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Biomedical subjects

A Girolami

Publications and source records attributed to A Girolami.

At least 505 records · Page 28Linked to original sources
PubMed1980

Hemophilia B with associated factor VII deficiency: a distinct variant of hemophilia B with low factor VII activity and normal factor VII antigen.

Factor VII activity and cross-reacting material was assayed in fresh and deep frozen non-contacted plasma in 43 patients with Hemophilia B belonging to different kindreds. Factor VII activity was found to be slightly decreased (about of 50% normal) in 12 patients, regardless of the thromboplastin used. In an additional patient (hemophilia BM) factor VII was slightly decreased in 1 : 10 diluted plasma but was normal in further diluted plasma. In the remaining 30 patients factor VII activity was normal. No significant variation was found between fresh and deep frozen plasmas. Factor VII antigen or cross-reacting material was normal.

Antigens↗
PubMed1980

Factor X assays using chromogenic substrate S-2222.

Factor X was assayed using chromogenic substrate S-2222 for four patients with severe factor X deficiency and for nine patients with homozygous or heterozygous factor X Friuli disorder. Factor X Friuli disorder is characterized by the presence of an abnormal factor X that is normally activated by Russell's viper venom, but is not activated by tissue thromboplastins. The levels of factor X found in factor X deficiency varied between 2 and 10% of normal and therefore were higher than those found in the same plasmas using "clotting" methods (1% or less than 1% of normal). The levels of factor X found in homozygous factor X Friuli patients varied between 4 and 11% of normal, and therefore were practically identical to those found by means of clotting methods that employed tissue thromboplastins (7-9% of normal). These values were definitely lower than those obtained using a Russell's viper venom and cephalin mixture as thromboplastin (82-92%). A similar pattern was observed for patients heterozygous for the abnormality. These findings indicate that "amidolytic" methods are not necessarily identical to clotting methods. Furthermore, they indicate that substrate S-2222 is not specific for factor X.

Blood Coagulation Tests↗
PubMed1980

Chromogenic substrate (S-2238) prothrombin assay in prothrombin deficiencies and abnormalities. Lack of identity with clotting assays in congenital dysprothrombinemias.

Prothrombin was assayed using chromogenic substrate of S-2238 for patients who were being treated with coumarin, for patients who had liver disease, and for patients who had congenital hypoprothrombinemias and dysprothrombinemias. In coumarin therapy and in patients with liver disease the levels found correlated well with the one-stage clotting methods. The same was true for heterozygous and homozygous "true" prothrombin deficiency. In the case of congenital dysprothrombinemias the levels observed with the chromogenic substrate were higher than the clotting counterparts, particularly so in the case of prothrombin Padua. In the latter case the levels observed were always about 100% of normal, as compared with the levels of about 50% of normal found with clotting methods. These data indicate that chromogenic substrates are not always equivalent to "clotting" substrates, namely, that amidolytic activity is not always equivalent to clotting activity. Therefore the two methods cannot be used interchangeably, lest some defects escape detection.

Blood Coagulation↗
PubMed1980

Increased number of pseudodrumsticks in neutrophils and large platelets. A "new' congenital leukocyte and platelet morphological abnormality.

2 members of a family, a child and his father, showed a combined morphological abnormality of leukocytes and platelets. The abnormality consisted of the presence of pseudodrumsticks in the neutrophils and of large platelets. One or more than one pseudodrumstick was present in about 40% of neutrophils. Leukocyte count, differential count and enzymatic stains were normal. Large platelets constituted about 25% of the platelet population. 1 patient also had mild thrombocytopenia which appeared to be unrelated to the basic defect since it appeared after a parotitis infection. Platelet function was normal but for a moderate prolongation of the bleeding time in the patient who had mild thrombocytopenia. No chromosomal abnormality was present in the propositi. The condition seems different from other leukocyte and platelet abnormalities so far described.

Adult↗
PubMed1980

Factor VII survival studies in factor VII Padua abnormality.

Two patients with factor VII Padua abnormality were studied during prophylactic administration of factor VII concentrates for tooth extractions. The amounts administered were 15.8 and 17.2 units/kg body weight in 30 and 20 min, respectively. The end infusion factor VII activity levels were 55 and 64%, respectively. Survival times of the exogenous components were 6.5 and 6 h, respectively. Satisfactory hemolstasis was obtained in both instances.

Adult↗
PubMed1980

Clotting changes in two patients with longitudinal sinus thrombosis.

Two patients with angiographically proven longitudinal sinus thrombosis were studied from a coagulation point of view. In the first case, marked primary thrombocytosis was found. This patient was treated with aspirin, dipyridamole and radioactive phosphorus. In the second case, fibrinogen and whole blood plasma viscosity were elevated. This patient developed deep vein thrombosis of the left leg a few weeks after the cerebral episode and was treated with coumarin drugs. The importance of a coagulation study in every patient with cerebral vein thrombosis is emphasized.

Adult↗
PubMed1980

Plasma and platelet beta-thromboglobulin levels in patients with May-Hegglin anomaly.

