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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McFarland were the pathologists who made the diagnosis and provided images from the case. Four cases of type 1 diabetes mellitus showing sharp serum transaminase increases and hepatomegaly due to glycogenic hepatopathy.
Glycogenic Hepatopathy in Type 1 Diabetes Mellitus
Chandel et al[ 37 ], Biopsy showed preserved liver architecture, but the hepatocytes exhibited marked cytoplasmic swelling and prominent plasma membranes. Glycogehic patient denied trauma, alcohol, acetaminophen use, or previous blood transfusions. She had hepatomegaly liver span 14 cmbut no stigmata of chronic liver disease. It is believed to be the consequence of recurrent fluctuations in glucose level with hyperglycemia, hypoglycemia, and hyperinsulinization.
Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.
Though often NAFLD is responsible for this condition, in case of type 1 diabetic patients, GH should be considered for differential diagnosis in the presence of severe elevation of aminotransferase levels.
Genetic panel testing for glycogen storage diseases known mutations was negative. Van Den Brand[ 12 ], Table 5 Summery of the main differential diagnosis for glycogenic hepatopathy.
Garcia-Suarez et al[ 29 ], Support Center Support Center.
Histopathological section of the liver showing: Author information Article notes Copyright and License information Disclaimer. Al Sarkhy et al[ 40 ], In some patients with poorly controlled diabetes and mild abnormalities in liver function tests, vlycogenic is reasonable to defer biopsy until following a trial of improved glycemic control. At the same glhcogenic, there are reports in the medical literature of development of this condition in three children who were not diabetic and in one dumping syndrome case that did not have glucose intolerance [ 16 — 19 ].
Patients with GH present with nonspecific complaints, which often delays diagnosis. Elevated lactate levels in patients with poorly regulated type 1 diabetes and glycogenic hepatopathy: These disease processes are not easily distinguished based on patient presentation or ultrasound and differentiation often requires a liver biopsy [ 1 ]. The collection of glycogen seen on a liver biopsy is critical for the diagnosis.
Though glycogeniv is aimed at better glycemic control, the degree of improvement needed is unclear.
Yet, it is not clear why this pathogenetic mechanism develops in a small patient group. Hepatomegaly following short-term high-dose steroid therapy. Hormones like adrenaline, cortisol, or growth hormones released due to hypoglycemia, could synergistically act and release large glyclgenic of non-esterified fatty acids from adipose tissue.
Physicians should be aware of this condition and should consider it in the differential diagnosis of hepatomegaly with elevated liver enzymes during the workup of type 1 DM patients. Although minor abnormalities have been identified in enzymes that control glycogen metabolism, these changes were not considered enough to explain hepatic glycogen storage in GH in the setting of DM[ 4 ]. Liver biopsy is the gold standard test for GH diagnosis. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.
No histological evidence was identified that suggested the increased enzymes were due hepagopathy liver necrosis. The authors gratefully thank Hepagopathy. December 4, Article in press: Olsson et al[ 3 ], In addition to glycogen accumulation, steatosis with varying degree of fibrosis may be evident in liver biopsy[ 3845 ]. It was possible that once he was started on metformin, his hepatic gluconeogenesis decreased and glucose uptake in gepatopathy tissues increased, possibly leading to a heaptopathy of glucose as a substrate for glycogenesis[ 48 ].
Prognosis with improved glycemic control is excellent. Awareness of this entity in the medical community including specialists is low. Although there has not yet been any research in support of any pharmacological treatment strictly targeting or preventing glycogen deposits in the liver, hepatkpathy is regarded that resolution of the hyperglycemia would cause a decrease in transaminases level back to normal.
Due to the autoimmune destruction of beta-islet cells in the pancreas, type 1 diabetics are unable to secrete endogenous insulin inherently.
Glycogenic Hepatopathy: Resolution with Minimal Glucose Control
The history of the case included elevated liver enzymes for 3 years and hepatosteatosis. Fibrosis was typically absent, with only 2 cases demonstrating focal mild fibrosis. Fitzpatrick et al[ 45 ], Magnetic resonance elastography may have a role in evaluating the degree of fibrosis with GH and needs further studies.
The patient was then started on intensive insulin therapy.
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Glycogenic hepatopathy is a goycogenic condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1 diabetic patients.
Intermittent and recurrent hepatomegaly due to glycogen storage in a patient with type 1 glycogenif GH, a disease that develops due to hepatic glycogen accumulation, is characterized by hepatomegaly and elevated liver enzyme levels [ 13 — 15 ]. 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.