Gauld Leanne M, Boynton Alison, Betts Grant A, Johnston Heather
Department of Respiratory Medicine, Sydney Children's Hospital, Sydney, New South Wales, Australia.
Pediatr Pulmonol. 2005 Nov;40(5):408-13. doi: 10.1002/ppul.20278.
Children with Duchenne muscular dystrophy (DMD) have progressive respiratory muscle weakness. Spirometry monitors progress, but is effort-dependent. Intelligence quotients (IQ) average one standard deviation below normal, and behavioral disturbance is common. Our aim was to assess if impaired intelligence or behavior influences spirometry in children with DMD, and if computerized visual incentives (CVI) are beneficial. Forty-seven boys with DMD, of mean age 12.6 years (range, 6-19), were recruited. Full-scale, performance, and verbal IQ, and parent-and-teacher-reported oppositional behavior scores, were recorded. Each was divided into moderate, mild, and no impairment groups. A randomized crossover design was applied to performing spirometry with or without CVI first. A Jaeger Masterscope (version 4.60) was used. Linear regression defined the relationship between spirometry and both IQ and behavior scores. The paired Student's t- test compared spirometry performed with and without CVI for the overall group and subgroups. Boys with DMD can adequately perform spirometry. There is an association between %FEV1 and %FVC with full-scale (r = 0.50, P = 0.002; r = 0.49, P = 0.003, respectively), performance (r = 0.68, P < 0.0005; r = 0.68, P < 0.0005, respectively) and verbal (r = 0.39, P = 0.043; r = 0.36, P = 0.037, respectively) IQ, but not with parent (P = 0.77, P = 0.70, respectively) or teacher (P = 0.90, P = 0.90, respectively)-reported oppositional behavior scores. The effect of CVI was significant in those with moderate full-scale (P = 0.03), performance (P = 0.002), and verbal (P = 0.02) intellectual impairment, and moderately severe teacher-reported oppositional behavior (P = 0.02). In conclusion, spirometry results are related to intelligence in DMD. Using CVI improves the FVC obtained in those with moderate intellectual or behavioral impairment.
杜兴氏肌营养不良症（DMD）患儿存在进行性呼吸肌无力。肺活量测定可监测病情进展，但受用力程度影响。其智商平均比正常水平低一个标准差，行为障碍也很常见。我们的目的是评估智力或行为受损是否会影响DMD患儿的肺活量测定，以及计算机化视觉激励（CVI）是否有益。招募了47名平均年龄为12.6岁（范围6 - 19岁）的DMD男孩。记录了全量表、操作和言语智商，以及家长和教师报告的对立行为得分。将每个指标分为中度、轻度和无损害组。采用随机交叉设计，先进行有或无CVI的肺活量测定。使用耶格Masterscope（版本4.60）。线性回归确定了肺活量测定与智商和行为得分之间的关系。配对t检验比较了全组及各亚组在有和无CVI情况下进行的肺活量测定。DMD男孩能够充分配合进行肺活量测定。第1秒用力呼气容积（%FEV1）和用力肺活量（%FVC）与全量表智商（分别为r = 0.50，P = 0.002；r = 0.49，P = 0.003）、操作智商（分别为r = 0.68，P < 0.0005；r = 0.68，P < 0.0005）和言语智商（分别为r = 0.39，P = 0.043；r = 0.36，P = 0.037）相关，但与家长报告（分别为P = 0.77，P = 0.70）或教师报告（分别为P = 0.90，P = 0.90）的对立行为得分无关。CVI对中度全量表智力损害（P = 0.03）、操作智力损害（P = 0.002）、言语智力损害（P = 0.02）以及教师报告的中度严重对立行为（P = 0.02）的患儿有显著效果。总之，肺活量测定结果与DMD患儿的智力相关。使用CVI可提高中度智力或行为损害患儿的用力肺活量。
