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  • 1
    Electronic Resource
    Electronic Resource
    Phagocyte dysfunction in common variable immune deficiency (1977)
    Springer
    Journal of molecular medicine 55 (1977), S. 735-742 
    Details
    ISSN: 1432-1440
    Keywords: Variables Immunmangelsyndrom ; Neutropathie ; Monozytendysfunktion ; Folsäuremangel ; Common variable immune deficiency ; Neutrophil-monocyte dysfunction ; Deficiency of folic acid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The history of a 13-year old boy is reported who suffered from frequent bacterial, enteroviral, and protozoal infections since late infancy. A decrease in the serum levels of IgG2, IgG3, IgA, a neutrophil dysfunction, and a partial cellular immune deficiency could be demonstrated. A deficiency of folic acid produced a pancytopenia which enhanced the patient's susceptibility to infections. The combined substitution of gammaglobulins and folic acid only was able to break this vicious cycle.
    Notes: Zusammenfassung Es wird über einen 13jährigen Jungen berichtet, der seit der späten Säuglingszeit an häufig wiederkehrenden bakteriellen, enteroviralen und protozoalen Infekten erkrankte. Neben der Erniedrigung der IgG2- IgG3- sowie IgA-Serumspiegel wurden eine Neutropathie und eine partielle Störung der zellulären Infektabwehr gefunden. Ein Folsäuremangel führte zur Panzytopenie und erhöhte damit noch die Infektanfälligkeit. Erst die kombinierte Substitution von Gammaglobulinen und Folsäure vermochte den Circulus vitiosus zu durchbrechen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01476960
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  • 2
    Electronic Resource
    Electronic Resource
    Neopterin in AIDS, other immunodeficiencies, and bacterial and viral infections (1986)
    Springer
    Journal of molecular medicine 64 (1986), S. 333-337 
    Details
    ISSN: 1432-1440
    Keywords: AIDS ; Neopterin ; Stimulated monocytes ; Immunodeficiencies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An increase in total urinary neopterin was observed in 12 of 13 patients with acquired immunodeficiency syndrome (AIDS), seven of 13 patients with lymphadenopathy, one of six healthy homosexual males, seven of ten adult patients with staphylococcal pneumonia, 11 of 12 children with viral infections, four of seven children with bacterial infections, and 12 of 13 children with various immune defects. Extremely high values of total urinary neopterin and monapterin were observed in severely ill patients with AIDS and those with familial hemophagocytic lymphohistiocytosis. Neopterin excretion was normal in two AIDS patients with Kaposi's sarcoma, but without opportunistic infections at that time. On reexamination of one of these patients later on, elevated neopterin values were noted. Parallel increases in neopterin and monapterin were found, whereas biopterin was usually normal. The increase in total neopterin was mainly due to 7,8-dihydroneopterin and was accompanied by an increase in 3′-hydroxysepiapterin. Increased neopterin in urine is assumed to reflect the increase in GTP pool and GTP cyclohydrolase I activity as observed in stimulated monocytes. Thus, neopterin, as a measure of the activation of the nonspecific cellular immune system, may be used diagnostically to detect allograft rejection after transplantations and to follow-up HTLV-III positive patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01711954
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  • 3
    Electronic Resource
    Electronic Resource
    Defects in granulocyte function in various chromosome abnormalities (down's-, edwards'-, cri-du-chat syndrome) (1976)
    Springer
    Journal of molecular medicine 54 (1976), S. 177-183 
    Details
    ISSN: 1432-1440
    Keywords: Granulocyten ; Chemotaxis ; Phagocytose ; Intrazelluläre Keimabtötung ; Down-, Edwards, Cri-du-chat-Syndrom ; Granulocytes ; Chemotaxis ; Phagocytosis ; Intracellular microbicidal activity ; Down's-, Edwards'-, Cri-du-chat Syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary In five infants with autosomal aberrations and diminished resistance to infection (in spite of intact humoral and cellular immune mechanisms) several granulocyte functions (chemotaxis, phagocytosis, intracellular killing and metabolism of killing) were measured. A serum-dependent or a cell-dependent disturbance of phagocytosis of Candida albicans was found in two infants with cat-cry syndrome and one with trisomy 18. In one of these children there was an additional serum dependent defect of the killing of Candida albicans and of Staphylococcus aureus, serum levels of opsonins (IgG, IgM, CH50 and C3) being within normal range. An infant with trisomy 21 showed, in addition to a cellular defect of chemotaxis, a reduced cellular ability of the killing of Staphylococcus aureus and of Escherichia coli in autologous and AB-pool-serum. Phagocytosis of these bacteria remained normal.
