Splenic function in sickle cell disease in the Eastern Province of Saudi Arabia

1984 ◽  
Vol 104 (5) ◽  
pp. 714-717 ◽  
Author(s):  
Baker Al-Awamy ◽  
Wendell A. Wilson ◽  
Howard A. Pearson
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Eastern Province ◽  
Splenic Function

Prevalence and Diversity of Haplotypes of Sickle Cell Disease in the Eastern Province of Saudi Arabia

Hemoglobin ◽  
2020 ◽  
Vol 44 (2) ◽  
pp. 78-81 ◽  
Author(s):  
Amein K. Al-Ali ◽  
Ahmed Alsulaiman ◽  
Alhusain J. Alzahrani ◽  
Obeid T. Obeid ◽  
Chitti Babu Vatte ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Eastern Province

scholarly journals High fetal hemoglobin production in sickle cell anemia in the eastern province of Saudi Arabia is genetically determined

Blood ◽  
1986 ◽  
Vol 67 (5) ◽  
pp. 1404-1410 ◽  
Author(s):  
BA Miller ◽  
M Salameh ◽  
M Ahmed ◽  
J Wainscoat ◽  
G Antognetti ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Chromosome 11 ◽  
Eastern Province ◽  
Genetically Determined ◽  
Hemoglobin Production

Abstract Homozygous sickle cell disease in the eastern province of Saudi Arabia is clinically mild. Circulating fetal hemoglobin levels of 16.0 +/- 7.4% were found in these anemic patients, but only 1.09 +/- 0.97% in their sickle trait parents. To determine whether these sickle cell anemia patients inherit an increased capacity to synthesize fetal hemoglobin, a radioimmunoassay of fetal and adult hemoglobin was performed on erythroid progenitor (BFU-E)-derived erythroblasts from Saudi Arabian sickle cell patients and their parents. Mean fetal hemoglobin content per BFU-E-derived erythroblast from Saudi Arabian sickle cell patients was 6.2 +/- 2.4 pg/cell or 30.4 +/- 8.6% fetal hemoglobin (normal 1.1 +/- 0.7 pg/cell and 5.1 +/- 1.8%). Linear regression analysis of % HbF in peripheral blood versus % HbF per BFU-E- derived cell showed a positive correlation with an r of 0.65. The variance of the intrinsic capacity to produce HbF may account for almost 40% (r2) of the variance of circulating fetal hemoglobin but other factors, particularly selective survival of F cells, must also contribute significantly. Despite virtually normal HbF levels in sickle trait parents of these Saudi patients, mean fetal hemoglobin production per BFU-E-derived erythroblast in these individuals was elevated to 3.42 +/- 1.79 pg/cell or 16.1 +/- 6.4% fetal hemoglobin, and the magnitude of fetal hemoglobin production found in parents correlated with that of the patients. These data indicate that the high fetal hemoglobin in Saudi sickle cell disease is genetically determined but expressed only during accelerated erythropoiesis. Further evidence of such genetic determination was provided by analysis of DNA polymorphisms within the beta-globin gene cluster on chromosome 11. This revealed a distinctive 5′ globin haplotype (+ + - + +) on at least one chromosome 11 in all high F SS and AS tested. The precise relationship of this haplotype to HbF production in this population remains to be defined.

Variability of sickle cell disease in the Eastern Province of Saudi Arabia

1989 ◽  
Vol 114 (6) ◽  
pp. 973-976 ◽  
Author(s):  
M.I. El Mouzan ◽  
Baker H. Al Awamy ◽  
M.T. Al Torki ◽  
G.A. Niazi
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Eastern Province

Neonatal screening for sickle cell disease in the Eastern Province of Saudi Arabia

1984 ◽  
Vol 78 (6) ◽  
pp. 792-794 ◽  
Author(s):  
B.H. Al-Awamy ◽  
M. Al-Muzan ◽  
M. Al-Turki ◽  
G.R. Serjeant
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Neonatal Screening ◽  
Cell Disease ◽  
Eastern Province

