Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus
A 22-year old female was referred to a hepatologist for evaluation of elevated liver enzymes. She had haven Type I DM for 8 years. When she visited our hospital one month ago, her liver function test had showed abnormal aspartate aminotransferase (AST) and alanine aminotransferase (ALT) as 169 U/L (normal value <35) and 172 U/L (normal value <35), respectively. Four weeks later, at the time of the referral, her AST and ALT were elevated strikingly as 1028 U/L and 365 U/L. Other laboratory findings were total bilirubin (TB) 0.5 mg/dL (normal value <1.3), Alkaline phosphatase (ALP) 346 U/L (normal value <85), γ-glutamyl transpeptidase (γ-GT) 321 U/L (normal value <40), hemoglobin A1c (HbA1c) 13.8% (normal value <6), triglyceride(TG) 160 mg/dL, high-density lipoprotein (HDL) 114 mg/dL creatine kinase (CK) 127 UL (normal value <220). Auto-antibody including anti-nuclear antibody (ANA), smooth muscle antibodies (ASMA) and mitochondrial antibodies (AMA) were all negative. Thyroid function test and serum ceruloplasmin were also normal. The serologic findings for viral infection such as IgM anti-HAV, HBsAg, anti-HCV, HCV-RNA, cytomegalovirus and Epstein-Barr virus were also all negative. Her height, weight, body mass index (BMI) and waist circumference were 165.1 cm, 50.8 kg, 18.6 kg/m2 and 72 cm respectively. Abdominal Ultrasonography (US) showed just a mild fatty change of liver and mild hepatomegaly (). She was not an alcohol or drug consumer and was not taking any medication except insulin. In addition, she did not show any symptom and sign compatible with acute hepatitis. So, we recommend her for a liver biopsy to elucidate the cause of acute elevation of liver enzyme. In hematoxylin and eosin (H&E) stain, liver biopsy showed normal liver architecture with no evidence of hepatocellular injury, inflammation, fibrosis, or steatosis. However, the hepatocytes were diffusely swollen with pale cytoplasm. The periodic acid-Schiff (PAS) staining showed abundant glycogen accumulation within hepatocytes. So, histologically, it was diagnosed as a glycogenic hepatopathy (). Eight weeks later, her transaminases were drooped suddenly without any special management. After this event, although we tried to control her sugar level strictly, her sugar control was still poor and HbA1c was maintained above 11% with mild fluctuation. Until now, her liver enzymes also have shown severe fluctuation continuously as like relapsing hepatitis ().