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Biomedical subjects

H Ekert

Publications and source records attributed to H Ekert.

At least 91 records · Page 5Linked to original sources
PubMed1979

A randomized study of factor VIII or prothrombin complex concentrate infusions in children with haemophilia and antibodies to factor VIII.

In four children with haemophilia A and antibodies to factor VIII, 18 bleeding episodes were randomized for treatment with factor VIII concentrate (30 units/kg) and 18 for treatment with a prothrombin-complex concentrate (prothrombinex) given in a dose of 30 units of factor IX/kg. Treatment with prothrombinex was associated with a better clinical response, a significantly greater shortening of the kaolin partial thromboplastin time and significantly lower incidence of post-infusion increase of levels of factor VIII antibodies. Although treatment with factor VIII concentrate was clinically successful in 15 episodes, treatment failures occurred in three instances leading to parental request for withdrawal from study in two families.

Antibodies↗
PubMed1978

Controlled study of hypertransfusion during remission induction in childhood acute lymphocytic leukaemia.

In a prospective, controlled trial 26 anaemic, neutropenic children with newly diagnosed acute lymphocytic leukaemia were randomised in pairs to receive either transfusion to a haemoglobin of 10--12 g/dl where clinically indicated (group A) or hypertransfusion to a haemoglobin of 16--18 g/dl (group B). Compared with group A (11 of 13 transfused), group B (all transfused) had a significantly more rapid rise in neutrophils at 7 and 10 days post-transfusion, a lower incidence of infection, and less interruption to chemotherapy. Hypertransfusion restored the myeloid/erythroid ratio to normal in bone-marrow of 5 of 6 children and the proportion of early myeloid precursors was greater than in controls.

Adolescent↗
PubMed1978

Cytokinetic studies in children with untreated acute lymphocytic leukaemia (ALL): relationship to "T" cell markers.

"The labelling index (LI), mitotic index (MI) of marrow lymphoblasts and the percent of peripheral blood lymphocytes and lymphoblasts that form rosettes with sheep red cells at 4 degrees C ("T4"), were measured at presentation in 33 children with ALL. There was a significant correlation between LI and MI, but no significant correlations between them and the total white cell count at diagnosis or the "T4" percent. Three patients had greater than 55% "T4" rosettes and showed increased LI and MI, and 2 have relapsed; 12 had less than 20% "T4" rosettes, showed an increased LI (LI greater than 8% in 8 of 12) but not MI, and 5 have relapsed; 18 had 20--55% "T4" rosettes and generally had the lowest LI (LI less than 8% in 11 of 18), and only 3 have relapsed. Our findings suggest that patients with high and low percentage of "T4" rosettes in the peripheral blood have an increased fraction of leukaemic cells in DNA synthesis and have a diminished chance of prolonged remission.

Child↗
PubMed1978

Treatment of childhood lymphocytic leukaemia with high white-cell counts.

Combination chemotherapy with cytosine arabinoside, cyclophosphamide and L-asparaginase (Asnase) was given to 22 children with acute lymphocytic leukaemia (ALL) with a white-cell count greater than 30 X 10(9)/1, and other features suggestive of poor prognosis. Complete remission was induced in all patients--in 19 after 2 courses of chemotherapy and in the remainder after a third course. During induction, neutropenia occurred in 18 and severe infection in 3. Anaphylaxis to Asnase occurred in 8 patients after the second course and one other had transient Asnase-induced diabetes. All patients received central-nervous-system prophylaxis after achieving remission, during which they were also treated with weekly vincristine and a 2-week course of prednisolone. Continuation therapy consisted of short cycles of intermittent chemotherapy and BCG inoculation or long cycles of intermittent chemotherapy +/- BCG. Life-table analysis shows 46% complete remission rate at 28 months, with 6 patients all in complete remission followed up between 28 and 41 months. There were minimal complications of continuation therapy, and BCG inoculation was well tolerated.

Adolescent↗
PubMed1978

Hematologic emergencies in children.

Hematologic emergencies may be defined as sudden or unexpected life-threatening events in clinical hematology and oncology which require immediate action predominantly based on clinical judgements and supported only by investigations that can be expected to produce results rapidly. It is convenient to classify these emergencies according to disorders affecting the erythrocytes; leukocytes; platelets; hemostasis; defence mechanisms against infection; the respiratory system; and emergencies related to drugs used for the treatment of neoplasia. A proposed classification is outlined. Part of treatment of hematologic emergencies is to ensure that the cicumstances which caused them will not recur in the same patient or those with similar disorders.

Acute Disease↗
PubMed1977

Intermittent chemotherapy and BCG in continuation therapy of children with acute lymphocytic leukemia.

