Glycogenic hepatopathy in young adults: a case series

Silva, Marco; Marques, Margarida; Cardoso, Hélder; Rodrigues, Susana; Andrade, Patrícia; Peixoto, Armando; Pardal, Joana; Lopes, Joanne; Carneiro, Fátima; Macedo, Guilherme

doi:10.17235/reed.2016.3934/2015

Cited by 17 publications

(17 citation statements)

References 12 publications

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“…Although a few reported cases had normal liver sizes on imaging studies, hepatomegaly was observed in more than 90% of the reported cases, with varying degrees of elevation in transaminases and rarely an elevation in alkaline phosphatase (ALP) level. Excessive glycogen with swollen hepatocytes eventually could cause sinusoidal compression and subsequent ascites[ 34 ]; therefore, ascites can also be a part of the clinical presentation, albeit rarely[ 34 , 44 ]. Physical examination of patients with GH generally exhibits tender hepatomegaly without splenomegaly.…”

Section: Clinicopathological Featuresmentioning

confidence: 99%

Glycogenic hepatopathy: A narrative review

Sherigar

Castro

Yin

et al. 2018

WJH

112

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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. It is predominantly seen in patients with longstanding type 1 diabetes mellitus and rarely reported in association with type 2 diabetes mellitus. Although it was first observed in the pediatric population, since then, it has been reported in adolescents and adults with or without ketoacidosis. The association of GH with hyperglycemia in diabetes has not been well established. One of the essential elements in the pathophysiology of development of GH is the wide fluctuation in both glucose and insulin levels. GH and non-alcoholic fatty liver disease (NAFLD) are clinically indistinguishable, and latter is more prevalent in diabetic patients and can progress to advanced liver disease and cirrhosis. Gradient dual-echo MRI can distinguish GH from NAFLD; however, GH can reliably be diagnosed only by liver biopsy. Adequate glycemic control can result in complete remission of clinical, laboratory and histological abnormalities. There has been a recent report of varying degree of liver fibrosis identified in patients with GH. Future studies are required to understand the biochemical defects underlying GH, noninvasive, rapid diagnostic tests for GH, and to assess the consequence of the fibrosis identified as severe fibrosis may progress to cirrhosis. Awareness of this entity in the medical community including specialists is low. Here we briefly reviewed the English literature on pathogenesis involved, recent progress in the evaluation, differential diagnosis, and management.

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Section: Clinicopathological Featuresmentioning

confidence: 99%

Glycogenic hepatopathy: A narrative review

Sherigar

Castro

Yin

et al. 2018

WJH

112

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“…GH is underrecognized and often confused with fatty liver disease and nonalcoholic steatohepatitis, which is more common in type 2 diabetes. e exact incidence and prevalence of the disease are unknown [1][2][3][4][5][7][8][9][10][11]. Classically it is seen in longstanding, poorly controlled, type 1 diabetes (98% of cases) with a mean duration of 10.5 years and a mean HbA1c of 11.8 [1][2][3].…”

Section: Discussionmentioning

confidence: 99%

“…e mechanism of ascites remains unclear [1]. Other laboratory abnormalities in patients with GH include a high amylase and/or lipase in patients with no clinical or radiographic evidence of acute pancreatitis [11]. e underlying mechanism of this is also unknown [11].…”

Section: Discussionmentioning

confidence: 99%

“…It is crucial that this condition is diagnosed and differentiated from other conditions which present with similar symptoms, as GH is reversible unlike other differentials including nonalcoholic fatty liver disease (NAFLD) which is more commonly seen in type 2 DM [3]. Despite the increasing incidence of diabetes in the United States [6], the real incidence and prevalence of GH are unknown [1][2][3][4][5][7][8][9][10][11][12][13][14][15][16], commonly being misdiagnosed or underdiagnosed. We present a case of a patient with poorly controlled type 1 diabetes who presented with abdominal pain, abnormal liver enzymes, and massive enlargement of the liver and was subsequently diagnosed with glycogenic hepatopathy.…”

Section: Introductionmentioning

confidence: 99%

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Hepatocellular Glycogen Accumulation in the Setting of Poorly Controlled Type 1 Diabetes Mellitus: Case Report and Review of the Literature

Aluko

Enofe

Burch

et al. 2020

Case Reports in Hepatology

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Glycogenic hepatopathy (GH) is the accumulation of glycogen in the hepatocytes and represents a rare complication in patients with diabetes mellitus (DM), most commonly type 1 DM. We present a case of a 23-year-old woman with a medical history of poorly controlled type 1 DM and gastroesophageal reflux disease (GERD) who presented with progressively worsening right-sided abdominal pain. Diagnostic workup resulted in a liver biopsy with hepatocytes that stained heavily for glycogen with no evidence of fibrosis or steatohepatitis. A diagnosis of glycogenic hepatopathy was made, and an aggressive glucose control regimen was implemented leading to resolution of symptoms and improvement in AST, ALT, and ALP. In addition to presenting this rare case, we offer a review of literature and draw important distinctions between glycogenic hepatopathy and other differential diagnoses with the aim of assisting providers in the diagnostic workup and treatment of glycogenic hepatopathy.

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“…After GH diagnosis, the treatment should aim the intense glycemic control as this is considered the backbone of its management 15 34 44. However, resolution of GH has also been described after minimal glucose control 34 45.…”

Section: Discussionmentioning

confidence: 99%

Glycogenic hepatopathy as a cause of severe deranged liver enzymes in a young patient with type 1 diabetes mellitus

et al. 2019

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Glycogenic hepatopathy (GH) is a rare complication of poorly controlled type 1 diabetes mellitus (T1DM). We present a 19-year-old woman with T1DM and autoimmune thyroiditis who admitted to our department because of abrupt onset intermittent abdominal pain in the right upper quadrant accompanied by laboratory evidence of acute anicteric hepatitis. Physical examination revealed significant hepatomegaly but the common imagining studies were negative. Following exclusion of common causes of acute hepatitis and because of the presence of smooth muscle antibodies in a young female patient with already established two autoimmune diseases, a liver biopsy was performed in order to exclude the potential presence of autoimmune hepatitis. However, liver histology showed typical findings of GH. Intense treatment targeting strict glycemic control resulted in normalisation of liver biochemistry. This case underlines that GH should be considered as a rare cause of acute hepatitis in T1DM patients with poor glycemic control.

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Glycogenic hepatopathy in young adults: a case series

Cited by 17 publications

References 12 publications

Glycogenic hepatopathy: A narrative review

Glycogenic hepatopathy: A narrative review

Hepatocellular Glycogen Accumulation in the Setting of Poorly Controlled Type 1 Diabetes Mellitus: Case Report and Review of the Literature

Glycogenic hepatopathy as a cause of severe deranged liver enzymes in a young patient with type 1 diabetes mellitus

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