特发性肺含铁血黄素沉着症患儿21例肺功能分析

Pulmonary function analysis of 21 children with idiopathic pulmonary hemosiderosis

目的评价特发性肺含铁血黄素沉着症（IPH）患儿的肺功能受损状况及治疗后改变，为指导诊治提供依据。方法分析2012年6月至2016年5月复旦大学附属儿科医院2l例IPH患儿的临床资料和肺功能检测结果。结果21例IPH患儿治疗前均行肺功能检查。测定结果显示4例（19．05％）患儿肺功能正常；17例（80．95％）患儿肺功能异常，包括限制性通气障碍11例（52．38％），混合性通气障碍4例（占19．05％），阻塞性通气障碍1例（4．76％），小气道病变1例（4．76％）。15例患儿激素治疗1～2个月复查肺功能显示最大肺活量占预计值百分比（VCmax％）[（77．91±18．86）％比（60．43±23．70）％]、用力肺活量占预计值百分比（FVC％）[（78．96±19．24）％比（61．03±24．62）％]、1秒钟用力呼气容积占预计值百分比（FEV，％）[（86．03±21．69）％比（65．17±26．89）％]均较治疗前明显升高，差异均有统计学意义（t=-4．13、-4．01、-4．54，均P〈0．05）。其中3例患儿肺功能随访18—40个月，动态监测肺功能显示FVC％存在波动[病例1为（69．6～84．2）％；病例2为（56．1～73．7）％；病例3为（40．4～70．2）％]。结论IPH患儿肺功能检查以限制性通气障碍为主，肺功能检测对本病的严重程度、疗效评估及预后具有重要意义。

Objective To evaluate the injury of pulmonary function of children with idiopathic pulmonary hemosiderosis（IPH） and the changes after treatment, and to provide some guidance for the diagnosis and treatment of IPH. Methods Twenty - one children with IPH who were admitted at Children＇s Hospital of Fudan University between June 2012 and May 2016 were selected. The pulmonary function and clinical data of them were analyzed. Results The general pulmonary function of 21 children with IPH before treatment with glucocorticoid was reported that 4 cases （ 19.05% ） were normal and 17 cases （80.95%） were abnormal,including 11 cases （52.38%） with restrictive ventilatory disorder,4 cases （19.05%） with mixed ventilatory disorder,1 case （4.76%） with obstructive ventilatory disorder,and 1 case （4.76%） with small airway dysfunction. Pulmonary function test was performed on 15 cases after 1 -2 months of treatment with ghicocorticoid . The results showed that maximal vital capacity （ VCmax% ） vs. the expected value was （77.91 ± 18.86）% vs. （60.43 ± 23.70）% ,forced vital capacity （FVC%） vs. the expected value was （78.96 ± 19.24 ） % vs. （61.03 ± 24.62 ） % and forced expiratory volume in one second （ FEV1 % ） vs. the expected value was （ 86.03 ± 21.69 ） % vs. （ 65.17 ± 26.89 ） %, which were significantly higher than those before treatment, and the differences were statistically significant （ t = - 4.13, - 4.01, - 4.54, all P 〈 0.05 ）. Three cases were followed up for 18 to 40 months by detecting pulmonary function and the results of dynamic monitoring of pulmonary function showed a fluctuation in FVC% [ case 1 ： （ 69.6 - 84.2） % ; case 2 ： （56.1 - 73.7 ） % ; case 3 ： （40.4 - 70.2） % ]. Conclusion The characteristic pulmonary function changes in children with IPH are restrictive ventilatory disorder. Pulmonary function test play a significant role in diagnosis, treatment and prognosis of IPH.