脉冲振荡法可识别腺苷脱氨酶缺乏症患儿的外周气道功能障碍。
Impulse oscillometry identifies peripheral airway dysfunction in children with adenosine deaminase deficiency.
作者信息
Komarow Hirsh D, Sokolic Robert, Hershfield Michael S, Kohn Donald B, Young Michael, Metcalfe Dean D, Candotti Fabio
机构信息
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, NIH/NIAID/LAD/Bldg. 10, Room 1C129A1, 10 Center Drive, Bethesda, MD, 20892-1960, USA.
National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, 20892, USA.
出版信息
Orphanet J Rare Dis. 2015 Dec 18;10:159. doi: 10.1186/s13023-015-0365-z.
Adenosine deaminase-deficient severe combined immunodeficiency (ADA-SCID) is characterized by impaired T-, B- and NK-cell function. Affected children, in addition to early onset of infections, manifest non-immunologic symptoms including pulmonary dysfunction likely attributable to elevated systemic adenosine levels. Lung disease assessment has primarily employed repetitive radiography and effort-dependent functional studies. Through impulse oscillometry (IOS), which is effort-independent, we prospectively obtained objective measures of lung dysfunction in 10 children with ADA-SCID. These results support the use of IOS in the identification and monitoring of lung function abnormalities in children with primary immunodeficiencies.
腺苷脱氨酶缺乏的重症联合免疫缺陷病（ADA-SCID）的特征是T细胞、B细胞和NK细胞功能受损。患病儿童除了早期出现感染外，还表现出非免疫性症状，包括可能归因于全身腺苷水平升高的肺功能障碍。肺部疾病评估主要采用重复X线摄影和依赖用力的功能研究。通过独立于用力的脉冲振荡法（IOS），我们前瞻性地获得了10例ADA-SCID患儿肺功能障碍的客观指标。这些结果支持将IOS用于原发性免疫缺陷患儿肺功能异常的识别和监测。
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