Search PubMed⌕ Search

Biomedical subjects

F Mandelli

Publications and source records attributed to F Mandelli.

At least 433 records · Page 24Linked to original sources
PubMed1981

Polymorphonuclear leukocyte transfusion for the treatment of sepsis in the newborn infant.

A therapeutic trial of transfusions with polymorphonuclear leukocyte concentrates was performed in newborn infants with bacterial sepsis proven by blood culture. With each transfusion, 20 ml/kg of a preparation obtained by continuous flow filtration leukapheresis, and containing 0.5 to 1 x 10(9) WBC, with less than 6% lymphocytes, was administered. Twenty newborn infants with sepsis received from 2 to 15 PMN transfusions. Results were compared with findings in 18 newborn infants with sepsis admitted during the trial period, and not treated because of unavailability of the PMN preparation (Group B). Infants with fulminant illness were excluded from both groups. Groups A and B were similar with respect to clinical characteristics and to etiology (in the majority cases a highly antibiotic-resistant Klebsiella). The mortality rate was significantly lower in Group A than in Group B in the whole series (10% vs 72%, P < 0.001), and also in the subgroups with birth weight equal or below 1,500 gm (10% vs 91%, P < 0.001). Major complications and associated conditions (i.e., necrotizing enterocolitis, meningitis, pneumonia, peritonitis, osteoarthritis, disseminated intravascular coagulation) were observed in 12 patients of Group B, and in only three infants of Group A. Untoward effects attributable to PMN transfusions were never observed. PMN transfusion was a highly effective therapeutic tool in our population of infected newborn infants.

Bacterial Infections↗
PubMed1981

Factor VII deficiency: immunological characterization of genetic variants and detection of carriers.

Twenty-one patients with congenital factor VII deficiency belonging to 16 different kindreds were investigated. The existence of three immunochemical variants on the ground of factor VII activity (VII:C) and factor VII-related antigen (VII:Ag) levels (VII-, VII+ and VIIR) was established. There was no correlation between the presence of factor VII:Ag and either the clinical picture or the specific function as studied with the Km calculations. Genetically the model of inheritance is the same whatever the immunological variant, but the identification of carriers is simplified when the presence of factor VII-related antigen is assayed throughout a kindred.

Antigens↗
PubMed1981

Combination chemotherapy for marrow relapse in children and adolescents with acute lymphocytic leukaemia.

38 children with acute lymphocytic leukaemia (ALL) in haematologic relapse were retreated with vincristine, daunomycin and prednisone (VPD) together with intrathecal methotrexate and prednisone, followed by asparaginase in those patients not in complete remission after 4 weeks. The overall complete remission (CR) rate was 79%; asparaginase was needed to achieve CR in 7 of the 30 responding patients. The median duration of second remission was only 36 weeks, but 6 out of 15 children receiving the COAP-POMP-CART consolidation regimen remain in continuous second remission after 37-260 weeks; 3 of them are currently off all therapy. It is concluded that a prolonged second remission can be achieved in children with ALL in bone marrow relapse by combining intensive chemotherapy with the prevention of meningeal leukaemia.

Adolescent↗
PubMed1981

Chronic idiopathic thrombocytopenic purpura (ITP): site of platelet sequestration and results of splenectomy. A study of 197 patients.

51Cr-platelet kinetics study was performed in 197 patients with chronic ITP after corticosteroid therapy had failed to induce a long lasting remission. The incidence of splenic, spleno-hepatic, hepatic and diffuse platelet sequestration site was 58%, 17%, 6% and 19%, respectively. Splenic and spleno-hepatic sequestration sites were more frequent in patients less than 30 years old and in patients with a platelet count lower than 50 x 10(9)/1. 111 patients were splenectomized shortly after the study. Normalization of the platelet count was obtained more frequently in patients with splenic and spleno-hepatic sequestration than in the others. Labelled platelet sequestration site was the best predictor of the outcome of splenectomy. Platelet kinetics is a non-invasive investigation that should be performed early after the diagnosis of chronic ITP in all patients eligible for splenectomy.

Adolescent↗
PubMed1981

Glucocorticoid receptor determinations in leukemia patients using cytosol and whole-cell assays.

