Borrelli Osvaldo, Salvia Gennaro, Mancini Valentina, Santoro Lucio, Tagliente Francesca, Romeo Erminia Francesca, Cucchiara Salvatore
Department of Pediatrics, Pediatric Gastroenterology Unit, University of Rome, Rome, Italy.
Am J Gastroenterol. 2005 Mar;100(3):695-702. doi: 10.1111/j.1572-0241.2005.41303.x.
Although muscular dystrophy (MD) affects primarily striated muscles, smooth muscle cells of the gastrointestinal tract may also be involved. We recorded gastric electrical activity and gastric emptying time (GET) in children with MD at initial presentation and at 3-yr follow-up in order to detect gastric motor abnormalities and study their evolution along the clinical course.
Twenty children with MD (median age: 4.6 yr; range age: 3-7 yr) were investigated by means of ultrasonography, for measuring GET, and by electrogastrography (EGG); 70 children served as controls.
Ten patients had Duchenne muscular dystrophy (DMD) and 10 Becker muscular dystrophy (BMD). GET was significantly more delayed in MD patients (DMD, median: 195 min; range 150-260 min; BMD, median: 197 min; range: 150-250 min) than in controls (median: 150 min; 110-180 min; p < 0.05); it markedly worsened at the follow-up in DMD (median: 270 min; range 170-310 min; p < 0.001 vs controls) but not in BMD patients (median: 205 min; 155-275 min; p < 0.05 vs DMD). Baseline EGG showed a significantly lower prevalence of normal rhythm and significantly higher prevalence of dysrhythmias in both groups of patients as compared to controls (% of normal rhythm: DMD 66.7 +/- 8.2, BMB 67.2 +/- 11.5, controls 85.3 +/- 7.2, p < 0.001; % of tachygastria: DMD 28.4 +/- 8.0, BMB 29.8 +/- 12.3, controls 10.6 +/- 5.1, p < 0.001; % of dominant frequency instability coefficient: DMD 36.1 +/- 6.0, BMB 33.2 +/- 2.9, controls 17.9 +/- 7.1, p < 0.001); furthermore, no difference in fed-to-fasting ratio of the dominant EGG power was found between the two groups and controls (DMD 2.84 +/- 1.27, BMB 2.82 +/- 0.98, controls 3.04 +/- 0.85, ns). However, at the follow-up no significant change in the prevalence of normal rhythm and dysrhythmias occurred in both groups (ns vs baseline values), whereas only DMD patients showed a marked reduction in fed-to-fasting power ratio (0.78 +/- 0.59; p < 0.001 vs controls and BMD; p < 0.05 vs baseline), which correlated with the progressive neuromuscular weakness occurring in DMD subjects (r, 0.75; p < 0.001).
In children with MD, there is an early abnormality in gastric motility that is due to deranged regulatory mechanisms, whereas contractile activity of smooth muscle cells seems to be preserved. At the follow-up, DMD patients exhibited a progressive failure in neuromuscular function, which was accompanied by a gastric motility derangement with worsening in GET and in EGG features suggesting an altered function of gastric smooth muscle cells.
尽管肌肉营养不良症（MD）主要影响横纹肌，但胃肠道的平滑肌细胞也可能受累。我们记录了MD患儿初诊时及3年随访时的胃电活动和胃排空时间（GET），以检测胃运动异常并研究其在临床病程中的演变。
通过超声检查测量GET，并采用胃电图（EGG）对20例MD患儿（中位年龄：4.6岁；年龄范围：3 - 7岁）进行研究；70例儿童作为对照。
10例患者为杜氏肌营养不良症（DMD），10例为贝克肌营养不良症（BMD）。MD患者的GET明显延迟（DMD，中位值：195分钟；范围150 - 260分钟；BMD，中位值：197分钟；范围：150 - 250分钟），而对照组为（中位值：150分钟；110 - 180分钟；p < 0.05）；在随访中，DMD患者的GET显著恶化（中位值：270分钟；范围170 - 310分钟；与对照组相比p < 0.001），但BMD患者未出现这种情况（中位值：205分钟；155 - 275分钟；与DMD相比p < 0.05）。基线EGG显示，与对照组相比，两组患者正常节律的患病率显著降低，心律失常的患病率显著升高（正常节律百分比：DMD 66.7 ± 8.2，BMB 67.2 ± 11.5，对照组85.3 ± 7.2，p < 0.001；快速胃动百分比：DMD 28.4 ± 8.0，BMB 29.8 ± 12.3，对照组10.6 ± 5.1，p < 0.001；优势频率不稳定系数百分比：DMD 36.1 ± 6.0，BMB 33.2 ± 2.9，对照组17.9 ± 7.1，p < 0.001）；此外，两组与对照组之间在EGG优势功率的进食与空腹比值上未发现差异（DMD 2.84 ± 1.27，BMB 2.82 ± 0.98，对照组3.04 ± 0.85，无显著性差异）。然而，在随访中，两组正常节律和心律失常的患病率均未发生显著变化（与基线值相比无显著性差异），而只有DMD患者的进食与空腹功率比值显著降低（0.78 ± 0.59；与对照组和BMD相比p < 0.001；与基线相比p < 0.05），这与DMD患者出现的进行性神经肌肉无力相关（r，0.75；p < 0.001）。
在MD患儿中，胃动力早期存在异常，这是由于调节机制紊乱所致，而平滑肌细胞的收缩活性似乎得以保留。在随访中，DMD患者表现出神经肌肉功能的进行性衰竭，同时伴有胃动力紊乱，GET和EGG特征恶化，提示胃平滑肌细胞功能改变。
