Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium

<h4>Introduction</h4> Sex-based clinical outcome differences in sickle cell disease (SCD) remain largely unknown despite evidence that female sex is associated with an increased lifespan. To better characterize sex-based differences in SCD, we assessed pain, treatment characteristics, la...

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Autores principales:	Rita V. Masese, Dominique Bulgin, Mitchell R. Knisely, Liliana Preiss, Eleanor Stevenson, Jane S. Hankins, Marsha J. Treadwell, Allison A. King, Victor R. Gordeuk, Julie Kanter, Robert Gibson, Jeffrey A. Glassberg, Paula Tanabe, Nirmish Shah, on behalf of the Sickle Cell Disease Implementation Consortium
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/afb52558f554465c8f3beb8b28bdfae7
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spelling	oai:doaj.org-article:afb52558f554465c8f3beb8b28bdfae72021-11-04T07:42:07ZSex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium1932-6203https://doaj.org/article/afb52558f554465c8f3beb8b28bdfae72021-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8555833/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Introduction</h4> Sex-based clinical outcome differences in sickle cell disease (SCD) remain largely unknown despite evidence that female sex is associated with an increased lifespan. To better characterize sex-based differences in SCD, we assessed pain, treatment characteristics, laboratory measures and complications among males and females currently enrolled in the Sickle Cell Disease Implementation Consortium (SCDIC) registry. <h4>Methods</h4> The SCDIC consists of eight comprehensive SCD centers and one data coordinating center that received funding from the National Heart Lung and Blood Institute to improve outcomes for individuals with SCD. Eligibility criteria included: 15 to 45 years of age and a confirmed diagnosis of SCD. Self-report surveys were completed and data were also abstracted from the participants’ medical records. <h4>Results</h4> A total of 2,124 participants were included (mean age: 27.8 years; 56% female). The majority had hemoglobin SS SCD genotype. Females had worse reports of pain severity (mean (SD) T-score 51.6 (9.6) vs 49.3 (10), p<0.001), more vaso-occlusive episodes (p = 0.01) and a higher occurrence of 3 or more hospital admissions in the past year (30.9% vs. 25.5, p = 0.03). On multivariable analysis, males had higher odds of acute chest syndrome (odds ratio (OR) 1.4, p = 0.002), cardiovascular (OR 1.70, p<0.001) and musculoskeletal (OR 1.33, p = 0.0034) complications and lower odds of depression (OR 0.77, p = 0.0381). Females had higher fetal hemoglobin levels with and without hydroxyurea use (9.6% vs 8.5%, p = 0.03 and 3% vs 2.2%, p = 0.0005, respectively). <h4>Conclusion</h4> Our data suggests that sex differences in clinical outcomes do occur among individuals with SCD. Future research needs to explore the mechanisms underlying these differences.Rita V. MaseseDominique BulginMitchell R. KniselyLiliana PreissEleanor StevensonJane S. HankinsMarsha J. TreadwellAllison A. KingVictor R. GordeukJulie KanterRobert GibsonJeffrey A. GlassbergPaula TanabeNirmish Shahon behalf of the Sickle Cell Disease Implementation ConsortiumPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 10 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Rita V. Masese Dominique Bulgin Mitchell R. Knisely Liliana Preiss Eleanor Stevenson Jane S. Hankins Marsha J. Treadwell Allison A. King Victor R. Gordeuk Julie Kanter Robert Gibson Jeffrey A. Glassberg Paula Tanabe Nirmish Shah on behalf of the Sickle Cell Disease Implementation Consortium Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium
description	<h4>Introduction</h4> Sex-based clinical outcome differences in sickle cell disease (SCD) remain largely unknown despite evidence that female sex is associated with an increased lifespan. To better characterize sex-based differences in SCD, we assessed pain, treatment characteristics, laboratory measures and complications among males and females currently enrolled in the Sickle Cell Disease Implementation Consortium (SCDIC) registry. <h4>Methods</h4> The SCDIC consists of eight comprehensive SCD centers and one data coordinating center that received funding from the National Heart Lung and Blood Institute to improve outcomes for individuals with SCD. Eligibility criteria included: 15 to 45 years of age and a confirmed diagnosis of SCD. Self-report surveys were completed and data were also abstracted from the participants’ medical records. <h4>Results</h4> A total of 2,124 participants were included (mean age: 27.8 years; 56% female). The majority had hemoglobin SS SCD genotype. Females had worse reports of pain severity (mean (SD) T-score 51.6 (9.6) vs 49.3 (10), p<0.001), more vaso-occlusive episodes (p = 0.01) and a higher occurrence of 3 or more hospital admissions in the past year (30.9% vs. 25.5, p = 0.03). On multivariable analysis, males had higher odds of acute chest syndrome (odds ratio (OR) 1.4, p = 0.002), cardiovascular (OR 1.70, p<0.001) and musculoskeletal (OR 1.33, p = 0.0034) complications and lower odds of depression (OR 0.77, p = 0.0381). Females had higher fetal hemoglobin levels with and without hydroxyurea use (9.6% vs 8.5%, p = 0.03 and 3% vs 2.2%, p = 0.0005, respectively). <h4>Conclusion</h4> Our data suggests that sex differences in clinical outcomes do occur among individuals with SCD. Future research needs to explore the mechanisms underlying these differences.
format	article
author	Rita V. Masese Dominique Bulgin Mitchell R. Knisely Liliana Preiss Eleanor Stevenson Jane S. Hankins Marsha J. Treadwell Allison A. King Victor R. Gordeuk Julie Kanter Robert Gibson Jeffrey A. Glassberg Paula Tanabe Nirmish Shah on behalf of the Sickle Cell Disease Implementation Consortium
author_facet	Rita V. Masese Dominique Bulgin Mitchell R. Knisely Liliana Preiss Eleanor Stevenson Jane S. Hankins Marsha J. Treadwell Allison A. King Victor R. Gordeuk Julie Kanter Robert Gibson Jeffrey A. Glassberg Paula Tanabe Nirmish Shah on behalf of the Sickle Cell Disease Implementation Consortium
author_sort	Rita V. Masese
title	Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium
title_short	Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium
title_full	Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium
title_fullStr	Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium
title_full_unstemmed	Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium
title_sort	sex-based differences in the manifestations and complications of sickle cell disease: report from the sickle cell disease implementation consortium
publisher	Public Library of Science (PLoS)
publishDate	2021
url	https://doaj.org/article/afb52558f554465c8f3beb8b28bdfae7
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Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium

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