Literature DB >> 14689090

[44-year-old patient with fulminant liver failure].

A Kerber1, C Sarrazin, C Allers, B Markus, K Engels, W Caspary, S Zeuzem.   

Abstract

The 44-year old female patient was admitted with acute hepatic failure and extensive haemolysis under the preliminary diagnosis of Wilson's disease. General characteristic criteria of Wilson's disease as Kayser-Fleischer ring, low serum copper and low ceruloplasmin levels were not observed. The preliminary diagnosis of acute Wilson's disease was established on the basis of the characteristic laboratory values with an AP/bilirubin ratio <2, an AST/ALT ratio >4, accompanying hemolysis and a highly elevated cupruresis. The definitive diagnosis of Wilson's disease was verified after orthotopic liver transplantation by quantitative copper evaluation in the explanted liver. The case represents the yet oldest patient reported with an acute manifestation of Wilson's disease.

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Year:  2003        PMID: 14689090     DOI: 10.1007/s00108-003-1016-3

Source DB:  PubMed          Journal:  Internist (Berl)        ISSN: 0020-9554            Impact factor:   0.743


  18 in total

1.  Mechanisms of copper incorporation during the biosynthesis of human ceruloplasmin.

Authors:  M Sato; J D Gitlin
Journal:  J Biol Chem       Date:  1991-03-15       Impact factor: 5.157

2.  Identification and analysis of mutations in the Wilson disease gene (ATP7B): population frequencies, genotype-phenotype correlation, and functional analyses.

Authors:  A B Shah; I Chernov; H T Zhang; B M Ross; K Das; S Lutsenko; E Parano; L Pavone; O Evgrafov; I A Ivanova-Smolenskaya; G Annerén; K Westermark; F H Urrutia; G K Penchaszadeh; I Sternlieb; I H Scheinberg; T C Gilliam; K Petrukhin
Journal:  Am J Hum Genet       Date:  1997-08       Impact factor: 11.025

3.  Wilson's disease in patients presenting with liver disease: a diagnostic challenge.

Authors:  P Steindl; P Ferenci; H P Dienes; G Grimm; I Pabinger; C Madl; T Maier-Dobersberger; A Herneth; B Dragosics; S Meryn; P Knoflach; G Granditsch; A Gangl
Journal:  Gastroenterology       Date:  1997-07       Impact factor: 22.682

Review 4.  Wilson's disease. Update of a systemic disorder with protean manifestations.

Authors:  J A Cuthbert
Journal:  Gastroenterol Clin North Am       Date:  1998-09       Impact factor: 3.806

Review 5.  Wilson disease: genetic basis of copper toxicity and natural history.

Authors:  M L Schilsky
Journal:  Semin Liver Dis       Date:  1996-02       Impact factor: 6.115

Review 6.  Wilson's disease.

Authors:  G Loudianos; J D Gitlin
Journal:  Semin Liver Dis       Date:  2000       Impact factor: 6.115

7.  Hepatocyte-specific localization and copper-dependent trafficking of the Wilson's disease protein in the liver.

Authors:  M Schaefer; R G Hopkins; M L Failla; J D Gitlin
Journal:  Am J Physiol       Date:  1999-03

8.  Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride.

Authors:  J M Walshe
Journal:  Lancet       Date:  1982-03-20       Impact factor: 79.321

9.  Neurological and neuropsychiatric spectrum of Wilson's disease: a prospective study of 45 cases.

Authors:  W Oder; G Grimm; H Kollegger; P Ferenci; B Schneider; L Deecke
Journal:  J Neurol       Date:  1991-08       Impact factor: 4.849

10.  The Wilson disease gene: spectrum of mutations and their consequences.

Authors:  G R Thomas; J R Forbes; E A Roberts; J M Walshe; D W Cox
Journal:  Nat Genet       Date:  1995-02       Impact factor: 38.330

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  1 in total

1.  Liver transplantation for late-onset presentations of acute liver failure in Wilson's disease: The UK experience over 2 decades.

Authors:  Samuel Shribman; Gwilym Webb; Rhiannon Taylor; Thomas T Warner; Adam Duckworth; Alexander Gimson; Achuth Shenoy; William Griffiths
Journal:  JHEP Rep       Date:  2020-03-09
  1 in total

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