Showing papers on "Sickle cell anemia published in 2021"
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23 Apr 2021
TL;DR: Studies from past decades related to such translational research as the use of hydroxyurea in treatment, as well as the therapeutic promise of red-cell ion-channel blockers, and antiadhesion and anti-inflammatory therapy are highlighted.
Abstract: Sickle-cell disease is one of the most common severe monogenic disorders in the world. Haemoglobin polymerisation, leading to erythrocyte rigidity and vaso-occlusion, is central to the pathophysiology of this disease, although the importance of chronic anaemia, haemolysis, and vasculopathy has been established. Clinical management is basic and few treatments have a robust evidence base. One of the main problems of sickle-cell disease in children is the development of cerebrovascular disease and cognitive impairment, and the role of blood transfusion and hydroxycarbamide for prevention of these complications is starting to be understood. Recurrent episodes of vaso-occlusion and inflammation result in progressive damage to most organs, including the brain, kidneys, lungs, bones, and cardiovascular system, which becomes apparent with increasing age. Most people with sickle-cell disease live in Africa, where little is known about this disease; however, we do know that the disorder follows a more severe clinical course in Africa than for the rest of the world and that infectious diseases have a role in causing this increased severity of sickle-cell disease. More work is needed to develop effective treatments that specifically target pathophysiological changes and clinical complications of sickle-cell disease.
966 citations
TL;DR: An overview of the current and emerging techniques for sickle cell disease detection and highlights the different potential methods that could be applied to help the early diagnosis of SCD can be found in this paper.
Abstract: Sickle cell disease (SCD) is a widespread disease caused by a mutation in the beta-globin gene that leads to the production of abnormal hemoglobin called hemoglobin S. The inheritance of the mutation could be homozygous or heterozygous combined with another hemoglobin mutation. SCD can be characterized by the presence of dense, sickled cells that causes hemolysis of blood cells, anemia, painful episodes, organ damage, and in some cases death. Early detection of SCD can help to reduce the mortality and manage the disease effectively. Therefore, different techniques have been developed to detect the sickle cell disease and the carrier states with high sensitivity and specificity. These techniques can be screening tests such as complete blood count, peripheral blood smears, and sickling test; confirmatory tests such as hemoglobin separation techniques; and genetic tests, which are more expensive and need to be done in centralized labs by highly skilled personnel. However, advanced portable point of care techniques have been developed to provide a low-cost, simple, and user-friendly device for detecting SCD, for instance coupling solubility tests with portable devices, using smartphone microscopic classifications, image processing techniques, rapid immunoassays, and sensor-based platforms. This review provides an overview of the current and emerging techniques for sickle cell disease detection and highlights the different potential methods that could be applied to help the early diagnosis of SCD.
25 citations
01 Apr 2021
TL;DR: The proposed approach tackles the limitations of manual research by implementing a powerful and efficient MLP (Multi-Layer Perceptron) classification algorithm that distinguishes Sickle Cell Anemia (SCA) into three classes: Normal (N), Sickle Cells (S) and Thalassemia (T) in red blood cells.
Abstract: A molecule called hemoglobin is found in red blood cells that holds oxygen all over the body. Hemoglobin is elastic, round, and stable in a healthy human. This makes it possible to float across red blood cells. But the composition of hemoglobin is unhealthy if you have sickle cell disease. It refers to compact and bent red blood cells. The odd cells obstruct the flow of blood. It is dangerous and can result in severe discomfort, organ damage, heart strokes, and other symptoms. The human life expectancy can be shortened as well. The early identification of sickle calls will help people recognize signs that can assist antibiotics, supplements, blood transfusion, pain-relieving medications, and treatments etc. The manual assessment, diagnosis, and cell count are time consuming process and may result in misclassification and count since millions of red blood cells are in one spell. When utilizing data mining techniques such as the multilayer perceptron classifier algorithm, sickle cells can be effectively detected with high precision in the human body. The proposed approach tackles the limitations of manual research by implementing a powerful and efficient MLP (Multi-Layer Perceptron) classification algorithm that distinguishes Sickle Cell Anemia (SCA) into three classes: Normal (N), Sickle Cells(S) and Thalassemia (T) in red blood cells. This paper also presents the precision degree of the MLP classifier algorithm with other popular mining and machine learning algorithms on the dataset obtained from the Thalassemia and Sickle Cell Society (TSCS) located in Rajendra Nagar, Hyderabad, Telangana, India. Doi: 10.28991/esj-2021-01270 Full Text: PDF
19 citations
TL;DR: No case has been reported in the literature with unilateral facial nerve palsy in SCA patient, and Bell’s palsy is a neuropathy which caused by traumatic, inflammatory, infective, or compressive conditions on the facial nerve.
