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PubMed2004

[Hemophilia and other inherited blood coagulation disorders in Poland].

The aim of the study was to analyze the data on 3224 patients with inherited blood coagulation disorders registered in Poland till December 1st, 2003. In 2269 registered hemophilia patients, 1953 were hemophilia A, and 316 were hemophilia B. Hemophilia A occurs in 1512 families, and hemophilia B in 240 families. Severe hemophilia constitutes the majority of hemophilia A and B cases (59.7% and 56.6% respectively). About 50% of the hemophiliacs have no history of bleeding diathesis in the family. The mean age of hemophilia A patients is 30.9 years, and that of hemophilia B patients--29.2 years. Prevalence of hemophilia in Poland is approximately 1:12 300 inhabitants. Hemophilia A has been diagnosed in 60.6% of all patients with inherited blood coagulation disorders registered in Poland, von Willebrand disease-- in 21.9%, hemophilia B-- in 9.8% and factor VII deficiency-- in 3.3%.

Adolescent↗
PubMed1993

[Participation of procoagulant activity of cultured rabbit lens epithelial cells in fibrin formation].

To clarify the participation of the lens epithelial cells in the postoperative fibrin formation in cataract surgery, the generation of procoagulant activity (PCA) by cultured rabbit lens epithelial (RLE) cells was investigated. PCA was detected in the homogenates of RLE cells cultured for three hours. PCA was increased approximately three- to four-fold by lipopolysaccharide (LPS) stimulation in a dose dependent manner. Treatment of the RLE cells with Concanavalin A resulted in a decrease of PCA. PCA of the RLE cells was not observed in Factor VII deficient plasma, as a standard tissue factor (TF). Parallelism was recognized between the dose response regression lines for the PCA in the RLE cells and standard TF. These results suggest that the PCA in the RLE cells is similar to TF, which has glycoprotein as an active site and requires coagulation factor VII. Some arachidonic acid metabolic cascade inhibitors which show an inhibitory effect for fibrin formation in vivo reduced the generation of PCA by the RLE cells in vitro. The activation of arachidonic acid cascade followed by the generation of PCA in the RLE cells may participate in postoperative fibrin formation.

Animals↗
PubMed1993

Buccal mucosa bleeding time is prolonged in canine models of primary hemostatic disorders.

Bleeding times are reported in many studies using canine models, with a variety of techniques employed to adapt these tests for dogs. We evaluated a canine model of template bleeding time, the buccal mucosa bleeding time (BMBT), by examining the test's sensitivity and specificity for defects of primary hemostasis. We examined thirty-five dogs having defined defects of either primary hemostasis (Types I, II, III von Willebrand's disease, thrombasthenia, thrombopathia) or secondary hemostasis (hemophilia A and B, Factor VII deficiency). Comparisons of BMBT and cuticle bleeding time were made in a subset of these dogs. All dogs having primary hemostatic disorders had long BMBT, and all factor deficient dogs had BMBT within normal range. The BMBT in canine models appears to be a specific and sensitive test of primary hemostasis; suitable for evaluating factors affecting template bleeding time and potential efficacy and thrombogenicity of treatment regimens.

Animals↗
PubMed1980

The congenital factor VII abnormalities (dysproconvertinemias). The genetic plot thickens.

Congenital factor VII deficiency is a well known clotting disorder first identified in 1951. Several congenital abnormalities of factor VII have been described during the past decade. These abnormalities were all characterized by the presence of a discrepancy between factor VII clotting activity and factor VII cross reacting material or antigen. Recently other factor VII abnormalities have been described which showed peculiar sensitivities to ox-brain thromboplastins as compared to thromboplastins of other origin. These discoveries have complicated the differential diagnosis of prothrombin complex factors deficiences and abnormalities. A correct diagnosis may be reached only by means of a battery of tests which employ tissue thromboplastins of different origin.

