INTRODUCTION: We tested the feasibility of using neuromuscular ultrasound for non-invasive real-time assessment of diaphragmatic structure and function in a canine model of X-linked myotubular myopathy (XLMTM). METHODS: Ultrasound images in 3 dogs [wild-type (WT), n=1; XLMTM untreated, n=1; XLMTM post-AAV8-mediated MTM1 gene replacement, n=1] were analyzed for diaphragm thickness, change in thickness with respiration, muscle echogenicity, and diaphragm excursion amplitude during spontaneous breathing. RESULTS: Quantitative parameters of diaphragm structure were different among the animals. WT diaphragm was thicker and less echogenic than the XLMTM control, whereas the diaphragm measurements of the MTM1-treated XLMTM dog were comparable to those of the WT dog. CONCLUSIONS: This pilot study demonstrates the feasibility of using ultrasound for quantitative assessment of the diaphragm in a canine model. In the future, ultrasonography may replace invasive measures of diaphragm function in canine models and in humans for non-invasive respiratory monitoring and evaluation of neuromuscular disease.
INTRODUCTION: We tested the feasibility of using neuromuscular ultrasound for non-invasive real-time assessment of diaphragmatic structure and function in a canine model of X-linked myotubular myopathy (XLMTM). METHODS: Ultrasound images in 3 dogs [wild-type (WT), n=1; XLMTM untreated, n=1; XLMTM post-AAV8-mediated MTM1 gene replacement, n=1] were analyzed for diaphragm thickness, change in thickness with respiration, muscle echogenicity, and diaphragm excursion amplitude during spontaneous breathing. RESULTS: Quantitative parameters of diaphragm structure were different among the animals. WT diaphragm was thicker and less echogenic than the XLMTM control, whereas the diaphragm measurements of the MTM1-treated XLMTM dog were comparable to those of the WT dog. CONCLUSIONS: This pilot study demonstrates the feasibility of using ultrasound for quantitative assessment of the diaphragm in a canine model. In the future, ultrasonography may replace invasive measures of diaphragm function in canine models and in humans for non-invasive respiratory monitoring and evaluation of neuromuscular disease.
Authors: Robert W Grange; Jon Doering; Erin Mitchell; Melanie N Holder; Xuan Guan; Melissa Goddard; Christopher Tegeler; Alan H Beggs; Martin K Childers Journal: Muscle Nerve Date: 2012-10 Impact factor: 3.217
Authors: Michael W Lawlor; Dustin Armstrong; Marissa G Viola; Jeffrey J Widrick; Hui Meng; Robert W Grange; Martin K Childers; Cynthia P Hsu; Michael O'Callaghan; Christopher R Pierson; Anna Buj-Bello; Alan H Beggs Journal: Hum Mol Genet Date: 2013-01-09 Impact factor: 6.150
Authors: Aarti Sarwal; Michael S Cartwright; Erin Mitchell; Koudy Williams; Francis O Walker; Martin K Childers Journal: Muscle Nerve Date: 2015-02 Impact factor: 3.217
Authors: Nicholas P Whitehead; Kenneth L Bible; Min Jeong Kim; Guy L Odom; Marvin E Adams; Stanley C Froehner Journal: J Physiol Date: 2016-10-13 Impact factor: 5.182