Abstract: Sickle cell anemia (SCA) is an inherited red blood cell disorder that results from the replacement of a valine residue for glutamic acid at position 6 in the beta-subunit of haemoglobin.This can lead to tissue ischemia, microcirculation obstructions, infarction, and acute stroke while Bell’s palsy is a neuropathy caused by traumatic, inflammatory, infective, or compressive conditions on the facial nerve. In patients with SCA, however, stroke may occur as an acute clinical syndrome presenting with hemiplegia with the unilateral facial nerve. Until now, no case has been reported in the literature with unilateral facial nerve palsy in SCA patients. Here, a rare case of idiopathic unilateral Bell’s palsy is presented in an SCA patient.
17 citations
TL;DR: In this paper, the authors tested the hypothesis that elevated cerebral infarcts or strokes increased the cognitive deficits of individuals with sickle cell anemia and found that the hypothesis was false.
Abstract: Background and Purpose: Individuals with sickle cell anemia experience cognitive deficits, even in the absence of cerebral infarcts or strokes. This study tested the hypothesis that elevated cerebr...
16 citations
TL;DR: In this paper, the Δ χ do was measured for both normal hemoglobin A (HbA) and sickle hemoglobin (SH) for both HbA and SH.
Abstract: Purpose Sickle cell anemia is a blood disorder that alters the morphology and the oxygen affinity of the red blood cells. Cerebral oxygen extraction fraction measurements using quantitative BOLD contrast have been used for assessing inadequate oxygen delivery and the subsequent risk of ischemic stroke in sickle cell anemia. The BOLD signal in MRI studies relies on Δ χ do , the bulk volume susceptibility difference between fully oxygenated and fully deoxygenated blood. Several studies have measured Δ χ do for normal hemoglobin A (HbA). However, it is not known whether the value is different for sickle hemoglobin. In this study, Δ χ do was measured for both HbA and sickle hemoglobin. Methods Six sickle cell anemia patients and 6 controls were recruited. Various blood oxygenation levels were achieved through in vivo manipulations to keep the blood close to its natural state. To account for the differences in oxygen affinity, Hill's equations were used to translate partial pressure of oxygen to oxygen saturation for HbA, sickle hemoglobin, and fetal hemoglobin (HbF) separately. The pH and PCO2 corrections were performed. Temperature and magnetic field drift were controlled for. A multivariate generalized linear mixed model with random participant effect was used. Results Assuming that Δ χ do is similar for HbA and HbF and that Δ χ metHb is 5/4 of Δ χ do for HbA, it was found that the Δ χ do values for HbA and sickle hemoglobin were not statistically significantly different from each other. Conclusion The same Δ χ do value can be used for both types of hemoglobin in quantitative BOLD analysis.
15 citations
13 citations
TL;DR: The primary hypothesis is that there will be a 66% relative risk reduction of strokes in children with SCA and abnormal TCD measurements, randomly allocated, for a minimum of three years to receive moderate fixed-dose versus low fixed- dose hydroxyurea therapy.
Abstract: Strokes in children with sickle cell anemia (SCA) are associated with significant morbidity and premature death. Primary stroke prevention in children with SCA involves screening for abnormal transcranial Doppler (TCD) velocity coupled with regular blood transfusion therapy for children with abnormal velocities, for at least one year. However, in Africa, where the majority of children with SCA live, regular blood transfusions are not feasible due to inadequate supply of safe blood, cost, and the reluctance of caregivers to accept transfusion therapy for their children. We describe the Primary Prevention of Stroke in Children with Sickle Cell Disease in Nigeria Trial [StrokePreventioninNigeria (SPRING) trial, NCT02560935], a three-center double-blinded randomized controlled Phase III clinical trial to 1) determine the efficacy of moderate fixed-dose (20 mg/kg/day) versus low fixed-dose (10 mg/kg/day) hydroxyurea therapy for primary stroke prevention; 2) determine the efficacy of moderate fixed-dose hydroxyurea for decreasing the incidence of all cause-hospitalization (pain, acute chest syndrome, infection, other) compared to low fixed-dose hydroxyurea. We will test the primary hypothesis that there will be a 66% relative risk reduction of strokes in children with SCA and abnormal TCD measurements, randomly allocated, for a minimum of three years to receive moderate fixed-dose versus low fixed-dose hydroxyurea (total n = 220). The results of this trial will advance the care of children with SCA in sub-Saharan Africa, while improving research capacity for future studies to prevent strokes in children with SCA.
