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Biomedical subjects

F Fabris

Publications and source records attributed to F Fabris.

At least 109 records · Page 6Linked to original sources

Hemorheologic and coagulative pattern in hypercholesterolemic subjects treated with lipid-lowering drugs.

The effects on the coagulative and rheologic pattern of two lipid-lowering drugs, bezafibrate and simvastatin, were studied in 36 hypercholesterolemic subjects. Patients were randomly divided into two groups (18 subjects each) and received bezafibrate R 400 mg/day or simvatatin 10-40 mg/day over a twelve week period. Besides a decrease in plasma fibrinogen and fibrinopeptide A (p less than 0.001 both), bezafibrate induced a reduction of factor VIIc and VIIIc activity (p less than 0.001 both), while antithrombin 3 activity was increased (p less than 0.001) and the hemorheologic pattern was greatly improved (p less than 0.001). Simvastatin caused a slight decrease in factor VIIIc activity and a moderate reduction of beta-thromboglobulin. The efficacy of bezafibrate in reducing the activation of the coagulative cascade and improving the hemorheologic pattern has been confirmed; the peculiar triglycerides- and fibrinogen-lowering effect of the drug, not observed with simvastatin, could be responsible for these modifications.

Adult↗

Decrease of fibrinogen in patients with peripheral atherosclerotic disease by ticlopidine.

Ticlopidine (Ticlodone: CAS 55142-85-3) is able to develop a positive effect on claudication in patients affected by peripheral atherosclerotic disease (PAD). It is also known that ticlopidine decreases fibrinogen levels in plasma. 15 PAD patients treated with ticlopidine for 3 months were studied, evaluating the drug's effect both on maximum walking distance and on fibrinogen. Plasma fibrinogen as coagulable protein, fibrinogen antigen (Partigen method), 125I-fibrinogen survival, euglobulin lysis time and plasminogen were determined with the aim of clarifying if the decrease of plasma fibrinogen induced by ticlopidine is due to an increased destruction of a decreased production of the molecule. The normal levels of all tests before and after therapy both in ticlopidine treated patients and in the control group (acetylsalicylic acid + dipyridamol) indicate that the two hypotheses are not true. It is proposed that ticlopidine developing the known effect in decreasing the binding of fibrinogen to platelets induces a decrease only of activated fibrinogen.

Adult↗

[Treatment of hypercholesterolemic patients of different age with simvastatin: 1-year study].

Nowadays there is no direct evidence that lowering cholesterol may reduce the CHD in the elderly; however, several evidences suggest the potential benefit of a dietetic and/or pharmacologic intervention in healthy young-old hypercholesterolemics. In this study we evaluated the efficacy and tolerability of simvastatin, alone or in combination with cholestyramine, in two groups of patients (group A: age 30-55 years and group B: age 60-75 years) with primary hypercholesterolemia, over a 48-week period. In both groups Simvastatin induced a significant reduction in total and LDL cholesterol (p less than 0.001 both), with a decrease of the total/HDL cholesterol ratio (p less than 0.001). A reduction of the apo-B100 levels was also observed (respectively p less than 0.01 and p less than 0.001) with a significant improvement of the apo-B100/apo-A1 ratio (p less than 0.01). Simvastatin was well tolerated and free of remarkable side effects in both groups.

Adult↗

Thrombocytosis in young people: evaluation of 57 cases diagnosed before the age of 40.

Over the past 13 years 57 cases of primary thrombocytosis in young people have been studied. Only patients with a platelet count over 500 x 10(9)/liter and a follow-up longer than 2 years were considered. Thrombocytosis in young people represents approximately 25% of total cases referred to our department during this period. The most common causes are essential thrombocythemia (20 cases) and secondary thrombocytosis (22 cases). The highest platelet counts are found in essential thrombocythemia patients. Most of our patients were discovered by a fortuitous hematological examination. In contrast, 5 out of the polycythemic patients were recognized after a thrombosis. The same was true for 2 out of 20 essential thrombocythemia subjects. Four subjects (2 essential thrombocythemia and 2 secondary thrombocytosis) were diagnosed after hemorrhages. The overall survival was very good except for leukemic patients and thrombocytosis secondary to neoplasms. Vascular complications after diagnosis were scarce: 2 polycythemia vera patients showed bleedings during antiaggregating therapy. None of our patients developed epithelial cancer, malignant lymphoma or myelofibrosis. Vascular traumata seem more frequent in polycythemia vera regardless of age. Therefore, it seems useful to treat polycythemic patients, while no therapy seems to be indicated in other forms of thrombocytosis.

