Respiratory manifestations of sickle cell disease in children: a comprehensive review for the pediatrician

Introduction: Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by the production of sickle hemoglobin, leading to red blood cells sickling and hemolysis in hypoxic conditions. The resulting acute and chronic endothelial inflammation leads to chronic organ damage. Respiratory manifestations in SCD usually start from childhood and represent the leading causes of morbidity and mortality. Nevertheless, they are generally poorly addressed or recognized later in life, often contributing to a more severe course and complications.

Areas covered: This narrative review aims to outline the significant acute and chronic respiratory manifestations in children with SCD, focusing on prevention and clinical management. Compelling issues that need to be addressed in the future are also discussed. We searched the PubMed database for original papers written in English. Age restrictions were set for children (birth to 18 years). No limitations were set for the date and study country.

Expert opinion: Early detection and treatment of respiratory manifestations in SCD should be central to follow-up with patients affected by SCD. Nonetheless, studies are lacking, especially in pediatric age, and there is still no consensus on their management. Further research is strongly needed to accomplish universally accepted guidelines to guarantee patients the best care possible.