ß-Globin Gene Cluster Haplotypes and Clinical Severity in Sickle Cell Anemia Patients in Southern BrazilReportar como inadecuado




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Hematopoietic stem cell transplantationHSCThas emerged as a curative strategy for sickle cell anemiaSCA;it is necessary to find markers of SCA clinical severity to spare those SCApatients whose clinical course is mild from the morbidity and mortalityassociated with HSCT.
Haplotypes have been correlated with the severity ofclinical manifestations in SCA patients, and fetal hemoglobinHbFand socioeconomic statusSeShave also been described as negative factors.
We studied these factors andtheir impact on clinical manifestations in a population of Southern Brazilianpatients attending the Center for Sickle Cell Anemia at Hospital de Clínicas dePorto Alegre-RS, Brazil.
Clinical severity was defined as two or moreveno-occlusive episodes per year.
The βS haplotypes were determined by PCR in 75 SCA patients.
Among the 150 βS chromosomes analyzed, 9966%were identified as BantuBan,4127%asBeninBen,and 107%as other haplotypes.
Mostpatients in our sample62.7%belongedto lower SeS groups, precluding meaningful statistical analysis of SeS impacton clinical severity.
There was no correlation between haplotypes or HbF leveland SCA clinical severity.
Gene polymorphisms and environmental issues have tobe taken into consideration.

KEYWORDS

Sickle Cell Anemia, ß-Globin, Fetal Hemoglobin and Clinical Severity

Cite this paper

A.
L.
da Silva, M.
, R.
Friedrisch, J.
, M.
Bittar, C.
, Urnau, M.
, Merzoni, J.
, S.
Valim, V.
, Amorin, B.
, Pezzi, A.
, Artur B.
Chies, J.
and M.
da Rocha Silla, L.
2014 ß-Globin Gene Cluster Haplotypes and Clinical Severity in Sickle Cell Anemia Patients in Southern Brazil.
Open Journal of Blood Diseases, 4, 16-23.
doi: 10.4236-ojbd.2014.42003.






Autor: Maria A.
L.
da Silva, João R.
Friedrisch, Christina M.
Bittar, Meide Urnau, Jóice Merzoni, Vanessa S.
Valim, Bruna Amorin, Anne


Fuente: http://www.scirp.org/





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