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Biomedical subjects

A Girolami

Publications and source records attributed to A Girolami.

At least 451 records · Page 25Linked to original sources
PubMed1983

Antithrombin III (AT III) Padua2: a "new" congenital abnormality with defective heparin co-factor activities but no thrombotic disease.

A "new" antithrombin III (AT III) abnormality is described in five members of the same family. None of the affected members showed thrombotic manifestations and no consanguinity was present in the family. The main laboratory features were: normal routine clotting tests, slightly decreased AT III activities in all assays carried out in the presence of heparin. In the absence of heparin, antithrombin III activities were instead within normal limitis. Progressive AT III activity and AT III antigen were also normal. Crossed immunoelectrophoresis in the absence of heparin showed a normal pattern both in plasma and serum. In the presence of heparin, the propositi's plasma showed a major, less anodal, abnormal peak and a smaller normal peak. Three peaks were present in the propositi's serum as compared with the two normal ones. This AT III abnormality is different from AT III Padua previously described by us and we propose the toponym of Antithrombin Padua-2 to define this condition.

Adult↗
PubMed1983

Normal and low molecular weight heparins: interaction with human platelets.

Porcine mucosal heparin was chemically depolymerized. The depolymerization was stopped at different steps to obtain two low molecular weight (LMW) heparins with a molecular weight of 10 000 and 6000, respectively. The LMW heparins were tested in vitro for anti-clotting activities and for platelet serotonin release in different systems in comparison with normal heparins, dermatan and heparan sulphate. After addition of various amounts of heparin preparations to washed platelets, no significant release was observed for all tested heparins. On the contrary, different heparins showed an inhibition of serotonin-release induced by collagen in platelet rich plasma, whereas the ADP-induced release was increased. The effect on the platelet release appears related to the molecular weight. In fact, it is significant only for normal heparins whereas it is not for LMW heparins. A good relation was observed, also, between anti-activated factor X activity/antiglobal clotting activity (Xa/APTT) ratio of different heparins and the effect on platelet release.

Adenosine Diphosphate↗
PubMed1983

Antithrombin III Padua: a "new" congenital antithrombin III abnormality with normal or near normal activity, normal antigen, abnormal migration and no thrombotic disease.

A "new" antithrombin III abnormality is described in four members of a family. The proposita is a 38 years old female who showed no thrombotic disease and the following laboratory pattern: normal routine clotting tests, normal or near normal AT III activity (chromogenic substrates S-2238 and Chromozym Th) both in plasma and in serum and in the presence or absence of heparin, slightly decreased antifactor Xa activity (chromogenic substrate S-2222), normal progressive antithrombin, normal AT III antigen but abnormal migration in the agarose-heparin bidimensional system. In the latter test, one major abnormal peak, less anodal than the normal counterpart, and a smaller, apparently normal peak, were seen. In agarose without heparin the pattern was similar to normal both in plasma and in serum. Heparin tolerance to heparin in vivo and in vitro was slightly increased but still within normal limits. The two sons and a paternal aunt showed the same pattern. The hereditary pattern seems therefore autosomal dominant. The abnormality described appears different from AT III Budapest. The toponym of antithrombin III Padua is proposed to define this peculiar abnormality.

Adult↗
PubMed1983

Clotting changes in Cushing's syndrome: elevated factor VIII activity.

Thirteen women and 2 men affected by Cushing's syndrome were investigated. The following parameters were used: plasma and urinary cortisol levels, factor VIII assay (antigen, activity and von Willebrand factor) together with other coagulative assays. Samples were taken before surgery or before medical and/or radiation therapy and every 30-50 days after treatment and continued for 11 months. Cortisol and factor VIII were increased before treatment and decreased slowly after treatment to become normal in 3-4 months. Other clotting tests did not show any significant changes. High plasma cortisol levels seem to stimulate the production of factor VIII. Patients with Cushing's syndrome often exhibit thromboembolic complications after surgery. The clotting abnormalities responsible for such complications may be due to increased factor VIII activities.

Adrenalectomy↗
PubMed1983

Report of the fifth homozygous patient with factor VII Padua defect.

A new patient with factor VII Padua abnormality is presented. The propositus is a 70 old man who showed a mild bleeding tendency characterized by occasional epistaxis and a laboratory pattern of prolonged prothrombin time corrected by normal serum, normal partial thromboplastin time and normal Thrombotest. Factor VII activity was 7% using rabbit brain thromboplastin and 105% of normal using ox-brain thromboplastin. Intermediate levels were found by using thromboplastin of human origin. Factor VII cross-reacting material was normal. Parents were not consanguineous but both came from the same area. Two children of the propositus were found, as expected, to be homozygous for the abnormality. No relationship could be traced between the propositus and the other homozygous patients already reported. However, the patient came from the same geographic area, namely the Piave river valley in northeastern Italy. The discovery of the present patient, the fifth in four years, indicates that the defect might be more frequent than originally assumed.

Aged↗
PubMed1983

Failure of sulphinpyrazone to affect platelet survival in patients with rheumatic heart valvular disease: a double blind study using 75Se-methionine labeled platelets.

