Terzi N, Orlikowski D, Fermanian C, Lejaille M, Falaize L, Louis A, Raphael J-C, Fauroux B, Lofaso F
Intensive Care Unit, Dept of Physiology-Functional Testing and Centre for Technological Innovation, Raymond Poincaré Teaching Hospital, Garches, France.
Eur Respir J. 2008 Jan;31(1):93-8. doi: 10.1183/09031936.00094707. Epub 2007 Sep 26.
Inspiratory muscle strength monitoring is crucial in patients with neuromuscular disorders. The sniff nasal inspiratory pressure (SNIP) and maximal inspiratory pressure (P(I,max)) are usually measured. The present study investigated whether the test yielding the best value at baseline continued to yield the best value during follow-up. The present study included 25 patients with Duchenne muscular dystrophy (DMD) and 61 with myotonic muscular dystrophy (MMD). SNIP and P(I,max) were measured at baseline and then annually. At baseline, SNIP was lower than P(I,max) in 20 (80%) DMD patients and 32 (52%) MMD patients. During follow-up in DMD patients, changes in the best method always occurred from SNIP to P(I,max). In MMD patients, when SNIP was better than P(I,max) at baseline, SNIP was usually (88%) better during follow-up, whereas a better P(I,max) than SNIP at baseline was frequently (50%) followed by a shift to SNIP. Maximal inspiratory pressure may be sufficient for monitoring inspiratory muscle function in Duchenne muscular dystrophy adults. In myotonic muscular dystrophy, the marked variability in the test yielding the best value at baseline indicates a need for performance of both tests at baseline. However, when sniff nasal inspiratory pressure measurement yields the best value at baseline, using sniff nasal inspiratory pressure alone during follow-up may be appropriate.
吸气肌力量监测在神经肌肉疾病患者中至关重要。通常会测量嗅吸鼻吸气压力（SNIP）和最大吸气压力（P(I,max)）。本研究调查了在基线时产生最佳值的测试在随访期间是否仍能产生最佳值。本研究纳入了25例杜氏肌营养不良症（DMD）患者和61例强直性肌营养不良症（MMD）患者。在基线时以及随后每年测量SNIP和P(I,max)。在基线时，20例（80%）DMD患者和32例（52%）MMD患者的SNIP低于P(I,max)。在DMD患者的随访期间，最佳方法的变化总是从SNIP变为P(I,max)。在MMD患者中，当基线时SNIP优于P(I,max)时，随访期间SNIP通常（88%）更好，而基线时P(I,max)优于SNIP时，随后经常（50%）会转变为SNIP。最大吸气压力可能足以监测成年杜氏肌营养不良症患者的吸气肌功能。在强直性肌营养不良症中，基线时产生最佳值的测试存在明显变异性，这表明在基线时需要同时进行这两项测试。然而，当嗅吸鼻吸气压力测量在基线时产生最佳值时，随访期间单独使用嗅吸鼻吸气压力可能是合适的。
