Antonio Girolami

..In case of doubt the thrombotic event should be considered as idiopathic. The two flow charts are not intended to eliminate the OCT-related VTE but represent only an attempt to decrease the prevalence of this complication...

..The significance of these findings is discussed with particular emphasis on the discrepancy between venous and arterial thrombosis seen in patients with Glanzmann thrombasthenia and Bernard-Soulier syndrome...

..Heparin and Coumadin together with adequate substitution therapy were carried out in 5 patients with satisfactory results. The FVII deficiency does not grant a sure protection from venous thromboembolism...

..Two patients with FXI deficiency had inhibitors. Dosage was variable. There was at least one fatality but in five cases evolution was not reported. The remaining patients recovered with variable sequels...

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..These clinical observations are in contrast with experimental studies which demonstrate that Bernard-Soulier-like strains of mice show a decreased thrombus generation in several experimental settings...

..Physicians should always keep in mind that a congenital hemorrhagic disorder may cause bleeding in any tissue of the body and therefore alter the normal clinical features of a given disease...

..The compound heterozygosis with the Glu 117 stop mutation, common among Jews, was not surprising because of the past strict ties of the Republic of Venice with the Middle East...

..This led to a decrease in transfusional needs and to improved general conditions...

..An early diagnosis, as often in medicine, is of paramount importance for a prompt treatment and a satisfactory outcome...

..OC are the treatment of choice in congenital bleeding disorders to control both the menorrhagia and, more importantly, ovulation-related hemoperitoneum...

..The study underlines the importance of a multipronged evaluation of all cases with Factor X deficiency. In fact only by this approach can an acceptable classification of the defect be reached...

..Only specific assays could allow one to reach the correct diagnosis. In cases of casual association with other defects, it is also important to study family members, as the two defects should segregate independently...

..The rarity of hemospermia in congenital bleeding disorders remains unexplained, although the strong perineal and sphincter muscles may exercise a compressive hemostatic effect which could prevent or reduce bleeding...

..Only seven of these 19 pregnancies were brought to term with fetal survival. Abortion was spontaneous in 5 cases and therapeutic in the additional 7. There were two maternal fatalities...

..Other noncoagulation-related conditions such as hypertension, liver damage, and obesity may represent absolute or relative contraindications to the use of OCT...

..A niece of the proposita was shown to be heterozygous for the abnormality. Molecular modeling suggested that the mutation does not alter significantly folding and stability of the protein but may be involved in the Ca2+ binding site...

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..As a conclusion of this review it seems safe to assume that both arterial and venous thrombosis appear rare in vWD. This is confirmed by the fact that arterial or venous thrombosis appears slightly more frequent in hemophilia A and B...

..In a few instances low molecular weight heparin was given for a few days. The frequent association of venous thrombosis with infusion of concentrates indicates the need for a careful evaluation of patients about to receive such therapy...

..The fact that thrombosis has never been described in patients with factor II or factor X seems to underscore the central antithrombotic role that these two factors have in the coagulation system...

..These observations may indirectly support the rationale for the use of direct thrombin or Factor X inhibitors in the prophylaxis and/or therapy of thrombotic manifestations...

..g. a platelet count for petechiae, a PTT for a patient with haemarthrosis, etc.)...

..Finally, the elevated incidence of mental and skeletal malformations present in these combined defects indicates the need for a careful evaluation of all these patients lest some aspects of the defect are missed...

..The low levels of activated FVIIa found in homozygotes with FVII Padua indicate that the normal FVII activity found with ox-brain thromboplastin cannot be attributed to higher than normal circulating levels of FVIIa...

..However, the occurrence of thrombotic events in only one of these conditions may be used as a new tentative differentiability feature...

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..The actual needs of substitution therapy in patients with some variants of FVII deficiency is discussed, together with comments on the therapeutic management of the thrombotic events in these patients...

..Unfortunately so far there is no sure way to distinguish the women with this polymorphism who will develop venous thrombosis from those who will remain asymptomatic during oral contraceptive therapy...

..The antigen/ox brain activity ratio of 1.1 fully confirms this assertion...

..Bradykinin causes inflammation, vasodilatation and an increase in vessel permeability. The activation of pro-urokinase results in enhanced fibrinolysis. However, fibrinolysis has been reported to be normal or defective in these patients...

..The defect is characterized by low FVII activity when rabbit brain thromboplastins are used in the assay system. On the contrary, FVII levels are normal when ox-brain thromboplastins are used. FVII antigen is always normal...

