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Haemophilia. 2016 Mar;22(2):218-224. doi: 10.1111/hae.12792. Epub 2015 Aug 28.

Early prophylaxis in children with severe haemophilia A: clinical and ultrasound imaging outcomes.

Haemophilia : the official journal of the World Federation of Hemophilia

C Altisent, M Martorell, A Crespo, L Casas, C Torrents, R Parra

Affiliations

  1. Haemophilia Centre, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
  2. Department of Rehabilitation, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
  3. Department of Radiology,, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

PMID: 26315845 DOI: 10.1111/hae.12792

Abstract

AIM: This observational study was undertaken with the aim to describe the characteristics and evaluate the outcomes of prophylactic treatment in children with severe haemophilia A (HA) treated at our centre.

METHODS: Twenty-five patients aged 4-19 years with severe HA, no history of inhibitors and treated with at least two infusions of factor VIII (FVIII) per week were studied. Prophylactic doses and annual joint bleeding rate (AJBR) were retrospectively evaluated over the last 5 years. Current joint status was assessed using the Haemophilia Joint Health Score (HJHS) (136 joints of 23 patients) and the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) procedure (124 joints of 21 patients).

RESULTS: Median AJBR was 0.2 and median prophylaxis dose 65.4 IU

CONCLUSION: A well-preserved joint status was found in our cohort. High-resolution US detected a higher percentage of abnormalities than the physical evaluation, but the clinical implications of these findings still need to be ascertained.

© 2015 John Wiley & Sons Ltd.

Keywords: annual joint bleeding rate; haemophilia joint health score; outcome assessment; prophylactic treatment; severe haemophilia A; ultrasound imaging

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