Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019087 (hemorrhagic diathesis)
 678 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Factor XI deficiency, also called hemophilia C, was first described in 1953. It is thought to constitute 7% of all disorders of the intrinsic pathway of blood coagulation. It represents the only deficiency of a contact phase protein associated with a bleeding tendency with widely variable expression. Eighteen members of 5 families with this deficiency have been investigated; only 3 exhibited a hemorrhagic diathesis: 2/4 homozygous (hemorrhages after surgical procedures and hematomas and after minor traumatism) and 1/10 heterozygous individuals (hematomas, epistaxis). All homozygotes had a prolongation of the PTT. The hemorrhagic tendency was higher among homozygous individuals (with very low factor XI activity), but 2 were asymptomatic. Heterozygotes may also bleed. This deficiency is found mainly in an Ashkenazy Jewish population. Our study shows that it occurs in an apparently autochthonous Swiss population. A detailed history and studies of other family members are necessary if there is suspicion of factor XI deficiency.
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PMID:[Hemophilia C, a deficiency of a contact phase protein which may involve a risk of hemorrhage]. 154 75

In children with bleeding tendency it is a dilemma to go in for sports. The risks and benefits are to be weighed against each other. Mostly there is no need to impede the child's activities. Several sports that safely can be played by children with hemorrhagic diathesis are summed up. Specific aspects of sports by children with thrombocytopenia, Von Willebrand's disease, hemophilia and thrombasthenia are discussed.
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PMID:[sports by children with a bleeding tendency]. 221 95

Bleeding disorders are frequent indications for reference to Hematologic Clinics. Won Willebrand's disease (vWD) is one of the commonest of inheritable hemorrhagic diathesis. In order to evaluate the frequency and clinical characteristics of vWD in the IX Region of Chile, we performed a clinical and laboratory investigation in 132 patients, children and adults, over a period of 3 years: 1983-1985. Within this group there was 26 close relatives. The laboratory tests used for the diagnosis were: bleeding time, factor VIII, von Willebrand factor and ristocetin cofactor. Diagnosis of vWD was made in 83 cases, most of them presenting the mild form of the disease. Two children had the severe form. The estimated prevalence of vWD in symptomatic patients in the IX Region of Chile is at least 113 per million, which makes it 3 or 4 times more frequent than hemophilia. vWD is observed at a much lower rate in mapuche than in caucasian population. The symptoms and their frequency were: post partum bleeding 75%, dental extraction bleeding 65%, surgical bleeding 65%, epistaxis 63%, menorrhagia 54%, easy bruising 45%, deep hematomas 8% and petechiae 5%. Five cases were asymptomatic. The bleeding time was the test most frequently abnormal: 72/83 cases (87%).
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PMID:[Von Willebrand's disease in the IX Region of Chile]. 251 97

The authors summarize the operative experiences performed with CO2 laser in 352 cases of oral surgery with special regard to the hemostatic effect. One of the patients suffered from hemophilia, 5 from thrombocytopenia and 5 from hypoprothrombinemia. Of the alterations to be eliminated 128 were especially inclined to hemorrhage (hemangioma, epulis, granuloma fissuratum). The operations were carried out with out-patients in local anesthesia. Due to the hemostatic effect of the laser the surgery could be performed in dry operative areas, the laser knife coagulates in the course of cutting the small vessels. Both in the group of patients with hemorrhagic diathesis and in the group of operation of alterations inclined to bleeding the experiences were highly favourable. No significant differences were found between the group of patients with hemorrhagic diathesis and other patients in the respect of the duration of operation, degree of bleeding, healing of the wound and complication. CO2 laser of high energy was found to be well applicable in the field of oral surgery owing to its excellent hemostatic effect, especially in operations where the patient or the alteration to be removed (possibly both) are disposed to bleeding.
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PMID:[Significance of the hemostatic effect of lasers in oral surgery]. 281 53

Rarely, a patient develops an antibody against factor VIII coagulant activity. The resultant hemorrhagic diathesis is clinically distinct from inherited hemophilia, being characterized by few hemarthroses but frequent skin and other soft-tissue hematomas. Hematuria may be troublesome. These patients represent therapeutic challenges. This study is one institution's results with 16 such patients followed up over an average of 31 months (range, four to 120 months; median, 19 months). It describes the largest group from a single institution receiving essentially no immunosuppressive agents, yet has one of the better overall results. Two patients experienced fatal hemorrhage and five patients underwent spontaneous remission. Long-term survival is not incompatible with persistence of the inhibitor. We conclude that this hemorrhagic diathesis is clinically distinct, less fatal than usually perceived, and may undergo spontaneous remission. Clearly, there is no mandate for any particular therapeutic regimen, such as immunosuppression, in the attempt to rid the patient of the antibody.
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PMID:Acquired hemophilia. A natural history study of 16 patients with factor VIII inhibitors receiving little or no therapy. 310 41

