Disease Information for Factor V deficiency

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Clinical Manifestations
Signs & Symptoms
Excessive/easy bruising tendency
Bleeding gums
Bleeding/prolonged dental surgery
Blood in mouth/gums/throat
Epistaxis Children
Typical Clinical Presentation
Presentation/Hereditary bleeder/normal labs
Disease Progression
Course/Chronic disorder
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Family History
Family history/Bleeding disorders
Sex & Age Groups
Population/Child
Population/Children/all
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Clotting factor assay/abnormal (Lab)
Coagulation/Clotting factors decreased (Lab)
Thrombin Clotting Time increased
Abnormal Lab Findings - Decreased
Proaccelerin/ Factor 5 (Lab)
Abnormal Lab Findings - Increased
Clotting time/Activated clotting time test (Lab)
Prothrombin consumption (Lab)
PT -Prothrombin time (Lab)
Thromboplastin generation time (Lab)
Associated Diseases & Rule outs
Associated Disease & Complications
Bleeding complications/Surgery
Bleeding diathesis/hemorrhagic diathesis
Bleeding Tendency
Epistaxis/nosebleed
Factor V Deficiency
Factor VII deficiency
Menorrhagia
Postpartum hemorrhage
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Hematologic (category)
Process
PROCESS/Acquired variant/congenital variant both seen
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Hereditofamilial (category)
PROCESS/INCIDENCE/Acquired disorder
PROCESS/INCIDENCE/Extremely rare disease
PROCESS/Reference organ/system (category)
PROCESS/Coagulation derangement/disorder (ex)
PROCESS/Hereditary hemorrhagic/coagulation disorder (ex)
Synonyms
Synonym
AC globulin deficiency, ACCELERATOR GLOBULIN DEFICIENCY, DEFIC FACTOR V, Deficiencies Factor V, Deficiency AC globulin, Deficiency Factor V, Deficiency labile, deficiency labile factor, Deficiency proaccelerin, Disease Owren, Disease Owren's, Diseases Owren, Diseases Owren's, FACTOR V DEFIC, Factor V Deficiencies, Factor V deficiency, Factor V deficiency (disorder), FACTOR V DEFICIENCY CONGENITAL, Hereditary factor V deficiency disease, Hereditary factor V deficiency disease (disorder), Hereditary hypoproaccelerinaemia, Hereditary hypoproaccelerinemia, LABILE FACTOR DEFICIENCY, Labile factor deficiency (disorder), Owren, OWREN DISEASE, Owren Diseases, Owrens Disease, Owren's Disease, Owren's Diseases, Parahaemophilia, Parahemophilia, Parahemophilias, Proaccelerin deficiency, Proaccelerin deficiency (disorder), Synonym/Acquired Factor V Deficiency variant, Synonym/Autoimmune Anti- factor V (Deficiency ) Variant, Synonym/Congenital Factor V Deficfiency (recessive), Synonym/Factor V and Factor VII Deficiency Variant, Synonym/Factor V Deficiency (Dominant variant), Synonym/Owren's disease, Synonym/Parahemophilia, Synonym/Proaccelerin deficiency
Treatment
Drug Therapy - Indication
RX/Fresh frozen plasma (FFP)
Definition

A deficiency of blood coagulation factor V (known as proaccelerin or accelerator globulin or labile factor) leading to a rare hemorrhagic tendency known as Owren"s disease or parahemophilia; It varies greatly in severity; Factor V deficiency is an autosomal recessive trait;

(Dorland, 27th ed)

Parahemophilia; Owren's disease: Factor V deficiency is an inherited abnormal blood coagulation disorder caused by a deficiency of the plasma protein Factor V;When certain coagulation factors are deficient or missing, the chain reaction does not take place normally; Factor V is rare, and can be caused by inheriting a defective Factor V gene or by acquiring an antibody that interferes with normal Factor V function; An inhibitor of Factor V can be acquired after giving birth, after surgery, with automimmune diseases and certain cancers, in patients being treated wtih a certain type of fibrin glue, and from unknown sources; The disease is similar to hemophilia, except bleeding into joints is less common; In the inherited form of Factor V deficiency, a family history of a bleeding disorder is a risk factor;Excessive bleeding with menstrual periods and after delivery occurs frequently; A family history of a bleeding disorder is a risk factor; Men and women are affected equally; Symptoms: Bleeding into the skin; Excessive bruising; Nose bleeds; Bleeding of the gums; Excessive menstrual bleeding; Prolonged or excessive loss of blood with surgery or trauma; Umbilical stump bleeding;Signs and tests ;Factor V assay showing decreased activity; Slightly prolonged bleeding time (in some people); Prolonged partial thromboplastin time; Prolonged prothrombin time; Normal thrombin time

Treatment; Fresh plasma or fresh frozen plasma infusions will correct the deficiency temporarily

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External Links Related to Factor V deficiency
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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