Animals↗
PubMed1991

Congenital factor VII abnormality discovered in an infant at a routine checkup.

Congenital coagulation factor VII abnormality in an infant was first discovered because of a decreased normotest value at a routine health checkup at the age of 1 month. No bleeding tendency had been noticed. The normotest value did not respond to an administration of vitamin K. The prothrombin time (PT) was prolonged, and the activated partial thromboplastin time (A-PTT) was in the normal range. Factor VII activity was extremely low, whereas the level of factor VII antigen was relatively low. These data indicate that the propositus is a factor VII reduced variant, a rare variant of congenital factor VII deficiency. The propositus and her brother are homozygotes, and her parents and sister are heterozygotes.

Blood Coagulation Tests↗
PubMed2001

Use of recombinant factor VIIa in hereditary bleeding disorders.

Recombinant factor VIIa is effective as a factor VIII or IX bypassing agent and is relatively safe for the management of bleeding and surgical procedures in patients with factor VIII or IX inhibitors (congenital or acquired hemophilia). It is one of several options in the overall treatment strategy for patients with these difficult conditions. This drug has also been used off label in a limited number of patients with other hereditary bleeding disorders (e.g., factor VII deficiency or antibodies to other clotting factors, and platelet disorders such as Glanzmann thrombasthenia). This paper reviews the proposed mechanisms of action, clinical effectiveness, and safety of rFVIIa for treatment of these disorders and points out areas that require further studies. With expanding indications and more widespread use, thromboembolic complications must be carefully monitored, especially in patients at risk for thromboembolism. The high cost of this drug may be a limiting factor.

Blood Coagulation Disorders, Inherited↗
PubMed1998

Antigenicity of activated recombinant factor VII followed through nine years of clinical experience.

Patients treated with activated recombinant factor VII (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) were followed for potential antibody formation. The recombinant product was used to control serious and mild-to-moderate bleeds in a hospital environment and mild-to-moderate bleeds at home. The 267 patients included 222 haemophilia A patients, 16 haemophilia B patients, 16 non-haemophilia patients with inhibitors, and 13 factor VII (FVII)-deficient patients. The individual exposure ranged from one to 121 episodes treated over a period of up to 6 years. Analysis for FVII antibodies in an immunoassay revealed that pre-treatment samples from 5% of the haemophilia A patients had values above the normal range. None of these reactions were specific. Increased post-treatment values were observed in two FVII-deficient patients; both patients had been previously treated with plasma-derived FVII. The FVII-specific antibodies reacted equally well with plasma FVII and rFVIIa. The overall result from antibody determination shows no indication of antibody formation against rFVIIa in haemophilia A or B patients or in non-haemophilia patients with acquired inhibitors; however, FVII-deficient patients represent a risk group for development of antibodies against FVII.

Adolescent↗
PubMed2003

Migraine, cortical blindness, multiple cerebral infarctions and hypocoagulopathy in celiac disease.

We describe the case of a female patient affected by migraine and untreated adult celiac disease who presented with a state of acute migraine accompanied by multiple neurological deficits, including transient cortical blindness with ischemic CT and MRI alterations, and hypocoagulation due to factor VII deficiency. She was receiving estroprogestin therapy. There was a prompt response to cortisone therapy followed by a state of complete well-being, which also led to the disappearance of migraine attacks after five years of dietary treatment alone.

Adult↗
PubMed2000

Current status of hemophilia patients and recombinant coagulation factor concentrates in Japan.

The first recombinant factor VIII concentrate was introduced in 1987 to treat hemophilia A patients, and the product was licensed in the United States in 1992. More than 10 years have passed since the recombinant products have been used for treatment of hemophilia A. The new therapeutic options seem to be safe and effective through the gathered experiences. Recently, recombinant factor VIIa concentrate has become available to treat hemophilia patients with inhibitor and factor VII deficiency patients in Europe and also recombinant factor IX for the treatment of hemophilia B has been licensed in the United States and Europe. The usage of recombinant coagulation factors has expanded the routine therapy for hemophilia in many countries. In Japan, the consumption of recombinant factor VIII is increasing year by year, because many patients have started to think that the recombinant technology seems to be safe. Unfortunately, though, the factor VIIa and factor IX products have not been licensed yet in Japan. This article discusses the current status of patients with hemophilia and recombinant coagulation factor products in Japan.

