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Biomedical subjects

E Dini

Publications and source records attributed to E Dini.

At least 55 records · Page 3Linked to original sources
PubMed1981

Reduced platelet factor X-activating activity: a possible contribution to bleeding complications in polycythaemia vera and essential thrombocythaemia.

A recently described platelet coagulant activity, factor X-activating activity (FXAA), was studied in 33 patients with polycythaemia vera and in 5 with essential thrombocythaemia, in order to gain information about possible mechanisms responsible for the haemostatic disorders observed in myeloproliferative diseases. Other parameters of platelet function including bleeding time, spontaneous and ADP-, epinephrine- or collagen-induced platelet aggregation, serotonin content and malondialdehyde (MDA) production in response to thrombin, were investigated simultaneously. Compared to the range obtained from 27 control subjects (60-150%), FXAA was low in all 9 patients with bleeding complications (range: 5-39%), in 1 out of 7 patients with thrombotic manifestations (range: 38-200%) and in 9 out of 22 patients without symptoms (range: 38-500%). Only the bleeding group differed significantly from each of the others. Moreover, of all patients with reduced FXAA, those with bleeding could be clearly distinguished from those without such symptoms on the basis of the degree of the defect. Increased FXAA (above 150%) was found in 2 out of 7 thrombotic patients and in 2 out of 22 asymptomatics. MDA production was significantly lower in the haemorrhagic group and enhanced in the thrombotic one as compared to control subjects or asymptomatic patients. However, a large area of overlap was observed between the four groups. None of the other platelet function parameters studied (bleeding time, platelet aggregation, platelet serotonin content) correlated with the clinical type of haemostatic complication. Our results suggest a significant association between reduced FXAA and bleeding tendency. These findings may be of relevance in understanding the pathogenesis of abnormal bleeding in patients with polycythaemia vera and essential thrombocythaemia.

Adolescent↗
PubMed1980

Abnormal aggregation and increased size of platelets in myeloproliferative disorders.

Platelet aggregation is frequently impaired in myeloproliferative disorders (MPD). The presence of spontaneous platelet aggregation (SPA) or alterations of the aggregation pattern after ADP, epinephrine and collagen have been a frequent feature in the series of 52 patients here presented. The occurrence of SPA was not related to the findings obtained with the aggregating agents or to the number of circulating platelets. The aggregation results did not correlate with the number of megathrombocytes. The presence of platelet abnormalities, not completely corrected after busulfan therapy, and the increased size of platelets should aid in diagnosis of myeloproliferative disorders.

Adult↗
PubMed1978

Subunits A and S inheritance in four families with congenital factor XIII deficiency.

Previous studies of the inheritance of the two molecular subunits of fibrin stablizing factor (factor XIII) refer to isolated cases. The present work investigates the hereditary mode of transmission of subunits A and S, measured by the Laurell technique, in seven homozygotes and in 29 heterozygotes belonging to four families with factor XIII deficiency. The results indicate that the homozygotes were devoid of immunologically identifiable A subunit, whereas the heterozygotes could be identified by measuring this protein. The subunit S has been found to be decreased both in homozygous and in heterozygous patients, so that it seems that the two subunits, even if their synthesis is controlled by different genes, are genetically related. The mode of transmission of this disorder, supported by quantitative determinations of plasma subunit A, is autosomal recessive.

Factor XIII↗
PubMed1977

The aspirin tolerance test in von Willebrand's disease.

Opinions about the clinical value of aspirin tolerance test proposed by Quick von Willebrand's disease are conflicting. The results of the present study seem to support the view that a platelet defect, induced by aspirin, causes a significant lengthening of bleeding time only in the cases with severe von Willebrand factor deficiency.

Aspirin↗
PubMed1976

Relationship between Hb and HbA2 concentrations in beta-thalassemia trait and effect of iron deficiency anaemia.

