Congenital Lung Anomalies (CLA) Swiss Database

Recruiting

• Conditions: Congenital Lung Malformation; Sequestrum; Bronchogenic Cancer; Congenital Lung Cyst; Acinar Dysplasia; Pleuropulmonary Blastoma
• Interventions: Procedure: Malformations resection

• Registration Number: NCT03044769
• Lead Sponsor: University Hospital, Geneva

Brief Summary

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.

Detailed Description

1. 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.

2. To create a tissue biobank

Outcomes:

1. Contribute to the definition of a standardized procedure at the Swiss level for the treatment of patients suffering from these malformations,

2. Improve the assessment of lesion evolution related to CLA, and

3. 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.

Study Timeline

• Study Start: 2016-04-01
• Study First Submitted: 2017-01-31
• Study First Submitted QC: 2017-02-02
• Study First Posted: 2017-02-07
• Status Verified: 2020-04
• Last Update Submitted: 2020-04-15
• Last Update Posted: 2020-04-16
• Primary Completion: 2022-06-01
• Study Completion: 2026-04-01

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 100

Inclusion Criteria

• All patients diagnosed with congenital lung anomalies

Exclusion Criteria

• None

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Patient with CLA with surgery	Malformations resection	-

Primary Outcome Measures

Name	Time	Method
Change in clinical of patients with CLA between different time point	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	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	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	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	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	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	22, 28, 32 and 37 weeks of gestation	Lesion description (micro cysts, macrocysts),lesion localisation

Secondary Outcome Measures

Name	Time	Method
Report biomarkers implicated in CLA with a potential role in lesion oncogenic transformation	1 year post surgery	Comparison using exome analysis : CLA lesion, healthy adjacent part and blood DNA

Trial Locations

Locations (1)

Geneva University Hospital; 🇨🇭 Geneva, Switzerland