Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2018 Apr 15;57(8):1087-1092.
doi: 10.2169/internalmedicine.9490-17. Epub 2017 Dec 27.

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

Affiliations
Case Reports

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

Shohei Asada et al. Intern Med. .

Abstract

Glycogenic hepatopathy (GH) is a rare complication of poorly controlled type 1 diabetes mellitus (T1DM), and is characterized by elevated liver enzymes, hepatomegaly, and glycogen accumulation. We herein present the case of a 23-year-old man with poorly controlled T1DM who had liver dysfunction. Imaging studies showed severe hepatomegaly and fatty liver. The examination of a liver biopsy specimen revealed fatty droplets, ballooning, inflammation, and mild fibrosis. Subsequent periodic acid-Schiff (PAS) staining after diastase digestion confirmed GH. In this case, the improvement of hyperglycemia, not HbA1c, resulted in the improvement of the patient's liver function. This is the first report on the use of continuous glucose monitoring in patients with GH to show that continuous hyperglycemia may worsen GH.

Keywords: glycogenic hepatopathy; hepatomegaly; periodic acid-Schiff staining; poorly controlled type 1 diabetes mellitus.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
(A, B) Ultrasound images showing severe hepatomegaly and fatty liver.
Figure 2.
Figure 2.
(A, B) Contrast-enhanced computed tomography scans showing severe hepatomegaly and fatty liver. (C, D) Computed tomography scans after the improvement of the liver function, showing the improvement of hepatomegaly and fatty liver.
Figure 3.
Figure 3.
Staining of the liver biopsy specimen. (A) Slight inflammation in the portal area, and mild intralobular inflammation [Hematoxylin and Eosin (H&E) staining, magnification ×40]. (B) Slight centrilobular ballooning of the hepatocytes (H&E staining, magnification ×100). (C) Mild pericellular fibrosis (azan staining, magnification ×40).
Figure 4.
Figure 4.
Periodic acid-Schiff (PAS) staining of the liver biopsy specimen. (A) Many PAS-positive granules were observed within the cytoplasm of the hepatocytes (magnification ×40). (B) The disappearance of PAS-positive granules after diastase digestion (magnification ×40).
Figure 5.
Figure 5.
Changes of AST, ALT, and GGT.

Comment in

References

    1. Mauriac P. Gros ventre, hepatomegaly, troubles de las croissance chez les enfants diabetiques traits depuis plusieurs annes par l'insuline. Gax Hebd Med Bordeaux 26: 402-410, 1930.
    1. El-Karaksy HM, Anwar G, Esmat G, et al. . Prevalence of hepatic abnormalities in a cohort of Egyptian children with type 1 diabetes mellitus. Pediatr Diabetes 11: 462-470, 2010. - PubMed
    1. Parmar N, Atiq M, Austin L, et al. . Glycogenic hepatopathy: thinking outside the box. Case Rep Gastroenterol 9: 221-226, 2015. - PMC - PubMed
    1. van den Brand M, Elving LD, Drenth JP, et al. . Glycogenic hepatopathy: a rare cause of elevated serum transaminases in diabetes mellitus. Neth J Med 67: 394-396, 2009. - PubMed
    1. Jeong HR, Shim YS, Kim YB, et al. . Glycogenic hepatopathy in a Korean girl with poorly controlled type 1 diabetes mellitus. Ann Pediatr Endocrinol Metab 19: 49-52, 2014. - PMC - PubMed

Publication types