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Biomedical subjects

A Casonato

Publications and source records attributed to A Casonato.

104 records · Page 6Linked to original sources

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↗

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↗

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↗

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↗

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↗

The effect of certain antiplatelet drugs on in vitro malonylaldehyde (MDA) production.

Several antiaggregating agents were tested with regard to their effect on platelet malonylaldehyde (MDA) production which seems to be a good marker of prostaglandin metabolism. Non-steroidal antiinflammatory compounds, namely acetylsalicylic acid (ASA), ditazole and sulfinpyrazone, showed a sure, statistically significant, inhibitory effect on MDA production while theophylline, adenosine and alpha-blocking agent nycergoline did not. Dipyridamole on the contrary, was demonstrated to have a mild but still statistically significant effect on the inhibition of platelet MDA production.

Blood Platelets↗

Platelet fibrinogen: subcellular localization by means of immunofluorescent studies in normals and in congenital afibrinogenemia.

We have studied the site of fibrinogen localization in normal platelets and in the platelets of a patient with congenital afibrinogenemia (CA). The methods employed were: direct immunofluorescence technique (DIT) and indirect immunofluorescence technique (IIT). By means of the DIT normal platelets were shown to have a clear peripheral staining. Such staining disappeared after treatment with proteolytic enzymes and after specific blocking experiments. Such peripheral staining of platelets was absent in congenital afibrinogenemia even after fibrinogen infusion. By means of the IIT platelets were shown to have a considerable amount of fibrinogen. Such protein was demonstrated to represent an important part of platelet surface, since intact platelets were able to absorb completely a specific antifibrinogen antiserum.

Afibrinogenemia↗

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↗

Antiphospholipid syndrome in a child with trisomy 21: the relationship between anticardiolipin G antibodies and the von Willebrand factor.

A young child with trisomy 21 developed severe multiple arterial thromboses in a three-phase clinical course which proved fatal. The episodes were characterized by high levels of both IgG anticardiolipin antibodies and the von Willebrand factor. The data suggest that there is a relationship between these two elements and the clinical events which followed.

Antibodies, Anticardiolipin↗

The use of fluorescence flow cytometry in the characterization of Bernard-Soulier syndrome and Glanzmann's thrombasthenia.

We have used flow cytometry analysis of fluorescence to study the binding of murine monoclonal antibodies to platelets. Anti-platelet glycoproteins Ib (AP1), the complex IIb-IIIa (LJP9) and the FITC-conjugated second antibody were added directly to the sample of platelet rich plasma without washing. The analysis was performed in normals and in patients affected by the Bernard-Soulier syndrome and Glanzmann's thrombasthenia and compared to the direct binding of radioiodinated monoclonas. Comparable results were obtained. A symmetric profile of fluorescence for both glycoproteins Ib and IIb-IIIa was observed in normals and in the patient group, which indicates homogeneous distribution of these glycoproteins on the platelet surface. The develop technique allows identification of homozygous and heterozygous carriers of the platelet disorders studied and quantification of the defect, which seems to be due to a homogeneous decrease of specific glycoproteins in all platelet populations.

Adult↗