Glycogenic hepatopathy (GH) is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal. Periodic acid Schiff stain for glycogen was positive in these hepatocytes. A diagnosis of glycogenic hepatopathy was made after clinicopathological correlation. We describe a presentation of glycogenic hepatopathy in a poorly controlled type I diabetic patient. As patients with glycogenic hepatopathy.

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Hyperglycemia and hyperinsulinemia are thought to glycogeniv the etiology for hepatic glycogen deposition in dumping syndrome. Auto immune hepatitis; DM: Histology of GH reveals pale appearance of hepatocytes with compression of sinusoids, glycogenated nuclei, giant mitochondria, and positive periodic acid-Schiff stained intracytoplasmic inclusions that disappear after digestion with diastase [ 1 — 36 ].

However, liver synthetic functions were usually preserved. All biopsies showed diffusely pale staining hepatocytes on hematoxylin and eosin stains, with excessive glycogen accumulation demonstrated by periodic acid-Schiff stains. Excessive glycogen with swollen hepatocytes eventually could cause sinusoidal compression and subsequent ascites[ 34 ]; therefore, ascites can also be a part of the clinical presentation, albeit rarely[ 3444 ]. It develops due to excessive accumulation of glycogen in the hepatocytes.

Glycogenic hepatopathy GH is considered as a benign glycogenjc condition. Future studies warranted to further assess for development of fibrosis in GH as it can progress to cirrhosis. We conclude that glycogenic hepatopathy can cause hepatomegaly and significant transaminase elevations in individuals with type I diabetes mellitus.

In conclusion, Although it is a well-known complication of poorly controlled type 1 DM, GH is still an underdiagnosed cause of elevated transaminase levels and hepatomegaly in these patients.


Glycogenic hepatopathy: A narrative review

Summary of the major case reports in English PubMed indexed on Glycogenic hepatopathy in type 2 diabetes mellitus. It was recorded that the liver enzymes had great fluctuations within the past 3 years, he was hospitalized due to hyperglycemia or ketoacidosis, and his liver enzymes declined when his blood sugar was regulated.

View at Google Scholar. Dumping syndrome, a cause of acquired glycogenic hepatopathy. Hepatic glycogen deposition has been described in several experimental models. Thinking Outside the Box.

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Despite showing a benign clinical course with strict glycemic control, GH could recur hhepatopathy relapse with uncontrolled glycemic levels[ 38 ]. Burda P, Hochuli M.

GSD is a group of inherited disorders in which excess glycogen accumulates in different tissues including liver, skeletal muscles, or both due to the deficiency of enzymes that regulate glycogenolysis or gluconeogenesis.

However, other possible etiologies for increasing hepatic attenuation on unenhanced CT include conditions where radiodense material is deposited in the liver, such as iodine in patients taking amiodarone and iron in patients with hemochromatosis [ 9 ]. Biochemical features Most patients with GH present with hepatocellular abnormality with a predominant elevation in aspartate transaminase AST and alanine transaminase ALT levels, although a mixed or predominantly cholestatic pattern can rarely occur[ 311203044 ].

Tight glycemic control via intensive insulin therapy provides full remission of clinical, laboratory, and histological abnormalities [ 4 ].

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

ALP, Total bilirubin may be elevated. Journal List World J Hepatol v. The extreme elevation over 10 times the normal values of the liver enzyme levels of our case, who was a type 1 diabetic, was a trigger for investigating secondary causes. Additionally, antinuclear antibody was positive in some cases, showing a mostly homogeneous speckled pattern.


Three patients had adequate follow-up and demonstrated improvement of liver enzyme levels with control of blood glucose. Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus.

Liver synthetic functions are typically preserved. Dual-Echo MRI; demonstrates decreased signal intensity in the affected tissues on the in-phase images compared with the out of- phase images opposite of steatosis.

He had a past medical history of T1DM and bipolar disorder that was being treated with the antipsychotic drugs paliperidone and asenapine.

Glycogenic hepatopathy: A narrative review

Martin J, Tomlinson P. While first described in association with recurrent hyper- and hypoglycemia with ketoacidosis in T1DM, it has since been reported without ketosis or acidosis in patients with T2DM and with variable insulin requirements[ 348 – 1642 ]. Glycogen-induced hepatomegaly glycogenlc type 1 DM was described first by Mauriac inas a component of a syndrome characterized by hepatomegaly, hepatopathj retardation, cushingoid appearance, and delayed puberty.

Chatila R, West AB.

Hepatic glycogenosis is thought to be an adaptive response that protects against potentially fatal hypoglycemia in malnutrition. Because there is an association between T1DM and autoimmune hepatitis, testing for autoimmune antibodies, such as ANA, anti-smooth muscle antibody and antimitochondrial antibody is essential.