School of Nursing, Duke University, Durham, North Carolina, United States of America.
College of Nursing, University of Tennessee Knoxville, Knoxville, Tennessee, United States of America.
PLoS One. 2021 Oct 29;16(10):e0258638. doi: 10.1371/journal.pone.0258638. eCollection 2021.
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.
尽管有证据表明女性性别与寿命延长有关，但镰状细胞病（SCD）中基于性别的临床结局差异在很大程度上仍未得到充分了解。为了更好地描述 SCD 中基于性别的差异，我们评估了目前参加镰状细胞病实施联合会（SCDIC）登记处的男性和女性的疼痛、治疗特征、实验室测量值和并发症。
SCDIC 由 8 个综合性 SCD 中心和 1 个数据协调中心组成，该中心获得了国家心肺血液研究所的资助，以改善 SCD 患者的结局。入选标准包括：15 至 45 岁，确诊为 SCD。完成自我报告调查，并从参与者的病历中提取数据。
共纳入 2124 名参与者（平均年龄：27.8 岁；56%为女性）。大多数人具有血红蛋白 SS SCD 基因型。女性疼痛严重程度报告较差（平均（SD）T 评分 51.6（9.6）与 49.3（10），p<0.001），血管阻塞发作更多（p = 0.01），过去一年有 3 次或以上住院的发生率更高（30.9%与 25.5%，p = 0.03）。多变量分析显示，男性急性胸痛综合征的几率更高（比值比（OR）1.4，p = 0.002），心血管（OR 1.70，p<0.001）和肌肉骨骼（OR 1.33，p = 0.0034）并发症的几率较低，抑郁的几率较低（OR 0.77，p = 0.0381）。女性在使用和不使用羟基脲时胎儿血红蛋白水平较高（9.6%与 8.5%，p = 0.03 和 3%与 2.2%，p = 0.0005）。
我们的数据表明，SCD 患者的临床结局存在性别差异。未来的研究需要探讨这些差异的潜在机制。
