The Pediatric Pulmonary Unit, The Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Ramat-Gan, Affiliated with the Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Pediatr Pulmonol. 2010 Oct;45(10):1030-6. doi: 10.1002/ppul.21291.
To explore the feasibility and validity of forced spirometry in patients with ataxia telangiectasia (A-T).
Twenty-eight patients (aged 3.7-19.3 years) performed spirometry on 47 occasions. Parameters studied were technical quality and relation to: predicted values, pulmonary illness.
Start of test criteria for correct expiratory effort was significantly prolonged (183 ± 115 ms; P < 0.001). The rise-time to peak flow in children free of respiratory symptoms (Group-FRS; n = 8) increased by 16.2 ± 12.5 ms/year above recommended and in children having recurrent infections (n = 8) 30.4 ± 16.1 ms/year, P < 0.01. Expiration-time was significantly shorter than requested (1.21 ± 0.47 sec) and was ended abruptly in 57% of the patients. FEV(1) could not be established by 8/20 patients. The intra-subject reproducibility met criteria (4.4 ± 2.7%, 5.2 ± 2.8%, 2.9 ± 3.2%, 6.3 ± 5.3%, for FVC, FEV(0.5), PEF, FEF(25-75), respectively). Group-FRS showed yearly deterioration in FVC of 2.2%, while patients with hyper-reactive airways (Group-HRA; n =12) had a deterioration rate of 3.6%/year. FEV(0.5) deterioration rate was similar in both groups (2.2 and 2.0, respectively), but baseline values in Group-HRA were significantly lower than those of Group-FRS (P = 0.029) in similar young ages, indicating airway obstruction at early ages in Group-HRA. FEV(0.5) values deterioration also correlated with body mass index (P < 0.017).
Forced spirometry in A-T patients is reproducible and has a distinct pattern, although curves do not meet other recommendations for acceptable criteria. The study insinuates that a rapid deterioration in lung function occurs in A-T patients with recurrent respiratory infection, suggesting that early intervention may prevent further deterioration or improve their lung function. Further studies are needed to confirm our results.
探讨在共济失调毛细血管扩张症（A-T）患者中使用强制肺活量测定法的可行性和有效性。
28 名患者（年龄 3.7-19.3 岁）在 47 次检查中进行了肺活量测定。研究的参数包括技术质量以及与以下因素的关系：预测值、肺部疾病。
正确呼气努力的测试标准起始时间明显延长（183±115ms；P<0.001）。无呼吸道症状的儿童（组-FRS；n=8）的升时峰值流量每年增加 16.2±12.5ms/年，超过了推荐值，而反复感染的儿童（n=8）每年增加 30.4±16.1ms/年，P<0.01。呼气时间明显短于要求（1.21±0.47 秒），并且在 57%的患者中突然结束。有 8/20 名患者无法确定 FEV1。个体内可重复性符合标准（FVC、FEV（0.5）、PEF 和 FEF（25-75）分别为 4.4±2.7%、5.2±2.8%、2.9±3.2%和 6.3±5.3%）。组-FRS 的 FVC 每年恶化 2.2%，而气道高反应性患者（组-HRA；n=12）的恶化率为 3.6%/年。两组 FEV（0.5）的恶化率相似（分别为 2.2%和 2.0%），但组-HRA 的基线值明显低于组-FRS（在相似的年龄时，P=0.029），表明组-HRA 中的气道阻塞发生在早期。FEV（0.5）值的恶化也与体重指数（P<0.017）相关。
尽管曲线不符合其他可接受标准的要求，但在 A-T 患者中进行强制肺活量测定法是可重复的，并且具有独特的模式。研究表明，反复呼吸道感染的 A-T 患者的肺功能迅速恶化，表明早期干预可能预防进一步恶化或改善其肺功能。需要进一步的研究来证实我们的结果。
