2304 Glycogen Hepatopathy: An Unusual Cause of Liver Enzymes Elevation in Diabetic Patients

Abstract

INTRODUCTION: Liver enzyme elevation is a common condition among diabetic patients and often Nonalcoholic Fatty Liver Disease is responsible for this,which can lead to fibrosis and cirrhosis. GH should be one of the differential diagnoses upon exam of a patient with uncontrolled DM with elevated transaminases and hepatomegaly. Compared to other liver diseases associated with DM, GH is a favorable diagnosis due to its benign nature and good prognosis. CASE DESCRIPTION/METHODS: An 18 years old female with type I diabetes had been non compliant with her basal insulin frequently. This had led to 7-8 hospitalizations within last year. This time, she was in the hospital for a ketoacidosis episode with HbA1C of 17%. During P/E, her liver was enlarged. Liver enzymes were, ALP-201 U/L, AST-120 U/L, ALT-125 U/L, GGT 98 U/L).Lipid profile- Cholesterol 315 mg/dl, HDL 50.4 mg/dl, LDL 205 mg/dl, Triglyceride 298 mg/dl. Her USG showed 19 cm liver with normal parenchyma. CT scan reiterated the findings with hyperdense liver. It was noted that the liver enzymes had great fluctuations within the last year, when she was hospitalized due to hyperglycemia ,and her liver enzymes declined when blood sugar was regulated. The patient was examined with regard to chronic hepatitis differential diagnosis since the aminotransferase levels were found to be elevated , intermittently from last year. Serology for viral hepatitis and autoimmune hepatitis had normal results. So, a liver biopsy was performed. Significant Glycogen rich hepatocytes were observed in the tissue, without fibrosis or inflammation. The case was diagnosed with GH due to the present clinical, laboratory, and pathological findings. Patient was advised diet and intensive insulin regimen. Though she remained non compliant. 6 years follow up revealed persistent elevated enzymes with CT scan showing liver size of 21 cm with liver enzymes elevation. DISCUSSION: Glycogenic hepatopathy is a rare complication of the poorly controlled diabetes mellitus characterized by the liver dysfunction with elevated liver enzymes and hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes. One of the essential elements in the pathophysiology of development of GH is the wide fluctuation in both glucose and insulin levels. Future progress is required in understanding the biochemical defects underlying GH and an ideal diagnostic test instead of biopsy to avoid the extensive workup in evaluating suspected cases of GH.