Investigation of Lipid Profile and Clinical Manifestations in SCA Children

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

doi:10.1155/2020/8842362

Cited by 10 publications

(12 citation statements)

References 44 publications

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“…Lipids have been suggested to play a significant role in the pathophysiology of SCD, although the pathophysiological implications of these changes have not been completely resolved [25], and we think this issue needs to be reinvestigated in association with low levels of TC and LDL-C that have been reported in previous reports [28,[31][32][33] and confirmed in the present study. In our study, the mean TC in ST females and SS and ST males have TC levels below 135 mg/dL and thus should have an increased mortality rate based on the findings of Song et al [29] Also, previous reports showed that adults and children with SCD tempt to have decreased levels of TC, HDL-C, and LDL-C and a significantly increased TG compared to age-sex-matched controls in normal population [28,29,34].…”

Section: Biomed Research Internationalsupporting

confidence: 65%

“…While in SS males, there was no significant difference in the triglyceride level when compared to AS individuals and AA controls. Previous reports by Ephraim et al and Essohona et al reported no significant difference in TG between SCD patients and controls [ 32 , 44 ].…”

Section: Discussionmentioning

confidence: 83%

“…TC and LDL-C were significantly lower (P value < 0.001) in SS and ST patients compared to AS individuals and AA controls (Table 3). Decreased TC and LDL-C in SCD have been reported in many studies that examined lipids in SCD patients [16,28,[30][31][32][33][34][35]. TC, in particular LDL-C, has a well-established role in atherosclerosis.…”

Section: Discussionmentioning

confidence: 99%

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Plasma Lipids and Lipoproteins in Sickle Cell Disease Patients in the Northern West Bank, Palestine

Samarah

Srour

Dumaidi

2021

BioMed Research International

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Background. Lipid metabolism may be altered in red cell genetic disorders. The erythrocyte and plasma lipids are defected which may increase the risk of cardiovascular disease. In the present study, we hypothesized a possible link between severity of anemia and altered lipid profile in SCD. Methods. A total of 151 SCD patients, including 62 patients with sickle cell anemia (SS), 54 patients with sickle β-thalassemia (ST), and 35 individuals with sickle cell trait (AS), were studied. The control group consisted of 160 healthy individuals. Total cholesterol (TC), triglyceride (TG), and high-density lipoprotein cholesterol (HDL-C) were enzymatically measured. Results. Total cholesterol and LDL-C were significantly lower ( P value < 0.001) in SS and ST patients compared to AS individuals and AA controls. However, LDL-C was significantly lower in AS individuals (both males and female) compared to AA controls ( P value < 0.001). The HDL-C in SS and ST patients (both males and females) was significantly lower than that in AS individuals ( P value < 0.001). In addition, the HDL-C was significantly higher in SS and ST males and AS (males and females) compared to AA controls ( P value < 0.001). The HDL-C was also significantly higher in SS males ( P value < 0.001) and females ( P value < 0.05) compared to ST patients. The HDL-C was significantly higher in AS individuals ( P value < 0.001) compared to AA controls. The triglycerides in SS males was significantly lower than that in ST patients ( P value < 0.001), but there was no significant difference when compared to AS individuals and AA controls. In contrast, triglycerides in SS females were significantly lower than those in ST ( P value < 0.05), AS ( P value < 0.001), and AA controls ( P value < 0.001). In males of ST patients, triglycerides were significantly higher than those observed in AS males and AA males ( P value < 0.001). In contrast, females of ST patients have a significantly lower triglycerides compared to AS and AA females ( P value < 0.001). Conclusions. In SCD, the plasma is affected in some way, especially the plasma cholesterol that was investigated in this study. Further prospective studies should examine the contribution of an altered lipid profile to the severity and clinical complications in SCD patients.

