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dc.contributor.author Kolonics-Farkas, Abigél Margit
dc.contributor.author Ágg, Bence
dc.contributor.author Benke, Kálmán
dc.contributor.author Odler, Balázs
dc.contributor.author Bohács, Anikó
dc.contributor.author Kováts, Zsuzsanna Ágnes
dc.contributor.author Szabolcs, Zoltán
dc.contributor.author Müller, Veronika
dc.date.accessioned 2019-10-15T07:20:35Z
dc.date.available 2019-10-15T07:20:35Z
dc.date.issued 2019
dc.identifier 85065962385
dc.identifier.citation journalVolume=197;journalIssueNumber=4;journalTitle=LUNG;pagerange=465-472;journalAbbreviatedTitle=LUNG;
dc.identifier.uri http://repo.lib.semmelweis.hu//handle/123456789/7912
dc.identifier.uri doi:10.1007/s00408-019-00236-1
dc.description.abstract 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, TLCO and quality of life measurements. Groups included patients who did not need surgery (Mf, n = 32) and those who underwent major thoracic surgery (Mfop, n = 23).Respiratory symptoms affected 20% of patients. Scoliosis was significantly more frequent in the Mfop group. LF demonstrated in all Marfan patients a tendency towards airway obstruction (FEV1/FVC = 0.77 ± 0.10), more prominent in Mfop patients (0.74 ± 0.08 vs. Mf: 0.80 ± 0.11; p = 0.03). Correction of LF values using a standing height modification by arm span (Hcorrected) revealed additional changes in FVC and FEV1. TLCO and quality of life did not differ between groups.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.
dc.format.extent 465-472
dc.relation.ispartof urn:issn:0341-2040
dc.title Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery
dc.type Journal Article
dc.date.updated 2019-10-14T08:35:15Z
dc.language.rfc3066 en
dc.rights.holder NULL
dc.identifier.mtmt 30678816
dc.identifier.wos 000476539100009
dc.identifier.pubmed 31089858
dc.contributor.department SE/AOK/K/Pulmonológiai Klinika
dc.contributor.institution Semmelweis Egyetem


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