Camelo A L, Awad R A, Madrazo A, Aguilar F
Hospital de Pediatría, Centro Médico Nacional Siglo XXI, IMSS, México D.F.
Acta Gastroenterol Latinoam. 1997;27(3):119-22.
Myopathies are entities that mainly involve striated muscle. In Duchenne's muscular dystrophy (DMD) there have been reported smooth muscle alterations in the pre-oral phase of swallowing, in gastric emptying, and pseudoobstruction. Nevertheless, esophageal motility alterations are not concluding. The objective of this work was to determine if there are motor esophageal alterations in this patients, and if this alterations are related to the clinical manifestations of disease.
Nine consecutive patients with DMD (mean age 8, range 6-11 years; males) were evaluated, comparing clinical and manometric findings.
Esophageal manometry alterations were found in all patients, mainly simultaneous non-peristaltic waves (60.86%) of diminished amplitude, in both striated and smooth muscle. Seventy seven percent presented with upper and lower gastrointestinal symptoms (dysphagia, regurgitation, epigastric pain, constipation, and distention). No correlation was found between esophageal motility alterations and gastrointestinal symptoms, nor with the clinical stage of disease in accordance to Brook (r = 0.27).
These results show that patients with DMD present esophageal motor disorders in both striated and smooth muscle, as well as upper and lower gastrointestinal symptoms. Specialized motility studies could yield a better understanding of disease, and, possibly with adequate treatment, provide for a better quality of life in children with DMD.
肌病主要累及横纹肌。在杜氏肌营养不良症（DMD）中，已有报道称在吞咽的口腔前期、胃排空和假性梗阻方面存在平滑肌改变。然而，食管动力改变尚无定论。本研究的目的是确定该疾病患者是否存在食管运动改变，以及这些改变是否与疾病的临床表现相关。
对9例连续的DMD患者（平均年龄8岁，范围6 - 11岁；均为男性）进行评估，比较临床和测压结果。
所有患者均发现食管测压改变，主要为横纹肌和平滑肌同时出现幅度减小的非蠕动性同步波（60.86%）。77%的患者出现上、下胃肠道症状（吞咽困难、反流、上腹部疼痛、便秘和腹胀）。根据布鲁克标准，未发现食管动力改变与胃肠道症状之间存在相关性，也未发现与疾病临床分期存在相关性（r = 0.27）。
这些结果表明，DMD患者在横纹肌和平滑肌中均存在食管运动障碍，以及上、下胃肠道症状。专门的动力研究可能有助于更好地理解该疾病，并且通过适当治疗，可能会改善DMD患儿的生活质量。