    Notes: Zusammenfassung Bei fünf Säuglingen mit Autosomenaberrationen, herabgesetzter Infektresistenz und intaktem humoralen und zellulären Immunapparat wurden die Granulocytenfunktionen Chemotaxis, Phagocytose, intrazelluläre Erregerabtötung und einige Stoffwechselvorgänge experimentell untersucht. Eine serumabhängige bzw. zelluläre Phagocytoseschwäche für Candida albicans bestand bei zwei Säuglingen mit Cri-du-chat-Syndrome und einem anderen mit Trisomie 18. Bei einem dieser Kinder bestand zusätzlich eine serumabhängige, verzögerte Erregerabtötung von Candida albicans sowie von Staphylococcus aureus, wobei die Serumspiegel der Opsonine IgG, IgM, CH 50 und C3 normal waren. Ein Säugling mit Trisomie 21 bot außer einem zellulären Chemotaxisdefekt ein verringertes zelluläres Abtötungsvermögen für Staphylococcus aureus sowie Escherichia coli im Eigen- und im Fremdserum. Die Phagocytose dieser Keime hingegen verlief ungestört.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01468883
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  • 4
    Electronic Resource
    Electronic Resource
    Neonatal lupus erythematosus with congenital heart block and severe heart failure due to myocarditis and endocardititis of the mitral valve (1996)
    Springer
    Intensive care medicine 22 (1996), S. 464-466 
    Details
    ISSN: 1432-1238
    Keywords: Neonatal lupus erythematosus ; Heart block ; Steroids ; Myocarditis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a case of neonatal lupus erythematosus (NLE) with congenital heart block and severe myocardial failure, which was followed from the 25th week of gestation because of fetal bradycardia. The child was delivered at the 37th week of gestation by elective cesarean section because of echocardiographically documented heart enlargement, pericardial effusion and moderate insufficiency of the mitral and tricuspid valves. In spite of immediate pacing, intubation and supportive treatment, the newborn developed progressive heart failure. Echocardiography showed endocarditis of the mitral valve and diffuse myocarditis. The heart failure resolved under steroid treatment. Our experience supports the early use of steroids in treating myocarditis due to NLE. Intrauterine steroid treatment in the presence of fetal hydrops and congenital heart block is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01712168
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  • 5
    Electronic Resource
    Electronic Resource
    Neonatal lupus erythematosus with congenital heart block and severe heart failure due to myocarditis and endocardititis of the mitral valve (1996)
    Springer
    Intensive care medicine 22 (1996), S. 464-466 
    Details
    ISSN: 1432-1238
    Keywords: Key words Neonatal lupus erythematosus ; Heart block ; Steroids ; Myocarditis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a case of neonatal lupus erythematosus (NLE) with congenital heart block and severe myocardial failure, which was followed from the 25th week of gestation because of fetal bradycardia. The child was delivered at the 37th week of gestation by elective cesarean section because of echocardiographically documented heart enlargement, pericardial effusion and moderate insufficiency of the mitral and tricuspid valves. In spite of immediate pacing, intubation and supportive treatment, the newborn developed progressive heart failure. Echocardiography showed endocarditis of the mitral valve and diffuse myocarditis. The heart failure resolved under steroid treatment. Our experience supports the early use of steroids in treating myocarditis due to NLE. Intrauterine steroid treatment in the presence of fetal hydrops and congenital heart block is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01712168
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  • 6
    Electronic Resource
    Electronic Resource
    Correlation between serum antibody-levels against group B streptococci and gestational age in newborns (1984)
    Springer
    European journal of pediatrics 142 (1984), S. 86-88 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00445584
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  • 7
    Electronic Resource
    Electronic Resource
    Oral chloramphenicol therapy for multiple liver abscesses in hyperimmunoglobulinemia E syndrome (1984)
    Springer
    European journal of pediatrics 142 (1984), S. 292-295 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00540256
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  • 8
    Electronic Resource
    Electronic Resource
    Familial deficiency of the seventh component of complement associated with recurrent meningococcal infections (1989)
    Springer
    European journal of pediatrics 148 (1989), S. 758-760 
    Details
    ISSN: 1432-1076
    Keywords: C7 deficiency ; Inherited complement deficiencies ; Neisseria infections ; C7 immunoblot
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe an 11-year-old girl suffering from recurrent meningitis with a complete absence of the seventh component of complement (C7). Diagnosis was established by haemolytic titration and western blotting. The patient's serum lacked the 85 kDa C7 chain. Haemolytic activity of serum was reconstituted with either pooled normal human serum or with purified C7. The relatives (parents and one sister) had halfnormal levels of both immunochemically and functionally determined C7, indicating a heterozygous state for C7 deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00443104
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  • 9
    Electronic Resource
    Electronic Resource
    Absence of complement receptor type 3 and lymphocyte function antigen 1 causing deficient phagocyte and lymphocyte functions (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 58-61 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00441816
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  • 10
    Electronic Resource
    Electronic Resource
    Recombinant human interferon-gamma in patients with chronic granulomatous disease – European follow up study (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 295-298 
    Details
    ISSN: 1432-1076
    Keywords: Key words Chronic granulomatous ; disease ; Interferon-gamma ; Infections
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This was an uncontrolled, open-label follow up study of a previous 12-month, randomized, double-blind, placebo-controlled trial performed to assess the long-term efficacy and safety of Recombinant Human Interferon Gamma (rIFN-γ) in patients with chronic granulomatous disease (CGD). In two centres, 28 patients (24 male, 4 female) with a mean age of 16 years (range 3–37) entered the open-label phase. The patients were treated for a mean of 880 days (range 97–1375 days). Visits were scheduled every 180 days and patients completed one to six visits. rIFN-γ was administered subcutaneously three times weekly at a dose of 0.05 mg per m2. During the open-label phase of the study 12 patients experienced a serious infection requiring hospitalization within 880 days. The median infection-free time was 993 days. No obvious increase of infections over time was seen. Phagocyte superoxide anion production and phagocyte staphylococcal killing were not influenced by therapy. Seven patients were withdrawn from the study, one because of an adverse reaction, three on their own wish and the other three because they changed to another trial. No patient died during the study. Conclusion Treatment of patients with CGD with intracellular active antibiotics and additional interferon gamma as infection prophylaxis is safe and justified.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957365
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