factor IX complex (redirected from Defix)

complex /com·plex/ (kom´pleks)

1. a combination of various things, e.g., a complex of symptoms; see syndrome.

2. sequence (2).

3. a group of interrelated ideas, mainly unconscious, that have a common emotional tone and strongly influence a person's attitudes and behavior.

4. that portion of an electrocardiogram representing the systole of an atrium or ventricle.

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AIDS dementia complex  HIV encephalopathy.

AIDS-related complex  (ARC) a complex of signs and symptoms representing a less severe stage of human immunodeficiency virus (HIV) infection, characterized by chronic generalized lymphadenopathy, fever, weight loss, prolonged diarrhea, minor opportunistic infections, cytopenia, and T-cell abnormalities of the kind associated with AIDS.

anomalous complex  in electrocardiography, an abnormal atrial or ventricular complex resulting from aberrant conduction over accessory pathways.

antigen-antibody complex  a complex formed by the binding of antigen to antibody.

anti-inhibitor coagulant complex  (AICC) a concentrated fraction from pooled human plasma, which includes various coagulation factors; used as an antihemorrhagic in hemophilic patients with factor VIII inhibitors.

atrial complex  the P wave of the electrocardiogram, representing electrical activation of the atria.

atrial premature complex  (APC) a single ectopic atrial beat arising prematurely, which may be associated with structural heart disease.

atrioventricular (AV) junctional escape complex  see under beat.

atrioventricular (AV) junctional premature complex  an ectopic beat arising prematurely in the atrioventricular junction and traveling toward both the atria and ventricles if unimpeded, causing the P wave to be premature and abnormal or absent and the QRS complex to be premature.

branched-chain α-keto acid dehydrogenase complex  a multienzyme complex that catalyzes the oxidative decarboxylation of the keto acid analogues of the branched chain amino acids; deficiency of any enzyme of the complex causes maple syrup urine disease.

calcarine complex  calcar avis.

castration complex  in psychoanalytic theory, unconscious thoughts and motives stemming from fear of damage to or loss of sexual organs as punishment for forbidden sexual desires.

Eisenmenger's complex  a defect of the interventricular septum with severe pulmonary hypertension, hypertrophy of the right ventricle, and latent or overt cyanosis.

Electra complex  the counterpart in females of the Oedipus complex, involving the daughter's love for her father and jealousy or resentment towards her mother; now rarely used since Oedipus complex (q.v.) has come to be applied to both sexes.

exstrophy-epispadias complex  a spectrum of congenital defects of the anterior abdominal wall, ranging from epispadias to exstrophy of the bladder to exstrophy of cloaca.

factor IX complex  a partially purified factor IX fraction also including factor II, VII, and X fractions, from venous human plasma. It is used in the treatment of hemophilia B, replacement of factor VII, and treatment of anticoagulant-induced hemorrhage.

Ghon complex  primary c. (1).

β-glycosidase complex  the enzyme complex comprising lactase and phlorhizin hydrolase activities, occurring in the brush border membrane of the intestinal mucosa and hydrolyzing lactose as well as cellobiose and cellotriose.

Golgi complex  Golgi apparatus; a complex cellular organelle consisting mainly of a number of flattened sacs (cisternae) and associated vesicles, involved in the synthesis of glycoproteins, lipoproteins, membrane-bound proteins, and lysosomal enzymes. The sacs form primary lysosomes and secretory vacuoles.

immune complex  antigen-antibody c.

inclusion complex  one in which molecules of one type are enclosed within cavities in the crystalline lattice of another substance.

inferiority complex  unconscious feelings of inadequacy, producing timidity or, as a compensation, exaggerated aggressiveness and expression of superiority.

junctional premature complex  atrioventricular junctional premature c.

LCMV-LASV complex  a group of antigenically related viruses comprising the Old World arenaviruses. Lassa virus (Lassa fever) and lymphocytic choriomeningitis virus are pathogenic for humans.

Lutembacher's complex  see under syndrome.

major histocompatibility complex  (MHC) the chromosomal region containing genes that control the histocompatibility antigens. In humans, it controls the HLA antigens.

membrane attack complex  (MAC) the pentamolecular complex of components C5b,6,7,8,9 formed in the final pathway of complement activation, inserting into the target cell membrane where it creates a pore and results in cytolysis.

Oedipus complex  the feelings and conflicts occurring in a child that result from sexual attraction to the opposite-sex parent, including envious, aggressive feelings toward the same-sex parent.

pore complex  a nuclear pore and its annulus considered together.

primary complex 

1. the combination of a Ghon focus and a corresponding lymph node focus in primary tuberculosis in children; similar lesions are seen with other mycobacterial and fungal infections.

