Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Kolonics-Farkas, AM; Agg, B; Benke, K; Odler, B; Bohacs, A; Kovats, Z; Szabolcs, Z; Müller, V, ;RITERM, study, group;RITERM, study, group.
Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery.
Lung. 2019; 197(4):465-472
Doi: 10.1007/s00408-019-00236-1
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- Odler Balazs
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- Abstract:
- INTRODUCTION: 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. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adult - administration & dosage
- Airway Obstruction - diagnosis, etiology, physiopathology
- Female - administration & dosage
- Forced Expiratory Volume - administration & dosage
- Humans - administration & dosage
- Lung - physiopathology
- Male - administration & dosage
- Marfan Syndrome - complications, diagnosis
- Middle Aged - administration & dosage
- Plethysmography - administration & dosage
- Pulmonary Diffusing Capacity - administration & dosage
- Quality of Life - administration & dosage
- Scoliosis - complications, physiopathology
- Spirometry - administration & dosage
- Thoracic Surgical Procedures - adverse effects
- Vital Capacity - administration & dosage
- Young Adult - administration & dosage
- Find related publications in this database (Keywords)
- Marfan syndrome
- Musculoskeletal disorder
- Lung function testing
- Airway obstruction
- Thoracic surgery