Biomedical subjects
Publications and source records attributed to M Rister.
Wegener's granulomatosis (WG) is a rare disease in pediatric patients. The case of a 14-year-old girl with generalized WG is described and the pediatric literature reviewed. Thirty additional cases of WG in childhood have been traced in previous studies. Diagnostic problems are discussed in the context of clinical, radiological and biopsy findings.
The usefullness of lithium carbonate for attenuation of leukopenia due to chemotherapy was studied in a prospective randomized trial. Chemotherapy for solid tumors was applied in a paired manner 24 times without and 24 times with lithiumcarbonate. Simultaneously, nucleotid-dependent functions of granulocytes were examined. An attenuation of leukopenia by lithium was observed. Toxic side effects within the therapeutic range of the serum level (0.3-1.4 mol/l) were not detected. Lithium did not impair adherence, chemotaxis and degranulation of neutrophile granulocytes. The influences of lithium on stem-cell maturation and granulocyte function are discussed.
The Torsten -Sjögren-syndrome is the combination of hereditary cataract associated with impaired central nervous coordination, mental retardation and hyperaminoacidurea . This recessive inheritable disease is due to an alteration of the aminoacidmetabolism also present in the not manifestly affected parents. In addition, the presented case exhibited an anal atresia with perinatal fistula. The pediatric and ophthalmologic aids have been started early. According to the etiology a genetic counceling is necessary.
Two hundred thirty cases of malignant non-Hodgkin's lymphomas were reclassified in a retrospective study according to the New Working Formulation for Clinical Usage of the NCI as compared to the Rappaport and Kiel classifications. The reproducibility for the individual schemes this study was 81% (Rappaport), 79% (Kiel), and 85% (New Working Formulation). In keeping with the results of the NCI international study, all lymphomas were subdivided into 3 prognostic groups: (1) low-grade malignancy (6.0 years median survival); (2) intermediate-grade malignancy (3.5 years median survival); and (3) high-grade malignancy (1.4 years median survival). The NCI-proposed New Working Formulation for Clinical Usage is thus recommended as practical and unprejudicing classification scheme for general application; however, its usefulness as tool for translating one classification scheme into another appears limited.
Intensive chemo- and radiotherapy in many cases leads to cure of malignant diseases and thus to an increasing number of former childhood and adult cases of cancer who now wish to start a family. A survey among 58 oncologic-paediatric and 26 obstetric-gynaecologic centres and a review of the literature was meant to demonstrate the effects of cytostatic treatment on the issue of treated persons. A total of 627 children of 537 preconceptionally treated patients could be assessed. 59 (9.4%) of these showed abnormalities. This corresponds to the frequency in a normal population. On the basis of these results effects of intensive tumour treatment on subsequent deliveries cannot be demonstrated. Thus, too pessimistic an attitude towards conception after treatment for cancer does at present not appear justified from a medical viewpoint.
Hyperoxia impairs cytoskeleton-dependent phagocytic functions in polymorphonuclear leukocytes (PMNs) and alveolar macrophages (AMs). To investigate the effect of different oxygen concentrations on the cytoskeleton, in particular the microtubule (MT) and microfilament (MF) system, the fluorescence pattern of Concanavalin A (Con A) receptors in AMs and PMNs was observed. Cells were obtained from guinea-pigs exposed to different oxygen concentrations. The exposure of guinea-pigs to oxygen concentrations of up to 50% induced in AMs and PMNs mainly spontaneous Con A capping, demonstrating an altered MT system. Oxygen tensions above 50% lead to an increased number of AMs and PMNs exhibiting a patchy Con A fluorescence distribution. Even in the presence of the microtubule-disrupting agent colchicine most AMs and PMNs were unable to form a Con A cap fluorescence distribution under these high oxygen tensions. This study demonstrates that the exposure of guinea-pigs to an oxygen concentration greater than 50% increases the relative number of AMs and PMNs demonstrating a patchy distribution of Con A. This patchy fluorescence pattern is associated with a severe cytoskeletal defect, i.e. MT and MF disruption. The various leukocyte function defects induced by hyperoxia, demonstrated in previous studies, are based on this MT and MF alteration in AMs and PMNs, representing an additional sepsis-promoting factor during hyperoxia.
