Prevalence of β(S)-globin gene haplotypes, α-thalassemia (3.7 kb deletion) and redox status in patients with sickle cell anemia in the state of Paraná, Brazil.
Eliana Litsuko Tomimatsu Shimauti,Eliana Litsuko Tomimatsu Shimauti,Danilo Grünig Humberto da Silva,Eniuce Menezes de Souza,Eduardo Alves de Almeida,Francismar Prestes Leal,Claudia Regina Bonini-Domingos +6 more
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TLDR
Both genotypes were associated with high LPO and TEAC levels and decreased melatonin concentration, suggesting that the level of oxidative stress in patients with Bantu/Bantu and BantU/Benin genotypes may overload the antioxidant capacity.Abstract:
The aim of this study was to determine the frequency of beta S-globin gene (β(S) globin) haplotypes and alpha thalassemia with 3.7 kb deletion (-α(3.7kb) thalassemia) in the northwest region of Parana state, and to investigate the oxidative and clinical-hematological profile of β(S) globin carriers in this population. Of the 77 samples analyzed, 17 were Hb SS, 30 were Hb AS and 30 were Hb AA. The β(S)globin haplotypes and -α(3.7kb) thalassemia were identified using polymerase chain reaction.Trolox equivalent antioxidant capacity (TEAC) and lipid peroxidation (LPO) were assessed spectophotometrically. Serum melatonin levels were determined using high-performance liquid chromatography coupled to coulometric electrochemical detection. The haplotype frequencies in the SS individuals were as follows: Bantu- 21 (62%), Benin - 11 (32%) and Atypical- 2 (6%). Bantu/Benin was the most frequent genotype. Of the 47 SS and AS individuals assessed, 17% (n = 8) had the -α(3.7kb) mutation. Clinical manifestations, as well as serum melatonin, TEAC and LPO levels did not differ between Bantu/Bantu and Bantu/Benin individuals (p > 0.05). Both genotypes were associated with high LPO and TEAC levels and decreased melatonin concentration. These data suggest that the level of oxidative stress in patients with Bantu/Bantu and Bantu/Benin genotypes may overload the antioxidant capacity.read more
Citations
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Journal ArticleDOI
Association between Oxidative Stress, Genetic Factors, and Clinical Severity in Children with Sickle Cell Anemia
Céline Renoux,Philippe Joly,Camille Faes,Pauline Mury,Buse Eglenen,Mine Turkay,Gokce Yavas,Ozlem Yalcin,Yves Bertrand,Nathalie Garnier,Daniela Cuzzubbo,Alexandra Gauthier,Marc Romana,Berenike Möckesch,Giovanna Cannas,Sophie Antoine-Jonville,Vincent Pialoux,Philippe Connes +17 more
TL;DR: Mild hemolysis in children with alpha‐thalassemia may limit oxidative stress and could explain the protective role of alpha‐halassemia in Hemolysis‐related sickle cell complications.
Dissertation
“Detección de células falciformes mediante la prueba de ciclaje para diagnóstico de anemia drepanocítica en pre-escolares de raza afroecuatoriana de la unidad educativa valle del chota.”
Zamora Sánchez,Germán Rodrigo +1 more
TL;DR: The prevalencia de celulas falciformes en pre-escolares identificado fenotipicamente bajo los rasgos del grupo etnico negroide de la Unidad Educación Valle del Chota (UEV) was investigated in this article.
Journal ArticleDOI
The frequency of βS-globin haplotypes in the state of Paraná, Brazil, and clinical manifestations of sickle cell anemia
TL;DR: No association was found between haplotypes and clinical manifestations, probably given the almost absolute predominance of CAR and BEN haplotypes, however, this fact alerts to the possible influence of other polymorphisms and miscegenation in the Brazilian population.
Journal ArticleDOI
Sickle cell anemia in the state of Maranhão: a haplotype study.
Ademilton Costa Alves,Verônica Avena Lisboa da Silva,Alexandro Dos Santos,Mariana Barreto Serra,Felipe Albuquerque Marques,Sônia Maria Pereira Cruz,Wermerson Assunção Barroso,Raimundo Antônio Gomes Oliveira +7 more
TL;DR: The high frequency of the CAR/CAR and CAR/BEN haplotypes in this study may ultimately reflect a severe clinical course and poor prognosis in patients with SCA in Maranhão.
Journal ArticleDOI
Alpha thalassemia, but not βS-globin haplotypes, influence sickle cell anemia clinical outcome in a large, single-center Brazilian cohort.
Betânia Lucena Domingues Hatzlhofer,Diego A Pereira-Martins,Diego A Pereira-Martins,Igor de Farias Domingos,Igor de Farias Domingos,Gabriela da Silva Arcanjo,Isabel Weinhäuser,Diego Arruda Falcão,Isabela Cristina Cordeiro Farias,Jéssica Vitória Gadelha de Freitas Batista,Luana Priscilla Laranjeira Prado,Jéssica Maria Florencio Oliveira,Thais Helena Chaves Batista,Marcondes José de Vasconcelos Costa Sobreira,Rodrigo Marcionilo Santana,A. S. Araújo,Manuela Albuquerque de Melo,Bruna Vasconcelos de Ancântara,Juan L Coelho-Silva,Juan L Coelho-Silva,Ana Beatriz Lucas de Moura Rafael,Danízia Menezes de Lima Silva,Flávia Peixoto Albuquerque,Flávia Peixoto Albuquerque,Magnun N. N. Santos,Ana Claudia Mendonça dos Anjos,Fernando Ferreira Costa,Aderson S Araujo,Antonio R. Lucena-Araujo,Marcos André Cavalcanti Bezerra +29 more
TL;DR: The results reinforce that alpha thalassemia can provide protective functions against hemolysis-related symptoms in SCA and should therefore always be considered first.
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