A 65-year-old man was admitted to the hospital because of extensive spontaneous ecchymoses of the trunk and huge hematomas of the arms and legs. He had no personal or family history of a hemorrhagic diathesis. Coagulation studies revealed a prolonged APTT, no detectable factor VIII:C activity, and a high titer of anti-factor VIII:C antibodies. A diagnosis of acquired hemophilia was made. No underlying disorder could be found. The inhibitor was an IgG antibody. Long-term management of bleeding including immunodepletion by plasma exchange and immunosuppression by corticosteroids and cytotoxic drugs alone and in combination had no effect on the bleeding tendency and coagulation data. The administration of Cs (10 mg/kg/d) in combination with prednisone induced a remission. After a stormy course and a 5-month stay in the hospital the patient could be discharged. A relapse occurred after the Cs and prednisone dosages were reduced. Increasing the Cs dosage induced a remission again.
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PMID:A patient with a spontaneous factor VIII:C autoantibody: successful treatment with cyclosporine. 313 76

A patient is reported in whom a meningioma of the lateral one-third of the sphenoid ridge was completely removed and long-term prophylaxis for seizures with diphenylhydantoin was prescribed. One and a half years later, a powerful inhibitor developed that specifically neutralized Factor VIII, the antihemophilic factor, and caused an acquired state of hemophilia. The 4-month hemorrhagic disorder was characterized initially by painless hematuria and later by intracerebral and extradural hematomas at the operative site of the previously excised meningioma. Despite the transfusion of massive quantities of concentrates of clotting factors, and the surgical evacuation of the recurrent hematomas on two occasions, the localized bleeding could not be staunched and the patient died. The types of inhibitors that cause acquired hemophilia and their modes of treatment are examined. Although it is possible that the Factor VIII inhibitor in this patient was induced by the meningioma, most previously reported tumors associated with acquired hemophilia have had an immunological basis. The most probable explanation for the acquired hemophilia in this patient was an inhibitor to Factor VIII from an autoantibody induced by the long-term use of diphenylhydantoin.
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PMID:Acquired hemophilia, meningioma, and diphenylhydantoin therapy. 677 40

A case of a rare post-traumatic complication of haemophilia A is reported. Similarly as other authors an atypical clinical course and growing internal hydrocephalus were observed in the present case. Haemorrhagic diathesis prolonged only the period of healing of the surgical wound.
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PMID:[Post-traumatic hematoma of the posterior cranial fossa in a 4-year-old boy with hemophilia A]. 725 69

A hemophilia care program should be able to provide comprehensive medical care to the person with hemophilia and fundamental to the provision of such care is the ability to provide adequate hemostatic support to the hemophilic person with his congenital hemorrhagic diathesis. Development of such a program depends on the presence and commitment of key medical and paramedical personnel and on the recognition of the fact that hemophilia exists in a particular country or community. Support programs ultimately require government assistance for provision of diagnostic facilities and most importantly for a blood transfusion service where production of blood and preparation of blood components for therapy can be undertaken. To maintain a hemophilia care program there should be viability and education of staff and an undertaking by governments for the provision of an on-going infrastructural financial support. As patients mature, new problems will arise, some of which may be a direct complication of hemophilia. Involved medical staff require support and continuing education to continue to provide essential support to the people with hemophilia. Finally, it must be stressed that hemophilia care does not stand alone but must be incorporated into the network of provision of health care based on an efficient and viable blood transfusion service.
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PMID:Development and maintenance of hemophilia programs in developing countries. 788 6

Von Willebrand's disease (vWd) and hemophilia are associated with hemorrhagic diathesis and disturbances in platelet aggregation to vessel wall. We compared the time course of thromboxane A2 (TXA2) formation by platelets during spontaneous clotting of blood of patients with von Willebrand syndrome and from patients with hemophilia A or B with that of healthy controls which were matched for sex, age and serum lipid status. In clotting blood of healthy females the TXA2 production rose at 37 degrees C in 60 min up to 228.2 +/- 32.3 ng/ml. In patients with vWd the TXA2 production at 60 min was significantly lower (129.1 +/- 26.7 ng/ml, p < 0/05). In hemophilia type A and B the TXA2 formation after 5-30 min was significantly diminished in comparison to healthy male controls (p < 0.05). From the diminished amount of TXA2 formed during spontaneous clotting of whole blood we conclude that the activation of platelets of patients with von Willebrand syndrome or hemophilia type A and B is diminished as compared to healthy controls possibly caused by reduced formation of thrombin in the blood coagulation process.
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PMID:von Willebrand's disease and hemophilia are associated with diminished thromboxane A2 (TXA2) formation in clotting whole blood. 814 8


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