..One patient who presented both a stroke and a pulmonary embolism had a fatal outcome. The article clearly indicates that prekallikrein deficiency does not protect from thrombosis in spite of the severe in vitro coagulation defect...

..In the remaining two pregnancies no bleeding was noted and no substitution therapy was given. No data are apparently available in the literature about the use of OCs in FV deficiency...

..On the contrary, no firm conclusion could be drawn for kindred who showed mutations in the Gla-domain associated with defects in other domains...

..The study analyzes the need to have a multipronged approach to the study of congenital FX deficiency. The indication of a mutation in a given domain does not provide clear information about the phenotype...

..As a consequence it may be only stated that FVII deficiency does not protect from thrombosis...

..By using this nosographic approach, all reported cases of FX deficiency can be adequately allocated to one of these groups...

..Because the majority of women took preparations that contained third-generation progestins during the second course of therapy, concomitant contributing effects of these compounds cannot be excluded...

..This study clearly indicates that the severe in vitro coagulation defect seen in these conditions does not protect from thrombosis...

..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...

..Comparison with other topologically equivalent mutations in other vitamin K-dependent proteins suggests that this disruption could adversely affect protein folding/stability, accounting for the cross-reactive material negative phenotype...

..In conclusion, PAI-1 4G/5G polymorphism may influence PAI-1 expression and thrombotic risk in patients with inherited thrombophilia...

..Contributing to the controversy is uncertainty about whether residual venous thrombosis, as assessed by repeated ultrasonography over time, increases the risk for recurrent thromboembolism...

..These risks correlate with the extent of cancer. Possibilities for improvement using the current paradigms of anticoagulation seem limited and new treatment strategies should be developed...

..Because only limited evidence suggests that elastic stockings prevent the post-thrombotic syndrome in patients with symptomatic deep venous thrombosis (DVT), these stockings are not widely used...

..No associated abnormality was apparent in the propositus or his parents. As expected, no tendency for bleeding was noted even after tonsillectomy...

..In conclusion, these data confirm the difficulty of plasminogen genetic analysis and may help researchers to better identify the true plasminogen gene mutations causing molecular defects...

..Although the frequency of HIT in hospitalized medical patients treated with subcutaneous heparin is lower than that observed in other clinical settings, this complication is associated with a similarly high rate of thromboembolic events...

..In all the examined districts, the mean IMT was significantly lower in patients with hemophilia than in controls. Hemophilia protects against asymptomatic atherosclerosis...

..These findings confirm that a normal consensus sequence for VWF propeptide cleavage and efficient cleavage are required in vivo for normal FVIII binding capacity of VWF...

..The increase in the prevalence of VTE episodes appeared to be proportional to the increased use of OC, regardless of the type of progestin contained in the oral contraceptive preparations...

..CSGE may be an effective and simple procedure for screening factor X gene mutations...

..In conclusion, variations in fibrinolytic test findings after HSCT, and PAI-1 in particular, may facilitate the early diagnosis of VOD in pediatric patients after HSCT...

..Screening families of symptomatic probands with the factor V Leiden mutation has the potential to identify those asymptomatic carriers who might benefit from thromboprophylaxis during risk periods...

..3; 95% CI, 1.3 to 8.6). Since aPT antibodies are more commonly identifiable in patients with multiple thrombotic episodes, they are a likely risk factor for recurrent thromboembolism...

..Whether atherosclerosis is associated with an increased risk of venous thrombosis is unknown...

..The substitution of Gly for Arg causes the introduction of a large charged amino acid which could affect the catalytic function of FX leading to secretion problem, accounting for the cross-reactive material (CRM) negative phenotype...

..In the index patient, an approximate 13-kb product was obtained, whereas no product was obtained from his mother. The mother investigated by means of polymorphism was shown not to be a carrier...

..The median time to clinical worsening was 2.1 months for MACE-positive patients and 27.7 months for MACE-negatives. The assay of specific platelet autoantibodies may be a useful prognostic tool for the clinical course of ITP...

..The need for a rapid response is determined in the case of hemophilia A patients, not only by the desire to reach a proper classification, but also by the urgency to inform the carrier status of the mother or of a female relative...

..Accordingly, screening for thrombophilia in otherwise healthy children younger than 15 years who belong to families with inherited defects predisposing to thrombosis seems unjustified...

..von Willebrand factor (VWF) is stored in the Weibel-Palade bodies of endothelial cells and may be released in response to different secretion stimuli such as stress, physical exercise, adrenaline, DDAVP and thrombin...

..2 to 30.1) in patients without the mutations. Prothrombin and factor V gene mutations occur frequently in patients with venous thrombosis and are associated with an increased risk for recurrent venous thromboembolic complications...

