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Biomedical subjects

A Girolami

Publications and source records attributed to A Girolami.

At least 379 records · Page 21Linked to original sources
PubMed1987

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↗
PubMed1987

A tentative classification of AT III congenital abnormalities.

Twenty two families with an abnormal antithrombin III have been described so far. A classification of these abnormality encounters many difficulties. In fact, the available classifications seem inadequate. On the basis of 5 tests, namely AT III progressive and/or global activity, heparin co-factor activities, crossed immunoelectrophoresis (CIE) without and with heparin, AT III antigen and heparin affinity studies, a "new" tentative classification is proposed. On the basis of these tests, AT III abnormalities may be subdivided in 5 groups: Group 1 includes asymptomatic patients with a variable defect in heparin cofactor activities with normal total or progressive AT III activity and with a slow peak in the heparin modified CIE. Group 2 comprises symptomatic patients with the same laboratory features as presented by group 1 patients. Group 3 includes families in which there is a variable reduction of all AT III activities. There is always a slow peak in the heparin modified CIE and patients are symptomatic. Group 4 includes patients with a variable decrease of all AT III activities but a normal CIE. Patients are symptomatic. Group 5 comprises symptomatic patients with variable decreased AT III activity, and with a fast moving peak in the plain (without heparin) CIE.

Antithrombin III↗
PubMed1987

PF 4 assay by an ELISA method: a good correlation with the usual RIA method.

PF 4 is a specific platelet protein. This protein is released from alpha granules during the platelet activation and later it adheres to endothelium. Intravenous heparin injection displaces PF 4 from vessels wall. Thus, PF 4 levels are an index of in act or past platelet activation. We have compared two methods of PF 4 dosage on 39 blood samples taken from healthy volunteers and patients. The samples has been shared out tree groups according to the procedure of collecting; so the values of PF 4 are widely enough distributed. There was no difference between the mean values of each group obtained with two methods. Equally the mean value of all samples processed with radioimmunoassay was similar to the mean value obtained with immunoenzymatic method. The correlation index between the values of PF 4 obtained with radioimmunoassay and immunoenzymatic method was 0.97. Therefore the new immunoenzymatic method for the dosage of PF 4 is as sensitive and precise as the radioimmunoassay.

Blood Specimen Collection↗
PubMed1987

Study on a new chromogenic substrate for the prothrombin time determination.

The aim of our study was to evaluate the possibility of using a chromogenic substrate for the prothrombin time determination. The reagent used by us (Chromoquick) is composed of a human placenta thromboplastin and chromogenic substrate (Tos-Gly-Pro-Arg-5-amino-2-nitrobenzoic acid-isopropylamide), calcium chloride and a buffer. Normal subjects, patients with liver disease, patients on oral anticoagulant therapy, patients on heparin therapy, heterozygous and homozygous patients for prothrombin complex defects and other miscellaneous conditions have been investigated. The results of chromoquick have been related with standard prothrombin time obtained using a human placenta thromboplastin (Thromborel) and rabbit brain and lung thromboplastin (Simplastin). The normal range was 18-23 s for chromoquick and 13.5-15.5 s for the standard prothrombin times using Thromborel and Simplastin. In all groups of patients examined we noticed a significant correlation between the chromogenic and the classic prothrombin times with r values varying between +0.505 and +0.947. The statistical significance resulted from p values varying between less than 0.05 and less than 0.001. Only in the case of some heterozygotes for prothrombin complex factor defects the values obtained have not been unequivocal in the sense that in a few instances the heterozygotes seemed to escape detection. Therefore, it seems that the introduction of chromogenic substrates in laboratory practice for the prothrombin time determination is possible and can offer considerable advantages like standardization and automation. The only disadvantage may be caused by costs involved.

Acenocoumarol↗
PubMed1987

A new family with congenital factor XIII deficiency showing a deficit of both subunit A and B. Type I factor XIII deficiency.

In this study we present a new case of Factor XIII deficiency. The proposita, a 34 year old woman, showed a deficiency of both subunit a and subunit b, and a moderate bleeding tendency. Because of the concomitant decrease of subunits a and b the proposita is considered to be an example of Type I disease. Factor XIII levels were less than 10% both as activity and antigen. Several family members showed intermediate levels of both subunit a and b and were asymptomatic. They were considered to be heterozygotes. The hereditary pattern is autosomal incompletely recessive. Type I disease appears much less frequent than Type II.

Adult↗
PubMed1987

Clotting changes in borderline hypertension.

