β-Thalassemia Distribution in the Old World: an Ancient Disease Seen from a Historical Standpoint
De Sanctis, Vincenzo; Kattamis, Christos; Canatan, Duran; Soliman, Ashraf T; Karimi, Mehran; Daar, Shahina; Wali, Yasser; Yassin, Mohamed; Soliman, Nada; Sobti, Praveen; Al Jaouni, Soad; mohamed, elkholy; Fiscina, Bernadette; Angastiniotis, Michael; Elsedfy, Heba;
Abstract
Haemoglobinopathies constitute the commonest recessive monogenic disorders worldwide, and the treatment of affected individuals presents a substantial global disease burden. β-thalassaemia is characterised by the reduced synthesis (β+) or absence (βo) of the β-globin chains in the HbA molecule, resulting in accumulation of excess unbound α-globin chains that precipitate in erythroid precursors in the bone marrow and in the mature erythrocytes, leading to ineffective erythropoiesis and peripheral haemolysis. Approximately 1.5% of the global population are heterozygotes (carriers) of the β-thalassemias; there is a high incidence in populations from the Mediterranean basin, throughout the Middle East, the Indian subcontinent, Southeast Asia, and Melanesia to the Pacific Islands.
Other data
Title | β-Thalassemia Distribution in the Old World: an Ancient Disease Seen from a Historical Standpoint | Authors | De Sanctis, Vincenzo; Kattamis, Christos; Canatan, Duran; Soliman, Ashraf T; Karimi, Mehran; Daar, Shahina; Wali, Yasser; Yassin, Mohamed; Soliman, Nada; Sobti, Praveen; Al Jaouni, Soad; mohamed, elkholy ; Fiscina, Bernadette; Angastiniotis, Michael; Elsedfy, Heba | Keywords | Ancient disease;Old world;Thalassemia distribution | Issue Date | 1-Jan-2017 | Publisher | MATTIOLI 1885 | Journal | Mediterranean Journal of Hematology and Infectious Diseases | Volume | 9 | Issue | 1 | ISSN | 2035-3006 | DOI | 10.4084/MJHID.2017.018 | PubMed ID | 28293406 | Scopus ID | 2-s2.0-85014656068 | Web of science ID | WOS:000397708300001 |
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