Successful management of acute liver failure in Italian children: A 16-year experience at a referral centre for paediatric liver transplantation. Issue 10 (October 2017)

• Record Type: Journal Article
• Authors: Di Giorgio, A.; Sonzogni, A.; Piccichè, A.; Alessio, G.; Bonanomi, E.; Colledan, M.; D'Antiga, L.
• Is Part Of: Digestive and liver disease. Volume 49:Issue 10(2017)
• Page Start: 1139
• Page End: 1145
• Publication Date: 2017-10
• Subjects: Acute liver failure -- Children -- Liver transplantation -- Prognostic criteria; Digestive organs -- Diseases -- Periodicals; Liver -- Diseases -- Periodicals; 616.33005
• DOI: 10.1016/j.dld.2017.05.026
• Languages: English
• ISSNs: 1590-8658

Abstract

Abstract: Background: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. Methods: Retrospective review of cases presenting between 1996–2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. Results: 55 children (median age 2.6 years, range 0.1–15.1; M/F = 31/24) had ALF due to autoimmune hepatitis (AIH) in 10 (18%), metabolic disorders in 9 (17%), paracetamol overdose in 6 (11%), mushroom poisoning in 3 (5%), viral infection in 1 (2%), indeterminate in 26 (47%); 25/55 recovered with supportive management (45%); 28/55 underwent LTX and 2 died on the waiting list (55%). On multivariate analysis severity of HE grade 3–4 and bilirubin ≥12 mg/dl were independent predictors of death or LTX (p < 0.05). After a median follow up of 4 years (range 2–15.0 years) the overall survival rate was 93%. Conclusion: Children with ALF can be managed successfully with combined medical treatment and transplantation, warranting a survival rate similar to children transplanted because of chronic conditions. In our cohort of patients severe HE and high bilirubin on admission were independent predictors of the need of LTX.