Infection and Potential Challenge of Childhood Mortality in Sickle Cell Disease: A Comprehensive Review of the Literature from a Global Perspective

• Published in: Thalassemia reports Vol. 13; no. 3; pp. 206 - 229
• Main Authors: Sahu, Tarun, Pande, Babita, Verma, Henu Kumar, Bhaskar, L V K S, Sinha, Meenakshi, Sinha, Ramanjan, Rao, Pasupuleti Visweswara
• Format: Journal Article
• Language: English
• Published: MDPI AG 01.09.2023
• Subjects: bacteria; children therapeutics; infection; molecular mechanism; sickle cell disease; virus
• ISSN: 2039-4365; 2039-4365
• DOI: 10.3390/thalassrep13030019

Sickle cell disease (SCD) is a complex genetic disorder associated with multiple clinical manifestations, including increased susceptibility to bacterial and viral infections. This review article presents a comprehensive analysis of the current literature obtained from various online databases focusing on the relationship between SCD and infections caused by specific pathogens, such as pneumonia- and influenza-causing pathogens, Escherichia coli, Staphylococcus aureus, parvovirus, and hepatitis viruses. We discuss the underlying mechanisms that contribute to the increased susceptibility of individuals with SCD to these infections, primarily related to the pathophysiology of variant hemoglobin (HbSS) and its impact on vascular occlusion, hemolysis, functional asplenia, and immune deficiency. Moreover, we highlight the significant burden of infections on SCD patients, particularly children under five years of age, where they are the leading cause of morbidity and mortality. Additionally, we address the challenges faced in attempts for reducing the global mortality rate associated with SCD, particularly in low-income countries, where factors such as increased pathogen exposure, co-morbidities like malnutrition, lower vaccination rates, and limited healthcare facilities contribute to the high disease burden. This review emphasizes the need for targeted interventions, improved healthcare access, vaccination programs, and infection prevention strategies to alleviate the impact of infections on individuals with SCD and reduce the global mortality rates associated with the disease.