Enter your Email address:
Wolters Kluwer Health may email you for journal alerts and information, but is committed
to maintaining your privacy and will not share your personal information without
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
Colleague's E-mail is Invalid
Your Name: (optional)
Separate multiple e-mails with a (;).
Thought you might appreciate this item(s) I saw at Journal of Pediatric Gastroenterology and Nutrition.
Send a copy to your email
Your message has been successfully sent to your colleague.
Some error has occurred while processing your request. Please try after some time.
An Existing Folder
A New Folder
The item(s) has been successfully added to "".
Login with your LWW Journals username and password.
Username or Email:
Enter and submit the email address you registered with. An email with instructions to reset your password will be sent to that address.
Link to reset your password has been sent to specified email address.
What does "Remember me" mean?
By checking this box, you'll stay logged in until you logout. You'll get easier access to your articles, collections,
media, and all your other content, even if you close your browser or shut down your
To protect your most sensitive data and activities (like changing your password),
we'll ask you to re-enter your password when you access these services.
What if I'm on a computer that I share with others?
If you're using a public computer or you share this computer with others, we recommend
that you uncheck the "Remember me" box.
Save my selection
Article Level Metrics