title: Prednisone and Deflazacort in Duchenne Muscular Dystrophy: Do They Play a Different Role in Child Behavior and Perceived Quality of Life?
abstract: The aim of this study was to determine whether prednisone and deflazacort play a different role in child behavior and perceived health related psychosocial quality of life in ambulant boys with Duchenne Muscular Dystrophy. As part of a prospective natural-history study, parents of sixty-seven ambulant boys with DMD (27 taking prednisone, 15 taking deflazacort, 25 were steroid naïve) completed the Child Behavior Checklist (CBCL) for assessment of behavioral, emotional and social problems and both parents and boys with DMD completed the PedsQL™4.0 generic core scale short form. Boys with DMD had higher rates of general behavioral problems than age-matched peers. No significant differences were found among the groups for any of the CBCL syndrome scales raw scores, including internalizing and externalizing behaviors; however, on average boys taking deflazacort demonstrated more withdrawn behaviors than those taking prednisone, while on average the boys taking prednisone demonstrated more aggressive behaviors than boys taking deflazacort. Age, internalizing and externalizing behaviors accounted for 39 and 48% of the variance in psychosocial quality of life for both parents and boys with DMD, respectively. Overall, the use of steroids was not associated with more behavioral problems in boys with DMD. As behavior played a significant role in psychosocial quality of life, comprehensive assessment and treatment of behavioral problems is crucial in this population.
authors.affiliation: Clinical Research, Shriners Hospitals for Children, Portland, Oregon, USA.
authors.forename: Susan
authors.initials: S
authors.lastname: Sienko
authors.affiliation: Clinical Research, Shriners Hospitals for Children, Portland, Oregon, USA.
authors.forename: Cathleen
authors.initials: C
authors.lastname: Buckon
authors.affiliation: Orthopaedic Surgery, University of California, Los Angeles, California, USA.
authors.forename: Eileen
authors.initials: E
authors.lastname: Fowler
authors.affiliation: Motion Analysis Laboratory, Shriners Hospitals for Children Northern California, Sacramento, California, USA.
authors.forename: Anita
authors.initials: A
authors.lastname: Bagley
authors.affiliation: Orthopaedic Surgery, University of California, Los Angeles, California, USA.
authors.forename: Loretta
authors.initials: L
authors.lastname: Staudt
authors.affiliation: Center for HealthCare Quality Licensing & Certification Division, California Department of Public Health, Sacramento, California, USA.
authors.forename: Mitell
authors.initials: M
authors.lastname: Sison-Williamson
authors.affiliation: Shriners Hospitals for Children, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Kathy
authors.initials: K
authors.lastname: Zebracki
authors.affiliation: Department of Physical Medicine and Rehabilitation, University of California, Davis, Sacramento, California, USA.
authors.forename: Craig M
authors.initials: CM
authors.lastname: McDonald
authors.affiliation: Shriners Hospitals for Children, Portland, Oregon, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Sussman