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Whole blood single nucleotide polymorphism genotyping was performed using a customized array, and a genome-wide association study (GWAS) was conducted to identify single nucleotide polymorphisms associated with priapism.<\/jats:p><\/jats:sec><jats:sec><jats:title>Main Outcome Measure<\/jats:title><jats:p>Of the 1,314 male patients in the cohort, 188 experienced priapism (14.3%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Priapism was more common among older patients (P\u00a0= .006) and more severe SCD genotypes such as homozygous SS (P &amp;lt; .0001). In the genotype- and age-matched analyses, associations with priapism were found for pulmonary hypertension (P\u00a0= .05) and avascular necrosis (P\u00a0= .01). The GWAS suggested replication of a previously reported candidate gene association of priapism for the gene transforming growth factor beta receptor 3 (TGFBR3) (P\u00a0= 2\u00a0\u00d7 10\u22124).<\/jats:p><\/jats:sec><jats:sec><jats:title>Clinical Implications<\/jats:title><jats:p>Older patients with more severe genotypes are at higher risk of priapism, and there is a lack of consensus on standard treatment strategies for priapism in SCD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Strengths &amp; Limitations<\/jats:title><jats:p>This study characterizes SCD patients with any history of priapism from a large multi-center cohort. Replication of the GWAS in an independent cohort is required to validate the results.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These findings extend the understanding of risk factors associated with priapism in SCD and identify genetic markers to be investigated in future studies to further elucidate priapism pathophysiology.<\/jats:p><\/jats:sec>","DOI":"10.1016\/j.jsxm.2019.09.012","type":"journal-article","created":{"date-parts":[[2019,10,24]],"date-time":"2019-10-24T22:30:41Z","timestamp":1571956241000},"page":"1988-1999","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":21,"title":["Clinical and Genetic Predictors of Priapism in Sickle Cell Disease: Results from the Recipient Epidemiology and Donor Evaluation Study III Brazil Cohort Study"],"prefix":"10.1093","volume":"16","author":[{"given":"Mina","family":"Cintho Ozahata","sequence":"first","affiliation":[{"name":"University of S\u00e3o 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