We report on the observation of a 26-year-old woman with portal vein obstruction, diagnosed at the age of 3, and liver iron overload. Celiac and superior mesenteric angiography showed large and multiple venous collaterals between the portal and caval systems. Liver biopsy demonstrated, on Perl's staining, an important hemosiderin deposition, confirmed by an increased hepatic iron concentration (15.6 mumol/100 mg dry weight). No other histologic abnormality was found. This report suggests that large spontaneous portosystemic shunting may stimulate hepatic iron deposition in an otherwise normal liver. This mechanism could, at least in part, explain the significant hepatic siderosis observed in some cirrhotic patients.
An increase in lower oesophageal sphincter pressure induced by domperidone has previously been reported to decrease superior portosystemic collateral flow in patients with portal hypertension owing to cirrhosis. Although a 10-mg intravenous dose of domperidone was effective in increasing lower oesophageal sphincter tone in a group of six patients with cirrhosis, the same dose failed to affect azygos blood flow in a matched group of six patients. The results do not support the hypothesis that an increase in lower oesophageal sphincter tone can decrease flow through oesophageal varices in patients with cirrhosis.
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