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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Liver synthetic function bilirubin, albumin, and coagulation profile and lipid profile were normal.

Glycogenic Hepatopathy: Resolution with Minimal Glucose Control

To receive news and publication updates for Case Reports in Hepatology, enter your email address in the box below. Hepatomegaly following short-term high-dose steroid therapy. Dumping syndrome, a cause of acquired glycogenic hepatopathy.

Glycogenic hepatopathy is a rare condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1 diabetic patients.

Tight glycemic control glyckgenic intensive insulin therapy provides full remission of clinical, laboratory, and histological abnormalities [ 4 ]. Abstract Glycogenic hepatopathy is a rare cause of high transaminase levels in type 1 diabetes mellitus.

Glycogenic hepatopathy: A narrative review

Mutations cause increased iron absorption and excessive deposition in the liver, heart, pancreas, and pituitary Asymptomatic or chronic liver disease with elevated transaminases, skin pigmentation, DM, arthropathy, impotence and cardiac enlargement, etc MRI is most sensitive and can estimate iron concentration in the liver.

The incidence of this syndrome has decreased significantly with the introduction of long-acting insulin and better control of blood sugar, but unfortunately, it still exists[ 52 ].


Fridell et al[ 5 ], In other words, hepatic glycogen accumulation occurs despite the high cytoplasmic glucose concentration in the presence of insulin. The excess glycogen accumulation has also been reported in diabetic patients without previous episodes of hypoglycemia[ 4453 ].

Some case reports described the utility of radiological imaging studies such as computed tomography CT scan and magnetic resonance imaging MRI in establishing the diagnosis of GH depending primarily on the interval changes in the liver density and its correlation with the clinical picture; however, further larger-scale studies are needed to confirm the findings from these reports, and to establish their sensitivity and specificity for this condition.

The hallmark of the GH is severe fluctuation in levels of glucose and administration of supraphysiologic levels of insulin to control the hyperglycemia. Absolute quantification of the hepatic glycogen content in a patient hepatopatny glycogen storage disease by 13C magnetic resonance spectroscopy. Expression of the mutant PHKG2 in a human liver cell line inhibited the enzyme activity of the phosphokinase complex and increased glycogen levels[ 55 ].

Anorexia nervos AN is characterized by the obsessive fear of gaining weight, distorted body image, and a significantly low body weight. The transaminases were dramatically elevated in 3 cases to greater than 10 times the upper limit of glycoogenic. Thereupon, a liver biopsy was performed.

Low intensity on subtraction. It is an underrecognized entity, and awareness of this clinical condition by clinicians, including gastroenterologists, is bepatopathy. Summary of the major studies in English pub med indexed on Glycogenic hepatopathy in type 1 diabetes mellitus. Olson et al[ 3 ], Once the diagnosis is made, improved glycemic control is the mainstay of management. This vicious cycle of excessive doses of short-acting insulin and subsequent administration of glucose to counteract the resulting hypoglycemia could have led to the continued accumulation of glycogen in the liver, a proposed mechanism by various authors[ 31954 ].


The patient who did not have a history of hepatotoxic agent use was examined with regard to chronic hepatitis differential diagnosis since the aminotransferase levels were found to be elevated to more than 10 times the normal values, intermittently for 3 years. In the setting of poorly controlled T1DM, the presence of excess glucose and insulin contributes to glycogen storage in the liver. Saracho et al[ 36 ], The family history was unremarkable for liver disease.

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Rev Esp Enferm Dig. Hepatomegaly glhcogenic soon after discontinuing the steroid therapy in all patients with hepatomegaly. Hepatic glycogenosis in an adolescent with diabetes. Isr Med Assoc J. Glucocorticoid monotherapy or in combination with immunomodulators.

Author information Article notes Copyright and License information Disclaimer. Three months later, her HgbA1c remained significantly elevated at