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Disarticulation of a knee joint in a haemophiliac with high inhibitor titre
Author(s) -
CHUANSUMRIT A.,
HATHIRAT P.,
KEOROCHANA S.,
TARDTONG P.,
PINTADIT P.,
RUEANGWETSAWAT Y.,
ISARANGKURA P.
Publication year - 1996
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/j.1365-2516.1996.tb00027.x
Subject(s) - medicine , cryoprecipitate , methylprednisolone , haemophilia a , surgery , knee joint , disarticulation , haemophilia , amputation , fibrinogen
Summary. Disarticulation of a knee joint in an 8‐year‐old haemophilia A patient with high inhibitor of 3450 Bethesda units (BU) is described. He had an infected compound fracture of the tibia and fibula. Surgery was successfully performed after extensive plasma exchange; administration of immunosuppressive agents such as cyclophosphamide, methylprednisolone, intravenous immunoglobulin and cyclosporine were combined with a loading dose of 100 units kg ‐1 of factor VIII concentrate, followed by continuous infusion of 16 units kg ‐1 h ‐1 of factor VIII in the form of factor VIII concentrate and cryoprecipitate for 7 days and decreased to 8 units kg ‐1 h ‐1 in the form of cryoprecipitate for 19 more days. During the 1st to 7th post‐operative days, the lowest factor VIII inhibitor was 18 BU and the factor VIII level ranged from < 1–2.1 IU dL ‐1 . On the 9th and 13th post‐operative day, although the inhibitor rose to 330 and 2700 BU, respectively, there was no serious bleeding. The suture was removed on the 21st post‐operative day. The inhibitor spontaneously decreased to 550, 232 and 14 BU at 1, 7 and 10 months, respectively.