TY - JOUR
T1 - Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion
T2 - Options for a shorter HJHS
AU - Kuijlaars, Isolde A R
AU - van der Net, Janjaap
AU - Feldman, Brian M
AU - Aspdahl, Magnus
AU - Bladen, Melanie
AU - de Boer, Wypke
AU - Cuesta-Barriuso, Rubén
AU - Matlary, Ruth E D
AU - Funk, Sharon M
AU - Hilliard, Pamela
AU - John, Judy A
AU - Kempton, Christine L
AU - de Kleijn, Piet
AU - Manco-Johnson, Marilyn
AU - Petrini, Pia
AU - Poonnoose, Pradeep
AU - St-Louis, Jean
AU - Thomas, Sylvia
AU - Timmer, Merel A
AU - Trakymiene, Sonata Saulyte
AU - van Vlimmeren, Leo
AU - Fischer, Kathelijn
N1 - © 2020 The Authors. Haemophilia published by John Wiley & Sons Ltd.
PY - 2020/11
Y1 - 2020/11
N2 - INTRODUCTION: The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption.AIM: This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real-life data and expert opinion.METHODS: A cross-sectional multicenter secondary analysis on pooled data of published studies using the HJHS (0-124, optimum score 0) in persons with haemophilia A/B aged 4-30 was performed. Least informative items, scoring options and/or joints were identified. An expert group of 19 international multidisciplinary experts evaluated the results and voted on suggestions for adaptations in a structured meeting (consensus set at ≥ 80%).RESULTS: Original data on 499 persons with haemophilia from 7 studies were evaluated. Median age was 15.0 years [range 4.0-29.9], 83.2% had severe haemophilia and 61.5% received prophylaxis. Median (IQR) HJHS total was 6.0 (1.0-17.0). The items 'duration swelling' and 'crepitus' were identified as clinically less informative and appointed as candidates for reduction.CONCLUSION: Analysis of 499 children and young adults with haemophilia showed that the HJHS is able to discriminate between children and adults and different treatment regimens. Reduction of the items 'duration swelling' and 'crepitus' resulted in the HJHSshort , which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment.
AB - INTRODUCTION: The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption.AIM: This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real-life data and expert opinion.METHODS: A cross-sectional multicenter secondary analysis on pooled data of published studies using the HJHS (0-124, optimum score 0) in persons with haemophilia A/B aged 4-30 was performed. Least informative items, scoring options and/or joints were identified. An expert group of 19 international multidisciplinary experts evaluated the results and voted on suggestions for adaptations in a structured meeting (consensus set at ≥ 80%).RESULTS: Original data on 499 persons with haemophilia from 7 studies were evaluated. Median age was 15.0 years [range 4.0-29.9], 83.2% had severe haemophilia and 61.5% received prophylaxis. Median (IQR) HJHS total was 6.0 (1.0-17.0). The items 'duration swelling' and 'crepitus' were identified as clinically less informative and appointed as candidates for reduction.CONCLUSION: Analysis of 499 children and young adults with haemophilia showed that the HJHS is able to discriminate between children and adults and different treatment regimens. Reduction of the items 'duration swelling' and 'crepitus' resulted in the HJHSshort , which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment.
KW - expert opinion
KW - haemophilia
KW - joints
KW - outcome measures
KW - physical examination
UR - http://www.scopus.com/inward/record.url?scp=85092482524&partnerID=8YFLogxK
U2 - https://doi.org/10.1111/hae.14180
DO - https://doi.org/10.1111/hae.14180
M3 - Article
C2 - 33058441
SN - 1351-8216
VL - 26
SP - 1072
EP - 1080
JO - Haemophilia
JF - Haemophilia
IS - 6
ER -