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: Fejas Brarn
Country: Mayotte
Language: English (Spanish)
Genre: Software
Published (Last): 18 April 2010
Pages: 452
PDF File Size: 9.9 Mb
ePub File Size: 9.22 Mb
ISBN: 237-3-94321-475-9
Downloads: 90269
Price: Free* [*Free Regsitration Required]
Uploader: Dasho

Some cases may require liver transplantation. This condition, characterized by elevated liver enzymes and hepatomegaly, is caused by irreversible and excessive accumulation of glycogen in hepatocytes. Munns et al[ 4 ], Though often NAFLD is responsible for hwpatopathy condition, in case of type 1 diabetic patients, GH should be considered for differential diagnosis in the presence of severe elevation of aminotransferase levels.

However, the best modality to accurately diagnose a GSD is next-generation genetic sequencing.

Hepatomegaly following short-term high-dose steroid therapy. Main etiology Clinical presentation Imaging characteristics Key, liver biopsy pathological features Diagnosis Management Cirrhosis GH Acquired excessive glycogen deposition in the liver mostly seen in patients with T1DM Hyperglycemia with hyperglycemic symptoms; could be asymptomatic.

Thereupon, a liver biopsy was performed. Acute liver cell damage in patients with hepatipathy nervosa: The hepatocyte cytoplasm is not stained with PAS. Fourteen cases were studied by stains, including hematoxylin and eosin, trichrome, periodic heparopathy, and periodic acid-Schiff with diastase.

Glucosephosphate is converted to glycogen by the enzyme glycogen synthase, which exists in an active dephosphorylated form and in an inactive phosphorylated form.

Case Reports in Hepatology

Glycogen accumulation further increases because insulin also activates glycogen synthase [ 20 ]. Butts et al[ 24 ], View at Google Scholar. 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 ]. In patients with a classic presentation of Mauriac syndrome, the wide fluctuations between hyper- and hypoglycemia-a pattern suggestive of under- and over-insulinization with secondary hyperadrenalism-possibly caused the cushingoid features.

The hepatopatuy of glycogen seen on a liver biopsy is critical for the diagnosis. High intensity on subtraction. Some case reports described the utility of radiological imaging studies such as computed tomography CT scan and magnetic resonance imaging MRI in establishing the hepatopthy 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.


After the administration of insulin and intravenous glucose the patient developed acute glycogen storage hepatomegaly. Mukewar et al[ 46 ], Diabetes mellitus and the liver. The case was diagnosed with GH due to the present clinical, laboratory, and pathological findings.

Neither CT abdomen nor liver US is a useful test for the definitive diagnosis of GH; however, gradient dual-echo MRI sequence was reported to be able to distinguish fat deposition from an edematous condition such as acute tissue injury; both of which appears as low-density areas on CT.

Recurrent hepatomegaly and transient alteration of liver functions in an adolescent with diabetic acidosis. The pathophysiology of Dumping syndrome can resemble that of GH seen in people with diabetes, eventually causing the hepatic accumulation of glycogen.

Thirty-eight percent of reported cases are male patients, with most cases occurring in adolescence. Introduction Liver enzyme elevation is more common among diabetic patients compared to the general population.

Glycogenic hepatopathy: A narrative review

At the same time, 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 ]. Stored mainly in the liver and skeletal muscles, glycogen is a polymer of glucose that acts as a reservoir for glucose.

Glycogenic hepatopathy GH is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction with elevated liver enzymes and associated hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes.

All figures or photographs should be submitted in a high resolution minimum DPI electronic version saved in jpeg or tiff format. Glycogenic hepatopathy associated with type 1 diabetes mellitus as a cause of recurrent liver damage. Varicella zoster virus; CMV; Cytomegalovirus. Table 1 Summary of the major case reports in English pub med indexed on Glycogenic hepatopathy in type 1 diabetes mellitus.


Subscribe to Table of Contents Alerts. A patient with GH may present with hepatomegaly, abdominal pain, nausea, vomiting, and abnormal liver function tests reflecting acute liver injury [ 1 — 9 ].

HE stain showing enlarged hepatocytes with cytoplasmic pallor with reddish pink globules consistent with glycogen accumulation blue arrowand prominent glycogenated nuclei green arrow. Mok edited the case report, assisted in manuscript preparation, and is the article guarantor. Scarpato wrote the case report.

GH develops due to excessive and irreversible accumulation of glycogen in the hepatocytes and causes liver function disorders and hepatomegaly [ 34 ].

Short-term high dose steroid therapy Steroids promote elevation in glucose, gluconeogenesis, and glycogen deposition. Charndrasekaran et al[ 33 ], Table 3 Summary of the major hepattopathy reports in English PubMed indexed on Glycogenic hepatopathy in type 2 diabetes mellitus.

Hepatomegaly can sometimes develop within days to weeks and hepatkpathy improve rapidly once the hyperglycemia is controlled[ 54 ]. Biochemical tests and hepatoathy lamp examination with or without genetic testing and liver biopsy.

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. The patient was then started on intensive insulin therapy.

There is still a need for larger-scale and long-term studies to explore the consequences of fibrosis over time. Presence of cirrhosis can be determined Biochemical tests including genetic testing, radiologic, and liver biopsy Phlebotomy or Chelation therapy if unable to tolerate phlebotomy Can progress to cirrhosis Wilson disease Autosomal recessive disorder with impaired cellular copper transport and impaired biliary copper excretion results in accumulation of copper hepatopatby notably the liver, brain, and cornea.

Tight glycemic control via intensive insulin therapy provides full remission of clinical, laboratory, and histological abnormalities [ 4 ].