Dataset Information

Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: Options for a shorter HJHS.

ABSTRACT:

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 HJHS_short , which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment.

SUBMITTER: Kuijlaars IAR

PROVIDER: S-EPMC7821332 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Publications

Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: Options for a shorter HJHS.

Kuijlaars Isolde A R IAR van der Net Janjaap J Feldman Brian M BM Aspdahl Magnus M Bladen Melanie M de Boer Wypke W Cuesta-Barriuso Rubén R Matlary Ruth E D RED Funk Sharon M SM Hilliard Pamela P John Judy A JA Kempton Christine L CL de Kleijn Piet P Manco-Johnson Marilyn M Petrini Pia P Poonnoose Pradeep P St-Louis Jean J Thomas Sylvia S Timmer Merel A MA Trakymiene Sonata Saulyte SS van Vlimmeren Leo L Fischer Kathelijn K

Haemophilia : the official journal of the World Federation of Hemophilia 20201015 6

<h4>Introduction</h4>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.<h4>Aim</h4>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 opi ...[more]

PMID: 33058441

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Dataset Information

Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: Options for a shorter HJHS.

Introduction

Aim

Methods

Results

Conclusion

Publications

Evaluating international Haemophilia Joint Health Score (HJHS) results combined with expert opinion: Options for a shorter HJHS.

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