PDF(Pubmed)
Abstract：
UNASSIGNED: Historically, sickle cell disease (SCD) patients experiencing frequent hospitalized vaso-occlusive crises (HVOC) have been associated with increased mortality, yet recent data reflecting the widespread use of hydroxyurea and advancements in disease management remain limited. Our study aims to assess the association between HVOC and mortality or severe complications in patients with SCD in this new treatment landscape.
UNASSIGNED: This was a retrospective observational cohort study using the French national health data system. Between 01-01-2012 and 12-31-2018, all SCD patients ≥16 years old (ICD-10 codes D57.0-2) were included and followed until 12-31-2018. HVOC was defined as a hospitalization of ≥1 night with primary diagnosis of SCD with crisis, following an emergency room visit. The association between HVOC and severe complications was assessed with a Cox proportional hazards model.
UNASSIGNED: In total, 8018 patients (56.6% females; 4538/8018) were included. The 2018 SCD standardized one-year period prevalence was 17.9 cases/100,000 person-years [17.4; 18.3]. The mean rate was 0.84 (1.88) HVOC/person-year. In 2018, 70% (5323/7605), 22% (1671/7605), and 8% (611/7605) of patients experienced 0, 1-2, or 3+ HVOCs, respectively. The median survival time between HVOCs was 415 days [386; 439]. Overall, 312 patients died (3.9%) with a mean age of 49.8 (19.4). Compared to patients without HVOC, the hazard ratios of death in patients with 1-2 or 3+ HVOCs the year prior to death were 1.67 [1.21; 2.30] and 3.70 [2.30; 5.93], respectively. Incidence of acute chest syndrome, pulmonary embolism, osteonecrosis, and sepsis increased with the HVOCs category, but not stroke. In 2018, 29.5% (180/611) of patients with 3+ HVOCs did not take hydroxyurea.
UNASSIGNED: Patients must be closely monitored during their hospitalizations to intensify treatment and check treatment compliance. Innovative therapies are also required.
UNASSIGNED: The study was funded by Novartis.
UNASSIGNED: This was a retrospective observational cohort study using the French national health data system. Between 01-01-2012 and 12-31-2018, all SCD patients ≥16 years old (ICD-10 codes D57.0-2) were included and followed until 12-31-2018. HVOC was defined as a hospitalization of ≥1 night with primary diagnosis of SCD with crisis, following an emergency room visit. The association between HVOC and severe complications was assessed with a Cox proportional hazards model.
UNASSIGNED: In total, 8018 patients (56.6% females; 4538/8018) were included. The 2018 SCD standardized one-year period prevalence was 17.9 cases/100,000 person-years [17.4; 18.3]. The mean rate was 0.84 (1.88) HVOC/person-year. In 2018, 70% (5323/7605), 22% (1671/7605), and 8% (611/7605) of patients experienced 0, 1-2, or 3+ HVOCs, respectively. The median survival time between HVOCs was 415 days [386; 439]. Overall, 312 patients died (3.9%) with a mean age of 49.8 (19.4). Compared to patients without HVOC, the hazard ratios of death in patients with 1-2 or 3+ HVOCs the year prior to death were 1.67 [1.21; 2.30] and 3.70 [2.30; 5.93], respectively. Incidence of acute chest syndrome, pulmonary embolism, osteonecrosis, and sepsis increased with the HVOCs category, but not stroke. In 2018, 29.5% (180/611) of patients with 3+ HVOCs did not take hydroxyurea.
UNASSIGNED: Patients must be closely monitored during their hospitalizations to intensify treatment and check treatment compliance. Innovative therapies are also required.
UNASSIGNED: The study was funded by Novartis.
摘要：
■历史上，镰状细胞病（SCD）患者经历频繁的住院血管闭塞危象（HVOC）与死亡率增加有关，然而,反映羟基脲广泛使用和疾病管理进展的最新数据仍然有限.我们的研究旨在评估在这种新的治疗环境中，HVOC与SCD患者的死亡率或严重并发症之间的关系。
■这是一项使用法国国家健康数据系统的回顾性观察性队列研究。在2012年1月1日至2018年12月31日期间，纳入所有≥16岁（ICD-10代码D57.0-2）的SCD患者，并随访至2018年12月31日。HVOC定义为住院≥1晚，主要诊断为SCD伴危象，在急诊室访问之后。使用Cox比例风险模型评估HVOC与严重并发症之间的关联。
■总共，包括8018例患者（56.6%为女性；4538/8018）。2018年SCD标准化一年期患病率为17.9例/100,000人年[17.4;18.3]。平均为0.84（1.88）HVOC/人年。2018年，70%(5323/7605)，22%（1671/7605），8%(611/7605)的患者出现0、1-2或3+HVOCs，分别。HVOCs之间的中位生存时间为415天[386;439]。总的来说,312例患者死亡（3.9%），平均年龄49.8岁（19.4岁）。与没有HVOC的患者相比，死亡前一年1-2或3+HVOCs患者的死亡风险比为1.67[1.21;2.30]和3.70[2.30;5.93]，分别。急性胸部综合征的发病率，肺栓塞,骨坏死,脓毒症随着HVOCs类别的增加而增加，但不是中风。2018年，29.5%(180/611)的3+HVOCs患者未服用羟基脲。
■患者在住院期间必须密切监测，以加强治疗并检查治疗依从性。还需要创新疗法。
■这项研究由诺华公司资助。
■这是一项使用法国国家健康数据系统的回顾性观察性队列研究。在2012年1月1日至2018年12月31日期间，纳入所有≥16岁（ICD-10代码D57.0-2）的SCD患者，并随访至2018年12月31日。HVOC定义为住院≥1晚，主要诊断为SCD伴危象，在急诊室访问之后。使用Cox比例风险模型评估HVOC与严重并发症之间的关联。
■总共，包括8018例患者（56.6%为女性；4538/8018）。2018年SCD标准化一年期患病率为17.9例/100,000人年[17.4;18.3]。平均为0.84（1.88）HVOC/人年。2018年，70%(5323/7605)，22%（1671/7605），8%(611/7605)的患者出现0、1-2或3+HVOCs，分别。HVOCs之间的中位生存时间为415天[386;439]。总的来说,312例患者死亡（3.9%），平均年龄49.8岁（19.4岁）。与没有HVOC的患者相比，死亡前一年1-2或3+HVOCs患者的死亡风险比为1.67[1.21;2.30]和3.70[2.30;5.93]，分别。急性胸部综合征的发病率，肺栓塞,骨坏死,脓毒症随着HVOCs类别的增加而增加，但不是中风。2018年，29.5%(180/611)的3+HVOCs患者未服用羟基脲。
■患者在住院期间必须密切监测，以加强治疗并检查治疗依从性。还需要创新疗法。
■这项研究由诺华公司资助。
