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Query: UMLS:C0684275 (haemophilia)
 10,958 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thrombotest clotting times of mixtures of coumarin plasmas and normal plasma yielded a patterm similar to that observed in mixtures of plasma with congenital coagulation disorders and normal plasma. The presence of 10 or 20% of test plasma in the mixture failed to affect the clotting times which resulted in normal limits. The only exception to this rule was the hemophilia BM plasma. In this case even the presence of 10-20% of patient plasma in the mixture caused a prolongation of the clotting time. This indicates that no inhibitor is present in coumarin plasmas and in the plasma of congenital coagulation disorders of the prothrombin complex save for hemophilia BM plasma which does contain an inhibitor.
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PMID:Thrombotest mixing experiments in congenital coagulation disorders of the prothrombin complex and in coumarin treated patients. An additional evidence against the presence of an inhibitor in the latter. 7 33

Three children with haemophilia and antibodies to factor VIII were treated with a non-activated prothrombin concentrate (Prothrombinex) for 12 bleeding episodes. There was clear clinical response and joint aspirations were performed after infusions of phothrombinex in a dose of 30--50 factor IX units/kg body weight and there was no clinical evidence of thrombosis or febrile reactions. There was a significant shortening of the activated partial thromboplastin time (PTT) at one and four hours after the initial infusion with a return to pre-infusion levels 9--24 hours after infusion. The shortening in the PTT was less marked in subsequent infusions. There were no changes in the level of factor VIII procoagulant activity, factor VIII related antigen or factor VIII antibodies after the infusion. In two patients platelet function studies were unaltered by the infusion and in one patient procoagulant levels of factor II, IX and X were no greater than expected from the infusion. We conclude that infusions of non-activated prothrombin concentrates are clinically effective in the treatment of children with haemophilia and factor VIII antibodies but the mechanism of action is unknown.
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PMID:The use of non-activated prothrombin concentrate in the management of haemophilia A with factor VIII antibodies. 26 89

In four children with haemophilia A and antibodies to factor VIII, 18 bleeding episodes were randomized for treatment with factor VIII concentrate (30 units/kg) and 18 for treatment with a prothrombin-complex concentrate (prothrombinex) given in a dose of 30 units of factor IX/kg. Treatment with prothrombinex was associated with a better clinical response, a significantly greater shortening of the kaolin partial thromboplastin time and significantly lower incidence of post-infusion increase of levels of factor VIII antibodies. Although treatment with factor VIII concentrate was clinically successful in 15 episodes, treatment failures occurred in three instances leading to parental request for withdrawal from study in two families.
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PMID:A randomized study of factor VIII or prothrombin complex concentrate infusions in children with haemophilia and antibodies to factor VIII. 28 89

Severe rectal bleeding in a 6-year-old boy with haemophilia A and factor VIII inhibitors could not be stopped with factor VIII concentrates. But a good effect was achieved with activated prothrombin complex concentrates (fraction FEIBA), given over eight days. Amaurosis occurred as a complication after injection of the first dose, but disappeared completely within several minutes. Tests revealed accelerated intravascular coagulation with increased fibrin monomers and fibrin/fibrinogen degradation products.
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PMID:[Emergency treatment of haemophilia A with factor VIII inhibitors using activated prothrombin complex concentrates (author's transl)]. 30 53

Factor VIII inhibitor levels were measured on 261 occasions in 76 hemophilia-A inhibitor patients before and after prothrombin complex concentrate infusion at 13 treatment centers. A rise in inhibitor level to at least twice the pretreatment value occurred in 35 treatment episodes (13.5%), in 27 patients (36%). In 16 patients (21%), such an anamnestic immune response occurred with the first treatment. Factors predisposing to anamnestic responses may include patient idiosyncrasy, low pretreatment inhibitor levels, and exposure to concentrate over several days.
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PMID:Effect of prothrombin complex concentrates on factor VIII inhibitor levels. 50 42

Nine patients with severe classic hemophilia and inhibitors against factor VIII were treated for 156 bleeding episodes with 503 infusions of Proplex, Konyne, or Auto-Factor IX, three preparations of prothrombin complex concentrates (PCCs). Approximately two thirds of the bleeding episodes were managed successfully. Although the prothrombin time (PT) and partial thromboplastin time (PTT) were shortened after most PCC infusions, there was no evidence of disseminated intravascular coagulation. The degree of shortening of PT or PTT was not related to the particular PCC preparation used, dose, or cessation of hemorrhage. All PCC preparations contained activated clotting factors, as manifested by their ability to shorten the PTT of normal plasma, factor-VIII-deficient plasma, and factor-IX-deficient plasma. Shortening, which was greater with Auto-Factor IX than with the other products, was inhibited partially by a factor IX antibody and blocked completely by prolonged incubation with plasma. Although the nature of the procoagulant material in PCCs is uncertain, these products are of proven benefit to hemophilic patients with high-titer inhibitors. Side effects have been minimal and inhibitor titers have not risen.
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PMID:Use of prothrombin complex concentrates in hemophiliacs with inhibitors: clinical and laboratory studies. 72 19

