodeficiency associated diarrhea is of particular concern.
In 376 children, between 6 months and 5 years of age, with suspected urinary tract infection, 4 parameters of a routine urine examination were correlated with culture reports. In diagnosing urinary infection, the sensitivity and specificity of proteinuria was 79 and 80% respectively, that of bacteriuria 78 and 96% and that of pyuria > 10 wbc/hpf 80 and 82% respectively. 61% among the culture positive groups had all these three parameter present, as against only 0.5% in the culture negative group (P < 0.001). All these 3 parameters were absent in 70% in the culture negative group, as against 8% in the culture positive group (P < 0.001). Bacteriuria in association with either proteinuria or Pyuria > 10 Wbc/hpf had 98% specialty is diagnosis. In diagnosis UTI, Pyuria > 10 wbc/hpf was significantly more specific (82:66.6) than the conventional > 5 wbc/hpf. Isolated proteinuria, isolated pyuria, isolated bacteriuria and microscopic haematuria were not features of urinary tract infection in children. Urine culture can be more selectively done if the routine urinalysis is well interpreted. In the absence of dependable culture facilities, a routine urine examination can be reliably used in the diagnosis of UTI in children.
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