title: Gastrointestinal Dysfunction in Patients with Duchenne Muscular Dystrophy.
abstract: BACKGROUND
  In adult patients with Duchenne muscular dystrophy (DMD) life-threatening constipation has been reported. Since gastrointestinal function in DMD has not been rigorously studied we investigated objective and subjective manifestations of gastrointestinal disturbances in DMD patients.
  
  
  METHODS
  In 33 patients with DMD, age 12-41 years, eating behavior and gastrointestinal symptoms were evaluated by questionnaires. Gastric emptying half time (T1/2) and oro-cecal transit time (OCTT) were evaluated by analyzing 13CO2 exhalation curves after ingestion of 13C labeled test meals. Colonic transit time (CTT) was measured by abdominal radiography following ingestion of radiopaque markers.
  
  
  RESULTS
  The median (quartiles) T1/2 was 187 (168, 220) minutes, the OCTT was 6.3 (5.0, 7.9) hours, both substantially longer than normal data (Goetze 2005, T1/2: 107±10; Geypens 1999, OCTT 4.3±0.1 hours). The median CTT was 60 (48, 82) hours despite extensive use of laxative measures (Meier 1995, upper limit of normal: 60 hours). T1/2 and OCTT did not correlate with symptoms evaluated by the Gastroparesis Cardinal Symptom Index (GCSI) (Spearman r = -0.3, p = 0.1; and r = -0.15, p = 0.4, respectively). CTT was not correlated with symptoms of constipation assessed by ROME III criteria (r = 0.12, p = 0.5).
  
  
  CONCLUSIONS
  DMD patients have a markedly disturbed gastrointestinal motor function. Since objective measures of impaired gastrointestinal transport are not correlated with symptoms of gastroparesis or constipation our findings suggest that measures assuring adequate intestinal transport should be taken independent of the patient's perception in order to prevent potentially life threatening constipation, particularly in older DMD patients.
authors.affiliation: Pulmonary Division, University Hospital of Zurich, Neuromuscular and Sleep Disorders Center, University Hospital of Zurich, Zurich, Switzerland.|Center for Human Integrative Physiology, University of Zurich, Zurich, Switzerland.
authors.forename: Christian M
authors.identifier: http://orcid.org/0000-0002-2163-4219
authors.initials: CM
authors.lastname: Lo Cascio
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital of Zurich, Zurich, Switzerland.|Division of Hepatology, Department of Medicine II, University Hospital Würzburg, Würzburg, Germany.
authors.forename: Oliver
authors.initials: O
authors.lastname: Goetze
authors.affiliation: Pulmonary Division, University Hospital of Zurich, Neuromuscular and Sleep Disorders Center, University Hospital of Zurich, Zurich, Switzerland.|Center for Human Integrative Physiology, University of Zurich, Zurich, Switzerland.
authors.forename: Tsogyal D
authors.initials: TD
authors.lastname: Latshang
authors.affiliation: Division of Gastroenterology and Hepatology, University Hospital of Zurich, Zurich, Switzerland.
authors.forename: Sena
authors.initials: S
authors.lastname: Bluemel
authors.affiliation: Institute of Diagnostic and Interventional Radiology, University Hospital of Zurich, Zurich, Switzerland.
authors.forename: Thomas
authors.initials: T
authors.lastname: Frauenfelder
authors.affiliation: Pulmonary Division, University Hospital of Zurich, Neuromuscular and Sleep Disorders Center, University Hospital of Zurich, Zurich, Switzerland.|Center for Human Integrative Physiology, University of Zurich, Zurich, Switzerland.
authors.forename: Konrad E
authors.initials: KE
authors.lastname: Bloch
mesh_terms: D000293:Adolescent; D000328:Adult; D002432:Cecum / Q000503:physiopathology; D002648:Child; D003106:Colon / Q000503:physiopathology; D003248:Constipation / Q000209:etiology* / Q000503:physiopathology*; D005260:Female; D005746:Gastric Emptying; D005767:Gastrointestinal Diseases / Q000209:etiology / Q000503:physiopathology; D005769:Gastrointestinal Motility; D041981:Gastrointestinal Tract / Q000503:physiopathology*; D005772:Gastrointestinal Transit; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000503:physiopathology*; D013270:Stomach / Q000503:physiopathology; D055815:Young Adult