1,720,971 research outputs found
Urokinase-type plasminogen activator release after DDAVP in von Willebrand disease: different behaviour of plasminogen activators according to the synthesis of von Willebrand factor.
No full textNine healthy volunteers and 23 patients with various types of von Willebrand disease were studied before and after DDAVP infusion. We investigated the behaviour of factor VIII/von Willebrand factor measurements, and of tissue plasminogen activator and urokinase-type plasminogen activator. In mild von Willebrand disease the increase of both plasminogen activators was similar to that seen in normal controls. A different fibrinolytic behaviour was found in the type I platelet low and in the type III von Willebrand disease patients. An impaired and absent fibrinolytic response to DDAVP was seen in the former and in the latter von Willebrand disease, respectively. A close relation between either u-PA and t-PA or von Willebrand factor was observed. The possibility of a linkage among these three proteins was discussed
Cerebral sinus venous thrombosis with parenchymal infarcts in a newborn with severe haemophilia A and subdural haematomas.
No full textThe case we report has three peculiar aspects
leading us to the following considerations:
1 This is the first reported case of cerebral SVT in a
haemophilic patient, either in the presence or
absence of cerebral haemorrhage, unrelated to
therapy or presence of CVC.
2 A complete recanalization of the venous sinuses
without heparin administration was observed,
probably favoured by the underlying disease.
3 MRI confirms to be the examination of choice in
the diagnosis of cerebral vascular diseases and in
the past the performance of CT alone in case of
cerebral haemorrhage in haemophiliacs may have
underdiagnosed the association with cerebral
venous thromboses
Risk factors, antithrombotic treatment and outcome in retinal vein occlusion: an age-related prospective cohort study
Full text linkObjectives:
Antithrombotic treatment for retinal vein occlusion (RVO) is controversial, although RVO has
been surmised as a predictor of a subsequent vascular event. We aimed to evaluate risk factors, the effects
of antithrombotic therapy and the occurrence of subsequent vascular events in patients with a first episode
of RVO, according to age of RVO onset.
Methods:
In this prospective cohort study, patients with central
(CRVO) and branch RVO (BRVO) confirmed by fluorescein angiography were studied; they were divided
according to age. Cardiovascular risk factors and thrombophilia were evaluated. Anticoagulants or aspirin
were given for at least 3 months
.
Patients were followed every 6
–
12 months and vascular events were
recorded.
Results:
One hundred CRVO and 32 BRVO patients were enrolled. Five of 60 (8.3%) patients
<
50 yr and 4/72 (5.5%) over 50 yr had a hereditary thrombophilic defect. One or more cardiovascular risk
factors were found in 35 (58%) patients of the younger group, and in 66 (91%) of the older group (
P
<
0.001).
Antithrombotic treatment led to both a satisfactory recanalization of occluded veins and visual acuity
improvement especially in younger patients. Vascular events occurred in 19 (14%) cases after 4
3.3 yr
from RVO, more frequently in older than in younger patients (22% vs. 5%,
P
=
0.005).
Conclusions:
Distribution of cardiovascular, but not of thrombophilic risk factors seems to be influenced by age in RVO
patients. Patients with a first RVO, especially those
>
50 yr, are likely at risk of a subsequent vascular event
The PAI-1 gene 4G/5G polymorphism and deep vein thrombosis in patients with inherited thrombophilia
No full textGenetic and acquired factors may influence phenotypic expression of inherited thrombophilia. Hypofibrinolysis due to excess PAI-1 can be found in patients with deep vein thrombosis (DVT) and 4G/5G polymorphism of the PAI-1 gene may modulate the inhibitor's synthesis. In 149 patients with inherited thrombophilia, the possible thrombotic contribution of both 4G/5G polymorphism and PAI-1 plasma levels was evaluated. Sixty-seven patients with idiopathic DVT and 98 normal subjects were also studied. By comparison with controls, a significantly higher prevalence of 4G/4G genotype was seen in idiopathic DVT and in thrombophilia patients, although in this latter group the difference only remained significant in cases symptomatic for thrombosis (p = 0.01). The 4G/4G genotype was associated with a greater risk of thrombosis both in symptomatic thrombophilia patients (OR 2.85, 95% CI 1.26-6.46) and in idiopathic DVT patients (OR 3.1, 95% CI 1.26-7.59). The greater frequency of 4G allele in symptomatic thrombophilia patients with respect to controls was statistically significant (p = 0.04). Compared to healthy subjects, PAI-1:Ag levels were higher in symptomatic thrombophilia patients and related to the 4G/5G polymorphism, with significantly higher values in the 4G/4G carriers. In conclusion, PAI-1 4G/5G polymorphism may influence PAI-1 expression and thrombotic risk in patients with inherited thrombophilia
High dose of human plasma-derived FVIII-VWF as first-line therapy in patients affected by acquired haemophilia A and concomitant cardiovascular disease: four case reports and a literature review
No full textPotential role of thrombelastography in the monitoring of acquired factor VIII inhibitor hemophilia A: report on a 78-year-old woman with life-threatening bleedings.