12 citations
TL;DR: It is suggested that the use of HU in young males does not adversely affect fertility.
12 citations
TL;DR: In this article, a unique pulmonary vascular iron accumulation in lungs of sickle cell anemia pulmonary hypertension patients at autopsy was defined within the concept of iron, which is unlike findings in idiopathic or other forms of pulmonary arterial hypertension.
Abstract: Sickle cell anemia and β-thalassemia intermedia are very different genetically determined hemoglobinopathies predisposing to pulmonary hypertension. The etiologies responsible for the associated development of pulmonary hypertension in both diseases are multi-factorial with extensive mechanistic contributors described. Both sickle cell anemia and β-thalassemia intermedia present with intra and extravascular hemolysis. And because sickle cell anemia and β-thalassemia intermedia share features of extravascular hemolysis, macrophage iron excess and anemia we sought to characterize the common features of the pulmonary hypertension phenotype, cardiac mechanics, and function as well as lung and right ventricular metabolism. Within the concept of iron, we have defined a unique pulmonary vascular iron accumulation in lungs of sickle cell anemia pulmonary hypertension patients at autopsy. This observation is unlike findings in idiopathic or other forms of pulmonary arterial hypertension. In this study, we hypothesized that a common pathophysiology would characterize the pulmonary hypertension phenotype in sickle cell anemia and β-thalassemia intermedia murine models. However, unlike sickle cell anemia, β-thalassemia is also a disease of dyserythropoiesis, with increased iron absorption and cellular iron extrusion. This process is mediated by high erythroferrone and low hepcidin levels as well as dysregulated iron transport due transferrin saturation, so there may be differences as well. Herein we describe common and divergent features of pulmonary hypertension in aged Berk-ss (sickle cell anemia) and Hbbth/3+ (intermediate β-thalassemia) mice and suggest translational utility as proof-of-concept models to study pulmonary hypertension therapeutics specific to genetic anemias.
11 citations
TL;DR: In this article, the prevalence of echocardiographic abnormalities in children and young adults with sickle cell anemia and examined the effects of hydroxyurea on reverse cardiac remodeling.
TL;DR: The 3‐year stenosis outcome in 60 stroke‐free children with sickle cell anaemia and an abnormal transcranial Doppler history, enrolled in the DREPAGREFFE trial, is reported, indicating better stenosis prevention and improved outcome after SCT.
Abstract: We report here the 3-year stenosis outcome in 60 stroke-free children with sickle cell anaemia (SCA) and an abnormal transcranial Doppler history, enrolled in the DREPAGREFFE trial, which compared stem cell transplantation (SCT) with standard-care (chronic transfusion for 1-year minimum). Twenty-eight patients with matched sibling donors were transplanted, while 32 remained on standard-care. Stenosis scores were calculated after performing cerebral/cervical 3D time-of-flight magnetic resonance angiography. Fourteen patients had stenosis at enrollment, but only five SCT versus 10 standard-care patients still had stenosis at 3 years. Stenosis scores remained stable on standard-care, but significantly improved after SCT (P = 0·006). No patient developed stenosis after SCT, while two on standard-care did, indicating better stenosis prevention and improved outcome after SCT.
TL;DR: In this paper, the authors assessed parasite biomass and plasma markers of inflammation and endothelial activation in children with sickle cell anemia (SCA) and P. falciparum parasitemia.
TL;DR: The data support long-term safety and tolerability of benserazide at doses ten times higher than used in preclinical models to induce fetal hemoglobin.
