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Disease Information for Factor XIII deficiency

Clinical Manifestations

Typical Clinical Presentation

Presentation/Hereditary bleeder/normal labs

Disease Progression

Course/Chronic disorder

Course/Chronic only

Demographics & Risk Factors

Population Group

Child

Population/Pediatrics population

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)

Abnormal Lab Findings - Decreased

Factor 13 (Lab)

Disease Mechanism & Classification

Class

CLASS/Hematologic (category)

Pathophysiology

Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)

Process

PROCESS/Hereditofamilial (category)

PROCESS/Coagulation derangement/disorder (ex)

PROCESS/Hereditary hemorrhagic/coagulation disorder (ex)

Synonyms

Synonym

DEFIC FACTOR XIII, Deficiencies Factor XIII, Deficiency factor XIII, deficiency fibrin stabilizing factor, Deficiency Laki Lorand factor, FACTOR XIII DEFIC, Factor XIII Deficiencies, Factor XIII Deficiency, FACTOR XIII DEFICIENCY CONGENITAL, Factor XIII deficiency disease, Factor XIII deficiency disease (disorder), Fibrin stabilising factor defi, Fibrin stabilising factor deficiency, Fibrin stabilizing factor defi, Fibrin stabilizing factor deficiency, FIBRIN STABILIZING FACTOR DEFICIENCY CONGENITAL, FIBRINASE DEFICIENCY CONGENITAL, Hereditary factor XIII deficiency disease, Hereditary factor XIII deficiency disease (disorder), LAKI LORAND FACTOR DEFICIENCY CONGENITAL, Laki Lorand factor deficiency disease, Synonym/Fibrin stabilizing factor deficiency, Synonym/Laki-Lorand factor deficiency

Definition

A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis.

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External Links Related to Factor XIII deficiency

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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