Gastric hypomotility in Duchenne's muscular dystrophy.
In Duchenne's muscular dystrophy, functional impairment of smooth muscle in the gastrointestinal tract can cause acute gastric dilatation and intestinal pseudo-obstruction that may be fatal. We describe a patient with this syndrome who at autopsy had smooth-muscle degeneration of the stomach. To provide objective evidence of functional smooth-muscle impairment in Duchenne's dystrophy, we performed gastric-emptying studies in 11 patients and 11 normal controls, using technetium-99m radionuclide scintigraphy in a test meal of oatmeal. The patients with Duchenne's dystrophy had delayed gastric-emptying times (118.18 +/- 32.21 minutes [mean +/- SEM]) as compared with controls (42.5 +/- 3.4 minutes, P less than 0.01). The cause of the pathological and functional abnormalities we describe in smooth muscle is unknown but may be a deficiency of dystrophin, the recently identified gene product of the Duchenne's muscular dystrophy locus.[1]References
- Gastric hypomotility in Duchenne's muscular dystrophy. Barohn, R.J., Levine, E.J., Olson, J.O., Mendell, J.R. N. Engl. J. Med. (1988) [Pubmed]
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