Animals↗
PubMed1989

Laboratory evaluation of hemorrhagic coagulopathies in small animal practice.

Assessment of animals with a suspected hemorrhagic diathesis of unknown cause(s) should be methodical. Most acquired coagulopathies result from thrombocytopenia. A platelet estimate (from a blood smear) and/or a platelet count on a fresh blood sample therefore are useful first steps in case evaluation. If thrombocytopenia is present, the most likely causes are immune-mediated destruction of platelets, DIC, or megakaryocytic hypoplasia. These diagnoses can be pursued by further test, including antiplatelet antibody assays (for example, the platelet factor 3 tests or an ELISA test), measurement of FDP, and bone marrow biopsy, respectively. If the platelet count is normal, a buccal mucosa bleeding time test is a useful second step. If this is prolonged, most likely causes are vWD or a thrombocytopathy (functional platelet defect). von Willebrand's disease can be diagnosed by measurement of vWf concentration or activity. A normal bleeding time does not exclude a diagnosis of vWD, but suggests that the functional activity of vWf is not compromised markedly. If the bleeding time is normal, APTT and PT should be measured. A prolonged APTT with normal PT, in the clinical setting, implies a deficiency of factor XI, IX, or VIII. A prolonged PT with normal APTT indicates factor VII deficiency. Prolongation of both APTT and PT usually is caused by a deficiency of several factors and is seen most often in cases with vitamin K deficiency or antagonism. Obviously, if a particular cause is suspected from the case history or for other reasons, appropriate tests should be evaluated at the beginning. If these do not confirm the provisional diagnosis, the just-described protocol might be a useful one to follow.

Animals↗
PubMed1995

Subgaleal hematoma with delayed proptosis and corneal ulceration.

We report the delayed occurrence in a child of proptosis caused by a subgaleal hematoma. A corneal ulcer resulted from the proptosis, which developed 9 days after mild head trauma. Evaluation revealed a heterozygous factor VII deficiency. The coagulopathy and, more likely, the use of the nonsteroidal antiinflammatory drug ibuprofen may have contributed to the extension of the hematoma into the orbital space. Careful follow-up of patients with subgaleal hematomas is necessary because the development of proptosis may be delayed.

Child↗
PubMed2000

Tissue factor and cancer procoagulant expressed by glioma cells participate in their thrombin-mediated proliferation.

The relationship between coagulation cascade activation and glioma cell proliferation was examined. The human glioma cell lines T98G, TM-1 and normal human astrocyte cell strain (NHA) were examined. Using anti-tissue factor (TF) antibody, immunocytochemical detection of TF antigen was obtained in both cell lines and cell strain. TF antigen in cell lysates was also measured by enzyme linked immunosorbent assay (ELISA). In a one-stage clotting assay, T98G, TM-1 and NHA revealed procoagulant activity (PCA) in normal human plasma and factor VII deficient plasma. PCA in normal human plasma was significantly inhibited by both inhibitory anti-TF antibody and cysteine protease inhibitor HgCl2. This result indicates that T98G, TM-1 and NHA cells express not only TF but also cancer procoagulant (CP) at the same time. In a cell proliferation assay, thrombin induced proliferation in T98G and TM-1 cells in a dose-dependent fashion and in NHA cell in a bell-shaped fashion. This mitogenic stimulant was inhibited by the specific thrombin inhibitor hirudin. The combinations of coagulation factors II, V, and X with or without factor VII induced proliferation in T98G, TM-1, and NHA cells. The maximal mitogenic stimulatory effects were larger in glioma cells than in NHA. These mitogenic stimulatory effects were also inhibited by hirudin. Each coagulation factor on its own or in any other combination of coagulation factors had no proliferative effect. Thus, these mitogenic stimulatory effects were considered to be the effect of thrombin. In conclusion, T98G and TM-1 human glioma cells express two different types of procoagulants TF and CP. In the presence of coagulation factors, these glioma cells can generate thrombin and this thrombin generation is capable of inducing glioma cell proliferation in vitro.

