FVC deterioration, airway obstruction determination, and life span in Ataxia telangiectasia. Issue 7 (July 2015)

• Record Type: Journal Article
• Authors: Vilozni, Daphna; Lavie, Moran; Sarouk, Ifat; Bar-Aluma, Bat-El; Dagan, Adi; Ashkenazi, Moshe; Ofek, Miryam; Efrati, Ori
• Is Part Of: Respiratory medicine. Volume 109:Issue 7(2015)
• Page Start: 890
• Page End: 896
• Publication Date: 2015-07
• Subjects: Chest -- Diseases -- Periodicals; Chest -- Diseases -- Great Britain -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory Tract Diseases -- Periodicals; Appareil respiratoire -- Maladies -- Périodiques; Thorax -- Maladies -- Périodiques; Appareil respiratoire -- Maladies -- Traitement -- Périodiques; Electronic journals; 616.2
• DOI: 10.1016/j.rmed.2015.05.013
• Languages: English
• ISSNs: 0954-6111

Abstract

<abstract xml:lang="en" abstract-type="author" id="abs0010"> <title id="sectitle0010">Summary</title> <sec> <title id="sectitle0015">Rationale</title> <p id="abspara0010">Forced vital capacity (FVC) values decrease with progress of the disease in Ataxia telangiectasia (AT).</p> </sec> <sec> <title id="sectitle0020">Objective</title> <p id="abspara0015">To study the effect of this process on airway obstruction determination and life span in AT.</p> </sec> <sec> <title id="sectitle0025">Methods</title> <p id="abspara0020">Clinical data and yearly best spirometry maneuvers were collected retrospectively from 37 AT patients (196 spirometry tests) during 5.4 ± 1.8yrs (initial age 4–21 y). Twelve patients were walking (7 of them had recurrent respiratory system infections); 25 subjects were confined to wheelchair, of them 8 patients were towards end-stage lung disease. Spirometry indices included Forced Vital Capacity (FVC), mid-expiratory-flow (FEF25-75), and tidal volume (VT). We calculated FEF25-75/FVC and VT/FVC ratios.</p> </sec> <sec> <title id="sectitle0030">Results</title> <p id="abspara0025">FVC declined from 67 ± 8 while walking to 19 ± 6 %predicted values. FEF25-75 values that were elevated (116 ± 23 %predicted) while walking, decreased to 69 ± 27 %predicted at end-stage where 7 patients responded to bronchodilators. VT/FVC ratio was 0.25 ± 0.06 while walking, increased to 0.35 once on wheelchairs, and further increased to 0.57 ± 0.19 at end-stage disease. FEF25–75/FVC ratio was 2.54 ± 0.70 above normal (∼1.0) increasing to 4.16 ± 0.75 at end stage. A sharp elevation was seen in FEF25–75/FVC ratio when FEV1 was still ∼45 %predicted and 2-years prior to death.</p> </sec> <sec> <title id="sectitle0035">Conclusions</title> <p id="abspara0030">Having a low baseline-FVC (60% predicted) artificially raises FEF25-75 values, so FEF25-75 of "mild obstruction" values may indicate severe airway obstruction in AT subjects. VT/FVC and FEF25-75/FVC ratios may therefore assist in revealing higher than normal breathing effort. The results further suggest adding VT/FVC and FEF25-75/FVC ratios to pulmonary function assessments in patients with AT.</p> </sec> </abstract>