Congenital Lung Anomalies (CLA) Swiss Database (CLADatabase)

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

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.

Study Overview

• Status: Recruiting
• Conditions: Pleuropulmonary Blastoma; Bronchogenic Cancer; Congenital Lung Malformation; Congenital Lung Cyst; Sequestrum; Acinar Dysplasia
• Intervention / Treatment: Procedure: Malformations resection

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 Type: Observational
• Enrollment (Anticipated): 100

Contacts and Locations

• Study Contact: Name: Isabelle Andrieu Vidal, MD PhD; Email: isabelle.andrieuvidal@hcuge.ch

Study Locations

• Switzerland
  • Geneva, Switzerland, 1205
    • Recruiting
    • Geneva University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: No older than 16 years (CHILD)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Recruitment for this study is performed through a discussion with parents of the child during prenatal and postnatal medical consultations.

Blood samples will be collected during surgery.

Description

Inclusion Criteria:

• All patients diagnosed with congenital lung anomalies

Exclusion Criteria:

• None

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Patient with CLA with surgery	Procedure: Malformations resection; Resection of the malformation lung sections with a healthy adjacent part
Patient with CLA without surgery

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change in clinical of patients with CLA between different time point	Clinical measurements: size (cm), weight (kg), saturation (%)	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
Analysis of CLA physiopathology	Analysis of growth factors, transcription factors and extracellular components implicated in CLA genesis by immunohistochemistry, transcriptomic and proteomic methods	samples collected during surgery
Change in lung function	FEV1 (l/min), FEV1/FVC (%)TLC (L) DLCO (ml/min/mmHg)	7 years old,10 years old, 12 years old, 16 years old
Change in Scar aspect and thoracic deformation of patients with CLA between different time point	Description	6 months post surgery, 1 year post surgery, 7 years old, 10 years old, 12 years old
Change in lung radiological images of patients with CLA between different time point	Chest X-ray and Thoracic CT Scan lesion description	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
Change in lesion size described by antenatal ultrasound of patients with CLA between different time point	lesion size (mm) CVR, estimated wight (gr), Head circumferences (cm)	22, 28, 32 and 37 weeks of gestation
Change in lesion description by antenatal ultrasound of patients with CLA between different time point	Lesion description (micro cysts, macrocysts),lesion localisation	22, 28, 32 and 37 weeks of gestation

Secondary Outcome Measures

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

Collaborators and Investigators

• Sponsor: University Hospital, Geneva
• Investigators: Principal Investigator: Isabelle Andrieu Vidal, MD PhD, University Hospital, Geneva; Principal Investigator: Isabelle Ruchonnet-Métrailler, MD PhD, University Hospital, Geneva

Study record dates

Study Major Dates

• Study Start (ACTUAL): April 1, 2016
• Primary Completion (ANTICIPATED): June 1, 2022
• Study Completion (ANTICIPATED): April 1, 2026

Study Registration Dates

• First Submitted: January 31, 2017
• First Submitted That Met QC Criteria: February 2, 2017
• First Posted (ESTIMATE): February 7, 2017

Study Record Updates

• Last Update Posted (ACTUAL): April 16, 2020
• Last Update Submitted That Met QC Criteria: April 15, 2020
• Last Verified: April 1, 2020

More Information

Terms related to this study

• Keywords: Congenital Lung Anomalies
• Additional Relevant MeSH Terms: Neoplasms by Histologic Type; Neoplasms; Neoplasms by Site; Respiratory Tract Neoplasms; Thoracic Neoplasms; Lung Neoplasms; Neoplasms, Complex and Mixed; Congenital Abnormalities; Pulmonary Blastoma
• Other Study ID Numbers: CLA-2016

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No