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[Wilson's disease in personal material--disturbances in hemostasis].

Genetically determined impairment of copper excretion from the liver into the bile in Wilson's disease (WD) cause that "free copper" is accumulated in toxic amounts not only in the liver, but also in other organs. In WD liver biopsy often could not be made because of serious disturbances in hemostasis. The aim of the study was: a) to demonstrate our 9 patients with various form of WD. b) to examine some blood clotting factors and compare the results with these obtained in other liver diseases. The diagnosis of Wilson's disease was made on the basis of disturbed copper metabolism. Among our 9 patients (8 women and 1 man, between 17-33 years old) we diagnosed: 3 patients with fulminant Wilson's disease with all day deep jaundice, hemolytic anemia, haemorrhagic diathesis and liver failure, died, 2 patients with active chronic hepatitis, hemolytic anemia and haemorrhagic diathesis, 2 patients with liver cirrhosis, haemorrhagic diathesis, Kayser-Fleisher ring, neuropsychiatric syndrome, 2 asymptomatic patients without haemorrhagic diathesis. The prothrombin index and the factors of prothrombin stem (II, V, VII, X) were lower than in other kinds of cirrhosis. After treatment with d-penicillamine the clothing factors returned near to the norm, similar as the biochemical and immunological results.

Adolescent↗

[Factor XI deficiency: do patients with hemorrhagic diathesis also have hemostasis defects?].

It is well known that the extent of bleeding in patients with a deficiency of factor XI does not parallel the residual factor activity. Therefore, additional hemostatic aspects must be taken into consideration. 27 patients of 18 different families--6 with severe (FXI < 0.01 to 0.09 U/ml) and 21 with moderate (FXI 0.20 to 0.252 U/ml) factor XI deficiency--were reinvestigated regarding hemostasis including bleeding time and platelet function. 16 had an enhanced bleeding tendency, while the others--including 3 with severe FXI deficiency of < 0.01 U/ml--never suffered from bleeding complications despite delicate surgery in some. All 16 of the symptomatic patients had, besides the reduction of factor XI activity to various extents, an additional hemostatic defect: 3 had a moderate alpha/delta storage pool detect of the platelets, 11 a platelet anomaly mainly characterized by reduced ADP aggregation and 2 an isolated prolonged bleeding time of unknown cause. Synthesis of platelet thromboxane was unimpaired in all patients as tested by formation of malondialdehyde after stimulation with N-ethyl maleimide. Hence, in patients with a known factor XI deficiency diagnostic and prophylactic measures before surgery should concentrate on such additional findings. Preoperative administration of desmopressin, and not replacement of factor XI, will be the treatment of choice in these cases.

Adult↗

Effect of tamoxifen on measurements of hemostasis in healthy women.

BACKGROUND: Tamoxifen citrate is being evaluated for primary prevention of breast cancer, but this drug with estrogen-like properties may cause changes in the hemostatic system that would increase the risk of thrombosis. METHODS: Women who had undergone hysterectomy were consecutively enrolled in the placebo-controlled, randomized, double-blind Breast Carcinoma Chemoprevention Tamoxifen Study, which was designed to evaluate the efficacy of oral tamoxifen citrate (20 mg/d). Our substudy of hemostasis and lipid measurements included the first 68 consecutive women assigned to tamoxifen (n = 31) or placebo (n = 37). Blood specimens were obtained before treatment and after 1,2,4, and 6 months of treatment. Measurements included blood cell counts, lipid levels, coagulation activation markers, clotting factors, and anticoagulant and fibrinolysis proteins. RESULTS: Hematocrit and hemoglobin and platelet levels fell slightly but significantly in women treated with tamoxifen. No between-treatment differences were observed in any of the clotting factors. Naturally occurring anticoagulant proteins such as antithrombin and protein C fell slightly in women treated with tamoxifen. However, no significant changes were observed in any of the markers of activated coagulation or fibrinolysis (fibrinopeptide A, prothrombin fragment 1 + 2, thrombin-antithrombin complex, D-dimer). Total and low-density lipoprotein cholesterol levels fell significantly in women treated with tamoxifen. CONCLUSIONS: Tamoxifen induced a modest decrease in anticoagulant proteins, but without biochemical signs of activation of coagulation and fibrinolysis. Tamoxifen improved the lipid profile and induced changes in blood cell counts, which should determine an improvement in blood rheologic factors. These preliminary findings seem to justify continuation of the double-blind study in healthy women, but only direct comparison of thromboembolic complications in the 2 treatment groups will establish whether tamoxifen carries a risk of thrombosis.

Administration, Oral↗

[The cause of upper gastrointestinal bleeding and the value of emergency endoscopic hemostasis].

Introduction of endoscopy and endoscopic hemostasis had substantial impact on the treatment of diseases of stomach and duodenum complicated by bleeding. We compared two 4-years' periods analysing etiology of bleeding and influence of endoscopic haemostasis on outcome of patients with upper gastrointestinal haemorrhage. Number of patients in both groups were comparable. Ratio of bleeding related to gastric and duodenal ulcer didn't change substantially. Number of patients with haemorrhagic gastritis decreased somehow while those with bleeding oesophageal varies increased. Number of patients submitted to emergency operation for bleeding ulcer decreased significantly. Mortality was lower in the second period of time only in patients with haemorrhage requiring transfusion of 4-7 units of blood.

Emergencies↗

[Efficacy of the MIDCAB doughnut: a new instrument for immobilization and hemostasis during MIDCAB].

A new instrument for the immobilization and hemostasis of an anastomotic site during off-pump bypass is described. The instrument called "MIDCAB doughnut" was used safely and accurately in 31 patients who underwent MIDCAB without CPB. In these, the instrument was applied in 44 anastomotic sites on the beating heart (30 left anterior descending, 7 right coronary, and 7 obtuse marginal arteries). It can make an operative field motionless and bloodless without distal snaring, because of air sucked fixation of an entire circuit of the anastomotic site. There was no operative and hospital deaths. Postoperative angiography revealed a 100% patency rate of the grafts. MIDCAB doughnut can improve a quality of the anastomosis during beating bypass surgery.

Cardiac Surgical Procedures↗

Hemostasis in acquired hemophilia--role of intracavitary instillation of EACA.

An 82 year old man developed antibodies against coagulation factor VIII:C without any apparent cause. Bleeding from the soft tissue cavity could not be controlled by factor VIII:C concentrates, immunosuppression with steroids and intravenous immunoglobulin therapy in the standard dosages. However, a single injection of Epsilon Aminocaproic Acid (EACA) instilled into the cavity under aseptic precautions achieved lasting hemostasis with resultant wound healing.

Aged↗

Murine tumor cell activity on in vitro hemostasis.

The procoagulant activity of various murine tumor cells was investigated. This activity is different from tumor to tumor in quality and strength. This parameter must be taken into account in any experiment designed to explore the metastatic spread or to cure tumors, including the use of drugs that modify hemostasis.

Animals↗