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Title of Journal: Infection

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Abbravation: Infection

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Springer Berlin Heidelberg

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DOI

10.1007/s11422-007-9050-4

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1439-0973

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Das BuckleySyndrom Rezidivierende schwere Staph

Authors: S Däumling M C Lalama B H Belohradsky D Buriot P Huu Trung C Griscelli
Publish Date: 1980/05/01
Volume: 8, Issue: 3, Pages: S248-S254
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Abstract

Fünfzehn Patienten im Alter von drei bis 27 Jahren wurden klinisch und infektionsimmunologisch untersucht Allen gemeinsam waren schwere rezidivierende kutane und pulmonale Staphylokokkeninfektionen ein chronisches Ekzem Bluteosinophilie und eine extreme Erhöhung des SerumIgESpiegels Acht der Patienten wiesen zudem eine faziale Dysmorphie auf die durch grobe Gesichtszüge Prognathie und schlecht modellierte Ohrmuscheln charakterisiert ist Eine deutliche Osteoporose vor allem der Wirbelkörper wurde bei acht Patienten beobachtet Ein konstanter GranulozytenChemotaxisDefekt fand sich nur in drei Fällen variable oder konstant normale ChemotaxisBefunde traten in sechs Fällen auf Eine polyklonale Hypergammaglobulinämie 14 Patienten erhöhtes IgD zehn Patienten wurden nachgewiesen ebenso ein partieller TZellDefekt zwei Patienten und eine fehlende anamnestische Antikörperantwort bei einem Patienten Therapieversuche mit Immunglobulinen TransferFaktor Levamisol und antiH2RezeptorAntihistaminika blieben bei der pathogenetisch ungeklärten Erkrankung ohne anhaltende oder befriedigende klinische und immunologische ErgebnisseFifteen patients aged between three and 27 years were examined clinically and immunologically Common to all patients were severe recurring cutaneous and pulmonary staphylococcal infections chronic eczema eosinophilia and an extremely elevated serum IgE level Eight of the patients had in addition facial dysplasia characterised by coarse features prognathism and poorly formed external ears Marked osteoporosis particularly of the vertebral bodies was observed in eight patients A constant defect of granulocyte chemotaxis was found in only three patients fluctuating or constantly normal chemotaxis occurred in six patients Polyclonal hypergammaglobulinemia was detected in 14 patients elevated IgD in two patients a partial Tcell defect in two patients and a history of lack of antibody response in one patient Therapeutic trials with immunoglobulins transfer factor levamisole and antiH2 receptorantihistamines did not produce lasting or satisfactory clinical or immunological results in the pathogenetically unidentified disease


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  1. Malassezia Species: A Rare Cause of Invasive Fungal Infections in Immunocompromised Patients
  2. Evaluation of the NanoCHIP ® Infection Control Panel test for direct detection and screening of methicillin-resistant Staphylococcus aureus (MRSA), Klebsiella pneumoniae carbapenemase (KPC)-producing bacteria and vancomycin-resistant Enterococcus (VRE)
  3. Endocarditis caused by Streptococcus canis : an emerging zoonosis?
  4. Increasing Occurrence of Multidrug-Resistance in Acinetobacter baumannii Isolates From Four German University Hospitals, 2002–2006
  5. Antineutrophil cytoplasmic antibody-associated vasculitis associated with Epstein–Barr virus infection: a case report and review of the literature
  6. Zur Diffusion von Cefotaxim in verschiedene Gewebe des urologischen Bereichs
  7. Validation of a multiplex reverse transcriptase PCR ELISA for the detection of 19 respiratory tract pathogens
  8. Interferon-γ releasing assay versus tuberculin skin testing for latent tuberculosis infection in targeted screening programs for high risk immigrants
  9. Complicated Community-Acquired Soft Tissue Infection by MRSA from Porcine Origin
  10. Human metapneumovirus infection after allogeneic hematopoietic stem cell transplantation
  11. Reasons for not starting antiretroviral therapy in HIV-1-infected individuals: a changing landscape
  12. Serum Citrate Levels, Haptoglobin Haplotypes and Transferrin Receptor (CD71) in Patients With HIV-1 Infection
  13. Immune-Mediated Severe Hemolytic Crisis with a Hemoglobin Level of 1.6 g/dl Caused by Anti-Piperacillin Antibodies in a Patient with Cystic Fibrosis
  14. Combination of Quinupristin/Dalfopristin and Glycopeptide in Severe Methicillin-Resistant Staphylococcal Infections Failing Previous Glycopeptide Regimens
  15. Challenging cases discussed by experts: retinal vasculitis following coinfection with HIV and syphilis
  16. Extended-spectrum β-lactamase-producing Escherichia coli strains in the feces of carriers contribute substantially to urinary tract infections in these patients
  17. Relevance of intracranial hypertension control in the management of Cryptococcus neoformans meningitis related to AIDS
  18. A case of postmalaria neurological syndrome in Japan
  19. Produktion und Reinigung des thermolabilen Escherichia coli-Enterotoxins
  20. Economic burden of Clostridium difficile associated diarrhoea: a cost-of-illness study from a German tertiary care hospital
  21. Current Antifungal Agents for Treatment of Central Nervous System Infections
  22. Experience with daptomycin daily dosing in ICU patients undergoing continuous renal replacement therapy
  23. Failure of Current Antibiotic First-Line Regimens and Mortality in Hospitalized Patients with Spontaneous Bacterial Peritonitis
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  25. Why is antimicrobial de-escalation under-prescribed for urinary tract infections?
  26. Is aciclovir prophylaxis necessary after bone marrow transplantation?
  27. Rare Fungal Infections in Children: An Updated Review of the Literature
  28. Combination antibiotic therapy for the treatment of infective endocarditis due to enterococci
  29. Diagnosis of tubercular uveitis by quantitative polymerase chain reaction
  30. New developments in the antifungal susceptibility testing of Candida
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  32. Reply: Is it really Aeromonas hydrophila ?
  33. Comparison of the clinical characteristics of Mycobacterium tuberculosis and nontuberculous mycobacteria patients with joint involvement
  34. Detection of Early Liver Fibrosis in Patients with Intestinal Schistosomiasis: Sonographic and Histologic Findings in Schistosoma mansoni Infection
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