Etiological Work-Up for Adults with Bronchiectasis: A Predictive Diagnostic Score for Primary Ciliary Dyskinesia and Cystic Fibrosis.

bronchiectasis cystic fibrosis primary ciliary dyskinesia

Journal

Journal of clinical medicine
ISSN: 2077-0383
Titre abrégé: J Clin Med
Pays: Switzerland
ID NLM: 101606588

Informations de publication

Date de publication:
06 Aug 2021
Historique:
received: 09 07 2021
revised: 30 07 2021
accepted: 02 08 2021
entrez: 27 8 2021
pubmed: 28 8 2021
medline: 28 8 2021
Statut: epublish

Résumé

etiological investigations are not done for all adult patients with bronchiectasis because of the availability and interpretation of tests. The aim of the study was to elaborate a score to identify patients at high risk of having cystic fibrosis or primary ciliary dyskinesia (CF/PCD), which require appropriate management. diagnostic work-ups were carried out on a French monocenter cohort, and results were subjected to logistic-regression analyses to identify the independent factors associated with CF/PCD diagnosis and, thereby, elaborate a score to validate in a second cohort. among 188 patients, 158 had no obvious diagnosis and were enrolled in the algorithm-construction group. In multivariate analyses, age at symptom onset (8.69 (2.10-35.99); a clinical score may help identify adult patients with bronchiectasis at higher risk of having CF or PCD.

Sections du résumé

BACKGROUND BACKGROUND
etiological investigations are not done for all adult patients with bronchiectasis because of the availability and interpretation of tests. The aim of the study was to elaborate a score to identify patients at high risk of having cystic fibrosis or primary ciliary dyskinesia (CF/PCD), which require appropriate management.
METHODS METHODS
diagnostic work-ups were carried out on a French monocenter cohort, and results were subjected to logistic-regression analyses to identify the independent factors associated with CF/PCD diagnosis and, thereby, elaborate a score to validate in a second cohort.
RESULTS RESULTS
among 188 patients, 158 had no obvious diagnosis and were enrolled in the algorithm-construction group. In multivariate analyses, age at symptom onset (8.69 (2.10-35.99);
CONCLUSIONS CONCLUSIONS
a clinical score may help identify adult patients with bronchiectasis at higher risk of having CF or PCD.

Identifiants

pubmed: 34441774
pii: jcm10163478
doi: 10.3390/jcm10163478
pmc: PMC8397090
pii:
doi:

Types de publication

Journal Article

Langues

eng

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Auteurs

Frederic Schlemmer (F)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.
Unité de Pneumologie, AP-HP, Hôpitaux Universitaires Henri Mondor, FHU SENEC, F-94010 Créteil, France.
Université Paris Est Créteil, INSERM, IMRB U955, F-94010 Créteil, France.

Agnes Hamzaoui (A)

Unité de Recherche (UR/12SP15), Départment des Maladies Respiratoires, Division de Pneumologie, Hôpital Abderrahman Mami, Ariana 2080, Tunisia.

Sonia Zebachi (S)

Département de Santé Publique, AP-HP, Hôpitaux Universitaires Henri Mondor, FHU SENEC, F-94010 Créteil, France.

Aurelie Le Thuaut (A)

Département de Santé Publique, AP-HP, Hôpitaux Universitaires Henri Mondor, FHU SENEC, F-94010 Créteil, France.

Gilles Mangiapan (G)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.

Isabelle Monnet (I)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.

Amel Boudjema (A)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.

Laurence Jabot (L)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.

Bruno Housset (B)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.

Sylvie Bastuji-Garin (S)

Département de Santé Publique, AP-HP, Hôpitaux Universitaires Henri Mondor, FHU SENEC, F-94010 Créteil, France.

Laurence Bassinet (L)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.

Bernard Maitre (B)

Service de Pneumologie, Centre Hospitalier Intercommunal, FHU SENEC, F-94000 Créteil, France.
Unité de Pneumologie, AP-HP, Hôpitaux Universitaires Henri Mondor, FHU SENEC, F-94010 Créteil, France.

Classifications MeSH