Background: Glycogenic hepatopathy (GH) is a well described, yet underdiagnosed disorder in type 1 diabetes. Erratic blood glucose values and high insulin levels promote the excessive deposition of glucose storage in the liver as glycogen, resulting in hepatomegaly, right upper quadrant pain and abnormal liver function. GH was first described with the introduction of insulin as a therapy to treat type 1 diabetes in the early 20th century. As our ability to effectively treat type 1 diabetes mellitus has improved, GH is seen much less commonly. Today, GH generally effects adolescent or young adult patients with poorly controlled type 1 diabetes mellitus and DKA. It is reversible with successful treatment of hyperglycemia.
Clinical Case: An 18 year old woman with a history of poorly controlled type 1 diabetes mellitus and frequent admissions for DKA was admitted for DKA and pyelonephritis. On admission, the patient complained of significant right upper quadrant pain and was found to have elevated transaminase values of: AST 1199 U/L (<37 U/L), ALT 371 U/L (56 U/L), an elevated alkaline-phosphatase of 319 IU/L (<135 IL/L) and normal indices of biosynthetic function (INR/PT). After inpatient treatment of DKA and pyelonephritis, the right upper quadrant pain persisted and required pharmacologic analgesia. Radiographic evaluation demonstrated severe hepatomegaly (24 cm in maximum length) without focal lesions. Laboratory evaluation for viral hepatitis, autoimmune hepatitis, Celiac Disease, Wilson’s Disease and hemochromatosis were unremarkable. Given the patient’s persistent symptoms and severity of hepatomegaly, hepatic biopsy was performed.Biopsy findings were consistent with glycogenic hepatopathy demonstrating steatosis and glycogen deposition with nucleic glycogenation and mega mitochondria.Our patient had higher than usual insulin requirements for type 1 diabetes (~1 unit/kg/day). Abdominal pain, hepatomegaly and elevated LFTs resolved over a 2 month duration with improvement in her blood glucose control. Conclusions: GH is an established yet rare complication of poorly controlled type 1 diabetes. Glycogen deposition in the liver leads to painful hepatomegaly due to stretching of the liver capsule. GH has a female predominance (77%) and is characterized by elevated AST >>ALT with preserved liver biosynthetic function.It is postulated that GH is a result of elevated blood glucose levels and elevated insulin levels. The patient we describe has long standing poorly controlled type 1 diabetes mellitus, frequent admissions for DKA and high insulin requirements. To our knowledge, insulin requirements have not been investigated or previously reported as a potential risk factor for this condition.