Department of Radiology, Cairo University, Cairo, Egypt.
Pediatr Radiol. 2013 Sep;43(9):1159-65. doi: 10.1007/s00247-013-2645-x. Epub 2013 Apr 6.
Current thalassemia major treatment includes blood transfusion and iron chelation, which is associated with growth disturbances and radiographic changes in the long bone metaphyses.
To explore and discuss the spectrum of deferoxamine-induced bone-dysplasia-like changes in children with thalassemia major in Egypt.
We studied 59 Egyptian children with thalassemia major and generalized arthralgia. All started deferoxamine treatment at 3 years of age. We conducted skeletal survey and MRI of both knees in radiographically positive children. Each child's age, serum ferritin, age of onset and duration of therapy were compared with the radiologic findings.
Twenty-two (37.3%) children had variable degrees of skeletal dysplasia-like changes similar to those described with deferoxamine intake, mostly around the knees. Mild dysplasia-like changes were seen in 4 (18%) children; moderate changes were seen in 11 (50%) children and severe changes were seen in 7 (31.8%) children. No statistically significant relationships were detected between bone changes and the children's age, age of starting deferoxamine, duration of therapy, or serum ferritin level.
A wider spectrum of deferoxamine-induced bone-dysplasia-like changes was recognized despite delayed onset and small doses of therapy. These changes should be considered as a possible cause of arthropathy in children with thalassemia major, especially symptomatic children.
目前,重型地中海贫血的治疗包括输血和铁螯合,这与长骨干骺端的生长障碍和放射影像学改变有关。
探讨和讨论埃及重型地中海贫血儿童中去铁胺引起的类骨质发育不良变化的谱。
我们研究了 59 名埃及重型地中海贫血和全身性关节炎儿童。所有患者均在 3 岁时开始接受去铁胺治疗。我们对放射影像学阳性的儿童进行了双侧膝关节的骨骼检查和 MRI。比较每个儿童的年龄、血清铁蛋白、发病年龄和治疗持续时间与放射学发现。
22 名(37.3%)儿童有不同程度的类骨质发育不良改变,类似于去铁胺摄入引起的改变,主要发生在膝关节周围。4 名(18%)儿童有轻度类骨质发育不良改变;11 名(50%)儿童有中度改变,7 名(31.8%)儿童有重度改变。骨改变与儿童年龄、开始使用去铁胺的年龄、治疗持续时间或血清铁蛋白水平之间无统计学显著相关性。
尽管发病时间延迟和治疗剂量较小,但仍认识到更广泛的去铁胺引起的类骨质发育不良改变。这些变化应被认为是重型地中海贫血儿童发生关节病的一个可能原因,尤其是有症状的儿童。