Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study.
Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study.
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DOI：
10.1002/ajh.25799
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发表时间：
2020-07
影响因子：
12.8
通讯作者：
Gordeuk VR
中科院分区：
文献类型：
--
作者：
Nouraie M;Darbari DS;Rana S;Minniti CP;Castro OL;Luchtman-Jones L;Sable C;Dham N;Kato GJ;Gladwin MT;Ensing G;Arteta M;Campbell A;Taylor JG 6th;Nekhai S;Gordeuk VR
在美国，镰状细胞病（SCD）的死亡率在18-20岁后增加。死亡风险的生物标志物可以识别需要密集随访和早期或新干预的患者。我们在2006-2010年前瞻性招募了510例年龄在3-20岁之间的SCD患者进入一项观察性研究，并对497例患者进行了中位88个月（范围1-105）的随访。我们假设三尖瓣反流速度（TRV）反映的肺动脉收缩压升高与死亡率相关。估计18年生存率为99%，25年生存率为94%。10例患者中有7例死亡原因已知：4例卒中（出血性2例，梗死性1例，未指明1例），1例多器官功能衰竭，1例细小病毒B19感染，1例猝死。在20.0%的死亡患者和4.6%的存活患者中观察到基线TRV ≥2.7 m/s（在年龄匹配和性别匹配的非SCD对照组中，高于平均值>2 SD）（通过对数秩检验，P = 0.012，用于生存平等）。与TRV升高最密切相关的基线变量是高溶血率。与死亡率相关的其他生物标志物为铁蛋白≥2000 μg/L（60%的死亡患者与7.8%的存活患者观察到，P < .001），1分钟用力呼气量与用力肺活量比值（FEV 1/FVC）<0.80（71.4%死亡患者vs 18.8%存活患者，P <0.001），中性粒细胞计数≥10×109/L（30.0%死亡患者vs 7.9%存活患者，P = 0.018）。在SCD儿童、青少年和年轻成人中，TRV、铁蛋白和中性粒细胞稳态升高以及FEV 1/FVC比值降低可能是与死亡风险增加相关的生物标志物。
In the US, mortality in sickle cell disease (SCD) increases after age 18–20 years. Biomarkers of mortality risk can identify patients who need intensive follow-up and early or novel interventions. We prospectively enrolled 510 SCD patients aged 3–20 years into an observational study in 2006–2010 and followed 497 patients for a median of 88 months (range 1–105). We hypothesized that elevated pulmonary artery systolic pressure as reflected in tricuspid regurgitation velocity (TRV) would be associated with mortality. Estimated survival to 18 years was 99% and to 25 years, 94%. Causes of death were known in seven of 10 patients: stroke in four (hemorrhagic two, infarctive one, unspecified one), multiorgan failure one, parvovirus B19 infection one, sudden death one. Baseline TRV ≥2.7 m/second (>2 SD above the mean in age-matched and gender-matched non-SCD controls) was observed in 20.0% of patients who died vs 4.6% of those who survived (P = .012 by the log rank test for equality of survival). The baseline variable most strongly associated with an elevated TRV was a high hemolytic rate. Additional biomarkers associated with mortality were ferritin ≥2000 μg/L (observed in 60% of patients who died vs 7.8% of survivors, P < .001), forced expiratory volume in 1 minute to forced vital capacity ratio (FEV1/FVC) <0.80 (71.4% of patients who died vs 18.8% of survivors, P < .001), and neutrophil count ≥10×109/L (30.0% of patients who died vs 7.9% of survivors, P = .018). In SCD children, adolescents and young adults, steady-state elevations of TRV, ferritin and neutrophils and a low FEV1/FVC ratio may be biomarkers associated with increased risk of death.
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影响因子：
12.8
作者：
Gordeuk, Victor R.;Reboussin, David M.;McLaren, Christine E.;Barton, James C.;Acton, Ronald T.;McLaren, Gordon D.;Harris, Emily L.;Reiss, Jacob A.;Adams, Paul C.;Speechley, Mark;Phatak, Pradyumna D.;Sholinsky, Phyliss;Eckfeldt, John H.;Chen, Wen-Pin;Passmore, Leah;Dawkins, Fitzroy W.
通讯作者：
Dawkins, Fitzroy W.
影响因子：
6.5
作者：
Couque, Nathalie;Girard, Delphine;Benkerrou, Malika
通讯作者：
Benkerrou, Malika
影响因子：
5.5
作者：
Levin, Adeera;Stevens, Paul E.
通讯作者：
Stevens, Paul E.
影响因子：
39.3
作者：
Damy, Thibaud;Bodez, Diane;Bartolucci, Pablo
通讯作者：
Bartolucci, Pablo
影响因子：
3.7
作者：
Gladwin, Mark T.;Barst, Robyn J.;Machado, Roberto F.
通讯作者：
Machado, Roberto F.