Associations between endothelial dysfunction and clinical and laboratory parameters in children and adolescents with sickle cell anemia

Abstract

BackgroundHematological changes can drive damage of endothelial cells, which potentially lead to an early endothelial dysfunction in patients with sickle cell anemia (SCA). An association may exist between endothelial dysfunction and several clinical manifestations of SCA. The present study aims to evaluate the links between changes in endothelial function and clinical and laboratory parameters in children and adolescents with SCA.MethodsThis study included 40 children and adolescents with stable SCA as well as 25 healthy children; aged 6–18 years. All study subjects were evaluated for endothelial function using Doppler ultrasonography. In addition, a number of laboratory assays were performed, including reticulocyte and leukocyte counts as well as measurement of circulating levels of total bilirubin, C-reactive protein (CRP), glucose, lipoproteins and peripheral oxyhemoglobin saturation. These parameters were also compared between SCA patients who were undertaking hydroxyurea (HU) and those who were not.ResultsFlow-mediated vasodilation (FMD) values were found to be reduced in SCA patients compared with those detected in healthy controls. SCA individuals with lower FMD values exhibited higher number of hospital admissions due to vaso-occlusive events. Additional analyses revealed that patients who had decreased FMD values exhibited higher odds of acute chest syndrome (ACS) episodes. A preliminary analysis with limited number of individuals failed to demonstrate significant differences in FMD values between SCA individuals who were treated with HU and those who were not.ConclusionsChildren and adolescents with SCA exhibit impaired endothelial function. Reductions in FMD values are associated with ACS. These findings underline the potential use of FMD as screening strategy of SCA patients with severe prognosis at early stages.

Sensorineural hearing loss in children with sickle cell anemia and its association with endothelial dysfunction

“…Other studies demonstrated an abnormal response in FMD after wall shear stress (WSS) for transient arterial occlusion in SCA adults [17,37] and children [38]. Studies by our research group showed similar results with decreased FMD in SCA [21,26].…”

“…Endothelial function was evaluated using a protocol established under the guideline for the ultrasonographic evaluation of FMD with reactive hyperemia of the brachial artery [23][24][25] and already used in previous studies carried out by our research group [21,26]. Briefly, the moving images were scanned, beginning 30 seconds before the cuff was deflated until 2 minutes later, allowing for subsequent analysis.…”

Higher values of triglycerides:HDL-cholesterol ratio hallmark disease severity in children and adolescents with sickle cell anemia

“…This study was comprised of secondary analyses of data collected and expanded on previously completed research (7), which included children and adolescents aged between 6 and 18 years, diagnosed with SCA, with or without the use of hydroxyurea and age-matched healthy children. The participants were assigned to two reference centers for the treatment of hematological diseases, including the Hematology Outpatient Clinic of the Magalhães Neto Ambulatory-HUPES Complex of the Federal University of Bahia Medical School, and Hematology and Hemotherapy Foundation of Bahia (HEMOBA).…”

Antisickling Effects of Quercetin may be Associated with Modulation of Deoxyhaemoglobin, 2, 3-bisphosphoglycerate mutase, Redox Homeostasis and Alteration of Functional Chemistry in Human Sickle Erythrocytes.

“…Low oxygen levels further cause increased red cell deformation and polymerization which also cause damage to vascular endothelial cells (Sedrak and Kondamudi, 2018). Vascular endothelial cells dysfunction results in altered vascular tone, vascular adhesion and receptor function related with inflammatory responses and oxidative damage were observed in SCD patients (Teixeira et al, 2017). Oxidative stress during SCD has also been reported to cause depletion of cellular antioxidants resulting in increased lipid peroxidation and increased fragility of RBCs (Okorie et al, 2018).…”