factor IX deficiency
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Management Factor IX concentrates
factor IX deficiencyChristmas disease, hemophilia B Hematology An X-R disorder due to a deficiency in Factor IX resulting in a bleeding diathesis Epidemiology Incidence–1/40,000 or 15-20% of hemophilias; severe disease is usually of neonatal onset Clinical, severe disease Factor IX activity is < 1% of normal; neonatal onset of bleeding, hematomas after injections or circumcision, hemarthroses and deep tissue hemorrhage, spontaneous bleeding Common sites Hemarthrosis of elbows, knees, ankles; pain, swelling, limited ROM of affected joint; muscle hematomas–pain, swelling, may cause muscle atrophy; mucosa–mouth, teeth, nose (epistaxis), GI tract; peripheral nerve lesions–femoral–iliopsoas muscle, sciatic–buttock; tibial–calf; perineal–anterior leg compartment; median & ulnar–forearm flexors; high-risk hemorrhages–intracranial, intraspinal, retropharyngeal, retroperitoneal; hematuria Lab ↑ PTT, ↓ factor IX, normal PT, bleeding time, thrombin time, platelets Management, supportive–avoid trauma, anticoagulants–ASA, add pad to crib/playpen; pressure compresses on bleeding sites; immunize against HBV Replacement Factor IX Concentrate; FFP. See Factor IX, Thombin.
Patient discussion about factor IX deficiency
Q. A christmas wish for each of you! To have all the presents in the world is not my desire for you! May your Christmas gift from me today be a sincere prayer that you will know: Peace that makes you sleep like a baby Joy that makes you laugh like a fool Happiness that lets you forget the size of your problems Hope that makes impossible dreams come true Love that makes you give from your heart Forgiveness when ‘you know not what you do”! Kindness that brings favor wherever you go And health that makes you act the age you choose.
Stay healthy always..