LBODP039 Glycogenic Hepatopathy- An Underrecognized Complication Of Diabetes

INTRODUCTION: Glycogenic hepatopathy (GH) is an infrequently described complication of predominantly type 1 diabetes characterized by liver dysfunction and hepatomegaly. This condition is different from the more common nonalcoholic fatty liver disease (NAFLD) which is clinically indistinguishable. Liver biopsy is currently the only way to differentiate the two conditions. We present a 19 year old female with persistent transaminasemia secondary to glycogenic hepatopathy associated with poorly controlled type 1 diabetes. CASE DESCRIPTION: A 19 year old female was admitted with DKA secondary to facial cellulitis. She had a past history of poorly controlled type 1 DM since age 9, proteinuria, and transaminasemia since age 14. Initial labs showed elevated plasma glucose 365 mg/dL, bicarbonate 15 mEq/L, anion gap 22, positive betahydroxybutyrate, AST 208 mmol/L, ALT 136 mmol/L, and HbA1c 12. 0%. She was successfully resuscitated from DKA. Repeat liver function tests showed further elevation of AST 819 mmol/L and ALT 346 mmol/L. Ultrasound of liver showed increased echotexture reported as steatosis. Further history revealed significant financial difficulty due to which patient was using different kinds of basal and bolus insulins interchangeably. She would sometimes use bolus insulin to cover for lack of basal insulin. Prior gastroenterology workup showed ANA positivity in a speckled pattern but was otherwise unrevealing for infective, autoimmune, or alcoholic hepatitis. Liver biopsy was therefore recommended by inpatient gastroenterology. Liver biopsy revealed spotty mild lobular hepatitis and focal portal fibrosis consistent with glycogenic hepatopathy. Features of autoimmune hepatitis were not seen in liver biopsy. DISCUSSION: Glycogen hepatopathy seems to represent an acquired form of glycogen storage disorder. Pathophysiology is suspected to be a combination of hyperglycemia and hyperinsulinemia leading to increased glycogen accumulation in hepatocytes while glycogenolysis is suppressed. This mechanism seems to correlate with our patient who reported use of large doses of short acting insulin followed by glucose administration to counteract hypoglycemia. Abnormal glucagon activity or enzymes deficiencies in glycogenolysis pathway (as reported with congenital glycogenstorage disorders) have not been elucidated. Recent data has also questioned reversibility and long term safety as liver biopsies of patients with glycogenic hepatopathy have demonstrated varying degrees of fibrosis. Newer MRI protocols seem to be able to distinguish GH from NAFLD, and this is important as these are pathophysiologically distinct and will have different management strategies. This case highlights glycogenic hepatopathy as an underrecognized complication of diabetes that will require improved recognition and further investigation to evaluate long term consequences. Presentation: No date and time listed