A Swiss database and biobank to better understand and manage congenital lung anomalies.

Isabelle Vidal,Constance Barazzone-Argiroffo,Barabara E Wildhaber,Daniel Trachsel,Ueli Moehrlen,Isabelle Ruchonnet-Mtrailler,Juerg Barben,Nicolas Regamey,Dietmar Cholewa

doi:10.4414/smw.2019.20081

Isabelle Vidal, Constance Barazzone-Argiroffo + Show 7 more

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https://doi.org/10.4414/smw.2019.20081

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Congenital lung anomalies are a group of rare malformations, often diagnosed during the prenatal period. Guidelines on how to manage these patients are currently under debate, especially with regard to prophylactic surgery in asymptomatic patients, or how to proceed with conservative follow-up. Currently, there is no clear consensus on management strategies. A Swiss congenital lung anomaly national database and biobank was created in 2016 to enable data recording and collection of surgical lung samples in order to help define the most appropriate management strategies. This national observational cohort study represents an important step towards a better understanding of the pathophysiology and clinical course of the diseases included under congenital lung anomalies, especially in the context of a small country like Switzerland.

Highlights

Congenital lung anomalies are a group of rare malformations, often diagnosed during the prenatal period
A Swiss congenital lung anomaly national database and biobank was created in 2016 to enable data recording and collection of surgical lung samples in order to help define the most appropriate management strategies. This national observational cohort study represents an important step towards a better understanding of the pathophysiology and clinical course of the diseases included under congenital lung anomalies, especially in the context of a small country like Switzerland
Pooling data from patients with a rare disease, such as congenital lung anomaly, in a longterm observational cohort study represents an important step to a better understanding of the natural clinical evolution of these patients

Summary

Congenital lung anomalies are a group of rare malformations, often diagnosed during the prenatal period. A Swiss congenital lung anomaly national database and biobank was created in 2016 to enable data recording and collection of surgical lung samples in order to help define the most appropriate management strategies. Reported complications are not more frequent during the first years of life regardless of which clinical, nonoperative follow-up is chosen [17] These still unanswered questions as to which is the best course of treatment to follow for congenital lung anomaly patients led to the creation of a Swiss national database and an associated biobank in 2016, with the aim of collecting surgically resected abnormal lung specimens. In France, several studies on congenital lung anomalies have been performed using the French database created in 2008 (Respirare) and dedicated to rare pulmonary diseases One of these studies accurately reported the various complications at birth and the need for delivery to occur in a tertiary care centre [3]. Information on the number of patients included in a register is rarely available and only a few websites, such as Clinicaltrial.gov, provide such details

A Swiss congenital lung anomaly database for improved patient management

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