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.

Author: Maull Vijar
Country: Honduras
Language: English (Spanish)
Genre: Politics
Published (Last): 2 November 2009
Pages: 105
PDF File Size: 7.67 Mb
ePub File Size: 13.31 Mb
ISBN: 980-9-94804-220-6
Downloads: 52659
Price: Free* [*Free Regsitration Required]
Uploader: Kajijas

These findings are infrequently observed today due to widespread use of long-acting insulin and possibly the decreased frequency of hypoglycemic events in this patient cohort.

Glycogenic hepatopathy: A narrative review

Her physical exam and symptoms of sharp abdominal pain prompted further evaluation. The pathogenesis of growth retardation in those initial cases reported in children was thought to be multifactorial[ 52 ].

Clinical presentation of GH varies from asymptomatic patients with elevated liver enzymes to various symptoms associated with hyperglycemia. While first described in association with recurrent hyper- and hypoglycemia with ketoacidosis in T1DM, it has since been reported without ketosis or acidosis in patients hepaatopathy T2DM and with variable insulin requirements[ 348 – 1642 ].

Although ischemic hepatitis is the result of hypotension and hypoperfusion, subjects with inadequate perfusion without the hypotension can hepatoparhy produce a significant elevation in transaminases without liver necrosis[ 26 ]. 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.


A total of fifty-one studies involving adolescents or adults were included for this review. HE glycogeic showing enlarged hepatocytes with cytoplasmic pallor with reddish pink globules consistent with glycogen accumulation blue arrowand prominent glycogenated nuclei green arrow. Patients presenting with tender hepatomegaly and abnormal liver function tests without hepatoathy identifiable causes should undergo early liver biopsy. The authors acknowledge John Wiley and Sons publications for providing the images.

Steroids promote elevation in glucose, gluconeogenesis, and glycogen deposition. Torbenson et al[ 44 ], There is still a need for larger-scale and long-term studies to explore the consequences of fibrosis over time. Resolution with Minimal Glucose Control.

Glycotenic glycemic control, provided via intensive insulin therapy, results in full remission of clinical, laboratory, and histologic abnormalities [ 4 ].

It occurs in patients with T1DM more often than in T2DM and is associated with severe microvascular disease in other organs. Address correspondence and reprint request to: However, recent studies have identified glycoyenic varying degree of fibrosis in glycogen hepatopathy as well, further emphasizing the need for future studies. Glycogenic hepatopathy GH is considered as a benign reversible condition.

Glycogenic Hepatopathy in Type 1 Diabetes Mellitus

Several mechanisms were involved in the pathogenesis of GH in steroid use. Unsolicited manuscript Peer-review started: Management for GSDs revolves around management of complex carbohydrate intake and surveillance for complications, whereas management for GH centers on proper glycemic control glucogenic insulin and prevention of hyperglycemic episodes. In some cases, though symptoms and histopathology may resolve with improved glycemic control, transaminitis may persist [ 6 ].


After the administration of insulin and intravenous glucose the patient developed acute glycogen storage hepatomegaly. He glycogemic a past medical history of T1DM and bipolar disorder that was being treated with the antipsychotic drugs paliperidone and asenapine.

Prior to the suicide attempt, glycogenicc baseline liver enzymes were initially normal. Glycogenic hepatopathy in type 1 diabetes mellitus.

National Center for Biotechnology InformationU.

Glycogenic hepatopathy GH is a rare disease that develops due to excessive accumulation of glycogen in the hepatocytes, leading to hepatomegaly and elevated transaminases levels in patients with uncontrolled type 1 DM. Gastroenterology and hepatology Glycgoenic of origin: Glycogen synthase converts glucose 1-phosphate into glycogen and increases glycogen storage in the liver while inhibiting glycogenolysis [ 126 ].

Pediatr Gastroenterol Hepatol Nutr.

Glycogenic Hepatopathy: Resolution with Minimal Glucose Control

Mild steatosis, mostly macrovesicular, was present. She had hepatomegaly liver span 14 cmbut no stigmata of chronic liver disease. Glycogen production persists for some time after insulin levels have declined while inhibiting the glycogenolysis[ 15 ].

Abstract Glycogenic hepatopathy is a hlycogenic cause of high transaminase levels in type 1 diabetes mellitus.