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.


an agent or element that contributes to the production of a result. In epidemiology and statistics called also a variable because the factor may have a number of values. In an experiment a factor is a type of treatment and in the experiment the factor will be represented in different groups by different values. Such a factor may originate spontaneously or be introduced by an investigator.

factor analysis
a statistical method for analyzing the correlations between several variables.
antihemorrhagic factor
vitamin K.
antinuclear factor (ANF)
antinuclear antibody.
antirachitic factor
vitamin D.
factor B
a complement component (C3 proactivator) that participates in the alternate complement pathway.
C3 nephritic factor
a gamma globulin that is not an immunoglobulin, which is found in the plasma of certain individuals with membranoproliferative glomerulonephritis associated with hypocomplementemia; it initiates the alternate complement pathway.
citrovorum factor
folinic acid.
clotting f's, coagulation f's
factors essential to normal blood clotting, whose absence, diminution or excess may lead to abnormality of the clotting mechanism. See also clotting factors.
factor D
a factor that, when activated, serves as a serine esterase in the alternate complement pathway.
extrinsic factor
a hematopoietic vitamin that combines with intrinsic factor for absorption from the intestine and is needed for erythrocyte maturation; called also cyanocobalamin and vitamin B12.
F factor, fertility factor
the plasmid that determines the mating type of conjugating bacteria, being present in the donor (male) bacterium and absent in the recipient (female).
f's I to XIII
see clotting factors and names of individual factors.
factor VIII activity
a test for hemophilia A; activity is measured in biologic assays using factor VIII-deficient plasma as the substrate.
factor VIII-related antigen
von Willebrand antigen.
factor IX deficiency
see plasma thromboplastin component (PTC).
factor IX complex
a sterile, freeze-dried powder containing coagulation factors II, VII, IX and X.
factor X deficiency
see stuart factor.
factor XI deficiency
see plasma thromboplastin antecedent (PTA).
factor XII deficiency
fibrin stabilizing factor
factor XIII, one of the blood clotting factors that converts soluble fibrin monomer to insoluble, stable fibrin polymer.
intrinsic factor
a glycoprotein secreted by the parietal cells of the gastric glands, necessary for the absorption of vitamin B12 (cyanocobalamin, extrinsic factor). Its absence in humans results in pernicious anemia. Porcine stomach is a very rich source.
LE factor
an immunoglobulin (a 7S antibody) that reacts with leukocyte nuclei, found in the serum in systemic lupus erythematosus.
factor loading
a relationship between observable manifestations (or variables) and the underlying factors affecting the variables.
lymph node permeability factor (LNPF)
a substance from normal lymph nodes which produces vascular permeability.
lymphocyte transforming factor (LTF)
a lymphokine causing transformation and clonal expansion of lymphocytes.
osteoclast activating factor
substance produced by lymphocytes which facilitates bone resorption.
platelet f's
factors important in hemostasis that are contained in or attached to the platelets. See also platelet factors.
platelet-activating factor (PAF)
an immunologically produced substance which leads to clumping and degranulation of blood platelets.
R factor, resistance factor
a bacterial plasmid (R plasmid) which carries genes for antimicrobial resistance; it can be transmitted to other bacterial cells by conjugation, as well as to daughter cells.
release factor
a protein that binds directly to any stop codon that reaches the A site on the ribosome.
releasing f's
factors elaborated in one structure (as in the hypothalamus) that effect the release of hormones from another structure (as from the anterior pituitary gland), including corticotropin releasing factor, melanocyte-stimulating hormone releasing factor and prolactin releasing factor. Applied to substances of unknown chemical structure, while substances of established chemical identity are called releasing hormones.
transfer factor (TF)
a factor released from sensitized lymphocytes that has the capacity to transfer delayed hypersensitivity to a normal (nonsensitized) animal. See also transfer factor.

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..

More discussions about factor IX deficiency
References in periodicals archive ?
In one of our earlier studies we had reported 45 documented cases with 36 (81%) factor VIII deficiency, 8 (17%) factor IX deficiency and one factor X deficiency case.
The programme is aimed at prevention and treatment of bleeding events in patients with factor IX deficiency.