AUTHOR=Ceglie Giulia , Di Mauro Margherita , Tarissi De Jacobis Isabella , de Gennaro Francesca , Quaranta Martina , Baronci Carlo , Villani Alberto , Palumbo Giuseppe TITLE=Gender-Related Differences in Sickle Cell Disease in a Pediatric Cohort: A Single-Center Retrospective Study JOURNAL=Frontiers in Molecular Biosciences VOLUME=6 YEAR=2019 URL=https://www.frontiersin.org/journals/molecular-biosciences/articles/10.3389/fmolb.2019.00140 DOI=10.3389/fmolb.2019.00140 ISSN=2296-889X ABSTRACT=
Sickle cell disease (SCD) is one of the most common monogenic disease worldwide. The incidence of SCD is not strictly gender-related as it is transmitted as an autosomal recessive disorder. In particular, the gender-related differences in pediatric SCD are not well-characterized. To address this matter, we retrospectively analyzed the clinical records of 39 pediatric patients with a diagnosis of SCD (hemoglobin SS genotype) focusing on gender differences analyzing various aspects of the disease and comprising both acute symptoms and late complications. We found various gender-related differences in our pediatric population. In particular, pain crisis frequency per year was significantly increased in the male population with a mean number of crisis per year of 1.6 vs. 0.6 in the female population (