在患有马凡综合征的儿童中自发性肺胸部治疗的积极方法?
Angelo Zarfati1, Simone Frediani1, Valerio Pardi1
1General and Thoracic Pediatric Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Frontiers in pediatrics
|January 4, 2024
概括
患有马方综合征 (MS) 的儿童面临自发性肺胸部 (SP) 并发症的风险更高. 由于复发和治疗失败率的增加,建议在MS患者中对SP进行积极的初始治疗.
科学领域:
- 儿科肺病学 儿科肺病学
- 遗传学 遗传学 是一个
- 胸部外科手术 胸部外科手术
背景情况:
- 马方综合征 (MS) 是一种全身结合组织疾病.
- 患有多发性硬化症的患者具有自发性肺胸部 (SP) 的风险较高.
- 有限的儿科文献和指导方针存在,用于在患有MS的儿童中管理SP.
研究的目的:
- 分析儿科患者自发性肺胸部 (SP) 的管理.
- 为了比较Marfan综合征 (MS) 和非综合征儿童之间的SP管理结果.
- 确定挑战,并为儿童多发性硬化症中多发性硬化症的治疗策略提供信息.
主要方法:
- 对被诊断患有SP的儿科患者 (18岁以下) 的回顾性分析.
- 包括诊断,治疗和随访 (FU) 数据.
- 多发性硬化症患者与非综合征患者的对照组之间的比较.
主要成果:
- 在66名SP患者中,有9人 (13%) 患有MS.
- 马方综合征患者的第一线治疗失败率显著增加,需要额外的手术.
- 马凡综合征患者的逆侧SP发生率和逆侧SP复发率较高,在随访期间需要手术或胸部排水.
结论:
- 儿科马方综合征中的自发性肺胸部管理因治疗失败,复发和副作用而复杂化.
- 对于被诊断为马方综合征的儿科患者,应考虑采取更积极的第一线管理方法.
- 鉴于此种人群中与SP相关的风险增加,主动管理至关重要.
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