Abstract

DMD results from deficiency in dystrophin, a sarcolemma protein of skeletal, cardiac and smooth muscle. It is characterized by progressive striated muscle degeneration in mdx mice and humans. Its effect on enteric smooth muscle function has been studied in mdx mice and duodenal contractility, intestinal transit and fecal output have been shown to be significantly decreased. Despite a high prevalence of constipation, segmental gastrointestinal transit has not been studied in DMD patients. The aim was to evaluate gastrointestinal transit in patients with an established diagnosis of DMD. Participants older than 18 years of age were screened for constipation using Rome-III questionnaire following informed consent, and underwent an assessment of gastric emptying time, small bowel and colonic transit using a wireless motility capsule. Of the six patients (age range 18–24 years) who completed the study, one screened positive for constipation using Rome-III questionnaire. Five of six patients had normal whole gut transit times, including the one with constipation. Mean gastric emptying time was 3.45 hours (range 2.8–4.4 hours), small and large bowel transit time was 24 hours (range 4.6–49.3 hours) and mean whole-gut transit time was 27.5 hours (range 8.5–52.2 hours). One patient with a negative Rome-III screen had mildly increased gastric emptying time (4.5 hours, upper limit of normal 4 hours) and small bowel transit time (8.8 hours, normal < 8 hours). This patient had normal colonic and whole gut transit times. Gut transit in DMD patients older than 18 years was not impaired; in contrary to the reported findings in animal models of DMD.

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