Transplant-free Survival in Chronic Liver Disease Presenting as Acute Liver Failure in Childhood

Autor: Lorenzo D'Antiga, Aurelio Sonzogni, Gabriella Nebbia, Davide Dalla Rosa, Emanuele Nicastro, Angelo Di Giorgio
Rok vydání: 2019
Předmět:
Male
congenital
hereditary
and neonatal diseases and abnormalities
medicine.medical_specialty
medicine.medical_treatment
Kaplan-Meier Estimate
030230 surgery
Liver transplantation
Chronic liver disease
Gastroenterology
End Stage Liver Disease
03 medical and health sciences
0302 clinical medicine
Hepatolenticular Degeneration
Internal medicine
medicine
Humans
Child
Hepatic encephalopathy
Survival analysis
Acetaminophen
Hepatitis
Transplantation
business.industry
Acetaminophen poisoning
Graft Survival
digestive
oral
and skin physiology
Liver failure
Infant
Liver Failure
Acute
respiratory system
medicine.disease
Survival Analysis
Liver Transplantation
respiratory tract diseases
Transplant free survival
Hepatitis
Autoimmune
Treatment Outcome
Child
Preschool
Hepatic Encephalopathy
Female
030211 gastroenterology & hepatology
business
Liver Failure
Metabolism
Inborn Errors
Zdroj: Transplantation. 103:544-551
ISSN: 0041-1337
DOI: 10.1097/tp.0000000000002367
Popis: In adults, the absence of a preexisting chronic liver disease (CLD) is required to diagnose acute liver failure (ALF). The pediatric classification does not consider 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.Patients admitted between 1996 and 2017 because of ALF defined by Pediatric Acute Liver Failure criteria (raised transaminases, International Normal Ratio ≥2.0, no history of liver disease) were classified as ALF-CLD if diagnosed with autoimmune hepatitis, Wilson disease, Budd-Chiari syndrome, hepatitis B virus reactivation, inborn errors of metabolism. The others were classified as ALF.Seventy-four children (median age, 4 years; 1.0-8.8; male/female, 36/38] with ALF were found; 18 of1 year of age were excluded. Fifty-six (median age, 6.6 years; 2.7-11.7; male/female, 23/33], 22 with ALF-CLD (autoimmune hepatitis, n = 14; Wilson disease, n = 6; inborn errors of metabolism, 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, alanine aminotransferase, albumin, and International Normal Ratio levels were lower, splenomegaly, ascites, and cirrhosis were more common (all P0.01). On multivariate analysis, the diagnosis of ALF-CLD was an independent predictor of transplant-free survival (P = 0.006).In children, ALF-CLD is common, has peculiar features, and is associated with a favorable outcome. This study suggests the need to distinguish this entity from other forms of ALF in children.
Databáze: OpenAIRE
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