SummaryThalassaemia patients with genotype 1 or 4 chronic hepatitis C virus (HCV) infection were randomised to receive peginterferon alpha-2a 180 mg/week ribavirin for 48 weeks. Primary efficacy variable was sustained viral response (SVR) at 72 weeks. Thirty-two patients were evaluated; 20 enrolled. Baseline characteristics were comparable. SVR occurred in four of 12 and five of eight patients in the monotherapy and combination groups (30% and 62AE5%; P ¼ 0AE19), respectively. Undetectable RNA at 12 weeks and age <18 years were associated with improved SVR (P < 0AE05). Transfusion requirements rose by 34% in the combination arm (P ¼ 0AE08). Peginterferon/ribavirin was effective in thalassaemics with HCV and moderate iron overload.
Cerebral vasculopathy is common in splenectomized adults with TI. However, large-vessel disease does not explain the occurrence of silent brain infarction. The combined use of MRA and MRI better identifies splenectomized TI adults with neuroimaging abnormalities.
L1 had comparable efficacy as deferoxamine with minimal side effects and better compliance. Provided long term side effects are not encountered, L1 seems to be a valuable alternative iron chelator for patients unable or unwilling to use deferoxamine effectively.
Beta-thalassemia is a hereditary anemia that is quite prevalent in Lebanon. Most patients with beta-Thalassemia are treated and followed up mostly at a multidisciplinary center, located in the suburban area of Beirut: the Chronic Care Center (CCC), operational since 1994. We will review the experience with beta-Thalassemia accumulated through this institution. Four hundred and twenty five patients, aged 2 to 68 years are followed up at the CCC. Sixty four percent have thalassemia major (TM) while 36% have thalassemia intermedia (TI). Lebanese patients with TM receive periodic packed red cell transfusions to maintain a pre-transfusional hemoglobin level of 10 gm/dl at all times and desferrioxamine is the standard iron chelator in use. Since 1994, 12 patients with TM have died from complications of their disease, with heart failure being responsible for the majority of deaths. The incidence of cardiac, endocrinologic, and infectious complications will be reviewed. Finally, both current and prospective preventive measures will be discussed, specifically educational campaigns and premarital screening. The effects of prevention are starting to show as the number of newly diagnosed disease is diminishing.
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