kaolinite and Hemophilia-B

To gain greater insight into the nature of the bleeding tendency in hemophilia, we compared the spatial dynamics of clotting in platelet-free plasma from healthy donors and from patients with severe hemophilia A or B (factor VIII:C or IX:C<1%). Clotting was initiated via the intrinsic or extrinsic pathway in a thin layer of nonstirred plasma by bringing it in contact with the glass or fibroblast monolayer surface. The results suggest that clot growth is a process consisting of two distinct phases, initiation and elongation. The clotting events on the activator surface and the preceding period free of visible signs of clotting are the initiation phase. In experiments with and without stirring alike, this phase is prolonged in hemophilic plasma activated by the intrinsic, but not the extrinsic pathway. Strikingly, both hemophilia A and B are associated with a significant deterioration in the elongation phase (clot thickening), irrespective of the activation pathway. The rate of clot growth in hemophilic plasma is significantly lower than normal and declines quickly. The resulting clots are thin, which may account for the bleeding disorder.

Topics: Blood Coagulation; Cells, Cultured; Factor IX; Factor VIII; Factor XI; Glass; Hemophilia A; Hemophilia B; Humans; In Vitro Techniques; Kaolin; Polyethylene Terephthalates; Thrombin; Time Factors

Comparative analysis of high molecular weight kininogen (HK) in various commercial congenital and immunodepleted deficiency plasmas was performed by immunoblotting of HK. It was found, that some artificially depleted deficiency plasmas contained proteolytically cleaved, kinin-free kininogen. In contrast, in all congenitally deficient plasmas, HK was present in the intact, single chain form. Thus, cleavage of kininogen could have been triggered by or during the immunodepletion procedure. It was seen, that the degree of proteolytic cleavage and degradation of HK in depleted plasmas differed among various manufacturers. E.g. depleted products of one company contained only trace amounts of cleaved HK, in contrast to products of another one, in which HK was completely degraded. The immunoblot analysis of HK reflects the occurrence of proteolytic events during the production of artificially deficient plasmas and can therefore serve as a quality control method.

Topics: Blood Coagulation Tests; Electrophoresis, Polyacrylamide Gel; Hemophilia A; Hemophilia B; Humans; Immunoblotting; Kaolin; Kininogens; Molecular Weight; Plasma; Protein C Deficiency

A simple microtechnique for carrying out partial thromboplastin time with kaolin tests with 2 microliter or less of test plasma is described. For single stage factor assays, less than 1 microliter of test solution may be used. Reagents and test plasma are loaded in sequence into a 10 microliter, long needle syringe and introduced into a micro test-tube immobilized in a water bath. The end-point is taken as a positive clearing of kaolin turbidity from the mixture while stirring. Correlation with normal techniques has been excellent.

Topics: Blood Coagulation Tests; Factor IX; Factor VIII; Hemophilia A; Hemophilia B; Humans; Kaolin; Partial Thromboplastin Time

Topics: Afibrinogenemia; Blood Coagulation Disorders; Blood Coagulation Tests; Factor V Deficiency; Factor VII Deficiency; Factor X Deficiency; Factor XI Deficiency; Factor XII Deficiency; Factor XIII Deficiency; Hemophilia A; Hemophilia B; Humans; Hypoprothrombinemias; Kaolin; Phosphatidylethanolamines; Prekallikrein; Prothrombin Time; Thrombin; Thromboplastin; von Willebrand Diseases

Reagents may be prepared from normal plasma and used with the prothrombin time and partial thromboplastin time tests to distinguish isolated defects of factors I, II, VII, VIII, IX, X, XI, or XII.

