Hemophilia with factor VIII and factor IX inhibitors, incidence, bleeding problems and management.

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dc.contributor.authorMahasandana, Cen_US
dc.contributor.authorPatharathienskul, Den_US
dc.contributor.authorSuvatte, Ven_US
dc.date.accessioned2009-05-27T16:06:00Z
dc.date.available2009-05-27T16:06:00Z
dc.date.issued1993-03-01en_US
dc.descriptionThe Southeast Asian Journal of Tropical Medicine and Public Health.en_US
dc.description.abstractAmong 117 cases of hemophilia, there were 7 hemophilia A and 2 hemophilia B with factor VIII and factor IX inhibitors diagnosed at the Department of Pediatrics, Siriraj Hospital, Bangkok, Thailand. The overall incidence of hemophilia with inhibitors was 7.7%. Eight cases (6 hemophilia A. 2 hemophilia B) were severe hemophilia and 1 moderate hemophilia A. The average age of the inhibitor detection was about 5 years. Of the 9 cases, 7 had high inhibitor titers and 2 had low inhibitor titers. The frequency of bleeding problems before and after inhibitor detection were not different. The bleedings included hemarthrosis, mucosal bleed, hematoma, oozing from wound, hematuria and intracranial hemorrhage. The treatment of hemarthrosis in hemophilia A with low inhibitor titers was the combination of short course of prednisolone and single large dose factor VIII. In high inhibitor titer patients with acute hemarthrosis (both hemophilia A and hemophilia B), the treatment consisted of prednisolone short course and single high dose of PCC. For bleeding control in both high and low inhibitor titer with mucosal bleeds, oozing from wounds, central nervous system bleeding and hematuria, the combination was used of high dose factor VIII or factor IX for 2 days, and tranexamic acid, prednisolone, cyclophosphamide were required. In life-threatening hemorrhage and surgical operation, plasmapheresis and large dosage factor VIII or factor IX were the treatment of choice. All supportive measures were also important in every case of mucosal bleeds, wounds and surgical operations. The result of treatment revealed one death from massive intracranial hemorrhage and 8 survivals, with joint contracture in 2 cases. All still have inhibitor detected, but in low titer.en_US
dc.description.affiliationDepartment of Pediatrics, Siriraj Hospital, Mahidol University, Bangkok, Thailand.en_US
dc.identifier.citationMahasandana C, Patharathienskul D, Suvatte V. Hemophilia with factor VIII and factor IX inhibitors, incidence, bleeding problems and management. The Southeast Asian Journal of Tropical Medicine and Public Health. 1993 ; 24 Suppl 1(): 106-12en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/34414
dc.language.isoengen_US
dc.source.urihttps://www.tm.mahidol.ac.th/seameo/publication.htmen_US
dc.subject.meshChilden_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshFactor IX --antagonists & inhibitorsen_US
dc.subject.meshFactor VIII --antagonists & inhibitorsen_US
dc.subject.meshHemophilia A --blooden_US
dc.subject.meshHemophilia B --blooden_US
dc.subject.meshHemorrhage --etiologyen_US
dc.subject.meshHumansen_US
dc.subject.meshIncidenceen_US
dc.subject.meshInfanten_US
dc.subject.meshRetrospective Studiesen_US
dc.subject.meshSeverity of Illness Indexen_US
dc.subject.meshThailanden_US
dc.subject.meshTreatment Outcomeen_US
dc.titleHemophilia with factor VIII and factor IX inhibitors, incidence, bleeding problems and management.en_US
dc.typeJournal Articleen_US
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