{"id":1862,"date":"2009-12-19T17:46:24","date_gmt":"2009-12-19T22:46:24","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=1862"},"modified":"2009-12-19T17:48:12","modified_gmt":"2009-12-19T22:48:12","slug":"hemophilia-2","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2009\/12\/hemophilia-2\/","title":{"rendered":"Hemophilia B"},"content":{"rendered":"
Hemophilia B is a disorder of the blood<\/p>\n
Pathophysiology<\/strong><\/p>\n 1)<\/strong> congenital bleeding disorder caused by deficiency in factor 9 2)<\/strong> factor 9 is synthesized in the liver and requires vitamin K for biologic activity 3)<\/strong> clinically indistinguishable from hemophilia A, but treatment is different<\/p>\n Biochemistry<\/strong><\/p>\n factor 9 is converted to its active form factor 9A by factor 11A or the factor 7A-tissue factor complex<\/p>\n Inheritance\/Epidemiology<\/strong><\/p>\n 1)<\/strong> occurs in 1\/100,000 in males 2)<\/strong> X-linked inheritance<\/p>\n Treatment<\/strong><\/p>\n 1)<\/strong> fresh frozen plasma (FFP) 2)<\/strong> monoclonal factor 9 preparation 3)<\/strong> prothrombin complex concentrates have been used but can cause embolism and thrombosis<\/p>\n Tips for USMLE<\/strong><\/p>\n 1)<\/strong> inhibitors to factor 9 do occur but much less frequently than in factor 8 deficiency (hemophilia A) 2)<\/strong> hemophilia A and B both have increased PTT, normal PT, normal bleeding time, and normal platelet count 3)<\/strong> differentiation between hemophilia A and B requires specific assays to detect absence of clotting factors 8 or 9<\/p>\n","protected":false},"excerpt":{"rendered":" Hemophilia B is a disorder of the blood Pathophysiology 1) congenital bleeding disorder caused by deficiency in factor 9 2) factor 9 is synthesized in the liver and requires vitamin K for biologic activity 3) clinically indistinguishable from hemophilia A, but treatment is different Biochemistry factor 9 is converted to its active form factor 9A […]<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_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":[1],"tags":[419,415,417,416,414,418,420],"yoast_head":"\n