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Biomedical subjects

A Girolami

Publications and source records attributed to A Girolami.

At least 541 records · Page 30Linked to original sources
PubMed1977

Normotest--thrombotest discrepancy in congenital coagulation disorders of the prothrombin complex and in coumarin-treated patients: a nonspecific phenomenon.

A Normotest (NT)-Thrombotest (TT) discrepancy is claimed to reflect the presence of coumarin-induced inhibitors or intravascular coagulation, or both. The results of this study indicate, however, that a significant NT-TT discrepancy is also present in all plasmas from patients who have congenital coagulation disorders of the prothrombin complex. None of these patients had received an anticoagulant or showed any sign of intravascular clotting; nevertheless the discrepancy observed was similar to that found in plasmas of coumarin-treated patients: average values were 0.437 and 0.450, respectively. As no inhibitor was present in the congenital coagulation disorders, except in hemophilia BM, the phenomenon does not appear to be specific for coumarin plasma. This indicates that the NT/TT discrepancy is a non-specific phenomenon that does not seem to provide any additional information about coumarin-treated patients compared with that obtainable by means of a simple prothrombin time test.

Acenocoumarol↗
PubMed1977

An immunological investigation of hemophilia B with a tentative classification of the disease into five variants.

23 patients with hemophilia B have been investigated by means of several immunological methods. 16 patients (69.9%) had no detectable factor XI antigen. Five had a normal factor IX antigen and the electrophoretic mobility of this abnormal factor IX was similar to that of its normal counterpart. One of these five patients had hemophilia Bm, since ox brain thromboplastin clotting time was severely prolonged. The remaining two patients had reduced or decreased factor IX antigen. Several patients showed a slight protongation of ox brain thromboplastin time due to an associated slight factor VII deficiency. On the basis of these results, a tentative classification of hemophilia B into five variants is proposed, namely: hemctor IX antigen; hemophilia Bra, or with reduced factor IX antigen; hemophilia Bm, or with normal factor IX antigen and severely prolonged ox brain thromboplastin; hemophilia B patients is feasible only by means of a battery of tests, namely:factor IX activity assay, factor IX antigen determination, ox brain thromboplastin clotting time, factor VII activity assay.

Factor IX↗
PubMed1977

Congenital deficiency of factor XIII with normal subunit S and lack of subunit A. Report of a new family.

Two sisters born from a nonconsanguineous marriage were found to have congenital factor XIII deficiency. In the electroimmunoassay system, using an anti-subunit S antiserum, two distinct peaks or rockets were seen in normal plasma and serum whereas only one peak was present in the propositae plasma or serum. In the bidimensional immunoelectrophoresis system, using the anti-subunit S antiserum, two major peaks were seen in normal plasma whereas only one peak was seen in the propositae plasma. Using an anti-subunit A antiserum no peak or precipitate was seen in our propositae in the electroimmunoassay or in the bidimensional immunoelectrophoresis systems. Both the parents and the children of our two propositae showed a normal coagulation pattern. Therefore, the heredity appears to be autosomal recessive. These data indicate that the defect is characterized by a normal factor XIII subunit S (support) and a lack of factor XIII subunit A (activity).

Adult↗
PubMed1977

Combined factor VII and factor VIII deficiency due to a casual association of heterozygosis for factor VII deficiency and hemophilia A.

A patient with combined factor VII and factor VIII deficiency is discussed. The propositus is a 21-year-old male who presented a mild bleeding tendency. The patient appears to be a hemophilia and at the same time heterozygote for factor VII deficiency. This conclusion is based on the fact that heterozygosis for factor VII deficiency was present in the father and in other relatives of the paternal side. On the contrary, no factor VII deficiency was present in the maternal side of the family. However, the maternal grandfather was known to have been a bleeder and the propositus' mother, his sister and his aunt had low-normal factor VIII levels and were probably hemophilia A carriers. This type of combined factor VII and factor VIII deficiency appears to be due to the casual association of two independently segregating defects.

Adult↗
PubMed1977

The effect of heparin on the interaction of factor VIII and human platelets in vitro.

Heparin was found to inhibit the interaction between human factor VIII and platelets. This was noted in two different systems, namely, platelet aggregation induced by neuraminidase-treated human cryoprecipitate and platelet aggregation induced by ristocetin-human factor VIII complex. Heparin appeared to have an inhibitory effect on the above systems similar to that reported on bovine factor VIII-induced platelet aggregation.

Blood Platelets↗
PubMed1977

Clotting factors and platelets. Immunofluorescence evidence that fibrinogen and factor VIII are present in human washed platelets, whereas prothrombin complex factors and factor XIII are lacking.

Factor I (fibrinogen) and factor VIII were shown, by an indirect immunofluorescence technique, to be present in human washed platelets. In the case of fibrinogen, the immunofluorescent pattern had a 'clod distribution' up to a 1:128 dilution of the antiserum. Factor VIII showed a 'speckled' pattern up to the 1:64 dilution. Cross-absorption studies confirmed the presence of such factors. Factor II, VII, IX, X and XIII, however, were not found in washed platelets. A diffuse fluorescence was noted using normal rabbit sera, but this was due to a cross-reaction, since it disappeared using a 1:32 diluted sera.

