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Biomedical subjects

J R Edson

Publications and source records attributed to J R Edson.

At least 37 records · Page 2Linked to original sources
PubMed1980

Plasminogen and antithrombin III deficiencies in the childhood nephrotic syndrome associated with plasminogenuria and antithrombinuria.

Three of four patients with the childhood nephrotic syndrome were found to have low plasma plasminogen concentrations; all four had low plasma antithrombin III concentrations. In the two patients who were tested, urinary concentrations of these proteins exceeded the plasma concentrations. As the urinary losses of plasminogen and antithrombin III decreased over the course of illness, the plasma concentrations of antithrombin III and plasminogen rose. One patient had multiple thromboembolic episodes. We conclude the deficiencies of antithrombin III and plasminogen, probably secondary to urinary excretion of these proteins, may contribute to the thrombotic diathesis associated with nephrotic syndrome.

Adolescent↗
PubMed1980

Factor V deficiency in Philadelphia-positive chronic myelogenous leukemia.

Factor V deficiency has been identified in 8 of 8 patients 7--20 yr of age, with Philadelphia-positive (Ph1+) chronic myelogenous leukemia (CML). In these 8 patients, factor V deficiency was not due to hepatic dysfunction, factor V inhibitors, or disseminated intravascular coagulation. In 3 patients, factor V activity rose 10%--12% (0.10--0.12 U/ml) after the infusion of 28--31 ml/kg body weight of fresh frozen plasma (FFP). The rise persisted less than 14 hr. The mean measured postinfusion rise in factor V was 18% of the expected rise calculated from the volume of FFP infused in the patients' plasma volume. In 4 patients, a small transient rise in factor V activity occurred after splenectomy or plateletpheresis. Factor V deficiency was completely corrected after a marked reduction in bone marrow cellularity in 2 patients with Ph1+ CML treated with extensive chemotherapy, total body irradiation, and bone marrow transplantation. Factor V deficiency was retrospectively observed in 6 of 20 patients, ages 20--80 yr, with Ph1+ CML and 3 of 6 patients with other myeloproliferative disorders. The factor V deficiency appears to be associated with the large myeloid-megakaryocytic cell mass characteristic of CML and other myeloproliferative disorders.

Adolescent↗
PubMed1978

Acute promyelocytic leukemia. Management of the coagulopathy during daunorubicin-prednisone remission induction.

Seven adults with acute promyelocytic leukemia (APL) and disseminated intravascular coagulation were treated for remission induction with daunorubicin hydrochloride and prednisone. In all patients the coagulopathy was managed with continuous-infusion heparin sodium and vigorous transfusion with platelets, cryoprecipitate, and fresh frozen plasma. Five patients survived induction; they all achieved complete remission (CR). Median duration of CR was 27 + months; two patients presently survive in their initial CR at 28 and 48 months. Recognition of APL as a distinct type of acute leukemia and prompt initiation of treatment aimed at rapid cytoreduction and control of the coagulopathy has resulted in a prolonged disease-free survival for the majority of patients.

Adolescent↗
PubMed1978

Retinal surgery complicated by a spontaneously acquired factor VIII inhibitor.

We studied a factor VIII inhibitor spontaneously occurring in an otherwise healthy patient who underwent retinal reattachment. The clotting defect first manifested itself as a delayed hemorrhagic choroidal detachment. His bleeding diathesis was successfully managed by infusion of factor VIII concentrate, prednisone, and cyclophosphamide. Surgical procedures in patients with severe bleeding disorders present a difficult therapeutic problem which can be effectively managed by the close cooperation of the surgeon, hematologist, coagulation laboratory, and blood bank.

Aged↗
PubMed1978

Spontaneously acquired factor IX inhibitor in a nonhemophiliac child.

A 2 1/2-year-old, previously healthy child developed progressive swelling of the left leg and a hematoma of the anterior chest wall associated with a falling hemoglobin concentration, as a result of a spontaneously acquired Factor IX inhibitor. Successful management of her condition required a four-volume exchange transfusion and immunosuppressive therapy consisting of cyclophosphamide for four days and prednisone for one month. A brief review of the literature regarding the occurrence and nature of acquired coagulation factor inhibitors and the role of immunosuppressive therapy is also presented.

Blood Coagulation↗
PubMed1977

The effect of L-asparaginase of plasma coagulation factors in acute lymphoblastic leukemia.

Serial coagulation studies were performed in 26 pediatric patients with acute lymphoblastic leukemia during initial induction therapy with vincristine, prednisone, and L-asparaginase. Prolongation of screening coagulation tests was frequent: prothrombin time (in 16 of 26 patients), partial thromboplastin time (23/26) and thrombin time (21/26). In all 26 patients fibrinogen levels fell below .20 g/100 ml and 16 had levels below .10 g/100 ml. Sixteen patients had plasma coagulation factor assays performed. In these 16 patients, Factor XI was less than 40% in 14 and Factor XI was less than 70% in 9, with only a few scattered low levels of other factors. There were no clinical bleeding episodes. Coagulation abnormalities returned to normal at the completion of L-asparaginase therapy while the patients remained on vincristine and prednisone.

Afibrinogenemia↗
PubMed1976

Antigenic differences among bovine and porcine Factor VIII concentrates.

Three anti-Factor-VIII antibodies from hemophiliacs were reacted with samples of batches of Maws commerical bovine and porcine Factor VIII concentrates manufactured over a 12-year period. The apparent antibody concentrations varied widely with the different batches of concentrates. The variations are probably due to intrinsic differences in the antigenic nature of the Factor VIII in the different preparations. With the older porcine concentrates, the low apparent concentrations may be in part due to the reaction of antibody with inactive Factor VIII. When animal Factor VIII concentrates are used in treating hemophiliac patients who have anti-Factor-VIII antibodies, the least reactive batch should be chosen. Random batches of animal concentrates are not suited as a standard for measuring anti-Factor-VIII antibody concentration.

Animals↗
PubMed1975

Low platelet adhesiveness and other hemostatic abnormalities in hypothyroidism.

Fifteen patients with definite hypothyroidism and two with probable hypothyroidism had extensive hemostatic profiles done. In 12 of the 16 patients tested, the platelet adhesiveness (platelet retention in a glass bead column) was abnormal, and in 1 more it was borderline. In the six patients who were studied repeatedly, hemostatic abnormalities either markedly improved or disappeared after treatment with L-thyroxine. It is concluded that, in addition to the previously reported coagulation factor deficiencies (which we also observed in some of our patients), low platelet adhesiveness occurs frequently in patients with hypothyroidism.

Adolescent↗