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  • Title: FVC deterioration, airway obstruction determination, and life span in Ataxia telangiectasia.
    Author: Vilozni D, Lavie M, Sarouk I, Bar-Aluma BE, Dagan A, Ashkenazi M, Ofek M, Efrati O.
    Journal: Respir Med; 2015 Jul; 109(7):890-6. PubMed ID: 26033643.
    Abstract:
    RATIONALE: Forced vital capacity (FVC) values decrease with progress of the disease in Ataxia telangiectasia (AT). OBJECTIVE: To study the effect of this process on airway obstruction determination and life span in AT. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
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