Mass Screens for Sickle Cell Disease

Bernstein, Ralph E.

doi:10.1001/jama.1973.03220040052016

Cited by 3 publications

(2 citation statements)

References 4 publications

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“…The present known distribution of HbS in non-blacks includes inhab itants of Greece, Sardinia, Italy, Turkey, Libya, Tunisia, Iran, Saudi Ara bia, India, and Southeast Asia [1]. HbS has not been found in Israeli Jews [24], but has been described previously in Israeli Arabs [19].…”

Section: Discussionmentioning

confidence: 99%

Blood Group Phenotypes and Hemoglobin S

Levene¹,

Rachmilewitz²,

Ezekiel³

et al. 1976

Acta Haematol

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Blood group phenotypes of anthropologic significance are described for inhabitants of two Israeli Arab communities with foci of hemoglobin S (HbS). The presence of Fy(a-b-), Rh₀, hr^V+, and Js(a+) among the Hulah Valley Bedouin, and of Fy(a-b-), Rh₀, and hr^V+ in Acre are indicative of genetic admixture of African origin. These non-African foci of HbS are thereby distinguished from previously described HbS foci in India, Greece, and Turkey where low or absent Rh₀phenotypes imply secondary dispersions of the HbS gene from the Arabian peninsula.

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Section: Discussionmentioning

confidence: 99%

Blood Group Phenotypes and Hemoglobin S

Levene¹,

Rachmilewitz²,

Ezekiel³

et al. 1976

Acta Haematol

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“…Of specific interest are reports of hemoglobin S occurring in white per sons with no African ancestry [4,17], reviving as an alternative to the single mutation theory, the possibility that sickle cell hemoglobin arose as multiple mutations in different geographic areas [32]. A review of the case reports of hemoglobin S in white individuals reveals that the majori ty are decendants of Sicilian emigrants [7, 10, 18-20, 24, 26, 29, 36] and, therefore, an assessment of African admixture in Sicilian populations is fundamental in evaluating the origin of hemoglobin S in these individu als.…”

mentioning

confidence: 99%

Blood Group Phenotypes and the Origin of Sickle Cell Hemoglobin in Sicilians

et al. 1978

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As an approach to investigating the origin of sickle cell hemoglobin (hemoglobin S) in white persons of Sicilian ancestry, two groups of native Sicilians were tested for blood group evidence of African admixture. Among 100 unrelated Sicilians, the phenotypes cDe(Rh_o) and Fy(a-b-), and the antigens V(hrv) and Jsa, which are considered to be African genetic markers, were detected in 12 individuals. Among 64 individuals from 21 families with at least one known hemoglobin S carrier, African blood group markers were detected in 7 (11%). These findings indicate that hemoglobin S is only one of multiple African genes present in contemporary Sicilian populations. The occurrence of hemoglobin S in white persons of Sicilian ancestry is considered to be a manifestation of the continuing dissemination of the original African mutation.

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Sickle Cell — Point, Counterpoint

1973

N Engl J Med

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Mass Screens for Sickle Cell Disease

Cited by 3 publications

References 4 publications

Blood Group Phenotypes and Hemoglobin S

Blood Group Phenotypes and Hemoglobin S

Blood Group Phenotypes and the Origin of Sickle Cell Hemoglobin in Sicilians

Sickle Cell — Point, Counterpoint

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