| Zdroj: |
Nagral, A, Sarma, M S, Matthai, J, Prashanth, L K, Devarbhavi, H, Sinha, S, Alam, S, Bavdekar, A, Dhiman, R K, Eapen, C E, Goyal, V, Mohan, N, Kandadai, R M, Sathiyasekaran, M, Poddar, U, Sibal, A, Sankaranarayanan, S, Srivastava, A, Thapa, B R, Wadia, P, Yachha, S K & Dhawan, A 2018, ' Wilson’s disease: Clinical practice guidelines of the Indian National Association for the study of Liver (INASL), The Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition (ISPGHAN) and the Movement Disorders Society of India (MDSI) ', Journal of Clinical and Experimental Hepatology . https://doi.org/10.1016/j.jceh.2018.08.009 |
| Popis: |
Clinical practice guidelines for Wilson's disease have been published by AASLD and EASL in 2008 and 2012 respectively. Their focus was on the hepatic aspects of the disease. Recently, a position paper on pediatric Wilson's disease was published by the ESPGHAN. A need was felt to harmonise guidelines for the hepatic, pediatric and neurological aspects of the disease and contextualise them to the resource constrained settings. Therefore, experts from national societies from India representing three disciplines, Hepatology (INASL: Indian National Association for the study of Liver), Pediatric Hepatology (ISPGHAN: Indian Society of Pediatric Gastroenterology, Hepatology and Nutrition) and Neurology (MDSI: Movement Disorders Society of India) got together to evolve fresh guidelines. A literature search on retrospective and prospective studies of Wilson's disease using MEDLINE (Pubmed) were performed. Members voted on each recommendation, using the nominal voting technique. GRADE 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 hour urine copper continue to be important, there is little role of serum copper and penicillamine challenge test in the diagnostic algorithm. A scoring system modified from the Leipzig scoring system has been suggested with extra points being added for family history and serum ceruloplasmin lower than 5 mg/dl. Liver dry copper estimation does not seem to be the gold standard and has been removed from the scoring system. Differences in pharmacological approach to Neurological and Hepatic disease as well as 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 Wilson's disease which will be of value to all specialities. |
