OBJECTIVE: To correlate the clinical and hematological features of β-globin gene haplotypes with the oxidative stress status in pediatric patients with sickle cell disease (SCD). METHODS: A total of 95 patients with SCD and 40 healthy children were studied. The β-globin cluster, plasma lipid peroxidation (LPO) and plasma nitrite plus nitrate (NOx), and erythrocyte content of glutathione (GSH) and glutathione disulfide (GSSG), and glutathione peroxidase (GPx), reductase (GRd), and superoxide dismutase (SOD) activities were measured. RESULTS: Plasma LPO (P < 0.001) and NOx (P < 0.05) were significantly higher in patients than in controls. In erythrocytes of patients with SCD, the activities of GRd (P < 0.001) and SOD (P < 0.05) were lower, and the GSSG/GSH ratio (P < 0.001) and GPx activity (P < 0.001) were higher than in controls. High LPO levels and low SOD plus GRd activities were associated with increased severity of clinical manifestations, which correspond mainly to patients with Bantu and Benin haplotypes. LPO levels were reduced in patients with high fetal hemoglobin (HbF) levels, whereas the NOx levels and GRd activity tended to increase in this group. CONCLUSION: Our results detected an important oxidative stress in patients with SCD and suggest that at least three redox markers, i.e., LPO, GRd, and SOD, were related with their clinical outcomes. Moreover, a relationship between high HbF and low LPO, and high HbF and high GRd activity and NOx levels were found.
OBJECTIVE: To correlate the clinical and hematological features of β-globin gene haplotypes with the oxidative stress status in pediatric patients with sickle cell disease (SCD). METHODS: A total of 95 patients with SCD and 40 healthy children were studied. The β-globin cluster, plasma lipid peroxidation (LPO) and plasma nitrite plus nitrate (NOx), and erythrocyte content of glutathione (GSH) and glutathione disulfide (GSSG), and glutathione peroxidase (GPx), reductase (GRd), and superoxide dismutase (SOD) activities were measured. RESULTS: Plasma LPO (P < 0.001) and NOx (P < 0.05) were significantly higher in patients than in controls. In erythrocytes of patients with SCD, the activities of GRd (P < 0.001) and SOD (P < 0.05) were lower, and the GSSG/GSH ratio (P < 0.001) and GPx activity (P < 0.001) were higher than in controls. High LPO levels and low SOD plus GRd activities were associated with increased severity of clinical manifestations, which correspond mainly to patients with Bantu and Benin haplotypes. LPO levels were reduced in patients with high fetal hemoglobin (HbF) levels, whereas the NOx levels and GRd activity tended to increase in this group. CONCLUSION: Our results detected an important oxidative stress in patients with SCD and suggest that at least three redox markers, i.e., LPO, GRd, and SOD, were related with their clinical outcomes. Moreover, a relationship between high HbF and low LPO, and high HbF and high GRd activity and NOx levels were found.
Authors: Betânia Lucena Domingues Hatzlhofer; Diego Antonio Pereira-Martins; 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 de Santana; Amanda Bezerra de Sá Araújo; Manuela Albuquerque de Melo; Bruna Vasconcelos de Ancântara; Juan Luiz Coelho-Silva; Ana Beatriz Lucas de Moura Rafael; Danízia Menezes de Lima Silva; Flávia Peixoto Albuquerque; Magnun Nueldo Nunes Santos; Ana Cláudia Dos Anjos; Fernando Ferreira Costa; Aderson da Silva Araújo; Antonio Roberto Lucena-Araújo; Marcos André Cavalcanti Bezerra Journal: Ann Hematol Date: 2021-02-13 Impact factor: 3.673
Authors: Tite M Mikobi; Prosper Lukusa Tshilobo; Michel N Aloni; Pierre Z Akilimali; Georges Mvumbi-Lelo; Jean Marie Mbuyi-Muamba Journal: J Clin Lab Anal Date: 2017-01-23 Impact factor: 2.352