Parallel determinations of glucocorticoid receptors in the cells of patients with various forms of leukemia were made by two assay methods, one using cell-free cytosolic extracts and the other using whole-cell preparations. Both assays revealed saturable binding of triamcinolone acetonide in all cases. The mean equilibrium dissociation constant for the interaction of triamcinolone acetonide with the cytoplasmic receptor at 2 degrees was 9.45 +/- 6.33 (S.D.) nM while that for the whole-cell binding at 37 degrees was 6.13 +/- 3.25 nM, suggesting an increase in receptor affinity at physiological temperatures. Competition experiments with various unlabeled steroids revealed a higher degree of glucocorticoid specificity at 37 degrees in whole-cell suspensions than at 2 degrees in cytosol. In a comparative analysis of 41 leukemic cell specimens, it was found that determinations carried out by whole-cell assay, calculated as number of sites per cell correlated well with those performed by cytosol assay, calculated as fmol/mg protein, independent of the type of leukemia. However, for cells with low receptor content, the two assay methods were more difficult to compare. In agreement with previous reports, the cytosol assay consistently underestimated the number of receptors with respect to the whole-cell assay, particularly in cases of lymphatic leukemia. Furthermore, the underestimation decreased for increasing levels of total cellular receptor. These results suggest that, in addition to possible defects in the cytoplasm-to-nucleus translocation process, the acceptor-binding capacity of the nucleus may also represent one of the factors which determines the levels of assayable cytoplasmic receptors. Moreover, they indicate that the two assay methods furnish nonequivalent information.

Cytosol↗
PubMed1980

Discontinuing therapy in childhood acute lymphocytic leukemia. A multicentric survey in Italy.

The results of discontinuing therapy in children with acute lymphocytic leukemia observed at four associated institutions are presented. Of the 247 patients who achieved complete remission, 122 (49.3%) reached the point of discontinuing therapy after 2-4 years of continuous remission. The median period off therapy was 13 months with a range of 1-69 months. Of the 122 children removed from therapy, 27 (22.1%) relapsed, mainly in the bone marrow; relapses occurred 1-32 months after cessation of therapy (median ten months) with only two relapses occurring later than two years. By actuarial analysis, 57% of the patients are projected in continuous remission after five years from cessation of therapy. Neither selected features at diagnosis nor single modalities of treatment were found to predict whether relapse would occur after discontinuing therapy. Long-term remission and possibly cure can be expected in over one-third of newly diagnosed children with ALL after 2-4 years of antileukemic treatment.

Adolescent↗
PubMed1980

Multiple myeloma and acute myelomonocytic leukemia: simultaneous occurrence without previous chemotherapy.

A patient with acute myelomonocytic leukemia was found to have IgG paraprotein on serum electrophoresis Bence Jones K proteinuria and increased plasma cells (30%) on marrow examination. The simultaneous occurrence of the two diseases was well documented by cytochemical immunological and electron-microscopic findings. Bone marrow chromosome investigations showed an abnormal karyotype: hypodiploidy was prevalent and marker chromosomes were present. A possible relationship between acute leukemia and multiple myeloma is discussed.

Aged↗
PubMed1980

B cell markers in Ph1-positive acute lymphoblastic leukemia.

A case of acute lymphoblastic leukemia (ALL) where the blast cells had B cell markers and displayed the presence of a typical Ph1 chromosome, originated by a standard t (9;22) translocation, is reported. Cytological and clinical aspects during the entire course of the disease were consistent with the diagnosis of ALL. Evidence of differentiation along a well-defined lymphoid cell line in a Ph1-positive cell confirms the presence of the Ph1 chromosome in conditions other than chronic granulocytic leukemia and shows that it possibly does not occur in an exclusively undifferentiated totipotent stem cell.

Adult↗
PubMed1979

'GPI Roma', a new glucose phosphate isomerase deficient variant: in vivo occurrence of postsynthetic modifications of the mutant enzyme.

In a 5-year-old Italian girl with severe congenital hemolytic anemia, red cell GPI deficiency was proven, and found to be due to a new variant, 'GPI Roma.' The parents are first cousins and have been proven to be heterozygous for this variant. GPI Roma was slightly unstable to heat and exhibited a slightly increased Michaelis constant for fructose-6-phosphate. A single predominant fast-migrating GPI form existed in the patient's white blood cells, while the electrophoretic pattern in the red cells was composed, in addition to this 'fast band,' of a major band migrating as normal GPI and of an additional slow band. It is shown that this phenomenon may be ascribed to postsynthetic events modifying the charge of the mutant enzyme.

Anemia, Hemolytic, Congenital↗