CFTR gene therapy, a method to rescue lung hypoplasia in congenital diaphragmatic hernia?

Jacques R. Bourbon; Alexandra Benachi

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CFTR gene therapy, a method to rescue lung hypoplasia in congenital diaphragmatic hernia?

机译：CFTR基因疗法是一种挽救先天性diaphragm肌疝的肺发育不全的方法吗？

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despite progress in neonatal care and despite changing concepts in treatment in the recent years, the mortality rate of infants with congenital diaphragmatic hernia (CDH) remains high (20). Pulmonary hypoplasia and vascular alterations associated with CDH have tremendous postnatal functional impact on respiratory function, including ventilatory insufficiency and pulmonary hypertension. In the current article in focus, Larson and Cohen (Ref. 14, see p. L4 in this issue), using an experimental model of CDH in the rat, propose a very innovative new approach to treat lung hypoplasia that consists of inducing transient overexpression of cystic fibrosis transmem-brane conductance regulator (CFTR) in fetal lung

机译：尽管近年来新生儿护理有所进步，尽管治疗观念发生了变化，但先天性diaphragm肌疝（CDH）婴儿的死亡率仍然很高（20）。与CDH相关的肺发育不全和血管改变对呼吸功能（包括通气功能不全和肺动脉高压）的产后功能产生巨大影响。在当前关注的文章中，Larson和Cohen（本期参考文献14，请参阅第L4页）使用大鼠CDH的实验模型，提出了一种非常创新的新方法来治疗肺发育不全，该方法包括诱导瞬时过表达肺肺囊性纤维化跨膜电导调节剂的测定

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《American Journal of Physiology》 |2006年第1期|共3页
作者
Jacques R. Bourbon; Alexandra Benachi;
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正文语种 eng
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Lung; Hypoplastic; Cystic Fibrosis Transmembrane Conductance Regulator; Hernia; Diaphragmatic; 肺; 囊性纤维化穿膜传导调节因子; 疝; 横膈;

机译：Lung;Hypoplastic;Cystic Fibrosis Transmembrane Conductance Regulator;Hernia;Diaphragmatic;肺;囊性纤维化穿膜传导调节因子;疝;横膈;

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1. CFTR gene therapy, a method to rescue lung hypoplasia in congenital diaphragmatic hernia? [J] . Jacques R. Bourbon, Alexandra Benachi American Journal of Physiology . 2006,第1期

机译：CFTR基因疗法是一种挽救先天性diaphragm肌疝的肺发育不全的方法吗？
2. Improvement of pulmonary hypoplasia associated with congenital diaphragmatic hernia by in utero CFTR gene therapy. [J] . Larson JE, Cohen JC American Journal of Physiology . 2006,第1期

机译：宫内CFTR基因治疗可改善与先天性diaphragm肌疝相关的肺发育不全。
3. The relationship of diaphragmatic defect, liver growth, and lung hypoplasia in nitrofen-induced congenital diaphragmatic hernia in the rat. [J] . Langwieler T, Fiegel HC, Alaamian M, Pediatric surgery international . 2004,第7期

机译：硝基芬诱发的先天性diaphragm肌疝的大鼠of肌缺损，肝脏生长和肺发育不良的关系。
4. Development of a robotic manipulator system for Congenital Diaphragmatic Hernia [C] . 2011 IEEE International Conference on Systems, Man, and Cybernetics . 2011

机译：先天性Dia肌疝的机械手系统的开发
5. Investigating the mechanisms underlying congenital diaphragmatic hernia and lung maturation. [D] . Branchfield, Kelsey. 2015

机译：研究先天性diaphragm肌疝和肺成熟的潜在机制。
6. Non-casual Association Between Congenital Pulmonary Airway Malformations/Primary Lung Hypoplasia and Congenital Diaphragmatic Hernia (CDH) [O] . Gloria Pelizzo, Sara Costanzo, Giorgio Giuseppe O. Selvaggio, 2020

机译：先天性肺气道畸形/原发性肺发育性和先天性膈疝（CDH）之间的非休闲协会
7. Improvement of Pulmonary Hypoplasia Associated with Congenital Diaphragmatic Hernia by In Utero CFTR Gene Therapy [O] . Janet E. Larson, J. Craig Cohen, Dr. Janet, 2013

机译：子宫CFTR基因治疗改善先天性膈疝相关肺发育不良

CFTR gene therapy, a method to rescue lung hypoplasia in congenital diaphragmatic hernia?

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