We have investigated platelet survival time in 18 patients suffering from valvular heart disease. Platelet survival was performed using the 75Se-methionine method modified by us. Nine of our eighteen patients underwent surgery for heart valve replacement. Platelet survival time was performed before treatment and six months afterwards with placebo or sulfinpyrazone in a double blind study. Before treatment and surgery, platelet survival time was significantly reduced in patients with a history of embolism (P less than 0.0048). In patients receiving valve replacement, platelet survival time was shortened both in the sulfinpyrazone and placebo groups six months after surgery. Of the nine patients not receiving prostheses and with a thrombotic history, treatment with placebo and sulfinpyrazone resulted in improved platelet survival times.

Adult↗
PubMed1983

A statistical analysis of some thromboelastographic parameters in normal subjects.

Thromboelastographic parameters r, k and am have been statistically evaluated in 777 normal subjects, 506 females and 271 males. The relationship existing among the parameters and their relationship with age have been investigated: r and k resulted to be correlated both in the male and female group. The same was true for k and am. On the contrary, a non-Gaussian distribution was noted together with the lack of correlation with age.

Adolescent↗
PubMed1983

In vitro stability studies of a Factor IX concentrate (Bebulin).

Factor IX: C activity decay was studied in lyophilized concentrates (Bebulin, IMMUNO) stored at room temperature and in reconstituted preparations frozen and stored at -20 degrees C. A very slight, practically identical, decay of Factor IX: C was found in lyophilized concentrates kept at room temperature, in reconstituted lyophilized concentrates frozen and stored at -20 degrees C and lyophilized concentrates kept at +4 degrees C. At the end of the experiment, which was 4-5 months after the expiration date of the product, a 15-30% decay of Factor IX: C could be found in the concentrates with respect to initial levels.

Blood Coagulation Factors↗
PubMed1982

Increased factor VIII associated activities in Cushing's syndrome: a probable hypercoagulable state.

Thirteen women and 2 men affected by Cushing's syndrome were investigated. The following parameters were used: plasma and urinary cortisol levels, factor VIII assay (antigen, activity and von Willebrand factor) together with other coagulative assays. Samples were taken before surgery or before medical and/or radiation therapy and every 30-50 days after treatment and continued for 11 months. Cortisol and factor VIII were increased before treatment and decreased slowly after treatment to become normal in 3-4 months. Other clotting tests did not show significant changes. It seems that high plasma cortisol levels may stimulate the production of factor VIII. Patients with Cushing's syndrome often exhibit thromboembolic complications after surgery. It is likely that the clotting abnormalities responsible for such complications is the increased factor VIII activities level.

Blood Coagulation Disorders↗
PubMed1982

Immunohistochemical identification of factor X-like antigen in the A cells of the normal human pancreas.

An indirect immunofluorescence technique using two rabbit anti-human factor X sera was used to investigate the presence of factor X in various human tissues. Of all tissues studied only the cytoplasm of some islet cells of the six human pancreases illustrated specific reactivity for factor X-like material. No reaction was seen in the exocrine pancreas. Cross-absorption studies with factor X-deficient plasma, glucagon or alpha-1-antitrypsin did not modify the pattern. On the contrary, cross-absorption with factor X Friuli or with normal plasma eliminated the positivity. No factor X-like material was seen in rat or guinea pig pancreas. The double-immunofluorescence technique demonstrated that the reactivity was localized only in the cytoplasm of the A cells.

Animals↗
PubMed1982

Further studies on factor VII Padua defect: the report of the fourth homozygous patient from the same valley.

A new patient with factor VII Padua abnormality is presented. The proposita is an 11-year-old girl who showed a mild bleeding tendency and a laboratory pattern characterized by a prolonged prothrombin time corrected by normal serum, normal partial thromboplastin time and normal Thrombotest. Factor VII assay was 10% using rabbit brain thromboplastin and 100% of normal using ox brain thromboplastin. Factor VII cross-reaction material was normal. The parents were not consanguineous but both came from the same area and were found to be heterozygous for the abnormality. The discovery of the present patient, the fourth in three years, indicates that the defect might be more frequent than originally thought.

Blood Coagulation Tests↗
PubMed1982

Incidence, significance, and subtypes of hemophilia BM in a large population of hemophilia B patients.

Eleven patients with hemophilia BM were found out of a population of 66 patients with hemophilia B. Factor IX activity in the hemophilia BM varied between less than 1% and 1.6% of normal but factor IX antigen was normal or only slightly reduced in each instance. Thrombotest clotting time was variably prolonged and was not corrected by the addition of normal plasma. Thrombotest mixing experiments and dilution curve studies confirmed the presence of the inhibitor in every patient. There are at least two forms of hemophilia BM, a severe one and a mild one. In the first form, Thrombotest is severely prolonged (90-120 s). In the other, the prolongation is mild or moderate (60-80 s). A positive correlation exists between the antigen-activity difference (delta antigen-activity) and the prolongation of Thrombotest both in the propositi and in obligatory carriers. The criteria for the diagnosis of hemophilia BM are the following: prolonged PTT, prolonged Thrombotest, lack of correction of Thrombotest by the addition of normal plasma while PTT is fully corrected. The lack of correction of Thrombotest in the presence of a full correction of PTT, is the unique clotting feature.

Adolescent↗