Coagulation , disorders

The hemorrhagic diathesis in patients with coagulation disorders is because of either an abnormaUty of one or more plasma proteins and/or platelets necessary for normal blood coagulation or the spontaneous presence of a circulating anticoagulant. Specific laboratory techniques are required for the precise identification of these disorders. 

Congenital deficiency of prothrombin is inherited in an autosomal recessive fashion and is the rarest of all the hereditary coagulation disorders. Congenital dysprothrombinemia has also been recognized. 

Fresh frozen plasma Multiple coagulation disorders... 

If the history dictates, testing may be done to identify coagulation disorder(s) as a cause. 

The primary treatment of recessively inherited coagulation disorders is single-donor fresh-frozen plasma (FFP) that contains all coagulation factors. 

Hemophilia A and B are coagulation disorders that result from defects in the genes encoding for plasma coagulation proteins. Hemophilia A (classic hemophilia) is caused by the deficiency of factor VIII, and hemophilia B (Christmas disease) is caused by the deficiency of factor IX. The incidences of hemophilia A and B are estimated at 1 in 5000 and 1 in 30,000 male births, respectively. Both types of hemophilia are evenly distributed across all ethnic and racial groups.1... 

Recessively inherited coagulation disorders (RICDs) refer to relatively rare deficiencies in factor II, V, VII, and X-XIII resulting in either decreased clotting factor production or production of a dysfunctional molecule with reduced activity.19 The clinical severity of bleeding varies and generally is poorly correlated with the factor blood levels. Table 64-6 illustrates these clotting factor deficiencies and some of their characteristics. 

The main goal of recessively inherited coagulation disorder (RICD) treatment is to prevent and control spontaneous and surgery-related bleeding episodes. Specifically, therapeutic options improve hemostasis via replacement of deficient blood coagulation factors while minimizing the development of immune tolerance.20... 

ACQUIRED COAGULATION DISORDERS DISSEMINATED INTRAVASCULAR COAGULATION (DIC)... 

Coagulation disorders Elevated prothrombin time, partial thromboplastin time, D-dimers hypofibrinogenemia... 

Chap. 64 - Coagulation Disorders Universal Program Number 014-999-07-079-H04... 

Table 12.2 Recombinant blood coagulation factors that have been approved for the management of coagulation disorders...

Cirrhosis results in elevation of portal blood pressure because of fibrotic changes within the hepatic sinusoids, changes in the levels of vasodilatory and vasoconstrictor mediators, and an increase in blood flow to the splanchnic vasculature. The pathophysiologic abnormalities that cause it result in the commonly encountered problems of ascites, portal hypertension and esophageal varices, HE, and coagulation disorders. 

Coagulation disorders Coagulation disorders due to faulty formation of factors II,... 

If possible, discontinue or reduce the dosage of drugs interfering with coagulation mechanisms (eg, salicylates, antibiotics) as an alternative to phytonadione. The severity of the coagulation disorder should determine whether the immediate administration of phytonadione is required in addition to discontinuation or reduction of interfering drugs. 

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