Investigation of Lipid Profile and Clinical Manifestations in SCA Children.

Caroline Conceição Da Guarda,Camylla Vilas Boas Figueiredo,Luciana Magalhães Fiuza,Valma Maria Lopes Nascimento,Cleverson Alves Fonseca,Rodrigo Mota De Oliveira,Rayra Pereira Santiago,Camila Felix De Lima Fernandes,Larissa Carneiro Rocha,Suellen Pinheiro Carvalho,Antonio Mateus De Jesus Oliveira,Milena Magalhães Aleluia,Marilda Souza Gonçalves,Joelma Santana Dos Santos Neres,Sètondji Cocou Modeste Alexandre Yahouédéhou

doi:10.1155/2020/8842362

Abstract

Introduction Clinical complications in sickle cell anemia (SCA) are heterogeneous and involve several molecules. It has been suggested that SCA individuals present a dyslipidemic phenotype and that lipid parameters are associated with severe clinical complications, such as pulmonary hypertension. We sought to investigate associations between lipid parameters and clinical manifestations, as well as other laboratory parameters in a population of pediatric SCA patients. Methods Our cross-sectional evaluation included 126 SCA patients in steady state and who were not undergoing lipid-lowering therapy. Hematological and biochemical parameters were characterized, and previous clinical manifestations were investigated. Results Total cholesterol and low-density lipoprotein cholesterol (LDL-C) levels were increased in patients with a previous history of pneumonia, which also positively correlated with HbS levels. Decreased LDL-C levels were also associated with leg ulcers and anemia. Elevated high-density lipoprotein cholesterol (HDL-C) levels were associated with pain crises, increased viscosity, and decreased hemolysis. Several studies have determined that lipids play a role in the vascular impairment seen in SCA, which was corroborated by our findings. Conclusions In sum, our results suggest that total cholesterol, HDL-C, and LDL-C levels are associated with hemolysis and anemia markers and, most importantly, with clinical complications related to vasculopathy in SCA.

Highlights

Clinical complications in sickle cell anemia (SCA) are heterogeneous and involve several molecules
hemoglobin S (HbS) polymerization is the first pathophysiological step leading to clinical manifestations in SCA; several different mechanisms are involved in the pathogenesis of the disease including ischemia reperfusion injury [2]; increased adhesiveness of leukocytes, reticulocytes, and endothelial cells culminating in vasoocclusion (VO) [3]; and the innate immune system activation with hemolysis products, known as erythrocyte damage-associated molecular pattern molecules [4]
We found that patients with a previous history of pneumonia presented increased total cholesterol levels (Figure 1(a)), a previous history of leg ulcers was associated with decreased low-density lipoprotein cholesterol (LDL-C) levels (Figure 1(b)), and patients with a previous history of pain crises had increased highdensity lipoprotein cholesterol (HDL-C) levels (Figure 1(c))

Summary

Introduction

Clinical complications in sickle cell anemia (SCA) are heterogeneous and involve several molecules. Our results suggest that total cholesterol, HDL-C, and LDL-C levels are associated with hemolysis and anemia markers and, most importantly, with clinical complications related to vasculopathy in SCA. HbS polymerization is the first pathophysiological step leading to clinical manifestations in SCA; several different mechanisms are involved in the pathogenesis of the disease including ischemia reperfusion injury [2]; increased adhesiveness of leukocytes, reticulocytes, and endothelial cells culminating in vasoocclusion (VO) [3]; and the innate immune system activation with hemolysis products, known as erythrocyte damage-associated molecular pattern molecules (eDAMPs) [4]. Abnormal lipid homeostasis is related to different inflammatory diseases, including Alzheimer, where alterations in sphingolipid and cholesterol metabolism result in accumulation of long-chain ceramides and cholesterol [10], and psoriasis, since patients present significantly higher cholesterol levels in the VLDL-C and HDL-C fractions [11]. It is thought that LDL-C plays an important proinflammatory role in vascular diseases, while HDL-C is thought to be anti-inflammatory depending on the context [12]

Methods

Results

Discussion

Conclusion