Adult↗

Benefits and risks of anemia correction with recombinant human erythropoietin in children maintained by hemodialysis.

Ten children with renal failure (age range 2 years 6 months to 18 years 9 months; median 11 years 10 months), maintained by long-term hemodialysis, had successful correction of their anemia after intravenous administration of recombinant human erythropoietin in a dosage escalating every 2 weeks (75 to 150 to 300 to 450 IU/kg/wk). Mean hemoglobin concentration increased from 6.4 +/- 0.9 to 11.5 +/- 1.0 gm/dl. Blood cell counts used to evaluate the correction of anemia were done after dialysis; this was especially important for children less compliant with water restriction. The higher hemoglobin concentration resulted in improvement of the quality of life, a greater tolerance for physical effort (exercise tolerance doubled and the ventilatory anaerobic threshold increased significantly), correction of some subclinical central nervous system abnormalities detected by evoked potentials testing, and reduction of bleeding time. Few side effects were noted; severe hypertension developed in one patient when postdialysis hematocrit was only 28%, and there were two episodes of hypertransaminasemia with no other evidence of liver dysfunction. We conclude that in children with renal failure the use of recombinant human erythropoietin to correct anemia is safe and strongly advisable, because of the resolution of many of the symptoms correlated with anemia.

Adolescent↗

Multiple, relapsing thrombosis in a young man with primary thrombocytosis.

We report the case of a relatively young man with multiple and fatal thrombosis associated with essential thrombocythaemia. The clinical and laboratory examinations performed before his death were in agreement with the diagnostic criteria proposed by PVSG. The autopsy revealed myocardial infarction and pulmonary embolisms. This case suggests that young patients with primary thrombocytosis should be treated with antiplatelet agents in spite of the absence of other thrombotic risks.

Humans↗

Platelet aggregation and pseudothrombocytopenia induced by 1-desamino-8-D-arginine vasopressin (DDAVP) in type IIB von Willebrand's disease patient.

Our study shows that the thrombocytopenia described in type IIB von Willebrand's disease (vWd) after 1-desamino-8-D-arginine vasopressin (DDAVP) infusion is, at least partially, a pseudothrombocytopenia. There was a discrepancy in platelet counts in blood anticoagulated with EDTA (less than 10 x 10(3)/microliters) or citrate (55 x 10(3)/microliters) in one patient with type IIB vWd and chronic thrombocytopenia (80 x 10(3)/microliters) after DDAVP infusion. Furthermore, DDAVP induced a normalization of patient's prolonged bleeding time. Spontaneous platelet aggregation (SPA) observed in platelet-rich plasma before DDAVP infusion was inhibited completely by monoclonal antibodies which block binding of fibrinogen, vWf and fibronectin to GPIIb-IIIa. SPA was partially inhibited by a monoclonal antibody which blocks the binding of vWf to GPIb. After DDAVP, in contrast, SPA partially persisted in the presence of anti-GPIIb-IIIa monoclonal antibodies but was completely inhibited by anti-GPIb monoclonal antibody. Therefore GPIb and GPIIb-IIIa complex seem to play a different role in SPA before and after DDAVP infusion into type IIB vWd.

Adult↗

Variant Bernard-Soulier syndrome type bolzano. A congenital bleeding disorder due to a structural and functional abnormality of the platelet glycoprotein Ib-IX complex.

