Γ-glutamyl transpeptidase level as a screening marker among diverse etiologies of infantile intrahepatic cholestasis
Journal
Journal of Pediatric Gastroenterology and Nutrition
Journal Volume
59
Journal Issue
6
Pages
695-701
Date Issued
2014
Author(s)
Abstract
Objectives: Low γ-glutamyl transpeptidase (GGT) level is an important marker for progressive familial intrahepatic cholestasis, yet the cutoff level and clinical application is not well defined. This study aimed to evaluate the role of GGT as a screening marker among diverse etiologies of infantile cholestasis.Methods: This retrospective study analyzed 256 cholestatic infants admitted to a tertiary referral center between 2000 and 2012. After excluding 121 infants of extrahepatic cholestasis, advanced investigations for 135 infants with intrahepatic cholestasis were performed. The etiologies, outcomes, and correlations with GGT levels were analyzed. Good prognosis was defined as clinical recovery before 1 year of age; poor prognosis as persistent disease, liver transplantation, or death before 1 year.Results: Among 135 patients of intrahepatic cholestasis, >12 different etiologies were found. Neonatal hepatitis (49.6%), progressive familial intrahepatic cholestasis (21.5%), and neonatal cholestasis caused by citrin deficiency (10.4%) were the leading causes. Patientswith initialGGT between 75 and 300 U/L had a higher chance of good prognosis (61/74, 82.4%) than those with GGT <75 U/L or >300 U/L (25/61, 41%, P<0.0001). In the low- GGT group (?100U/L), 52.6% (30/57) of the patients have good prognosis; and GGT level ?75U/L has a sensitivity, specificity, and positive predictive value of 100%, 43.3%, and 61.4% in predicting poor prognosis.Conclusions: Patients with GGT levels ?75 or ?300 U/L should receive advanced investigations such as genetic/metabolic assays early; otherwise, the amount of diagnostic workupmay be limited if no signs of progressive disease. Copyright ? 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
Other Subjects
biochemical marker; citrinin; gamma glutamyltransferase; biological marker; gamma glutamyltransferase; Article; cholestasis; death; diagnostic test accuracy study; disease course; enzyme blood level; extrahepatic bile duct obstruction; familial disease; heredity; hospital admission; human; human tissue; infant; infant disease; intrahepatic cholestasis; liver transplantation; major clinical study; medical record review; molecular diagnosis; morbidity; newborn hepatitis; outcome assessment; predictive value; prognosis; progressive familial intrahepatic cholestasis,; retrospective study; screening; tertiary care center; blood; Cholestasis, Intrahepatic; epidemiology; female; genetics; male; mutation; newborn; sensitivity and specificity; Taiwan; Biological Markers; Cholestasis, Intrahepatic; Female; gamma-Glutamyltransferase; Humans; Infant; Infant, Newborn; Liver Transplantation; Male; Mutation; Prognosis; Retrospective Studies; Sensitivity and Specificity; Taiwan
Publisher
Lippincott Williams and Wilkins
Type
journal article