窒息性胸廓发育不良手术治疗的现状及展望
【摘要】 窒息性胸廓发育不良(ATD)是一种罕见的、死亡率高的常染色体隐性遗传病,可分为致死型、重度型、轻度型和潜伏型,其发病年龄与疾病的严重程度相关。患者因胸廓狭窄导致限制性通气功能障碍,反复肺炎,易引起呼吸窘迫和呼吸衰竭。疾病同时也可累及肾脏、肝脏、眼、胃肠道、心脏等器官。目前手术是唯一有效的治疗方法。手术主要分三大类:第一类正中胸骨切开术,第二类侧胸壁扩张开胸术,第三类切除或抬高肋骨扩胸术。现就窒息性胸廓发育不良手术方法展开综述,对这些方法进行比较分析,为患者提供个体化手术思路。【关键词】 窒息性胸廓发育不良 儿童 手术治疗
Current Status and Future Perspectives in the Surgical Treatment of Asphyxiating Thoracic Dysplasia/LI Na, WANG Mingzhi, LIU Yang, LI Xuejun. //Medical Innovation of China, 2025, 22(09): -183
[Abstract] Asphyxiating thoracic dysplasia (ATD) is a rare and highly lethal autosomal recessive disorder, which may be categorized as lethal, severe, mild and latent, with the age of onset correlating with the severity of the disease. Patients are prone to respiratory distress and respiratory failure due to restrictive ventilatory dysfunction caused by thoracic stenosis and recurrent pneumonia. The disease can also involve the kidneys, liver, eyes, gastrointestinal tract, heart and other organs. Surgery is the only effective treatment method at present. There are three main types of surgery: the first is median sternotomy ......
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