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Fatal deterioration of neurological disease after orthotopic liver transplantation for valproic acid‐induced liver damage
Author(s) -
Kayihan Nilüfer,
Nennesmo Inger,
Ericzon BoGöran,
Németh Antal
Publication year - 2000
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1034/j.1399-3046.2000.00115.x
Subject(s) - medicine , valproic acid , fulminant , fulminant hepatic failure , liver transplantation , pathological , transplantation , liver disease , chronic liver disease , pathology , gastroenterology , cirrhosis , epilepsy , psychiatry
We describe a 12‐year‐old girl with an early onset neurologic disease of slow progressiveness and electro‐encephalography showing epileptic activity. The girl developed fulminant liver failure 5 months after the start of valproic acid treatment. Repeated mitochondrial assays failed to prove a mitochondrial disorder, but muscle biopsies were slightly pathological. Liver histology indicated acute‐on‐chronic liver disease. Six weeks after a successful orthotopic liver transplantation her neurological condition deteriorated rapidly, soon leading to generalized cortical disease and death. Post‐mortem brain examination showed advanced central nervous destruction. We suggest that this is a late‐onset Huttenlocher variant of Alpers’ syndrome, where fulminant liver failure can be triggered by valproic acid, and orthotopic liver transplantation can subsequently trigger a fatal neurologic deterioration. Our case illustrates that when a referral center receives a previously unknown patient with hepatocellular insufficiency, it might be impossible to differentiate between fulminant vs. acute‐on‐chronic liver failure, and the decision whether to perform a liver transplantation or not would become difficult.