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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Pediatric allergy and immunology 3 (1992), S. 0 
    ISSN: 1399-3038
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Chronic granulomatous disease (CGD) is a syndrome, the unifying characteristics of which arc a predisposition to recurrent bacterial and fungal infections, an impaired ability of phagocytes to kill microorganisms and the failure of these cells to produce microbicidal oxygen metabolites. Four genetically different molecular defects have been found to underlie the x-linked and autosomal recessive forms of CGD. Since the relevant normal genes are cloned, molecular analysis of the genetic lesions in CGD is rapidly progressing. Diagnosis of carriers is possible in most instances and prenatal diagnosis by trophoblast biopsy or at least cordoccntcsis is now feasible. Until recently, therapy has been limited to aggressive antimicrobial prophylaxis and in very selected cases bone marrow transplantation. A new development is the introduction of recombinant human interferon-λ as infection prophylaxis. Finally, our current knowledge of the genetic defects in CGD may allow the development of somatic gene therapy directed at monocytes, or more permanently at bone marrow or peripheral blood stem cells, and promises definitive cure of this still life-threatening disease.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1399-3038
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Four different types of chronic granulomatous disease (CGD) were analysed with the monoclonal antibody, 7D5, directed against the small 23 kD subunit of cytochrome b558 using a flow cytometric fluorescence analytical method. 7D5 immunofluorescent surface staining of granulocytes was absent in 12 patients with X-linked cytochrome b558 deficiency, in 2 patients with variant X-linked CGD with residual (X-forming activity and in 2 patients with autosomal recessive cytochrome b558 deficiency. The mothers of the patients with the X-linked form of CGD had 2 cell populations, one 7D5 negative or weakly positive and one 7D5 positive. The granulocytes of both parents of one patient with autosomal recessive cytochrome b558 deficiency had slightly reduced fluorescence intensity comparable to their reduced cytochrome b558 content. Three CGD patients with normal cytochrome b558 and their parents had granulocytes normally stained with antibody 7D5. 7D5 antibody enables rapid detection and classification of CGD patients with cytochrome b558 deficiency as well as rapid identification of heterozygous carriers.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of clinical periodontology 10 (1983), S. 0 
    ISSN: 1600-051X
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract Recently we had the opportunity 10 examine and follow up over a period of 2 years an unusual case of Papillon-Lefèvre syndrome (PLS). A 10 year old boy exhibited all symptoms typical of PLS except periodontitis and premature loss of deciduous teeth. The present report aimed at studying the functional capacity of his neutiophilic granulocytes. It integrates clinical observations, histopathological findings and results of in vitro tests, The bioptic material examined included one gingival biopsy, extracted teeth, suppurative material discharged from periodontal pockets, pus emanating from a mucosal abscess, and peripheral blood leucocytes. The neutrophils were sampled on two separate occasions in two independent laboratories and tested for a variety of functions, i.e. motility, random and directional locomotion (chemotaxis), phagocytosis, membrane potential depolarization, oxygen consumption, NBT reduction, and intracellular killing of bacteria and fungi. Findings and test data indicated that in this case of PLS, neutrophilic granulocytes behaved normally with respect to all these functions including margination in blood vessels, emigration, phagocytosis of a broad range of bacteria, degranulation of lysosomes, and intracellular destruction. The data imply that factors other than neutrophil defects may be responsible for rapidly destructive periodontitis in cases where PLS is not associated with an increased susceptibility to infection.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 145 (1997), S. 98-103 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Bakterien ; Viren ; Immunabwehr ; Anpassung ; Key words Bacteria ; Viruses ; Immune system ; Adaptation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Our topic is the old conflict, “immune system versus microbes”, but this time from the microbes' point of view. In order to multiply, microbes need food and a place where they feel at home, a metabolic niche. In the background, danger is already lurking: a defence force is after their lives. They have to outwit it. As a rule, it is advantageous for the microbes not to kill their victim immediately, but to come to an arrangement with him to let him live a little longer, or even to share the greater part of their lives with him, at least until their reproductive cycle is completed and a new victim has been found. The microbes that slip through our immune defences today have been selected as pathogens in the course of evolution, in the arms race. In contrast to our normal flora, all pathogens, without exception, get the better of the immune system by means of tricks and deception. From the great treasure chest of our virulence factors, some of the most successful survival strategies are presented. The assumption that in normal individuals pathogens and defence are in a state of equilibrium is an illusion. Even in immune-competent individuals, all pathogens slip through the immune defences. Some get in through the back door in disguise. Capsules, biofilms, antigen drift and antigen shift are all successful deception manoeuvres. Or they spit poison and bile to paralyse the gatekeepers. Chemical blockages of the intracellular killing, antigen processing and antigen presentation are the tricks. Luckily for us, it is usually not in the parasite's interests to kill its host. If the parasite is not willing to share most of its life with its host, we doctors must intervene and shut the gates by force.