Animals↗
PubMed1977

Glass activation-induced decrease or disappearance of normotest-thrombotest discrepancy in congenital coagulation disorders of the prothrombin complex.

Exposure of plasma to glass for 60 minutes will cause a sharp, progressive shortening of Thrombotest clotting times in all congenital disorders of the prothrombin complex. On the other hand Normotest clotting times will change only slightly or moderately after the same glass activation. Consequently the Normotest-Thrombotest percentile discrepancy, quite marked at zero time, became progressively smaller. This behaviour is similar to that observed in a group of coumarin treated patients. These studies indicate that the Normotest/Thrombotest discrepancy does not allow any differentiation to be made between congenital coagulation disorders and coumarin induced defect. This is consistent with the non-specificity of the phenomenon.

Blood Coagulation Disorders↗
PubMed1977

Thrombotest mixing experiments in congenital coagulation disorders of the prothrombin complex and in coumarin treated patients. An additional evidence against the presence of an inhibitor in the latter.

Thrombotest clotting times of mixtures of coumarin plasmas and normal plasma yielded a patterm similar to that observed in mixtures of plasma with congenital coagulation disorders and normal plasma. The presence of 10 or 20% of test plasma in the mixture failed to affect the clotting times which resulted in normal limits. The only exception to this rule was the hemophilia BM plasma. In this case even the presence of 10-20% of patient plasma in the mixture caused a prolongation of the clotting time. This indicates that no inhibitor is present in coumarin plasmas and in the plasma of congenital coagulation disorders of the prothrombin complex save for hemophilia BM plasma which does contain an inhibitor.

Acenocoumarol↗
PubMed1977

Platelet adhesiveness and aggregation in combined factor V and factor VIII deficiency and in combined factor VII and factor VIII deficiency.

Platelet aggregation and adhesiveness were studied in 3 patients with combined factor V and factor VIII deficiency and in 3 patients with combined factor VII and factor VIII deficiency. The first three patients belonged to three different kindreds whereas the second group belonged to the same kindred. Serotonin C14 uptake and release was also found to be normal in these patients. These studies indicate that platelet function is normal in combined defects of factor VIII. These findings were in agreement with the presence of a normal bleeding time and a normal factor VIII antigen level in all these patients.

Blood Coagulation Disorders↗
PubMed1977

Apparent decrease of "inhibition" in the dilution curve system with the increase of anticoagulation. A paradox that is against the presence of inhibitors in coumarin treated patients.

The apparent "inhibition" found in coumarin plasma by means of the dilution curve system seems to decrease with the increase of anticoagulation. Three groups of anticoagulated patients with a P/N prothrombin time ratio of less than 1.8, between 1.8 and 2.3 and over 2.3, showed an "inhibition" of 1.6, 1.1 and 0.9 conventional units, respectively. An inverse correlation was found to exist between Thrombotest clotting times, P/N ratio, antigen/activity ratios on one side and inhibition units on the other side. These findings do not confirm the presence of "inhibitors" in coumarin treated patients.

Blood Coagulation Disorders↗
PubMed1977

[Hematic and plasmatic viscosity in cyanotic congenital heart diseases (author's transl)].

57 patients with cyanotic congenital heart diseases were studied. 39 patients presented t. of Fallot, 18 patients had patent ductus arterious with pulmonary hypertension and right to left shunt. The average whole blood viscosity in patients with t. of Fallot was 6.86+/-0.32 cps. This parameter was correlated with hematocrit, fibrinogen, euglobulin lysis time, pulmonary output, systemic output, and O2 saturation. The value of "r" resulted as statistically significant only for the correlation between viscosity and hematocrit and between viscosity and fibrinogen (t=13.22, p less than 0.0005; t=3.35, p less than 0.001 respectively). The average value of plasma viscosity in patients with t. of Fallot was 1.61+/-0.024 cps. The correlations of this parameter with fibrinogen, euglobulin lysis time, pulmonary output and systemic output did not result as statistically significant. The average fibrinogen value was 256.10+/-20.63 mg%, whereas that of euglobulin lysis time was 12,67+/-1.50 hours. In patients with patent ductus arteriosus the average blood viscosity was 6.65+/-0.56 cps. Such parameter was correlated with hematocrit, fibrinogen, euglobulin lysis time, pulmonary output, systemic output and O2 saturation. The value of "r" resulted as statistically significant only between blood viscosity and hematocrit and between blood viscosity and O2 saturation (t=9.30, p less than 0.0005; t=5.800, p less than 0.001 respectively). The average value of plasma viscosity was 1.61+/-0.04 cps. This parameter was correlated with fibrinogen, euglobulin lysis time, pulmonary output and systemic output. The correlation index "r" never resulted as statistically significant. The average fibrinogen value was 299.20+/-20.30 mg. The average euglobulin lysis time was 16.02+/-2.73 hours.

Adolescent↗