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Biomedical subjects

F Fabris

Publications and source records attributed to F Fabris.

At least 127 records · Page 7Linked to original sources

Increased proportion of giant platelets and platelet distribution width are better indicators of altered platelet homeostasis than mean platelet volume in liver cirrhosis.

Platelet count, mean platelet volume (MPV), giant platelet percentage, expressed by us as megathrombocytic index (MTI) and platelet distribution width (PDW) have been evaluated in 32 control subjects and in 27 patients suffering from liver cirrhosis (LC) and thrombocytopenia. MTI and PDW were linearly and inversely correlated to platelet count both in controls and patients. MTI and PDW were markedly increased in LC as compared to controls, while MPV was not significantly different. It is concluded that MTI and PDW are good indices of thrombopoietic stimulus both in controls and in CL and better indicators of altered platelet homeostasis than MPV in LC.

Adolescent↗

The use of antiproteolytic mixture fails to modify the abnormal von Willebrand factor pattern in myeloproliferative disease.

Abnormalities of von Willebrand factor, with reduction of higher molecular weight multimers, was described in patients with increased platelet number. We have studied twelve patients with myeloproliferative disease (9 patients with Essential Thrombocytosis and 3 patients with Polycythemia Vera). Blood samples were collected either with citrate and antiproteolytic mixture (EDTA 6 mM, Aprotinin 200 U/ml, N-ethylmaleimide 5 mM in sodium citrate 3.8%). VIIIR: RCoF was found decreased in all patients studied, using both anticoagulants, while VIIIR: Ag was within normal range. Higher molecular weight multimers were found decreased or absent both in samples collected with citrate and in those collected using the antiproteolytic mixture. These data suggested that von Willebrand factor abnormalities observed in myeloproliferative disease are not due to a proteolytic degradation that's supposed to happen in vitro during blood samples collection and manipulation.

Anticoagulants↗

Electromyography of anal sphincters after radical gynecological surgery.

In this study, the Authors report their own experience in electromyography of anal sphincters. The clinical evaluation concerns a clinical series of 14 patients suffering from carcinoma of the uterine cervix at SPS stage A and B, and undergoing a personalized radical hysterectomy during 1986. The protocol included a preoperative electromyographic and urodynamic evaluation and a post-operative re-evaluation 30-60 days after surgery. The pre-operative evaluation in both the urodynamic test and the electromyographic test showed bladder-urethral function in all the cases examined. The post-operative electromyographic evaluation was considered normal and did not show alterations in the sphincteral function in 9 cases (64.3%). In the remaining 5 cases (35.7%) the presence of sphincteral incoordination was shown. The analysis of the results shows that the use of electromyography, connected with the usual urodynamic study in gynaecological oncology, objectively recognises the sphincter-detrusorial situations characterized by dysuria and retention from a clinical-anamnestic point of view.

Adult↗

Increased factor VIII/vWf levels in patients with reduced platelet number.

Factor VIII/von Willebrand factor (VIII/vWf) related properties were studied in twenty six patients with thrombocytopenia. Fifteen patients were affected by idiopathic thrombocytopenic purpura (ITP) and 11 patients by thrombocytopenia of a different nature or non-ITP (n-ITP). All patients showed an enhancement of platelet associated IgG (PAIgG). A significant increase of factor VIII ristocetin cofactor (VIII R: RCoF) and factor VIII related antigen (VIII R:Ag) was found in ITP patients while normal values were observed for factor VIII coagulant (VIII:C). All factor VIII/vWf components, on the contrary, were increased in n-ITP group with a prevalence of VIII R:RCoF as observed in ITP group even though with lower mean values. Multimeric analysis of VIII/vWf demonstrated a higher concentration of all multimeric components, with major representation of higher molecular weight multimers (HMWM) in patients of both groups. Two patients were studied before and after improvement in platelet count. A decrease of vWf related properties (VIII R:RCoF and VIII R:Ag) concomitant with the increase in platelet count was found. In n-ITP patients a statistical correlation between VIII R:RCoF and PAIgG was also observed while no correlation was found between other factor VIII/vWf components and PAIgG both in ITP and n-ITP patients.

