Mörl M, Schwalbach G, Wannagat L
Z Gastroenterol. 1979 Jun;17(6):381-6.
In contrast to hepatic and alcoholic cirrhosis of the liver, the life-threatening portal hypertension seen in idiopathic haemochromatosis (IH) responds well to therapy. With the aid of laparoscopic transhepatic manometry, we determined the pressures in the branches of the portal vein and the hepatic vein in four patients presenting with IH, and compared the values obtained with clinico-chemical and histological parameters. Three patients who had been suffering from the disease for periods varying between two and ten years, responded well to therapy. The fourth patient, who presented with the highest pressure in the portal vein (26.5 mm Hg) died, three months after the initial establishment of the diagnosis of advanced IH, in consequence of unmanagable haemorrhage from oesophageal varices.
与肝性肝硬化和酒精性肝硬化不同，特发性血色素沉着症（IH）中危及生命的门静脉高压对治疗反应良好。借助腹腔镜经肝测压法，我们测定了4例IH患者门静脉分支和肝静脉的压力，并将所得数值与临床化学和组织学参数进行了比较。3例患病时间在2至10年之间的患者对治疗反应良好。第四例患者门静脉压力最高（26.5毫米汞柱），在确诊晚期IH三个月后，因食管静脉曲张无法控制的出血而死亡。
