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Measuring Physical Function Capacity in Persons With Haemophilia: A Systematic Review of Performance-Based Methods

IF 3 2区医学 Q2 HEMATOLOGY

Haemophilia Pub Date : 2025-07-20 DOI:10.1111/hae.70081

Catherine Holdsworth, Melanie Bladen, Hannah Harbidge, Wendy Drechsler, Ryan Mahaffey, Sofia Perez-Alenda, Fionnuala Sayers, Karen Strike, Merel Timmer, David Stephensen

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The aim of the systematic review was to review and apply standardised criteria to evaluate reliability, responsiveness and construct validity of performance-based instruments evaluating physical capability in persons with haemophilia.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>Medline, CINAHL, Embase, EMCARE, and Cochrane (inception-March 2024) were searched using COSMIN filters for 7 performance-based tests in haemophilia, supplemented by manual searches. Reliability, responsiveness and construct validity of the six-minute walk test (6MWT), timed up and go test (TUG), timed up and down stairs (TUDS), 30-second sit-to-stand (30-STS), single leg stance (SLS), tandem stance (TS) and single hop for distance (SH) were evaluated.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>The search yielded 88 abstracts; 25 studies remained after full-text screening, covering 5 of 7 performance-based instruments: 6MWT, TUG, TUDS, SLS, and 30-STS. 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Abstract

Introduction

Currently, physical health assessments in persons with haemophilia focus on bleed-related events and after-effects. The aim of the systematic review was to review and apply standardised criteria to evaluate reliability, responsiveness and construct validity of performance-based instruments evaluating physical capability in persons with haemophilia.

Methods

Medline, CINAHL, Embase, EMCARE, and Cochrane (inception-March 2024) were searched using COSMIN filters for 7 performance-based tests in haemophilia, supplemented by manual searches. Reliability, responsiveness and construct validity of the six-minute walk test (6MWT), timed up and go test (TUG), timed up and down stairs (TUDS), 30-second sit-to-stand (30-STS), single leg stance (SLS), tandem stance (TS) and single hop for distance (SH) were evaluated.

Results

The search yielded 88 abstracts; 25 studies remained after full-text screening, covering 5 of 7 performance-based instruments: 6MWT, TUG, TUDS, SLS, and 30-STS. No performance-based test was evaluated for all properties across all ages. Only TUG in adults and older adults and 6MWT in children and adolescents has been tested for all properties. No test received a high grading. Low and very low grades were given mostly for indeterminate results, small or single studies and lack of a similar construct of comparator. The 6MWT in all age groups was the only performance-based test graded moderate, and this was for responsiveness.

Conclusion

With increasing use of performance-based methods of physical function capacity, evaluating measurement properties is a priority. Until evidence is generated, we can only advocate the 6MWT to monitor responsiveness in adult persons with haemophilia affected with marked arthropathy.

Summary

Understanding Physical Health in People with Haemophilia

Currently, when we check the physical health of people with haemophilia, we mostly look at problems caused by bleeding. But we wanted to see if there are better ways to measure how well people with haemophilia can move and do daily activities.

What We Did

We looked through a lot of medical studies (up to March 2024) to find information on 7 specific physical tests. These tests measure things like:

How far someone can walk in six minutes (6-minute walk test or 6MWT)
How long it takes to stand up, walk a short distance, and sit down (Timed Up and Go test or TUG)
How long it takes to go up and down stairs (timed up and down stairs or TUDS)
How many times someone can stand up from a chair in 30 seconds (30-second sit-to-stand or 30-STS)
How long someone can stand on one leg (single leg stance or SLS)
How long someone can stand with one foot directly in front of the other (Tandem Stance or TS)
How far someone can hop on one leg (single hop for distance or SH)

We wanted to see how reliable (consistent), responsive (can detect changes), and valid (measures what it's supposed to) these tests were for people with haemophilia.

What We Found

We found 25 studies that looked at 5 of the 7 tests (6MWT, TUG, TUDS, SLS, and 30-STS).

Here's what stood out:

No single test was good for everything and for all ages.
Only the TUG test (for adults) and the 6MWT (for children) had been fully studied for all aspects (reliability, responsiveness, and validity).
None of the tests were rated as highly effective overall. Most got low ratings because the results weren't clear, studies were small, or there wasn't enough good information to compare them to.
The 6MWT was the only test that received a ‘moderate‘ rating, but only for its ability to show changes in all age groups.

What This Means

It's becoming more common to use these kinds of physical tests to understand how well people function. So, it's very important to know if these tests work well for people with haemophilia.

For now, based on the evidence, we can only suggest using the 6-minute walk test (6MWT) to track how much someone's physical ability changes over time, especially for adults with haemophilia who have severe joint problems. We need more research to find better tests.

Abstract Image

查看原文本刊更多论文

测量血友病患者的身体功能能力：基于性能的方法的系统回顾。

目前，血友病患者的身体健康评估侧重于出血相关事件和后遗症。系统评价的目的是审查和应用标准化标准来评估血友病患者身体能力评估的可靠性、反应性和构建效度。方法：使用COSMIN筛选器检索Medline、CINAHL、Embase、EMCARE和Cochrane（初刊- 2024年3月）的7项血友病性能检测，并辅以人工检索。对6分钟步行测验（6MWT）、定时上下楼梯测验（TUG）、定时上下楼梯测验（TUDS）、30秒坐立测验（30-STS）、单腿站立测验（SLS）、串联站立测验（TS）和单跳距离测验（SH）进行信度、反应性和结构效度评估。结果：检索到88篇摘要；全文筛选后仍有25项研究，涵盖了7种基于性能的仪器中的5种：6MWT、TUG、TUDS、SLS和30-STS。没有基于性能的测试对所有年龄段的所有属性进行评估。只有成人和老年人的TUG和儿童和青少年的6MWT进行了所有性质的测试。没有测试获得高分。低和极低的评分主要是由于不确定的结果，小型或单一的研究和缺乏类似的比较物结构。在所有年龄组中，6MWT是唯一被评为中度的基于表现的测试，这是针对反应性的。结论：随着基于性能的身体功能能力测量方法的使用越来越多，评估测量性能是一个优先考虑的问题。在证据产生之前，我们只能提倡6MWT监测成人血友病伴明显关节病患者的反应性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Haemophilia 医学-血液学

CiteScore

6.50

自引率

28.20%

发文量

226

审稿时长

3-6 weeks

期刊介绍： Haemophilia is an international journal dedicated to the exchange of information regarding the comprehensive care of haemophilia. The Journal contains review articles, original scientific papers and case reports related to haemophilia care, with frequent supplements. Subjects covered include: clotting factor deficiencies, both inherited and acquired: haemophilia A, B, von Willebrand''s disease, deficiencies of factor V, VII, X and XI replacement therapy for clotting factor deficiencies component therapy in the developing world transfusion transmitted disease haemophilia care and paediatrics, orthopaedics, gynaecology and obstetrics nursing laboratory diagnosis carrier detection psycho-social concerns economic issues audit inherited platelet disorders.