Pierre et Marie Curie University Paris 06, France.
Pediatr Pulmonol. 2011 Jun;46(6):587-94. doi: 10.1002/ppul.21409. Epub 2011 Jan 28.
Upper airway obstruction is common in children with mucopolysaccharidosis. The acoustic reflection method is a noninvasive technique that can analyze the caliber of the upper airways. The aim of the study was to evaluate the feasibility of the acoustic reflection method in children with mucopolysaccharidosis, and to compare the characteristics of the upper airways evaluated by the acoustic reflection method in patients with mucopolysaccharidosis to matched healthy counterparts.
Open, single center, prospective, study.
Accurate acoustic reflection measurements could be obtained in 7 of 10 patients (mean age: 10.4 ± 3.9 years; mucopolysaccharidosis type II (n = 3); type IV (n = 2), type VI (n = 1), and fucosidosis (n = 1)). The mean minimum cross-sectional area was lower in mucopolysaccharidosis patients (1.6 ± 0.3 cm(2) ) as compared to 14 healthy counterparts (1.8 ± 0.3 cm(2); P = 0.03). The mean resistance of the airways was significantly higher in the MPS group (7.9 ± 1.8 cmH(2) O l(-1) sec) as compared to the controls (5.5 ± 1.2 cmH(2) O l(-1) sec; P = 0.006).
This study is the first to analyze the upper airways by the noninvasive acoustic reflection method in children with mucopolysaccharidosis. Due to a lack of cooperation, reliable measurements could only be obtained in 70% of a selected group of patients. Children with mucopolysaccharidosis have significant upper airway obstruction as assessed by the reduction of the minimal cross-sectional area of the upper airways and the increase in airway resistance.
上气道阻塞在黏多糖贮积症患儿中很常见。声反射法是一种非侵入性技术，可分析上气道的口径。本研究旨在评估声反射法在黏多糖贮积症患儿中的可行性，并比较声反射法评估的黏多糖贮积症患儿与匹配健康对照者的上气道特征。
开放性、单中心、前瞻性研究。
10 例患者中有 7 例（平均年龄：10.4 ± 3.9 岁；黏多糖贮积症 II 型（n = 3）；IV 型（n = 2），VI 型（n = 1）和岩藻糖苷贮积症（n = 1））可获得准确的声反射测量值。黏多糖贮积症患者的最小横截面积平均值较低（1.6 ± 0.3 cm²），而 14 例健康对照者的最小横截面积平均值较高（1.8 ± 0.3 cm²；P = 0.03）。MPS 组气道阻力的平均值明显高于对照组（7.9 ± 1.8 cmH2O l-1 sec）（P = 0.006）。
本研究首次采用非侵入性声反射法分析黏多糖贮积症患儿的上气道。由于合作程度不够，仅在选择的一组患者中 70%的患者获得了可靠的测量值。黏多糖贮积症患儿的上气道阻塞明显，表现为上气道最小横截面积减小和气道阻力增加。
