土耳其儿童间歇性肺病:国家登记处的首批数据
Halime Nayır-Büyükşahin1,2, Nagehan Emiralioğlu3, Ayşe Ayzıt Kılınç4
1Division of Pulmonology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey. hnayirbuyuksahin@gmail.com.
European journal of pediatrics
|October 24, 2023
概括
土耳其儿童间歇性肺病 (chILD) 登记处的首批结果揭示了婴儿发病和所有年龄段的扩散性对细胞性肺病之间的表现和治疗的关键差异. 研究结果强调需要考虑营养,特别是在患有CHILD的婴儿中.
科学领域:
- 儿科肺病学 儿科肺病学
- 罕见疾病 罕见疾病
- 医疗登记簿 医疗登记簿
背景情况:
- 儿童间歇性肺病 (chILD) 是一组异质的罕见疾病,带来诊断和管理的挑战.
- 建立国家注册表对于了解chILD的流行病学和临床特征至关重要.
- 儿童火注册表 (chILD-TR) 是为了提高认识,诊断和治疗标准而创建的.
研究的目的:
- 介绍chILD-TR的初步发现,这是一项前性队列研究.
- 分析土耳其登记的CHILD患者的人口,临床,放射和治疗特征.
- 为了比较患有chILD的婴儿和所有年龄段被诊断的婴儿之间的临床特征.
主要方法:
- 使用数据输入软件系统进行前性队列研究.
- 包括来自19个中心的416名患者,在排除后分析了370人.
- 两组之间临床特征的比较:第一组 (婴儿期发病的DPLD) 和第二组 (所有年龄段的DPLD).
主要成果:
- 诊断时的中位数年龄为6.05岁. 第一组包括21.8%的患者,第二组包括78.1%的患者.
- 第1组在CT扫描上显示了明显较低的重量z分数和更频繁的地面玻璃不透明度.
- 第2组更频繁地出现结节不透明,支气管切除,马赛克 perfusion,和中间腺淋巴病;口服类固醇的使用在第2组更高.
结论:
- 该chILD-TR提供了关于土耳其chILD的频率,类型和治疗的宝贵见解,识别了诊断和管理方面的挑战.
- 在临床表现和放射学发现中,婴儿发病和晚期发病的CHILD之间存在显著的差异.
- 营养状况是所有CHILD患者的关键考虑因素,特别是那些患有婴儿发病的患者.
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