We have studied a patient with a congenital bleeding disorder and phenotypic manifestations typical of Bernard-Soulier syndrome, including giant platelets with absent ristocetin-induced von Willebrand factor binding. Two monoclonal antibodies reacting with distinct epitopes in the amino-terminal domain of the alpha-chain of glycoprotein (GP) Ib were used to estimate the number of GP Ib molecules on the platelet membrane. In the patient, binding of one antibody (LJ-Ib10) was approximately 50% of normal, while binding of the other (LJ-Ib1) was absent. Binding of both antibodies was reduced to approximately 50% of normal in the mother and one sister of the propositus, and their platelets exhibited approximately 70% of normal von Willebrand factor binding. Immunoblotting studies confirmed the presence of GP Ib alpha, as well as GP IX, in patient platelets. Antibody LJ-Ib10, but not LJ-Ib1, could immunoprecipitate the patient's GP Ib alpha from surface-labeled proteins. Thus, platelets from the propositus contained a structurally and functionally altered GP Ib-IX complex lacking a specific antibody epitope and the ability to bind von Willebrand factor. In contrast, the binding of human alpha-thrombin to the patient's platelets was normal, and three classes of binding sites with high, intermediate, and low affinity could be detected. These studies define a distinct variant form of Bernard-Soulier syndrome and provide evidence, based on a naturally occurring mutant molecule, that the amino-terminal region of GP Ib alpha contains a von Willebrand factor-binding domain distinct from the high affinity thrombin-binding site. Use of different monoclonal antibodies with distinct epitope specificities appears to be essential for a correct identification of variant Bernard-Soulier syndrome.

Antibodies, Monoclonal↗

Effect of cellulose acetate 0.2 micron filter on platelet specific proteins plasma levels.

We are reporting the effect of a cellulose acetate 0.20 micron filter (Flow Pore D26) on preparation of platelet poor plasma (PPP) for subsequent assay of platelet specific proteins. PPP was obtained using the Edinburgh anticoagulant mixture and a centrifugation at 2,300 g for 20 minutes. The mean levels of platelet factor 4 (PF4) and beta-thromboglobulin (beta TG) before filtration were: PF4 15.7 ng/ml (1.5-42.8 ng/ml); beta TG 62.0 ng/ml (25-140 ng/ml). After filtration we obtained: PF4 4.8 ng/ml (1.0-8.9 ng/ml), beta TG 28.4 ng/ml (8.5-51.1 ng/ml). Thus difference for both the platelet specific proteins was statistically significant (p less than 0.005). The contamination by platelet-like material was also greatly affected by the filter (before filtration 4.3 x 10(6)/ml), (2-15 x 10(6)/ml), after filtration 2.0 x 10(6)/ml (1-5 x 10(6)/ml), p less than 0.005). If a high speed centrifuge is not available, the use of this simple filter method could avoid falsely elevated values of platelet specific proteins.

Adult↗

Haematological complications in polycythaemia vera and thrombocythaemia patients treated with radiophosphorus (32P).

We have evaluated 230 patients with myeloproliferative disorders treated in the last 15 years with 32P. None of the patients affected by essential thrombocythaemia developed haematological complications. In the larger group of polycythaemia patients (214 subjects) only 38 patients (17 males and 21 females) developed complications. 60.5% of these subjects had a minor complications: 1.8% showed a thrombocytopenia lower than 100.10e9/lt, 2.3% anaemia with Hb lower than 10 g%, 2.6% leukopenia lower than 40.10e9/lt and 2.3% a pancytopenia. All these complications were transient and eventually treated with limited blood transfusions. We could not identify a correlation between the dose used and the development of such complications. We noted only that the occurrence of anaemia, given a similar dose, was more frequent in females. Only 7% of all patients presented a major complication after 32P administration. In this case too, there was no correlation with the dose administered. Myelofibrosis and chronic myeloid leukaemia resulted to be the more frequent complication (9 out of 15) but we could not clarify if they represented a natural evolution of polycythaemia vera or were due to the treatment with 32P. Acute leukaemia developed only in 5 patients and again we could not recognized a correlation with the dose administered. Moreover, the time from the diagnosis of polycythaemia vera the onset of acute leukaemia ranged widely. 32P has a definite effect on the prevention of thrombotic and haemorrhagic complications in polycythaemia patients since it prolongs their life but it also increases the incidence of acute leukaemia.

Anemia↗

"Anti-platelet antibodies" in HIV infected haemophiliacs.

We have studied anti platelet antibodies and circulating immunocomplexes in 16 haemophiliacs with mild thrombocytopenia eight of which were infected by human immunodeficiency virus (HIV). No difference in platelet count was observed between HIV+ (143 +/- 31 x 10(9)/l) and HIV- patients (148 +/- 30 x 10(9)/l). On the contrary, HIV+ haemophiliacs had serum platelet bindable IgG (SPBIgG), normal platelet associated IgG (PAIgG), high serum IgG and circulating immunocomplexes (CIC). Considering all 16 patients serum IgG correlated with CIC (r = 0.7 p less than 0.01) and SPBIgG (r = 0.6 p less than 0.01) respectively. We obtained also a positive correlation between serum CIC and SPBIgG (r = 0.51 p less than 0.05). Immunoblotting of patients' sera showed no specific binding to target platelet antigens. In conclusion there is no evidence of HIV related immune thrombocytopenia in our haemophiliacs but the study confirms the appearance of immunocomplexes in the HIV+ subjects.

