Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2
dc.contributor.author | Ferner, RE | |
dc.contributor.author | Shaw, A | |
dc.contributor.author | Evans, DG | |
dc.contributor.author | McAleer, D | |
dc.contributor.author | Halliday, D | |
dc.contributor.author | Parry, A | |
dc.contributor.author | Raymond, FL | |
dc.contributor.author | Durie-Gair, J | |
dc.contributor.author | Hanemann, Clemens Oliver | |
dc.contributor.author | Hornigold, R | |
dc.contributor.author | Axon, P | |
dc.contributor.author | Golding, JF | |
dc.date.accessioned | 2015-10-06T13:24:47Z | |
dc.date.available | 2015-10-06T13:24:47Z | |
dc.date.issued | 2014-05 | |
dc.identifier.issn | 0340-5354 | |
dc.identifier.issn | 1432-1459 | |
dc.identifier.uri | http://hdl.handle.net/10026.1/3574 | |
dc.description.abstract |
Advances in molecular biology have resulted in novel therapy for neurofibromatosis 2-related (NF2) tumours, highlighting the need for robust outcome measures. The disease-focused NF2 impact on quality of life (NFTI-QOL) patient questionnaire was assessed as an outcome measure for treatment in a multi-centre study. NFTI-QOL was related to clinician-rated severity (ClinSev) and genetic severity (GenSev) over repeated visits. Data were evaluated for 288 NF2 patients (n = 464 visits) attending the English national NF2 clinics from 2010 to 2012. The male-to-female ratio was equal and the mean age was 42.2 (SD 17.8) years. The analysis included NFTI-QOL eight-item score, ClinSev graded as mild, moderate, or severe, and GenSev as a rank order of the number of NF2 mutations (graded as mild, moderate, severe). The mean (SD) 8.7 (5.4) score for NFTI-QOL for either a first visit or all visits 9.2 (5.4) was similar to the published norm of 9.4 (5.5), with no significant relationships with age or gender. NFTI-QOL internal reliability was good, with a Cronbach's alpha score of 0.85 and test re-test reliability r = 0.84. NFTI related to ClinSev (r = 0.41, p < 0.001; r = 0.46 for all visits), but weakly to GenSev (r = 0.16, p < 0.05; r = 0.15 for all visits). ClinSev related to GenSev (r = 0.41, p < 0.001; r = 0.42 for all visits). NFTI-QOL showed a good reliability and ability to detect significant longitudinal changes in the QOL of individuals. The moderate relationships of NFTI-QOL with clinician- and genetic-rated severity suggest that NFTI-QOL taps into NF2 patient experiences that are not encompassed by ClinSev rating or genotype. | |
dc.format.extent | 963-969 | |
dc.format.medium | Print-Electronic | |
dc.language | en | |
dc.language.iso | eng | |
dc.publisher | Springer Science and Business Media LLC | |
dc.subject | Neurofibromatosis 2 | |
dc.subject | NF2 | |
dc.subject | NFTI-QOL | |
dc.subject | Vestibular schwannoma | |
dc.title | Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2 | |
dc.type | journal-article | |
dc.type | Evaluation Study | |
dc.type | Journal Article | |
dc.type | Multicenter Study | |
dc.type | Research Support, Non-U.S. Gov't | |
plymouth.author-url | https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000335772500015&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=11bb513d99f797142bcfeffcc58ea008 | |
plymouth.issue | 5 | |
plymouth.volume | 261 | |
plymouth.publication-status | Published | |
plymouth.journal | Journal of Neurology | |
dc.identifier.doi | 10.1007/s00415-014-7303-1 | |
plymouth.organisational-group | /Plymouth | |
plymouth.organisational-group | /Plymouth/Faculty of Health | |
plymouth.organisational-group | /Plymouth/Faculty of Health/Peninsula Medical School | |
plymouth.organisational-group | /Plymouth/REF 2021 Researchers by UoA | |
plymouth.organisational-group | /Plymouth/REF 2021 Researchers by UoA/UoA01 Clinical Medicine | |
plymouth.organisational-group | /Plymouth/Research Groups | |
plymouth.organisational-group | /Plymouth/Research Groups/Institute of Translational and Stratified Medicine (ITSMED) | |
plymouth.organisational-group | /Plymouth/Research Groups/Institute of Translational and Stratified Medicine (ITSMED)/CBR | |
plymouth.organisational-group | /Plymouth/Users by role | |
plymouth.organisational-group | /Plymouth/Users by role/Academics | |
plymouth.organisational-group | /Plymouth/Users by role/Researchers in ResearchFish submission | |
dc.publisher.place | Germany | |
dcterms.dateAccepted | 2014-02-25 | |
dc.identifier.eissn | 1432-1459 | |
dc.rights.embargoperiod | Not known | |
rioxxterms.versionofrecord | 10.1007/s00415-014-7303-1 | |
rioxxterms.licenseref.uri | http://www.rioxx.net/licenses/all-rights-reserved | |
rioxxterms.licenseref.startdate | 2014-05 | |
rioxxterms.type | Journal Article/Review |