Chronic Pulmonary Complications of Sickle Cell Disease.
Chest. 2016 Jan 13;
Authors: Mehari A, Klings ES
Sickle cell disease (SCD), the most common genetic hemolytic anemia worldwide, affects 250,000 births annually. In the United States, SCD affects approximately 100 000 individuals, mostly of African descent. Hemoglobin S (HbS) results from a GLU to VAL mutation of the 6(th) codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is the most prevalent and severe. Other SCD genotypes include HbSC disease, comprised of one HbS and one HbC (GLU to LYS mutation) allele, and HbS-β thalassemia(0 or +) comprised of one HbS allele and one β thalassemia allele with reduced or absent β chain production. Despite recent advances in care, the median survival remains in the 5(th) decade, due in large part to chronic complications of the disease. Chronic pulmonary complications in SCD are major contributors to this early mortality. While our understanding of these conditions has improved much over the past 10-15 years, there remains no specific treatment for pulmonary complications of SCD. It is unclear if conventional treatment regimens directed at non-SCD populations have equivalent efficacy in SCD patients. This represents a critical research need. In this review, the authors will overview the state of the art understanding of the following pulmonary complications of SCD: 1) Pulmonary hypertension; 2) Venous thromboembolic disease; 3) Sleep-disordered breathing; 4) Asthma and recurrent wheezing; and 5) Pulmonary function abnormalities. This manuscript will highlight the recent advances as well as the knowledge gaps in this field to update clinicians and other health care providers and garner research interest from the medical community.
PMID: 26836905 [PubMed - as supplied by publisher]