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Kallas, M. R.E.; Cheeseman, P.; Bhaduri, B.**; Heaton, N.*; Rela, M.*; Mieli-Vergani, G.
Depts of Child Health, Transplant Surgery*, King's College Hospital, London. **Maidstone Hospital, Kent,UK
Acute liver failure in childhood is associated with > 90% mortality if INR is ≥4, unless liver transplant (LT) is performed. Prognosis is particularly severe in younger children. To assess etiology and prognosis of acute liver failure in infancy, we have reviewed retrospectively the case notes of 57 infants referred to our Unit between 1983-96. Acute liver failure was diagnosed on the basis of submassive liver cell necrosis and coagulopathy (INR >2), with or without encephalopathy, which may be absent or a late event in this age group. Age was <1 month in 22, 1 month-1 year in 25, 1-2 years in 10. Etiology was haemophagocytic lymphohistiocytosis (HLH) in 10, cryptogenic in 16, metabolic in 16 (10 neonatal haemocromatosis, 3 tyrosinaemia, 1 ornithine transcarbamylase deficiency, 1 fatty acid oxidation defect, 1 mitochrondial defect), toxic in 4 (1 Amanita phalloides, 1 paracetamol, 1 isoniazid, 1 copper), infective in 8 (4 post septic shock, 2 hepatitis B, 1 Herpes simplex Type II, 1 Herpes simplex Type I), autoimmune in 3 (all liver-kidney microsomal positive). Maximum INR was ≥4 in 45. Overall 13 of 57 patients (23%) survived, 9 after LT, 4 patients without LT; 1 tyrosinaemia treated with NTBC, 1 HLH, 1 fatty acid oxidation defect, and 1 with Amanita phalloides. LT was not indicated in 10 with HLH and was not available for 10 patients referred before 1990. Of the remaining 37 patients 9 of 19 (47%) transplanted survived compared to 3 of 18 (16.6%) without LT (p<0.02). All the transplanted survivors are well (median(range) age at LT: 4.3 months(16 days - 1.99 years); follow up: 1.4 years (4 months-5.3 years). In conclusion: a) acute liver failure in infancy has a poor prognosis; b) is associated with a particularly severe coagulopathy; c) is often caused by HLH; d) is treatable by LT even in very young infants, but the 47% survival after LT is considerably less satisfactory than reported survival in older children with fulminant hepatic failure (60%).
© Lippincott-Raven Publishers
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