title: Development, validation, and evaluation of the PBC-40, a disease specific health related quality of life measure for primary biliary cirrhosis.
abstract: OBJECTIVE
  Study of health related quality of life (HRQOL) and the factors responsible for its impairment in primary biliary cirrhosis (PBC) has, to date, been limited. There is increasing need for a HRQOL questionnaire which is specific to PBC. The aim of this study was to develop, validate, and evaluate a patient based PBC specific HRQOL measure.
  
  
  METHODS
  A pool of potential questions was derived from thematic analysis of indepth interviews carried out with 30 PBC patients selected to represent demographically the PBC patient population as a whole. This pool was systematically reduced, pretested, and cross validated with other HRQOL measures in national surveys involving a total of 900 PBC patients, to produce a quality of life profile measure, the PBC-40, consisting of 40 questions distributed across six domains. The PBC-40 was then evaluated in a blinded comparison with other HRQOL measures in a further cohort of 40 PBC patients.
  
  
  RESULTS
  The six domains of PBC-40 relate to fatigue, emotional, social, and cognitive function, general symptoms, and itch. The highest mean domain score was seen for fatigue and the lowest for itch. The measure has been fully validated for use in PBC and shown to be scientifically sound. PBC patient satisfaction, measured in terms of the extent to which a questionnaire addresses the problems that they experience, was significantly higher for the PBC-40 than for other HRQOL measures.
  
  
  CONCLUSIONS
  The PBC-40 is a short easy to complete measure which is acceptable to PBC patients and has significantly greater relevance to their problems than other frequently used HRQOL measures. Its scientific soundness, shown in extensive testing, makes it a valuable instrument for future use in clinical and research settings.
authors.affiliation: Division of Public Health, University of Liverpool, Whelan Building, The Quadrangle, Brownlow Hill, Liverpool L69 3GB, UK. ajacoby@liv.ac.uk
authors.forename: A
authors.initials: A
authors.lastname: Jacoby
authors.forename: A
authors.initials: A
authors.lastname: Rannard
authors.forename: D
authors.initials: D
authors.lastname: Buck
authors.forename: N
authors.initials: N
authors.lastname: Bhala
authors.forename: J L
authors.initials: JL
authors.lastname: Newton
authors.forename: O F W
authors.initials: OF
authors.lastname: James
authors.forename: D E J
authors.initials: DE
authors.lastname: Jones
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006305:Health Status Indicators; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000523:psychology / Q000534:rehabilitation*; D008297:Male; D008875:Middle Aged; D011594:Psychometrics; D011788:Quality of Life; D015203:Reproducibility of Results; D011795:Surveys and Questionnaires