Congenital Lung Anomalies (CLA) Swiss Database

NCT03044769 — Unknown, 100 participants, University Hospital, Geneva

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

Registry	NCT03044769, acronym CLADatabase
Status	Unknown
Study type	Observational
Enrollment	100 (Estimated)
Sponsor	University Hospital, Geneva (Other)
Conditions	Congenital Lung Malformation, Congenital Lung Cyst, Sequestrum, Bronchogenic Cancer, Pleuropulmonary Blastoma, Acinar Dysplasia
Interventions	Malformations resection (Procedure)
Dates	start 2016-04-01, primary completion 2022-06-01 (estimated), completion 2026-04-01 (estimated), first posted 2017-02-07, last updated 2020-04-16
Locations	1 site in Switzerland

-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.

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+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.

+Arms and interventions

Arm	Type	Interventions	Description
Patient with CLA with surgery		Procedure: Malformations resection
Patient with CLA without surgery

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

+Outcome measures

Primary outcomes

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 outcomes

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

+Eligibility

Sex: All, ages: any to 16 Years

Inclusion Criteria:

* All patients diagnosed with congenital lung anomalies

Exclusion Criteria:

* None

+Locations (1)

Facility	City	Country	Status
Geneva University Hospital	Geneva	Switzerland	Recruiting

+Investigators

Isabelle Andrieu Vidal, MD PhD, Principal investigator, University Hospital, Geneva
Isabelle Ruchonnet-Métrailler, MD PhD, Principal investigator, University Hospital, Geneva