The exposure of guinea pigs to 85% oxygen increased in alveolar macrophages and granulocytes the Superoxid Dismutase activity. This enzyme protects aerobic organisms against the toxic superoxide anion. In contrast both cell types exhibited a decrease in hydrogen peroxide metabolizing enzymes Catalase and Glutathione perioxidase. In addition, the animal exposure to FiO2 of 85% impaired various phagocytic functions. A decrease of adherence, chemotaxis, ingestion rates as well as the bacterialcidal activity was observed in alveolar macrophages and granulocytes. In alveolar macrophages the degranulation was also diminished. These phagocytic function defects are caused by a cytoskeleton alteration, consistent of the microtubulus and microfilaments. The guinea pig exposure to a FiO2 of 85% did enhance the number of alveolar macrophages and granulocytes demonstrating a patchy FITC-Concannavalin A fluorescence, which is associated with a disruption of the microtubulus and the microfilaments. The phagocytic defects described in alveolar macrophages and granulocytes obtained from guinea pigs exposed to a FiO2 of 85% are based upon a microtubulus and microfilament disturbance in both cell types.
Polymorphonuclear leukocytes (PMNs) release superoxide anion (O-2) when they are exposed to a phagocytic stimulus. Intracellularly the copper-containing enzyme superoxide dismutase (SOD) protects against the toxic effects of O-2. To investigate the role of O-2 and SOD in the inflammatory process we determined the O-2 release and SOD content in PMNs. In PMNs of children with rheumatoid arthritis the SOD activity was diminished compared to healthy controls. Upon stimulation with opsonized zymosan PMNs obtained from children with rheumatoid arthritis generated greater amounts of superoxide anion than control cells. The "SOD deficiency" in PMNs of children with rheumatoid arthritis may promote this extreme release of the toxic superoxide radical inducing the damage of the connective tissue. The involvement of superoxide dismutase and superoxide anion in inflammatory process may induce further studies, leading hopefully to an appropriate understanding or even to new principles in the treatment of the rheumatoid arthritis.
The development of bacterial infections is a common complication during treatment with high concentrations of oxygen. To study the effect of hyperoxia on phagocytes, the adherence, chemotaxis, ingestion rates, degranulation as well as the bactericidal activity were measured in alveolar macrophages (AMs) and polymorphonuclear leukocytes (PMNs) obtained from guinea pigs exposed to 85% oxygen. The animal exposure to a Fi O2 of 85% impaired the adherence to nylon-wool, the chemotactic activity and the phagocytic rate of paraffinoil-droplets of AMs and PMNs. In AMs the secretion of beta-glucuronidase upon stimulation with opsonized zymosan was also diminished. In addition, the bacterial activity of AMs and PMNs demonstrated a reduction of 50%. These phagocytic defects may be caused by cytoskeleton alteration, induced by the increase of oxygen derived metabolites, representing an additional sepsis promoting factor during hyperoxia.
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Granulocyte adherence to endothelial surfaces associated with their chemotactic property enables these cells to leave the peripheral blood and to migrate into the tissue. This study was performed to investigate the effect of bacterial and viral infections as well as various kinds of therapies on these leukocyte functions. The adherence of control granulocytes to nylon fibers was 70%. In contrast to viral infections bacterial infections increased the adherence to 91%. Following the treatment with high dose methotrexate or vincristine the adherence was reduced to 20%. This granulocyte function defect was evident up to 14 days after the high dose methotrexate infusion. An age dependency of granulocyte adherence was not observed. In addition, viral infections as well as cytostatic therapy did not effect granulozyte chemotaxis. But bacterial infections and various defined phagocytic defects impaired the granulocyte chemotactic activity.
Since the age of nine weeks a red haired girl suffered from purulent dermatitis and recurrent, systemic E. coli infections. She had an excessive hyperimmunoglobulinemia E, as well as impaired granulocyte adherence and chemotaxis. Though a sepsis was evident, the granulocytes exhibited a random FITC-Concanavalin A fluorescence. In spite of intensive treatment with various antibiotics and several granulocyte transfusions the child died at the age of 2 years and 11 months. As shown by the FITC-Concanavalin A distribution, the hyperimmunoglobulinemia E may have caused a decreased membrane fluidity causing the impaired adherence and chemotaxis. This could explain the pathophysiology of the Job's Syndrome.
