Issn Print: 0277-2116
Publication Date: 1996/05/01
53 GASTROINTESTINAL MOTILITY IN CHILDREN WITH MUSCULAR DYSTROPHY
A. Staiano; E. Del Giudice; G. Marsullo; D. Simeone; A. Marino; C. Villano; L. Santoro
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Excerpt
There is increasing evidence that progressive muscular dystrophy (MD) may involve gastrointestinal (GI) smooth muscle. We evaluated GI motility in 29 children with progressive MD (mean age: 8.4 yrs) to detect early GI smooth muscle involvement even when overt, symptomatic skeletal muscle disease was absent. Diagnosis of MD was based on clinical, electromyographic and muscle biopsy findings. On the basis of the dystrophin test, we segregated the subjects by type of MD into the following subgroups: Duchenne MD in 15 children, Becker MD in 8, and nonspecific progressive MD in 6. Fifteen of the patients had no GI symptoms. In all children we evaluated scintigraphic gastric emptying of solid food and bowel frequency for one week. Total gastrointestinal transit time (TGTT), using radipaque markers, was evaluated in 25 patients; 15 also underwent anorcctal and esophageal manometry, using constantly perfused multilumen catheters. The results of each study were compared with those from a group of 11 age- and sex-matched control children. RESULTS: Gastric emptying was significantly (p<0.01) prolonged over the control values in 25 (86%) MD children. In comparison with the controls, weekly bowel frequency was significantly (p<0.01) decreased in 6 (21%) MD children, and TGTT was significantly (p<0.01) prolonged in 17 (59%) MD children. Furthermore, in MD children, contraction amplitudes in the upper portion of the esophagus and anal squeeze pressures were significantly lower than similar values determined in the controls (p<0.05 for each comparison). No differences were found among the types of progressive MD in the degree of GI motility abnormalities. CONCLUSIONS: Children with progressive MD show a multitude of GI motility abnormalities consistent with expression of smooth muscle weakness. These abnormalities are subclinical in many patients and can be detected early in the course of the disease, even when skeletal muscle involvement is not evident.
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