title: Successful Treatment of Rapid Onset, Symptomatic de novo Non-alcoholic Steatohepatitis Following Liver Transplantation: A Case Report.
abstract: A 45 year old female with a body mass index (BMI) of 24 underwent successful liver transplantation (LT) for alcoholic cirrhosis using a donor liver from an obese woman with microvesicular steatosis (80%) and minimal macrovesicular steatosis (5-10%) on liver biopsy. Ascites and hepatosplenomegaly developed soon after LT with progressive increase of serum alkaline phosphatase to 1340 IU/L while aspartate aminotransferase (AST), and alanine transaminase (ALT), and total bilirubin remained normal. Imaging showed marked hepatomegaly, extensive fatty infiltration of the liver, and compression of the hepatic veins with narrowing of the intrahepatic inferior vena cava (IVC). Liver biopsy on post-operative day 39 revealed 90-100% macrovesicular steatosis, steatohepatitis, and portal fibrosis. A hepatic venogram showed a 10 cm segment of intrahepatic IVC stenosis that was stented, improving portal venous pressure measurements. However, portal hypertension requiring diuretic therapy and multiple paracenteses remained. By 3 months after LT, her liver had grown to 22 cm, transaminases increased 2-4 times the upper limit of normal with a 2:1 AST to ALT ratio. Liver biopsy at post-LT day 82 showed no change in steatosis and steatohepatitis despite corticosteroid withdrawal and interval periportal and perisinusoidal fibrosis. 12 weeks after LT, the patient was found to have low apolipoprotein B (65 mg/dL), high-density lipoprotein (HDL) (<10 mg/dL), low-density lipoproteins (LDL) (9 mg/dL), and total cholesterol (<50 mg/dL) levels. Therapy was started for NASH with high dose (800 IU daily) vitamin E and pioglitazone 15 mg daily, and she received topical vegetable oil and oral essential fatty acid supplements. Liver enzymes normalized after 3 months and her lipid profile improved markedly (HDL 27 mg/dL, total cholesterol 128 mg/dL), with progressive decrease in liver size and resolution of ascites after 5 months of therapy. At 2 years post-LT, the liver enzymes remain normal and lipids have normalized.
authors.affiliation: Division of Gastroenterology, Albert Einstein Medical Center, Philadelphia, PA, United States.
authors.forename: Justin R
authors.initials: JR
authors.lastname: Cuschieri
authors.affiliation: Division of Gastroenterology, University of Florida College of Medicine, United States.
authors.forename: Bijo K
authors.initials: BK
authors.lastname: John
authors.affiliation: Department of Pathology, Albert Einstein Medical Center, United States.
authors.forename: Ronald
authors.initials: R
authors.lastname: Miick
authors.affiliation: Division of Transplant Surgery, Albert Einstein Medical Center, United States.
authors.forename: Jorge A
authors.initials: JA
authors.lastname: Ortiz
authors.affiliation: Division of Hepatology and Liver Transplantation, Albert Einstein Medical Center, United States.
authors.forename: Nikroo
authors.initials: N
authors.lastname: Hashemi
keywords: ALT, alanine transaminase; AST, aspartate aminotransferase; BMI, body mass index; CT, computed tomography; EFAD, essential fatty acid deficiency; HDL, high-density lipoproteins; HVPG, hepatic venous pressure gradient; IVC, inferior vena cava; LDL, low-density lipoproteins; LT, liver transplantation; NAFLD; NAFLD, non-alcoholic fatty liver disease; NASH; NASH, non-alcoholic steatohepatitis; PIVENS, pioglitazone versus vitamin E versus placebo for the treatment of non-diabetic patients with non-alcoholic steatohepatitis; PO, per os (oral); liver transplantation; non-alcoholic fatty liver disease; portal hypertension