摘要:

Patients with the hypereosinophilic syndrome (HES) are at increased risk of thrombosis and have signs of fibrin deposition in the myocardial cavity; the pathogenesis of these complications is still unknown. We have studied a 51-year-old man affected by HES with heart, lung, skin, and gastrointestinal involvement. Routine laboratory parameters of the hemostatic system were normal with the exception of blood fibrinolytic activity. The latter was evaluated by both diluted blood clot lysis time and euglobulin lytic activity on fibrin plates before and after 10 min venous occlusion. The fibrinolytic activity measured on four occasions during a 3-month period, was impaired both in basal conditions and following venous occlusion. Platelet studies on two different occasions before and during therapy showed spontaneous platelet aggregation, lowered threshold concentrations of various aggregating agents, reduced platelet regeneration time and increased plasma β-thromboglobulin concentration. The patient’s polymorphonuclear cells (more than 75% eosinophils) were devoid of any procoagulant activity (PCA). Instead, patient’s mononuclear cells studied before therapy generated significantly higher PCA on stimulation by endotoxin than cells from control subjects. The procoagulant response to endotoxin decreased markedly during therapy. The observed abnormalities could, at least partially, contribute to fibrin deposition in

ISSN:1424-8832    

DOI:10.1159/000215886

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

In three different studies we tested the hypothesis that early-onset vascular disease is associated with impaired homocysteine metabolism which could contribute to the development of arteriosclerosis and thrombosis. In patients with occlusive vascular disease before the age of 60, a post-methionine load increase of plasma homocysteine exceeding the highest value for comparable healthy control subjects was found in 1 of 21 with myocardial infarction (5%), 14 of 37 with aorto-iliac disease (38%), and 17 of 53 with cerebrovascular disease (32%). This might indicate heterozygosity for homocystinuria due to cystathionine β-synthase deficiency. Concentrations of serum vitamin B12 and red cell folate had an important modulating effect on plasma homocysteine concentrations in the fasting state

ISSN:1424-8832    

DOI:10.1159/000216094

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

A more concentrated desmopressin (DDAVP) preparation (40 μg/ml), which required small injection volumes (< 1 ml), was studied in a double-blind trial in 10 healthy volunteers, 12 patients with haemophilia A, and 8 patients with uraemic bleeding. DDAVP was administered by subcutaneous injection at a dose of 0.4 μg/kg body weight. In healthy subjects, peak levels of DDAVP ranging from 480 to 638 pg/ml were reached 1 h after the subcutaneous injection and DDAVP was eliminated with a mean half-life of 3.1 h. DDAVP produced a 2.5-fold (3.0-fold) increase of factor VIII·C (factor VIII·Ag) and a 1.9-fold (2.2-fold) increase of von Willebrand factor:Ag (ristocetin cofactor) over baseline levels. Additionally, a 2.1-fold increase of tissue-type plasminogen activator antigen was observed. Factor VIII and von Willebrand factor were rapidly eliminated with a half-life ranging from 1.3 to 5.7 h and from 1.1 to 11.4 h, respectively. In haemophilia A patients, DDAVP produced a 2.3-fold increase of factor VIII·C 1 h after the injection. DDAVP was given on 8 occasions for management of bleeding, and only in 1 patient did a wound haematoma (after herniotomia) occur. In 7 of the 8 patients with uraemia the bleeding time shortened, and in all patients an increase of platelet retention and a decrease of platelet count was observed (p < 0.05). No serious local or systemic untoward side effects were observed.

ISSN:1424-8832    

DOI:10.1159/000216097

出版商:S. Karger AG    

年代:1989

数据来源: Karger

摘要:

Protein-C activity and antigen were measured in 141 full-term infants during the first month of life. The levels of both protein-C activity and antigen were about one third the level for normal adults in cord blood, and significantly lower than the cord blood during the 1st to 2nd days of life. They increased with age progressively, but did not reach the lowest limit in normal adults even in the first month. The low ratio of protein-C activity and antigen was demonstrated in some infants within the first 4 days of life. The precipitin arc of neonatal infants, which had a discrepancy between protein-C activity and antigen levels, showed an anodal shift upon agarose gel electrophoresis in the presence of Ca2+. The abnormal protein C in the neonatal period may be regarded as protein induced by vitamin-K absence or antagonist.

ISSN:1424-8832    

DOI:10.1159/000215887

出版商:S. Karger AG    

年代:1989

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000216096

出版商:S. Karger AG    

年代:1989

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000215888

出版商:S. Karger AG    

年代:1989

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000215889

出版商:S. Karger AG    

年代:1989

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000215890

出版商:S. Karger AG    

年代:1989

数据来源: Karger

ISSN:1424-8832    

DOI:10.1159/000216089

出版商:S. Karger AG    

年代:1989

数据来源: Karger