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儿童镰状细胞病急性胸部综合征的早期无创通气和非常规输血:描述性研究。

Early Noninvasive Ventilation and Nonroutine Transfusion for Acute Chest Syndrome in Sickle Cell Disease in Children: A Descriptive Study.

机构信息

Pediatric Intensive Care Unit, AP-HP, Hôpital Necker Enfants Malades, Paris, France.

Department of Pediatric, AP-HP, Hôpital Necker Enfants Malades, Paris, France.

出版信息

Pediatr Crit Care Med. 2018 May;19(5):e235-e241. doi: 10.1097/PCC.0000000000001468.

DOI:10.1097/PCC.0000000000001468
PMID:29356722
Abstract

OBJECTIVES

To describe the need for transfusion and short- and long-term evolutions of pediatric sickle cell disease patients with acute chest syndrome for whom early continuous noninvasive ventilation represented first-line treatment.

DESIGN

Single-center retrospective chart study in PICU.

SETTING

A tertiary and quaternary referral PICU.

PATIENTS

All sickle cell disease patients 5-20 years old admitted with confirmed acute chest syndrome and not transfused in the previous month were included.

INTERVENTIONS

None.

MEASUREMENTS AND MAIN RESULTS

Demographic data, laboratory and radiologic findings, transfusions, invasive ventilation, oxygen and noninvasive ventilation settings, duration of opioid treatment, length of hospital stay, and severe sickle cell disease complications in the ensuing 2 years were extracted from medical charts. Sixty-six acute chest syndrome in 48 patients were included. Continuous early noninvasive ventilation was well tolerated in 65 episodes, with positive expiratory pressure 4 cm H2O and pressure support 10 cm H2O (median) administered continuously, then discontinued during 7 days (median). No patient necessitated invasive ventilation or died. Twenty-three acute chest syndrome (35%) received transfusions; none received blood exchange. Transfused patients had more frequent upper lobe radiologic involvement, more severe anemia, higher reticulocyte counts, and higher C-reactive protein than nontransfused patients. Their evolution was more severe in terms of length of opioid requirement, length of noninvasive ventilation treatment, overall time on noninvasive ventilation, and length of stay. At 2-year follow-up after the acute chest syndrome episode, no difference was observed between the two groups.

CONCLUSIONS

Early noninvasive ventilation combined with nonroutine transfusion is well tolerated in acute chest syndrome in children and may spare transfusion in some patients. Early recognition of patients still requiring transfusion is essential and warrants further studies.

摘要

目的

描述急性胸痛综合征伴镰状细胞病患儿输血需求及短期和长期演变情况,这些患儿的一线治疗为早期持续无创通气。

设计

重症监护病房(PICU)的单中心回顾性图表研究。

地点

三级和四级转诊 PICU。

患者

所有年龄在 5-20 岁、确诊为急性胸痛综合征且在过去一个月内未输血的镰状细胞病患者均纳入研究。

干预措施

无。

测量和主要结果

从病历中提取人口统计学数据、实验室和影像学发现、输血、有创通气、氧疗和无创通气设置、阿片类药物治疗持续时间、住院时间以及随后 2 年内严重镰状细胞病并发症等信息。48 例患者中有 66 例发生急性胸痛综合征。65 例患者早期持续无创通气耐受良好,呼气末正压 4cmH2O,压力支持 10cmH2O(中位数)持续给予,7 天内停止(中位数)。无一例患者需要有创通气或死亡。23 例(35%)急性胸痛综合征患者接受输血;无一例患者接受换血治疗。输血患者的上肺叶影像学受累更频繁,贫血更严重,网织红细胞计数和 C 反应蛋白更高。与未输血患者相比,他们在阿片类药物需求时间、无创通气治疗时间、总体无创通气时间和住院时间方面的病情更严重。急性胸痛综合征发作后 2 年随访时,两组之间未观察到差异。

结论

急性胸痛综合征患儿早期接受无创通气联合非常规输血治疗耐受性良好,某些患者可能无需输血。早期识别仍需输血的患者至关重要,需要进一步研究。

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