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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Thirty-eight percent of reported cases are male patients, with most cases occurring in adolescence.

Glycogenic Hepatopathy: Resolution with Minimal Glucose Control

In the presence of hyperglycemia, glucose passively diffuses into hepatocytes insulin-independent mechanismand is subsequently converted to glucosephosphate, which is then converted to glycogen under the influence of insulin; these changes are exaggerated in the presence of hyperglycemia and over-insulinization, which are believed to represent the primary mechanism in the development of GH.

The patient was then started on intensive insulin therapy. Type 1 diabetes patients compose majority of the case reports on this rare condition. Imtiaz et al[ 23 ], Liver enzyme elevation is more common among diabetic patients compared to the general population. Thyroid stimulating hormone TSHceliac screen, ammonia, lactate, and metabolic screenings were all normal.

Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.

Most cases showed no evidence for fatty liver disease: Excessive glycogen with swollen hepatocytes heppatopathy could cause sinusoidal compression and subsequent ascites[ 34 ]; therefore, ascites can also be a part of the clinical presentation, albeit rarely[ 3444 ]. Most patients with GH will have elevated transaminases. J Pediatr Gastroenterol Nutr. Glucocorticoid monotherapy or in combination with immunomodulators. Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus.


In addition to glycogen accumulation, steatosis with varying degree of fibrosis may be evident in liver biopsy[ 3845 ]. Pathology results of majority of the cases reported in the literature did not show any significant fibrosis of the liver; however, further long-term studies are required to assess for the consequence of the mild fibrosis identified in some reports, and few cases of bridging fibrosis as severe fibrosis may further progress to cirrhosis.

Unusual glycogenic hepatopathy causing abnormal liver enzymes in a morbidly obese adolescent with well-controlled type 2 diabetes: The phosphatase enzyme is stimulated by elevated glucose and insulin levels.

Future progress is required glyccogenic understanding the biochemical defects underlying GH and development of fibrosis. On the 36th hour of his hospitalization, urine ketone was negative and nausea had regressed. All figures or photographs should be submitted in a high resolution minimum DPI electronic version saved in jpeg or tiff format.

Case Reports in Hepatology

Introduction Diabetes is associated with several etiologies of hepatic dysfunction. Hepatic complications in poorly controlled type 1 diabetes mellitus: 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 ].

Sinusoidal compression by the swollen hepatocytes can produce a paved appearance to the liver parenchyma. Type 1 diabetes mellitus; US: Murata et al[ 19 ], Prospective evaluation of unexplained chronic liver transaminase abnormalities in asymptomatic and symptomatic patients. The prevalence of elevated alanine aminotransferase level is 9.

Hyperglycemia with hyperglycemic symptoms; could be asymptomatic. 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. 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 ].


J Paediatr Child Health. A case has also been reported in a patient following intentional insulin overdose [ 67 ]. Biopsy showed preserved liver architecture, but the hepatocytes exhibited marked cytoplasmic swelling and prominent plasma membranes.

Like GH, it involves fluctuation between hyperglycemia from the rapid nutrition glucose load and hyperinsulinemia. Massive hepatomegaly in poorly controlled insulin-dependent diabetes mellitus.

Once the diagnosis is made, improved glycemic control is the mainstay of management. Further research is needed for an ideal noninvasive, rapid diagnostic test to avoid the extensive workup and associated costs in evaluating suspected cases of GH.

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

The use of high-dose corticosteroids may precipitate or exacerbate Hepatoparhy. This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. Both her parents were immunized for hepatitis and there was no family history of liver, gastrointestinal, or autoimmune diseases. Hepatomegaly with or without raised transaminase levels is common in patients with DM.

Brouwers et al[ 32 ], Hepatopathy of Mauriac syndrome: