{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T10:49:55Z","timestamp":1792838995606,"version":"4.5.0"},"reference-count":13,"publisher":"BMJ","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Gut"],"published-print":{"date-parts":[[1986,11]]},"abstract":"<jats:p>As the results of treatment in Wilson's disease are so dependent on the stage at which penicillamine therapy is started, the antecedent history in 34 patients with Wilson's disease was analysed with particular respect to the earliest manifestations of the disease. Lethargy and anorexia (70%) jaundice (56%) and abdominal pain (48%) were the commonest symptoms and less common were intellectual deterioration (22%) and recurrent epistaxes (22%). The duration of symptoms before diagnosis ranged from five days to three years (mean 10.5 months) and in only five of the patients was the diagnosis established before referral. Analysis of the physical signs at presentation showed hepatomegaly (81%) and splenomegaly (70%) to be common and the only signs which were significantly more common in the 13 fatal cases were jaundice and ascites. In three of these and in one other patient who survived the clinical course was exceptionally severe and was indistinguishable from fulminant hepatic failure. Based on the severity of abnormality of serum aspartate aminotransferase, bilirubin, and prothrombin time on admission a prognostic index was derived which enabled complete separation of fatal and nonfatal cases and when subsequently used in a further nine index cases correctly predicted the outcome. Two further cases found to have indices in the fatal category did well after liver transplantation, which needs to be considered as soon as the diagnosis is established in cases with such severe liver damage.<\/jats:p>","DOI":"10.1136\/gut.27.11.1377","type":"journal-article","created":{"date-parts":[[2007,9,18]],"date-time":"2007-09-18T17:41:31Z","timestamp":1190137291000},"page":"1377-1381","source":"Crossref","is-referenced-by-count":212,"title":["Wilson's disease: clinical presentation and use of prognostic index."],"prefix":"10.1136","volume":"27","author":[{"given":"H","family":"Nazer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R J","family":"Ede","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A P","family":"Mowat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R","family":"Williams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[1986,11,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1177\/000992288302201104","article-title":"Wilson's disease in childhood: variability of clinical presentation","volume":"22","author":"Nazer, H.; Ede, R.J.; Mowat, A.P.; Williams, R.","year":"1983","unstructured":"Nazer H, Ede RJ, Mowat AP, Williams R. Wilson's disease in childhood: variability of clinical presentation. Clin Paediatr 1983; 22: 755-7.","journal-title":"Clin Paediatr"},{"issue":"30","key":"ref_2","first-page":"402","article-title":"The liver in juvenile Wilson's disease","volume":"1962","author":"Silverberg, M.; Gellis, S.S.","unstructured":"Silverberg M, Gellis SS. The liver in juvenile Wilson's disease. Paediatrics 1962; 30: 402-13.","journal-title":"Paediatrics"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1056\/NEJM196702232760804","article-title":"Haemolytic anaemia in Wilson's disease","volume":"276","author":"McIntyre, N.; Clink, H.M.; Levi, A.J.; Cumings, J.N.; Sherlock, S.","year":"1967","unstructured":"McIntyre N, Clink HM, Levi AJ, Cumings JN, Sherlock S. Haemolytic anaemia in Wilson's disease. N Engi J Med 1967; 276: 439.","journal-title":"N Engi J Med"},{"key":"ref_4","first-page":"472","article-title":"Maladie de Wilson's a debut trompeur. Hematurie puis syndrome d'hypertension portale","volume":"20","author":"Pellett, H.; Gallard, L.; Monier, D.; Ruitton-Ugliengo","year":"1965","unstructured":"Pellett H, Gallard L, Monier D, Ruitton-Ugliengo. Maladie de Wilson's a debut trompeur. Hematurie puis syndrome d'hypertension portale. Pediatrie 1965; 20: 472-3.","journal-title":"Pediatrie"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1136\/ard.36.2.99","article-title":"Arthropathy