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Biomedical subjects

C S August

Publications and source records attributed to C S August.

At least 73 records · Page 4Linked to original sources
PubMed1977

A hyperimmunoglobulin E syndrome with normal chemotaxis in vitro and defective leukotaxis in vivo.

A 26-yr-old male with a lifelong history of atopic dermatitis and recurrent severe staphylococcal abscesses was found to have hyperimmunoglobulinemia E. Evaluation of both the humoral and cellular aspects of chemotaxis in vitro showed both neutrophils and monocytes to be normal. However, quantitative neutrophil migration in vivo was significantly suppressed using the patient's own serum as the attractant. This defective migration in vivo was partially corrected by serum from normal donors as the attractant and also partially corrected following plasma infusion in this patient. Evaluation of quantitative leukocyte migration in vivo may be most useful in patients suspected of defects of leukocyte mobility.

Abscess↗
PubMed1977

Psychological issues in bone marrow transplantation.

We studied the psychological and emotional problems experienced by seven children and their families who underwent bone marrow transplantation at the University of Colorado Medical Center from 1973 to 1975. These problems included (1) anxiety and depression relating to isolation, fear of death, and painful procedures; (2) an overdependence associated with a feeling of helplessness; (3) anger directed toward both the staff and the parents; (4) a reduced tolerance for medical procedures; and (5) periodic refusal to cooperate. Initially we had been concerned that patients might become agitated, psychotic, or even suicidal. These did not occur. Severe anxiety over bodily changes was not a problem. We did not encounter prolonged refusal to cooperate, refusal to remain in isolation, or drug addiction. Important aspects in management included an honest, straightforward, and direct discussion of all aspects of transplantation, including the potential complications and the risks of death from the underlying disease or from complications of transplantation. A firm but understanding approach to the patients appeared to be the most effective method to develop their continuing cooperation. The opportunity for patients to express verbally their fears of procedures and of death was essential. The donors needed help in working through their feelings of guilt if a transplant was not successful. The parents needed continuing psychological support for the many personal, social, and psychological difficulties which they had to face.

Adolescent↗
PubMed1976

Clinical and physiologic studies of two siblings with prekallikrein (Fletcher factor) deficiency.

Two siblings with hereditary Fletcher factor (prekallikrein) deficiency were studied for alterations of fibrinolysis, platelet function, skin inflammatory responses, permeability factor (PF/dil) formation and leukocyte chemotaxis. In vivo stimulation of fibrinolytic activity was normal; the bleeding time and platelet functions (adhesivity, aggregation, release reaction) were also normal. Both immediate (wheal-flare reaction to histamine, bradykinin, prostaglandin E1, physical agents) and delayed sensitivity skin test reactions were within normal limits. Migration of subjects' leukocytes to attractants in skin windows and in Boyden-type chambers was the same as that of control leukocytes. Serum complement components were essentially normal. One subject's leukocytes showed normal tissue factor production on stimulation by endotoxin, although prekallikrein deficiency did impair the endotoxin-stimulated generation of serum procoagulant activity. PF/dil caused increased vessel permeability in human skin; in vitro generation of PF/dil required both the Hageman factor and prekallikrein. The Fletcher factor-deficient subjects responded in a normal manner to PF/dil. Based on the Fletcher factor-coagulation assay, the biologic half-disappearance time of prekallikrein (after the transfusion of normal plasma in one of the subjects) was estimated at 35 hours. Therefore, these studies suggest that severe prekallikrein (Fletcher factor) deficiency in man is not associated with any clinically significant impairment in hemostasis, fibrinolysis, inflammatory responses or leukocyte function.

Adolescent↗
PubMed1976

Establishment of erythropoiesis following bone marrow transplantation in a patient with congenital hypoplastic anemia (Diamond-Blackfan syndrome).

