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

Publication ,  Journal Article
Masese, RV; Bulgin, D; Knisely, MR; Preiss, L; Stevenson, E; Hankins, JS; Treadwell, MJ; King, AA; Gordeuk, VR; Kanter, J; Gibson, R; Shah, N ...
Published in: PLoS One
2021

INTRODUCTION: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Duke Scholars

Published In

PLoS One

DOI

EISSN

1932-6203

Publication Date

2021

Volume

16

Issue

10

Start / End Page

e0258638

Location

United States

Related Subject Headings

  • Young Adult
  • Surveys and Questionnaires
  • Sex Characteristics
  • Self Report
  • Patient Admission
  • Pain
  • Male
  • Humans
  • Hemoglobin, Sickle
  • General Science & Technology
 

Citation

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Masese, R. V., Bulgin, D., Knisely, M. R., Preiss, L., Stevenson, E., Hankins, J. S., … Sickle Cell Disease Implementation Consortium, . (2021). Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium. PLoS One, 16(10), e0258638. https://doi.org/10.1371/journal.pone.0258638
Masese, Rita V., Dominique Bulgin, Mitchell R. Knisely, Liliana Preiss, Eleanor Stevenson, Jane S. Hankins, Marsha J. Treadwell, et al. “Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium.PLoS One 16, no. 10 (2021): e0258638. https://doi.org/10.1371/journal.pone.0258638.
Masese RV, Bulgin D, Knisely MR, Preiss L, Stevenson E, Hankins JS, et al. Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium. PLoS One. 2021;16(10):e0258638.
Masese, Rita V., et al. “Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium.PLoS One, vol. 16, no. 10, 2021, p. e0258638. Pubmed, doi:10.1371/journal.pone.0258638.
Masese RV, Bulgin D, Knisely MR, Preiss L, Stevenson E, Hankins JS, Treadwell MJ, King AA, Gordeuk VR, Kanter J, Gibson R, Glassberg JA, Tanabe P, Shah N, 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. PLoS One. 2021;16(10):e0258638.

Published In

PLoS One

DOI

EISSN

1932-6203

Publication Date

2021

Volume

16

Issue

10

Start / End Page

e0258638

Location

United States

Related Subject Headings

  • Young Adult
  • Surveys and Questionnaires
  • Sex Characteristics
  • Self Report
  • Patient Admission
  • Pain
  • Male
  • Humans
  • Hemoglobin, Sickle
  • General Science & Technology