Laboratório de Investigação em Genética e Hematologia Translacional, Instituto Gonçalo Moniz, Salvador, BA, Brazil.
Department of Biochemistry, Ahmadu Bello University, Zaria, Nigeria.
Dis Markers. 2019 Sep 15;2019:1580485. doi: 10.1155/2019/1580485. eCollection 2019.
The present study aimed to investigate the association of N -carboxymethyllysine (CML) with laboratory parameters and haplotypes in pediatric sickle cell anemia (SCA) patients with or without hydroxyurea (HU) therapy. We included 55 children with SCA (SCA), where 27 were on HU treatment (SCA-HU) and 28 without HU treatment (SCA-HU). Laboratory characteristics were determined using electronic methods while CML was measured using competitive ELISA. haplotypes were determined by RFLP-PCR. Significant increases in MCV and MCH and significant decreases in leukocytes, eosinophils, basophils, atypical lymphocytes, lymphocytes, and monocytes were found in SCA-HU compared to SCA-HU. SCA-HU presented significant reduction in aspartate transaminase and lactate dehydrogenase and increase in creatinine levels compared to SCA-HU. CML levels were significantly higher in both SCA-HU and SCA-HU compared to the healthy control. In addition, a negative correlation was found between CML and alanine transaminase in SCA-HU and SCA ( < 0.01). A significant association was found between CML levels and haplotypes. The results suggest that CML has a role to play in SCA complications, independent of HU therapy.
本研究旨在探讨 N -羧甲基赖氨酸(CML)与伴有或不伴有羟基脲(HU)治疗的小儿镰状细胞贫血(SCA)患者的实验室参数和单倍型之间的关系。我们纳入了 55 名 SCA 患儿(SCA),其中 27 名接受 HU 治疗(SCA-HU),28 名未接受 HU 治疗(SCA-HU)。使用电子方法测定实验室特征,使用竞争性 ELISA 测定 CML。通过 RFLP-PCR 确定单倍型。与 SCA-HU 相比,SCA-HU 中 MCV 和 MCH 显著增加,白细胞、嗜酸性粒细胞、嗜碱性粒细胞、异型淋巴细胞、淋巴细胞和单核细胞显著减少。与 SCA-HU 相比,SCA-HU 中的天冬氨酸转氨酶和乳酸脱氢酶显著降低,肌酐水平显著升高。与健康对照组相比,SCA-HU 和 SCA-HU 中的 CML 水平均显著升高。此外,在 SCA-HU 和 SCA 中,CML 与丙氨酸转氨酶呈负相关(<0.01)。CML 水平与单倍型之间存在显著相关性。结果表明,CML 在 SCA 并发症中起作用,与 HU 治疗无关。