Etiological and Clinical Spectrum of Acute Liver Failure of Infancy in Pakistan

Abstract

Objective: To describe the aetiology and clinical spectrum of acute liver failure of infancy at a tertiary care hospital

Study Design: Cross-sectional study.

Place and Duration of Study: Department of Paediatric Gastroenterology, Hepatology & Nutrition, Children Hospital and Institute of Child Health, Lahore, from Nov 2020 to May 2021.

Methodology: Infants under 12 months of age were enrolled having liver-based coagulopathy (not corrected after two doses of parenteral vitamin K, 10 mg) with INR > 2, whether encephalopathy was present or not. Encephalo-pathy is difficult to identify in infants, so it was not essential for the diagnosis of ALFI in our study. Infants diagnosed with chronic liver disease at presentation or those without final etiological diagnosis were excluded.

Results: A total of 31 infants were enrolled fulfilling the criteria of acute liver failure of infancy and were studied about aetiology and clinical presentation. The mean age of presentation was 4.64±3.16 months, and males predominated in the study group (64.5%). Common clinical features were in descending order ascites in 29 (93.5%), jaundice in 28 (90.3%), pallor in 24 (77.4%) and peripheral oedema in 21 (67.7%). Metabolic liver diseases were the common cause of ALFI, constituting around(18, 58%) followed by sepsis (9, 29%).Galactosemia (11, 35.5%) stands out among the metabolic causes.

Conclusion: Metabolic disorders followed by sepsis are the most common cause of ALFI.