Marostica Paulo J C, Weist Andrea D, Eigen Howard, Angelicchio Connie, Christoph Kathy, Savage Julie, Grant Debra, Tepper Robert S
Department of Pediatric Pulmonology and Critical Care, James Whitcomb Riley Hospital for Children, Indianapolis, Indiana 46202-5225, USA.
Am J Respir Crit Care Med. 2002 Jul 1;166(1):67-71. doi: 10.1164/rccm.200111-056OC.
Spirometry is routinely used to assess pulmonary function of older children and adults with cystic fibrosis (CF); however, few data exist concerning the preschool age group. We have reported normative spirometric data for 3- to 6-year-old children. The current study was designed to assess a similarly aged group of clinically stable patients with CF. Thirty-three of 38 children with CF were able to perform 2 or 3 technically acceptable maneuvers. These patients had significantly decreased FVC, FEV(1), FEV(1)/FVC, and FEF(25-75) when expressed as z scores (number of SD from predicted): -0.75 +/- 1.63, -1.23 +/- 1.97, -0.87 +/- 1.33, and -0.74 +/- 1.63, respectively. There were significant positive correlations of the Brasfield radiological score with FVC and FEV(1) z scores (r(2) = 0.26, p < 0.01 and r(2) = 0.24, p < 0.01). In addition, homozygous patients for the DeltaF508 mutation had lower z scores for FVC (-1.21 versus 0.47, p < 0.01) and FEV(1) (-1.38 versus 0.21, p < 0.05) than heterozygous patients. Of the 14 patients who had full flow-volume spirometric measurements during infancy, 10 had FEF(25-75) z scores greater than -2 at both evaluations. Our findings suggest that spirometry can successfully be used to assess lung function in preschool children with CF and has the potential for longitudinal assessment from infancy through adulthood.
肺活量测定法常用于评估患有囊性纤维化（CF）的大龄儿童和成人的肺功能；然而，关于学龄前儿童的数据却很少。我们已经报告了3至6岁儿童的肺活量测定法标准数据。本研究旨在评估一组年龄相仿、临床病情稳定的CF患者。38名CF患儿中有33名能够完成2次或3次技术上可接受的动作。当以z分数（相对于预测值的标准差数量）表示时，这些患者的用力肺活量（FVC）、第1秒用力呼气容积（FEV₁）、FEV₁/FVC和呼气中期流速（FEF₂₅₋₇₅）显著降低，分别为-0.75±1.63、-1.23±1.97、-0.87±1.33和-0.74±1.63。布拉斯菲尔德放射学评分与FVC和FEV₁的z分数之间存在显著正相关（r² = 0.26，p < 0.01和r² = 0.24，p < 0.01）。此外，与杂合子患者相比，ΔF508突变纯合子患者的FVC（-1.21对0.47，p < 0.01）和FEV₁（-1.38对0.21，p < 0.05）的z分数更低。在14名婴儿期进行过全流速-容量肺活量测定的患者中，有10名在两次评估时FEF₂₅₋₇₅的z分数均大于-2。我们的研究结果表明，肺活量测定法可成功用于评估学龄前CF儿童的肺功能，并且有潜力从婴儿期到成年期进行纵向评估。