OSTEOMYELITIS AND SEPTIC ARTHRITIS IN SICKLE CELL DISEASE IN THE EASTERN PROVINCE OF SAUDI ARABIA

1993 ◽  
Vol 13 (4) ◽  
pp. 558
Author(s):  
A. H. Al-Salem ◽  
H. A. Ahmed ◽  
S. Qaisaruddin
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Septic Arthritis ◽  
Sickle Cell ◽  
Cell Disease ◽  
Eastern Province

scholarly journals Sickle cell disease in the Eastern Province of Saudi Arabia: Clinical and laboratory features

2021 ◽  
Author(s):  
Amein K. Al‐Ali ◽  
Ahmed Alsulaiman ◽  
Mohammed Alfarhan ◽  
Surinder Safaya ◽  
Chitti Babu Vatte ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Eastern Province ◽  
Laboratory Features

scholarly journals High fetal hemoglobin production in sickle cell anemia in the eastern province of Saudi Arabia is genetically determined

Blood ◽  
1986 ◽  
Vol 67 (5) ◽  
pp. 1404-1410
Author(s):  
BA Miller ◽  
M Salameh ◽  
M Ahmed ◽  
J Wainscoat ◽  
G Antognetti ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Chromosome 11 ◽  
Eastern Province ◽  
Genetically Determined ◽  
Hemoglobin Production

Homozygous sickle cell disease in the eastern province of Saudi Arabia is clinically mild. Circulating fetal hemoglobin levels of 16.0 +/- 7.4% were found in these anemic patients, but only 1.09 +/- 0.97% in their sickle trait parents. To determine whether these sickle cell anemia patients inherit an increased capacity to synthesize fetal hemoglobin, a radioimmunoassay of fetal and adult hemoglobin was performed on erythroid progenitor (BFU-E)-derived erythroblasts from Saudi Arabian sickle cell patients and their parents. Mean fetal hemoglobin content per BFU-E-derived erythroblast from Saudi Arabian sickle cell patients was 6.2 +/- 2.4 pg/cell or 30.4 +/- 8.6% fetal hemoglobin (normal 1.1 +/- 0.7 pg/cell and 5.1 +/- 1.8%). Linear regression analysis of % HbF in peripheral blood versus % HbF per BFU-E- derived cell showed a positive correlation with an r of 0.65. The variance of the intrinsic capacity to produce HbF may account for almost 40% (r2) of the variance of circulating fetal hemoglobin but other factors, particularly selective survival of F cells, must also contribute significantly. Despite virtually normal HbF levels in sickle trait parents of these Saudi patients, mean fetal hemoglobin production per BFU-E-derived erythroblast in these individuals was elevated to 3.42 +/- 1.79 pg/cell or 16.1 +/- 6.4% fetal hemoglobin, and the magnitude of fetal hemoglobin production found in parents correlated with that of the patients. These data indicate that the high fetal hemoglobin in Saudi sickle cell disease is genetically determined but expressed only during accelerated erythropoiesis. Further evidence of such genetic determination was provided by analysis of DNA polymorphisms within the beta-globin gene cluster on chromosome 11. This revealed a distinctive 5′ globin haplotype (+ + - + +) on at least one chromosome 11 in all high F SS and AS tested. The precise relationship of this haplotype to HbF production in this population remains to be defined.

scholarly journals Reasons for Hospitalization of Sickle Cell Disease Patients in the Eastern Province of Saudi Arabia: A Single-Center Study

Cureus ◽  
2021 ◽  
Author(s):  
Ossama M Zakaria ◽  
Rayan A Buhalim ◽  
Faisal A Al Jabr ◽  
Mohammed N AlSaeed ◽  
Ibrahim A Al-Hajji ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Single Center ◽  
Cell Disease ◽  
Eastern Province ◽  
Single Center Study ◽  
Center Study

scholarly journals Vitamin D deficiency in sickle cell disease patients in the Eastern Province of Saudi Arabia

2018 ◽  
Vol 38 (2) ◽  
pp. 130-136 ◽  
Author(s):  
Ali AlJama ◽  
Mohammed AlKhalifah ◽  
Ibrahim Abdulla Al-Dabbous ◽  
Ghada Alqudaihi
Keyword(s):  
Vitamin D ◽  
Saudi Arabia ◽  
Sickle Cell Disease ◽  
Vitamin D Deficiency ◽  
Sickle Cell ◽  
Cell Disease ◽  
Eastern Province
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