Fibrinogen Baltimore IV: congenital dysfibrinogenemia with delayed fibrin monomer polymerization. 1985

R F Ebert, and W R Bell

A congenital dysfibrinogenemia, fibrinogen Baltimore IV, has been found in a 56-year-old Caucasian man. Clinical laboratory studies disclosed a slightly prolonged prothrombin time, but were otherwise unremarkable. Release of fibrinopeptides by thrombin occurs normally, as does ligation of the fibrin polymer by Factor XIII. Approximately half of the isolated fibrin monomers polymerize normally, but the remainder polymerize at about 2% of the initial rate. The functional defect is thus limited to a decrease in the rate of fibrin monomer polymerization.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010375 Pedigree The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition. Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D011108 Polymers Compounds formed by the joining of smaller, usually repeating, units linked by covalent bonds. These compounds often form large macromolecules (e.g., BIOPOLYMERS; PLASTICS). Polymer
D001778 Blood Coagulation Disorders Hemorrhagic and thrombotic disorders that occur as a consequence of abnormalities in blood coagulation due to a variety of factors such as COAGULATION PROTEIN DISORDERS; BLOOD PLATELET DISORDERS; BLOOD PROTEIN DISORDERS or nutritional conditions. Coagulation Disorders, Blood,Disorders, Blood Coagulation,Blood Coagulation Disorder,Coagulation Disorder, Blood,Disorder, Blood Coagulation
D005337 Fibrin A protein derived from FIBRINOGEN in the presence of THROMBIN, which forms part of the blood clot. Antithrombin I
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000347 Afibrinogenemia A deficiency or absence of FIBRINOGEN in the blood. Fibrinogen Deficiency,Afibrinogenemia, Congenital,Congenital Afibrinogenaemia,Congenital Afibrinogenemia,Deficiency, Fibrinogen,Familial Afibrinogenemia,Hypofibrinogenemia, Congenital,Afibrinogenaemia, Congenital,Afibrinogenaemias, Congenital,Afibrinogenemia, Familial,Afibrinogenemias,Afibrinogenemias, Congenital,Afibrinogenemias, Familial,Congenital Afibrinogenaemias,Congenital Afibrinogenemias,Congenital Hypofibrinogenemia,Congenital Hypofibrinogenemias,Familial Afibrinogenemias,Fibrinogen Deficiencies,Hypofibrinogenemias, Congenital

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