of Wilson's disease. Study of clinical and radiological features in 32 patients","volume":"36","author":"Golding, D.N.; Walshe, J.M.","year":"1977","unstructured":"Golding DN, Walshe JM. Arthropathy of Wilson's disease. Study of clinical and radiological features in 32 patients. Ann Rheum Dis 1977; 36: 99-111.","journal-title":"Ann Rheum Dis"},{"key":"ref_6","first-page":"161","article-title":"Diagnosis of Wilson's disease presenting","volume-title":"as fulminant hepatic failure. Gastroenterology 1983; 84","author":"McCullough, A.J.; Fleming, C.R.; Thistle, J.L.","unstructured":"McCullough AJ, Fleming CR, Thistle JL et al. Diagnosis of Wilson's disease presenting as fulminant hepatic failure. Gastroenterology 1983; 84: 161-7."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1016\/S0016-5085(79)80024-4","article-title":"The role of radiocopper in the diagnosis of Wilson's disease","volume":"77","author":"Sternlieb, I.; Scheinberg, I.H.","year":"1979","unstructured":"Sternlieb I, Scheinberg IH. The role of radiocopper in the diagnosis of Wilson's disease. Gastroenterology 1979; 77: 138-42.","journal-title":"Gastroenterology"},{"key":"ref_8","first-page":"95","article-title":"Wilson's disease","volume":"1984","author":"Scheinberg, I.; Sternlieb, I.","unstructured":"Scheinberg I, Sternlieb I. Wilson's disease. Philadelphia. Saunders, 1984: 95.","journal-title":"Philadelphia. Saunders"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1016\/S0022-3476(71)80458-4","article-title":"The varied manifestations of Wilson's disease","volume":"78","author":"Slovis, T.L.; Dubois, R.S.; Rodgerson, D.O.","year":"1971","unstructured":"Slovis TL, Dubois RS, Rodgerson DO et al. The varied manifestations of Wilson's disease. J Pediatr 1971; 78: 578-84.","journal-title":"J Pediatr"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1148\/10.1148\/94.1.127","article-title":"Skeletal changes in Wilson's disease. A radiological study","volume":"94","author":"Mindelzun, R.; Ekin, M.; Scheinberg, I.H.; Sternleib, I.","year":"1970","unstructured":"Mindelzun R, Ekin M, Scheinberg IH, Sternleib I. Skeletal changes in Wilson's disease. A radiological study. Radiology 1970; 94: 127-32.","journal-title":"Radiology"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1016\/S0140-6736(82)92201-2","article-title":"Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride","volume":"1","author":"Walshe, J.M.","year":"1982","unstructured":"Walshe JM. Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride. Lancet 1982; 1: 643-7.","journal-title":"Lancet"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"314","DOI":"10.7326\/0003-4819-99-3-314","article-title":"Oral zinc therapy for Wilson's disease","volume":"99","author":"Brewer, G.J.; Gretchen, M.H.; Prasad, A.S.","year":"1983","unstructured":"Brewer GJ, Gretchen MH, Prasad AS et al. Oral zinc therapy for Wilson's disease. Ann Intern Med 1983; 99: 314-20.","journal-title":"Ann Intern Med"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"398","DOI":"10.7326\/0003-4819-99-3-398","article-title":"Treatment of Wilson's disease","volume":"99","author":"Deiss, A.","year":"1983","unstructured":"Deiss A. Treatment of Wilson's disease. Ann Intern Med 1983; 99: 398-400.","journal-title":"Ann Intern Med"}],"container-title":["Gut"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/gut.27.11.1377","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,13]],"date-time":"2025-12-13T12:17:29Z","timestamp":1765628249000},"score":1,"resource":{"primary":{"URL":"https:\/\/gut.bmj.com\/lookup\/doi\/10.1136\/gut.27.11.1377"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1986,11]]},"references-count":13,"journal-issue":{"issue":"11","published-online":{"date-parts":[[1986,11,1]]},"published-print":{"date-parts":[[1986,11]]}},"alternative-id":["10.1136\/gut.27.11.1377"],"URL":"https:\/\/doi.org\/10.1136\/gut.27.11.1377","relation":{},"ISSN":["0017-5749","1468-3288"],"issn-type":[{"value":"0017-5749","type":"print"},{"value":"1468-3288","type":"electronic"}],"subject":[],"published":{"date-parts":[[1986,11]]}}}