PubMed Health⌕ Search

Biomedical subjects

E Dini

Publications and source records attributed to E Dini.

At least 37 records · Page 2Linked to original sources
PubMed1987

Successful pregnancy in a woman with congenital factor XIII deficiency treated with substitutive therapy. Report of a second case.

A syndrome of marked fetal wastage is associated with congenital factor XIII deficiency in adult women. A previously unreported case of a woman with factor XIII deficiency is described, in which substitutive treatment with normal plasma or placental factor XIII concentrate permitted two normal pregnancies. Factor XIII activity was maintained above 1-2% with intermittent infusion of 300 ml to 450 ml of plasma every 14 days or of 500 units of concentrate every 21 days. This case confirms the only other case so far reported in which factor XIII substitutive therapy was able to permit a normal pregnancy in a woman with factor XIII deficiency and seems to suggest factor XIII to be involved in the process of annidation.

Abortion, Spontaneous↗
PubMed1987

Treatment of the lymphoid blast crisis of chronic myeloid leukemia.

Ten patients with lymphoid-type blast crisis of chronic myeloid leukemia were treated with combination chemotherapy comprising doxorubicin, vincristine, L-asparaginase, and prednisone. Once remission was achieved in 9 (90%), consolidation with doxorubicin, vincristine and cyclophosphamide was given, and then maintenance chemotherapy with 6-mercaptopurine and methotrexate. Median remission duration was 12 months (range 2-18) and survival 17 (range 3-29). Drug-related toxicity was manageable, leading to a major schedule alteration in 3 cases. These data suggest that combination chemotherapy including doxorubicin improves the prognosis of lymphoid blast crisis in chronic myeloid leukemia.

Adult↗
PubMed1987

Epidemiological investigation of the prevalence of von Willebrand's disease.

To evaluate the prevalence of von Willebrand's disease (vWd) we carried out an epidemiological investigation among school children of the Veneto region in northern Italy. A total of 1,218 of 1,281 possible children participated in the study. They were 11 to 14 years of age, and all attended secondary schools in two distinct small areas, 70 km apart, between which there is no social contact. A blood sample was taken from each subject for determination of the blood group and von Willebrand factor (vWf) level (measured as ristocetin cofactor and expressed in IU/dL after calibration of the internal pool against an international standard), and the parents were given a questionnaire concerning hemorrhagic symptoms in the members of the family in the last three generations. Separate normal ranges were calculated for blood group O and non-O subjects (1,166 children and 289 adults) with a nonparametric method because the distribution curves of the reference values did not fit the gaussian distribution. Diagnoses of vWd were considered only for children who had low vWf levels and were members of a family with a convincing bleeding history (case of "probable vWd"). A final diagnosis was assigned if, in addition to these criteria, at least one other family member on the side with hemorrhagic history had a low vWf level. Of the 1,218 children examined, ten were classified as having vWd (0.82%). Taking into account the 90% confidence interval for the lower limit of the normal range, this figure could range from 7 (0.57%) to 14 (1.15%). All these subjects were mildly to moderately affected and presented features of heterozygous classic vWd (type I). Affected subjects were distributed evenly in the two areas examined. Our results suggest that the prevalence of vWd might be much higher than previously reported and that a different screening approach might be of use for patients with mild bleeding diathesis.

Adolescent↗
PubMed1986

Preliminary observations on intravenous idarubicin (4-demethoxydaunorubicin) in the chronic and accelerated phase of chronic myelogenous leukemia.