Abstract: Induction of fetal hemoglobin production with hydroxyurea is an effective strategy in sickle cell disease and beta thalassemias, but up to 20% of patients do not respond to or cannot tolerate it. Benserazide is used in the treatment of Parkinson's disease and was noticed to induce gamma globin in preclinical models. We hypothesized that chronic treatment with benserazide-containing medication may be associated with increase in HbF production and in circulating F-cells. Blood samples were collected from 50 subjects including 35 patients on benserazide for Parkinson's disease, 10 healthy controls, and 5 patients with sickle cell anemia as positive controls for high fetal hemoglobin. We found a strong correlation between HbF and circulating F-cells in the entire population, but we found no significant increase in HbF and F-cell percentage in patients taking benserazide up to 700 mg daily. No hematologic abnormalities attributable to benserazide use after up to 22 years were detected. Our data support long-term safety and tolerability of benserazide at doses ten times higher than used in preclinical models to induce fetal hemoglobin. Further clinical trials enrolling patients with sickle cell disease and thalassemia are warranted to provide insight into its efficacy to treat those populations.
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08 Sep 2021
TL;DR: G6PD deficiency significantly contributes to recurrent painful vasoocclusive crisis in SCA persons in the steady state and there was no correlation between disease severity resulting from reduced G6PD activity and Heinz body formation in SC a persons in a steady state.
Abstract: Background and ObjectivesSickle cell anemia (SCA) and glucose-6-phosphate dehydrogenase (G6PD) deficiency are both hereditary diseases of the red blood cells that cause hemolysis. The impact of the...
TL;DR: Sickle cell anemia (SCA) is the commonest life-threatening genetic disorder in tropical regions, particularly in sub-Saharan Africa as discussed by the authors, and between 50-90% of SCA children will die in Africa before the age of 5, corresponding to a number of 150,000-300,000 annual SCA child deaths, which represents 5-10 % of total child mortality.
Abstract: Sickle cell anemia (SCA) is the commonest life-threatening genetic disorder in tropical regions, particularly in sub-Saharan Africa. It has been estimated that between 50-90% of SCA children will die in Africa before the age of 5, corresponding to a number of 150,000-300,000 annual SCA child deaths, which represents 5-10 % of total child mortality. Transfusion support remains an essential component in the management of patients with SCA and has made a significant contribution to improving patient morbidity and mortality. In Africa where the majority of patients with SCA reside, many blood transfusion challenges remains, including shortage of blood supplies, risks related to infectious and immunologic potential side effects and limitation on the diagnosis and management of post-transfusion iron overload. The proportion of transfused SCA patients varies from different studies, between 30% and 90%. This variation can be related to environmental factors, disease genetic factors and other factors including the low availability of blood, difficulties in accessing to health care and inadequacies of the transfusion system. Because blood transfusion therapy is an integral component of the management of SCA, improved efforts and strategies to overcome these challenges and optimize blood transfusion practices are needed in African countries.
TL;DR: Progressive muscle relaxation exercises were found to be effective in the pain management of patients with sickle cell anemia.
TL;DR: In this article, the frequencies of UGT1A1 promoter alleles, alpha thalassemia, and βS haplotypes were determined using DNA sequencing and PCR-based assays in 913 patients with sickle cell anemia (SCA).
Abstract: Hyperbilirubinemia in patients with sickle cell anemia (SCA) as a result of enhanced erythrocyte destruction, lead to cholelithiasis development in a subset of patients Evidence suggests that hyperbilirubinemia may be related to genetic variations, such as the UGT1A1 gene promoter polymorphism, which causes Gilbert syndrome (GS) Here, we aimed to determine the frequencies of UGT1A1 promoter alleles, alpha thalassemia, and βS haplotypes and analyze their association with cholelithiasis and bilirubin levels The UGT1A1 alleles, -37 kb alpha thalassemia deletion and βS haplotypes were determined using DNA sequencing and PCR-based assays in 913 patients with SCA The mean of total and unconjugated bilirubin and the frequency of cholelithiasis in GS patients were higher when compared to those without this condition, regardless of age (P 005) However, not cholelithiasis but total and unconjugated bilirubin levels were associated with βS haplotype These findings confirm in a large cohort that the UGT1A1 polymorphism influences cholelithiasis and hyperbilirubinemia in SCA HbF and alpha thalassemia also appear as modulators for cholelithiasis risk
TL;DR: The pathophysiology of sickle cell anemia, a hereditary hemoglobinopathy, has fascinated clinicians and scientists alike since its description over 100 years ago A single gene mutation in the HBB was found in this paper.
