Ataxia Telangiectasia National Clinic, Chaim Sheba Medical Center, Tel Hashomer, affiliated with the Sackler Medical School, Tel-Aviv University, Israel.
Pediatr Pulmonol. 2010 Mar;45(3):230-5. doi: 10.1002/ppul.21095.
Lung disease is a significant cause of the short life span of ataxia telangiectasia (A-T) patients. Objective lung function measurements are difficult to achieve in A-T.
To assess lung function by spirometry in relation to the clinical characteristics of A-T patients followed up at the Israeli Ataxia Telangiectasia National Clinic.
Medical and spirometry data were collected from 27 A-T patients during 2004-2007. Laboratory, nutritional condition, mode of treatment, pulmonary status, and malignancies were assessed. The spirometry values FVC, FEV(1), FEV(0.5), FEF(25-75), PEF and time rise to peak flow were analyzed individually and values were compared to those of healthy age-matched children.
Eleven patients (40.7%) were found to suffer from asthma according to clinical symptoms and response to bronchodilators. We found significant reduction in FEV(1) and FEV(0.5) (z-scores: -0.84 + or - 0.7 SD, -0.7 + or - 0.6 SD; P = 0.0014 and P = 0.003, respectively), in relation to healthy predicted values. FEF(25-75) was significantly lower than that in healthy children in 5 of 11 asthmatic patients. All 27 patients showed higher than healthy FEV(1)/FVC and FEV(0.5)/FVC ratios (z-scores 0.68 + or - 0.99 SD, P < 0.0015, and 2.12 + or - 1.50 SD, P < 0.0015, respectively). The rise time to peak flow was three-fold longer than that of healthy children.
Obstructive lung disease is common among A-T patients. Maximal peak flow reduction and prolonged rise time to peak flow may be the first signs of pulmonary involvement in these patients. Early treatment with anti-asthma therapy, bronchodilators, and steroids, may prevent further pulmonary deterioration and improve the prognosis of A-T patients.
肺部疾病是共济失调毛细血管扩张症（A-T）患者寿命缩短的重要原因。A-T 患者的目标肺功能测量难以实现。
通过肺活量测定法评估在以色列共济失调毛细血管扩张症国家诊所接受随访的 A-T 患者的肺功能与临床特征的关系。
2004 年至 2007 年期间，从 27 名 A-T 患者中收集了医学和肺活量测定数据。评估了实验室、营养状况、治疗方式、肺部状况和恶性肿瘤。分别分析了 FVC、FEV（1）、FEV（0.5）、FEF（25-75）、PEF 和峰值流量上升时间等肺活量值，并将其与健康同龄儿童的相应值进行了比较。
根据临床症状和对支气管扩张剂的反应，11 名患者（40.7%）被诊断为哮喘。我们发现 FEV（1）和 FEV（0.5）明显降低（z 分数：-0.84±0.7 SD，-0.7±0.6 SD；P=0.0014 和 P=0.003），与健康预测值相比。在 11 名哮喘患者中，有 5 名患者的 FEF（25-75）明显低于健康儿童。27 名患者的 FEV（1）/FVC 和 FEV（0.5）/FVC 比值均高于健康儿童（z 分数 0.68±0.99 SD，P<0.0015 和 2.12±0.99 SD，P<0.0015）。峰值流量上升时间是健康儿童的三倍。
A-T 患者中常见阻塞性肺部疾病。最大峰值流量减少和峰值流量上升时间延长可能是这些患者肺部受累的最初迹象。早期使用抗哮喘治疗、支气管扩张剂和类固醇治疗可能会防止肺部进一步恶化并改善 A-T 患者的预后。
