Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease
Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease
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DOI：
10.1002/ajh.21569
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发表时间：
2010-01-01
影响因子：
12.8
通讯作者：
De Castro, Laura M.
中科院分区：
文献类型：
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作者：
Fitzhugh, Courtney D.;Lauder, Naudia;De Castro, Laura M.
镰状细胞病（SCD）与早期死亡率有关。我们试图确定成人SCD患者死亡的发生率、原因和危险因素。所有2000年1月至2005年4月在杜克大学医学中心成人镰状细胞中心就诊的年龄≥ 18岁的患者均入选。43例患者（21例男性和22例女性）在研究期间死亡。女性的中位生存年龄为39岁（95% CI：34-56），男性为40岁（95% CI：34-48），总体为40岁（95% CI：35-48）。心源性死亡占25.6%（11/43例患者）;肺部死亡占14.0%（6例患者）;其他SCD相关死亡占32.6%（14例患者）;未知死亡占14.0%（6例患者）;其他死亡占14.0%（6例患者）。无脉性电活动停止、肺栓塞、多器官衰竭和中风是最常见的死亡原因。在死亡患者中，最常见的病前疾病为心肺疾病：急性胸部综合征/肺炎（58.1%）、肺动脉高压（pHTN; 41.9%）、全身性HTN（25.6%）、充血性心力衰竭（25.6%）、心肌梗死（20.9%）和心律失常（14.0%）。与存活患者相比，死亡患者的三尖瓣返流射流速度显著更高（3.1 m/sec vs. 2.6 misec，P < 0.001），血红蛋白显著更低（8.3 g/dL vs. 9.2 g/dL，P < 0.05）。随着预防和治疗方面的进步，包括羟基脲治疗，感染等急性并发症不再是死亡的主要原因;相反，死亡原因和发病前状况正在转变为慢性心肺并发症。此外，心律失常导致过早死亡在SCD中认识不足，需要进一步研究。Am. J. Hematol. 85：36-40，2010. (C)2009 Wiley-Liss，Inc.
Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >= 18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and April 2005 were enrolled. Forty-three patients (21 males and 22 females) died during the study period. The median age of survival was 39 years for females (95% CI: 34-56), 40 years for males (95% CI: 34-48), and 40 years overall (95% CI: 35-48). Cardiac causes of death accounted for 25.6% (11/43 patients); pulmonary, 14.0% (six patients); other SCD related, 32.6% (14 patients); unknown, 14.0% (six patients); and others, 14.0% (six patients). Pulseless electrical activity arrest, pulmonary emboli, multiorgan failure, and stroke were the most frequent causes of death. Among the deceased patients, the most common premorbid conditions were cardiopulmonary: acute chest syndrome/pneumonia (58.1%), Pulmonary hypertension (pHTN; 41.9%), systemic HTN (25.6%), congestive heart failure (25.6%), myocardial infarction (20.9%), and arrhythmias (14.0%). Tricuspid regurgitant jet velocity was significantly higher (3.1 m/sec vs. 2.6 misec, P < 0.001) and hemoglobin significantly lower (8.3 g/dL vs. 9.2 g/dL, P < 0.05) in deceased patients when compared with patients who lived, respectively. With improved preventive and therapeutic advances, including hydroxyurea therapy, acute complications such as infection are no longer the leading cause of death; instead, causes of death and premorbid conditions are shifting to chronic cardiopulmonary complications. Further, arrhythmia leading to premature death is under-recognized in SCD and warrants further investigation. Am. J. Hematol. 85:36-40, 2010. (C) 2009 Wiley-Liss, Inc.