Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom
Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom
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DOI：
10.1371/journal.pone.0099489
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发表时间：
2014-07-02
期刊：
影响因子：
3.7
通讯作者：
Machado, Roberto F.
中科院分区：
文献类型：
--
作者：
Gladwin, Mark T.;Barst, Robyn J.;Machado, Roberto F.
背景：在镰状细胞病(SCD)中，肺动脉高压作为死亡原因的作用是有争议的。方法和结果：我们评估了SCD患者估计的肺动脉收缩压升高与死亡率之间的关系。我们对WAK-PHaSST筛查队列中的患者进行了中位数29个月的跟踪调查。三尖瓣返流速度(TRV)=3.0m/S，对平均肺动脉压<Gt=25 mm Hg有67-75%的阳性预测值。在572名受试者中，11.2%的受试者TRV>；=3.0m/s。在582例测定NT-proBNP的患者中，24.1%的患者>；=160pg/m L。随访期死亡22例，TRV=3.0m/s者占50.00%，TRV为3.0m/s者占98%(p=3.0m/s)，NT-proBNP和NT-proBNP>；=160pg/m l组分别为4.6%(1.8~11.3；p=0.001)和14.9%(p=3.0m/s和160pg/mL)。年龄47岁、男性、慢性输血、WHO III-IV级、溶血标志物升高、铁蛋白和肌酐升高也与死亡风险增加相关。结论：TRV&>=3.0m/s的个体约占10%，是所有测量变量中死亡风险最高的。
Background: The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial.Methods and Results: We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)>= 3.0 m/s cuttof, which has a 67-75% positive predictive value for mean pulmonary artery pressure >= 25 mm Hg was used. Among 572 subjects, 11.2% had TRV >= 3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values >= 160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV= 3.0 m/sec and 98% with TRV, 3.0 m/sec (p= 3.0 m/sec, 4.6 (1.8-11.3; p = 0.001) for NT-proBNP >= 160 pg/mL, and 14.9 (5.5-39.9; p= 3.0 m/sec and NT-proBNP >= 160 pg/mL. Age >47 years, male gender, chronic transfusions, WHO class III-IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death.Conclusions: A TRV >= 3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable.