Matsui Kiyoshi, Yamashita Sumimasa, Shibasaki Jun, Watanabe Tatsuya
Division of Neonatalogy, Kanagawa Children's Medical Center, Yokohama.
No To Hattatsu. 2007 Jul;39(4):304-8.
Although gastrointestinal symptoms are frequently observed in congenital myotonic dystrophy (congenital MD) during early neonatal periods, there are few reports of gastrointestinal smooth muscle involvement and its management. We report two cases with congenital MD treated with bethanechol (0.25 mg/kg/dose 30 min before breast milk, 8 doses/day) for gastrointestinal dismotility. Two patients showed gastrointestinal symptoms characterized by increased gastric residua prior to the next feeding and gasless abdomen with relative gastric dilatation on abdominal X ray. Treatment with bethanechol resolved the gastrointestinal symptoms and allowed increase of daily feeding volume. We speculate that the main pathogenesis of transient gastrointestinal dismotility in neonates with congenital MD is gastroparesis probably due to "maturation arrest" of smooth muscle. Bethanechol may be one of the alternative prokinetic drugs to increase gastric emptying.
尽管先天性肌强直性营养不良(先天性MD)在新生儿早期常出现胃肠道症状,但关于胃肠道平滑肌受累及其治疗的报道却很少。我们报告了2例先天性MD患者,使用氨甲酰甲胆碱(0.25mg/kg/剂量,在母乳喂养前30分钟给药,每天8次)治疗胃肠道动力障碍。2例患者均表现出胃肠道症状,特征为下次喂养前胃残余量增加,腹部X线显示无气腹伴相对胃扩张。氨甲酰甲胆碱治疗缓解了胃肠道症状,并使每日喂养量增加。我们推测,先天性MD新生儿短暂性胃肠道动力障碍的主要发病机制可能是由于平滑肌“成熟停滞”导致的胃轻瘫。氨甲酰甲胆碱可能是增加胃排空的替代促动力药物之一。