Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of SCD, contributing to the vascular complications and tissue damage. This review is centered on exploring the role of the inflammatory complement system in the pathophysiology of SCD. Our goal is to offer a comprehensive summary of the existing evidence regarding complement activation in patients with SCD, encompassing both steady-state conditions and episodes of vaso-occlusive events. Additionally, we will discuss the proposed mechanisms by which the complement system may contribute to tissue injury in this pathology. Finally, we will provide an overview of the available evidence concerning the effectiveness of therapeutic interventions aimed at blocking the complement system in the context of SCD and discuss the perspective of complement inhibition.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.lpm.2023.104205 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Thrombotic Microangiopathy Associated with Systemic Adeno-Associated Virus Gene Transfer: Review of Reported Cases.
Hum Gene Ther
January 2025
BridgeBio Gene Therapy, Palo Alto, California, USA.
Complement-mediated thrombotic microangiopathy (TMA) in the form of atypical hemolytic uremic syndrome (aHUS) has emerged as an immune complication of systemic adeno-associated virus (AAV) gene transfer that was unforeseen based on nonclinical studies. Understanding this phenomenon in the clinical setting has been limited by incomplete data and a lack of uniform diagnostic and reporting criteria. While apparently rare based on available information, AAV-associated TMA/aHUS can pose a substantial risk to patients including one published fatality.
View Article and Find Full Text PDFNon-canonical Roles of Complement in the CNS: From Synaptic Organizer to Presynaptic Modulator of Glutamate Transmission.
Curr Neuropharmacol
January 2025
Department of Pharmacy, DIFAR, Pharmacology and Toxicology Section, University of Genoa, Viale Cembrano 4, 16148, Genoa, Italy.
The central nervous system (CNS) is not an immune-privileged compartment, but it is intimately intertwined with the immune system. Among the components shared by the two compartments is the complement, a main constituent of innate immunity, which is also produced centrally and controls the development and organization of synaptic connections. Complement is considered a doubled-faced system that, besides controlling the physiological development of the central network, also subserves synaptic engulfment pivotal to the progression of neurodegenerative diseases.
View Article and Find Full Text PDFPreprocedural imaging guiding ventricular tachycardia ablation in structural heart disease.
J Arrhythm
February 2025
Department of Cardiology ULSSM Lisbon Portugal.
Background: Integration of preprocedural imaging techniques in ventricular tachycardia (VT) ablation may improve the identification of arrhythmogenic substrates, particularly relevant for patients with nonischemic cardiomyopathy (NICM) with sub-optimal outcomes. We assessed the impact of advanced preprocedural imaging on the safety and long-term efficacy of radiofrequency catheter ablation (RCA) for VT, comparing patients with NICM and ischemic cardiomyopathy (ICM).
Methods: In this prospective, single-center study, consecutive patients referred for scar-related VT ablation underwent multidetector computed tomography (MDCT) and late gadolinium enhancement cardiac magnetic resonance (LGE-CMR).
Association of branched-chain amino acids and risk of three urologic cancers: a Mendelian randomization study.
Transl Cancer Res
December 2024
Department of Urology, Affiliated Hospital of North Sichuan Medical College, Nanchong, China.
Background: Multiple studies suggest a plausible connection between urologic cancers and branched-chain amino acids (BCAAs) breakdown metabolic enzymes. Nevertheless, there is scarce exploration into the variations in circulating BCAAs. In our research, we utilize bidirectional, two-sample Mendelian randomization (MR) analysis to predict the link between BCAAs levels and three distinct types of urological tumors.
View Article and Find Full Text PDFStreptococcus pyogenes M1UK Variant-Associated Sartorius Muscle Necrotizing Soft Tissue Infection: A Case Report and Literature Review.
Cureus
December 2024
Laboratory of Infectious Diseases, Graduate School of Infection Control Sciences & Ōmura Satoshi Memorial Institute, Kitasato University, Tokyo, JPN.
Necrotizing soft tissue infections (NTSIs) represent a concept of necrotizing infections involving the skin, subcutaneous tissue, fascia, and muscle, and it is a potentially fatal disease. Early exploratory incision is strongly recommended for both the diagnosis and treatment of necrotizing soft tissue infections. Treatment of necrotizing soft tissue infections requires the administration of appropriate antimicrobial agents and adequate surgical debridement.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!