Adolescent↗

The significance of thrombocytosis in old age.

We performed a retrospective study on 21 patients, aged 70 years or more, affected by polycythemia vera (PV) or essential thrombocythemia (ET). As controls, we evaluated 10 younger ET patients. The results indicate that in older ET subjects there was a lower incidence of hemorrhagic and thrombotic complications than in younger patients and PV patients. However, platelet number and platelet function tests were similar in all the patients studied. We suppose that an increase in hematocrit as seen in PV is much more dangerous as compared to an isolated increase in platelet count, and that thrombocytosis alone in old age can be an isolated expression of a natural involution of blood marrow similar to myelofibrosis.

Adult↗

Failure of two anti-platelet drugs (indobufen and dipyridamole) to improve thrombocytopenia in liver cirrhosis.

The hypothesis that thrombocytopenia in liver cirrhosis (LC) could be due to platelet activation was investigated. 18 patients with thrombocytopenia and LC have been studied. Circulating beta-thromboglobulin (beta TG) was normal, but appeared elevated when referred to platelet count. However, this may not accurately reflect alpha-granule release because of reduced liver cell function. Intraplatelet beta TG, on the contrary, should not be affected by liver cell function. It was markedly depressed, thus truly suggesting the existence of an exhausted state caused by platelet activation. Thromboxane B2 production was slightly increased. This could be due to a compensatory mechanism, or simply to platelet size, which was slightly increased, too. Anti-platelet therapy failed to improve thrombocytopenia. A platelet activation state seems therefore to be present in LC, but seems not to be the only cause of thrombocytopenia. Platelet factor 4 approximated zero, regardless of platelet count and therapy. This confirms that elevated values of such a protein represent only a laboratory artifact, due to platelet activation in vitro.

Aged↗

Thrombocytopenia in liver cirrhosis complicated by variceal haemorrhage: lack of increase in platelet count after spleno renal distal shunt.

10 patients with liver cirrhosis (LC) and previous variceal bleeding have been studied. Platelet count was markedly depressed. Giant platelet percentage (MTI) was significantly increased, reflecting bone marrow compensatory hyperactivity. Platelet associated IgG (PAIgG) was elevated. Such elevation, however, was inconstant and did not correlate with platelet count. Beta-thromboglobulin was markedly increased when referred to circulating platelet number, but its level did not accurately reflect platelet activation, because of decreased liver cell function. Platelet Factor 4 was always zero, thus confirming that high values of this protein represent only a laboratory artifact, due to platelet activation in vitro. 5 patients underwent spleno renal distal shunt, which transiently improved hypersplenism only in one case. Despite this, MTI became perfectly normal in all patients, suggesting a decrease in the thrombopoietic stimulus. PAIgG dramatically fell in the two patients with the highest pre-operative values. Since thrombocytopenia persisted, the non-specific nature of PAIgG in LC seems to be further supported.

Adult↗

Evaluation of platelet function in postsplenectomy thrombocytosis.

The usually transient post-splenectomy thrombocytosis has no well defined effect on the development of thromboembolism. We studied 14 patients submitted to splenectomy for different causes: portal vein thrombosis (PVT), idiopathic thrombocytopenia purpura (ITP) and lymphoma. The mean platelet count in PVT patients was significantly higher than in all other subjects. In PVT group we demonstrated various alterations in platelet aggregation curves and a frequent increase of platelet 5-HT levels. In effect, we noted that all the PVT patients showed a myeloproliferative disease (MPD) before the surgical procedure. We therefore conclude that the increased incidence of thromboembolism in the patients who underwent splenectomy is probably caused by a preexisting MPD rather than the post-splenectomy thrombocytosis.

