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. 2020 Aug;40(7):357-360.
doi: 10.1097/BPO.0000000000001527.

Patient-reported Outcomes in Arthrogryposis

Lindley B Wall  1 , Carley Vuillerman  2 , Patricia E Miller  2 , Donald S Bae  2 , Charles A Goldfarb  1 ; CoULD Study Group
Affiliations

Patient-reported Outcomes in Arthrogryposis

Lindley B Wall et al. J Pediatr Orthop. 2020 Aug.

Abstract

Background: Little is known about patient-reported health status in children and adolescents with arthrogryposis. Utilizing the Patient-Reported Outcome Measurement Information System (PROMIS) and Pediatric Outcomes Data Collection Instrument (PODCI) questionnaires, we investigated functional and psychosocial measures in arthrogryposis.

Methods: A total of 118 patients with arthrogryposis were identified from a prospective longitudinal cohort (the Congenital Upper Limb Difference Registry) from 2014 to 2018. Demographics and patient-reported outcome measures were evaluated, including the PROMIS [upper extremity (UE) function, pain, depression, anxiety, and peer relations] and PODCI questionnaires (UE function, pain, happiness, and global function).

Results: A total of 29 arthrogrypotic patients had complete PROMIS and PODCI data. This cohort was divided into distal arthrogryposis and amyoplasia groups, with 15 and 14 patients in each group, respectively. There were 8 males in the distal arthrogryposis group with a median age of 9 years and 7 males in the amyoplasia group with a median age of 8 years. For both cohorts, the median UE function PROMIS scores were significantly below population norms, 31 for distal arthrogryposis and 22 for amyoplasia. PODCI UE function was statistically lower for amyoplasia compared with the distal arthrogryposis cohort. PROMIS pain, depression, anxiety, and peer relations were in the normal range for both amyopasia and distal arthrogryposis. Median PODCI pain and happiness ranged from 85 to 88 for all patients with no statistical difference between groups.

Conclusions: Arthrogryposis patients have lower UE function scores compared with population normals, but they have emotional states that are consistent with populations norms. Amyoplasia patients were functionally worse than distal arthrogryposis patients.

Levels of evidence: Level II.

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References

    1. Daltroy LH, Liang MH, Fossel AH, et al. The POSNA pediatric musculoskeletal functional health questionnaire: report on reliability, validity, and sensitivity to change. Pediatric Outcomes Instrument Development Group. Pediatric Orthopaedic Society of North America. J Pediatr Orthop. 1998;18:561–571.
    1. Cella D, Riley W, Stone A, et al. The Patient-Reported Outcomes Measurement Information System (PROMIS) developed and tested its first wave of adult self-reported health outcome item banks: 2005-2008. J Clin Epidemiol. 2010;63:1179–1194.
    1. Fries JF, Bruce B, Cella D. The promise of PROMIS: using item response theory to improve assessment of patient-reported outcomes. Clin Exp Rheumatol. 2005;23(5 suppl 39):S53–S57.
    1. DeWalt DA, Gross HE, Gipson DS, et al. PROMIS® pediatric self-report scales distinguish subgroups of children within and across six common pediatric chronic health conditions. Qual Life Res. 2015;24:2195–2208.
    1. Howell CR, Thompson LA, Gross HE, et al. Responsiveness to change in PROMIS® measures among children with asthma: a report from the PROMIS® pediatric asthma study. Value Health. 2016;19:192–201.