鼻窦一氧化氮检测对原发性纤毛运动障碍患儿的诊断价值

Diagnostic value of nasal nitric oxide for children with primary ciliary dyskinesia

目的探究鼻窦一氧化氮(nNO)检测对原发性纤毛运动障碍(PCD)患儿的诊断价值。方法回顾性病例总结,纳入2018年3月至2022年9月于复旦大学附属儿科医院呼吸科就诊的40例PCD患儿为PCD组,纳入75例症状与PCD相似[内脏异位、内脏反位、囊性纤维化(CF)、支气管扩张、慢性化脓性肺病、哮喘]的患儿为PCD症状相似组。纳入2022年12月至2023年1月于复旦大学附属儿科医院儿童保健科或泌尿外科就诊的55例患儿为nNO正常对照组。对入组患儿进行nNO检测,总结性别、年龄及nNO数据等资料,组间比较采用Mann-WhitneyU检验或χ2检验,根据nNO数据绘制受试者工作特征曲线,计算曲线下面积,根据约登指数最大时确定nNO最佳诊断阈值。结果PCD组40例、PCD症状相似组75例(内脏异位或反位23例、CF 8例、慢性化脓性肺病或支气管扩张26例、哮喘18例)、nNO正常对照组55例,就诊年龄分别为9.7(6.7,13.4)、9.3(7.0,13.0)、9.9(7.3,13.0)岁。PCD组nNO水平均低于PCD症状相似组及nNO正常组[12(9,19)比182(121,222)、209(165,261)nl/min,U=143.00、2.00,均P<0.001]。PCD症状相似组中内脏异位或反位、CF、慢性化脓性肺病或支气管扩张、哮喘的患儿nNO水平均高于PCD组[185(123,218)、97(52,132)、154(31,202)、266(202,414)比12(9,19)nl/min,U=1.00、9.00、133.00、0,均P<0.001]。PCD组与非PCD患儿组(PCD症状相似组和nNO正常对照组)鉴别时的诊断阈值为84 nl/min,灵敏度为0.98,特异度为0.92,曲线下面积0.97(95%CI 0.95~1.00,P<0.001)。结论nNO检测能有效区分PCD患儿,并推荐84 nl/min作为PCD患儿的诊断阈值。

Objective To evaluate the value of nasal nitric oxide(nNO)measurement as a diagnostic tool for Chinese patients with primary ciliary dyskinesia(PCD).MethodsThis study is a retrospective study.The patients were recruited from those who were admitted to the respiratory Department of Respiratory Medicine,Children′s Hospital of Fudan University from March 2018 to September 2022.Children with PCD were included as the PCD group,and children with situs inversus or ambiguus,cystic fibrosis(CF),bronchiectasis,chronic suppurative lung disease and asthma were included as the PCD symptom-similar group.Children who visited the Department of Child health Care and urology in the same hospital from December 2022 to January 2023 were selected as nNO normal control group.nNO was measured during plateau exhalation against resistance in three groups.Mann-Whitney U test was used to analyze the nNO data.The receiver operating characteristic of nNO value for the diagnosis of PCD was plotted and,the area under the curve and Youden index was calculated to find the best cut-off value.ResultsnNO was measured in 40 patients with PCD group,75 PCD symptom-similar group(including 23 cases of situs inversus or ambiguus,8 cases of CF,26 cases of bronchiectasis or chronic suppurative lung disease,18 cases of asthma),and 55 nNO normal controls group.The age of the three groups was respectively 9.7(6.7,13.4),9.3(7.0,13.0)and 9.9(7.3,13.0)years old.nNO values were significantly lower in children with PCD than in PCD symptom-similar group and nNO normal controls(12(9,19)vs.182(121,222),209(165,261)nl/min,U=143.00,2.00,both P<0.001).In the PCD symptom-similar group,situs inversus or ambiguus,CF,bronchiectasis or chronic suppurative lung disease and asthma were significantly higher than children with PCD(185(123,218),97(52,132),154(31,202),266(202,414)vs.12(9,19)nl/min,U=1.00,9.00,133.00,0,all P<0.001).A cut-off value of 84 nl/min could provide the best sensitivity(0.98)and specificity(0.92)with an area under the curve of 0.97(95%CI 0.95-1.00,P<0.001).ConclusionsnNO value can draw a distinction between patients with PCD and others.A cut-off value of 84 nl/min is recommended for children with PCD.