Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

At the present time, the only treatment available to correct the anemia in Cooley's disease is the use of blood transfusions. The patients maintained at a higher baseline level of Hb (Hypertransfusion Regimen = HTR, with Hb levels of 8 divided by 10 g/100 cm3) are in general, in better health and have smaller organs: particularly, the changes in the skull and in other bones become less pronounced and may recede. The authors have reviewed in 77 patients with beta-thalassemia syndromes a spectrum of rib changes, including widening, osteoporosis with or without localized lucencies, "rib-within-a-rib" appearance, subcortical lucency and extramedullary hematopoietic masses. These radiological changes are explained on the basis of the relationship of the proliferating marrow to the medulla, cortex and periosteum. The type of abnormalities depends on the type of transfusion regimen. HTR begun early in life will prevent the development of any abnormal rib changes; on the other hand, in the majority of cases, abnormal findings did not regress with LTR (= Low Transfusion Regimen, with Hb level of 4 divided by 6 g/100 cm3).
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PMID:[The ribs in beta-thalassemia. Course of the changes in relation to transfusion therapy]. 667 51

In 32 patients with thalassemia the spectrum of rib changes, including widening, osteoporosis, localized lucencies, cortical erosions, "rib-within-a-rib" appearance, subcortical lucency, and extramedullary hematopoiesis, is reviewed. This material is supplemented by the radiographic and computed tomographic findings in a specimen of spine, posterior ribs, and extramedullary hematopoietic masses. The relevant literature on thalassemia, including two cases of "costal osteoma," is reviewed. A mechanism is offered to explain the various rib changes based on (a) the relationship of the proliferating marrow to the medulla, cortex, and periosteum, (b) the patient's age at the onset of a transfusion regimen, and (c) the type of transfusion regimen used.
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PMID:The ribs in thalassemia. II. The pathogenesis of the changes. 728 Feb 34