Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery.
Adult
Airway Obstruction
/ diagnosis
Female
Forced Expiratory Volume
Humans
Lung
/ physiopathology
Male
Marfan Syndrome
/ complications
Middle Aged
Plethysmography
Pulmonary Diffusing Capacity
Quality of Life
Scoliosis
/ complications
Spirometry
Thoracic Surgical Procedures
/ adverse effects
Vital Capacity
Young Adult
Airway obstruction
Lung function testing
Marfan syndrome
Musculoskeletal disorder
Thoracic surgery
Journal
Lung
ISSN: 1432-1750
Titre abrégé: Lung
Pays: United States
ID NLM: 7701875
Informations de publication
Date de publication:
08 2019
08 2019
Historique:
received:
24
01
2019
accepted:
02
05
2019
pubmed:
16
5
2019
medline:
9
4
2020
entrez:
16
5
2019
Statut:
ppublish
Résumé
Marfan syndrome is a genetic disorder affecting the connective tissue. Changes in lung tissue might influence respiratory function; however, a detailed respiratory functional assessment according to the need for major thoracic surgery is missing. Comprehensive pulmonary examinations were performed in 55 Marfan patients including respiratory symptoms, lung function (LF) testing using European Coal and Steel Community (ECSC) reference values, TL Respiratory symptoms affected 20% of patients. Scoliosis was significantly more frequent in the Mf Marfan syndrome is associated with airway obstruction, especially in patients who have undergone major thoracic surgery, indicative of more severe connective tissue malfunction. The use of arm span for height correction is suitable to evaluate LF changes in this special patient group including patients with significant scoliosis.
Identifiants
pubmed: 31089858
doi: 10.1007/s00408-019-00236-1
pii: 10.1007/s00408-019-00236-1
pmc: PMC6647216
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
465-472Références
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