Chew Kenneth, Carey Kate, Ho Genevieve, Mallitt Kylie-Ann, Widger John, Farrar Michelle
Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, The University of New South Wales, Sydney, Australia.
Discipline of Paediatrics, School of Women's and Children's Health, UNSW Medicine, The University of New South Wales, Sydney, Australia; Department of Respiratory Medicine, Sydney Children's Hospital, Randwick, NSW 2031, Australia.
Respir Med. 2016 Oct;119:35-40. doi: 10.1016/j.rmed.2016.08.018. Epub 2016 Aug 22.
The multidisciplinary care of Duchenne muscular dystrophy (DMD) incorporates management of nutrition and the respiratory system, however the effect of body habitus on respiratory function in DMD is poorly understood. The present study examined the impact of nutritional status on respiratory function in DMD to guide further treatment strategies.
Anthropometric and respiratory parameters, such as body mass index (BMI) z-scores, forced vital capacity (FVC) and forced expiratory volume in one second (FEV) were retrospectively analysed with a mixed linear model in 34 DMD patients. Cross-sectional analysis of cough peak flow (CPF) in upright and supine positions and body fat mass were examined in 12 DMD patients.
Respiratory function in DMD patients was significantly related to BMI Z-score (P < 0.001), age (P < 0.05) and mobility (P < 0.001). DMD patients with greater BMI Z-score had increased respiratory function, even when adjusting for age and mobility status, with a 1 unit increase in BMI z-score associated with a 7.43% increase in FVC% predicted (P < 0.001). Body fat mass was adversely associated with FVC with a 1% body fat increase associated with a 1.5% reduction in FVC (P < 0.05). CPF values were significantly lower in supine compared to upright position (P = 0.005) and greater postural reductions in CPF were associated with higher body fat percent, with a 1% body fat increase associated with a 1.5% increase in postural CPF difference (P < 0.05).
The present study reinforces the importance of weight management in DMD, showing that a higher weight profile and lower adiposity have better respiratory outcomes. Furthermore, attention to body position with airway clearance techniques will maximize their effectiveness.
杜氏肌营养不良症（DMD）的多学科护理包括营养和呼吸系统管理，然而，身体体型对DMD患者呼吸功能的影响尚不清楚。本研究旨在探讨营养状况对DMD患者呼吸功能的影响，以指导进一步的治疗策略。
采用混合线性模型对34例DMD患者的人体测量和呼吸参数进行回顾性分析，如体重指数（BMI）z评分、用力肺活量（FVC）和一秒用力呼气量（FEV）。对12例DMD患者进行了直立位和仰卧位咳嗽峰流速（CPF）及体脂量的横断面分析。
DMD患者的呼吸功能与BMI Z评分（P<0.001）、年龄（P<0.05）和活动能力（P<0.001）显著相关。即使在调整年龄和活动状态后，BMI Z评分较高的DMD患者呼吸功能仍有所改善，BMI z评分每增加1个单位，预计FVC%增加7.43%（P<0.001）。体脂量与FVC呈负相关，体脂增加1%，FVC降低1.5%（P<0.05）。仰卧位CPF值显著低于直立位（P=0.005），CPF的姿势性降低越大，体脂百分比越高，体脂增加1%，姿势性CPF差异增加1.5%（P<0.05）。
本研究强化了DMD患者体重管理的重要性，表明较高的体重和较低的肥胖程度具有更好的呼吸结局。此外，采用气道清理技术时注意身体姿势将使其效果最大化。
