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Cited by 52Hemoglobinopathies and Related DisordersNeonatal Health and BiochemistryBlood groups and transfusion

Sickle cell disease in Saudi Arabs in early childhood

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Perrine, R P, John, P, Pembrey, M, Perrine, S·1981·Archives of Disease in Childhood
DOI 10.1136/adc.56.3.187PMC PMC1627161
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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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  • When HbF is near 30%, disease can be relatively benign and 70% of cells can be protected.

    “When HbF is near 30%, disease can be relatively benign and 70% of cells can be protected.[52][53][54]”

    From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [54]

    The Results report a mean HbF level of 29% in one group of Saudi Arab infants with HbSS, and the introductory passage says the unusually high HbF is believed to account for their relatively mild clinical course. The paper does not report that 70% of cells were protected; the Subjects and methods passage describes HbF distribution as heterocellular without giving a protected-cell percentage. Page numbers were not available in our copy. (LLM, conf=0.87)

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Research works printed but not linked: 3

  1. [5]5. Perrine R P, Pembrey M E, John P, Perrine S, Shoup F. Natural history of sickle cell anemia in Saudi Arabs. Ann Intern Med 1978; 88: 1-6.
  2. [7]7. Porter F S, Thurman W G. Studies of sickle cell disease. Diagnosis in infancy. Am JDis Child 1963; 106: 35-42.
  3. [10]10. Pembrey M E, Perrine R P, Wood W G, Weatherall D J. Sickle-PO thalassemia in eastern Saudi Arabia. Am J Hum Genet 1980; 32: 26-41.

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Authors
Perrine, R. P., John, P., Pembrey, M., & Perrine, S.
Affiliations
Boston Children's Hospital (US)
ORCID
Venue
Archives of Disease in Childhood
Publisher
BMJ
Published
1981-03-01
Volume
56
Issue
3
Pages
187-192
ISSN
  • 0003-9888 (ISSN-L)
  • 1468-2044
DOI
10.1136/adc.56.3.187
PMC
PMC1627161
OpenAlex
W2135276771
Semantic Scholar
bcc2e07e17272b1707ba8f9f29753accae182890
Cited by
52
References
21
Citations by year
2022: 3 · 2021: 2 · 2020: 2 · 2019: 1 · 2017: 1 · 2016: 1 · 2012: 1
FWCI
1.45
Type
journal article
Language
en
Open access
Locations
  • Archives of Disease in Childhood (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
  • Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Neonatal Health and Biochemistry
  • Health Sciences › Medicine › Hematology › Blood groups and transfusion
Keywords
sickle cell disease, sickle cell trait, HbSS, beta-thalassemia, early childhood, infant morbidity and mortality, fetal hemoglobin, alpha-thalassemia, functional asplenia, Howell-Jolly bodies, eastern Saudi Arabia
MeSH terms
Anemia, Sickle Cell, Child, Child, Preschool, Female, Fetal Hemoglobin, Hemoglobin, Sickle, Hemoglobinometry, Humans, Infant, Male, Saudi Arabia, Thalassemia

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