Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatment for granulomatous hepatitis： A case report and review of the literature

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 granuloma-induced 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 contrast-enhanced echocardiography along with the normalization of cholestatic enzymes and improvement of the patient' s 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.     

进一步重视肝硬化临床诊治工作的系统性和规范性

肝硬化是各种慢性肝病的晚期阶段，其病理学定义为弥漫性肝脏纤维化伴有异常结节形成。从临床角度看，肝硬化主要表现为肝细胞功能衰竭(血清白蛋白降低、胆红素升高、凝血酶原时间延长等)和门静脉高压症(食管胃底静脉曲张及其破裂出血、腹水、自发性细菌性腹膜炎及肝肾综合征、肝性脑病等)。近年有关肝硬化的临床研究尚无突破性进展，目前尚缺乏对肝硬化的特效治疗方法。因此，临床医师更应树立以人为本的观点，为患者制定系统、规范的诊断治疗方案及随访计划。