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ICL670(一种每日一次口服铁螯合剂)在β地中海贫血输血性铁过载患者III期临床试验中的评估。

Evaluation of ICL670, a once-daily oral iron chelator in a phase III clinical trial of beta-thalassemia patients with transfusional iron overload.

作者信息

Galanello Renzo

机构信息

Ospedale Regionale Microcitemie, Via Jenner s/n, 09121 Cagliari, Sardinia, Italy.

出版信息

Ann N Y Acad Sci. 2005;1054:183-5. doi: 10.1196/annals.1345.021.

DOI:10.1196/annals.1345.021
PMID:16339664
Abstract

Osteoporosis and osteopenia are frequent complications of thalassemia major (TM) and intermedia (TI). Osteoporosis was found in 23/25 patients with TI and in 115/239 patients with TM. In TM, no association was found with specific polymorphisms in candidate genes (vitamin D receptor, estrogen receptor, calcitonin receptor, and collagen type 1 alpha 1). Osteoporosis in TM female was strongly associated with primary amenorrhea (P < .0001), while in male patients with TM hypogonadism was not significantly related to BMD (P = .0001). Low BMD was also associated with cardiomiopathy (P = .01), diabetes mellitus (P = .0001), chronic hepatitis (P = .0029), and increased ALT (P = .01).

摘要

骨质疏松症和骨质减少是重型地中海贫血(TM)和中间型地中海贫血(TI)的常见并发症。在25例TI患者中有23例发现骨质疏松,在239例TM患者中有115例发现骨质疏松。在TM中,未发现与候选基因(维生素D受体、雌激素受体、降钙素受体和I型胶原α1)的特定多态性有关。TM女性的骨质疏松与原发性闭经密切相关(P <.0001),而在TM男性患者中,性腺功能减退与骨密度无显著相关性(P =.0001)。低骨密度还与心肌病(P =.01)、糖尿病(P =.0001)、慢性肝炎(P =.0029)和谷丙转氨酶升高(P =.01)有关。

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