Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Chronic Pulmonary Complications of Sickle Cell Disease
authors: Mehari A, Klings ESsummary/abstract:
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, most of African descent. Hemoglobin S (HbS) results from a glutamate-to-valine mutation of the sixth codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is associated with the most prevalent and severe form of the disease. Other SCD genotypes include HbSC, composed of one HbS allele and one HbC (glutamate-to-lysine mutation) allele; and HbS-β-thalassemia(0) or HbS-β-thalassemia(+), composed of one HbS allele and one β-thalassemia allele with absent or reduced β-chain production, respectively. Despite advances in care, median survival remains in the fifth decade, due in large part to chronic complications of the disease. Chronic pulmonary complications in SCD are major contributors to this early mortality. Although our understanding of these conditions has improved much over the past 10 to 15 years, there remains no specific treatment for pulmonary complications of SCD. It is unclear whether conventional treatment regimens directed at non-SCD populations have equivalent efficacy in patients with SCD. This represents a critical research need. In this review, the authors review 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 review highlights the advances as well as the knowledge gaps in this field to update clinicians and other health care providers and to garner research interest from the medical community.organization: Howard University College of Medicine, Washington, DC; Boston University School of Medicine
read more full text
Contrary to expectations: seasonal variation in sickle cell crisis – a nation wide data analysisBackground: Sickle cell crisis is a pain...
Phase 1 trial to test under-the-skin injection of sevuparin in sickle cell patientsModus Therapeutics is going to launch ...
Chronic Opioid Use Pattern in Adult Patients with Sickle Cell DiseaseBackground: Pain, the hallmark complicat...
Living With Sickle Cell Disease Is a Constant Battle With Pain, According to one Woman Who Has It“You don’t look sick.” That’s ...
Sickle Cell Treatments can Destroy Germ Cells in Boys, Affecting Fertility in Adulthood, Study SuggestsSome treatments for sickle cell disease ...
Randomized Trials Needed to Assess Benefits of Salmonella Vaccines in SCD Patients, Study SaysRandomized controlled trials are needed ...
Sickle Cell Patients are Innocent Victims of the Opioid Epidemic: Samir K. Ballas, MDhttps://www.youtube.com/watch?v=BOhY_gJo...
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.