{"id":9564,"date":"2014-03-10T18:28:00","date_gmt":"2014-03-10T22:28:00","guid":{"rendered":"https:\/\/crashingpatient.com\/?p=9564"},"modified":"2014-03-10T18:28:00","modified_gmt":"2014-03-10T22:28:00","slug":"hemophilia-von-willebrands-disease","status":"publish","type":"post","link":"https:\/\/crashingpatient.com\/medical-surgical\/hematology\/hemophilia-von-willebrands-disease.htm\/","title":{"rendered":"Hemophilia and von Willebrand’s Disease"},"content":{"rendered":"
x linked recessive causing defective VIII:c,<\/p>\n
Abnormal ptt.<\/p>\n
Replace c cryoprecipitate of factor VIII 1 unit=100 units VIII<\/p>\n
1 cc of FFP=1 unit VIII and less of IX<\/p>\n
dDAVP raises factor VIII by causing release from endothelial cells, It has ADH-like effects.\u00a0 Dose:\u00a0 0.3 mcg\/kg.<\/p>\n
Number of units=0.5 x (Desired Factor Level) x (Weight in Kg)\u00a0\u00a0\u00a0\u00a0\u00a0 (1\/2 life of 12 hours)\u00a0 (Each unit of factor\/kg raises levels by 2%)<\/p>\n
Raise to 100% in head injury, mult. Trauma, neck\/tongue\/throat hematoma, ABD pn\/GI bleed, eye trauma.<\/p>\n
VIII-plasma or recombinant<\/p>\n
<\/p>\n
L<\/strong><\/p>\n evels for Hem A\/B<\/p>\n 10 minute post transfusion, get PTT and factor level<\/p>\n <\/p>\n Factor IX, replace c factor<\/p>\n 1 x Factor Level x Kg (1\/2 life of 24 hours)<\/p>\n <\/p>\n (Benefix is only 80% factor IX so multiply by 1.25 instead of 1)<\/p>\n <\/p>\n IX-older plasma derived can be thrombogenic, use recombinant<\/p>\n Autosomal (usually dominant)<\/p>\n Presents with mucocutaneous\u00a0 bleeding, commonly epistaxis, gi bleeds, and prolonged menorrhagia.<\/p>\n Patients will have prolonged PTT, increased bleeding time, Normal PT and Platelet count.<\/p>\n 1 bag cryo per 10 kg or FFP in emergency<\/p>\n Consider if prolonged vaginal bleeding in menarche or in women in the perimenopausal period.\u00a0 Estrogen and progesterone raise vWF level.\u00a0 When Anovulatory cycles are present, may see prolonged bleeding.<\/p>\n Treat with dDAVP, Factor VIII concentrates with high vWF levels (Humate-P or Koate-HS), Cryoprecipitate (10 units\/kg), FFP<\/p>\n","protected":false},"excerpt":{"rendered":" Hemophilia A x linked recessive causing defective VIII:c, Abnormal ptt. Replace c cryoprecipitate of factor VIII 1 unit=100 units VIII 1 cc of FFP=1 unit VIII and less of IX dDAVP raises factor VIII by causing release from endothelial cells, It has ADH-like effects.\u00a0 Dose:\u00a0 0.3 mcg\/kg. Number of units=0.5 x (Desired Factor Level) x […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[25],"tags":[],"yoast_head":"\n\n
<\/span>Hemophilia B (Christmas)<\/span><\/h2>\n
<\/span>von Willebrand\u2019s Disease<\/span><\/h2>\n