Abstract

SICKLE CELL DISEASE IN CHILDREN: A REVIEW OF DIAGNOSIS, TREATMENT AND MANAGEMENT

Suraj G. Kokare*, Neha L. Zod, Harigopal S. Sawarkar, Nikita V. Mahalle and Umesh R. Bansod

ABSTRACT

This review focuses on the diagnosis, treatment, and management of sickle cell disease (SCD) in children. Sickle cell disease is an inherited hemoglobinopathy that primarily affects individuals of African, Mediterranean, and Middle Eastern descent. The condition is caused by a mutation in the β-globin gene, leading to abnormal hemoglobin production. The resulting sickled red blood cells cause vaso-occlusion, hemolysis, and a range of complications including pain crises, anemia, infections, and organ damage. The review discusses the pathophysiology of SCD, its clinical manifestations, diagnostic techniques, and available treatments, including blood transfusions, pain management, bone marrow transplantation, gene therapy, and stem cell therapy. Prevention strategies and the role of early intervention through neonatal screening and prophylactic care are also highlighted.

Keywords: Sickle cell disease (SCD), Hemoglobinopathy, Vaso-occlusion, Pain crisis Anemia, Hemolysis, Bone marrow transplantation, Gene therapy, Stem cell therapy, Pediatric sickle cell management, Neonatal screening Prophylactic care, Organ damage.