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婴儿重度支气管肺发育不良的肺活检。

Lung biopsy in infants with severe bronchopulmonary dysplasia.

机构信息

Department of Pediatrics, Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Department of Neonatology, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, China.

出版信息

Pediatr Pulmonol. 2023 Jul;58(7):2068-2075. doi: 10.1002/ppul.26433. Epub 2023 May 3.

DOI:10.1002/ppul.26433
PMID:37133233
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10502733/
Abstract

INTRODUCTION

Lung biopsy is infrequently performed in the population of infants with severe bronchopulmonary dysplasia (BPD). Yet, its presentation may overlap with other infant diffuse lung diseases, including those within the spectrum of childhood interstitial lung diseases (chILD). Lung biopsy might differentiate between these entities or identify those with an extremely poor prognosis. Both might alter the clinical management of some infants diagnosed with BPD.

METHODS

In this tertiary referral center, we drew on a retrospective cohort of 308 preterm infants with severe BPD. Of these, nine underwent lung biopsy between 2012 and 2017. We aimed to assess the indication for lung biopsy, the prior clinical history, safety of the procedure, and describe the biopsy findings. Finally, we considered management decisions in relation to the biopsy results in these patients.

RESULTS

All nine infants undergoing biopsy survived the procedure. The mean gestational age and birth weight of the nine patients were 30 ± 3 (range 27-34) weeks and 1421 ± 571 (range 611-2140) grams. All infants received serial echocardiograms to assess pulmonary hypertension, genetic testing, and computed tomography angiography (CTA) before biopsy. In all nine patients moderate to severe alveolar simplification was present and eight had some degree of pulmonary interstitial glycogenosis (PIG) ranging from focal to diffuse. Following biopsy, two infants with PIG received high dose systemic steroids and two separate infants had care redirected.

CONCLUSION

In our cohort, lung biopsy was safe and well tolerated. Findings from lung biopsy may aid decision making in selected patients as a part of a step-wise diagnostic algorithm.

摘要

简介

在患有严重支气管肺发育不良(BPD)的婴儿人群中,肺活检的实施频率较低。然而,其表现可能与其他婴儿弥漫性肺疾病重叠,包括儿童间质性肺疾病(chILD)谱内的疾病。肺活检可能有助于区分这些疾病,或识别预后极差的疾病。这两种情况都可能改变一些被诊断为 BPD 的婴儿的临床管理。

方法

在这个三级转诊中心,我们回顾性分析了 308 名患有严重 BPD 的早产儿的队列。其中,9 名婴儿在 2012 年至 2017 年间接受了肺活检。我们旨在评估肺活检的适应证、既往临床病史、操作的安全性,并描述活检结果。最后,我们考虑了这些患者的活检结果与管理决策之间的关系。

结果

所有接受活检的 9 名婴儿均在操作过程中存活。9 名患者的平均胎龄和出生体重分别为 30±3(范围 27-34)周和 1421±571(范围 611-2140)克。所有婴儿在接受活检前均接受了连续超声心动图检查以评估肺动脉高压、基因检测和计算机断层血管造影(CTA)。在所有 9 名患者中,均存在中至重度肺泡简化,8 名患者存在不同程度的肺间质糖原沉积(PIG),范围从局灶性到弥漫性。在接受活检后,2 名患有 PIG 的婴儿接受了大剂量全身类固醇治疗,2 名婴儿的护理方向发生了变化。

结论

在我们的队列中,肺活检是安全且耐受良好的。肺活检的结果可能有助于在选定的患者中作为逐步诊断算法的一部分做出决策。

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Respir Med. 2018 Jul;140:11-20. doi: 10.1016/j.rmed.2018.05.009. Epub 2018 May 17.
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Interstitial lung disease in newborns.新生儿间质性肺疾病
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Bronchopulmonary Dysplasia: Chronic Lung Disease of Infancy and Long-Term Pulmonary Outcomes.支气管肺发育不良:婴儿期慢性肺病及长期肺部结局
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Functional Characterization of ATP-Binding Cassette Transporter A3 Mutations from Infants with Respiratory Distress Syndrome.患有呼吸窘迫综合征婴儿的ATP结合盒转运蛋白A3突变的功能特性
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