Background: Sickle cell cardiomyopathy is becoming a neglected tropical cardiovascular disease, yet the most common cause of death in HbSS patients. Myocardial strain using speckle tracking has recently been validated as a tool for the detection of subclinical left ventricular systolic dysfunction. Its utility in sickle cell disease in Nigeria is unknown.
Objectives: The study aims to evaluate global longitudinal left ventricular (GL LV) strain pattern in black SCD patients in steady state, and correlate that with clinico-laboratory and electrocardiographic (ECG) measurements. Furthermore, we will evaluate the timeline for the earliest detectable cardiac changes in SCD detectable by ECG or speckle tracking.
Method: 60 sickle cell patients between ages 5 and 40 years will be matched with 60 controls. Baseline clinic-laboratory data will be collected. All participants will have electrocardiography and speckle-tracking echocardiography of the left ventricle. The primary outcome is the global LV longitudinal strain in HBSS and matched control. Secondary outcomes are the clinico-laboratory and electrocardiographic correlates of LV strain in black HBSS patients as well as tracking the earliest detectable cardiovascular change in the life spectrum from childhood to adulthood. Regression analysis and other statistical models will be used.
Conclusion: The simplicity of the study design is crafted to spark interest in life-threatening cardiovascular complications of sickle cell disease beyond pulmonary hypertension.
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Psychol Health Med
December 2024
Department of Psychology, University of Nigeria, Enugu, Nigeria.
Despite extensive research on the impact of stigma on the health-related quality of life (HRQoL) of people with sickle cell disease, gaps remain in understanding the mechanisms through which this association occurs. We investigated how stigma impacts HRQoL among people with sickle cell disease (SCD) through distress and the moderating role of social support in this association. Utilizing a cross-sectional design, we sampled 165 people with SCD in Nigeria, who completed relevant measures.
View Article and Find Full Text PDFCytotherapy
December 2024
Molecular and Clinical Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland, USA.
Sickle cell disease (SCD) is the most common inherited blood disorder worldwide, impacting millions and imposing severe healthcare challenges, particularly in resource-limited regions. Current treatments have variable efficacy and require lifelong adherence. Allogeneic Hematopoietic Stem Cell Transplantation can be curative but comes with significant side effects and limited donor availability limits its widespread applicability.
View Article and Find Full Text PDFTrop Med Infect Dis
November 2024
School of Health Systems & Public Health, University of Pretoria, Pretoria 0028, South Africa.
Sickle cell disease (SCD) is a prevalent inherited blood disorder, particularly affecting populations in Africa. This review examined the disease's burden, its diverse clinical presentations, and the challenges associated with its management in African settings. Africa bears a significant burden of SCD, with prevalence varying across countries and age groups.
View Article and Find Full Text PDFInt J Neonatal Screen
December 2024
Laboratory of Genomic, Epigenetics, Precision and Predictive Medicine, School of Medicine, Mohammed VI University of Sciences and Health, Casablanca 82403, Morocco.
Unlabelled: Newborn screening (NBS) represents an important public health measure for the early detection of specified disorders; such screening can prevent disability and death, not only from metabolic disorders but also from endocrine, hematologic, immune, and cardiac disorders. Screening for critical congenital conditions affecting newborns' health is a great challenge, especially in developing countries such as Morocco, where NBS program infrastructure is lacking. In addition, the consanguinity rate is high in Morocco.
View Article and Find Full Text PDFInt J Neonatal Screen
November 2024
Laboratoire de Biologie Médicale Multi-Sites (LBMMS), Service de Biochimie et Biologie Moléculaire Grand Est, Hospices Civils de Lyon, 69500 Bron, France.
In France, sickle cell disease newborn screening (SCD NBS) has been targeted to at-risk regions since 1984, but generalization to the whole population will be implemented from November 2024. Although tandem mass spectrometry (MS/MS) is already used for the NBS of several inherited metabolic diseases, its application for SCD NBS has not been widely adopted worldwide. The aim of this study was to evaluate a dedicated MS/MS kit (Targeted MS/MS Hemo, ZenTech, LaCAR Company, Liege, Belgium) for SCD NBS and to compare the results obtained with those from an NBS reference center using matrix-assisted laser desorption/ionization time of flight (MALDI-TOF) and cation-exchange high-performance liquid chromatography (CE-HPLC, Variant NBS, Biorad Laboratories, Inc.
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