Acute chest syndrome in adults with sickle cell disease - Therapeutic approach, outcome, and results of BAL in a monocentric series of 107 episodes
Acute chest syndrome in adults with sickle cell disease - Therapeutic approach, outcome, and results of BAL in a monocentric series of 107 episodes
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DOI：
10.1378/chest.117.5.1386
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发表时间：
2000-05-01
期刊：
影响因子：
9.6
通讯作者：
Godeau, B
中科院分区：
文献类型：
--
作者：
Maitre, B;Habibi, A;Godeau, B
研究目的：急性胸部综合征（ACS）是镰状细胞病（SCD）中常见的潜在严重肺部疾病。本研究的目的是报告法国SCD拉幅机中成人患者连续ACS发作的临床特征和结局。所有患者均按照统一的治疗方案进行治疗，仅在ACS的更严重临床形式中应用输血。结果：在6年期间，77名成人患者（平均年龄29 ± 7岁; 78%血红蛋白[Hb] SS; 14% Hb SC; 8% Rb SP +地中海贫血）中有107次连续发作。78%的患者有相关的血管闭塞危象，在一半的病例中，这些危象先于胸部体征。急性和基线水平之间的比较显示Hb水平存在统计学显著差异（下降1.6 - 2.25 g/dL，取决于Hb基因型），WBC计数（增加9.2 +/- 8.3 x 10（9）/L）;血小板计数（增加67 +/- 209 x 10（9）/L）和乳酸脱氢酶值（增加358 +/- 775 IU/L），在42%没有麻醉剂滥用迹象的患者中检测到高碳酸血症。我们在43例接受BAL的患者中发现了31例（77%）含有脂肪滴的肺泡巨噬细胞的高百分比。细菌培养结果几乎总是阴性，但在96次发作中开始给予抗生素治疗后进行。107例ACS事件中有50例（47%）需要输血。5例患者死亡，所有的transfused.Conclusions：这些结果证实，脂肪栓塞可能是一个常见的机制，ACS在成人患者，但是，脂肪栓塞是不相关的一个更严重的临床过程中，这表明支气管镜检查和BAL对这些患者的管理影响不大。限制最严重的ACS病例输血似乎不会增加死亡率。
Study objectives: Acute chest syndrome (ACS) is a frequent and potentially severe pulmonary illness in sickle cell disease (SCD), The aim of the study was to report the clinical features and outcome of consecutive ACS episodes in adult patients in a French SCD tenter. All patients were treated according to an uniform therapeutic protocol applying transfusion only in the more severe clinical form of ACS,Results: There were 107 consecutive episodes in 77 adult patients (mean age, 29 +/- 7 years; 78% hemoglobin [Hb] SS; 14% Hb SC; and 8% Rb SP + thalassemia) over a 6-year period. Seventy-eight percent of our patients had an associated vaso-occlusive crisis that preceded the chest signs in half of the cases. Comparison between acute and baseline levels showed a statistically significant difference in Hb levels (drop of 1.6 to 2.25 g/dL depending on Hb genotype), WBC count (increase of 9.2 +/- 8.3 x 10(9)/L); platelet count (increase of 67 +/- 209 x 10(9)/L); and lactate dehydrogenase values (increase of 358 +/- 775 IU/L) in ACS patients, Hypercapnia was detected in 42% of patients without sign of narcotic abuse. We identified a high percentage of alveolar macrophages containing fat droplets in 31 of 43 (77%) patients who underwent BAL. Bacterial culture findings were almost always negative, but were performed after starting antibiotic therapy that was administered in 96 episodes. Transfusion was required in 50 of 107 ACS events (47%). Five patients died, and all were transfused.Conclusions: These results confirm that fat embolism is probably a frequent mechanism of ACS in adult patients, However, fat embolism was not associated with a more severe clinical course, suggesting that bronchoscopy and BAL have Little impact on the management of these patients. Restricting transfusion to the most severe ACS cases does not seem to increase the mortality rate.