α:Non–α and Gγ:Aγ globin chain ratios in thalassemia intermedia patients treated with hydroxyurea
| Autor: | Masoumeh Rezaei Moshaei, Haleh Akhavan Niaki, Seyyedeh Masoumeh Eslami, Ramin Ataee, Ladan Hosseini Gouhari, Hassan Abolghasemi, Ali Ahmadi, Amir Sasan Mozaffari Nejad, Abdol Ali Ebrahimi, Mohsen Asouri, Abbas Najjari |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: |
medicine.medical_specialty
Pediatrics Globin chain lcsh:Arctic medicine. Tropical medicine lcsh:RC955-962 Thalassemia Biochemistry Genetics and Molecular Biology (miscellaneous) Clinical Research Internal medicine hemic and lymphatic diseases Fetal hemoglobin Medicine Hydroxyurea Globin Hemoglobin lcsh:QH301-705.5 AUT-PAGE business.industry Thalassemia intermedia Therapeutic effect medicine.disease Endocrinology Mechanism of action lcsh:Biology (General) medicine.symptom business |
| Zdroj: | Asian Pacific Journal of Tropical Biomedicine, Vol 4, Iss S1, Pp S177-S185 (2014) |
| ISSN: | 2221-1691 |
| Popis: | Objective To elucidate the possible ways by which hydroxyurea molecules affect globin chain (α or β-like) synthesis. Methods A total of 23 thalassemia intermedia patients (13 male and 10 female) aged between 5 and 26 years were treated for five months with 15 mg/(kg·day) of hydroxyurea. Hemoglobins electrophoresis and globin chain electrophoresis was performed on each sample at different time points before and during the treatment. Results Fetal hemoglobin increased significantly in most patients and average episodes of transfusion decreased. Both Gγ and Aγ-globin chains increased significantly and α-globin:Nonα-globin chain as well as Gγ-globin:Aγ globin chains ratios decreased. Conclusions Improvement in α:non-α ratio and consequent decrease of free α-globin chain might be the cause of beneficial effects of hydroxyurea therapy. Two patients who felt better didn't show significant increase in their fetal hemoglobin level, and this is in contradiction with the hypothesis claiming that the HbF level increase is the cause of such therapeutic effect. In spite of the unclear mechanism of action of this drug, hydroxyurea therapy had noticeable impacts on thalassemia intermedia and also sickle cell disease and even patients suffering from thalassemia major. |
| Databáze: | OpenAIRE |
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