[Incidence and significance of disease of the paranasal sinuses in small children].
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Biomedical subjects
Publications and source records attributed to W Friedrich.
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In 15 infants with severe combined immunodeficiency (SCID), immunological reconstitution was attempted by bone marrow transplantation (BMT) from HLA-haplo-identical parents. To prevent graft versus host disease (GvHD), marrow grafts were depleted of contaminating T-lymphocytes using lectin agglutination and rosette formation with sheep red blood cells. Thirteen patients received transplants without undergoing prior cytoreductive conditioning. Eleven of these developed donor-dependent T-cell functions, two failed to do this. One of these two as well as two further patients received cytoreductive treatment prior to repeat and to first transplants and in two, complete lymphohemopoietic reconstitution was observed. Of the 15 patients who received transplants, 11 are currently alive. Two recently treated patients remain in the hospital, nine are at home with stable T-cell functions. Normal humoral immune functions have developed upto now in three patients. In the others, gamma globulins are regularly substituted. Complications of acute or chronic GvHD were not observed with the exception of one case who developed transient GvHD of the skin. These results suggest that in a majority of patients with SCID, T-cell functions can develop without GvHD following haploidentical, T-cell-depleted BMT. Exceptional patients require preconditioning to allow donor cell engraftment, an approach that also appears to facilitate reconstitution of humoral immune functions.
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The effect of hyperimmune globulin for the prevention of cytomegalovirus (CMV) infections following bone marrow transplantation (BMT) was evaluated in 21 children with various diseases and compared to a historical group of 23 children without prophylaxis. A higher incidence of interstitial pneumonia (19%) as well as CMV-associated infections with or without pneumonitis (29%) could be demonstrated in patients with CMV-prophylaxis as against the rate of interstitial pneumonia (4%) and CMV-infections (8%) in children without prophylaxis. This surprising observation was very likely due to selection of patients with high risk features and higher incidence of GVHD in the prophylaxis group. The analysis of patients with prophylaxis failure shows a low CMV-infection rate in initially seronegative marrow recipients and a high risk for CMV-infections in seropositive patients. Therefore, even though CMV-infections could not be completely abrogated, hyperimmune globulin administration might have reduced CMV-complications in seronegative patients. In CMV-seropositive marrow recipients, however, this type of prophylaxis remains unsatisfactory in preventing severe CMV-infections caused by virus reactivation.
In a prospective study in 220 children at crèche age altogether 2,443 bacteriological investigations of the urine were performed over two years. As investigation method an electrochemical technique was used. About 3% of the bacteriological investigations of the urine revealed a bacteriurine of greater than 10(5) germs/ml. More than 60% of these findings were falsely positive. The frequency of the urinary tract infections in children from the 6th month up to the 3rd year was 1.36%. The proportion of the sterile findings of the urine reveals a clear dependence on age and is essentially influenced by the taking technique. The findings were compared with the results of other screening investigations and discussed.
Bone marrow transplantation (BMT) represents the therapy of choice in severe combined immunodeficiency (SCID). Besides a short description of the clinical picture and of the diagnostical criteria of the disorder, the basis and results of BMT in SCID are presented. This treatment can result in the complete immunological reconstitution with resulting cure of affected patients. Recently, the use of other than HLA-identical marrow donors has become possible, such as haploidentical parents. Threatening complications from graft versus host disease are preventable by depleting T-cells from marrow grafts. Early results of this new approach are very encouraging, and represent significant advance in clinical BMT.
The reaction of phenylalanine with 2,4-dinitrofluorobenzene is increased by substances forming micells. As against the basic test, the increase is not dependent on conditions using solutions of 2.42 X 10(-3) mol phenylalanine and 1.62 X 10(-4) mol dinitrofluorobenzene the rate constant of the reaction proportionally increases by addition of 6.2 X 10(-4) to 4.4 X 10(-3) mol cetyltrimethyl ammonium bromide solution, then remains constant and finally decreases with further increase of the concentration. These curves could continuously be registered with varying pH values, although the basic and the catalytic reaction rate of both the reactions will increase according to the pH value with any buffer. A further increase of the basic and the catalyzed reactions can be enabled by increasing the temperature. The validity of the Arrhenius equation could be proved.
