Congenital Lung Anomalies (CLA) Swiss Database

NCT03044769 | Recruiting

• 1 other identifier: CLA-2016
• Study Type: observational
• Target: 100
• Locations: 1 country
• Sites: 1

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.

Conditions

Congenital Lung Malformation; Congenital Lung Cyst; Sequestrum; Bronchogenic Cancer; Pleuropulmonary Blastoma; Acinar Dysplasia

Keywords

Congenital Lung Anomalies

Outcome Measures

Primary Outcomes (7)

• 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 Outcomes (1)

• Report biomarkers implicated in CLA with a potential role in lesion oncogenic transformation

  1 year post surgery

Study Arms (2)

Patient with CLA with surgery

Procedure: Malformations resection

Patient with CLA without surgery

Interventions

Malformations resection PROCEDURE

Resection of the malformation lung sections with a healthy adjacent part

Patient with CLA with surgery

Eligibility Criteria

• Age: Up to 16 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)
• 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.

You may qualify if:

• All patients diagnosed with congenital lung anomalies

You may not qualify if:

• None

Sponsors & Collaborators

• University Hospital, Geneva: lead

Study Sites (1)

Geneva University Hospital

Geneva, 1205, Switzerland

RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

To create a biobank with the different samples of patients undergoing surgery, healthy edge of resections being considered as control tissue. Leukocyte DNA will be compared with lung tissue DNA.

MeSH Terms

Conditions

Cystic Adenomatoid Malformation of Lung, Congenital; Pleuropulmonary blastoma

Condition Hierarchy (Ancestors)

Lung Diseases; Respiratory Tract Diseases; Respiratory System Abnormalities; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Isabelle Andrieu Vidal, MD PhD

  University Hospital, Geneva

  PRINCIPAL INVESTIGATOR

• Isabelle Ruchonnet-Métrailler, MD PhD

  University Hospital, Geneva

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Isabelle Ruchonnet-Métrailler, MD PhD

CONTACT

isabelle.ruchonnet-metrailler@hcuge.ch

Isabelle Andrieu Vidal, MD PhD

CONTACT

isabelle.andrieuvidal@hcuge.ch

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 10 Years
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: January 31, 2017
• First Posted: February 7, 2017
• Study Start: April 1, 2016
• Primary Completion: June 1, 2022
• Study Completion: April 1, 2026
• Last Updated: April 16, 2020

Record last verified: 2020-04

Locations

CH (1)