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  Česky / Czech version Čes.-slov. Pediat., 2007, roč. 62, č. 9, s. 505-510.
 
The Problems of Inhibitor in Children Hemophilic Patients Observed at the Pediatrie Hematology and Oncology Clinic, University Hospital Prague- Motol  
Komrska V., Čepeláková V., Starý J. 

Klinika dětské hematologie a onkologie UK 2. LF, FN Motol, Praha přednosta prof. MUDr. J. Starý, DrSc
 


Summary:

       Among 230 pediatrie hemophiliacs registered by 31st March 2007 in the HemlS register (Hemophilia - Information System), there are 26 patients (11.3%) with a history of inhibitor, and only 6 of them (2.3%) háve the evidence of an active inhibitor requiring treatment. Between 1990 and 2006 in the Second Department of Pediatrics and later in the Department of Pediatrie Hematology and Oncology of the University Hospital Motol, 19 patients with an inhibitor were followed up, thirteen of them were treated with the objective of inhibitor elimination. Immunosuppressive and immune toleran¬ce therapy was ušed. In 10 of 13 treated patients the therapy led to a permanent elimi¬nation of inhibitor, in 3 patients it did not succeed due to an early termination of therapy. One of these patients died at home due to an unrecognized retroperitoneal bleeding. A treatment algorithm based on our experience is proposed in the discussion.

        Key words: register HemlS, pediatrie hemophiliacs, inhibitor, immunosuppressive treatment, immune tolerance treatment
       

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