scholarly journals <i>ß</i>-Globin Gene Cluster Haplotypes and Clinical Severity in Sickle Cell Anemia Patients in Southern Brazil

2014 ◽  
Vol 04 (02) ◽  
pp. 16-23 ◽  
Author(s):  
Maria A. L. da Silva ◽  
João R. Friedrisch ◽  
Christina M. Bittar ◽  
Meide Urnau ◽  
Jóice Merzoni ◽  
...  
Keyword(s):  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Clinical Severity ◽  
Southern Brazil ◽  
Globin Gene Cluster

scholarly journals Variants of ZBTB7A (LRF) and its β-globin gene cluster binding motifs in sickle cell anemia

2016 ◽  
Vol 59 ◽  
pp. 49-51 ◽  
Author(s):  
Elmutaz M. Shaikho ◽  
Alawi H. Habara ◽  
Abdulrahman Alsultan ◽  
A.M. Al-Rubaish ◽  
Fahad Al-Muhanna ◽  
...  
Keyword(s):  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Binding Motifs ◽  
Globin Gene Cluster

scholarly journals The Senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients

Blood ◽  
1991 ◽  
Vol 77 (6) ◽  
pp. 1371-1375 ◽  
Author(s):  
RL Nagel ◽  
S Erlingsson ◽  
ME Fabry ◽  
H Croizat ◽  
SM Susuka ◽  
...  
Keyword(s):  
New York ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Inhibitory Effect ◽  
Hemoglobin F ◽  
Globin Gene Cluster ◽  
Alpha Gene ◽  
Hbf Level

Abstract We have previously determined that in African sickle cell anemia (SS) patients three different beta-like globin gene cluster haplotypes are associated with different percent G gamma (one of the two types of non- alpha chains comprising hemoglobin F [HbF]), mean percent HbF, and percent dense cells. We report now that in adult New York SS patients, the presence of at least one chromosome with the Senegal haplotype is associated with higher Hb levels (1.2 g/dL higher) than is found for any other non-Senegal haplotype (P less than .004). The percent reticulocytes and the serum bilirubin levels were lower in these patients. When the effect of alpha-gene number was analyzed by examining a sample of SS patients with concomitant alpha-thalassemia, the same results were obtained. Because the HbF level is significantly higher among the Senegal haplotype carriers in this sample, the inhibitory effect on sickling of this Hb variant may be one of the reasons for the haplotype effect. We conclude that the Senegal beta- like globin gene cluster haplotype is associated with an amelioration of the hemolytic anemia that characterizes sickle cell disease.

scholarly journals The β‐Globin Gene Cluster Haplotypes in Sickle Cell Anemia Patients from Northeast Brazil: A Clinical and Molecular View

Hemoglobin ◽  
2004 ◽  
Vol 28 (3) ◽  
pp. 267-271 ◽  
Author(s):  
Elisângela Vitória Adorno ◽  
Ângela Zanette ◽  
Isa Lyra ◽  
Cyntia Cajado Souza ◽  
Leandro Ferraz Santos ◽  
...  
Keyword(s):  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Northeast Brazil ◽  
Globin Gene Cluster

Sickle Cell Anemia and β-Globin Gene Cluster Haplotypes in Colombia

Hemoglobin ◽  
2000 ◽  
Vol 24 (3) ◽  
pp. 221-225 ◽  
Author(s):  
F. Cuéllar-Ambrosi ◽  
M. C. Mondragón ◽  
M. Figueroa ◽  
C. Préhu ◽  
F. Galactéros ◽  
...  
Keyword(s):  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Globin Gene Cluster

β-Globin Gene Cluster Haplotypes in a Cohort of 221 Children with Sickle Cell Anemia or Sβ⁰-Thalassemia and Their Association with Clinical and Hematological Features

2010 ◽  
Vol 124 (3) ◽  
pp. 162-170 ◽  
Author(s):  
André Rolim Belisário ◽  
Marina Lobato Martins ◽  
Ana Mercy Siebra Brito ◽  
Cibele Velloso Rodrigues ◽  
Célia Maria Silva ◽  
...  
Keyword(s):  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Globin Gene Cluster

Effect of β-Globin gene cluster haplotype on the hematological and clinical features of sickle cell anemia

1991 ◽  
Vol 36 (3) ◽  
pp. 184-189 ◽  
Author(s):  
R. F. Rieder ◽  
S. Safaya ◽  
P. Gillette ◽  
S. Fryd ◽  
H. Hsu ◽  
...  
Keyword(s):  
Gene Cluster ◽  
Clinical Features ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Globin Gene Cluster

scholarly journals The Senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients

Blood ◽  
1991 ◽  
Vol 77 (6) ◽  
pp. 1371-1375
Author(s):  
RL Nagel ◽  
S Erlingsson ◽  
ME Fabry ◽  
H Croizat ◽  
SM Susuka ◽  
...  
Keyword(s):  
New York ◽  
Gene Cluster ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Globin Gene ◽  
Inhibitory Effect ◽  
Hemoglobin F ◽  
Globin Gene Cluster ◽  
Alpha Gene ◽  
Hbf Level

We have previously determined that in African sickle cell anemia (SS) patients three different beta-like globin gene cluster haplotypes are associated with different percent G gamma (one of the two types of non- alpha chains comprising hemoglobin F [HbF]), mean percent HbF, and percent dense cells. We report now that in adult New York SS patients, the presence of at least one chromosome with the Senegal haplotype is associated with higher Hb levels (1.2 g/dL higher) than is found for any other non-Senegal haplotype (P less than .004). The percent reticulocytes and the serum bilirubin levels were lower in these patients. When the effect of alpha-gene number was analyzed by examining a sample of SS patients with concomitant alpha-thalassemia, the same results were obtained. Because the HbF level is significantly higher among the Senegal haplotype carriers in this sample, the inhibitory effect on sickling of this Hb variant may be one of the reasons for the haplotype effect. We conclude that the Senegal beta- like globin gene cluster haplotype is associated with an amelioration of the hemolytic anemia that characterizes sickle cell disease.

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