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

Haemophilia : the Official Journal of the World Federation of Hemophilia
C AltisentR Parra

Abstract

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. 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). Median AJBR was 0.2 and median prophylaxis dose 65.4 IU-1  kg-1  week-1 . Median total HJHS was 0 (range 0-13) and total HEAD-US 1 (0-8). At the joint level, 85.3% of joints were normal on HJHS and 79.0% on US. The ankle was the joint most commonly affected, considering bleeding and ultrasound results. Correlation was found between HEAD-US scores and bleeding scores but not between HEAD-US and HJHS scores. HJHS and HEAD-US scores were concordant in 91/124 (73.4%) joints (86 joints normal and five abnormal). Ultrasound detected minimal chang...Continue Reading

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Citations

Sep 2, 2016·Haemophilia : the Official Journal of the World Federation of Hemophilia·M RechtS Pipe
Mar 17, 2017·Haemophilia : the Official Journal of the World Federation of Hemophilia·M A TimmerK Fischer
Jul 20, 2017·Haemophilia : the Official Journal of the World Federation of Hemophilia·N Bakeer
Aug 2, 2017·Journal of Clinical Medicine·Matteo Nicola Dario Di MinnoCarlo Martinoli
Mar 27, 2018·Haemophilia : the Official Journal of the World Federation of Hemophilia·A SeuserM E Mancuso
Mar 28, 2018·Haemophilia : the Official Journal of the World Federation of Hemophilia·A KlukowskaR J Liesner
Mar 2, 2018·Haemophilia : the Official Journal of the World Federation of Hemophilia·D StephensenA Wells
Jul 19, 2018·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Axel SeuserLily Heijnen
Jul 15, 2018·Journal of Thrombosis and Haemostasis : JTH·J Puetz
Nov 15, 2018·Haemophilia : the Official Journal of the World Federation of Hemophilia·Samantha C GouwKathelijn Fischer
May 3, 2019·Haemophilia : the Official Journal of the World Federation of Hemophilia·Novie A ChozieMarcel Prasetyo
May 28, 2019·Haemophilia : the Official Journal of the World Federation of Hemophilia·Atanas BanchevAnna Pavlova
May 12, 2020·British Journal of Haematology·Rachel RaymentUNKNOWN British Society for Haematology
Feb 1, 2018·Expert Review of Hematology·Hortensia De la Corte-RodriguezVíctor Jimenez-Yuste
Nov 18, 2018·Haemophilia : the Official Journal of the World Federation of Hemophilia·Victor Jiménez-YusteHae Kyung Kim
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Aug 21, 2020·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Hortensia De la Corte-RodriguezVíctor Jimenez-Yuste
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Sep 28, 2018·HSS Journal : the Musculoskeletal Journal of Hospital for Special Surgery·Hortensia De la Corte-RodriguezVíctor Jimenez-Yuste
Feb 8, 2021·Haemophilia : the Official Journal of the World Federation of Hemophilia·Ragnhild J MåseidePål A Holme
Feb 24, 2021·Haemophilia : the Official Journal of the World Federation of Hemophilia·Víctor Jiménez-YusteHae Kyung Kim
May 11, 2021·Haemophilia : the Official Journal of the World Federation of Hemophilia·Carla DaffunchioHoracio Caviglia

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