Adult↗

A new congenital platelet abnormality characterized by spontaneous platelet aggregation, enhanced von Willebrand factor platelet interaction, and the presence of all von Willebrand factor multimers in plasma.

A case is reported of a 49-year-old woman with a mild bleeding tendency. Her bleeding time, platelet count and size, plasma ristocetin cofactor activity, von Willebrand factor (vWF) antigen, and vWF multimeric pattern are all within normal limits. Spontaneous platelet aggregation is observed when citrated platelet-rich plasma (PRP) is stirred in an aggregometer cuvette. This aggregation is completely is only slightly diminished by an antiglycoprotein (GP) IIb/IIIa or by an anti GPIb monoclonal antibody. The patient's PRP shows increased sensitivity to ristocetin. The distinct feature of this patient, also present in two family members studied, is that platelet aggregation is initiated by purified vWF in the absence of any other agonist. The vWF-induced platelet aggregation is abolished by anti-GPIb and anti-GPIIb/IIIa monoclonal antibodies and by EDTA (5 mmol/L). Apyrase inhibits the second wave of aggregation. Patient's platelets in PRP are four to six times more reactive to asialo vWF-induced platelet aggregation than normal platelets. The amount of radiolabeled vWF bound to platelets in the presence of either low concentration of ristocetin or asialo vWF was increased 30% compared with normal. The patient's platelet GPIb was analyzed by SDS page and immunoblotting and by binding studies with anti-GPIb monoclonal antibodies showed one band with slightly increased migration pattern and a normal number of GPIb molecules. Unlike the previously reported patients with pseudo or platelet-type von Willebrand disease, this patient has normal vWF parameters.

Antibodies, Monoclonal↗

Response to splenectomy in idiopathic thrombocytopenic purpura: prognostic value of the clinical and laboratory evaluation.

Sixteen patients with chronic idiopathic thrombocytopenic purpura underwent splenectomy after failure of steroid therapy. The average time of follow-up was 69 months. Immediately after splenectomy, complete response (platelets above 150 x 10(9)/l) was obtained by 68% of patients while 25% had partial response (platelets from 50 to 150 x 10(9)/l) and only in 1 patient splenectomy failed. During the long-term follow-up, 2 patients relapsed 9 and 20 years, respectively, after splenectomy (20% of non-responders). Partial recurrence of thrombocytopenia (partial response) was observed in 33% of the patients. The persistent complete response rate was then 47%. The young patient age appears to be the only positive predictive factor both for short-term and long-term response to splenectomy. The platelet recovery rate and postsplenectomy thrombocytosis are also early features of lasting response. Positive serum and platelet-associated immunoglobulins became negative after splenectomy, but there is no correlation with clinical response.

Adult↗

Anterior spread of gynaecological neoplasias: reliability between some diagnostic procedures.

The Authors report their experience regarding the comparison between pre-surgical clinico-instrumental staging and intra-operative staging in 134 patients with gynaecological neoplasias, particularly considering the spread to the lower urinary tract. The analysis of the results shows the moderate reliability of the cystoscopy in routinely evaluating extension of gynaecologic malignant diseases. The association of cytological urinary sediment examination with cystoscopy improves the possibility of a better evaluation. On the contrary, vesical biopsy, performed on the indications of cystoscopy and urinary cytology, and standard excretory urography prove to be insensitive indicators in diagnosing the spread of gynaecological malignancies.

Cystoscopy↗

Low incidence of familial occurrence of thrombocythaemia and/or thrombocytosis.

Several reports have been published about familial polycythaemia vera (PV) but no information is available about the incidence of thrombocytosis in the same family. In our population of thrombocytosic patients, both with primary thrombocytosis (133 cases) and secondary (37 cases), we found only two family related subjects. One of them had PV and the other essential thrombocytosis (ET). Our results seem to indicate that familial thrombocytosis is a rare phenomenon, much less frequent then familial thrombocytopenia.

Adolescent↗