The Problems of Inhibitor in Children Hemophilic Patients Observed at the Pediatric Hematology and Oncology Clinic, University Hospital Prague-Motol
Problematika inhibitoru u dětských hemofiliků, sledovaných na Klinice dětské hematologie a onkologie FN Motol
Mezi 230 dětskými hemofiliky, registrovanými k 31. 3. 2007 v celostátním registru hemofiliků HemIS (Hemofilie – Informační Systém), je 26 pacientů s inhibitorem (11,3 %), z nich pak pouze 6 (2,3 %) s „aktivním“, klinicky významným inhibitorem. V letech 1990 až 2006 bylo na II. dětské klinice a později Klinice dětské hematologie a onkologie FN Motol sledováno celkem 19 pacientů s inhibitorem, z nichž 13 bylo léčeno s cílem trvale inhibitor odstranit. Použita byla imunosupresivní a imunotoleranční léčba. U 10 pacientů, z 13 léčených, došlo k trvalému vymizení inhibitoru, u 3 pacientů se nepodařilo inhibitor odstranit, a to pro předčasné ukončení léčby. Jeden z těchto pacientů zemřel doma na nerozpoznané retroperitoneální krvácení. Na základě získaných zkušeností je v diskusi navržen léčebný algoritmus.
Klíčová slova:
registr HemIS, dětští hemofilici, inhibitor, imunosupresivní léčba, imunotoleranční léčba
Authors:
V. Komrska; V. Čepeláková; J. Starý
Authors‘ workplace:
Klinika dětské hematologie a onkologie UK 2. LF, FN Motol, Praha
přednosta prof. MUDr. J. Starý, DrSc.
Published in:
Čes-slov Pediat 2007; 62 (9): 505-510.
Category:
Original Papers
Overview
Among 230 pediatric hemophiliacs registered by 31st March 2007 in the HemIS register (Hemophilia – Information System), there are 26 patients (11.3%) with a history of inhibitor, and only 6 of them (2.3%) have the evidence of an active inhibitor requiring treatment. Between 1990 and 2006 in the Second Department of Pediatrics and later in the Department of Pediatric 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 tolerance therapy was used. In 10 of 13 treated patients the therapy led to a permanent elimination 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 HemIS, pediatric hemophiliacs, inhibitor, immunosuppressive treatment, immune tolerance treatment
Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2007 Issue 9
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