Congenital Lung Anomalies (CLA) Swiss Database

Pleuropulmonary Blastoma Clinical Trial

— CLADatabase  

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT03044769
• Other study ID #: CLA-2016
• Status: Recruiting
• Start date: April 1, 2016
• Est. completion date: April 1, 2026

Study information

• Verified date: April 2020
• Source: University Hospital, Geneva
• Contact: Isabelle Ruchonnet-Métrailler, MD PhD
• Email: isabelle.ruchonnet-metrailler[@]hcuge.ch
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 100
• Est. completion date: April 1, 2026
• Est. primary completion date: June 1, 2022
• Accepts healthy volunteers: No
• Gender: All
• Age group: N/A to 16 Years

Eligibility

Inclusion Criteria:

• All patients diagnosed with congenital lung anomalies

Exclusion Criteria:

• None

Related Conditions & MeSH terms

• Acinar Dysplasia
• Bronchogenic Cancer
• Congenital Abnormalities
• Congenital Lung Cyst
• Congenital Lung Malformation
• Pleuropulmonary Blastoma
• Sequestrum

Intervention

Procedure:
• Malformations resection
Resection of the malformation lung sections with a healthy adjacent part

Locations

Country	Name	City	State
Switzerland	Geneva University Hospital	Geneva

Sponsors (1)

Lead Sponsor	Collaborator
University Hospital, Geneva

Country where clinical trial is conducted

Switzerland, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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
Primary	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
Primary	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
Primary	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
Primary	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
Primary	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
Primary	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	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