The quantitative and/or qualitative distribution of liver iron was assessed in 81 transfusion-dependent thalassemia major patients with chronic liver disease (36 with chronic active hepatitis, 23 with chronic persistent hepatitis, 22 with siderosis). Viral marker studies showed only 3 cases with both HBsAg and anti-HBc posiüvity in the serum, while the others had anti-HBc and anti-HBs or only anti-HBs or no B viral markers. A significantly higher iron overload was found in chronic hepatitis, particularly chronic active hepatitis, than in siderosis. This increased iron overload may be due to less intensive chelation treatment, higher intestinal absorption secondary to lower mean Hb levels, and/or to liver inflammation-depedent iron deposition. The liver iron overload in turn may facilitate the development or persistence of chronic progressive liver disease.

We evaluated the agreement between wedged hepatic vein pressure (WHVP), portal vein pressure (PVP), and its relationship with portal hemodynamics in 21 patients with HCV-related cirrhosis with esophageal varices. Direct measurements of the portohepatic gradient (HVPG) were obtained by ultrasound-guided fine needle puncture of the right hepatic and the portal veins. In five cases PVP was 6.4-10.4 mm Hg higher than WHVP. In 12 cases measurements were similar (WHVP - PVP < or = 3 mm Hg). In the remaining four cases WHVP was 3.6-9.6 mm Hg higher than PVP. WHVP and PVP agreement was not related to HVPG mean value, Child-Pugh score, or grading of esophageal varices. By contrast, the difference between WHVP and PVP was inversely related to the portal flow velocity (P = 0.053) and directly related to the portal vascular resistance (P = 0.02). Whereas the portal branches were visualized in patients with WHVP lower or similar to PVP, a predominant left portosystemic collateral flow was observed in patients with WHVP > PVP. Our data point out that, in patients with cirrhosis due to hepatitis C virus infection, discrepant HVPG values reflect true hemodynamic differences.

In this study maximum urinary iron elimination with continuous desferrioxamine subcutaneous infusion was obtained in thalassemia major patients with chronic persistent or active hepatitis with lower doses (60 mg/kg) than those necessary in patients without hepatitis (80 mg/kg). Since dose-response curves were highly variable the treatment schedule should be tailored to the individual needs of each patient. Both groups may achieve iron balance but chronic hepatitis patients have more frequently a net urinary iron excretion. In patients with chronic hepatitis no correlation was found between serum ferritin levels or serum ferritin/aspartate aminotransferase ratios and transfusional iron overload while serum ferritin/aspartate aminotransferase ratios were seen to be correlated with liver iron stores.

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