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鼻腔一氧化氮在生命早期含量较低:两名原发性纤毛运动障碍婴儿的病例研究。

Nasal nitric oxide is low early in life: case study of two infants with primary ciliary dyskinesia.

作者信息

Baraldi E, Pasquale M F, Cangiotti A M, Zanconato S, Zacchello F

机构信息

Dept of Paediatrics, University of Padova, Padova, Italy.

出版信息

Eur Respir J. 2004 Nov;24(5):881-3. doi: 10.1183/09031936.04.00000504.

DOI:10.1183/09031936.04.00000504
PMID:15516684
Abstract

Nasal nitric oxide levels are low in patients with primary ciliary dyskinesia, but it is not known whether this defect is already present in the first months of life. The current authors measured nasal nitric oxide in two infants with situs inversus and primary ciliary dyskinesia, diagnosed by electron microscopy at 4 and 6 months of age, and in five healthy control infants. Nasal nitric oxide values in the primary ciliary dyskinesia infants (85 and 115 parts per billion (ppb)) were markedly lower than in the healthy controls (mean: 295 ppb, range: 225-379 ppb). This is the first report to show that nasal nitric oxide values are already low in early life in primary ciliary dyskinesia children, supporting the hypothesis that a reduced production of nasal nitric oxide is an intrinsic feature of this disease. The current authors suggest that the nasal nitric oxide test may be a useful, noninvasive method for screening young children for primary ciliary dyskinesia in clinical practice.

摘要

原发性纤毛运动障碍患者的鼻腔一氧化氮水平较低,但尚不清楚这种缺陷在生命的最初几个月是否已经存在。本文作者测量了两名患有内脏反位和原发性纤毛运动障碍的婴儿的鼻腔一氧化氮水平,这两名婴儿分别在4个月和6个月大时通过电子显微镜诊断,同时还测量了五名健康对照婴儿的鼻腔一氧化氮水平。原发性纤毛运动障碍婴儿的鼻腔一氧化氮值(85和115十亿分之一(ppb))明显低于健康对照组(平均值:295 ppb,范围:225 - 379 ppb)。这是第一份表明原发性纤毛运动障碍儿童在生命早期鼻腔一氧化氮值就已经较低的报告,支持了鼻腔一氧化氮产生减少是该疾病固有特征的假说。本文作者建议,在临床实践中,鼻腔一氧化氮测试可能是一种用于筛查幼儿原发性纤毛运动障碍的有用的非侵入性方法。

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引用本文的文献

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J Clin Med. 2014 May 9;3(2):491-503. doi: 10.3390/jcm3020491.
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Primary ciliary dyskinesia: recent advances in pathogenesis, diagnosis and treatment.原发性纤毛运动障碍:发病机制、诊断与治疗的最新进展
Drugs. 2007;67(13):1883-92. doi: 10.2165/00003495-200767130-00006.
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Exhaled nitric oxide measurements: clinical application and interpretation.呼出一氧化氮测量:临床应用与解读
Thorax. 2006 Sep;61(9):817-27. doi: 10.1136/thx.2005.056093.
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Nasal nitric oxide to diagnose primary ciliary dyskinesia in newborns.用鼻腔一氧化氮诊断新生儿原发性纤毛运动障碍。
Arch Dis Child Fetal Neonatal Ed. 2006 May;91(3):F233. doi: 10.1136/adc.2005.086702.