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Section: Biomed Research Internationalsupporting

confidence: 65%

Section: Discussionmentioning

confidence: 83%

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Plasma Lipids and Lipoproteins in Sickle Cell Disease Patients in the Northern West Bank, Palestine

Samarah

Srour

Dumaidi

2021

BioMed Research International

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“…In addition, TGF- β 1 levels were also found to be positively correlated with VLDL-C and triglycerides in these individuals. HDL-C exerts important anti-inflammatory activity in vascular diseases [ 34 – 36 ] In SCD, decreased HDL-C levels have been associated with an inflammatory state [ 7 , 34 ]. Triglycerides and VLDL-C have been shown to induce inflammatory events in atherosclerosis, supporting the notion that these molecules participate in proinflammatory activity [ 37 ].…”

Section: Discussionmentioning

confidence: 99%

Associations between TGF-β1 Levels and Markers of Hemolysis, Inflammation, and Tissue Remodeling in Pediatric Sickle Cell Patients

Santiago

Carvalho

Figueiredo

et al. 2021

Mediators of Inflammation

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Transforming growth factor beta (TGF-β) is a cytokine with important involvement in biological processes related to the pathogenesis of sickle cell disease (SCD), including endothelial and vascular dysfunction, inflammation, and hematopoietic homeostasis. This study is aimed at investigating associations between levels of TGF-β1 and classical laboratory biomarkers and inflammatory mediators, as well as the tissue inhibitor of metalloproteases-1 (TIMP-1) and matrix metalloproteinase-9 (MMP-9), in pediatric patients ( n = 123 ) with SCD in steady state: 84 with sickle cell anemia (HbSS) and 39 with hemoglobin SC disease (HbSC). A healthy control (HC) group of 59 individuals was also included. Hematological and biochemical analyses were carried out using electronic methods. TGF-β1, TIMP-1, and MMP-9 plasma quantifications were performed by ELISA. TGF-β1 plasma levels were higher in HbSS individuals than in HbSC and HC. In individuals with HbSS, TGF-β1 levels were positively correlated with red blood cells, hemoglobin, hematocrit, platelets, and TIMP-1. In addition, HbSS individuals with TGF-β1 levels above the median (≥72.29 ng/mL) also presented increased monocyte counts and decreased albumin levels. In patients with HbSC, TGF-β1 levels were positively correlated with leukocytes, eosinophils, lymphocytes, monocytes, and platelets, as well as levels of TIMP-1, VLDL-C, triglycerides, heme, and AST. Additionally, HbSC individuals with TGF-β1 levels above the median (≥47.80 ng/mL) presented increased leukocyte and platelet counts, as well as increased levels of triglycerides, VLDL-C, MMP-9, and TIMP-1, and decreased HDL-C. Our findings suggest that TGF-β1 may play important roles in vascular remodeling, vasculopathy, angiogenesis, and inflammation in pediatric patients with SCD.

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“…Conversely, two retrospective studies, Guarda et al (2020) 20 and Darbari et al (2013), 21 found an association between higher HDL-C levels and more frequent VOS. Lalanne-Mistrih et al (2018), 22 also through a retrospective analysis, did not detect a correlation between clinical history of VOS and this lipid component.…”

Section: Discussionmentioning

confidence: 94%

Association Between Lipid Profile and Clinical Manifestations in Sickle Cell Anemia: A Systematic Review

Dantas¹,

Lopes²,

Ladeia³

2022

International Journal of Cardiovascular Sciences

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Every year, 250,000 children with SCA are born worldwide. 2 In Brazil, this number reaches 3,500 new cases/year. 3 being the most frequent hereditary disease in the country. 4 It is a chronic inflammatory disease permeated with acute events, with high morbidity and early mortality. 5 The pathophysiology of SCA is complex, involving changes in lipid metabolism, endothelial dysfunction, and hemolysis, in addition to a chronic inflammatory process. These mechanisms are associated with markers of disease severity.

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Investigation of Lipid Profile and Clinical Manifestations in SCA Children

Cited by 10 publications

References 44 publications

Plasma Lipids and Lipoproteins in Sickle Cell Disease Patients in the Northern West Bank, Palestine

Plasma Lipids and Lipoproteins in Sickle Cell Disease Patients in the Northern West Bank, Palestine

Associations between TGF-β1 Levels and Markers of Hemolysis, Inflammation, and Tissue Remodeling in Pediatric Sickle Cell Patients

Association Between Lipid Profile and Clinical Manifestations in Sickle Cell Anemia: A Systematic Review

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