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Results for “CHRISTMAS DISEASE”

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At least 235 records · Page 13Linked to original sources
PubMed1986

Danazol therapy in haemophilia.

Three patients with severe haemophilia A and one patient with moderately severe Christmas disease were given danazol and placebo in a double blind cross over trial to study the effects on haemostatic variables and bleeding tendency. In each case there was a shortening of the activated partial thromboplastin time (APTT) and euglobulin clot lysis time (ECLT) during danazol therapy but factor VIII and IX levels did not show any consistent change and there was no improvement in the bleeding tendency.

Adult↗
PubMed1985

Effect of stanozolol on factors VIII and IX and serum aminotransferases in haemophilia.

The treatment of haemophilia has been dramatically improved since the introduction of factor VIII and IX concentrates, however these concentrates have brought new problems such as hepatitis and A.I.D.S. An oral agent which could raise endogenous levels of factor VIII and IX would be of great benefit. Danazol, an anabolic steroid, has recently been shown to increase levels of factors VIII and IX in haemophilia. We therefore studied the effect of stanozolol, a closely related anabolic steroid, in 15 patients with haemophilia A or Christmas disease over a 2-4 week period. There was no consistent change in factor VIIIc or factor IX, and fibrinolysis was significantly enhanced. No effect was apparent on the incidence of spontaneous bleeds. However serum aminotransferases which were abnormal in 11 of the 15 patients at the start of the study fell significantly with stanozolol therapy. This raises the interesting possibility that anabolic steroids may be beneficial in patients with chronic liver diseases.

Blood Viscosity↗
PubMed1985

Effect of danazol on coagulation parameters and bleeding in hemophilia.

Danazol was given orally at a dose of 600 mg/day to six hemophiliacs for eight to 14 weeks. All patients showed a significant decrease in activated partial thromboplastin time (APTT) beginning with the first measurement (two weeks) and persisting until use of the drug was discontinued. However, a corresponding increase in the deficient factor activity could not be consistently demonstrated. Despite the shortened APTT, bleeding episodes continued in the severe hemophiliacs and the patient with Christmas disease. In four patients, bleeding appeared to increase in severity or change in pattern, and in two cases the bleeding manifestations did not respond to usual factor infusions but responded to discontinuation of the drug therapy and further factor replacement. Euglobulin lysis times were measured in five patients (one hemophiliac and four with nonhemophilic conditions) who were receiving danazol. The lysis times were markedly shortened. Increased fibrinolytic activity may be responsible for the increased bleeding manifestations in danazol-treated hemophiliacs.

Adult↗
PubMed1971

The activated coagulation time of whole blood as a routine pre-operative sceening test.

Patients with disorders of hemostasis who undergo surgical procedures are in danger of hemorrhage. While the careful medical history remains the most sensitive test of a bleeding tendency, some such patients can give no suggestive history. In three patients with coagulopathy-one with mild classical hemophilia, one with Christmas disease, and one with warfarin toxicity-the abnormality was missed by routine preoperative history but promptly detected by the routine preoperative use of the activated coagulation time (act). Either this test or the activated partial thromboplastin time should be included in the routine preoperative work-up, along with appropriate additional tests of the hemostatic mechanism.

Adult↗
PubMed1984

Mild bleeding disorders: review of 120 patients.

Of 120 patients presenting with mild bleeding disorders, 63 were found to have a definite coagulopathy. The commonest disorders were haemophilia, Christmas disease and von Willebrand's disease (vWd), the latter being predominant. Diagnosis led to prophylactic treatment prior to surgery in 18 patients with prevention of excessive haemorrhage. Three patients who had received blood products developed hepatitis. DDAVP (desamino-cys-1-8-D-arginine vasopressin) is the treatment of choice in suitable mildly affected patients with haemophilia A and vWd. Examination of blood group distribution suggests an excess of group O among patients with bleeding disorders, especially those with vWd.

Adult↗
PubMed1983

The management of musculoskeletal problems in hemophilia. Part I. Principles of medical management of hemophilia.

Musculoskeletal bleeding in general and arthropathy in particular are the central problems among many in the two major forms of hemophilia: classic hemophilia, caused by factor VIII deficiency, and Christmas disease, caused by factor IX deficiency. Currently available replacement therapy, if properly used in a multidisciplinary setting, should provide significant benefit to hemophilic patients despite its cost and occasional complications.

Anemia, Hemolytic↗
PubMed1980

Management of common congenital disorders of haemostasis.

The management of haemophilia, Christmas disease and von Willebrand's syndrome are reviewed. The principal advances in diagnosis are the measurement of procoagulant activity, the ristocetin cofactor and the levels of the protein associated with procoagulant activity by immunologic methods. The mainstay of treatment is replacement of the coagulation factors by partially or highly purified blood products.

Antibodies↗
PubMed1982

Preparation of factor IX deficient human plasma by immunoaffinity chromatography using a monoclonal antibody.

A murine hybridoma clone is described that grows continuously in culture and produces a monoclonal antibody we have called Royal Free Monoclonal Antibody to factor IX No. 1 (RFF-IX/1). This has high affinity for a coagulation site on factor IX. RFF-IX/1 immobilised on sepharose can be used to deplete factor IX from normal human plasma. This immunoaffinity depleted plasma is indistinguishable from severe Christmas disease plasma and can be used as the substrate in a one stage coagulation assay for factor IX. The affinity column has high capacity and can be regenerated so that large scale production from normal plasma of factor IX deficient plasma as a diagnostic reagent is now feasible.

Animals↗
PubMed1976

[Prophylactic replacement therapy in hemophilia. A case report (author's transl)].

In a six year old boy with severe hemophilia prophylactic substitution of factor VIII was started in 1973 in order to prevent early invalidity due to series of bleeding in the right anklejoint. A substitution of factor VIII over 8 months with 21 resp. 30 U/kg body-weight did not lead to a significant improvement. But since the factor VIII in a dosage of 18 U/kg body-weight is given three times a week no bleeding occurred during the treatment time of 14 months and also the ability to walk improved to an excellent degree.--So far no signs of hepatitis or an factor-VIII-antibody could be detected.--Some results from the prophylactic treatment of severe hemophilia and Christmas disease are cited from the literature.

Child↗