A rather large proportion of the hemophilia B patients can be characterized as hemophilia B+ because of the presence in their plasma of a protein which is immunologically identical with human factor IX. In a group of 33 hemophilia B patients we found 14 cases of hemophilia B+ belonging to 11 independent pedigrees. The variant factor IX molecules of these families have been compared with respect to the following properties: 1) factor IX activity and its dependence on phospholipid concentration; 2) factor IX antigen; 3) prolongation of prothrombin time with an ox brain thromboplastin; 4) electrophoretic mobility; 5) Ca(+) binding capacity; 6) affinity for binding to heparin and 7) susceptibility of the factor IX antigen to contact-induced activation. In the study of these parameters the use of a precipitating antibody against highly purified human factor IX showed to be of great value. According to our criteria at least 7 different factor IX variants were present in the 11 families with hemophilia B+ studied. Because of this rather high heterogeneity a suitable nomenclature for subclassification of hemophilia B+ variants is proposed.
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PMID:The abnormal factor IX of hemophilia B+ variants. 73 33

In a 33-year-old man with haemophilia A, due to the presence of a factor VIII inhibitor, severe bleeding followed a tooth extraction. It could not be stopped by ordinary factor-VIII substitution or administration of activated prothrombin complex. Before starting substitution treatment it was necessary to decrease the inhibitor concentration in plasma. This was possible by plasma exchange with a blood-cell separator: an exchange volume of 4 litres proved to be sufficient. The inhibitor-containing plasma was replaced by fresh plasma and cryoprecipitate. It was possible to increase the haemostatic activity of the transfused factor VIII by combination with activated prothrombin complex. The inhibitor kinetics remained uninfluenced. A delayed plasmapheresis was without effect as a result of an antibody booster by previous factor-VIII substitution.
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PMID:[Treatment of haemorrhages in a haemophiliac with factor-VIII inhibitor (author's transl)]. 75 78

A Normotest (NT)-Thrombotest (TT) discrepancy is claimed to reflect the presence of coumarin-induced inhibitors or intravascular coagulation, or both. The results of this study indicate, however, that a significant NT-TT discrepancy is also present in all plasmas from patients who have congenital coagulation disorders of the prothrombin complex. None of these patients had received an anticoagulant or showed any sign of intravascular clotting; nevertheless the discrepancy observed was similar to that found in plasmas of coumarin-treated patients: average values were 0.437 and 0.450, respectively. As no inhibitor was present in the congenital coagulation disorders, except in hemophilia BM, the phenomenon does not appear to be specific for coumarin plasma. This indicates that the NT/TT discrepancy is a non-specific phenomenon that does not seem to provide any additional information about coumarin-treated patients compared with that obtainable by means of a simple prothrombin time test.
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PMID:Normotest--thrombotest discrepancy in congenital coagulation disorders of the prothrombin complex and in coumarin-treated patients: a nonspecific phenomenon. 83 55

Twenty specialized centres for the care of hemophilia and allied disorders have now been established in Italy. Their geographic distribution is uneven and the number is considered inadequate. Financial support is provided by regional Health Departments or by University and General Hospitals; a bill recognizing hemophilia as a social disease entitles the centres to an annual grant from the State or Regional Boards. Less than half of factor VIII employed in the management of hemophilia A is supplied as a cryoprecipitate from hospital blood banks; the rest is imported from commercial manufacturers who also satisfy all the demand for prothrombin complex concentrates. Commercial freeze-dried concentrates can also be obtained free of charge at pharmacies by hemophiliacs covered by national social security schemes. This arrangement facilitates home treatment, which appears the ideal form of management in a country with the geographical features of Italy. In Lombardy, a bill allows hemophiliacs or their relatives to give infusions after adequate instruction. All this has been a great help in achieving more modern management of hemophilia; however, dependence on imported commercial concentrates appears unreasonable on a long-term basis, and major efforts should be directed towards developing a national program for manufacturing blood components.
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PMID:Hemophilia care in Italy. 98 58


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