No full textAssociation between ABO blood group and bleeding phenotype in patients with mild rare bleeding disorders
No full textGlobal hemostatic profiling in patients with decompensated cirrhosis and bacterial infections
Full text linkBACKGROUND & AIMS: Bacterial infections in cirrhosis are associated with increased bleeding risk. To assess the factors responsible for bleeding tendency in patients with bacterial infections, we conducted a prospective study comparing all 3 aspects of hemostasis (platelets, coagulation, and fibrinolysis) in hospitalized patients with decompensated cirrhosis with vs. without bacterial infections. METHODS: Primary hemostasis assessment included whole blood platelet aggregation and von Willebrand factor (VWF). Coagulation assessment included procoagulant factors (fibrinogen, factor II, V, VII, VIII, IX, X, XI, XII, XIII), natural anticoagulants (protein C, protein S, antithrombin) and thrombomodulin-modified thrombin generation test. Fibrinolysis assessment included fibrinolytic factors (plasminogen, t-PA, PAI-1, α2-AP, TAFIa/ai) and plasmin-antiplasmin complex (PAP). RESULTS: Eighty patients with decompensated cirrhosis were included (40 with and 40 without bacterial infections). Severity of cirrhosis and platelet count were comparable between groups. At baseline, patients with cirrhosis and bacterial infections had significantly lower whole blood platelet aggregation, without significant differences in VWF. Regarding coagulation, bacterial infections were associated with reduced procoagulant factors VII and XII, and a significant reduction of all natural anticoagulants. However, thrombomodulin-modified thrombin generation was comparable between the study groups. Finally, although mixed potentially hypo-fibrinolytic (lower plasminogen) and hyper-fibrinolytic (higher t-PA) changes were present in bacterial infections, a comparable level of PAP was detected in both groups. Upon resolution of infection (n = 29/40), platelet aggregation further deteriorated whereas coagulation and fibrinolysis factors returned to levels observed in patients without bacterial infections. CONCLUSION: In hospitalized patients with decompensated cirrhosis, bacterial infections are associated with reduced whole blood platelet aggregation and a significant decrease of all natural anticoagulants, which may unbalance hemostasis and potentially increase the risk of both bleeding and thrombosis. LAY SUMMARY: Bacterial infections are a common issue in hospitalized patients with decompensated cirrhosis (i.e. patients hospitalized due to severe complications of advanced chronic liver disease). Patients with decompensated cirrhosis who acquire infections may be at increased risk of bleeding complications following invasive procedures (that is a procedure in which the body is penetrated or entered, for instance by a needle or a tube). As bleeding complications in decompensated cirrhosis are associated with a high risk of further decompensation and death, there is an urgent need to understand the factors responsible for such increased bleeding tendency. Herein, we investigated the alterations of hemostasis (that is the physiological process responsible for clot formation and stability) in patients with decompensated cirrhosis and bacterial infections. We found that development of bacterial infections in these patients is associated with alterations of hemostasis (particularly of platelets and clotting cascade) that may increase the risk of both bleeding and thrombotic complications
Activated prothrombin complex concentrate (FEIBA®) for the treatment and prevention of bleeding in patients with acquired haemophilia: A sequential study
No full textDespite anti-haemorrhagic therapy with proper doses of activated prothrombin complex concentrate (aPCC, Feiba®), patients with acquired haemophilia A (AHA) have a considerable risk of recurrent bleeding complications. Evidence in support of the benefit-to-risk ratio of prevention strategies with the use of lower doses of aPCC following the end of the initial treatment period is scarce and inconclusive. We report our experience in the management of 18 consecutive patients with AHA admitted to two Haemophilia centres in Italy. We managed the first 11 according to current guidelines (e.g., with conventional aPCC doses until bleeding resolution). Then, we decided to prolong the treatment beyond bleeding resolution with lower doses of the same concentrate (short-term prophylaxis) in the 7 additional patients. In these patients, the treatment was continued for as long as the titre of FVIII inhibitor was found to decrease by at least 50% when compared to the baseline one. We observed six relapses of bleeding in patients in whom aPCC was confined to the treatment of the qualifying bleeding episode, and none in patients to whom lower doses were administered until the pre-specified decrease in the titre of FVIII inhibitor was achieved. No patients experienced thrombotic complications during the study period. Prolonging the treatment with lower doses of aPCC beyond the initial phase in patients with AHA in whom the titre of FVIII inhibitor is still high is likely to safely prevent further bleeding complication
Contraceptive pills induce an improvement in congenital hypoplasminogenemia in two unrelated patients with ligneous conjunctivitis.
No full textAbstract
Severe type I plasminogen deficiency is the underlying cause of ligneous conjunctivitis, a rare disease characterized by wood-like pseudomembranes developing on the ocular and extraocular mucosa. Two unrelated female patients with ligneous conjunctivitis and moderate hypoplasminogenemia are described. Being of fertile age, they were treated with oral contraceptives, which determined a marked increase in plasminogen levels. Moreover, a palpebral pseudomembrane stopped growing in one patient and disappeared completely in the other while on the estroprogestinic treatment. In patient n. 2, who also suffered from von Willebrand's disease, prior Cushing's disease induced an increase in both von Willebrand factor and plasminogen levels, which dropped after curative hypophysectomy. Genetic plasminogen study showed a 19Lys>Glu mutation in a heterozygous state in the first proposita and in a homozygous state in the second proband. In addition, both index patients were homozygous for a new intron F-14T>G mutation, which was found to reduce the acceptor splicing site prediction score. In conclusion, oral contraceptive therapy may improve plasminogen deficiency and deserves attention as an alternative therapeutic approach in selected cases of ligneous conjunctivitis with low, but not absent, plasminogen synthesis
- …
