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Upper gastrointestinal tract motility in children with progressive muscular dystrophy
A Staiano1, E Del Giudice, A Romano
1Department of Pediatrics, University of Naples, II Medical School, Italy.
Insights
Children with progressive muscular dystrophy show early gastrointestinal smooth muscle dysfunction, detectable via gastric emptying studies, even with minimal symptoms. This suggests upper gastrointestinal tract involvement in early-stage myopathy.
Area of Science:
- Pediatric Gastroenterology
- Neuromuscular Disorders
- Gastrointestinal Motility
Background:
- Progressive muscular dystrophy (PMD) primarily affects skeletal muscles.
- Gastrointestinal (GI) smooth muscle involvement in pediatric PMD is often undiagnosed early.
- Early detection of GI dysfunction can inform disease management.
Purpose of the Study:
- To assess early gastrointestinal smooth muscle involvement in children with PMD.
- To evaluate gastric emptying and esophageal motility in pediatric PMD patients.
- To correlate GI findings with the stage of neuromuscular disease.
Main Methods:
- Gastric emptying studies using technetium 99m-sulfur colloid scintigraphy in 15 children with PMD.
- Esophageal manometry in 10 of the PMD patients.
- Comparison with 11 age-matched healthy children.
Main Results:
- Significantly greater gastric isotope retention in PMD patients compared to controls, indicating delayed gastric emptying.
- No significant differences in distal esophageal motility or sphincter function.
- Reduced contraction amplitudes in the upper esophagus of PMD patients.
Conclusions:
- Early upper gastrointestinal smooth muscle dysfunction is detectable in children with PMD.
- Gastric emptying studies can identify GI involvement before overt symptoms appear.
- Esophageal motility changes, specifically in the upper esophagus, may also indicate early disease progression.
Abstract:
Gastric emptying was evaluated in 15 children (mean age, 8.0 years) with progressive muscular dystrophy to detect early gastrointestinal smooth muscle involvement; 10 of the children also underwent esophageal manometry. Clinical evidence of skeletal muscle dysfunction was minimal in 14 of the 15 patients; 10 of them had no gastrointestinal symptoms. Gastric emptying studies were performed by using 500 muCi of technetium 99m-sulfur colloid bound to a scrambled egg, and scintigraphic measurements were made continuously for 60 to 90 minutes. Gastric emptying studies and manometric tracings were compared with those from 11 children (mean age, 8.4 years) without gastrointestinal or muscular disorders. Mean (+/- SD) percentage retention of gastric isotope was significantly greater in patients with muscular dystrophy than in control subjects. No differences were found between the two groups in distal esophageal motility or in upper and lower esophageal sphincter pressures or relaxation. Contraction amplitudes in the upper portion of the esophagus, however, were significantly lower in patients with myopathy than in control subjects. These data suggest that dysfunction of smooth muscle of the upper gastrointestinal tract is detectable in children with muscular dystrophy early in the course of the disease, even when gastrointestinal symptoms are absent and skeletal muscle symptoms are minimal.