Fulminant Wilsonian hepatitis unmasked by disease progression: Report of a case and review of the literature

Digestive Diseases and Sciences

Volumn 44, Issue 5, 1999, Pages 1054-1058

  Ferlan Marolt, Vera ^a,b   Stepec S ^a,b  

^a INSTITUTE OF PATHOLOGY   (Slovenia)

^b UNIVERSITY MEDICAL CENTRE LJUBLJANA   (Slovenia)

Author keywords

Fulminant hepatic failure; Hepatic morphology; Wilson's disease; Wilsonian hepatitis

Indexed keywords

BIOCHEMICAL MARKER;

ADULT; ARTICLE; CLINICAL FEATURE; COPPER METABOLISM; DIFFERENTIAL DIAGNOSIS; DISEASE COURSE; FEMALE; HEMOLYTIC ANEMIA; HEPATITIS; HUMAN; LIVER FAILURE; LIVER HISTOLOGY; PRIORITY JOURNAL; WILSON DISEASE;

ADULT; CERULOPLASMIN; COPPER; DISEASE PROGRESSION; FATAL OUTCOME; FEMALE; HEPATIC ENCEPHALOPATHY; HEPATITIS; HEPATOLENTICULAR DEGENERATION; HUMANS; KIDNEY TUBULES, PROXIMAL;

EID: 0032959057     PISSN: 01632116     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1026637403894     Document Type: Article

References (26)

1. Wilson SAK: Progressive lenticular degeneration: A familial nervous disease associated with cirrhosis of the liver. Brain 34:295-509, 1912
2. Berman DH, Leventhal RJ, Gavaler JS, Cadoff EM, Van Thiel DH: Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure. Gastroenterology 100:1129-1134, 1991
3. Walshe JM: Wilson's disease presenting with features of hepatic dysfunction. Q J Med 70:253-263, 1989
4. Rector WG, Uchida T, Kanel GC, Redeker AG, Reynolds TB: Fulminant hepatic and renal failure complicating Wilson's disease. Liver 4:341-347, 1984
5. McCullough AJ, Wiesner RH, Fleming CR, Dickson ER: Antemortem diagnosis and shortterm survival of a patient with Wilson's disease presenting as fulminant hepatic failure. Dig Dis Sci 29:862-864, 1984
6. Schilsky ML, Scheinberg IH, Sternlieb I: Prognosis of Wilsonian chronic active hepatitis. Gastroenterology 100:762-767, 1991
7. Johnson PJ, Williams R: Excellent prognosis in Wilsonian chronic active hepatitis: New data or an article of faith? Hepatology 14:1293-1295, 1991
8. Zilker TR, Felgenhauer N, Hibler A, von Charmann CM: Indications for liver transplantation in Wilson's disease with a fulminant course, Dtch Med Wochenschr 114:871-875, 1989
9. Davies SE, Williams R, Portmann B: Hepatic morphology and histochemistry of Wilson's disease presenting as fulminant hepatic failure: A study of 11 cases. Histopathology 15:385-394, 1989
10. Gur H, Aderka D, Finkelstein A, Yoram L: Fulminant Wilsonian hepatitis: Difficulties in diagnosis and treatment. Am J Gastroenterol 83:679-681, 1988
11. Fleming CR, Dickson ER, Wahner HW, Hollenhorst RW: Pigmented corneal rings in non-Wilsonian liver disease. Am Intern Med 86:285-288, 1977
12. Kaplinsky C, Sternlieb I, Javitt N, Rotem Y: Familial cholestatic cirrhosis associated with Kayser-Fleischer rings. Pediatrics 65:782-788, 1980
13. Sallie R, Katsiyiannakis L, Baldwin D, Davies S, O'Grady J, Mowat A, Mieli-Vergani G, Williams R: Failure of simple biochemical indexes to reliably differentiate fulminant Wilson's disease from other causes of fulminant liver failure. Hepatology 16:1206-1211, 1992
14. Schilsky ML, Scheinberg IH, Sternlieb I: Prognosis of Wilsonian chronic active hepatitis. Gastroenterology 100:762-767, 1991
15. Walshe JM, Briggs J: Ceruloplasmin in liver disease: a diagnostic pitfall. Lancet 2:263-265, 1962
16. McCullough AJ, Fleming R, Thistle JL, Baldus WP, Ludwig J, McCall JT, Dickson R: Diagnosis of Wilson's disease presenting as fulminant hepatic failure. Gastroenterology 84:161-167, 1983
17. Nazer H, Ede RJ, Mowat AP, Williams R: Wilson's disease: Clinical presentation and use of prognostic index. Gut 27:1377-1381, 1986
18. Deiss A, Lee GR, Carwright GE: Hemolytic anemia in Wilson's disease. Ann Intern Med 73:413-418, 1979
19. Chan TK, Todd D: Haemolysis complicating viral hepatitis in patients with glucose-6-phosphate dehydrogenase deficiency. Br Med J 1:131-133, 1975
20. O'Grady JG, Fagan EA, Williams R: Fulminant viral hepatitis. Clin Trop Med Communic Dis 1:361-376, 1986
21. Lashner BA, Jones RB, Tang HS, Evans AA, Ozeran SE, Baker AL: Chronic hepatitis: Disease factors at diagnosis predictive of mortality. Am J Med 85:609-614, 1988
22. Scheinberg IH, Sternlieb I, Schilsky ML, Stockert RJ: Penicillamine may detoxify copper in Wilson's disease. Lancet 2:95, 1987
23. Durand F, Bernuan J, Samuel D, Saliba F, Shouval D, Benhamou JP: Severe acute Wilson's disease: an indication for emergency administration of high dose D-penicillamine. J Hepatol 11(suppl 2):S20, 1990
24. Walshe JM: Diagnosis and treatment of presymptomatic Wilson's disease. Lancet 11:435-437, 1988
25. Ludwig J, Mayer TP, Rakela J: The liver biopsy diagnosis of Wilson's disease: Methods in pathology. Am J Clin Pathol 102:443-446, 1994
26. Zilker TR, Felgenhauer N, Hibler A, von Clarmann CM: Indications for liver transplantation in Wilson's disease with a fulminant course. Dtsch Med Wochenschr 114:871-875, 1989