Marrow transplantation was attempted in a 13-yr-old boy with congenital hypoplastic anemia who had never responded to corticosteroid therapy. Prior to the transplant, he had received 238 transfusions, at least 12 of which were from his father. He was prepared for grafting with antilymphocyte globulin, procarbazine, and total body irradiation (1000 rads). The patient, whose red cells were Group B, then received marrow cells from his Group O, histocompatible, sister. Thereafter, reticulocytes, Group O erythrocytes, and female leukocytes appeared in the peripheral blood. Erythroid precursors were seen in the patient's marrow for the first time in his life, and all lacked fluorescent Y chromosomes. Dividing cells were all female. After initially progressing well, the patient developed interstitial pneumonia and died 55 days after the transplant. The successful erythroid graft suggested that this patient's failure to produce red blood cells was due to a defective stem cell rather than to a humoral defect, plasma inhibitor, or abnormal marrow microenvironment. It suggested further that sibling marrow may be engrafted in patients who have received multiple transfusions, even from a parent.

Adolescent↗
PubMed1975

Missing Y chromosome in juvenile chronic myelogenous leukemia.

A child with Ph1-negative juvenile chronic myelogenous leukemia (CML) is presented. The only chromosomal abnormality in hematopoietic tissues consisted of an absent Y chromosome. While a missing Y chromosome in adult patients with CML may be associated with a better prognosis, the clinical course in our patient was as malignant as that usually observed in other children with Ph1-negative juvenile CML.

Aneuploidy↗
PubMed1975

Preliminary report: treatment of the hemolytic-uremic syndrome with aspirin and dipyridamole.

Three children with the hemolytic-uremic syndrome were treated with heparin, aspirin, and dipyridamole. Two of the children had remained profoundly thrombocytopenic in spite of platelet transfusion and heparin therapy. All three patients responded with prompt elevation of their platelet counts and apparent termination of the pathologic consumption of platelets. Our experience suggests not only that primary platelet consumption may play a critical role in the pathogenesis of the HUS, but also that such patients may benefit from therapy with drugs which inhibit platelet function.

Acute Disease↗
PubMed1973

Human chorionic gonadotropin: its possible role in maternal lymphocyte suppression.

Human chorionic gonadotropin completely inhibits the response of lymphocytes to phytohemagglutinin. The effect is both reversible and noncytotoxic. These observations support the theory that the fetus is accepted because human chorionic gonadotropin represents trophoblastic surface antigen and blocks the action of maternal lymphocytes.

Chorionic Gonadotropin↗
PubMed1972

The Wiskott-Aldrich syndrome. Results of transfer factor therapy.

12 patients with Wiskott-Aldrich syndrome were treated with therapeutic doses of transfer factor in an attempt to induce cellular immunity. Clinical improvement was noted after transfer factor therapy in 7 of the 12 patients treated. Because this disease has a variable course and temporary spontaneous improvement can occur, the observed improvement cannot necessarily be attributed to the transfer factor. However, in two patients repeated remissions consistently followed transfer factor administration on repeated occasions. This included freedom from infections, regression of splenomegaly, and clearing of eczema. An unexpected finding was a decrease in bleeding in 3 of the 10 patients who had bleeding. Conversion of skin reactivity was obtained in all seven patients who clinically seemed to respond to transfer factor. In vitro studies performed after the administration of transfer factor demonstrated that the lymphocytes of the patients now produced migration inhibitory factor in response to appropriate test antigens, but did not undergo increased radioactive thymidine incorporation in response to the same antigens. A defect in the monocyte IgG receptors has been found in certain patients with the disease, and the current study shows that all patients with defective monocyte IgG receptors responded to transfer factor, whereas only one patient with normal receptors showed any response. This test may thus prove to be useful in predicting the results of transfer factor therapy in patients with Wiskott-Aldrich syndrome, although evaluation of a larger series of patients will be necessary to confirm this point. We conclude that cellular immunity can be induced, that there appears to be clinical benefit in certain patients with Wiskott-Aldrich syndrome by the use of transfer factor, and that this mode of therapy warrents trial in these patients and others with defects of cellular immunity.

Cell Migration Inhibition↗