Congenital dysfibrinogenemias. A review

Rocha, Eduardo; Páramo, José A.; Aranda, A.; Cuesta, B; Fernández, J

doi:10.1007/bf03029192

Cited by 9 publications

(3 citation statements)

References 102 publications

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“…Aunque la mayoría de los pacientes con anomalías funcionales del fibrinógeno tienen tendencia hemorrágica, aproximadamente un 1 O % de las disfibrinogenemias descritas hasta la actualidad presentan clínica trombótica. En estos pacientes la molécula de fibrinógeno anómalo forma geles de fibrina rígidos y resistentes a la acción de la plasmina 12 • Patología del sistema contacto El factor XII, la prekalicreína y el kininógeno de alto peso molecular están no solamente implicados en la iniciación de la coagulación intrínseca, sino que intervienen en la activación del sistema fibrinolítico y en la generación de kininas. Del balance de estas acciones dependerá que la deficiencia de factores contacto pueda conducir al desarrollo de fenómenos trombóticos, presentes en un 8 % de los casos como trombosis venosa o infarto de miocardio a pesar del alargamiento constante del tiempo de tromboplastina parcial activado 13 • Patología del sistema fibrinolítico Los defectos congénitos y adquiridos del mecanismo fibrinolítico pueden condicionar una alteración de la digestión de la fibrina que conllevará una mayor tendencia trombótica.…”

Section: Disfibrinogenemiasunclassified

Trombosis y estados hipercoagulables

Páramo¹,

Cuesta²,

Fernández³

et al. 2017

revista-de-medicina

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las estructuras vasculares, el enlentecimiento circulatorio y los factores circulantes. La incidencia creciente y la gravedad de las trombosis llevan a estas afecciones a ocupar el primer plano en lo que respecta a morbilidad. Se conoce la existencia de unos factores generales de riesgo como edad, sobrepeso, diabetes, hipertensión, tabaquismo o hiperlipidemia, pero se desconocen algunos mecanismos implicados en su patogénesis.

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Section: Disfibrinogenemiasunclassified

Trombosis y estados hipercoagulables

Páramo¹,

Cuesta²,

Fernández³

et al. 2017

revista-de-medicina

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“…More than 100 qualitative abnormalities of fibrinogen (dysfibrinogenemia) have been reported [16]. Dysfibrinogenernias are inherited in an autosomal dominant manner with most patients representing the heterozygous state.…”

Section: Dysfibrinogenemiamentioning

confidence: 99%

“…The majority of these have either no clinical symptoms or symptoms of a bleeding disorder. A minority, approximately 11% of the reported dysfibrinogens, have clinical features of a recurrent thromboembolic disorder [16,17]. The most commonly observed functional defect of the dysfibrinogenemias which is associated with thrombosis is an abnormality of fibrin monomer polymerization with resistance to fibrinolysis.…”

Section: Dysfibrinogenemiamentioning

confidence: 99%

Acquired and congenital clotting syndromes

1990

World j. surg.

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Although Virchow postulated 100 years ago that hypercoagulability states exist, it has only been in recent years that methods of documenting hypercoagulability have been developed. These clotting tendencies can be acquired or congenital. The common causes of acquired clotting tendencies include conditions which result in tissue and cellular damage, shock, transfusion reactions, and tissue necrosis. Certain drugs and drug reactions, and certain disease states which include blood dyscrasias and cancer are also associated with clotting problems. In certain diseases such as homocystinuria, hyperlipidemia, and lupus erythematosus, abnormal clotting tendencies may also develop. Important advances in the recognition of hypercoagulability have come with the documentation that congenital clotting abnormalities exist. Moreover, these abnormalities are proving to be more common than are congenital bleeding syndromes. Patients who appear to have spontaneous clotting manifestations and are under 40 years of age should be screened for one of these abnormalities. These congenital clotting tendencies can be classified as defects in thrombosis inhibitors, dysfibrinogenemias, or defects in fibrinolysis. The first thrombotic inhibitor defect recognized was antithrombin III deficiency which was reported in 1965. Subsequently, Protein C, Protein S, and Heparin cofactor II deficiencies have been recognized as contributing to thrombotic tendencies. Dysfibrinogenemias are relatively rare and most are associated with bleeding problems; however, 11% of the abnormal fibrinogens are associated with a clotting tendency. The reason appears to be that these fibrins are resistant to fibrinolysis. The most common defects which are associated with thrombotic tendencies appear, at the present time, to be due to defects in fibrinolysis. These include hypoplasminogenemia, decreases in plasminogen activator, increases in plasminogen activator inhibitor, and Factor XII deficiency.

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Screening for congenital coagulation disorders

Tripodi

1989

Res. Clin. Lab.

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Congenital dysfibrinogenemias. A review

Cited by 9 publications

References 102 publications

Trombosis y estados hipercoagulables

Trombosis y estados hipercoagulables

Acquired and congenital clotting syndromes

Screening for congenital coagulation disorders

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