Adult↗

Simple asymptomatic orthotopic ureterocele in pregnancy: analysis of a clinical case.

In the present paper the authors, taking for a starting point the occasional recognition of an asymptomatic orthotopic ureterocele in pregnancy, refer to the present knowledge of its embryology, pathological anatomy and the form of therapeutic approach. In the light of such experience they confirm the role of clinical-instrumental diagnostic investigations of purely gynecological and obstetric pertinence in the recognition of pathologies of urological interest.

Adult↗

Abnormalities of von Willebrand factor in myeloproliferative disease: a relationship with bleeding diathesis.

We studied factor VIII related properties in 24 patients with increased platelet number. Twenty-one were affected by myeloproliferative disorders (eight had polycythaemia vera, 13 had essential thrombocythaemia) and three had secondary thrombocytosis. Normal levels of VIII:C and VIIIR:Ag were found while a significant (P less than 0.05) decrease of VIIIR:RCOF (43 +/- 13%) related to a lack of larger multimers of VWF (39 +/- 12%) was observed in 57% of patients with myeloproliferative disorders. A normal VWF pattern was found in the three patients with secondary thrombocytosis. The highest incidence of VWF abnormalities occurred in patients with essential thrombocythaemia (70%) in comparison with polycythaemic patients (38%). A significant (P less than 0.03) correlation between platelet count and the values of both VIIIR:RCOF and VWF multimeric pattern was observed only in patients with polycythaemia vera. The lowest levels of VIIIR:RCOF and the greatest loss of larger VWF multimers (less than 30%) were observed in two patients who presented bleeding symptoms at the time of study and a prolonged bleeding time. In addition, the relationship between VWF pattern and bleeding diathesis was supported by the fact that 75% of the patients with VWF abnormalities had bleeding history.

Adult↗

Clinical significance of surface and internal pools of platelet-associated immunoglobulins in immune thrombocytopenia.

We have investigated serum platelet bindable IgG(SPBIgG) and platelet-associated IgG(PAIgG) in patients with immune thrombocytopenia (IT) to ascertain the significance of the larger amounts of PAIgG reported both in normals and in patients using homogenized (Total:T-PAIgG) instead of intact platelets (Surface:S-PAIgG). 12 patients, during active immune thrombocytopenia (A-IT), and 18 patients in steroid-induced remission (S-IT), were studied. As control we considered 20 patients with non-immune thrombocytopenia (N-IT) and 29 subjects with normal platelet count. The average positivity of SPBIgG was 41% with a higher percentage in A-IT (66%) than in S-IT (16%). The results of PAIgG also indicate a different behaviour in A-IT and S-IT of surface and cytoplasmatic pools. During A-IT both pools are enhanced with prevalence towards the surface one (S-PAIgG). On the contrary, during steroid-induced remission, despite the normal amount of the S-PAIgG present the internal pool still increases, indicating a steady-state of IT and the possible existence of a platelet phagocytic activity.

Adult↗

Bernard-Soulier syndrome: diagnosis by an ELISA method using monoclonal antibodies in 2 new unrelated patients.

Two unrelated patients with Bernard-Soulier syndrome and their relatives were studied. The patients demonstrated severe bleeding diathesis, the relatives were asymptomatic. The propositi showed the characteristic abnormalities of the syndrome: thrombocytopenia, a percentage of giant platelets higher than 65%, prolonged bleeding time and defective platelet aggregation to ristocetin and bovine plasma. On the contrary, in the heterozygotes, the typical abnormalities were not fully evident. We introduce a simple ELISA method for the precise definition of both homozygous and heterozygous states for the syndrome by the quantitation of platelet glycoprotein (GP) Ib. Specific binding of monoclonal antibodies anti-platelet GPIb was performed both by direct binding of radioiodinated antibody and by ELISA. Comparable results were obtained. In fact, we demonstrated near absence of GPIb in the 2 propositi and about half the amount in the heterozygotes studied compared to normal platelets.

Adolescent↗