Fulminant Wilson's disease in children: Appraisal of a critical diagnosis

Pediatric Critical Care Medicine

Volumn 4, Issue 3, 2003, Pages 338-343

  Tissières, Pierre ^a   Chevret, Laurent ^a   Debray, Dominique ^a   Devictor, Denis ^a  

^a BICÊTRE HOSPITAL   (France)

Author keywords

Child; Diagnosis; Fulminant hepatic failure; Liver transplantation; Outcome; Pediatric risk of mortality score; Pediatrics; Wilson's disease

Indexed keywords

ALKALINE PHOSPHATASE; BILIRUBIN;

ADOLESCENT; ARTICLE; BLOOD; CHILD; COMPARATIVE STUDY; CONFIDENCE INTERVAL; ENZYME ASSAY; FEMALE; HEPATIC ENCEPHALOPATHY; HUMAN; INTENSIVE CARE; LIVER FAILURE; LIVER TRANSPLANTATION; MALE; MORTALITY; PATIENT REFERRAL; RETROSPECTIVE STUDY; SENSITIVITY AND SPECIFICITY; TIME; TREATMENT OUTCOME; WILSON DISEASE;

ADOLESCENT; ALKALINE PHOSPHATASE; BILIRUBIN; CHILD; CONFIDENCE INTERVALS; ENZYME TESTS; FEMALE; HEPATIC ENCEPHALOPATHY; HEPATOLENTICULAR DEGENERATION; HUMANS; INTENSIVE CARE UNITS, PEDIATRIC; LIVER FAILURE; LIVER TRANSPLANTATION; MALE; REFERRAL AND CONSULTATION; RETROSPECTIVE STUDIES; SENSITIVITY AND SPECIFICITY; TIME FACTORS; TREATMENT OUTCOME;

EID: 0142155638     PISSN: 15297535     EISSN: 19473893     Source Type: Journal    
DOI: 10.1097/01.PCC.0000074268.77622.DE     Document Type: Article

References (30)

1. Saito T: Presenting symptoms and natural history of Wilson's disease. Eur J Pediatr 1987; 146:261-265
2. Narkewicz MR, Sokol RJ. Copper and iron storage disorders. In: Liver Disease in Children. Second Edition. Suchy FJ, Sokol RJ, Balistreri WF (Eds). Philadelphia, Lippincott Williams & Wilkins, 2001, pp 595-640
3. Sternleib I: Wilson's disease: Indications for liver transplants. Hepatology 1984; 4 (Suppl):15S-17S.
4. Rakela J, Kurtz SB, McCarthy JT, et al: Fulminant Wilson's disease treated with postdilution hemofiltration and orthotopic liver transplantation. Gastroenterology 1986; 90: 2004-2007
5. Berman DH, Leventhal RI, Gavaler JS, et al: Clinical differentiation of fulminant wilsonian hepatitis from other causes of hepatic failure. Gastroenterology 1991; 100: 1129-1134
6. Bernuau J, Rueff B, Benhamou JP: Fulminant and subfulminant liver failure: Definitions and causes. Semin Liver Dis 1986; 6:97-106
7. Devictor D, Desplanques L, Debray D, et al: Emergency liver transplantation for fulminant liver failure in infants and children. Hepatology 1992; 16:1156-1162
8. Navelet Y, Girier B, Clouzeau J, et al: Insuffisance hépato- cellulaire aiguë grave de l'enfant: Aspect EEG pronostiques. Neurophysiol Clin 1990; 20:237-245
9. Tissieres P, Devictor D: Drug management of hepatic encephalopathy associated with fulminant liver failure. CNS Drugs 1999; 11: 335-349
10. Pollack MM, Ruttimann UE, Getson PR: Pediatric risk of mortality (PRISM) score. Crit Care Med 1988; 16:1110-1116
11. O'Grady JG, Alexander GJ, Hayllar KM, et al: Early indicators of prognosis in fulminant hepatic failure. Gastroenterology 1989; 97: 439-445
12. Nazer H, Ede RJ, Mowat AP, et al: Wilson's disease: Clinical presentation and use of prognostic index. Gut 1986; 27:1377-1381
13. Tissieres P, Prontera W, Chevret L, et al: The pediatric risk of mortality score in infants and children with fulminant liver failure. Pediatr Transpl 2003; 7:64-68
14. Schiodt FV, Atillasoy E, Shakil O, et al: Etiology and outcome for 295 patients with acute liver failure in the United States. Liver Transpl 1999; 5:29-34
15. Eghtesad B, Nezakatgoo N, Geraci LC, et al: Liver transplantation for Wilson's disease: A single-center experience. Liver Transpl 1999; 5:467-474
16. McCullough AJ, Fleming CR, Thistle JL, et al: Diagnosis of Wilson's disease presenting as fulminant hepatic failure. Gastroenterology 1983; 84:161-167
17. Sanchez-Albisua I, Garde T, Hierro L, et al: A high index of suspicion: The key to an early diagnosis of Wilson's disease in childhood. J Pediatr Gastroenterol Nutr 1999; 28: 186-190
18. Giacchino R, Marazzi MG, Barabina A, et al: Syndromic variability of Wilson's disease in children. Clinical study of 44 cases. Ital J Gastroenterol Hepatol 1997; 29:155-161
19. Hillenbrand P, Parbhoo SP, Jedrychowski A, et al: Significance of intravascular coagulation and fibrinolysis in acute hepatic failure. Scand J Gastroenterol 1973; 19:133-134
20. Steindl P, Ferenci P, Dienes HP, et al: Wilson's disease in patients presenting with liver disease: A diagnostic challenge. Gastroenterology 1997; 113:212-218
21. Sallie R, Katsiyiannakis L, Baldwin D, et al: Failure of simple biochemical indexes to reliably differentiate fulminant Wilson's disease from other causes of fulminant liver failure. Hepatology 1992; 16:1206-1211
22. Emre S, Atillasoy EO, Ozdemir S, et al: Orthotopic liver transplantation for Wilson's disease. Transplantation 2001; 72:1232-1236
23. Rivera-Penera T, Moreno J, Skaff C, et al: Delayed encephalopathy in fulminant hepatic failure in the pediatric population and the role of liver transplantation. J Pediatr Gastroenterol Nutr 1997; 24:128-134
24. Bellary S, Hassanein T, Van Thiel DH: Liver transplantation for Wilson's disease. J Hepatol 1995; 23:373-381
25. Devlin J, Wendon J, Heaton N, et al: Pretransplantation clinical status and outcome of emergency transplantation for acute liver failure. Hepatology 1995; 21:1018-1024
26. Schilsky ML, Scheinberg IH, Sternlieb I: Liver transplantation for Wilson's disease: Indications, and outcome. Hepatology 1994; 19:583-587
27. Rela M, Heaton ND, Vougas V, et al: Orthotopic liver transplantation for hepatic complications of Wilson's disease. Br J Surg 1993; 80:909-911
28. Eghtesad B, Mazariegos GV, Cacciarelli T, et al: Liver transplantation in pediatric patients with Wilson's disease. Abstr. Pediatr Transpl 1998; 2:77
29. Kreymann B, Seige M, Schweigart U, et al: Albumin dialysis: Effective removal of copper in a patient with fulminant Wilson disease and successful bridging to liver transplantation: A new possibility for the elimination of proteinbound toxins. J Hepatol 1999; 31:1080-1085
30. Kiss JE, Berman D, Van Thiel D: Effective removal of copper by plasma exchange in fulminant Wilson's disease. Transfusion 1998; 38:327-331