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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
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Gastrointestinal Dysfunction in Patients with Duchenne Muscular Dystrophy
Christian M Lo Cascio1,2, Oliver Goetze3,4, Tsogyal D Latshang1,2
1Pulmonary Division, University Hospital of Zurich, Neuromuscular and Sleep Disorders Center, University Hospital of Zurich, Zurich, Switzerland.
Plos One
|October 14, 2016
Summary
Gastrointestinal transit is significantly delayed in Duchenne muscular dystrophy (DMD) patients, even when they don't report symptoms. Proactive measures are crucial to prevent severe constipation in DMD individuals.
Area of Science:
- Gastroenterology
- Neuromuscular Disorders
- Clinical Physiology
Background:
- Duchenne muscular dystrophy (DMD) patients experience life-threatening constipation.
- Gastrointestinal function in DMD has not been extensively studied.
Purpose of the Study:
- To investigate objective and subjective gastrointestinal disturbances in adult DMD patients.
- To evaluate gastric emptying, oro-cecal transit, and colonic transit times.
- To correlate objective measures with patient-reported symptoms.
Main Methods:
- 33 adult DMD patients (age 12-41) completed questionnaires on eating behavior and GI symptoms.
- Gastric emptying (T1/2) and oro-cecal transit time (OCTT) measured using 13C-labeled test meals and 13CO2 exhalation.
- Colonic transit time (CTT) assessed via abdominal radiography with radiopaque markers.
Main Results:
- Significantly prolonged T1/2 (187 min) and OCTT (6.3 hrs) compared to normal values.
- Markedly prolonged CTT (60 hrs) despite laxative use.
- No correlation found between objective GI transit measures (T1/2, OCTT, CTT) and patient-reported symptoms (GCSI, ROME III).
Conclusions:
- DMD patients exhibit significantly impaired gastrointestinal motor function.
- Objective measures of GI transit impairment do not correlate with subjective symptoms.
- Proactive interventions for intestinal transport are essential, irrespective of patient perception, to prevent severe constipation in DMD.
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