factor IX deficiency


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An X-linked recessive hemophilia, seen in 1/50,000 men, due to a defect in coagulation factor IX
Management Factor IX concentrates

factor IX deficiency

Christmas 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.

A. Happy Christmas, everybody.. May all the joy of this wonderful season follow our life in upcoming year ahead..
Stay healthy always..

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References in periodicals archive ?
Blood analyses reported as factor IX deficiency (35%) and decreased free protein S (44%).
Among other coagulation factor deficiencies factor IX deficiency was the second most common after factor VIII deficiency.
sex values Results 1 9/M PT ([dagger]) [down arrow] Returned to normal 2 4/M aPTT ([dagger]) [up arrow] Returned to normal 3 5/M aPTT [up arrow] Antiphospholipid antibodies (lupus anticoagulant) 4 4/M PT [down arrow] Factor VII activity: 20% (lupus anticoagulant) 5 6/M aPTT [up arrow] Hereditary factor IX and XII deficiency 6 40/M Normal Factor XIII deficiency (activity: 56%) 7 5/M Normal Factor IX deficiency (activity: 41%) Pt.
Haemophilia A (factor VIII deficiency) and haemophilia B (factor IX deficiency) are the most common and serious congenital coagulation factor deficiencies.
Refixia is the brand name for nonacog beta pegol; N9-GP, which is indicated for prophylaxis, on-demand treatment of bleeding and surgical procedures in adolescent (>12 years of age) and adult patients with haemophilia B (congenital factor IX deficiency).
Idelvion is used to replace Factor IX, a naturally occurring clotting factor that is missing (functionally deficient) or defective in people with Hemophilia B (also called congenital Factor IX deficiency or Christmas disease).