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

Abstract

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.

Prevalence of elevated liver enzymes and its association with type 2 diabetes: A Descriptive Cross-Sectional Study among Nepalese Adults from Biratnagar, Nepal

“…13 Some studies reported that accumulation of intracellular glycogen in hepatocytes leads to liver injury with typical biochemical findings of mild to moderate rise in ALT, AST, and normal synthetic function with or without ALP elevation. 14 There are several previous reports from various parts of the world that showed altered liver parameters among the diabetic subjects. There is lacking such study in the Nepali population.…”

Conditions for maintenance of hepatocyte differentiation and function in 3D cultures

“…The PLIN2 staining confirmed the steatotic phenotype after cultivation in hyperglycemic media ( Figure 2 B) and its absence in normo-glycemic media ( Figure 2 E). Further, an increase of hollow spherical structures, typical of glycogen deposits ( Aluko et al., 2020 ), was found in the hyperglycemic cultures ( Figure 2 A).…”

Advances in paediatrics in 2019: current practices and challenges in allergy, endocrinology, gastroenterology, public health, neonatology, nutrition, nephrology, neurology, respiratory diseases and rheumatic diseases

“…It has been defined in various ways: liver glycogen storage, hepatic or liver glycogenosis, DM-associated glycogen storage hepatomegaly and lately "glycogenic hepatopathy" (GH). Real incidence and prevalence of GH are unknown, commonly being misdiagnosed or underdiagnosed [37]. However, if we consider some series of patients, we find that about 10% of diabetic children had hepatic hyperechogeneity decreasing after a period of several months of better adherence to therapy [38].…”