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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National Center for Biotechnology InformationU. We briefly reviewed the literature on mechanisms involved in the development of GH, evaluation of these patients, recent progress heepatopathy on diagnostic tests and management. 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.

Hepatopathy of Mauriac syndrome: T1 weighted gradient-dual-echo MRI images with in-phase and opposed-phase conditions could efficiently differentiate hepatic glycogen from the fat seen in NAFLD[ heptopathy22 ]. On the 36th hour of his hospitalization, urine ketone was negative and nausea hepatooathy regressed. GH was first defined as glycogen accumulation inas a component of Mauriac syndrome type 1 diabetes, delayed development, hepatomegaly, cushingoid appearance, and delayed puberty [ 5 ].

Depletion of intracellular inorganic phosphate for which there is an absolute requirement may be responsible for limitation of phosphorylase activity. Type 1 diabetes patients compose majority of the case reports on this rare condition. 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.

Gycogenic hepatocytes exhibit glycogenated nuclei. Table of Contents Alerts. The medical history of the case showed that he was followed up due to type 1 diabetes for 8 years and for hepatosteatosis for 3 years, had poor blood glucose regulation despite insulin analogue and basal insulin therapy, and was hospitalized and followed up 8—10 times for diabetic ketoacidosis.


One of the essential elements in the pathophysiology of development of GH is the wide fluctuation in both glucose and insulin levels. Clin Pediatr Phila ; 9: He became hypoglycemic and was later administered a large dose of intravenous glucose to counteract the persistent hypoglycemia. GH can resolve with both clinical and biochemical resolution as quickly as it develops within days to weeks with good glycemic control[ 40 ].

In other words, hepatic glycogen accumulation occurs despite the high cytoplasmic glucose concentration in the presence of insulin. A study by MacDonald and colleagues of an adolescent boy with Mauriac syndrome identified a mutation in PHKG2, is the catalytic subunit of glycogen phosphorylase kinase[ 55 ].

Hepatitis A and E are transmitted by feco- oral route; Rest of the viruses spread either by sexual contact, contact with body glucogenic or blood or from birth from an infected mother.

Note that some nuclei have cleared chromatin nuclear glycogenosiswhich can be seen in various metabolic conditions, such as diabetes mellitus Hematoxylin and Eosin staining at x magnification. Cui LJ L- Editor: The hepatocytes in both conditions are markedly swollen and filled with glycogen, while the subtle difference can be the presence of higher cytoplasmic clumping of glycogen in GSD[ 44 ].

Nonalcoholic fatty liver glycoegnic NAFLD is the most common cause of chronic liver disease for both the general and the diabetic populations today. Histological examination of a liver biopsy specimen from a patient with NAFLD could likewise show macro vesicular steatosis, mild lobular and portal inflammation, and varying degrees of fibrosis. Histological examination of hepatocytes from patients with GH normally does not exhibit significant portal inflammation, steatosis, or significant fibrosis.

Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.

The family history was unremarkable for liver disease. Arch Pathol Lab Med. Ultrasound of the abdomen showed hepatomegaly A bright liver compared to the spleen in CT scan imaging can be the clue to diagnosing GH.

May need liver transplantation in selected cases.

It develops due to excessive accumulation of glycogen in the hepatocytes. High glucose levels cause an influx of glucose into the hepatocytes via facilitated diffusion through the glucose transporter 2 GLUT2independent of insulin[ 43 ]. Saadi T[ glycogenix ], Tight glycemic control, provided via intensive insulin therapy, results in full remission of clinical, laboratory, and histologic abnormalities [ 4 ]. Symptomatic treatment to dietary changes to maintain the blood glucose level and pharmacologic therapy in different types of GSDs.


Hyperglycemia activates glycogen synthase by inhibiting glycogenesis via glycogen phosphorylation inactivation. Evidence of hepatocellular injury and fibrosis would indicate increased severity and progression to actual steatohepatitis.

Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.

As patients with glycogenic hepatopathy often have nonspecific complaints, diagnosis tends to be delayed and laboratory and imaging data are often indistinguishable from nonalcoholic fatty liver disease.

Dual-Echo MRI; iso-intense between the in-phase and out-of-phase images, and low intensity on subtraction. High intensity on subtraction. The mother of the boy with Mauriac syndrome also had the mutant PHKG2 but did not have diabetes or hepatomegaly. Prognosis with improved glycemic control is excellent. In conclusion, Although it is hepatopsthy well-known complication of poorly controlled type 1 DM, GH is still an underdiagnosed cause of elevated transaminase levels and hepatomegaly in these 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 ]. Although ischemic hepatitis is the result of hypotension and hypoperfusion, subjects with inadequate perfusion without the hypotension can hepatopayhy produce a significant elevation in transaminases without liver necrosis[ 26 ].

The fact that the patient was in the early stages of diabetes and had not decompensated enough to cause total insulin resistance might explain the preserved effect of insulin on glycogenesis[ 48 ]. Munns et al[ 4 ], Ultrastructural examination revealed marked glycogen accumulation in the cytoplasm and nuclei.

The collection of glycogen seen on a liver biopsy is critical for the diagnosis.