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.
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.
Department of Metabolism/Diabetes and Clinical Nutrition, Nagasaki University Hospital, Japan.
Fumi
F
Murata
Ichiro
I
Horie
Takao
T
Ando
Eriko
E
Isomoto
Hideyuki
H
Hayashi
Satoru
S
Akazawa
Ikuko
I
Ueki
Kan
K
Nakamura
Masakazu
M
Kobayashi
Hironaga
H
Kuwahara
Norio
N
Abiru
Eiji
E
Kawasaki
Hironori
H
Yamasaki
Atsushi
A
Kawakami
D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging; D016883:Diabetic Ketoacidosis / Q000150:complications; D006801:Humans; D007333:Insulin Resistance / Q000502:physiology; D008107:Liver Diseases / Q000175:diagnosis* / Q000209:etiology; D008112:Liver Glycogen; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography; D055815:Young Adult
D008112:Liver Glycogen