https://www.ncbi.nlm.nih.gov/pubmed/30028785 Chronic liver disease

Transplantation. 2018 Jul 19. doi: 10.1097/TP.0000000000002367. [Epub ahead of print].Transplant-free Survival in Chronic Liver Disease Presenting as Acute Liver Failure in Childhood.Di Giorgio A1, Nicastro E1, Rosa DD1, Nebbia G2, Sonzogni A3, D'Antiga L1.

Abstract

BACKGROUND:
in adults, the absence of a preexisting chronic liver disease (CLD) is required to diagnose acute liver failure (ALF). The paediatric classification does not considered this aspect, thus previous studies pooled together children with ALF and children with unknown CLD presenting with acute hepatic decompensation (ALF-CLD). We aimed to compare prevalence, features and outcome of children with ALF-CLD to those with a proper ALF.

METHODS:
Patients admitted between 1996-2017 because of ALF defined by PALF criteria (raised transaminases, INR ≥2.0, no history of liver disease) were classified as ALF-CLD if diagnosed with autoimmune hepatitis (AIH), Wilson disease (WD), Budd-Chiari syndrome, HBV reactivation, inborn errors of metabolism (IEM). The others were classified as ALF.

RESULTS:
74 children [median age 4 years, 1.0-8.8, M/F =36/38] with ALF were found ; 18 of <1 year of age were excluded. Fifty-six [median age 6.6 years (2.7-11.7), M/F = 23/33], 22 with ALF-CLD (AIH n=14, WD n=6, IEM n=2) and 34 with ALF (paracetamol overdose n=6, viral infections n=3, mushroom poisoning n=5, indeterminate n=20) were compared. In ALF-CLD the median age at onset was higher, ALT, albumin and INR levels were lower, splenomegaly, ascites and cirrhosis were more common (all p<0.01). On multivariate analysis the diagnosis of ALF-CLD was an independent predictor of transplant free survival (p = 0.006).

CONCLUSIONS:
In children ALF-CLD is common, has peculiar features and is associated with a favourable outcome. This study suggests the need to distinguish this entity from other forms of ALF in children.