Rev Mal Respir. 2023 Apr 12:S0761-8425(23)00107-9. doi: 10.1016/j.rmr.2023.03.002. Online ahead of print.
Sickle-cell disease is an autosomal recessive genetic disorder of hemoglobin that causes systemic damage. Hypoxia is the main actor of sickle-cell disease. It initiates acutely the pathogenic cascade leading to tissue damages that in turn induce chronic hypoxia. Lung lesions represent the major risk of morbidity and mortality. Management of sickle-cell disease requires a tight collaboration between hematologists, intensivists and chest physicians. Recurrent episodes of thrombosis and hemolysis characterize the disease. New therapeutic protocols, associating hydroxyurea, transfusion program and stem cell transplantation in severe cases allow a prolonged survival until the fifth decade. However, recurrent pain, crisis, frequent hospital admissions due to infection, anemia or acute chest syndrome and chronic complications leading to organ deficiencies degrade the patients' quality of life. In low-income countries where the majority of sickle-cell patients are living, the disease is still associated with a high mortality in childhood. This paper focuses on acute chest syndrome and chronic lung manifestations.
PMID:37059617 | DOI:10.1016/j.rmr.2023.03.002