Clinical Trial: Congenital Lung Anomalies (CLA) Swiss Database

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational [Patient Registry]

Official Title: Follow up of Congenital Lung Anomalies (CLA) With Antenatal Diagnosis - a Swiss Multicentric Database

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 Summary:

  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.

Sponsor: University Hospital, Geneva

Current Primary Outcome:

  • Change in clinical of patients with CLA between different time point [ Time Frame: 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 [ Time Frame: 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 [ Time Frame: 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 [ Time Frame: 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 [ Time Frame: 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 [ Time Frame: 22, 28, 32 and 37 weeks of gestation ]
    Same as current

    Current Secondary Outcome: Report biomarkers implicated in CLA with a potential role in lesion oncogenic transformation [ Time Frame: 1 year post surgery ]

    Comparison using exome analysis : CLA lesion, healthy adjacent part and blood DNA


    Original Secondary Outcome: Same as current

    Information By: University Hospital, Geneva

    Dates:
    Date Received: January 31, 2017
    Date Started: April 1, 2016
    Date Completion: April 1, 2026
    Last Updated: February 2, 2017
    Last Verified: February 2017