Bara Zsolt, Gozar Horea, Nagy Nándor, Gurzu Simona, Derzsi Zoltán, Forró Timea, Kovács Evelyn, Jung Ioan
Department of Pediatric Surgery and Orthopedics, George Emil Palade University of Medicine, Pharmacy, Science and Technology of Targu Mures, 540142 Targu Mures, Romania.
Clinic of Pediatric Surgery and Orthopedics, Targu Mures, County Emergency Clinical Hospital, 540136 Targu Mures, Romania.
Int J Mol Sci. 2025 Feb 14;26(4):1639. doi: 10.3390/ijms26041639.
Congenital diaphragmatic hernia (CDH) is a relatively rare and severe developmental disease. Even with the most recent multidisciplinary therapies, the risk for neonatal mortality and morbidity remains high. Recent advancements in prenatal treatments, alongside experimental and clinical data, suggest that fetoscopic endoluminal tracheal occlusion (FETO) promotes lung development and offers a promising strategy against lung hypoplasia and pulmonary hypertension. It is the only existing direct mechanical therapy that intervenes in the regulation of pulmonary pressure. Its influence on lung development also interferes with tissue homeostasis and cell differentiation; it also enhances inflammation and apoptosis. Its physiopathology on cellular and molecular levels is still poorly understood. Unfortunately, the procedure also carries significant pregnancy-, maternal-, and fetus-related risks. Assessing a multifaceted intervention requires a collective view of all aspects. This scoping review uncovers potential materno-fetal procedure-related risks and highlights innovative solutions. Future research on lung development therapies in CDH may focus on the "dual hit" mechanism, combining molecular-targeting drugs and regenerative medicine with the mechanical nature of FETO for synergistic effects.
先天性膈疝(CDH)是一种相对罕见且严重的发育性疾病。即便采用最新的多学科治疗方法,新生儿死亡和发病风险依然很高。产前治疗的最新进展,连同实验和临床数据表明,胎儿镜下腔内气管阻塞术(FETO)可促进肺发育,并为对抗肺发育不全和肺动脉高压提供了一种有前景的策略。它是现有的唯一一种直接干预肺压力调节的机械疗法。其对肺发育的影响也会干扰组织稳态和细胞分化;还会加剧炎症和细胞凋亡。其在细胞和分子水平上的病理生理学仍知之甚少。不幸的是,该手术也存在与妊娠、母体和胎儿相关的重大风险。评估一项多方面的干预措施需要全面考虑所有方面。本综述揭示了与母婴手术相关的潜在风险,并突出了创新解决方案。未来关于CDH肺发育治疗的研究可能集中在“双重打击”机制上,将分子靶向药物和再生医学与FETO的机械特性相结合以产生协同效应。