[Portal hypertension in idiopathic haemochromatosis (author's transl)].

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.