Acute and Relapsing Hepatitis Caused by Glycogenic Hepatopathy

Shah, Neil; Sasatomi, Eizaburo; Baron, Todd H.

doi:10.1016/j.cgh.2017.02.004

Cited by 7 publications

(8 citation statements)

References 2 publications

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“…Additionally, antinuclear antibody was positive in some cases, showing a mostly homogeneous speckled pattern. These may be nonspecific as well, as there were no reports of any associated systemic disease[ 18 , 26 , 38 , 41 , 48 ].…”

Section: Clinicopathological Featuresmentioning

confidence: 99%

“…The architectural structure of the liver parenchyma likewise remains intact. Furthermore, an addition of diastase to the Periodic-Acid Schiff (PAS) stained specimen would cause enzymatic breakdown of glycogen in the hepatocytes, causing these hepatocytes to turn into “ghost cells”[ 41 ] (Figure 4 ). Histological examination of hepatocytes from patients with GH normally does not exhibit significant portal inflammation, steatosis, or significant fibrosis.…”

Section: Diagnosismentioning

confidence: 99%

“…In a review of cases of GH by Torbenson et al[ 44 ], mild steatosis was noted in 14% of cases, mild steatohepatitis in 7% of cases, and mild fibrosis in 14% of cases with GH. Histopathology of a case reported by Shah et al[ 41 ]. also showed GH with focal portal tract fibrosis.…”

Section: Diagnosismentioning

confidence: 99%

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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%

Section: Diagnosismentioning

confidence: 99%

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Glycogenic hepatopathy: A narrative review

Sherigar

Castro

Yin

et al. 2018

WJH

112

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“…Resolution of GH has also been published after pancreatic transplantation in diabetic patients, further supporting the notion that GH is completely reversible after the effective management of diabetes 29. However, recurrences are not uncommon if metabolic targets are not achieved 15 21 46 47. For this reason, close follow-up for recurrence of symptoms may be needed in patients with a past history of GH and poorly controlled diabetes.…”

Section: Discussionmentioning

confidence: 82%

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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“…Adding diastase to the Periodic-Acid Schiff stained specimen, enabling digestion of glycogen in the hepatocytes, would lead to the appearance of "ghost cells" (Shah et al, 2017). It is important to mention, that in the histology of NAFLD, besides steatosis, inflammation, and fibrosis, hepatocellular glycogenated nuclei are also often described, facilitating differentiation between NASH and alcoholic steatohepatitis, since they are rarely seen in the latter (Pinto et al, 1996;Takahashi and Fukusato, 2014).…”

Section: Glycogenic Hepatopathy: Epidemiologymentioning

confidence: 99%

Hepatopathy Associated With Type 1 Diabetes: Distinguishing Non-alcoholic Fatty Liver Disease From Glycogenic Hepatopathy

et al. 2021

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Autoimmune destruction of pancreatic β-cells results in the permanent loss of insulin production in type 1 diabetes (T1D). The daily necessity to inject exogenous insulin to treat hyperglycemia leads to a relative portal vein insulin deficiency and potentiates hypoglycemia which can induce weight gain, while daily fluctuations of blood sugar levels affect the hepatic glycogen storage and overall metabolic control. These, among others, fundamental characteristics of T1D are associated with the development of two distinct, but in part clinically similar hepatopathies, namely non-alcoholic fatty liver disease (NAFLD) and glycogen hepatopathy (GlyH). Recent studies suggest that NAFLD may be increasingly common in T1D because more people with T1D present with overweight and/or obesity, linked to the metabolic syndrome. GlyH is a rare but underdiagnosed complication hallmarked by extremely brittle metabolic control in, often young, individuals with T1D. Both hepatopathies share clinical similarities, troubling both diagnosis and differentiation. Since NAFLD is increasingly associated with cardiovascular and chronic kidney disease, whereas GlyH is considered self-limiting, awareness and differentiation between both condition is important in clinical care. The exact pathogenesis of both hepatopathies remains obscure, hence licensed pharmaceutical therapy is lacking and general awareness amongst physicians is low. This article aims to review the factors potentially contributing to fatty liver disease or glycogen storage disruption in T1D. It ends with a proposal for clinicians to approach patients with T1D and potential hepatopathy.

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Acute and Relapsing Hepatitis Caused by Glycogenic Hepatopathy

Abstract: Conflicts of interest This author discloses the following: Todd H. Baron has been a speaker and consultant with Medtronic, BSCI, Cook, and Olympus. The remaining authors disclose no conflicts.

Cited by 7 publications

References 2 publications

Glycogenic hepatopathy: A narrative review

Glycogenic hepatopathy: A narrative review

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

Hepatopathy Associated With Type 1 Diabetes: Distinguishing Non-alcoholic Fatty Liver Disease From Glycogenic Hepatopathy

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