Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium
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 complication...
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| creator | Masese, Rita V Bulgin, Dominique Knisely, Mitchell R Preiss, Liliana Stevenson, Eleanor Hankins, Jane S Treadwell, Marsha J King, Allison A Gordeuk, Victor R Kanter, Julie Gibson, Robert Glassberg, Jeffrey A Tanabe, Paula Shah, Nirmish |
| description | 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.
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.
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 |
| doi_str_mv | 10.1371/journal.pone.0258638 |
| format | Article |
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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.
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).
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.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0258638</identifier><identifier>PMID: 34714833</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Acute Chest Syndrome - epidemiology ; Acute Chest Syndrome - etiology ; Adolescent ; Adult ; Age ; Anemia, Sickle Cell - complications ; Anemia, Sickle Cell - genetics ; Biology and Life Sciences ; Complications ; Complications and side effects ; Consortia ; Cross-Sectional Studies ; Demographic aspects ; Diagnosis ; Emergency medical care ; Female ; Females ; Fetuses ; Gender aspects ; Gender differences ; Genotypes ; Gibson, Jeffrey ; Health services utilization ; Hemoglobin ; Hemoglobin, Sickle - genetics ; Hostage negotiations ; Humans ; Hydroxyurea ; Life span ; Male ; Males ; Medical records ; Medical screening ; Medicine ; Medicine and Health Sciences ; Nursing schools ; Pain ; Pain - epidemiology ; Pain - etiology ; Patient Admission - statistics & numerical data ; Quality of life ; Self Report ; Sex Characteristics ; Sex differences ; Sickle cell anemia ; Sickle cell disease ; Social Sciences ; Stevenson, Jane ; Surveys and Questionnaires ; Young Adult</subject><ispartof>PloS one, 2021-10, Vol.16 (10), p.e0258638-e0258638</ispartof><rights>COPYRIGHT 2021 Public Library of Science</rights><rights>2021 Masese et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2021 Masese et al 2021 Masese et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c692t-a7deab0f12637ca00270a6ea15b795728d12c1d8073b936b44ad0607b0cc6563</citedby><cites>FETCH-LOGICAL-c692t-a7deab0f12637ca00270a6ea15b795728d12c1d8073b936b44ad0607b0cc6563</cites><orcidid>0000-0002-1431-8840</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8555833/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8555833/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,860,881,2096,2915,23845,27901,27902,53766,53768,79343,79344</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/34714833$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Wonkam, Ambroise</contributor><creatorcontrib>Masese, Rita V</creatorcontrib><creatorcontrib>Bulgin, Dominique</creatorcontrib><creatorcontrib>Knisely, Mitchell R</creatorcontrib><creatorcontrib>Preiss, Liliana</creatorcontrib><creatorcontrib>Stevenson, Eleanor</creatorcontrib><creatorcontrib>Hankins, Jane S</creatorcontrib><creatorcontrib>Treadwell, Marsha J</creatorcontrib><creatorcontrib>King, Allison A</creatorcontrib><creatorcontrib>Gordeuk, Victor R</creatorcontrib><creatorcontrib>Kanter, Julie</creatorcontrib><creatorcontrib>Gibson, Robert</creatorcontrib><creatorcontrib>Glassberg, Jeffrey A</creatorcontrib><creatorcontrib>Tanabe, Paula</creatorcontrib><creatorcontrib>Shah, Nirmish</creatorcontrib><creatorcontrib>Sickle Cell Disease Implementation Consortium</creatorcontrib><creatorcontrib>on behalf of the Sickle Cell Disease Implementation Consortium</creatorcontrib><title>Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>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.
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.
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).
