A case of glycogenic hepatopathy developed in a patient with new-onset fulminant type 1 diabetes: the role of image modalities in diagnosing hepatic glycogen deposition including gradient-dual-echo MRI

Fumi Murata; Ichiro Horie; Takao Ando; Eriko Isomoto; Hideyuki Hayashi; Satoru Akazawa; Ikuko Ueki; Kan Nakamura; Masakazu Kobayashi; Hironaga Kuwahara; Norio Abiru; Eiji Kawasaki; Hironori Yamasaki; Atsushi Kawakami

doi:10.1507/endocrj.EJ12-0081

ORIGINALS

A case of glycogenic hepatopathy developed in a patient with new-onset fulminant type 1 diabetes: the role of image modalities in diagnosing hepatic glycogen deposition including gradient-dual-echo MRI

Fumi Murata, Ichiro Horie, Takao Ando, Eriko Isomoto, Hideyuki Hayashi, Satoru Akazawa, Ikuko Ueki, Kan Nakamura, Masakazu Kobayashi, Hironaga Kuwahara, Norio Abiru, Eiji Kawasaki, Hironori Yamasaki, Atsushi Kawakami

Author information

Fumi Murata
Department of Metabolism/Diabetes and Clinical Nutrition, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Ichiro Horie
Department of Internal Medicine, National Hospital Organization Saga National Hospital, Saga 849-8577, Japan
Department of Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Takao Ando
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Eriko Isomoto
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Hideyuki Hayashi
Department of Radiology, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Satoru Akazawa
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Ikuko Ueki
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Kan Nakamura
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Masakazu Kobayashi
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Hironaga Kuwahara
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Norio Abiru
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Eiji Kawasaki
Department of Metabolism/Diabetes and Clinical Nutrition, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Hironori Yamasaki
Center for Health and Community Medicine, Nagasaki University, Nagasaki 852-8521, Japan
Atsushi Kawakami
Department of Metabolism/Diabetes and Clinical Nutrition, Nagasaki University Hospital, Nagasaki 852-8501, Japan
Department of Endocrinology and Metabolism, Nagasaki University Hospital, Nagasaki 852-8501, Japan

Keywords: Glycogenic hepatopathy, Glycogen, Fulminant type 1 diabetes, Hepatomegaly, Magnetic resonance imaging (MRI)

JOURNAL FREE ACCESS

2012 Volume 59 Issue 8 Pages 669-676

DOI https://doi.org/10.1507/endocrj.EJ12-0081

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Abstract

Glycogenic hepatopathy (GH) has been reported as a very rare and under recognized complication in long-standing poorly controlled type 1 diabetes (T1D) patients. GH is characterized by transient elevation of liver transaminase and hepatomegaly caused by reversible and excessive glycogen accumulation in hepatocytes. It has been reported that GH is indistinguishable from non-alcoholic fatty liver disease, which is more commonly seen in diabetic patients, even after a history is taken and a physical examination or imaging studies have been performed. GH can only be diagnosed by liver biopsy. We here demonstrate a 21-year-old male patient with new-onset fulminant T1D complicated with diabetic ketoacidosis who subsequently developed GH just after the initiation of insulin treatment. The marked liver dysfunction (serum levels of aspartate aminotransferase 769 IU/L and alanine aminotransferase 1348 IU/L) and hepatomegaly improved spontaneously via glycemic control without any specific treatments thereafter. Moreover, the insulin requirement dramatically decreased from 168 to 80 units per day as GH improved, suggesting a potential role of GH in insulin resistance. GH was diagnosed based on the histological findings of the liver in our case, but we were able to predict GH before the biopsy based on the findings in the gradient-dual-echo magnetic resonance imaging sequence combined with ultrasound and/or computed tomography examinations of the liver.

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