The study included 13 patients with chronic myelogenous leukemia (8 in the chronic phase with high WBC counts at onset, and 5 in the accelerated phase, poorly responding to conventional drugs for the chronic phase). They were treated with 4-demethoxydaunorubicin (idarubicin), a new anthracycline analog more active than daunorubicin (DNR) and doxorubicin (DX) in experimental tumor models which offers a higher therapeutic index than existing anthracyclines. Idarubicin was administered i.v. at the dose of 8 mg/m2 on days 1, 3 and 5. All patients in the chronic phase (8/8) developed significant leukopenia. Five of these 8 patients showed complete reduction of splenomegaly, and 4 of hepatomegaly as well. In all the other cases, hepato-splenomegaly was reduced by more than 70%. Three of the 5 patients in the accelerated phase of chronic myelogenous leukemia also showed massive cytolysis. More important, all of them showed complete or major reduction of hepato-splenomegaly and renewed responsiveness to conventional drugs for the chronic phase of the disease. Idarubicin was fairly well tolerated by all patients with only minor gastrointestinal side effects and no liver damage or acute cardiotoxic effects. These findings indicate that idarubicin--although it cannot replace established drugs for the chronic phase of the disease--represents an added therapeutic resource for producing rapid cytolysis at onset and, above all, in the accelerated phase of chronic myelogenous leukemia.

Administration, Oral↗
PubMed1985

One year follow-up study of T-cell subsets and incidence of seropositivity for HTLV-I and HTLV-III antibodies in patients treated "on demand" or sporadically with clotting concentrates.

A 1-year follow-up study of the T-cell subset abnormalities was carried out in 16 severe haemophilia A patients, treated "on demand" with an average amount of 500 U/kg/yr of factor VIII concentrate (group A) and in 15 mild haemophiliacs or von Willebrand patients treated only sporadically with less than 3000 U of factor VIII and no longer exposed to any other blood component in the 2 years preceding the beginning of the study (group B). In group A, 50% and 70% of patients showed a reduced or inverted T 4/T 8 ratio, respectively, at the beginning and at the end of follow-up. These values were of 30% and 20% in patients of group B, suggesting a long-lasting effect of concentrate therapy on T-cell subsets. The low T 4/T 8 ratio was mainly due to an increase of suppressor cells. None of the patients was found positive for anti HTLV-I, whereas 3 patients, all belonging to the group A, showed antibodies against HTLV-III. Thus, in these patients, HTLV-III seems not to be the only cause of low T 4/T 8 ratio.

Antibodies, Viral↗
PubMed1984

Defective fibrin crosslinking in acute leukemia.

Fibrin crosslinking was assayed in 22 patients with acute leukemia showing secondary coagulation abnormalities of variable severity. In 9 patients fibrin crosslinking was found to be normal, whereas 10 patients presented impaired polymerization of alpha-chains and 3 of both alpha- and gamma-chains. Only a rough correlation was found between transamidating activity of factor XIII and the fibrin crosslinking pattern in these patients. Moreover, incomplete fibrin crosslinkage occurred at levels of factor XIII far in excess of that required for full polymerization of fibrin in "normal" plasma. This latter finding suggests that, in addition to factor XIII deficiency, other causes are responsible for the high rate of fibrin crosslinking impairment in acute leukemia.

Acute Disease↗
PubMed1984

Bleeding on patients with autoimmune thrombocytopenic purpura and normal platelet count.

80% of patients with chronic autoimmune thrombocytopenic purpura (ATP) may reach normalization of platelet count after steroids or splenectomy. In some of these cases a bleeding tendency may still persist and this has been attributed to abnormalities of platelet function resulting from the effect of platelet-bound antibodies. We have studied 49 ATP patients in order to assess the frequency and the pattern of this immune thrombopathia, and the correlation with the levels of platelet associated IgG (PAIgG). Only 3 patients (6%) had prolonged bleeding time and persistence of mucocutaneous haemorrhages, whereas 36% presented an altered platelet aggregation pattern. Platelet serotonin content, platelet and plasma beta-thromboglobulin concentration, and malondialdehyde generation after thrombin stimulus were found also altered, but the findings were not clearly correlated with the concentration of PAIgG.

Adolescent↗
PubMed1984

Liver dysfunction rather than intravascular coagulation as the main cause of low protein C and antithrombin III in acute leukemia.