Coagulation factors evaluated in a group of patients with borderline hypertension. The following tests were carried out: prothrombin time (PT) and partial thromboplastin time (PTT), Factor VIII coagulant activity, Factor VIII antigen and Factor VIII ristocetin cofactor, Factor XII and Factor XI activities. These tests were selected for their relationship to the contact coagulative activation near the vascular wall. Comparing the results with those of normal controls, Factor VIII coagulant activity, Factor XII and PTT levels were significantly higher. Other tests were all within normal limits in both groups. High Factor VIII and Factor XII levels associated with PTT shortening suggest that an increased synthesis and/or release of these coagulation factors was present in our patients. Activated coagulation seems to be present in borderline hypertension before the appearance of clinical signs of vascular lesions.

Adult↗
PubMed1986

Associated von Willebrand disease as a possible cause of lack of thrombosis in an AT III abnormality (AT III Trento).

In a family with a known antithrombin III abnormality (AT III Trento) an associated von Willebrand defect (Type I) was found. The two defects seem to segregate independently. In fact four types of individuals were present, namely: subjects with isolated AT III abnormality, subjects with isolated von Willebrand defect, patients with double defect and normal subjects. Only one of the two patients with isolated AT III abnormality showed a thrombotic tendency. None of the patients with double defect showed thrombotic disease, indicating a possible protective action of the von Willebrand defect against thrombotic manifestations. Patients with isolated von Willebrand defect showed neither thrombotic nor bleeding manifestations. The study emphasizes the need for a careful evaluation of the hemostatic balance of patients with AT III abnormalities before concluding that they are symptomatic or asymptomatic.

Antigens↗
PubMed1986

Erythrocyte Na+-Li+ countertransport and blood viscosity in arterial hypertension.

The aim of this study was to evaluate together the main hemorheologic parameters and one of the transmembrane ion transport systems in erythrocytes of subjects with normal and elevated blood pressure. Three sex-, age-, and weight-matched groups consisting of 15 normotensive subjects (NT) with no parental hypertension, 15 patients with essential hypertension (EH) at stage 1-II WHO, and eight patients with secondary hypertension (Sec.H), respectively, were studied. Red blood cell Na+-Li+ countertransport (CTT), blood viscosity (eta B) at shear rates of 230 X S-1, 115 X S-1, and 46 S-1 and plasma viscosity (eta P) at shear rate of 46 X S-1 were measured. Plasma proteins and fibrinogen were also evaluated. CTT was higher in EH than in NT (P less than 0.01), while no significant difference was found between NT and Sec. H patients. eta B at 115 X S-1 and 46 X S-1 was higher in EH, but not in Sec. H, than in NT patients (P less than 0.05). No difference in eta P, plasma proteins and fibrinogen levels was observed between EH and NT. Elevated eta B and/or CTT may indicate a structural alteration in the erythrocyte membrane of some essential hypertensive patients. This is consistent with the hypothesis that a widespread membrane disorder is involved in the pathogenesis of primary hypertension.

Adolescent↗
PubMed1986

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↗
PubMed1986

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↗
PubMed1986

First case in Italy of fatal AIDS in a hemophiliac.

The first fatal case of AIDS in an hemophiliac observed in Italy is reported. The propositus is a 53-year-old hemophilia A patient who died on the 8th December, 1984. AIDS was documented clinically and in the laboratory by serum antibodies to HTLV-III detected by ELISA and Western blot assays. A progressive intellectual worsening of the patient due to diffuse cerebral atrophy was followed by CT scan, EEG and by evaluation of proper neurological signs and symptoms.

Acquired Immunodeficiency Syndrome↗
PubMed1986

Congenital heterozygous plasminogen deficiency associated with a severe thrombotic tendency.

Heterozygous plasminogen deficiency was found in 2 patients (mother and daughter). The mother, aged 55 years, was symptomatic while the daughter, aged 10 years, was asymptomatic so far. The thrombotic tendency presented by the proposita (mother) was severe and included recurrent superficial, portal, mesenteric, subclavian thrombophlebitis. No arterial thrombosis was noted. Oral anticoagulants have been of some benefit. The main laboratory features were: plasminogen activity about 50% of normal in two amidolytic methods and in a caseinolytic method. Plasminogen antigen was also about 50% of normal using electroimmunoassay and radial immunodiffusion. Crossed immunoelectrophoresis revealed a normal, even though reduced pattern, thus excluding dysplasminogenemia. Routine coagulation tests were negative. Euglobulin lysis time, fibrinogen level and fibrinogen degradation products (FDP) were within normal limits. Antithrombin III, protein C and protein S were also within normal limits.

Child↗
PubMed1986

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↗