Skip Navigation

Journal of Tropical Pediatrics 1993 39(4):209-213; doi:10.1093/tropej/39.4.209
© 1993 by Oxford University Press
This Article
Right arrow Full Text (PDF)
Right arrow E-letters: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when E-letters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by El-Hazmi, M. A. F.
Right arrow Articles by Warsy, A. S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by El-Hazmi, M. A. F.
Right arrow Articles by Warsy, A. S.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

other

On the Molecular Interactions Between {alpha}-thalassaemia and Sickle Cell Gene

M. A. F. El-Hazmi, BSc (Hons), MB, BChir, PhD (Cantab), MRCPath*, and A. S. Warsy, MSc, PhD, FACB**

*Medical Biochemistry Department, College of Medicine, King Saud University Riyadh, Saudi Arabia
**Department of Biochemistry, Centre for Scientific and Medical Studies for Girls, King Saud University Riyadh, Saudi Arabia

Correspondence: Professor Dr M. A. F. El-Hazmi, Medical Biochemistry Department and WHO Collaborating Centre, College of Medicine and King Khalid Hospital, PO Box 2925, Riyadh 11461, Saudi Arabia

Using the restriction endonucleases, Bam HI, Bgl II, Hind III and EcoRI, the {alpha}-gene arrangements were investigated in heterozygotes and homozygotes for the sickle cell haemoglobin (Hb S). In the heterozygotes (Hb AS) group the Hb S level showed a trimodal distribution due to presence of the normal {alpha}-globin genes ({alpha}{alpha}/{alpha}{alpha}) or of one (– {alpha}/{alpha}{alpha}) or two (– {alpha}/–{alpha}) {alpha}-genes deletions. The haematological analytes inversely correlated with the associated {alpha}-thalassaemia ({alpha}-thal.) genes. In the Hb S homozygotes (Hb SS), associated {alpha}-thalassaemia was found to ameliorate the clinical manifestations and improved the haematological values.

Co-existing triple {alpha}-gene arrangement, {alpha}{alpha}{alpha}anti 3.7/, with Hb AS did not influence the haematological analytes. In Hb SS, presence of {alpha}{alpha}{alpha}anti 3.7/, resulted in a severe sickle cell anaemia (SCA) with a high severity index (>11) and with frequent crises, transfusion requirements and hospitalizations. It is suggested that reduced level of {alpha}-chain ameliorates SCA while excess of {alpha}-globin chain production gives rise to a severe form of SCA.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 J. Nutr.Home page
R. T. Jackson and Z. Al-Mousa
Iron Deficiency Is a More Important Cause of Anemia than Hemoglobinopathies in Kuwaiti Adolescent Girls
J. Nutr., May 1, 2000; 130(5): 1212 - 1216.
[Abstract] [Full Text]


Disclaimer:
Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.