Study of Carnitine/Acylcarnitine and Aminoacid Profile in children and adults with Acute Liver Failure.

Objectives: Fatty acid oxidation defects (FAODs) may underlie or modify the course of acute liver failure (ALF). Overall significance of carnitine/acylcarnitine and aminoacid profile in ALF is similarly undetermined. Thus, this study was undertaken to study the abnormalities in carnitine/acylcarnitine and aminoacid profile in ALF. Patients and Methods: A prospective study was performed including all cases of ALF and detailed evaluation including metabolic testing was done. Results: A total of 55 patients (33 pediatric and 22 adult cases) were included in the study. Three patients (a 1 year 6-month-old child, a 13 year old adolescent and a 21 year old adult) i.e 5.5% of all, were detected to be having underlying metabolic etiology i.e Carnitine Palmitoyl Transferase-1 or CPT-1 deficiency based on abnormal carnitine/acylcarnitine profile. Almost three-fourths of patients (78%) had evidence of serum hyperaminoacidemia. Thirty-one patients (56%) had evidence of abnormal carnitine/acylcarnitine profile with predominant abnormality being low free carnitine (C0). Higher levels of serum tyrosine (p 0.002) and lower levels of serum C0 (p 0.032) in children and higher levels of serum phenyalanine (p 0.047) in adults predicted poor outcome (death/liver transplant) on univariate analysis. Conclusion: FAODs are not uncommon in ALF with a suggested prevalence of around 5.5%. FAODs can cause ALF or modify the natural course of ALF caused by other etiologies. Serum hyperaminoacidemia and low serum free carnitine may predict poor outcome in ALF cases. (C) 2016 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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