Characterization of Pulmonary Function in Duchenne Muscular Dystrophy
Characterization of Pulmonary Function in Duchenne Muscular Dystrophy
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DOI：
10.1002/ppul.23172
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发表时间：
2015-05-01
影响因子：
3.1
通讯作者：
Meier, T.
中科院分区：
文献类型：
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作者：
Mayer, O. H.;Finkel, R. S.;Meier, T.
杜氏肌营养不良症（DMD）患者肺功能下降导致显著的发病率和寿命降低。肺活量测定法是一种广泛使用且相当容易执行的技术，用于评估肺功能，特别是肺容量；然而，美国胸科学会（ATS）的可接受性标准可能过于严格，不适合神经肌肉疾病患者。我们检查了60名DMD患者（中位年龄10.3岁，范围5-24岁）的前瞻性肺活量测定数据（用力肺活量[FVC]和呼气峰值流量[PEF]）。由肺科医生检查呼气流量-体积曲线，并根据神经肌肉疾病患者的能力修改ATS标准，评估数据的可接受性。然后分析数据随年龄、活动状况和糖皮质激素使用的变化。在44名受试者（73%）中至少获得了一项可接受的研究，131项研究中有81项（62%）可接受。随着年龄的增长，植被覆盖度和植被覆盖度的绝对值呈现出相似的相对变化趋势，即在10年时相对增加，在10-18年时相对稳定，然后随着年龄的增长而下降。从5岁到24岁，FVC和PEF的预测百分比呈线性下降，约为5% /年。令人惊讶的是，步行或类固醇治疗在FVC或PEF方面没有观察到差异。可接受的肺活量测定法可用于各种年龄的DMD患者。使用改良的ATS标准，整理肺活量测定数据，排除技术上不可接受的数据，可以提供更可靠的方法来确定肺功能随时间的变化。儿科肺科杂志2015；50:487 - 494。(c) 2015 Wiley期刊公司
Decline in pulmonary function in Duchenne Muscular Dystrophy (DMD) contributes to significant morbidity and reduced longevity. Spirometry is a widely used and fairly easily performed technique to assess lung function, and in particular lung volume; however, the acceptability criteria from the American Thoracic Society (ATS) may be overly restrictive and inappropriate for patients with neuromuscular disease. We examined prospective spirometry data (Forced Vital Capacity [FVC] and peak expiratory flow [PEF]) from 60 DMD patients enrolled in a natural history cohort study (median age 10.3 years, range 5-24 years). Expiratory flow-volume curves were examined by a pulmonologist and the data were evaluated for acceptability using ATS criteria modified based on the capabilities of patients with neuromuscular disease. Data were then analyzed for change with age, ambulation status, and glucocorticoid use. At least one acceptable study was obtained in 44 subjects (73%), and 81 of the 131 studies (62%) were acceptable. The FVC and PEF showed similar relative changes in absolute values with increasing age, i.e., an increase through 10 years, relative stabilization from 10-18 years, and then a decrease at an older age. The percent predicted, FVC and PEF showed a near linear decline of approximately 5% points/year from ages 5 to 24. Surprisingly, no difference was observed in FVC or PEF by ambulation or steroid treatment. Acceptable spirometry can be performed on DMD patients over a broad range of ages. Using modified ATS criteria, curated spirometry data, excluding technically unacceptable data, may provide a more reliable means of determining change in lung function over time. Pediatr Pulmonol. 2015; 50:487-494. (c) 2015 Wiley Periodicals, Inc.