Wilson's Disease: Clinical Practice Guidelines of the Indian National Association for Study of the Liver, the Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition, and the Movement Disorders Society of India

Abstract

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

Clinicohistological correlation of etiological spectrum of chronic liver disease diagnosed during noncirrhotic stages in children: Can need of liver biopsy be obviated?

“…16 For WD, the Leipzig score could not be calculated in our cases as the dry weight of Cu estimation was beyond our scope. 17 The proportion of AIH as etiology in our case series of CLD is higher in contrast to what has been reported in the literature on adult populations as diseases like AIH, with a faster rate of progression, will obviously manifest more frequently in the younger age group. 18 All the four children with SH had high elastography values (Mean ± SD, 10 ± 0.5 kPa) and significant fibrosis (≥F2) and are adolescents with CHA, with poor chelation.…”

Comparative effectiveness of common therapies for Wilson disease: A systematic review and meta‐analysis of controlled studies

“…Probably mostly owing to the fact that DPen was introduced as a successful treatment for WD in the late 1950s – at least 30 years before any other treatment used today – it has remained the standard of care for WD patients in most countries . The dominance of DPen or, more generally, chelator therapy is also reflected in current guidelines . These suggest that symptomatic patients should be treated with a chelating agent, although Zn may be used as first‐line therapy in those with neurological disease .…”

“…17 The dominance of DPen or, more generally, chelator therapy is also reflected in current guidelines. [18][19][20][21] These suggest that symptomatic patients should be treated with a chelating agent, although Zn may be used as first-line therapy in those with neurological disease. [18][19][20][21] In presymptomatic patients, either a chelator or Zn can be used.…”

“…[18][19][20][21] These suggest that symptomatic patients should be treated with a chelating agent, although Zn may be used as first-line therapy in those with neurological disease. [18][19][20][21] In presymptomatic patients, either a chelator or Zn can be used. [18][19][20][21] These recommendations were partly based on a systematic review on initial treatment of WD from 2009 that included all studies published at that time describing outcome, both controlled and non-controlled.…”

“…[18][19][20][21] In presymptomatic patients, either a chelator or Zn can be used. [18][19][20][21] These recommendations were partly based on a systematic review on initial treatment of WD from 2009 that included all studies published at that time describing outcome, both controlled and non-controlled. 22 This systematic review was limited by the small number of symptomatic patients that were treated with Zn.…”