2. the primary cutaneous lesion at the site of skin infection, e.g., a chancre in syphilis or tuberculosis.

primary inoculation complex , primary tuberculous complex tuberculous chancre.

pyruvate dehydrogenase complex  a multienzyme complex that catalyzes the formation of acetyl coenzyme A from pyruvate and coenzyme A; deficiency of any component of the complex results in lacticacidemia, ataxia, and psychomotor retardation.

QRS complex  the portion of the electrocardiogram comprising the Q, R, and S waves, together representing ventricular depolarization.

sucrase-isomaltase complex  the enzyme complex comprising sucrase and isomaltase activities, occurring in the brush border of the intestinal mucosa and hydrolyzing maltose as well as maltotriose and some other glycosidic bonds.

symptom complex  syndrome.

synaptonemal complex  the structure formed by the synapsis of homologous chromosomes during the zygotene stage of meiosis I.

Tacaribe complex  a group of antigenically related viruses comprising the New World arenaviruses, including Junin virus, the agent of Argentinian hemorrhagic fever, and Machupo virus, the agent of Bolivian hemorrhagic fever.

VATER complex  an association of congenital anomalies consisting of v ertebral defects, imperforate a nus, t racheoe sophageal fistula, and r adial and r enal dysplasia.

ventricular complex  the combined QRS complex and T wave, together representing ventricular electrical activity.

ventricular premature complex  (VPC) an ectopic beat arising in the ventricles and stimulating the myocardium prematurely.

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factor IX complex,

a hemostatic containing factors II, VII, IX, and X.

indication It is prescribed in the treatment of hemophilia B. It is a vitamin K-dependent protein synthesized in the liver.

contraindication Liver disease with associated intravascular coagulation and fibrinolysis is the only contraindication.

adverse effects Among the more serious adverse effects are hepatitis, intravascular coagulation, circulatory collapse, and hypersensitivity reaction.

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complex [kom´pleks]

1. the sum, combination, or collection of various things or related factors, like or unlike; e.g., a complex of symptoms (see syndrome).

2. a group of interrelated ideas, mainly unconscious, that have a common emotional tone and strongly influence a person's attitudes and behavior.

3. that portion of an electrocardiographic tracing which represents the systole of an atrium or ventricle.

AIDS-related complex (ARC) a complex of signs and symptoms occurring in HIV infection including fever, weight loss, prolonged diarrhea, minor opportunistic infections, lymphadenopathy, and changes in cells of the immune system.

antigen-antibody complex here the complex formed by the noncovalent binding of an antibody and antigen. Complexes of antibodies belonging to certain immunoglobulin classes may activate complement. Called also immune complex.

anti-inhibitor coagulant complex (AICC) a concentrated fraction from pooled human plasma, which includes various coagulation factors. It is administered intravenously as an antihemorrhagic in hemophilic patients with inhibitors to coagulation factor VIII.

atrial complex the P wave of the electrocardiogram, representing electrical activity of the atria. See also ventricular complex.

castration complex in psychoanalytic theory, unconscious thoughts and motives stemming from fear of loss of the genitals as punishment for forbidden sexual desires.

Electra complex libidinous fixation of a daughter toward her father. This term is rarely used, since oedipus complex is generally applied to both sexes.

factor IX complex a sterile, freeze-dried powder consisting of partially purified coagulation factor IX fraction, as well as concentrated factor II, VII, and X fractions, of venous plasma from healthy human donors. It is used in the prophylaxis and treatment of bleeding in patients with hemophilia B, replacement of factor VII in patients deficient in that factor, and treatment of anticoagulant-induced hemorrhage. Administered intravenously.

Ghon complex primary complex (def. 1).

Golgi complex golgi apparatus.

HLA complex the human major histocompatibility complex, which contains the hla antigens.

immune complex antigen-antibody complex.

inclusion complex one in which molecules of one type are enclosed within cavities in the crystalline lattice of another substance.

inferiority complex unconscious feelings of inadequacy, producing shyness or timidity or, as a compensation, exaggerated agressiveness and expression of superiority; based on Alfred Adler's concept that everyone is born with a feeling of inferiority stemming from real or imagined physical or psychological deficiency, with the manner in which the inferiority is handled determining behavior.

interpolated premature ventricular complex a premature ventricular complex that does not interfere with the conduction of the next sinus beat, i.e., it lacks the usual following compensatory pause.

major histocompatibility complex (MHC) the chromosomal region containing genes that control the histocompatibility antigens; in humans it controls the hla antigens.

membrane attack complex (MAC) C5b,6,7,8,9, the five-molecule complex that is the cytolytic agent of the complement system.

