Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatme

• 作 者: Nikolaos Tzovaras Aggelos Stef
• 机 构: Department of Medicine,Department of Pathology,Division of Cardiology
• 出 处: 世界胃肠病学杂志（英文版）
• 发表日期: 2004年期
• 关键词: Hepatopulmonary syndrome Granulomatous hepatitis Liver cirrhosis Orthodeoxia Pla

Hepatopulmonary syndrome (HPS) is defined as a clinical triad including liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations. We report a 61-year-old male presented with fatigue, long-lasting fever, loss of weight, signs of portal hypertension, hepatosplenomegaly, cholestasis and progressive dyspnoea over the last year. Clinical, laboratory and histological findings confirmed the diagnosis of granulomatous hepatitis. HPS due to hepatic granulomainduced portal hypertension was proved to be the cause of severe hypoxemia of the patient as confirmed by contrast-enhanced echocardiography. Reversion of HPS after corticosteroid therapy was confirmed by a new contrastenhanced echocardiography along with the normalization of cholestatic enzymes and improvement of the patients conditions. This is the first case of complete reversion of HPS in a non-cirrhotic patient with hepatic granuloma,indicating that intrapulmonary shunt in liver diseases is a functional phenomenon and HPS can be developed even in miscellaneous liver involvement as in this case.