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.</description><subject>Acute Chest Syndrome - epidemiology</subject><subject>Acute Chest Syndrome - etiology</subject><subject>Adolescent</subject><subject>Adult</subject><subject>Age</subject><subject>Anemia, Sickle Cell - complications</subject><subject>Anemia, Sickle Cell - genetics</subject><subject>Biology and Life Sciences</subject><subject>Complications</subject><subject>Complications and side effects</subject><subject>Consortia</subject><subject>Cross-Sectional Studies</subject><subject>Demographic aspects</subject><subject>Diagnosis</subject><subject>Emergency medical care</subject><subject>Female</subject><subject>Females</subject><subject>Fetuses</subject><subject>Gender aspects</subject><subject>Gender differences</subject><subject>Genotypes</subject><subject>Gibson, Jeffrey</subject><subject>Health services utilization</subject><subject>Hemoglobin</subject><subject>Hemoglobin, Sickle - genetics</subject><subject>Hostage negotiations</subject><subject>Humans</subject><subject>Hydroxyurea</subject><subject>Life span</subject><subject>Male</subject><subject>Males</subject><subject>Medical records</subject><subject>Medical screening</subject><subject>Medicine</subject><subject>Medicine and Health Sciences</subject><subject>Nursing schools</subject><subject>Pain</subject><subject>Pain - epidemiology</subject><subject>Pain - etiology</subject><subject>Patient Admission - statistics & numerical data</subject><subject>Quality of life</subject><subject>Self Report</subject><subject>Sex Characteristics</subject><subject>Sex differences</subject><subject>Sickle cell anemia</subject><subject>Sickle cell disease</subject><subject>Social Sciences</subject><subject>Stevenson, Jane</subject><subject>Surveys and Questionnaires</subject><subject>Young Adult</subject><issn>1932-6203</issn><issn>1932-6203</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><sourceid>DOA</sourceid><recordid>eNqNk89u1DAQxi0EomXhDRBYQkJw2MWOY8fhgFQt_1aqVKlbcbUcZ7zrksTbOEHlOXhhnE1a7aIeUA6JJr_vG8-MB6GXlCwoy-iHa9-3ja4WO9_AgiRcCiYfoVOas2QuEsIeH3yfoGchXBPCmRTiKTphaUZTydgp-rOG23mhA5S4dNZCC42BgF2Duy3gWjfOQuh053wTsG5KbHy9q5yZIt7i4MzPCrCBqooWAaLXR3wJO9922La-3hutR2g5QJ9HCK-iEdTQjO54Gf2ixvX1c_TE6irAi-k9Q1dfv1wtv8_PL76tlmfncyPypJvrrARdEEsTwTKjCUkyogVoyoss51kiS5oYWkqSsSJnokhTXRJBsoIYI7hgM_R6tN1VPqipnUHFTkpGOYmyGVqNROn1tdq1rtbtb-W1U_uAbzdKxxObClTOScEkJDZLZQosKwqdW2aT3FjOi9JEr09Ttr6ooTSx7lZXR6bHfxq3VRv_S0nOeRxVNHg3GbT-po9DUbULQ9d1A74fzp0TysQw5Bl68w_6cHUTtdGxANdYH_OawVSdCUlEImVMPEOLB6j4lFA7E--edTF-JHh_JIhMB7fdRvchqNX68v_Zix_H7NsDdgu66rbBV_3-Ih6D6Qia1ofQgr1vMiVqWJ27bqhhddS0OlH26nBA96K7XWF_Ae4OFgM</recordid><startdate>20211029</startdate><enddate>20211029</enddate><creator>Masese, Rita V</creator><creator>Bulgin, Dominique</creator><creator>Knisely, Mitchell R</creator><creator>Preiss, Liliana</creator><creator>Stevenson, Eleanor</creator><creator>Hankins, Jane S</creator><creator>Treadwell, Marsha J</creator><creator>King, Allison A</creator><creator>Gordeuk, Victor R</creator><creator>Kanter, Julie</creator><creator>Gibson, Robert</creator><creator>Glassberg, Jeffrey A</creator><creator>Tanabe, Paula</creator><creator>Shah, Nirmish</creator><general>Public Library of Science</general><general>Public Library of Science (PLoS)</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>IOV</scope><scope>ISR</scope><scope>3V.</scope><scope>7QG</scope><scope>7QL</scope><scope>7QO</scope><scope>7RV</scope><scope>7SN</scope><scope>7SS</scope><scope>7T5</scope><scope>7TG</scope><scope>7TM</scope><scope>7U9</scope><scope>7X2</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABJCF</scope><scope>ABUWG</scope><scope>AEUYN</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>ATCPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>D1I</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB.</scope><scope>KB0</scope><scope>KL.