Bilateral lung transplantation in a 9-year-old girl with bronchopulmonary dysplasia with pulmonary hypertension.
Bilateral lung transplantation in a 9-year-old girl with bronchopulmonary dysplasia with pulmonary hypertension.
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一名患有支气管肺发育不良并肺动脉高压的 9 岁女孩接受双侧肺移植。
DOI：
10.1002/ppul.25597
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发表时间：
2021
影响因子：
3.1
通讯作者：
Yoshinori Okada
中科院分区：
文献类型：
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作者：
Chiharu Ota;Ryoko Saito;Junya Tominaga;Shinya Iwasawa;Takashi Hirama;Yasushi Matsuda;Katsunori Ono;Takehiko Onoki;Masato Kimura;Yoshinori Kawabata;Yoshinori Okada
研究背景支气管肺发育不良（BPD）是一种发生于早产儿的慢性呼吸系统疾病，其预后取决于合并症，包括纤维化、肺气肿或肺动脉高压（PH）。我们报告了一例9岁女孩，她发生了与重度BPD相关的PH（BPD-PH），并接受了双侧肺移植（BLTx）。病例描述一名9岁女孩被收治到我科接受BLTx。她在妊娠23周零4天出生，体重507 g。她在前2个月接受了通气，由于反复的严重呼吸道感染，需要进一步的呼吸护理。她在6个月大时被诊断为BPD-PH，开始口服肺血管扩张剂。在危及生命的急性加重后，她在4岁时登记为肺移植候选人。胸部计算机断层扫描（CT）显示重度肺部疾病，上叶和下叶存在磨玻璃样阴影和肺气肿性低密度区。进行了来自脑死亡男性供体的BLTx。她切除的肺的病理结果显示囊状、发育不全的肺，伴有肺泡修复/再生，以及外周动脉的中膜肥大和肌肉化。术后病程基本平稳。她是免费的氧气管理，并没有表现出PH的迹象后，6个月的surgical.ConclusionThis是第一个案例报告BLTx在儿科，不可逆的BPD-PH患者详细的病理组织学结果和临床检查。肺移植是重度BPD-PH的治疗选择之一。
BackgroundBronchopulmonary dysplasia (BPD) is a chronic respiratory disease that occurs in premature infants and the prognosis is variable depending on the comorbidities including fibrosis, emphysema, or pulmonary hypertension (PH). We present a case of a 9‐year‐old girl who developed PH associated with severe BPD (BPD‐PH) and underwent bilateral lung transplantation (BLTx). Case description A 9‐year‐old girl was admitted to our department to undergo BLTx. She was born at 23 weeks and 4 days gestation with a weight of 507 g. She received ventilation for the first 2 months and required further respiratory care due to repetitive, severe respiratory infections. She was diagnosed with BPD‐PH at 6 months of age and oral administration of pulmonary vasodilators were initiated. She was registered as a lung transplant candidate at 4 years of age after the life‐threatening exacerbation. Chest computed tomography (CT) revealed severe lung conditions with ground‐glass opacities and emphysematous low‐density areas in the upper and lower lobes. BLTx from a brain‐dead male donor was performed. The pathological findings of her resected lung revealed saccular, hypoplastic lung with alveolar repair/regeneration, and medial hypertrophy and muscularization of peripheral arteries. The postoperative course was mostly uneventful. She was free from oxygen administration and showed no signs of PH after 6 months of the surgery.ConclusionThis is the first case report of BLTx in a pediatric, irreversible BPD‐PH patient with detailed pathohistological findings and clinical examination. Lung transplantation is one of the treatment options for severe BPD‐PH.