Abstract
Objectives:To determine etiologies, clinical presentations and outcomes of children with fulminant hepatic failure in the first liver transplant center of Pakistan.Methods:It was a retrospective, observational study, conducted in Paediatric Gastroenterology Department of Shifa International Hospital. Patients between one month to 16 years were included who fulfilled the Pediatric Acute Liver Failure study group (PALFSG) definition of acute liver failure as biochemical evidence of liver injury with no known co-existing chronic liver disease, coagulopathy not corrected by vitamin K, an International Normalized Ratio (INR) greater than 1.5 if the patient has encephalopathy, or greater than 2.0 if the patient does not have encephalopathy. The data collected was recorded on a self-constructed proforma after IRB approval.Results:There were 28 patients in the study which ncluded 17 males and 11 females with a mean age of 72.86±52.50 months. The most common etiologies were Hepatitis A (29%) in isolation or co-infection with Wilson Disease, typhoid fever. It was followed by seronegative hepatitis (29%). Majority (64%) had acute presentation (7 to 28 days), jaundice (82%) being the most common symptom. Severity of encephalopathy was significantly associated with outcome (p=0.02). There were 6 (21%) patients who succumbed to death.Conclusions:The study highlights infective diseases as the predominant etiology causing fulminant liver failure in children. Our study highlights lower mortality in children
Highlights
Fulminant hepatic failure refers to a highly specific and fatal albeit rare clinical syndrome, manifesting as various abnormalities in liver function tests in a patient without pre-existing hepatic pathology
Other causes leading to fulminant hepatic failure include seronegative hepatitis, metabolic liver diseases, autoimmune hepatitis, hemophagocytic lymphohistiocytosis and sepsis
Pediatric Acute Liver Failure study groups (PALFSG) consensus definition of acute liver failure in children was used as inclusion criteria which states biochemical injury of liver injury with no known co-existing chronic liver disease, coagulopathy not corrected by vitamin K, an International Normalized Ratio (INR) greater than 1.5 if the patient has encephalopathy, or greater than 2.0 if the patient does not have encephalopathy.[10]
Summary
Fulminant hepatic failure refers to a highly specific and fatal albeit rare clinical syndrome, manifesting as various abnormalities in liver function tests in a patient without pre-existing hepatic pathology. The deterioration in liver function is associated with coagulopathy and altered level of consciousness due to hepatic encephalopathy.[1] owing to the difficulty in the assessment of hepatic encephalopathy in young children and infants, presence of signs of encephalopathy is not a part of diagnostic criteria in children as opposed to adults.[2,3] There is wide variation in the etiology leading to fulminant. Pak J Med Sci September - October 2020 Vol 36 No 6 www.pjms.org.pk 1252 hepatic failure depending on the age group and geographical location, with the acute viral hepatitis[4] and drugs among the leading causes.[5] Other causes leading to fulminant hepatic failure include seronegative hepatitis, metabolic liver diseases, autoimmune hepatitis, hemophagocytic lymphohistiocytosis and sepsis. Mortality associated with this devastating event is reported to be between 506 and 70 percent.[7]
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