</scope><scope>L6V</scope><scope>LK8</scope><scope>M0K</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>M7S</scope><scope>NAPCQ</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PATMY</scope><scope>PDBOC</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PTHSS</scope><scope>PYCSY</scope><scope>RC3</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope><orcidid>https://orcid.org/0000-0002-1431-8840</orcidid></search><sort><creationdate>20211029</creationdate><title>Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium</title><author>Masese, Rita V ; Bulgin, Dominique ; Knisely, Mitchell R ; Preiss, Liliana ; Stevenson, Eleanor ; Hankins, Jane S ; Treadwell, Marsha J ; King, Allison A ; Gordeuk, Victor R ; Kanter, Julie ; Gibson, Robert ; Glassberg, Jeffrey A ; Tanabe, Paula ; Shah, Nirmish</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c692t-a7deab0f12637ca00270a6ea15b795728d12c1d8073b936b44ad0607b0cc6563</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Acute Chest Syndrome - 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Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PloS one</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Masese, Rita V</au><au>Bulgin, Dominique</au><au>Knisely, Mitchell R</au><au>Preiss, Liliana</au><au>Stevenson, Eleanor</au><au>Hankins, Jane S</au><au>Treadwell, Marsha J</au><au>King, Allison A</au><au>Gordeuk, Victor R</au><au>Kanter, Julie</au><au>Gibson, Robert</au><au>Glassberg, Jeffrey A</au><au>Tanabe, Paula</au><au>Shah, Nirmish</au><au>Wonkam, Ambroise</au><aucorp>Sickle Cell Disease Implementation Consortium</aucorp><aucorp>on behalf of the Sickle Cell Disease Implementation Consortium</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2021-10-29</date><risdate>2021</risdate><volume>16</volume><issue>10</issue><spage>e0258638</spage><epage>e0258638</epage><pages>e0258638-e0258638</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>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.
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.
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).
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.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>34714833</pmid><doi>10.1371/journal.pone.0258638</doi><tpages>e0258638</tpages><orcidid>https://orcid.org/0000-0002-1431-8840</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1932-6203 |
| ispartof | PloS one, 2021-10, Vol.16 (10), p.e0258638-e0258638 |
| issn | 1932-6203 1932-6203 |
| language | eng |
| recordid | cdi_plos_journals_2588315007 |
| source | Public Library of Science (PLoS) Journals Open Access; MEDLINE; DOAJ Directory of Open Access Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central; Free Full-Text Journals in Chemistry |
| subjects | Acute Chest Syndrome - epidemiology Acute Chest Syndrome - etiology Adolescent Adult Age Anemia, Sickle Cell - complications Anemia, Sickle Cell - genetics Biology and Life Sciences Complications Complications and side effects Consortia Cross-Sectional Studies Demographic aspects Diagnosis Emergency medical care Female Females Fetuses Gender aspects Gender differences Genotypes Gibson, Jeffrey Health services utilization Hemoglobin Hemoglobin, Sickle - genetics Hostage negotiations Humans Hydroxyurea Life span Male Males Medical records Medical screening Medicine Medicine and Health Sciences Nursing schools Pain Pain - epidemiology Pain - etiology Patient Admission - statistics & numerical data Quality of life Self Report Sex Characteristics Sex differences Sickle cell anemia Sickle cell disease Social Sciences Stevenson, Jane Surveys and Questionnaires Young Adult |
| title | Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-30T06%3A20%3A34IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Sex-based%20differences%20in%20the%20manifestations%20and%20complications%20of%20sickle%20cell%20disease:%20Report%20from%20the%20Sickle%20Cell%20Disease%20Implementation%20Consortium&rft.jtitle=PloS%20one&rft.au=Masese,%20Rita%20V&rft.aucorp=Sickle%20Cell%20Disease%20Implementation%20Consortium&rft.date=2021-10-29&rft.volume=16&rft.issue=10&rft.spage=e0258638&rft.epage=e0258638&rft.pages=e0258638-e0258638&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0258638&rft_dat=%3Cgale_plos_%3EA680628858%3C/gale_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2588315007&rft_id=info:pmid/34714833&rft_galeid=A680628858&rft_doaj_id=oai_doaj_org_article_950b38e2f7484e37bba9f3f29cf55bdc&rfr_iscdi=true |