Characteristics, clinical laboratory, histopathology, and outcomes of glycogenic hepatopathy in children

TL;DR

This study examines the features and outcomes of glycogenic hepatopathy in children with type 1 diabetes, showing it resolves with modest glycemic control.

Contribution

The study provides detailed clinical and histopathological insights into glycogenic hepatopathy in pediatric patients with type 1 diabetes.

Findings

GH occurs after years of diabetes and recurrent diabetic ketoacidosis in teenagers.

Liver biopsy shows hepatocyte swelling, minimal fibrosis, and steatosis without steatohepatitis.

Elevated aminotransferases, especially AST, improve with modest glycemic control.

Abstract

Glycogenic hepatopathy (GH) is a rare complication of type I diabetes mellitus (DM1), resulting in abnormal deposition of glycogen in the liver due to poor glycemic control. Clinical characteristics and natural history of GH are not completely understood in children. In this study, we investigated clinical, biochemical, histologic parameters and outcomes in children with GH.

This was a retrospective review of patients less than 18 years old diagnosed with GH and DM. GH was confirmed on liver biopsy. Medical records were reviewed for clinical presentation, laboratory tests, and clinical outcomes. Liver biopsy findings were reviewed by a pediatric pathologist (I. A. G.).

Nine children were diagnosed with GH and type 1 DM. The median age at diagnosis of GH was 16 (IQR 14.5−17) years. Duration of diagnosis of DM until GH diagnosis was 7 (IQR 5−11) years. The median frequency of diabetic ketoacidosis before GH diagnosis was three times (IQR 2−5.25). Peak Aspartate transaminase (AST) and Alanine transaminase (ALT) ranged from 115 to 797, and 83−389 units/L, respectively. Only two children had mild fibrosis. Seven of nine had steatosis without steatohepatitis. There was no correlation between glycosylated hemoglobin (HbA1c), or other laboratory tests and liver fibrosis on biopsy. HbA1c was 11.2 (IQR 10.2−12.8) at GH diagnosis and 9.8 (IQR 9.5−10.8) with normalization of liver enzymes.

GH appears to be related to poor glycemic control in teenagers with long‐term diabetes. GH presents with high to very high aminotransferase especially AST > ALT and resolves with modestly improved glycemic control. Diffuse hepatocyte swelling, steatosis, minimal fibrosis without hepatocyte ballooning or lobular inflammation are most common histological features.

Glycogenic hepatopathy (GH) is a rare complication of poorly controlled diabetes mellitus (DM) characterized by elevated aminotransferases and hepatomegaly.Elevated aminotransferases improve drastically after glycemic control in diabetes.

Glycogenic hepatopathy (GH) is a rare complication of poorly controlled diabetes mellitus (DM) characterized by elevated aminotransferases and hepatomegaly.

Elevated aminotransferases improve drastically after glycemic control in diabetes.

GH occurs after years of diabetes (7, IQR 5−11 years) and recurrent diabetic ketoacidosis in teenagers.Liver biopsy in GH can show hepatocyte swelling with minimal liver fibrosis and steatosis without steatohepatitis.GH tends to have aspartate transaminase‐predominant elevation of aminotransferase.GH resolves with only very mild improvement in glycosylated hemoglobin, indicating continued poor glycemic control.

GH occurs after years of diabetes (7, IQR 5−11 years) and recurrent diabetic ketoacidosis in teenagers.

Liver biopsy in GH can show hepatocyte swelling with minimal liver fibrosis and steatosis without steatohepatitis.

GH tends to have aspartate transaminase‐predominant elevation of aminotransferase.

GH resolves with only very mild improvement in glycosylated hemoglobin, indicating continued poor glycemic control.

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