PT  - JOURNAL ARTICLE
AU  - Vilozni, Daphna
AU  - Lavie, Moran
AU  - Sarouk, Ifat
AU  - Levi, Yonit
AU  - Efrati, Ori
TI  - Longitudinal follow-up of lung function in ataxia telangiectasia
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - P1087
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/P1087.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/P1087.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - Rationale: Individuals with Ataxia Telangiectasia (AT) are vulnerable to the development of significant pulmonary complications with age. This condition calls for a follow up on lung function evaluation.Objectives: To follow the individual patient's lung functions during 3-5 yearsMethods: Yearly best spirometry data were collected from 39 AT patients (age 10.4±5.3years; 13 patients younger than 7 years). The yearly decline in spirometry indices were defined in relation to the preceding year, initial age, and airway hyper-reactivity (a positive response to β2-agonist).Results: Young children showed low FVC (%predicted) between 43.1-70.7%. Yearly decline in spirometry was -3.4±9.4%, -2.7±7.6%; -5.9±10.4; for FVC, FEV1 and in PEF respectively. FEF25-75 increased by 1.6±12.1%/year. Patients having airway hyper-reactivity (n=21) showed initially worse flows (%predicted) than others in FEV0.5 (41.6±18.5% vs. 57.7±15.7%, p<0.0055) and in FEF25-75=83.3±19.2% vs. 106.3±20.8%; p<0.0006).Conclusions: Young A-T patients showed a restricted FVC pattern that deteriorated with age. Patients having hyperactive airways showed initially restrictive/obstructive worse lung function than others. Airway to volume ratio increases with age implying hypoventilation. The findings call for a restricted follow up on lung function in A-T patients.