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Functional limitations in Romanian children with haemophilia: further testing of psychometric properties of the Paediatric Haemophilia Activities List

Authors

  • W. Groen,

    1. Child Development and Exercise Center, University Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
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  • J. van der Net,

    Corresponding author
    • Child Development and Exercise Center, University Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
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  • A. M. Lacatusu,

    1. Clinical Center for Evaluation and Rehabilitation “Cristian Serban”, Buzias, Romania
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  • M. Serban,

    1. Clinical Center for Evaluation and Rehabilitation “Cristian Serban”, Buzias, Romania
    2. University Emergency Children Hospital Louis Turcanu, Timisoara, Romania
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  • P. J. M. Helders,

    1. Child Development and Exercise Center, University Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
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  • K. Fischer

    1. Van Creveldkliniek, Department of Haematology, University Medical Center Utrecht, Utrecht, The Netherlands
    2. Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands
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Correspondence: Janjaap van der Net, Child Development and Exercise Center, University Children's Hospital, UMC Utrecht, Suite KB02.056.0, PO Box 85090, 3508 AB Utrecht, The Netherlands.

Tel.: +31 88 755 4030; fax: +31 88 755 5333;

e-mail: j.vandernet@umcutrecht.nl

Summary

Children with haemophilia often experience limitations in activities of daily life. Recently the Paediatric Haemophilia Activities List (PedHAL) has been developed and tested in Dutch children with intensive replacement therapy. The psychometric properties of the PedHAL in children not receiving intensive replacement therapy are not known. The objective was to gain further insight into the psychometric properties of the PedHAL and to study the functional health status of Romanian children and adolescents with haemophilia. Children attending to the rehabilitation centre of Buzias in Romania were sampled consecutively. Construct validity of the PedHAL was evaluated by concurrent testing with objective and subjective measures of physical function and functional ability. Reproducibility was tested by a 3-day test–retest by intraclass correlation coefficient (ICC) and limits of agreement (LOA). Responsiveness to rehabilitation was assessed by Haemophilia Joint Health Score (HJHS) and PedHAL. Twenty-nine children with severe (n = 25) or moderate (n = 4) haemophilia participated. Mean age was 13.2 years (SD 4.0). Median score of the PedHAL was 83.5 (IQR 47.9–90.5). The PedHAL correlated moderately with HJHS (rho = −0.59), Functional Independence Score in& Haemophilia (rho = 0.65) and Child Health Questionnaire-physical function (rho = 0.40) and not with Child Health Questionnaire-mental health, Child Health Questionnaire-behaviour and 6MWT. Test-retest reliability was good (ICC = 0.95). LOA was 17.4 points for the sum score. HJHS scores improved slightly after rehabilitation, whereas PedHAL scores did not change. In general, construct validity and test–retest reliability were good, test–retest agreement showed some variability. Therefore, currently the PedHAL may be more appropriate for research purposes than for individual patient monitoring in clinical practice.

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