The relationship of HbA2 to total Hb has been measured in 204 cases of Beta-Thalassaemia (beta-th-t) where the total Hb varied from 3.6 to 16 g/100 ml. The variation of total Hb in the study population was due, as far as could be defined, only to beta-th-t and a superimposed iron deficiency anaemia (IDA). Superimposed IDA decreases HbA2 proportionately more than it does total Hb (P less than 0.001). A not previously described relationship between HbA2 and total Hb was demonstrated and probably conformed to a second degree parabola.

Anemia, Hypochromic↗
PubMed1975

Relationship between Hb and HbA2 concentrations in untreated and Fe-treated iron deficiency anaemia.

The absolute concentrations of Hb (x) and HbA2 (y) were statistically correlated (a = 0.0208; b = 0.0170; r = 0.4700; P less than 0.05) in 29 subjects with iron deficiency anaemia. 15 days after a month course of iron therapy the mean percentage increase of HbA2 was greater (123.9%) than that of Hb (62.0%). HbA2 concentration was still correlated with that of Hb (r = 0.6632; P less than 0.001), but the values for intercept and slope (a = - 0.1476; b = 0.0383) indicated a relatively greater production of HbA2.

Anemia, Hypochromic↗
PubMed1975

Relationship between Hb and HbA2 concentrations in healthy and iron deficient subjects.

In 23 healthy subjects and in 115 patients with various degrees of chronic iron deficiency anaemia without congenital abnormalities of globin synthesis, Hb ranged from 3.4 to 16.3 g/100 ml. HbA2 ranged from 0.0550 to 0.5250 g/100 ml. Hb and HbA2 were statistically correlated, as shown by linear regression analysis (a equals --0.1387; b equals 0.0372; r equals 0.8198; P smaller than 0.001). The second degree parabola was not statistically different, but it gave a biologically preferable Figure for intercept (a equals --0.0006; b equals 0.0070; c equals 0.0015; r equals 0.8324; P smaller than 0.001). The second degree parabola was to be preferred also on the basis of previous literature results. Shortness of iron seems to reduce more the HbA2 than the Hb levels.

Anemia, Hypochromic↗
PubMed1975

Desferrioxamine B: reversible side effects of high daily doses.

16 patients with chronic liver or haematologic diseases were parenterally given various doses of Desferrioxamine B (DF). Each daily dose of DF (from 1 to 4 g) was given for a 7 days cycle. Liver, kidney and blood functions were investigated at the first and seventh day of each cycle, and 1 and 2 weeks after therapy was stopped. 1 g/day and 2 g/day had no side effects, with the exception of a fall of white blood cell (WBC) count in a single case on 2 g/day. 3 g/day (15 patients) were followed by rises of blood urea, creatinine, alkaline phosphatase and glutamyl-transpeptidase respectively in 4 cases, and by falls of WBC count in 3 cases. 4 g/day (9 patients) caused rises of creatinine, GPT and GOT (1 case) or LDH (1 case), while WBC count dropped in 4 cases. All changes were reversible within one-two weeks. These recorded changes were outside the range of pretreatment values as obtained over the previous four weeks.

Adolescent↗
PubMed1975

A new family with congenital factor XII deficiency.

The case of a patient with Hageman trait and his family study are reported. Commercial plasma thromboplastin time (PTT) reagents showed a good sensitivity for detecting the plasma defect. By prolonging the incubation time of the mixture containing PTT reagent and factor XII-deficient plasma the abnormal coagulation times were not corrected. Thus, a concomitant Fletcher factor deficiency could be excluded.

Blood Coagulation Disorders↗
PubMed1975

Dissociation between factor VIII (activity and antigen) and ristocetin-induced platelet aggregation in von Willebrand's disease.

Previously we showed that von Willebrand patients could be separated into 2 groups on the basis of ristocetin-induced platelet aggregation and factor VII (antigen and activity). Ristocetin-induced platelet aggregation and factor VIII exhibited a positive correlation. This paper deals with 5 out of 31 examined von Willebrand patients in which ristocetin-induced platelet aggregation was not in correlation either with factor VIII activity or antigen. The importance of these findings is discussed.

Antigens↗