Herzer Kerstin, Post Felix, Canbay Ali, Gerken Guido
Zentrum für Innere Medizin, Gastroenterologie und Hepatologie, Essen, Germany.
Med Klin (Munich). 2010 Dec;105(12):916-23. doi: 10.1007/s00063-010-1157-9. Epub 2011 Jan 16.
Patients suffering from severe chronic liver disease, in particular cirrhosis, are at risk for pulmonary complications. The leading clinical symptom is shortness of breath, which can accompany the actual disease as indirect effect because of anemia, faint muscles or ascites. On the other hand, dyspnea can have multiple additive causes in case of accompanying cardial or pulmonary disease. The hepatopulmonary syndrome (HPS) and the portopulmonary hypertension (PoPH) belong to the most relevant pulmonary complications in liver cirrhosis. HPS appears to be more common than PoPH and the presence of either entity increases morbidity and mortality in patients with liver disease. The two diseases have to be strictly distinguished, as they have opposed histological and pathophysiological origin. While the HPS is a dilatative pulmonary- vascular disease, the PoPH is a constrictive or obliterative pulmonary-vascular disease in the context of a liver disease or a portal hypertension. Therefore, these diseases are separate entities also when it comes to diagnostics and therapy.
患有严重慢性肝病,尤其是肝硬化的患者有发生肺部并发症的风险。主要临床症状是呼吸急促,由于贫血、肌肉无力或腹水,它可能作为间接影响伴随实际疾病出现。另一方面,如果伴有心脏或肺部疾病,呼吸困难可能有多种叠加原因。肝肺综合征(HPS)和门脉性肺动脉高压(PoPH)属于肝硬化中最相关的肺部并发症。HPS似乎比PoPH更常见,任何一种情况的存在都会增加肝病患者的发病率和死亡率。这两种疾病必须严格区分,因为它们有相反的组织学和病理生理学起源。虽然HPS是一种扩张性肺血管疾病,但PoPH是在肝病或门静脉高压背景下的一种收缩性或闭塞性肺血管疾病。因此,在诊断和治疗方面,这些疾病也是不同的实体。