Vilozni Daphna, Lavie Moran, Sarouk Ifat, Bar-Aluma Bat-El, Dagan Adi, Ashkenazi Moshe, Ofek Miryam, Efrati Ori
The Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Clinic, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Israel.
The Pediatric Pulmonary Unit and the Ataxia Telangiectasia National Clinic, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Israel.
Respir Med. 2015 Jul;109(7):890-6. doi: 10.1016/j.rmed.2015.05.013. Epub 2015 May 21.
Forced vital capacity (FVC) values decrease with progress of the disease in Ataxia telangiectasia (AT).
To study the effect of this process on airway obstruction determination and life span in AT.
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.
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.
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.
在共济失调毛细血管扩张症（AT）中，用力肺活量（FVC）值会随着疾病进展而降低。
研究这一过程对AT患者气道阻塞判定及寿命的影响。
回顾性收集37例AT患者（196次肺量计测试）在5.4±1.8年（初始年龄4 - 21岁）期间的临床数据及每年最佳肺量计测量结果。12例患者可行走（其中7例有反复呼吸系统感染）；25例患者需借助轮椅，其中8例已处于终末期肺病阶段。肺量计指标包括用力肺活量（FVC）、呼气中期流速（FEF25 - 75）和潮气量（VT）。我们计算了FEF25 - 75/FVC和VT/FVC比值。
FVC从可行走时预测值的67±8下降至终末期的19±6%预测值。可行走时升高的FEF25 - 75值（预测值的116±23%），在终末期降至预测值的69±27%，其中7例患者对支气管扩张剂有反应。VT/FVC比值在可行走时为0.25±0.06，坐上轮椅后升至0.35，在终末期疾病时进一步升至0.57±0.19。FEF25 - 75/FVC比值高于正常（约1.0）2.54±0.70，在终末期升至4.16±0.75。当第一秒用力呼气容积（FEV1）仍为预测值的约45%且距死亡前2年时，FEF25 - 75/FVC比值急剧升高。
基线FVC较低（预测值的60%）会人为提高FEF25 - 75值，因此“轻度阻塞”值的FEF25 - 75可能表明AT患者存在严重气道阻塞。VT/FVC和FEF25 - 75/FVC比值因此可能有助于揭示高于正常的呼吸用力情况。结果进一步提示在AT患者的肺功能评估中增加VT/FVC和FEF25 - 75/FVC比值。
