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.
■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。