An enlarging liver in a young diabetic male.


Question: A 22-year-old man with history of type 1 diabetes mellitus, alpha-1-antitrypsin deficiency (ZZ phenotype), gastroesophageal reflux disease, hyperlipidemia, and depression presented to the hospital for increasing right upper quadrant pain and nausea for 2 weeks. The patient was a former heroin user and an active smoker, but denied significant alcohol use. The patient’s home medications included insulin, atorvastatin, dexlansoprazole, paroxetine, buprenorphine, and trazodone. The patient had poorly controlled diabetes, requiring 6 hospitalizations for diabetic ketoacidosis within the last year. Physical examination revealed a soft, nondistended abdomen with diffuse tenderness and severe hepatomegaly without ascites, jaundice, spider angioma, or other stigmata of advanced liver disease. Laboratory studies showed an alanine aminotransferase of 223 U/L, aspartate aminotransferase of 331 U/L, alkaline phosphatase of 223 U/L, total bilirubin of 0.3, albumin of 3.4, platelet count of 302 U/L, International Normalized Ratio of 0.9, and hemoglobin A1C of 14.6%. Hepatitis B and C serologies were negative and the alpha-1-antitrypsin level was <60 mg/dL. MRI revealed a significantly enlarged liver with marked interval increase of 21.2 to 25.8 cm from 8 months prior. No focal hepatic lesions were identified. There was no intrahepatic or extrahepatic biliary ductal dilatation (Figure A, B). A core biopsy of the liver was performed showing hepatocytes are swollen with cleared cytoplasm (Figure C). Figure D, E, shows hepatocytes are strongly and diffusely positive for Periodic acid–Schiff stain and largely negative for Periodic acid–Schiff diastase stain suggesting that the hepatocytes were swollen with glycogen that was digested with diastase. Periodic acid–Schiff diastase also highlights alpha1-antitrypsin globules. Based on the clinical scenario, imaging, and pathologic findings, what is the diagnosis? See the Gastroenterology web site ( for more information on submitting your favorite image to Clinical Challenges and Images in GI.

DOI: 10.1053/j.gastro.2015.02.024

Cite this paper

@article{Xu2015AnEL, title={An enlarging liver in a young diabetic male.}, author={Jason Xu and Deepti Dhall and Vinay Sundaram}, journal={Gastroenterology}, year={2015}, volume={149 6}, pages={e8-e10} }