Complications of β-thalassemia major in North America

摘要: Treatment of patients with beta-thalassemia major has improved dramatically during the past 40 years; however, current clinical status these remains poorly characterized. We performed a cross-sectional study 342 in Registry National Institutes Health-sponsored Thalassemia Clinical Research Network. Evidence hepatitis C exposure was present 35% tested patients, associated age, and had rate spontaneous viral clearance 33%. Ferritin levels ranged from 147 to 11 010 ng/mL (median, 1696 ng/mL). Median hepatic iron content 7.8 mg/g dry weight 23% values 15 or higher. No years younger 5% aged 16 24 heart disease requiring medication. Ten percent cirrhosis on biopsy. Endocrinologic complications were common among adults. Seventy-four (22%) recent implantable central venous access devices (CVADs) placed. Among 80 episodes bacteremia 38 90% attributable CVAD. 330 who received deferoxamine chelation therapy, 224 (68%) reported no complications. conclude that C, iron-related organ dysfunction, therapy are strongly age-dependent North American beta-thalassemia.