Santiago Rayra Pereira, Vieira Camilo, Adanho Corynne Stephanie Ahouefa, Santana Sanzio Silva, Guarda Caroline Conceição, Figueiredo Camylla Vilas Boas, Fiuza Luciana Magalhães, Pitanga Thassila Nogueira, Ferreira Junia Raquel Dutra, Aleluia Milena Magalhães, Oliveira Rodrigo Mota, Zanette Dalila Luciola, Lyra Isa Menezes, Goncalves Marilda Souza
Centro de Pesquisas Gonçalo Moniz, Fundação Oswaldo Cruz, Rua Waldemar Falcão, 121, Candeal, 40.296-710 Salvador, BA, Brazil.
Universidade Federal da Bahia, Avenida Adhemar de Barros, s/n, Ondina, 40.170-110 Salvador, BA, Brazil.
Dis Markers. 2017;2017:6359871. doi: 10.1155/2017/6359871. Epub 2017 Jul 16.
Reference values for cerebral blood flow velocity (CBFV) in hemoglobin SC disease (HbSC) have not been established. We aimed to investigate associations between laboratory and genetic biomarkers associated with CBFV in HbSC children. Sixty-eight HbSC children were included; CBFV was analyzed by transcranial Doppler, and the time-averaged maximum mean velocity (TAMMV) was estimated. Hematological, biochemical, immunological, and genetic analyses were performed. TAMMV was negatively correlated with red blood cell count (RBC) count, hemoglobin, hematocrit, and direct bilirubin (DB), yet positively correlated with monocytes and ferritin. We found that children with TAMMV ≥ 128 cm/s had decreased red blood cell distribution width (RDW) and nitric oxide metabolite (NOx) concentration. Children with TAMMV ≥ 143.50 cm/s had decreased hemoglobin and hematocrit, as well as increased ferritin levels. Decreased hemoglobin, hematocrit, RDW, and NOx and increased ferritin were detected in children with TAMMV ≥ 125.75 cm/s. The CAR haplotype was associated with higher TAMMV. In association analyses, RBC, hemoglobin, hematocrit, RDW, monocyte, DB, NOx, and ferritin, as well as the CAR haplotype, were found to be associated with higher TAMMV in HbSC children. Multivariate analysis suggested that high TAMMV was independently associated with hematocrit, RDW, and NOx. Additional studies are warranted to validate the establishment of a cutoff value of 125.75 cm/s associated with elevated TAMMV in HbSC children.
血红蛋白SC病（HbSC）中脑血流速度（CBFV）的参考值尚未确定。我们旨在研究HbSC儿童中与CBFV相关的实验室和遗传生物标志物之间的关联。纳入了68名HbSC儿童；通过经颅多普勒分析CBFV，并估计时间平均最大平均速度（TAMMV）。进行了血液学、生化、免疫学和遗传学分析。TAMMV与红细胞计数（RBC）、血红蛋白、血细胞比容和直接胆红素（DB）呈负相关，但与单核细胞和铁蛋白呈正相关。我们发现，TAMMV≥128 cm/s的儿童红细胞分布宽度（RDW）和一氧化氮代谢物（NOx）浓度降低。TAMMV≥143.50 cm/s的儿童血红蛋白和血细胞比容降低，铁蛋白水平升高。TAMMV≥125.75 cm/s的儿童血红蛋白、血细胞比容、RDW和NOx降低，铁蛋白升高。CAR单倍型与较高的TAMMV相关。在关联分析中，发现RBC、血红蛋白、血细胞比容、RDW、单核细胞、DB、NOx和铁蛋白以及CAR单倍型与HbSC儿童较高的TAMMV相关。多变量分析表明，高TAMMV与血细胞比容、RDW和NOx独立相关。有必要进行更多研究来验证确定与HbSC儿童TAMMV升高相关的125.75 cm/s的临界值。