Oedipus complex see oedipus complex.

primary complex

1. the combination of a parenchymal pulmonary lesion (Ghon focus) and a corresponding lymph node focus, occurring in primary tuberculosis, usually in children. Similar lesions may also be associated with other mycobacterial infections and with fungal infections.

2. the primary cutaneous lesion at the site of infection in the skin, e.g., chancre in syphilis and tuberculous chancre.

QRS complex a group of waves seen on an electrocardiogram, representing ventricular depolarization. Called also QRS wave. It actually consists of three distinct waves created by the passage of the cardiac electrical impulse through the ventricles and occurs at the beginning of each ventricular contraction. In a normal surface electrocardiogram the R wave is the upward deflection; the first downward deflection represents a Q wave and the final downward deflection is the S wave. The Q and S waves may be extremely weak and sometimes are absent.

One abnormality of the QRS complex is increased voltage resulting from enlargement of heart muscle, which produces increased quantities of electric current. A low-voltage QRS complex may result from toxic conditions of the heart, most commonly from fluid in the pericardium. Pleural effusion and emphysema also can cause a decrease in the voltage of the QRS complex.

VATER complex an association of congenital anomalies consisting of vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia.

ventricular complex the Q, R, S, and T waves of the electrocardiogram, representing ventricular electrical activity. See also atrial complex.

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factor

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.

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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

see hageman factor.

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.

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factor IX complex

Bebulin VH, Defix (UK), Hipfix (UK), Profilnine SD, Proplex T (heat-treated), Replenine (UK)

Pharmacologic class: Blood modifier

Therapeutic class: Antihemophilic

Pregnancy risk category C

Action

Converts fibrinogen to fibrin, increasing levels of clotting factors

Availability

Powder for injection: Various strengths; units specified on label

⊘Indications and dosages

➣ Factor IX deficiency (hemophilia B or Christmas disease); anticoagulant overdose

Adults and children: Dosage individualized; drug administered I.V. Use following equations to calculate approximate units needed:

Human product - 1 unit/kg times body weight (in kg) times desired increase in factor IX level, expressed as percentage of normal

Recombinant product - 1.2 units/kg times body weight (in kg) times desired increase in factor IX level, expressed as percentage of normal

Proplex T - 0.5 unit/kg times body weight (in kg) times desired increase in factor IX level, expressed as percentage of normal

Off-label uses

• Hepatic dysfunction
• Esophagitis
• Unspecified GI hemorrhage (human product)

Contraindications

• Hypersensitivity to mouse or hamster protein (with BeneFix)
• Fibrinolysis

Precautions

Use cautiously in:
• recent surgery
• pregnant patients
• children younger than age 6 (safety and efficacy not established).

Administration

☞ Give by slow I.V. infusion. Average infusion rate is 100 units (2 to 3 ml)/minute; don't exceed 10 ml/minute.
• If prescribed, administer hepatitis B vaccine before giving factor IX.
• Know that dosage is highly individualized according to degree of factor IX deficiency, patient's weight, and bleeding severity.
• Don't use glass syringe. Don't shake reconstituted solution or mix with other I.V. solutions.

Route	Onset	Peak	Duration
I.V.	Immediate	10-30 min	Unknown

Adverse reactions

CNS: light-headedness, paresthesia, headache

CV: blood pressure changes, thromboembolic reactions, myocardial infarction (MI)

EENT: allergic rhinitis

GI: nausea, vomiting

Hematologic: disseminated intravascular coagulation (DIC)

Respiratory: pulmonary embolism

Skin: rash, flushing, diaphoresis, pruritus, urticaria

Other: altered taste, fever, chills, burning sensation in jaw and skull, pain at I.V. injection site, hypersensitivity reactions including anaphylaxis

Interactions

Drug-drug. Aminocaproic acid: increased risk of thrombosis

Patient monitoring

• Be aware that factor IX complex may transmit hepatitis.
• Closely monitor vital signs during infusion.
☞ Observe for hemolytic reaction. If it occurs, stop infusion, flush line with saline solution, and notify prescriber immediately.
• Monitor I.V. injection site closely.
☞ Monitor coagulation studies closely. Know that drug may cause thromboembolic disorders, including MI and DIC.

Patient teaching

• Inform patient that drug may transmit diseases.
☞ Tell patient to immediately report signs and symptoms of hypersensitivity reaction, including rash, hives, tightness in chest, wheezing, shortness of breath, and swelling of throat or lips.
☞ Advise patient to immediately report unusual bleeding or bruising.
• Caution patient to avoid activities that can cause injury.
• Tell patient to wear medical identification stating that he has a blood-clotting disorder.
• Instruct patient to notify surgeon or dentist of his blood-clotting disorder before surgery or invasive dental procedures.
• As appropriate, review all other significant and life-threatening adverse reactions and interactions, especially those related to the drugs mentioned above.