Continuation therapy using intermittent chemotherapy and BCG inoculation was commenced in 28 children with acute lymphocytic leukemia (ALL) immediately after remission induction and "CNS prophylaxis." At a median followup time of 17 months, 71% remain in total remission and 86% in bone marrow remission. Complications of the therapy were minimal. Major infections occurred on two occasions and there were no deaths in remission. Neutropenia, "minor" infections and postponement of chemotherapy occurred most often during the first three courses of treatment. There were no local or systemic BCG infections. Tuberculin sensitivity was tested in 25 patients. It was positive in 17 of 18 patients in total remission and all four patients with only CNS relapse, and was negative prior to relapse in three patients who developed bone marrow disease.

BCG Vaccine↗
PubMed1977

Changes in electrophoretic mobility of human factor VIII-related antigen: evidence for subunit structure.

Therapeutic factor-VIII concentrates were found to have factor VIII-related antigen (FVIIIRAg) with an electrophoretic mobility faster than that of plasma using two-dimensional crossed immunoelectrophoresis. Immediately after infusion of factor-VIII concentrates into patients, the electrophoretic mobility of FVIIIRAg in the patients' plasmas was increased to that of the antigen in the infused concentrates. Two hours after infusion, a proportion of the antigen had an electrophoretic mobility intermediate between that of the pre-infusion antigen and that of the concentrate antigen, and by 24 h after infusion the reversion of electrophoretic mobility to pre-infusion values was complete. The return of electrophoretic mobility to normal did not occur in vitro after 24 h. In vitro mixing experiments between pre-infusion plasma and concentrate resulted in antigen with a range of intermediate mobilities which were related to the relative proportions of slow and fast antigen in the mixture. In vitro mixing experiments with slow and fast antigen separated from intermediate purity factor VIII concentrate by agarose gel filtration resulted in the formation of a relatively large proportion of antigen with intermediate electrophoretic mobility. The most reasonable interpretation of the results is the formation of hybrids between the two electrophoretically different populations of antigen. This implies that VIIIRAg normally exists in a polymeric form which can spontaneously dissociate into the exist in equilibrium with a pool of partially and/or completely dissociated subunits.

Antigens↗
PubMed1977

The use of non-activated prothrombin concentrate in the management of haemophilia A with factor VIII antibodies.

Three children with haemophilia and antibodies to factor VIII were treated with a non-activated prothrombin concentrate (Prothrombinex) for 12 bleeding episodes. There was clear clinical response and joint aspirations were performed after infusions of phothrombinex in a dose of 30--50 factor IX units/kg body weight and there was no clinical evidence of thrombosis or febrile reactions. There was a significant shortening of the activated partial thromboplastin time (PTT) at one and four hours after the initial infusion with a return to pre-infusion levels 9--24 hours after infusion. The shortening in the PTT was less marked in subsequent infusions. There were no changes in the level of factor VIII procoagulant activity, factor VIII related antigen or factor VIII antibodies after the infusion. In two patients platelet function studies were unaltered by the infusion and in one patient procoagulant levels of factor II, IX and X were no greater than expected from the infusion. We conclude that infusions of non-activated prothrombin concentrates are clinically effective in the treatment of children with haemophilia and factor VIII antibodies but the mechanism of action is unknown.

Adolescent↗
PubMed1976

Studies on the altered electrophoretic type of the factor VIII related antigen.

A distinct sub-group of von Willebrand's disease is characterized by an electrophoretically faster mobility of the factor VIII related antigen. Some of the physico-chemical properties of this variant antigen were investigated in this communication. The effect of temperature was tested by heating aliquots (0.5 ml) for 20 minutes at 45 degrees C, 56 degrees C and 65 degrees C. The variant was found to be denatured at 56 degrees C while the control was denatured at 65 degrees C. The effect of pH was tested by assessing the quantity (Laurell technique) and electrophoretic mobility (two dimensional immunoelectrophoresis) of the antigen in a variety of buffers ranging in pH from 7.0 to 9.5. The quantity of antigen was variable both among variants and controls and the electrophoretic mobility of the variant antigen was faster at all pH's. Molecular weight differences between the variant and controls were not detected since the chromatographic profile of the variant was similar to that of the controls in Sepharose 6 B using a 0.02 M Tris-NaCL buffer at pH 7.0. The affinity of the antigen for human antibody was heterogeneous although the variant exhibited less affinity for one of the human antibodies but not the other. The inhibitory effect was more pronounced in serum than in plasma. Purified IGG, however, did not show any inhibition, as the residual antigen assayed by the Laurell technique, was similar to the expected values. This would imply that non-IgG plasmatic factors could also play a part in the observed inhibition.

Antigen-Antibody Reactions↗