OUTCOME OF IDIOPATHIC ACUTE LIVER FAILURE IN CHILDREN: 15 YEARS, SINGLE CENTRE EXPERIENCE

Abstract

Acute liver failure (ALF) in children is uncommon but carries high mortality without liver transplantation. The outcome also depends on the aetiology, however in many children with ALF the aetiology remains unknown. Aim: To review clinical and biochemical characteristics in children with idiopathic ALF and identify factors that could determine outcome. Method: Medical records of 204 children presenting with ALF between 1989-Sept 2004 were reviewed. Of these 61 (32 male, age 4.6yrs [12 days-17.5 yrs]) had idiopathic ALF. All the known causes of ALF were excluded. ALF was defined as either INR > 2 and abnormal liver function or INR > 1.5 with encephalopathy and abnormal liver function tests. Known causes of ALF were: drug induced in 44 (31%), metabolic in 27 (19%), infectious in 21 (15%), neonatal haemochromatosis in 14 (10%), autoimmune in 13 (9%) and miscellaneous in 24 (17%). Results: Presenting features were jaundice in 53 (87%); hepatomegaly in 46 (75%); encephalopathy in 37 (61%). Grade of encephalopathy was >II in 25 (41%), with invasive ICP monitoring in 17 (28%). Forty-three (71%) required PICU admission. Laboratory indices on admission: median AST, 1355 [range 64-12860] iu/l; SBR, 368 [12-1018] umol/l; GGT, 43 [19-175] iu/l; albumin, 32 [13-49] g/l; INR 3.07 [1.28-15]. Median length of hospital stay was 11 [1-166] days. Eighteen (30%) survived with their own liver. Seven (12%) died without transplantation 4 [1-75] days from admission. Thirty-six (59%) received a liver transplant. Eleven (31%) children required re-transplantation due to: chronic rejection in 5, biliary complications in 3, primary non-function in 1, reoccurence of disease in 1, HAT in 1. Five of them required a 3rd transplant. Thirteen (36%) died after transplantion due to multi-organ failure and sepsis in 8, primary non-function in 4, chronic rejection in 1. Factors related to survival without transplantation were no hepatomegaly (p = 0.05) or encephalopathy (p = 0.03). Conclusion: Idiopathic ALF accounts for a third of ALF children and carries high mortality despite transplantation.