    Notes: Zusammenfassung Die Vermutung, Infekterreger und Abwehr befänden sich bei normalen Individuen in sicherem Gleichgewicht, ist eine Illusion. Auch beim Immunkompetenten unterlaufen alle Infekterreger die Immunabwehr. Zum Teil kommen sie „verkleidet durch die Hintertür“: Kapsel, Biofilm, Antigen-Drift und -Shift sind wirksame Täuschungsmanöver. Oder sie „speien Gift und Galle“, um die Torhüter zu lähmen; d. h. sie rufen eine chemische Blockade der intrazellulären Abtötung, der Antigenverarbeitung und der Antigenpräsentation hervor. Zu unserem Glück ist es für einen Parasiten meist nicht von Vorteil, seinen Wirt zu töten. Bei Parasiten jedoch, die letzlich zum Tod des Wirts führen, müssen wir Ärzte eingreifen und die Infektion verhindern bzw. den Parasiten bekämpfen.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Human genetics 〈Berlin〉 64 (1983), S. 207-215 
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Chronic granulomatous disease (CGD) is a clinical syndrome, the unifying characteristics of which are a severe predisposition to bacterial and fungal infections, an impaired ability of phagocytic leukocytes to kill certain microorganisms and the failure of these cells to produce microbicidal oxygen metabolites. In CGD the causal biochemical defect and the mechanism of genetic transmission vary from family to family. At least six different molecular defects have been found to underly the X-linked and at least three other the autosomal recessive form of CGD. Diagnosis of carriers is possible in most instances, and prenatal diagnosis by fetoscopic placental vessel puncture has become feasible.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Group B streptococci: types Ia, Ib, II, III ; IgG antibody levels ; Premature infants ; Early onset septicemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sera from 33 newborn infants with gestational ages ranging from 27 to 41 weeks were tested by radioimmunoassay for IgG antibodies to surface antigens of group B streptococci (GBS) types Ia, Ib, II and III. Antibody levels to GBS antigens were positively correlated to gestational age and birthweight. However, only the correlations for anti-Ia and anti-II antibody levels reached statistical significance. Mean antibody concentrations in infants below 34 weeks of gestation were significantly lower for type Ia (P〈0.001), type II (P〈0.001) and type III (P=0.05) than in infants above this limit. These findings might explain the higher rate of serious GBS-infections found among prematures as compared to full-term infants.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Hyper-IgE-syndrome ; Multiple liver abscesses ; Percutaneous needle aspiration ; Oral chloramphenicol
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a patient with Hyper-IgE-syndrome multiple liver abscesses developed in spite of prophylactic treatment with trimethoprim and sulfamethoxazol. Ultrasound confirmed the clinical diagnosis and percutaneous needle aspiration under ultrasonographic guidance and culture of the aspirated pus allowed specific antibiotic treatment by oral chloramphenicol alone without surgical drainage. The isolated Staph. aureus strain was resistant to trimethoprim and sulfamethoxazol.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Adhesive proteins ; LFA-1 ; CR3 ; Recurrent infections
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a patient with delayed umbilical cord detachment, recurrent bacterial infections, and inability to form pus, despite persistent leucocytosis. Immunofluorescence studies with specific monoclonal antibodies showed a severe deficiency in the expression of α-chains of the receptor for the C3bi fragment of C3, complement receptor type 3, and the lymphocyte function antigen 1 molecule, found on neutrophil, monocyte and lymphocyte membranes. These membrane antigen defects were responsible for abnormalities in adhesive cell functions. Polymorphonuclear leucoytes demonstrated a markedly reduced chemiluminescence response as well as an impaired nitroblue tetrazolium test and superoxide generation to a particulate stimulus (zymosan), while the responses to a soluble stimulus (phorbol myristate acetate) were normal. In addition, random migration und chemotactic response to zymosan-activated serum were impaired. The lymphocytes demonstrated abolished natural killer cell cytotoxicity as well as abnormal humoral immunity and a lack of antibody response to pertussis and tetanus antigens.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Chronic mucocutaneous candidiasis ; Bronchiectases
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 22-year-old man with chronic mucocutaneous candidiasis (CMC) and hypothyroidism developed severe bronchiectases following recurrent bronchopneumonia. Immunological investigations revealed IgG2/IgG4 subclass deficiency and absence of antibodies against pneumococcal and Haemophilus polysaccharides. Under regular immunoglobulin substitution every 3 weeks pulmonary symptoms improved markedly.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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