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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Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.

Rarely may require liver transplantation. D-Periodic-Acid Schiff stain remove glycogen leaving empty looking cytoplasm gycogenic arrow and nuclei green arrow. Case reports have not demonstrated success in differentiating glycogenic hepatopathy from NAFLD with ultrasound or other imaging modalities. A study conducted in rats with insulin deficiency has shown that, after a single dose of insulin injection, glycogenesis continues for a significant amount of time after blood glucose levels return to gylcogenic preinjection levels [ 21 ].

Similarly, the case presented here also attained blood glucose regulation, accompanied by reduction in the liver size and significant decreases in ALT and AST levels with intensive insulin therapy. Histopathological section of the liver showing: Several authors have also demonstrated elevated hepatic glycogen concentration in subjects with glycogen storage disease using 13C MRS Magnetic Resonance Spectroscopywhich has the advantage of assessing the entire liver while avoiding the risks of liver biopsy[ 61 ].

Glycogenic hepatopathy: A narrative review

Hepatomegaly and abnormal liver tests due to glycogenosis in adults with diabetes. Abstract We describe a presentation of glycogenic hepatopathy in a poorly controlled type I diabetic patient. Larger-scale and long-term studies are still needed to clarify the incidence and prevalence of the condition, and to tests the utility of noninvasive diagnostic modalities such as MRI in the diagnosis of this condition.

The signs and symptoms of Wilson disease often begin during the teenage years and should be ruled out. Butts et al[ 24 ], Awareness of this entity in the medical community including specialists is low.


Support Center Support Center. Therefore, lactic acidosis could be secondary to reduced gluconeogenesis and a lack of conversion of pyruvate to glucose[ 35 ]. Mild steatosis, mostly macrovesicular, was present.

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

A patient with Glyfogenic may present with hepatomegaly, abdominal pain, nausea, vomiting, and abnormal liver function tests reflecting acute liver injury [ 1 — 9 ]. The case presented was followed up for the past three years with NAFLD diagnosis due to elevated liver enzyme levels and ultrasonographic hepatosteatosis appearance. Support Center Support Center.

Ultrastructural analysis was performed in 2 cases. The authors gratefully thank Dr. Case Reports in Hepatology. These may be nonspecific as well, as there were no reports of any associated systemic disease[ 1826384148 ]. Liver biopsy and Histological features A liver biopsy typically shows swollen hepatocytes with an accumulation of glycogen in the cytoplasm. This is an open-access article distributed under the glcogenic of the Creative Glycogeniv Attribution-Noncommercial-Share Alike 3.

Management for GSDs revolves around management of complex carbohydrate intake and surveillance for complications, whereas management for GH centers on proper glycemic control with insulin and prevention of hyperglycemic episodes.

Can be stained for copper Biochemical tests and slit lamp examination with or without genetic testing and liver biopsy Treatment with a chelating agent. Periodic acid-Schiff stain revealed glycogen accumulation which dissolved upon diastase application Figure 2. Afterwards, the case was diagnosed with GH upon liver biopsy. Abstract Glycogenic hepatopathy is a rare cause of high transaminase levels in type 1 diabetes mellitus.

It has been reported in the medical literature that remission was attained in a case with GH by a continuous insulin infusion pump implanted under the skin [ 7 ]. Glycogenic hepatopathy, an underdiagnosed cause of relapsing hepatitis in uncontrolled type 1 diabetes mellitus. Medical records were reviewed for clinical presentations, laboratory findings, and clinical outcomes. The rapid enlargement of the liver causes stretching of the liver capsule, resulting in visceral pain.

Histological examination of hepatocytes from patients with GH normally does not exhibit significant portal inflammation, steatosis, or significant fibrosis. Kransdorf et al[ 57 ], Hepatomegaly with or without elevated transaminases levels is a common condition in patients with uncontrolled type 1 diabetes mellitus DM.


Long-term follow-up and future studies are required to examine the natural history and to explore treatment options for this form of microvascular complication involving the liver. The pathology is distinct from steatohepatitis. 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 ].

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Original hard copies of all figures may be requested when necessary. Unsolicited manuscript Peer-review started: On the 36th hour of his hospitalization, urine ketone was negative and nausea had regressed. It has been reported that 1 in 10 patients with AN will have abnormal liver hepatoptahy.

Alkaline phosphatase; T bili: Glucosephosphate is converted to glycogen by the enzyme glycogen synthase, which exists in an active dephosphorylated form and in an inactive phosphorylated form. This is an open access article distributed under the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Auto immune hepatitis; DM: Hepatitis A and E are transmitted by feco- oral route; Rest of the viruses spread either by sexual contact, contact with body fluids or blood or from birth from an infected mother. It was possible that once he was started on metformin, his hepatic gluconeogenesis decreased and glucose uptake in glycohenic tissues increased, possibly leading to a paucity of glucose as a substrate for glycogenesis[ 48 ]. Ruschhaupt et al[ 1 ], The cardiovascular and respiratory system examination results were normal.

Prognosis with improved glycemic control is excellent.