The radiological appearances of thalassaemia

Talasemi Osteoporozunda Zoledronik Asit Kullanımı: İki Olgu Sunumu ve Literatürün Gözden Geçirilmesi

“…Bu nedenle transfüzyonlar, şelasyon ve seks hormonlarıyla optimal tedaviye rağmen jeneralize osteoporoz ve osteopeni sık görülmektedir (1). Talasemiye bağlı osteoporoz, uygun transfüzyon ve demir şelasyon tedavisine rağmen talasemi majörlü hastaların %30-50'sinde görülmektedir (6,8).…”

“…Demir aşırı yükünü ortadan kaldırmak için önerilen desferrioksamin şelasyon tedavisi ise, DNA sentezini, osteoblast ve fibroblast proliferasyonunu, osteoblast prekürsör farklılaşmasını ve kollajen formasyonunu inhibe etmekte ve osteoblast apopitozunu arttırmaktadır. Ayrıca kemik iliği ekspansiyonu, vitamin C ve vitamin D eksiklikleri, fizik aktivite azlığı, artmış osteoklast fonksiyonu ve azalmış osteoblast fonksiyonu da talasemi majördeki osteoporoz nedenleri arasında sıralanmaktadır (1,8,9,11).…”

What We Learn from Bone Complications in Congenital Diseases? Thalassemia, an Example

“…They say , Deferoxamine (DFX) also exacerbates the observed growth retardation. DFX-induced skeletal dysplasia, may cause toxicity, which is associated with visual and auditory impairment (Tyler et al, 2006). Among the spinal deformities observed, Papanastasiou et al suggest that an increased prevalence of frontal curves was reported of at least, 5° in 67% of patients with TM.…”

“…They suggest that even with an optimal transfusion regimen, the bone marrow remains hyperactive. The appearance of "cob-webbing" in the pelvis is the reason of the expanded bone marrow that destroys the medullary trabeculae with initial cortical and trabecular thinning and subsequent trabecular coarsening (Tyler et al, 2006). Salehi et al, even reported spinal cord compression that is seen in these patients, which can cause neurologic compromise and is, in part, due to extramedullary hematopoiesis (Salehi et al, 2004).…”

“…Tyler et al suggest that the skeletal changes in untreated thalassemia are due to ineffective erythropoiesis and expansion of the bone marrow which affect every part of the skeleton. These changes include osteoporosis, growth retardation, platyspondyly and kyphosis (Tyler et al, 2006). Anemia, hemosiderosis, iron chelation therapy, and associated hormonal disorders, are described as main causes of spinal deformity (Haidar et al, 2011).…”