Plasmatic beta-thromboglobulin (BTG) has been assayed in 5 patients with May-Hegglin anomaly. All patients showed a normal BTG plasmatic level. The plasma BTG/number of platelets ratio resulted to be elevated but this was due to the low platelet count typical of the disease. The platelet content in BTG was studied in only 2 patients and was found to be four times the normal value; however, the circulating BTG platelet mass in these patients resulted to be within normal limits.

Adolescent↗
PubMed1980

In vivo platelet release reaction in patients with heart valve prosthesis.

Platelets play an important role in the genesis of thromboembolic episodes frequently observed in patients following cardiac valve replacement. No simple and reliable method as yet exists for quantitating platelet function in vivo. Platelet-specific proteins, serum beta-thromboglobulin related antigen and platelet factor 4 as well as thromboxane B2 were measured in 50 healthy subjects and in 100 patients who had cardiac valve replacement at least 6 months previously; these were related to the type, site and number of valves replaced. Highly significant differences were observed in the mean plasma beta-thromboglobulin related antigen, platelet factor 4 and thromboxane B2 levels in patients compared with healthy controls. The elevated levels of platelet release products observed 6 months after cardiac valve replacement suggest a continuous activation by the prosthesis. No significant differences were observed in patients with different types and site of valve prosthesis.

Adult↗
PubMed1980

The congenital factor VII abnormalities (dysproconvertinemias). The genetic plot thickens.

Congenital factor VII deficiency is a well known clotting disorder first identified in 1951. Several congenital abnormalities of factor VII have been described during the past decade. These abnormalities were all characterized by the presence of a discrepancy between factor VII clotting activity and factor VII cross reacting material or antigen. Recently other factor VII abnormalities have been described which showed peculiar sensitivities to ox-brain thromboplastins as compared to thromboplastins of other origin. These discoveries have complicated the differential diagnosis of prothrombin complex factors deficiences and abnormalities. A correct diagnosis may be reached only by means of a battery of tests which employ tissue thromboplastins of different origin.

Animals↗
PubMed1980

A study of platelet function and morphology in a new family with May-Hegglin anomaly.

A new family with May-Hegglin anomaly is presented. 9 patients were found to be affected, namely to present thrombocytopenia, giant platelets, and leukocytes inclusion bodies. A mild to moderate hemorrhagic diathesis was present in 8 patients (easy bruising, excessive bleeding after tooth extraction, menomethrorrhagia). One patient was asymptomatic. The bleeding tendency seemed to be relatively more pronounced in those patients who have larger platelets. Bleeding time was slightly prolonged in 4 of the affected patients. Platelet aggregation to Ristocetin and serotonin release was normal; on the contrary, platelet adhesiveness was slightly decreased in all patients. Plasma Btg was investigated in 7 patients, found to be normal in 5 and elevated in 2. Platelet Btg was found to be increased in all patients investigated. The ratio between Btg and platelet number was elevated in every instance. The circulating platelet mass (Btg platelet mass microgram/ml) was investigated in 7 patients, found normal in two and decreased in the remaining three. The disorder is transmitted as an autosomal dominant trait but there seems to be a variable phenotypic expression from one patient to the other.

Adolescent↗
PubMed1980

Normal platelet adhesiveness and aggregation in congenital PTA or Hageman factor deficiency.

Platelet adhesiveness and aggregation were studied in two patients with congenital factor XI deficiency and in a patient with congenital factor XII deficiency. A normal aggregation pattern was observed in every instance, regardless of the aggregating agent. The same was true for platelet adhesiveness. It is concluded that factor XI and factor XII play no role in platelet aggregation and adhesiveness.

Bleeding Time↗
PubMed1979

Warfarin treatment during pregnancy: a clinical note.

The case history is presented of a woman aged 38 who received warfarin treatment because of a mitral valve replacement. During the first trimester of pregnancy, her prothrombin time was 20% of normal and kept at 40% until the 38th week of gestation. Then the patient received heparin until a few days after delivery of a normal child.

Adult↗
PubMed1979

Factor VII Padua 2: another factor VII abnormality with defective ox brain thromboplastin activation and a complex hereditary pattern.

A new factor VII abnormality is presented. The propositus was a 9-yr-old child who presented a mild bleeding tendency characterized by epistaxis and easy bruising. The parents were not consanguineous, but they came from the same area. The laboratory features were mild prolongation of prothrombin time and P.P. test and normal partial thromboplastin and Stypven cephalin clotting times. The Thrombotest was moderately prolonged. Factor VII was 40%-50% of normal using rabbit or human brain thromboplastin, but only 13%-24% using ox brain thromboplastin. Factor VII cross-reacting material (CRM) was about 50% of normal. The father, a paternal aunt, and a paternal cousin showed similar clinical and laboratory findings. The brother of the propositus, the mother, and other members of her family showed about 50% factor VII activity and CRM and were considered to be heterozygotes for true factor VII deficiency. Similar findings were also present in the father and in the brother of the affected cousin. The defect in the propositus seems to consist of a double heterozygosity between abnormal factor VII and heterozygous factor VII true deficiency. The factor VII abnormality appears to consist of abnormal reactivity toward ox brain tissue thromboplastins and appears to be different from previously described factor VII abnormalities. The name factor VII Paudua2 is proposed for this condition.

Adolescent↗