Division of Respiratory Medicine, Children's Hospital of Eastern Ontario, Ontario, Canada.
Pediatr Pulmonol. 2013 Jan;48(1):59-66. doi: 10.1002/ppul.22548. Epub 2012 Mar 26.
Respiratory management of Duchenne muscular dystrophy (DMD) is not well studied and may vary across centers and practitioners. Our objective was to describe and compare the respiratory management practices of Canadian Pediatric Respirologists and Neuromuscular specialists for children with DMD.
A web-based survey was sent to all 56 practicing Canadian Pediatric Respirologists and to all 24 members of the Canadian Pediatric Neuromuscular Group (CPNG) who follow children with neuromuscular diseases. The survey included 28 questions about timing and indications for respiratory consultation, sleep disordered breathing (SDB) assessments, and treatments.
Thirty eight (68%) pediatric respirologists and 17 (71%) CPNG members responded. Respirologists provide initial consultation after a patient's first admission to hospital with respiratory complications (14/38, 37%) and when symptoms of SDB are present (14/38, 37%). Half of the CPNG members request initial Respirology consultation at the time of DMD diagnosis. Both groups request routine pulmonary function tests. Ninety-six percent of respirologists use maximal inspiratory (MIP) and expiratory pressures (MEP) to assess respiratory muscle strength, whereas 82% of CPNG members additionally use peak cough flow. Assessment for SDB is requested by both groups when pulmonary function is abnormal or patients are symptomatic. Respirologists favor polysomnography, whereas CPNG members use overnight pulse oximetry. Nocturnal non-invasive ventilation and lung volume recruitment (LVR) are used in a minority of patients.
Respirologists and CPNG members provide similar respiratory management of DMD patients, but differ in timing of consultation and choice of tests for pulmonary function and SDB. Canadian practices differ from the American Thoracic Society and Centre for Disease Control guidelines.
杜兴肌营养不良症（DMD）的呼吸管理尚未得到充分研究，并且可能因中心和医生而异。我们的目的是描述和比较加拿大儿科呼吸科医生和神经肌肉专家对患有 DMD 的儿童的呼吸管理实践。
向所有 56 名加拿大儿科呼吸科医生和所有 24 名加拿大儿科神经肌肉小组（CPNG）成员发送了一份基于网络的调查，这些成员负责随访患有神经肌肉疾病的儿童。该调查包括 28 个问题，涉及呼吸咨询的时机和指征、睡眠呼吸障碍（SDB）评估和治疗。
38 名（68％）儿科呼吸科医生和 17 名（71％）CPNG 成员做出了回应。呼吸科医生在患者因呼吸并发症首次住院后（14/38，37％）和出现 SDB 症状时（14/38，37％）提供初步咨询。一半的 CPNG 成员在 DMD 诊断时要求进行初始呼吸科咨询。两组均要求进行常规肺功能检查。96％的呼吸科医生使用最大吸气（MIP）和呼气压力（MEP）评估呼吸肌力量，而 82％的 CPNG 成员还使用峰值咳嗽流量。当肺功能异常或患者有症状时，两组均要求进行 SDB 评估。呼吸科医生赞成多导睡眠图，而 CPNG 成员则使用夜间脉搏血氧饱和度。少数患者使用夜间无创通气和肺容积募集（LVR）。
呼吸科医生和 CPNG 成员为 DMD 患者提供了类似的呼吸管理，但在咨询时机以及肺功能和 SDB 测试的选择上存在差异。加拿大的做法与美国胸科学会和疾病控制中心的指南不同。