Abstract: The pathophysiology of sickle cell anemia, a hereditary hemoglobinopathy, has fascinated clinicians and scientists alike since its description over 100 years ago A single gene mutation in the HBB
TL;DR: The purpose of this letter is to determine if the onset of menopause is early or late in women with SCA and if hydroxyurea affects the onset.
TL;DR: In this paper, silent cerebral infarcts and arteriopathy are common and progressive in individuals with sickle cell anemia, however, most data describing brain lesions in SCE are cross-sectional or...
Abstract: Silent cerebral infarcts and arteriopathy are common and progressive in individuals with sickle cell anemia. However, most data describing brain lesions in sickle cell anemia are cross-sectional or...
TL;DR: In this paper, the authors explored how muscle dysfunction may explain the reduced functional capacity of patients with sickle cell disease (SCD) due to anemia and cardio-respiratory abnormalities.
Abstract: Patients with sickle cell disease (SCD) have reduced functional capacity due to anemia and cardio–respiratory abnormalities. Recent studies also suggest the presence of muscle dysfunction. However, the interaction between exercise capacity and muscle function is currently unknown in SCD. The aim of this study was to explore how muscle dysfunction may explain the reduced functional capacity. Nineteen African healthy subjects (AA), and 24 sickle cell anemia (SS) and 18 sickle cell hemoglobin C (SC) patients were recruited. Maximal isometric torque (Tmax) was measured before and after a self-paced 6-min walk test (6-MWT). Electromyographic activity of the Vastus Lateralis was recorded. The 6-MWT distance was reduced in SS (p < 0.05) and SC (p < 0.01) patients compared to AA subjects. However, Tmax and root mean square value were not modified by the 6-MWT, showing no skeletal muscle fatigue in all groups. In a multiple linear regression model, genotype, step frequency and hematocrit were independent predictors of the 6-MWT distance in SCD patients. Our results suggest that the 6-MWT performance might be primarily explained by anemia and the self-paced step frequency in SCD patients attempting to limit metabolic cost and fatigue, which could explain the absence of muscle fatigue.
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TL;DR: Sickle cell disease (SCD) is a multisystem disorder and the most common genetic disease in the United States, affecting 1 in 500 African Americans as discussed by the authors, and approximately 300,000 infants are born with SCD annually.
Abstract: Sickle cell disease (SCD) is a multisystem disorder and the most common genetic disease in the United States, affecting 1 in 500 African Americans. About 1 in 12 African Americans carry the autosomal recessive mutation, and approximately 300,000 infants are born with sickle cell anemia annually. The understanding of the phenotypic expression of the disease is still limited. However, environmental factors such as cold weather and air quality, infections, fetal hemoglobin level, and other genetic subtypes play a role in the manifestation of the disease. Clinical manifestations are variable and affect multiple systems, and generally cause lower life expectancy.
TL;DR: A comprehensive review of the pathophysiology, epidemiology, evaluation, and treatment of SCA-related stroke in developed and developing countries is presented in this article, where the authors identify the role of vascular injury, hypercoagulability and vaso-occlusion in SCA.
TL;DR: In this article, the authors investigated the effect of DMTs on tricuspid regurgitant velocity (TRV) elevation in children with SCA and concluded that hydroxyurea therapy may mitigate TRV elevation, possibly as a result of a reduction in hemolysis and improvement in anemia.
TL;DR: In this article, the transcranial Doppler (TCD) is used as a risk-screening tool for ischemic stroke in children with sickle cell anemia (SCA).
TL;DR: In this article, the authors evaluated the correlation between oxygenscan parameters and more well-known biomarkers of SCA such as fetal hemoglobin (HbF), F-cells, and dense red blood cells (DRBCs).
