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Late-breaking abstract: The Effect of lung volume deterioration on tidal volume, airway flows and life span in ataxia telangiectasia

Daphna Vilozni, Moran Lavie, Ifat Sarouk, Bat-El Bar Aluma, Adi Dagan, Yonit Levi, Moshe Askenazi, Miryam Ofek, Ori Efrati
European Respiratory Journal 2014 44: P4518; DOI:
Daphna Vilozni
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Moran Lavie
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Ifat Sarouk
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Bat-El Bar Aluma
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Adi Dagan
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Yonit Levi
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Moshe Askenazi
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Miryam Ofek
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Ori Efrati
1Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Center, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Ramat-Gan, Israel
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Abstract

Introduction: Forced Vital Capacity (FVC) gradually decrease in Ataxia telangiectasia (A-T).

Aim: to determine the effect of FVC decline on life span in A-T.

Methods: Clinical data and yearly best spirometry maneuvers (195 visits) were collected retrospectively during 6yrs, from 37 A-T patients (age 4-21y) attending the A-T national clinic in Israel. 11 patients were walking (ambulated); 26 were confined to wheelchair (Wch) from whom 7 patients died. Spirometry included tidal-volume (TV), FVC and mid-flows (FEF25-75) measurements. Findings were related to recurrent respiratory infections (rRI)

Results: Ambulant patients initial and last FVC were 67+8 and 51+17 %predicted (%P) with a TV/FVC ratio of 23+4% and 26+7% respectively (lower than healthy). Deterioration in FVC accelerated from 2yrs after being confined to the Wch. In deceased patients initial FVC was 31+7%p and 19+6%p prior to death (P=0.0125) while TV/FVC ratio increased from 39+9% 5yrs and to 73+11% 2yrs prior to death (p=0.0001). Ambulant patients without rRI (n=6) had FEF25-75 of 121+16%p indicating restrictive pattern. Once rRI occurred (n=31) FEF25-75 values decreased gradually to 69+27%p at the last measure near death (p=0.0001). FEF25–75/FVC ratio was 2.54+0.70 above normal in all patients until 2yrs prior to death when values increased significantly reaching 4.17+2.08 ratios.

Conclusion: Findings indicate that 5yrs prior to death TV/FVC ratios elevate to levels implying respiratory distress. Moderately reduced FEF25-75 may indicate severe airway obstruction. TV/FVC and FEF25-75/FVC ratios increase abruptly 2yrs prior to death. Treating physicians should be aware of these red flags.

  • Chronic disease
  • Children
  • Lung function testing
  • © 2014 ERS
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Late-breaking abstract: The Effect of lung volume deterioration on tidal volume, airway flows and life span in ataxia telangiectasia
Daphna Vilozni, Moran Lavie, Ifat Sarouk, Bat-El Bar Aluma, Adi Dagan, Yonit Levi, Moshe Askenazi, Miryam Ofek, Ori Efrati
European Respiratory Journal Sep 2014, 44 (Suppl 58) P4518;

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Late-breaking abstract: The Effect of lung volume deterioration on tidal volume, airway flows and life span in ataxia telangiectasia
Daphna Vilozni, Moran Lavie, Ifat Sarouk, Bat-El Bar Aluma, Adi Dagan, Yonit Levi, Moshe Askenazi, Miryam Ofek, Ori Efrati
European Respiratory Journal Sep 2014, 44 (Suppl 58) P4518;
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