Fulminant Wilson's disease in children: appraisal of a critical diagnosis.
OBJECTIVE
To characterize fulminant Wilson's disease in children, outline clinical and biochemical differences to fulminant hepatic failure of other etiologies, and compare sensitivity and specificity in diagnosing fulminant Wilson's disease of alkaline phosphatase to total bilirubin ratio and aspartate aminotransferase to alanine aminotransferase ratio.


METHODS
Retrospective case study.


METHODS
Twenty-two-bed multidisciplinary pediatric intensive care unit in a tertiary university hospital, national referral center for pediatric liver transplantation.


METHODS
Between March 1986 and December 1999, seven children, aged between nine and 16 yrs, were admitted in our unit for fulminant Wilson's disease. During the same period of time, aged-matched analysis identified 12 other children with fulminant hepatic failure of other etiologies.


METHODS
None.


RESULTS
On admission, pediatric risk of mortality score, coagulation variables, and hepatic encephalopathy grade were similar in both groups. Children with fulminant Wilson's disease (n = 7) were characterized by lowered hemoglobin and serum transaminases, elevated serum bilirubin, and the occurrence of Kayser-Fleischer rings. Although some admission variables were significantly different between both groups, the elevated variability did not allow us to define acceptable cutoff values. Receiver operating characteristics curve for alkaline phosphatase to total bilirubin ratio showed a good discriminative power (Az = 0.87) in differentiating fulminant Wilson's disease from fulminant hepatic failure of other causes, and a ratio <1 showed a 86% sensitivity and 50% specificity (odds ratio, 6; 95% confidence interval, 66-0.5; p =.173) for fulminant Wilson's disease diagnosis.


CONCLUSIONS
Although requiring prospective study to confirm, Kayser-Fleischer rings and serum alkaline phosphatase to total bilirubin ratio may assist in the early diagnosis of fulminant Wilson's disease.
Tissières
Pierre
P
Unité de Soins Intensifs, Département de Pédiatrie, Hôpital de Bicêtre, 94275 Le Kremlin-Bicêtre, France.
Chevret
Laurent
L
Debray
Dominique
D
Devictor
Denis
D
D000293:Adolescent; D000469:Alkaline Phosphatase / Q000097:blood; D001663:Bilirubin / Q000097:blood; D002648:Child; D004796:Clinical Enzyme Tests; D016001:Confidence Intervals; D005260:Female; D006501:Hepatic Encephalopathy / Q000097:blood / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D006801:Humans; D015278:Intensive Care Units, Pediatric; D017093:Liver Failure / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D012017:Referral and Consultation; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D013997:Time Factors; D016896:Treatment Outcome
D000469:Alkaline Phosphatase; D001663:Bilirubin