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A combined treatment with surgery, radiotherapy and chemotherapy is necessary for children with adenomyosarcomas. In order to reduce the side effects of the treatment, it seems reasonable to establish a therapy plan taking into consideration the risk of recurrences. Besides the classification of stages according to the N.W.T.S., the following factors should be considered: histologic findings, manifestations of the lymph nodes, number of tumor nodes, size of the adenomyosarcoma, and a rupture of the adenomyosarcoma. A postoperative polychemotherapy is sufficient for children of stage I presenting favorable histological findings. For the stages II and III, an irradiation of the tumor and the lymph outflow regions or the total abdomen with 24 Gy is recommendable in addition to chemotherapy. For stage IV, an irradiation of the lung up to 12 Gy in addition to chemotherapy has proved to be useful. A pre-operative radio- or chemotherapy is indicated especially in case of a great tumor volume and stage III and IV.
Chronic granulomatous disease is caused by an X chromosome-linked granulocyte function defect whereby the superoxide radical (O2-) and hydrogen peroxide are not formed during phagocytosis. In the course of the investigation of the family of a boy with chronic granulomatosis disease carriers could not be established using the NBT test. Only determination of O2- formation by granulocytes during phagocytosis led to identification of disease carriers. This may possibly represent a means for prenatal diagnosis.
Various polymorphonuclear leukocyte (PMN) functions are dependent on an intact intracellular cytoskeleton consisting of the microtubules and the microfilaments. To investigate the microtublule system in PMNs we observed the spontaneous, Colchicine and Diamide induced cap-formation by fluorescence microscopy ion PMNs obtained from children with bacterial and viral infections demonstrated with 47 +/- 1% a significantly increased number of spontaneous capped PMNs compared to 22 +/- 1% capped cells obtained from controls. Furthermore, 52 +/- 2% PMNs of patients on immunosuppressive therapy exhibited spontaneous surface capping. There was no significant elevation in the number of capped PMNs (30 +/- 2%) obtained from children with viral infections. Colchicine and Diamide increased the number of capped cells in control PMNs as well as in PMNs from patients to 69 +/- 1% and 67 +/- 1%, respectively. Since the increased spontaneous cap formation in PMNs is associated with a defect of microtubule assembly, the various leukocyte function defects described in patients with bacterial infections, bronchial asthma or on immunosuppressive therapy may have to be considered the consequence of an altered microtubule system.
Alveolar macrophages and granulocytes protect the organism against invaded microorganisms under various conditions. To investigate whether the environmental difference may influence the various phagocytic functions the adherence, chemotaxis, ingestion rate, degranulation as well as the bactericidal activity were determined in alveolar macrophages and granulocytes obtained from guinea pigs. In addition, the cytoskeleton was examined by observing the fluorescence of Concanavalin A receptors. Various functional differences between both cell types have been demonstrated. Both cell types exhibited the same adherence. In contrast, granulocytes demonstrated a greater chemotactic activity and ingestion rate than alveolar macrophages. Following the exposure to opsonized zymosan alveolar macrogphages secreted almost totally the lysosomal enzyme beta-glucuronidase during the degranulation. Nevertheless, alveolar macrophages exhibited a lower bactericidal activity than granulocytes. This functional differences were not caused by an alteration of the cytoskeleton. A random fluorescence distribution of FITC-Concanavalin A receptors was observed in 72% alveolar macrophages and 73% granulocytes, demonstrating polymerisation of the microtubuli. Only 12% alveolar macrophages and 11% granulocytes showed a capped FITC-Concanavalin A fluorescence which is associated with an alteration of the microtubulin. This study demonstrates that the difference in adherence, chemotaxis, ingestion rate, as well as the bactericidal activity of alveolar macrophages and granulocytes is a specific cell-type property.
The Steinbrinck-Chediak-Higashi Syndrome is characterized by morphological and functional alterations of polymorphonuclear leukocytes. The impaired function in the polymorphonuclear leukocytes may be related to abnormal microtubular assembly. The ingestion of 200 mg/d of ascorbic acid potentiated chemotaxis and bactericidal activity of polymorphonuclear leukocytes from a patient with this disease. Furthermore, the treatment with ascorbic acid reduced the spontaneous cap formation by these leukocytes, demonstrating the improvement of microtubular assembly. The life long treatment with ascorbic acid may affect the clinical course of these patients favourably.