Protein C, a newly identified inhibitor of blood coagulation, was measured immunologically in 58 patients with untreated acute leukemias and compared with that of normal subjects. On the average, slightly lower values were found. However, the 17 patients with overt laboratory pictures of decompensated disseminated intravascular coagulation (DIC), including 11 cases with acute promyelocytic leukemia, had protein C concentrations no lower than those of the remaining 41 patients without DIC. Antithrombin III activity and antigen were normal and, like protein C, not lowered in DIC. The concentrations of both proteins were closely correlated with changes in the indexes for liver synthetic function. A subgroup of 13 patients with hyperleukocytic leukemias had lower protein C and antithrombin III, in line with the more compromised synthetic function of their livers. Our findings indicate that liver impairment rather than DIC is the main cause of the changes in the two naturally occurring inhibitors of blood coagulation.

Adult↗
PubMed1983

Treatment of early stage Hodgkin's disease with radiation therapy plus short-cycle (3 MOPP) adjuvant chemotherapy.

Eighty-four consecutive, previously untreated patients with stage I, II A-B and IIIA Hodgkin's disease were treated with combined modality therapy including subtotal or total nodal irradiation, followed by three cycles of MOPP. MOPP was administered before radiotherapy in patients with systemic symptoms or with bulky disease. Seventy-six of 84 patients (90.5%) achieved complete remission, and 8 died from disease progression after a variable period of incomplete remission. Three of 76 (3.9%) relapsed, and 2 of them have been subsequently salvaged. Up to the present time, 70 patients are alive, without evidence of disease; 9 have died from Hodgkin's disease, 2 from acute non-lymphoblastic leukemia, and 3 from intercurrent causes. No death occurred from acute toxicity due to chemotherapy. Actuarial overall survival is 82.3% and freedom from relapse is 81.8% after 48 months' median observation (range: 12-111 months). No significant difference in survival and freedom from relapse has been observed with respect to age, sex, stage, presence or absence of unfavorable prognostic factors. The role of adjuvant chemotherapy and its use in a reduced number of cycles in early stage Hodgkin's disease are discussed.

Age Factors↗
PubMed1983

Thrombohaemorrhagic complications in 101 cases of myeloproliferative disorders: relationship to platelet number and function.

A series of 101 consecutive patients with chronic myeloproliferative disorders including polycythaemia vera, chronic myelogenous leukaemia, idiopathic myelofibrosis and essential thrombocythaemia have been studied. The aim was to establish the incidence of thrombotic and haemorrhagic complications and the possible role played by platelet number and function. The total incidence of haemostatic complications was 21% and the platelet functional tests investigated (platelet aggregation, generation of malondialdehyde, endogenous serotonin, beta-thromboglobulin and platelet coagulant activity) were of little help for predicting these clinical complications.

Adolescent↗
PubMed1983

Immunoelectrophoretic evidence of a thrombin-induced abnormality in a new variant of hereditary dysfunctional antithrombin III (AT III 'Vicenza').

An Italian family with thrombosis and hereditary dysfunctional AT III, i.e. reduced biological activity and normal levels of the immunoreactive protein is presented. The abnormal molecule, called AT III 'Vicenza', was characterized by two-dimensional crossed immunoelectrophoresis either in the absence or presence of heparin. In the presence of heparin, a normal pattern was found in plasma, but abnormality was evident in serum. A similar abnormality was found in plasma artificially clotted with thrombin. The role of thrombin in inducing the immunoelectrophoretic abnormality of AT III 'Vicenza' is discussed.

Adolescent↗
PubMed1983

Fatal pulmonary embolism and antithrombin III deficiency in adult lymphoblastic leukaemia during L-asparaginase therapy.

We describe 2 adult patients with acute lymphoblastic leukaemia (ALL) who died from pulmonary embolism following L-asparaginase treatment. Since this drug is known to cause a decrease in antithrombin III, the most important protein physiologically involved in the neutralization of thrombin, we studied the behaviour of this inhibitor in 14 ALL patients treated with a protocol including a 14-day course of L-asparaginase. A significant but transient fall of biological and immunological antithrombin III and a concomitant reduction of fibrinogen were documented.

Acute Disease↗