Abstract: Sickle cell anemia (SCA) is a hereditary hemoglobinopathy with a variable phenotype. There is no single biomarker that adequately predicts disease severity and can be used to monitor treatment response in patients in clinical trials and clinical care. The use of clinical outcomes, such as vaso-occlusive crises (VOC), requires long and expensive studies, sometimes with inconclusive results. To address these limitations, there are several biomarkers under study to improve the ability to predict complications and assess treatment response in both clinical and research settings. Oxygen gradient ektacytometry, also called as oxygenscan, is an assay that measures the effects of deoxygenation and reoxygenation on red blood cell (RBC) deformability and is gaining popularity in SCA research, because it captures the dynamic sickling capacity of a patient's RBCs as they are subjected to an oxygen gradient under steady shear stress. We describe here the oxygenscan methodology and evaluate the correlation between oxygenscan parameters and more well-known biomarkers of SCA such as fetal hemoglobin (HbF), F-cells, and dense red blood cells (DRBCs). Our data indicate that the oxygenscan curve is affected by all these parameters and the result incorporates the effects of %HbF, %F-cells, RBC hydration, and RBC membrane deformability.
TL;DR: It is unclear what types of anemia are most prevalent in patients with hidradenitis suppurativa, including females, because very few studies have investigated this correlation.
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01 Nov 2021TL;DR: In this paper, a multivariate logistic regression model was constructed to assess the risk of stillbirth using the following risk factors: SCD, numbers of pain crises and blood transfusions before delivery, delivery episode (as a proxy for parity), prior cesarean delivery, multiple gestation, patient age, marital status, race and ethnicity, ABO blood type, Rhesus (Rh) factor, and year of delivery.
Abstract: Importance Relative to what is known about pregnancy complications and sickle cell disease (SCD), little is known about the risk of pregnancy complications among those with sickle cell trait (SCT). There is a lack of clinical research among sickle cell carriers largely due to low sample sizes and disparities in research funding. Objective To evaluate whether there is an association between SCT and a stillbirth outcome. Design, setting, and participants This retrospective cohort study included data on deliveries occurring between January 1, 2010, and August 15, 2017, at 4 quaternary academic medical centers within the Penn Medicine health system in Pennsylvania. The population included a total of 2482 deliveries from 1904 patients with SCT but not SCD, and 215 deliveries from 164 patients with SCD. Data were analyzed from May 3, 2019, to September 16, 2021. Exposures The primary exposure of interest was SCT, identified using clinical diagnosis codes recorded in the electronic health record. Main outcomes and measures A multivariate logistic regression model was constructed to assess the risk of stillbirth using the following risk factors: SCD, numbers of pain crises and blood transfusions before delivery, delivery episode (as a proxy for parity), prior cesarean delivery, multiple gestation, patient age, marital status, race and ethnicity, ABO blood type, Rhesus (Rh) factor, and year of delivery. Results This cohort study included 50 560 patients (63 334 deliveries), most of whom were aged 25 to 34 years (29 387 of 50 560 [58.1%]; mean [SD] age, 29.5 [6.1] years), were single at the time of delivery (28 186 [55.8%]), were Black or African American (23 777 [47.0%]), had ABO blood type O (22 879 [45.2%]), and were Rhesus factor positive (44 000 [87.0%]). From this general population, 2068 patients (4.1%) with a sickle cell gene variation were identified: 1904 patients (92.1%) with SCT (2482 deliveries) and 164 patients (7.9%) with SCD (215 deliveries). In the fully adjusted model, SCT was associated with an increased risk of stillbirth (adjusted odds ratio [aOR], 8.94; 95% CI, 1.05-75.79; P = .045) while adjusting for the risk factors of SCD (aOR, 26.40; 95% CI, 2.48-280.90; P = .007) and multiple gestation (aOR, 4.68; 95% CI, 3.48-6.29; P Conclusions and relevance The results of this large, retrospective cohort study indicate an increased risk of stillbirth among pregnant people with SCT. These findings underscore the need for additional risk assessment during pregnancy for sickle cell carriers.
TL;DR: In this paper, the authors investigated the associations between TGFβ1 and classical laboratory biomarkers and inflammatory mediators, as well as the tissue inhibitor of metalloproteases-1 (TIMP-1) and matrix metallopease-9 (MMP-9), in pediatric patients with SCD in steady state: 84 with sickle cell anemia and 39 with hemoglobin SC disease (HbSC).
Abstract: 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 ( ) 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.