THU368 A Rare Case Of Glycogenic Hepatopathy With Persistent Lactic Acidosis

Abstract

Abstract Disclosure: M. Shah: None. S. Challagulla: None. Background: Glycogenic hepatopathy (GH) is an under-recognized complication of poorly controlled diabetes. It is caused by abnormal glycogen accumulation in hepatocytes leading to hepatomegaly and transient elevation of liver transaminases. Diagnosis is often delayed as liver biopsy is needed to distinguish it from other causes of liver injury. Complete resolution can be achieved with intensive glucose control. We present a case of GH diagnosed in a patient who developed increasing liver transaminases and persistent lactic acidosis after resolution of DKA. Clinical Case: We present a 21-year-old female with uncontrolled Type 1 diabetes who presented with fatigue, nausea, and generalized body aches. Patient was hemodynamically stable. Laboratory work up on admission showed a serum glucose of 834 mg/dL, serum bicarbonate less than 5 mEq/L, beta hydroxybutyrate was elevated to 8.7 mmol/L (normal <0.4 mmol/L), lactic acid was also elevated to 7.9 mmol/L (normal <2.0 mmol/L). She was diagnosed with DKA and treated with IV fluids and insulin drip. Over the next five days the patient’s symptoms improved and DKA resolved with beta hydroxybutyrate falling to <0.1 mmol/L. Her lactic acidosis however persisted, she had no apparent infection and no episodes of hypotension to explain this finding. CT scan of the abdomen revealed hepatomegaly not compatible with steatosis. Liver enzymes on admission were mildly elevated AST 57 IU/L and ALT 61 IU/L however values continued to rise and peaked at AST 2372 IU/L and ALT 681 IU/L five days later. Further laboratory studies revealed no evidence of viral hepatitis, no presence of drugs or toxins. Chronic liver disease work up revealed normal ANA, AMA, SPEP, ceruloplasmin, and iron levels. Patient ultimately underwent liver biopsy which revealed glycogenic hepatopathy, mild steatosis, and mild fibrosis. Patient’s lactic acidosis eventually improved with supportive care, and she was discharged. On follow up 3 months later the patient's liver enzymes had returned to normal. Discussion: Glycogenic hepatopathy is a rare diagnosis usually seen in patients with uncontrolled diabetes and elevated liver transaminases. It is hypothesized that pathogenesis of GH involves insulin increasing glycogen synthesis in the liver resulting in congested hepatocytes. Persistent lactic acidosis can also be seen; it has been proposed that increased insulin and dextrose administration prevents gluconeogenesis in the liver, therefore decreased conversion of pyruvate to glucose and increased lactate production. GH is important to understand and differentiate from other causes of liver injury and disease as treatment and prognosis is unique. Prompt liver biopsy should be pursued to elucidate diagnosis of GH. If patients are treated with aggressive glycemic control, hepatopathy can completely resolve. Presentation: Thursday, June 15, 2023