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Pediatr Endocrinol Rev ; 6 Suppl 1: 123-6, 2008 Oct.
Article de Anglais | MEDLINE | ID: mdl-19337165

RÉSUMÉ

The modern radiologist is unlikely to encounter the classic radiographic features of thalassemia other than in teaching files. The main pathological change that leads to radiological skeletal changes in beta thalassemia major is extensive marrow proliferation. The severity of the skeletal responses is related to the type of thalassemia, the extent and duration of the disease, the type of treatment and the volume of blood transfusions given to the patient, as well as the side effects of transfusion-chelation therapy, and also depends on the bone involved. The radiographic features can be divided into those affecting the skeleton (axial and appendicular) and those occurring extra-medullary. Axial skeletal changes mainly include skull and facial bones, paranasal sinuses, vertebral bodies, weight-bearing bones, while appendicular skeleton manifestations are more pronounced in peripheral bones, mainly hands and feet as well as ribs. Patients on repeated blood transfusions and iron-chelation therapy may demonstrate variable range of manifestations than the scope of untreated patients.


Sujet(s)
Os et tissu osseux/anatomopathologie , bêta-Thalassémie/anatomopathologie , Os et tissu osseux/imagerie diagnostique , Humains , Agents chélateurs du fer/effets indésirables , Radiographie , Réaction transfusionnelle , bêta-Thalassémie/imagerie diagnostique , bêta-Thalassémie/thérapie
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