PMID: 6163399Mar 1, 1981Paper

Sickle cell disease in Saudi Arabs in early childhood

Archives of Disease in Childhood
R P PerrineS Perrine

Abstract

Haemoglobin electrophoresis screening of 2341 infants from the oases of eastern Saudi Arabia, performed in an attempt to detect cases early and then to follow up and give better management to patients with sickle cell disease, showed 20% with S-trait and 43 with sickle cell disease (37 HbSS and 6 S-beta(0) thalassaemia). On follow-up from birth (or from 3 months) for a mean of 3 1/2 years there was more morbidity and mortality than in matched Saudi Arab controls, but these rates were lower than for affected black infants in the US or Jamaica. Serial haemoglobin levels were l g/dl lower than for controls; height and weight increases were the same. Mean fetal haemoglobin level was 32% at 4-5 years. Half of a subsample of 23 cases had evidence of coexistent alpha-thalassaemia which appeared to protect against functional asplenia, judged by the presence of Howell-Jolly bodies; however there was no difference in degree of anaemia or clinical course. Early screening is recommended for infants at risk for sickle cell disease, and comprehensive care should be given even if the infant has the less severe type.

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Citations

Jan 1, 1984·Transactions of the Royal Society of Tropical Medicine and Hygiene·B H Al-AwamyG R Serjeant
Feb 7, 1985·The New England Journal of Medicine·S P PerrineD V Faller
Dec 2, 2010·Annals of Tropical Paediatrics·A-W M Al-SaqladiB J Brabin
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Aug 30, 2008·Annals of Tropical Paediatrics·A-W M Al-SaqladiB J Brabin
Jan 1, 1989·Annals of the New York Academy of Sciences·B A MillerD G Nathan
Apr 20, 2017·The New England Journal of Medicine·Frédéric B PielDavid C Rees
Jun 1, 1995·Journal of the Royal Society of Health·R K PejaverH Bedawi
Oct 14, 2004·Blood·Rona S WeinbergGeorge F Atweh
Jan 29, 1987·The New England Journal of Medicine
Jun 1, 1988·Annals of Tropical Paediatrics·I el ArabiJ Acquaye
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Aug 17, 2004·Annals of Saudi Medicine·Graham R Serjeant, Beryl E Serjeant
May 1, 1995·Annals of Saudi Medicine·I A Al-DabbousA M El-Bashier
Jun 1, 1984·Annals of Tropical Paediatrics·A MallouhM S Ahmad
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Jul 17, 2021·Journal of Pediatric Endocrinology & Metabolism : JPEM·Ugo Nnenna ChikaniIfeoma Emodi

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