Liver glycogen storage associated with uncontrolled type 1 diabetes mellitus

Torres, Miquel; López, Dolores

doi:10.1016/s0168-8278(01)00132-5

Cited by 20 publications

(17 citation statements)

References 1 publication

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“…As mentioned above, especially in case of mild transaminases elevation in diabetic patients, liver biopsy is essential in differential diagnosis of GH with NAFLD. In addition, US finding is not helpful in distinguishing NAFLD from GH because both fatty liver and glycogen overload could show similar US finding as mild hyperechoic hepatic parenchyma 3,6,10,11. Consequently, the condition is often misdiagnosed and it result in wasting medical resources.…”

Section: Discussionmentioning

confidence: 99%

Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus

Hwang

Park

et al. 2013

Clin Mol Hepatol

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Glycogenic hepatopathy (GH) is an uncommon cause of serum transaminase elevation in type I diabetes mellitus (DM). The clinical signs and symptoms of GH are nonspecific, and include abdominal discomfort, mild hepatomegaly, and transaminase elevation. In this report we describe three cases of patients presenting serum transaminase elevation and hepatomegaly with a history of poorly controlled type I DM. All of the cases showed sudden elevation of transaminase to more than 30 times the upper normal range (like in acute hepatitis) followed by sustained fluctuation (like in relapsing hepatitis). However, the patients did not show any symptom or sign of acute hepatitis. We therefore performed a liver biopsy to confirm the cause of liver enzyme elevation, which revealed GH. Clinicians should be aware of GH so as to prevent diagnostic delay and misdiagnosis, and have sufficient insight into GH; this will be aided by the present report of three cases along with a literature review.

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Section: Discussionmentioning

confidence: 99%

Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus

Hwang

Park

et al. 2013

Clin Mol Hepatol

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“…It has since been recognized that glycogen loading and hepatic disease may be present in the absence of the extrahepatic manifestations described earlier by Mauriac (2). This process has variably been referred to as hepatic glycogenosis (3), liver glycogenosis (4), liver glycogen storage (5,6), diabetes mellitus-associated glycogen storage hepatomegaly (7), and most recently glycogenic hepatopathy (8).…”

Section: Discussionmentioning

confidence: 99%

“…This is a reflection of the rarity of GH coupled with the common occurrence of fatty liver disease; a patient with hepatomegaly and elevated transaminases is therefore assumed to have fatty liver unless a biopsy is obtained. Furthermore, ultrasound does not distinguish fatty liver from glycogen overload (3)(4)(5)8).…”

Section: Discussionmentioning

confidence: 99%

Complete Reversal of Glycogen Hepatopathy With Pancreas Transplantation: Two Cases

et al. 2007

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Glycogen hepatopathy is the pathological overloading of hepatocytes with glycogen that is associated with poorly controlled diabetes mellitus. Clinically, it presents with abdominal discomfort, tender hepatomegaly and elevated transaminases. In this report, we describe our experience with two cases of type I diabetes mellitus and glycogen hepatopathy. The patients underwent isolated pancreas transplantation, following which, we have been able to demonstrate complete histological resolution of glycogen hepatopathy associated with control of glucose metabolism.

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“…Generally, it is reversible and caused by excessive glycogen deposition. Over the years, this abnormality has been referred to as hepatic glycogenosis, liver glycogen storage, liver glycogenosis, and DM‐associated glycogen storage hepatomegaly and was then termed GH by Torbenson et al…”

Section: Introductionmentioning

confidence: 99%

“…The cause of GH has been reported as type 1 DM in 95% and type 2 DM in 5% of cases . In uncontrolled diabetes, the frequent hypoglycemic episodes and subsequent glucose administration are potential exacerbating factors in GH .…”

Section: Introductionmentioning

confidence: 99%

Four cases of type 1 diabetes mellitus showing sharp serum transaminase increases and hepatomegaly due to glycogenic hepatopathy

Ikarashi

Kogiso

Hashimoto

et al. 2016

Hepatology Research

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Poorly controlled diabetes mellitus (DM) patients sometimes show serum transaminase elevations due to steatohepatitis. However, we experienced four cases with type 1 DM with sharp elevations in serum transaminases that could not be explained by steatohepatitis alone and showed bright liver. They were diagnosed with glycogenic hepatopathy (GH) clinicopathologically. The four patients had a median age of 22.5 years (range, 19-29 years) and 12.5 (4-15)-year histories of type 1 DM and showed marked increases in serum transaminases. Diabetes mellitus control was poor and hemoglobin A1c was 12.7% (11-16.5%). Three cases had a past history of diabetic ketoacidosis. Hepatomegaly and hyperdense liver were seen on computed tomography scans. Magnetic resonance imaging showed low intensity in T2-weighted images. The pathological findings revealed pale and swollen hepatocytes and glycogenated nuclei. The architecture of the liver was preserved, and steatosis and fibrosis were mild. The cytoplasm of hepatocytes stained densely positive with periodic acid-Schiff, and the positive staining disappeared after diastase digestion, suggesting glycogen deposition. No other cause of hepatitis was evident, and the diagnosis was GH. Elevated transaminases improved within 1 month with good glycemic control. Transaminase elevations were observed several times in three cases with poor glycemic control. Glycogenic hepatopathy is rare, but extremely high serum elevations of transaminases are important to identify clinically. Despite showing a good clinical course in general, GH sometimes recurs and requires strict glycemic control. Clinicians should be aware of and recognize GH when dealing with uncontrolled DM patients.

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Liver glycogen storage associated with uncontrolled type 1 diabetes mellitus

Cited by 20 publications

References 1 publication

Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus

Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus

Complete Reversal of Glycogen Hepatopathy With Pancreas Transplantation: Two Cases

Four cases of type 1 diabetes mellitus showing sharp serum transaminase increases and hepatomegaly due to glycogenic hepatopathy

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