Topics: Adolescent; Adult; Aluminum Hydroxide; Blood Coagulation Disorders; Blood Coagulation Tests; Child; Child, Preschool; Factor XI Deficiency; Female; Hemophilia A; Hemophilia B; Humans; In Vitro Techniques; Kaolin; Male; Middle Aged; Phospholipids; Plasma; Prothrombin Time; Thromboplastin; Tromethamine; von Willebrand Diseases

Topics: Adenocarcinoma, Mucinous; Animals; Arginine; Blood Coagulation; Blood Coagulation Tests; Brain Chemistry; Bronchi; Chemical Precipitation; Disseminated Intravascular Coagulation; Esterases; Factor V Deficiency; Factor VII; Factor VII Deficiency; Factor X; Fibrinogen; Hemophilia B; Humans; Hypoprothrombinemias; Kaolin; Mucins; Mucus; Muramidase; Phospholipids; Prothrombin; Rabbits; Thromboplastin; Time Factors; Venoms

Topics: Adult; Blood Coagulation Tests; Blood Protein Electrophoresis; Blood Transfusion; Factor IX; Factor VIII; Freeze Drying; Hemophilia A; Hemophilia B; Humans; Kaolin; Male; Thromboplastin

Topics: Blood Coagulation Disorders; Blood Coagulation Tests; Hemophilia A; Hemophilia B; Humans; Kaolin; Thromboplastin

Topics: Blood Coagulation Disorders; Blood Coagulation Factors; Blood Coagulation Tests; Factor VIII; Hemophilia B; Humans; Hypoprothrombinemias; Kaolin; Methods; Prothrombin Time; Thromboplastin; Time Factors

Deficiencies of factor VIII (in haemophilia) and factor IX (in Christmas disease) prolong the partial thromboplastin time. If normal plasma is treated with alumina, the factor VIII remains but the factor IX is removed and can subsequently be recovered by elution of the alumina. If a long partial thromboplastin time is found on investigating a male patient whose history suggests a life-long bleeding disorder, the plasma may be retested after adding either alumina-adsorbed normal plasma or eluate. If the patient's partial thromboplastin time is shortened (relative to the control) by adding adsorbed normal plasma the patient is likely to be a haemophiliac; but if it is shortened by adding eluate then he is likely to have Christmas disease. Practical details for carrying out these manoeuvres are given and experiments on the validity of the test described.

Topics: Blood Coagulation Tests; Hemophilia A; Hemophilia B; Kaolin; Thromboplastin; Time Factors

Topics: Animals; Blood Coagulation; Blood Coagulation Disorders; Collagen; Elastin; Factor XII; Hemophilia B; Humans; Kaolin; Rabbits; Thrombosis

Topics: Adenine Nucleotides; Blood Coagulation Disorders; Blood Coagulation Factors; Blood Coagulation Tests; Blood Platelet Disorders; Blood Platelets; Edetic Acid; Erythrocytes; Factor XII; Hemophilia A; Hemophilia B; Kaolin; Lipoproteins; Microscopy; Microscopy, Phase-Contrast; Pharmacology; Platelet Factor 3; Platelet-Rich Plasma; Research; von Willebrand Diseases

The partial thromboplastin time test provides a convenient and sensitive screening procedure for deficiencies of thromboplastic factors, especially factors VIII and IX. The test is carried out after preincubating the plasma for 10 minutes with kaolin, and Inosithin is used as a platelet substitute. The ;normal range' of the test has been estimated in terms of the differences encountered between random normal plasmas tested in pairs, because individual patients are usually tested against single control subjects. A patient's partial thromboplastin time should be regarded as abnormal if it is more than six seconds longer than the control time. In the diagnosis of haemophilia, patients' plasmas with concentrations of factor VIII as low as about 20% might be regarded as being within the range of normal, if the selected control subject's factor VIII happened to lie near the lower end of the normal range. When mild haemophilia is suspected, discrimination may be improved by diluting both the patient's and the control plasmas 1 in 20 in haemophilic plasma. With the test modified in this way the clotting time is prolonged, though the range of differences among normal subjects is unaltered, and plasmas with factor VIII concentrations below about 30%, i.e., in undiluted plasma, would be unlikely to be regarded as normal. The partial thromboplastin time may be similarly modified as a screening test for factor IX deficiency.Some clinical examples are reported.

Topics: Aged; Blood Coagulation Tests; Diagnosis; Factor VIII; Hemophilia A; Hemophilia B; Humans; Indicators and Reagents; Kaolin; Partial Thromboplastin Time; Plasma; Reference Values; Thromboplastin