The features of sickle cell disease in Saudi children

Journal of Tropical Pediatrics

Volumn 36, Issue 4, 1990, Pages 148-155

  El hazmi, M A F ^a   Bahakim, H M ^a   Al swailem, A M ^b   Warsy, A S ^c  

^a KING SAUD UNIVERSITY   (Saudi Arabia)

^b MINISTRY OF HEALTH   (Saudi Arabia)

^c KING SAUD UNIVERSITY   (Saudi Arabia)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA THALASSEMIA; ARTICLE; BETA THALASSEMIA; CHILD; CLASSIFICATION; DISEASE ASSOCIATION; DISEASE SEVERITY; GENE DELETION; HUMAN; MAJOR CLINICAL STUDY; SAUDI ARABIA; SICKLE CELL ANEMIA;

ANEMIA, SICKLE CELL; CHILD; CHILD, PRESCHOOL; HEMATOLOGIC TESTS; HOMOZYGOTE; HUMAN; INFANT; PROSPECTIVE STUDIES; RETROSPECTIVE STUDIES; SAUDI ARABIA; SUPPORT, NON-U.S. GOV'T; THALASSEMIA;

EID: 0025182059     PISSN: 01426338     EISSN: None     Source Type: Journal    
DOI: 10.1093/tropej/36.4.148     Document Type: Article

References (34)

1. Lehmann H, Maranjin G, Mourant AE. Distribution ofsickle cell haemoglobin in Saudi Arabia. Nature 1983;198: 492-3.
2. El-Hazmi MAF. Hemoglobin disorders: a pattern forthalassaemia and haemoglobinopathies in Arabia. ActaHaematol 1982;68:43-51.
3. El-Hazmi MAF. Abnormal hemoglobins and allied disorders in the Middle East-Saudi Arabia. In: BowmanJE (ed.) Distribution and evolution of hemoglobin andglobin loci 1983; 239-49.
4. El-Hazmi MAF. The distribution and nature of hemoglobinopathies in Arabia. In: Winter WP (ed.) Hemoglobin variants in human populations. Boca Raton, Florida: CRC Press Inc., 1987; 65-77.
5. El-Hazmi MAF. Haemoglobinopathies, thalassaemiasand enzymopathies in Saudi Arabia: the present status.ActaHaemat 1987; 78: 1311.
6. Perrine RP, Brown MJ, Oegg JB, Weatherall DJ, MayA. Benign sickle cell anaemia. Lancet 1972; 2: 1163-7.
7. Gelpi AP. Sickle cell disease in Saudi Arabia. Acta Hae-mat 1970; 43: 88-99.
8. Perrine RP, John P, Pembrey M, Perrine S. Sickle celldisease in Saudi Arabs in early childhood. Arch DisChildh 1981; 56: 187-92.
9. El-Hazmi MAF, Jabbar FA, Al-Faleh FZ, Al-Swailem AR, Warsy AS. The haematological, biochemical and clinical presentation of haemoglobin S in SaudiArabia-(i) Haematological and clinical expression.Trop geogr Med 1987; 39: 157-62.
10. Perrine RP, Pembrey ME, John P, Perrine S, Shoup F.Natural history of sickle cell anaemia in Saudi Arabia: astudy of 270 subjects. Ann Intern Med 1978; 88: 1-6.
11. El-Hazmi MAF, Warsy AS. On the nature of sickle celldisease in the South-Western Province of Saudi Arabia.Acta Haematol 1986; 76: 212-15.
12. Acquaye JK, Omer A, Ganeshagura K, Sejeny SA, Hoffhrand AV. Non-benign sickle cell anaemia in western Saudi Arabia. Br J Haemat 1985; 60: 99-108.
13. Babiker MA, Taha SA. Two different patterns of sicklecell disease in children in Saudi Arabia. Ann Trop Pae-diat 1982; 2: 179-81.
14. Kunkel LM, et al. Analysis of human Y-chromosome-specific reiterated DNA in chromosome variants. ProcNat Acad Sci USA 1977; 74: 1245-9.
15. Southern EM. Detection of specific sequence amongDNA fragments separated by gel electrophoresis. J MolBiol 1975; 98: 503-17.
16. Jeffreys AJ, Flavell RA. A physical map of the DNAregions flanking the rabbit β-globin. Cell 1977; 12: 429-39.
17. El-Hazmi MAF. Thalassaemia in Saudi Arabia: deletion pattern. Hum Genet 1987; 76: 196-8.
18. Marengo-Rowe AJ. Rapid electrophoresis and quantitation of haemoglobins on cellulose acetate. J Clin Path1965; 18: 790-2.
19. Robinson AR, Robson M, Harrison AP, Zuelzer WW.A new technique for differentiation of haemoglobin.J Lab Clin Med 1957; 50: 745-52.
20. Betke K, Marti HR, Schlicht L. Estimation of small percentage of fetal haemoglobin. Nature Lond 1959: 184:1877-8.
21. England JM, Fraser PM. Differentiation of iron deficiency from thalassaemia trait by routine blood count.Lancet 1973; 1: 449-52.
22. Serjeant GR. Sickle cell disease. Oxford: Oxford University Press 1985.
23. Weatherall DJ, Clegg JB, Blankson J. A new sicklingdisorder resulting from interaction of the genes forhaemoglobin S and a-thalassaemia. Br J Haemat 1969;17: 517-26.
24. Weatherall DJ, Clegg JB. The thalassaemia syndrome, 3rd edn. Oxford: Blackwell Scientific Publications 1981.
25. Pembrey ME, Wood WG, Weatherall DJ, Perrine RP.Fetal haemoglobin production and the sickle cell gene inthe oasis of Eastern Saudi Arabia. Br J Haematol 1978;40:415-29.
26. Powers DR, Weiss JN, Chan LS, Schroeder WA. Isthere a threshold level of fetal haemoglobin that ameliorates morbidity in sickle cell anaemia? Blood 1984; 63:921-6.
27. Wasi P, Disthasangchan P, Na-Nakom S. The effect ofiron deficiency on the level of Hb A2 and E. J Lab ClinMed 1968;71:85-91.
28. Steiner J, Marti HR, Dean D. Decreased haemoglobinA2 concentration in iron deficiency anemia. Acta Haematol 1971; 45: 77-81.
29. Kan YM, Dozy AM. Polymorphism of DNA sequenceadjacent to the human globin structural gene. Relationship to sickle mutation. Proc Nat Acad Sci USA1978; 75: 5631-5.
30. El-Hazmi MAF. Xmn I polymorphism in the y-globingene region among Saudis. Hum Hered 1981; 39: 12-19.
31. El-Hazmi MAF. /f-globin gene polymorphism in theSaudi Arab population. Hum Genet 1986; 73: 31-4.
32. Wainscoat JS, et al. A genetic marker for elevated levelof haemoglobin F in homozygous sickle cell disease. Br JHaemat 1985; 60:215-68.
33. Miller BA, et al. Molecular analysis of the high haemoglobin F phenotype in Saudi Arabian sickle cell anaemia. New Engl J Med 1987; 316: 244-50.
34. El-Hazmi MAF. Globin gene haplotypes in the Saudisickle cell anaemia patients (Hum. Hefed, 1990; (in press))