We performed a prospective, observational, cohort study of children newly diagnosed with children’s interstitial lung disease (ChILD), with structured follow-up at 4, 8, 12 weeks and 6 and 12 months. 127 children, median age 0.9 (IQR 0.3–7.9) years had dyspnoea (68%, 69/102), tachypnoea (75%, 77/103) and low oxygen saturation (SpO2) median 92% (IQR 88–96). Death (n=20, 16%) was the most common in those <6 months of age with SpO2<94% and developmental/surfactant disorders. We report for the first time that ChILD survivors improved multiple clinical parameters within 8–12 weeks of diagnosis. These data can inform family discussions and support clinical trial measurements.

中文翻译：

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儿童罕见弥漫性肺实质疾病 (ChILD) 多中心队列研究的一年结果

我们对新诊断为儿童间质性肺病 (ChILD) 的儿童进行了一项前瞻性、观察性队列研究，并在 4、8、12 周和 6 个月和 12 个月时进行了结构化随访。127 名儿童，中位年龄 0.9 (IQR 0.3-7.9) 岁有呼吸困难 (68%, 69/102)、呼吸急促 (75%, 77/103) 和低氧饱和度 (SpO2) 中位数 92% (IQR 88-96)。死亡 (n=20, 16%) 在 SpO2 <94% 和发育/表面活性剂障碍的 <6 个月大的儿童中最常见。我们首次报告儿童幸存者在诊断后 8-12 周内改善了多项临床参数。这些数据可以为家庭讨论提供信息并支持临床试验测量。