PMID- 24316419
OWN - NLM
STAT- MEDLINE
DCOM- 20141021
LR  - 20211021
IS  - 1873-2763 (Electronic)
IS  - 8756-3282 (Print)
IS  - 1873-2763 (Linking)
VI  - 60
DP  - 2014 Mar
TI  - Disease severity and functional factors associated with walking performance in 
      polyostotic fibrous dysplasia.
PG  - 41-7
LID - S8756-3282(13)00484-5 [pii]
LID - 10.1016/j.bone.2013.11.022 [doi]
AB  - The purpose of this study was to determine the association between measures of 
      disease severity, impairment, and ambulation ability in persons with polyostotic 
      fibrous dysplasia (PFD). A cross-sectional sample of 81 patients (ages 5-57) with 
      polyostotic fibrous dysplasia was evaluated as part of an ongoing study. Subjects 
      were scored on the Skeletal Disease Burden Score (SDBS), completed a 9-minute 
      walk test (9MW), manual muscle testing (MMT), and measurements of range of motion 
      (ROM). Correlations between continuous variables were calculated using the 
      Pearson correlation coefficient and ordinal variables by Spearman correlation 
      coefficient. It was found that subjects with more severe disease walked slower 
      than those with less skeletal disease, with the exception of the youngest 
      subjects. Walking velocity was faster in subjects with better hip strength and 
      range of motion and slower in those with bilateral coxa vara. Those subjects with 
      more severe disease had less range of motion, were weaker at the hips, and more 
      likely to have leg length discrepancy. Skeletal disease severity was associated 
      with hip weakness, leg length discrepancy, and loss of range of motion. In most 
      cases, findings did not differ in the presence or absence of associated 
      endocrinopathies. Skeletal disease severity, MMT and ROM each has an impact on 
      walking efficiency in persons with PFD. These findings suggest that treatment 
      focused on strategies to improve or, at least, maintain hip strength and range of 
      motion, correct leg length discrepancies and hip malalignment may help preserve 
      ambulation ability in persons with PFD and that treatment should begin at a young 
      age.
CI  - Published by Elsevier Inc.
FAU - Paul, Scott M
AU  - Paul SM
AD  - Rehabilitation Medicine Department, Clinical Center, National Institutes of 
      Health, Bethesda, MD, USA. Electronic address: spaul@cc.nih.gov.
FAU - Gabor, Lisa R
AU  - Gabor LR
AD  - Rehabilitation Medicine Department, Clinical Center, National Institutes of 
      Health, Bethesda, MD, USA. Electronic address: lisa.gabor@gmail.com.
FAU - Rudzinski, Scott
AU  - Rudzinski S
AD  - Department of Radiology, University of Pittsburgh, Pittsburgh, PA, USA. 
      Electronic address: rudzinskism@upmc.edu.
FAU - Giovanni, David
AU  - Giovanni D
AD  - PRMC Wound Care Center, Salisbury, MD, USA. Electronic address: 
      giovannidavidmd@msn.com.
FAU - Boyce, Alison M
AU  - Boyce AM
AD  - Craniofacial and Skeletal Diseases Branch, National Institute of Dental and 
      Craniofacial Research, National Institutes of Health, Bethesda, MD, USA; Division 
      of Endocrinology and Diabetes and Bone Health Program, Division of Orthopaedics 
      and Sports Medicine, Children's National Medical Center, Washington, DC, USA. 
      Electronic address: boyceam@nidcr.nih.gov.
FAU - Kelly, Marilyn R N
AU  - Kelly MR
AD  - Craniofacial and Skeletal Diseases Branch, National Institute of Dental and 
      Craniofacial Research, National Institutes of Health, Bethesda, MD, USA. 
      Electronic address: marilynhkelly@gmail.com.
FAU - Collins, Michael T
AU  - Collins MT
AD  - Craniofacial and Skeletal Diseases Branch, National Institute of Dental and 
      Craniofacial Research, National Institutes of Health, Bethesda, MD, USA. 
      Electronic address: mc247k@nih.gov.
LA  - eng
GR  - Z99 CL999999/Intramural NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Intramural
DEP - 20131204
PL  - United States
TA  - Bone
JT  - Bone
JID - 8504048
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Demography
MH  - Female
MH  - Fibrous Dysplasia, Polyostotic/*pathology/*physiopathology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Regression Analysis
MH  - *Severity of Illness Index
MH  - Walking/*physiology
MH  - Young Adult
PMC - PMC3985279
MID - NIHMS552121
OTO - NOTNLM
OT  - Ambulation
OT  - Functional measures
OT  - McCune-Albright Syndrome
OT  - Polyostotic fibrous dysplasia
COIS- Conflicts of interest The authors have no conflicts to report.
EDAT- 2013/12/10 06:00
MHDA- 2014/10/22 06:00
PMCR- 2015/03/01
CRDT- 2013/12/10 06:00
PHST- 2013/04/25 00:00 [received]
PHST- 2013/11/21 00:00 [revised]
PHST- 2013/11/22 00:00 [accepted]
PHST- 2013/12/10 06:00 [entrez]
PHST- 2013/12/10 06:00 [pubmed]
PHST- 2014/10/22 06:00 [medline]
PHST- 2015/03/01 00:00 [pmc-release]
AID - S8756-3282(13)00484-5 [pii]
AID - 10.1016/j.bone.2013.11.022 [doi]
PST - ppublish
SO  - Bone. 2014 Mar;60:41-7. doi: 10.1016/j.bone.2013.11.022. Epub 2013 Dec 4.