Antibodies↗
PubMed2005

Of four mutations in the factor VII gene in Tunisian patients, one novel mutation (Ser339Phe) in three unrelated families abrogates factor X activation.

Hereditary factor VII (FVII) deficiency is a rare bleeding disorder. Dysfunctional FVII variants characterized by normal or reduced levels of FVII antigen and discordantly low FVII activity have been described. In this study, seven unrelated Tunisian patients with FVII deficiency were examined. Molecular analysis revealed that three probands harbored a novel Ser339Phe mutation, one proband was inferred to have a novel splice site mutation in intron 2, c.226-2 A>G and three probands had two previously described mutations, Arg304Gln and Cys310Phe. Expression of Ser339Phe in baby hamster kidney cells yielded secretion of FVII antigen at a concentration of 225+/-50 ng/ml, compared with 181+/-47 ng/ml in cells transfected with wild-type FVII but with no demonstrable FVII activity. FVII Ser339Phe bound to tissue factor similarly to the binding of commercial recombinant activated FVII or recombinant wild-type FVII and was normally activated by activated factor X. The major defect of FVII Ser339Phe was its inability to activate factor X in the presence of tissue factor. Modeling predicted that the substitution of Ser339 by Phe abrogated substrate docking.

DNA Mutational Analysis↗
PubMed1981

Plasma thromboplastin antecedent (Factor XI) deficiency in a black family.

A black man with a prolonged partial thromboplastin time has a severe deficiency of plasma thromboplastin antecedent (PTA) (factor XI) measured both in clotting assays and immunoassays, suggesting a diagnosis of homozygous PTA deficiency. His offspring seemed to be heterozygous carriers of PTA deficiency. Additionally, the proband and two of his children had decreased Hageman factor (factor XII) levels consistent with those of heterozygous carriers of Hageman trait. To our knowledge, this is the first case known of PTA deficiency in a black person. Its pattern of inheritance was independent of that of factor VII deficiency.

Black People↗
PubMed1993

The role of tissue factor in the production of thrombin.

Tissue factor is now widely accepted to be the major physiological initiator of blood coagulation. The importance of this protein to normal haemostasis may be inferred from the clinical effects of factor VII deficiency and by the fact that congenital tissue factor deficiency has not been described. Tissue factor is a single polypeptide with an established amino acid sequence that may be divided into cytoplasmic, extracellular and transmembrane regions. There are no close structural similarities with other proteins although tissue factor may be a member of the cytokine receptor family. This paper reviews current knowledge of the role of tissue factor in the production of thrombin with particular reference to its expression, location, control and relevance to haemostasis.

Amino Acid Sequence↗
PubMed1990

Vitamin K-dependent multifactor coagulopathy in Devon Rex cats.

A coagulopathy attributable to a deficiency of vitamin K-dependent clotting factors (II, VII, IX, and X) was diagnosed in 3 Devon Rex cats. There was no evidence for exposure to vitamin-antagonist-related rodenticides. The cats did not have evidence of hepatic disease, gastrointestinal disease, or fat malassimilation. Oral treatment with vitamin K1 resulted in normalization of clotting factor concentrations. However, when treatment was discontinued in 2 cats, prothrombin and activated partial thromboplastin values became prolonged again, although the cats did not have clinical signs of a bleeding disorder.

Animals↗