title: Muscle ultrasound quantifies disease progression over time in infants and young boys with duchenne muscular dystrophy.
abstract: BACKGROUND
  Quantitative muscle ultrasound (QUS) in boys with Duchenne muscular dystrophy (DMD) shows increased echointensity as muscle is replaced with fat and fibrosis. Studies of quantitative ultrasound in infants/young boys with DMD over time have not been reported.
  
  
  METHODS
  We used calibrated muscle backscatter (cMB), a reproducible measure of ultrasound echointensity, to quantify muscle pathology in 5 young boys with DMD (ages 0.5-2.8 years) over 17-29 months. We compared the results with repeated assessments of function (n = 4) and with muscle ultrasound images from a cross-section of 6 male controls (0.6-3.1 years).
  
  
  RESULTS
  cMB in boys with DMD increased (worsened) over time (P < 0.001), whereas function improved. After age 2 years, cMB in most (4 of 5) boys with DMD was higher than in any control.
  
  
  CONCLUSIONS
  QUS measures disease progression in young boys with DMD despite functional improvements. QUS could be employed as an outcome measure for serial assessment of young boys with DMD.
authors.affiliation: Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8111, St. Louis, Missouri, 63110, USA.|Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: Craig M
authors.initials: CM
authors.lastname: Zaidman
authors.affiliation: Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8111, St. Louis, Missouri, 63110, USA.
authors.forename: Elizabeth C
authors.initials: EC
authors.lastname: Malkus
authors.affiliation: Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, Box 8111, St. Louis, Missouri, 63110, USA.|Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
authors.forename: Anne M
authors.initials: AM
authors.lastname: Connolly
mesh_terms: D001132:Arm; D016022:Case-Control Studies; D002675:Child, Preschool; D018450:Disease Progression; D006801:Humans; D007223:Infant; D008297:Male; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000000981:diagnostic imaging* / Q000503:physiopathology; D052097:Quadriceps Muscle / Q000000981:diagnostic imaging* / Q000503:physiopathology; D014463:Ultrasonography
keywords: Duchenne muscular dystrophy; backscatter; children; muscle; myopathy; ultrasound