Follow up of Congenital Lung Anomalies (CLA) With Antenatal Diagnosis - a Swiss Multicentric Database
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 100
- 试验地点
- 1
- 主要终点
- Change in clinical of patients with CLA between different time point
研究概览
简要总结
Congenital lung anomalies include different pathologies such as congenital cystic adenomatoid malformation, pulmonary sequestration, bronchial atresia, emphysema, bronchogenic cyst. They concern less than 1/10000 births and their physiopathological origin is still poorly understood. The main goal of this project is to pool the cases from different swiss centers on a prospective cohort study, first to increase knowledge of clinical and radiological evolution and their correlation with histological data, and second to analyse the pathological embryological mechanism underlying these malformations.
详细描述
- To register prospectively clinical, biological, radiological and histological datas in a multicentric database (secured internet link, via Secutrial® software). for children with prenatal diagnosis of this kind of malformation. This study is conducted by a multidisciplinary team, involving obstetricians, neonatologists, pneumologists, pediatric surgeons, radiologists and anatomopathologists.
- To create a tissue biobank
Outcomes:
- Contribute to the definition of a standardized procedure at the Swiss level for the treatment of patients suffering from these malformations,
- Improve the assessment of lesion evolution related to CLA, and
- Possibly validate some biomarkers, which could help to identify individuals at risk. On the long term, these results could also support the development of innovative therapies targeting the factors involved in lung development.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- — 至 16 Years（Child）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •All patients diagnosed with congenital lung anomalies
排除标准
- 未提供
结局指标
主要结局
Change in clinical of patients with CLA between different time point
时间窗: Birth, 1 month of life, 4 months of life, 9-18 months of life, 6 months post surgery, 1 year post surgery, 7 years old, 10 years old, 12 years old
Clinical measurements: size (cm), weight (kg), saturation (%)
Analysis of CLA physiopathology
时间窗: samples collected during surgery
Analysis of growth factors, transcription factors and extracellular components implicated in CLA genesis by immunohistochemistry, transcriptomic and proteomic methods
Change in lung function
时间窗: 7 years old,10 years old, 12 years old, 16 years old
FEV1 (l/min), FEV1/FVC (%)TLC (L) DLCO (ml/min/mmHg)
Change in Scar aspect and thoracic deformation of patients with CLA between different time point
时间窗: 6 months post surgery, 1 year post surgery, 7 years old, 10 years old, 12 years old
Description
Change in lung radiological images of patients with CLA between different time point
时间窗: 1 month of life, 4 months of life, 9-18 months of life, 6 months post surgery, 1 year post surgery, 7 years old, 10 years old, 12 years old
Chest X-ray and Thoracic CT Scan lesion description
Change in lesion size described by antenatal ultrasound of patients with CLA between different time point
时间窗: 22, 28, 32 and 37 weeks of gestation
lesion size (mm) CVR, estimated wight (gr), Head circumferences (cm)
Change in lesion description by antenatal ultrasound of patients with CLA between different time point
时间窗: 22, 28, 32 and 37 weeks of gestation
Lesion description (micro cysts, macrocysts),lesion localisation
次要结局
- Report biomarkers implicated in CLA with a potential role in lesion oncogenic transformation(1 year post surgery)
研究者
Isabelle Ruchonnet-Métrailler
Principal Investigator
University Hospital, Geneva