Disorders of coagulation factor function

Introduction Coagulation dysfunction is a hemorrhagic disease caused by clotting factor deficiency or dysfunction, which can be divided into two major categories: hereditary and acquired. Hereditary coagulopathy is generally a single clotting factor deficiency, mostly bleeding symptoms in infants and young children, mainly family history; acquired coagulopathy is more common, rehabilitation patients often have multiple clotting factor deficiency, mostly in adulthood Clinically, in addition to bleeding, there are symptoms and signs of primary disease. Hemophilia is the most common group of hereditary coagulation factor deficiency.

Cause

1. Hereditary single coagulation factor deficiency.

2, due to other diseases lead to a lack of a variety of clotting factors.

an examination

Related inspection

Platelet activating factor simple thrombin generation test platelet factor III effectiveness test simple thrombin generation test ethanol gel test

Diagnosis: To determine hyperuricemia is a hypersynthetic or excretory disorder requiring uric acid clearance, uric acid clearance or urinary uric acid concentration and urinary uric acid concentration. Urinary clearance rate, uric acid anhydride clearance ratio: 5% or less is uric acid excretion disorder, 5% to 12% is mixed type, that is, both uric acid production and uric acid excretion disorder, more than 12% of uric acid synthesis. The ratio of urinary uric acid concentration and urinary uric anhydride concentration: 0.4%~0.5% is uric acid excretion disorder; 0.4%~0.8% is mixed type, and 0.8% or more is excessive uric acid synthesis. Excessive synthesis of uric acid can be divided into two major categories: primary and secondary. The primary is uric acid over-synthesis and enzyme deficiency. Secondary is found in blood diseases, tumors, burns and other large amounts of nucleic acid. Excretory disorders can also be divided into primary and secondary, primary is mostly idiopathic or hereditary; secondary is more common in renal dysfunction, dehydration, hyperthyroidism, acidosis, etc.; More common in alcohol, sorghum, high-calorie diet, after strenuous exercise, obesity and so on.

Can be used for laboratory identification of coagulopathy:

Corresponding examinations are performed for the relevant possible coagulation mechanism disorders.

1. Factor VIII, IX, XI, XII or Fletcher, Fitzerald factor deficiency can be used for thromboplastin production test.

2. When factor VII, V, X or prothrombin is deficient, prothrombin time correction test, Stypven venom time, and activity of related factors can be determined.

3, fibrinogen deficiency or abnormality, disseminated intravascular coagulation can be done fibrinogen quantification and molecular structure examination related to disseminated intravascular coagulation multiple tests. Finally, combined with the results of various inspections to make a comprehensive judgment.

Differential diagnosis

1. Vascular hemophilia is an inherited hemorrhagic disease whose clinical features are prolonged bleeding from childhood, prolonged bleeding of platelets, reduced platelet aggregation induced by ristocetin or non-aggregated plasma The von willebrand factor (vWF) is deficient or has abnormal molecular structure.

2. Vitamin K deficiency: Vitamin K plays an important role in the process of blood coagulation. In the absence of vitamin K-dependent coagulation factors (prothrombin, factor VII, IX and X), these factors need to be involved in liver synthesis by vitamin K. Through the release of the cell membrane to the outside of the cell, spontaneous bleeding often occurs in severe cases.

3. Disseminated intravascular coagulation is a complex pathological process in the development of the disease, and is a group of severe hemorrhagic syndrome. It is characterized by a transient hypercoagulable state under certain pathogenic factors. Platelet aggregation and fibrin deposition form extensive microthrombus, followed by consumptive hypocoagulability with secondary fibrinolysis. The clinical manifestation is Acute types such as hemorrhage, embolism, microcirculatory disorders and hemolysis are dangerous and dangerous, and can be life-threatening if not treated in time.

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