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From 1972-1983 53 patients underwent bone marrow transplantation. The median age was 18 years (3-41). 27 patients suffered from severe aplastic anaemia, 22 patients had acute leukaemia and 4 patients had chronic granulocytic leukaemia in chronic phase. Out of 22 patients with acute leukaemia, 2 had florid leukaemia, 2 had an early relapse and 18 patients were in first or second remission of their disease. 2/53 patients received a syngeneic transplant, 51/53 patients an allogeneic transplant. 47/51 patients had a HLA-A, B, C-identical, MLC-negative sibling donor, 1/51 had a HLA-A, B-C-identical, MLC-positive sibling donor, 2/51 a HLA-phaenotypical identical parental donor and 1/51 a HLA-identical, MLC-negative unrelated donor. The comparison of the results obtained in patients with severe aplastic anaemia transplanted from 1972-1979 with those transplanted from 1980-1983 shows that the bone marrow transplantation has to be performed in an early stage of the disease before the patients become multiple transfused, sensitized and severely infected and that the conditioning regimen for polytransfused patients has to be more intensive than in untransfused patients. From the patient group transplanted 1972-1979, only 1/14 patients is a long-term survivor in contrast to 8/13 patients transplanted from 1980-1983. 11/22 patients with acute leukaemia are alive between more than 5 years and 14 days after bone marrow transplantation. Only 1/4 patients, who were transplanted not in remission, is alive.(ABSTRACT TRUNCATED AT 250 WORDS)
Immunological reconstitution by transplantation of HLA-haploidentical, paternal bone marrow was attempted in four infants with severe combined immunodeficiency who lacked HLA-identical donors. To prevent graft-versus-host disease, T lymphocytes were removed from the grafts by agglutination with soybean agglutinin and rosette formation with sheep red blood cells. None of the patients received conditioning treatment before transplantation. Normal, donor-derived cellular immune functions developed in all four patients within several months of transplantation. Threatening complications of graft-versus-host reactions were not seen. All four patients remain in excellent health 12-15 months after discharge home, with persisting normal T-cell functions.
Arsenate-resistant (AsR) clones were obtained with high frequency from colony populations of streptothricin-producing strains of Streptomyces noursei by the paper strip method. Whereas in the AsR-strains obtained from both wild type and mutant NG 13 the antibiotic production was reduced to approx. 60% of the parental level, the AsR clones isolated from colony populations of mutant UV 12 displayed increased productivity (less than or equal to 150%). However, their improved capacity to produce streptothricins was lost rapidly after repeated cell propagation in submerged cultures, suggesting that unstable genetic elements were involved in enabling S. noursei to grow in the presence of toxic concentrations of arsenate.
Mycelial levels of ATP and glucose-6-phosphate were investigated in mutants of streptothricin-producing S. noursei JA 3880b differing from the wild-type strain in antibiotic formation, in the control by inorganic phosphate of the secondary metabolism, and in the resistance to growth inhibition by toxic arsenate ions. As compared with the ancestral strain, mutants exhibited a lower content of ATP in the mycelium while addition of 0.1 M arsenate to growing cultures provoked only moderate changes in the level of this high-energy metabolite. The results suggest that there exists a correlation between growth resistance to arsenate and insensitivity to phosphate inhibition of the secondary metabolism, on the one hand, and the capacity to produce streptothricin-type antibiotics, on the other.
In a program of in vitro fertilization, laparoscopies for oocyte aspiration were performed on 35 patients receiving human pituitary gonadotropin (hPG) and human chorionic gonadotropin (hCG). Of the 122 preovulatory oocytes which were recovered from these patients, 44 (36%) were fertilized and cleaved, and were transferred. 144 immature oocytes were collected, and attempts were made to mature these in vitro. 40 oocytes (28%) could be fertilized and cleaved, and were transferred. Eight pregnancies resulted from 32 embryo transfers (25%), and 22,9% from laparoscopies, respectively. Of the eight pregnancies, there were three preclinical abortions and two clinical abortions, and three patients are well along in the pregnancy. Among the three patients there is one twin pregnancy.
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