..05). A slight and nonsignificant trend of association between genotype and t-PA:Ag 20/0 ratio was seen in the t-PA group patients. In conclusion, these data suggest a possible genetic modulation of t-PA-regulated secretion...

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..We include this variant in type 2M VWD, focusing on the abnormality in GPIb-dependent VWF function. We advance that this defect depends on the mutation in the GPIb binding domain of VWF rather than the abnormal VWF multimer pattern...

..Occasional reports suggesting a link between this polymorphism and oral contraception-related venous thrombosis should be carefully evaluated in order to avoid premature and incorrect conclusions...

..The significance and importance of genetic analysis in addition to clotting and immunological studies in FXII deficiency is emphasized...

..The patient was given low-molecular-weight heparin with a dramatic reduction in previously elevated fibrinogen level and a good control of the hepatic function...

..The protocol proposed in the present study makes dental extractions in anticoagulated patients possible on an outpatient basis with a cost reduction for the community and minor discomfort for the patients...

..In conclusion, it is safe to withhold anticoagulant treatment from patients with suspected recurrent ipsilateral DVT in whom compression ultrasonography showed improved or stable vein diameters...

..There was also a significant falling circaseptanal rhythm on Fridays. These observations may optimize an adequate and precise anticoagulant therapy in patients with protein C or protein S deficits...

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..Finally CRP levels in preeclampsia are believed to correlate with preeclamptic process severity...

..Although oral anticoagulants are still widely used for the prevention of venous thromboembolism after hip replacement, to our knowledge no study has assessed the benefit of prolonging anticoagulation beyond the hospital stay...

..In conclusion, the bio-chemical findings support a role for AT in controlling the haemostasis at conventional doses, and the inflammatory state at higher doses...

..In addition, because normal VWF-platelet GPIb interaction was observed before or after DDAVP administration, it is proposed that type Vicenza VWD not be considered a 2M subtype...

..We evaluated plasma fibrinolytic behavior in two groups of RTRs treated with different immunosuppressive regimens...

..We describe a single-institution experience in patients with ET and PV, diagnosed in agreement with the Polycthemia Vera Study Group criteria, with portal vein thrombosis who did or did not undergo splenectomy...

..The aim of the present study was to compare the diagnostic value of PAIgG with a modified antigen capture ELISA (MACE) in patients with primary and secondary immune thrombocytopenia and in patients with non-immune thrombocytopenia...

..Impaired interaction of elongated APC molecules with a membrane-surface and/or factor Va which is the physiological substrate for APC, is manifested in vivo by thrombophilia...

..Until this problem is clarified, the clinician must abstain from attributing a prothrombotic effect to this polymorphism...

..The most serious hemophilic patients had fewer plaques than controls or than patients with mild hemophilia. Both hemophilia and von Willebrand disease seem to protect against atherosclerosis...

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..Since subjects with central obesity are at high risk for cardiovascular disease, the effects of the 4G/5G polymorphism on PAI-1 concentration may further enhance this risk...

..The implications of this unusual association are discussed...

..The results suggest that vWF abnormalities are present in uremia. Moreover, thrombopathy caused by impaired collagen-induced platelet aggregation is constantly present and apparently not improved by dialytic treatment...

..There is no consensus on the efficacy of physical training, smoking cessation, and pharmacological therapy (pentoxifylline or nafronyl oxalate) in the treatment of patients with intermittent claudication at Fontaine stage II of disease...

..The results show that the parents paid great attention to the problems of their sons, but mothers were more depressed and anxious about the disease of their child...

..There is no consensus on the efficacy of the antithrombotic drugs available for patients with intermittent claudication...

..Treatment with FVIII concentrate is probably the most important triggering factor. Monitoring these patients for autoimmune manifestations is recommended...

..It is likely that acquired triggering conditions play a major role in determining VTE in carriers of a mild genetic predisposition. This has to be taken into account when recommendation for thromboprophylaxis is given...

..33+/-409.96; P<0.02), it did not significantly change even in patients with adequate correct count increment. Our data confirm the importance of anti-HLA antibodies in determining adequate post-transfusion recovery or refractoriness...

..002); however, the mean level was not as high as in ITP patients (345 +/- 167 pg/mL; p < 0.001). This finding suggests that CHMT syndrome is not secondary to a defective production of TPO and that megakaryocyte mass is nearly normal...

..X Lys(408)-->Asn substitution. This is the first characterisation of a naturally occurring F.X variant with a mutation at the COOH-terminal end of the molecule...