Pulmonary Artery Thrombosis during Acute Chest Syndrome in Sickle Cell Disease

Pulmonary Artery Thrombosis during Acute Chest Syndrome in Sickle Cell Disease
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DOI:
10.1164/rccm.201105-0783oc
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发表时间:
2011-11-01
影响因子:
24.7
通讯作者:
Maitre, Bernard
Maitre, Bernard
中科院分区:
医学1区
文献类型:
--
作者:
Dessap, Armand Mekontso;Deux, Jean-Francois;Maitre, Bernard

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理由:镰状细胞病患者急性胸综合征(ACS)的病理生理是复杂的,肺动脉血栓形成(PT)可能是导致这一并发症的原因之一。目的:应用多探测器计算机断层扫描(MDCT)评价急性冠脉综合征(ACS)中PT的发生率。方法:我们筛选了125例连续144次ACS发作的患者。121例mdct(103例连续患者)纳入研究。测量和主要结果:20例mdct显示PT阳性,确定患病率为17%(95%置信区间,10-23%)。经修订的日内瓦临床概率评分在有PT和没有PT的患者之间相似。在ACS期间,d -二聚体检测通常呈阳性(95%)。与没有PT的患者相比,有PT的患者较少发现ACS的诱发因素。PT患者在ACS发病时血小板计数(517[273-729]比307 [228-412]10(9)/L, P < 0.01)和胆红素(28[19-43]比44 [31-71]mu mol/L, P < 0.01)水平明显高于其他患者。此外,PT患者住院期间血小板计数峰值(537[345-785]比417 [330-555]10(9)/L, P = 0.048)、胆红素峰值(36[18-51]比46[32-83]mu mol/L, P = 0.048)和乳酸脱氢酶峰值(357[320-704]比604 [442-788]IU/L, P = 0.01)均高于其他患者。结论:PT在ACS中并不罕见,更可能发生在血小板计数较高、溶血率较低的ACS患者中。镰状细胞病患者表现为提示ACS的呼吸系统症状,可从PT评估中获益。
Rationale: The pathophysiology of acute chest syndrome (ACS) in patients with sickle cell disease is complex, and pulmonary artery thrombosis (PT) may contribute to this complication.Objectives: To evaluate the prevalence of PT during ACS using multi-detector computed tomography (MDCT).Methods: We screened 125 consecutive patients during 144 ACS episodes. One hundred twenty-one MDCTs (in 103 consecutive patients) were included in the study.Measurements and Main Results: Twenty MDCTs were positive for PT, determining a prevalence of 17% (95% confidence interval, 10-23%). Revised Geneva clinical probability score was similar between patients with PT and those without. D-dimer testing was very often positive (95%) during ACS. A precipitating factor for ACS was less frequently found in patients with PT as compared with those without. Patients with PT exhibited significantly higher platelet counts (517 [273-729] vs. 307 [228-412] 10(9)/L, P < 0.01) and lower bilirubin (28 [19-43] vs. 44 [31-71] mu mol/L, P < 0.01) levels at the onset of ACS as compared with others. In addition, patients with PT had a higher platelet count peak (537 [345-785] vs. 417 [330-555] 10(9)/L, P = 0.048) and smaller bilirubin peak (36[18-51] vs. 46[32-83] mu mol/L, P = 0.048) and lactate dehydrogenase peak (357 [320-704] vs. 604 [442-788] IU/L, P = 0.01) during hospital stay as compared with others.Conclusions: PT is not a rare event in the context of ACS and seems more likely in patients with higher platelet counts and lower hemolytic rate during ACS. Patients with sickle cell disease presenting with respiratory symptoms suggestive of ACS may benefit from evaluation for PT.