Department of Pediatric Pulmonology and Reference Center for Rare Lung Diseases, RespiRare, Armand Trousseau Hospital, APHP, Sorbonne University, Paris, France.
Department of Pediatric and Reference Center for Rare Lung Diseases, RespiRare, Centre Hospitalier Intercommunal de Créteil, Créteil, France.
Pediatr Pulmonol. 2024 Feb;59(2):417-425. doi: 10.1002/ppul.26765. Epub 2023 Nov 22.
Childhood Interstitial Lung Disease (chILD) represents a rare and severe group of diseases for which the etiologic workup, classification, and management remain a challenge for most pediatric pulmonologists. In France in 2018, the RespiRare network established the first multidisciplinary team meetings (MDTm) dedicated to chILD. This study aims to investigate the impact of MDTm in chILD diagnosis and management as well as user satisfaction.
The MDTm took place on a monthly basis through video conferences. The participants consisted of a quorum and included pediatric pulmonologists, radiologists, geneticists, and pulmonologists, with an average of 10.5 participants per meeting. Patients provided consent to participate in MDTm and for data collection. Data were retrospectively extracted from MDTm reports. To evaluate the usefulness of the MDTm and the satisfaction of the participants, a survey was sent by email at least 3 months after the MDTm to the participants.
A total of 216 chILD cases were discussed during 56 MDTm sessions. The median age of onset was 0.5 years (interquartile range 0-7). The MDTm sessions resulted in the correction of chILD etiology in 25% of cases (neuroendocrine cell hyperplasia of infancy 17%, surfactant metabolism disorder 8%, pulmonary alveolar proteinosis 4%, hemosiderosis 3%, sarcoidosis 3%, and others 34%), and chILD was ruled out in 7% of cases. A change in therapy was proposed for 46% of cases. User satisfaction was significant, particularly regarding their confidence in managing these rare diseases.
Dedicated MDTm sessions offer a unique opportunity to enhance chILD etiologic diagnosis and management, leading to increased physician knowledge and confidence in managing these patients.
儿童间质性肺病（chILD）代表了一组罕见且严重的疾病，大多数儿科肺病专家在病因学检查、分类和管理方面仍然面临挑战。2018 年，法国的 RespiRare 网络成立了首个多学科团队会议（MDTm），专门针对 chILD。本研究旨在调查 MDTm 对 chILD 诊断和管理的影响以及用户满意度。
MDTm 通过视频会议每月举行一次。参与者包括法定人数，包括儿科肺病专家、放射科医生、遗传学家和肺病专家，每次会议平均有 10.5 名参与者。患者同意参加 MDTm 并提供数据收集。数据从 MDTm 报告中回顾性提取。为了评估 MDTm 的有用性和参与者的满意度，在 MDTm 后至少 3 个月，向参与者发送电子邮件进行调查。
在 56 次 MDTm 会议中讨论了 216 例 chILD 病例。发病中位年龄为 0.5 岁（四分位距 0-7）。MDTm 会议使 25%的 chILD 病因得到纠正（婴儿期神经内分泌细胞增生 17%，表面活性剂代谢障碍 8%，肺泡蛋白沉积症 4%，血色素沉着症 3%，结节病 3%，其他 34%），7%的病例排除了 chILD。46%的病例提出了治疗方案的改变。用户满意度显著，尤其是在管理这些罕见疾病的信心方面。
专门的 MDTm 会议为提高 chILD 的病因诊断和管理提供了独特的机会，从而提高了医生管理这些患者的知识和信心。
