RT Journal Article
SR Electronic
T1 Management of chronic respiratory complications in children and adolescents with sickle cell disease
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 200054
DO 10.1183/16000617.0054-2020
VO 29
IS 157
A1 Michele Arigliani
A1 Atul Gupta
YR 2020
UL http://err.ersjournals.com/content/29/157/200054.abstract
AB Sickle cell disease (SCD) is a life-threatening hereditary blood disorder that affects millions of people worldwide, especially in sub-Saharan Africa. This condition has a multi-organ involvement and highly vascularised organs, such as the lungs, are particularly affected. Chronic respiratory complications of SCD involve pulmonary vascular, parenchymal and airways alterations. A progressive decline of lung function often begins in childhood. Asthma, sleep-disordered breathing and chronic hypoxaemia are common and associated with increased morbidity. Pulmonary hypertension is a serious complication, more common in adults than in children. Although there is a growing attention towards respiratory care of patients with SCD, evidence regarding the prognostic meaning and optimal management of pulmonary issues in children with this condition is limited.This narrative review presents state-of-the-art evidence regarding the epidemiology, pathophysiology and therapeutic options for chronic respiratory complications commonly seen in paediatric patients with SCD. Furthermore, it highlights the gaps in the current knowledge and indicates future directions for studies that aim to improve our understanding of chronic respiratory complications in children with SCD.Respiratory disease has a high burden in sickle cell disease (SCD), with chronic pulmonary complications often beginning in childhood. We provide an overview of pathophysiology and therapeutic aspects of chronic respiratory disease in children with SCD. https://bit.ly/3165pNk