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选择鼻一氧化氮技术作为原发性纤毛运动障碍的一线检测方法。

Choice of nasal nitric oxide technique as first-line test for primary ciliary dyskinesia.

机构信息

Danish PCD Center, Paediatric Pulmonary Service, 5003, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.

出版信息

Eur Respir J. 2011 Mar;37(3):559-65. doi: 10.1183/09031936.00032610. Epub 2010 Jun 4.

DOI:10.1183/09031936.00032610
PMID:20525709
Abstract

Nasal nitric oxide (nNO) has a well-known potential as an indirect discriminative marker between patients with primary ciliary dyskinesia (PCD) and healthy subjects, but real-life experience and usefulness in young children is sparsely reported. Three nNO sampling methods were examined and compared as first-line tests for PCD. Healthy subjects, confirmed PCDs, consecutive referrals with PCD-like symptoms and patients with cystic fibrosis (CF) had nNO sampled during breath hold (BH-nNO), oral exhalation against resistance (OE-R-nNO) and tidal breathing (TB-nNO) aiming to expand age range into infancy. 282 subjects, 117 consecutive referrals, 59 PCDs, 49 CF patients and 57 healthy subjects, were included. All methods separated significantly between PCD and non-PCD, including CF with reliability, in ranking order BH-nNO>OE-R-nNO>TB-nNO. Acceptability in children ranked in reverse order. A problematic high fraction (39%) of false positive TB-nNO was found in young children. An unexpected large fraction (6.8%) of PCDs had nNO values above cut-off. nNO is a helpful first-line tool in real-life PCD work-up in all age groups if the sampling method is chosen according to age. nNO can be misleading in a few patients with true PCD. Further studies are strongly needed in young children.

摘要

鼻一氧化氮(nNO)作为原发性纤毛运动障碍(PCD)患者与健康受试者之间的间接鉴别标志物具有良好的潜力,但在幼儿中的实际经验和应用却鲜有报道。本文共检查并比较了 3 种 nNO 采样方法,作为 PCD 的一线检测方法。在屏气(BH-nNO)、口腔呼气抵抗(OE-R-nNO)和潮气呼吸(TB-nNO)时对健康受试者、确诊的 PCD 患者、具有 PCD 样症状的连续转诊患者和囊性纤维化(CF)患者进行 nNO 采样,旨在将年龄范围扩展至婴儿期。共纳入 282 名受试者、117 例连续转诊患者、59 例 PCD 患者、49 例 CF 患者和 57 例健康受试者。所有方法均能可靠地区分 PCD 和非 PCD,排序为 BH-nNO>OE-R-nNO>TB-nNO。在儿童中,可接受性则相反。在幼儿中,TB-nNO 出现了 39%的高比例假阳性,这是一个有问题的结果。出乎意料的是,有 6.8%的 PCD 患者 nNO 值超过了临界值。如果根据年龄选择采样方法,nNO 是所有年龄段 PCD 实际检测中的一种有用的一线工具。但在少数真正的 PCD 患者中,nNO 可能会产生误导。在幼儿中还需要进行更多的研究。

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