Sickle Cell Disease in the Kingdom of Saudi Arabia: East and West

Abstract

Letters to the EditorSickle Cell Disease in the Kingdom of Saudi Arabia: East and West Adebayo OpawoyeMD Adebayo Opawoye Department of Pediatrics, Faifa General Hospital, Faifa, Gizan, Saudi Arabia Search for more papers by this author Published Online:1 May 1999https://doi.org/10.5144/0256-4947.1999.281SectionsPDF ToolsAdd to favoritesDownload citationTrack citations ShareShare onFacebookTwitterLinked InRedditEmail AboutIntroductionTo the Editor. In his editorial, Dr. Pearson1 mentions that “it is well known that the sickle cell disease seen in many patients from the Arabian Gulf area has distinct clinical and electrophoretic differences from that observed in the United States and Africa.” This may hold true only in some of the Arabian Gulf areas, but not in the Kingdom of Saudi Arabia.It has been widely reported here by Al-Awamy,2 El-Hazmi,3 Sergent,4 and Opawoye,5’6 that there are two distinct types of sickle cell anemia in the Kingdom. In the Eastern Province, the Saudi-Indian haplotype has been identified, and in the southern regions, the Benin haplotype has been found among Africans and Black Americans. The studies showed that, in general, the sickle cell disease in the Eastern Province runs a milder clinical course. The mildness in symptoms is attributed to the protective effect of the high level of HbF, and also partly because of the co-existence of α-thalassemia, while in the other provinces, especially the Western/Southwestern provinces, a severe form of sickle cell anemia was encountered with features similar to those reported in communities in Africa and the US. The majority of the patients from these regions of Saudi Arabia suffered from severe and frequent episodes of crises, required blood transfusions frequently, and some suffered from hand and foot syndromes.I agree with Dr. Pearson that in an area such as the Western/Southwestern provinces of the Kingdom where the Benin haplotype of sickle cell disease is found, the need for neonatal screening of the disease cannot be overemphasized.ARTICLE REFERENCES:1. Pearson HA. "Sickle cell disease in the Kingdom of Saudi Arabia: East and West" . Ann Saudi Med. 1998; 18:287-8. Google Scholar2. Al-Awamy BH. "Sickle cell anemia: its clinical manifestations and therapeutic management" . Saudi Med J. 1987; 8:553-62. Google Scholar3. El-Hazmi MAF. "Hemoglobinopathies, thalassemias and enzomyo-pathies in Saudi Arabia" . Saudi Med J. 1992; 13:483-8. Google Scholar4. Sergent GR. "The geography of the sickle cell disease: opportunities for understanding its diversity" . Ann Saudi Med. 1994; 4:237-46. Google Scholar5. Opawoye AD. "Sickle cell anemia in Faifa" . Practioner (East Medit edition). 1994; 6:460-2. Google Scholar6. Opawoye AD, et al.. "Bacterial infections in sickle cell pediatric patients" . Practioner (East Medit edition). 1996; 7:225-7. Google Scholar Previous article Next article FiguresReferencesRelatedDetails Volume 19, Issue 3May 1999 Metrics History Published online1 May 1999 InformationCopyright © 1999, Annals of Saudi MedicinePDF download