Search Results - (Author, Cooperation:I. Tezcan)
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1X. Zhang ; D. Bogunovic ; B. Payelle-Brogard ; V. Francois-Newton ; S. D. Speer ; C. Yuan ; S. Volpi ; Z. Li ; O. Sanal ; D. Mansouri ; I. Tezcan ; G. I. Rice ; C. Chen ; N. Mansouri ; S. A. Mahdaviani ; Y. Itan ; B. Boisson ; S. Okada ; L. Zeng ; X. Wang ; H. Jiang ; W. Liu ; T. Han ; D. Liu ; T. Ma ; B. Wang ; M. Liu ; J. Y. Liu ; Q. K. Wang ; D. Yalnizoglu ; L. Radoshevich ; G. Uze ; P. Gros ; F. Rozenberg ; S. Y. Zhang ; E. Jouanguy ; J. Bustamante ; A. Garcia-Sastre ; L. Abel ; P. Lebon ; L. D. Notarangelo ; Y. J. Crow ; S. Boisson-Dupuis ; J. L. Casanova ; S. Pellegrini
Nature Publishing Group (NPG)
Published 2014Staff ViewPublication Date: 2014-10-14Publisher: Nature Publishing Group (NPG)Print ISSN: 0028-0836Electronic ISSN: 1476-4687Topics: BiologyChemistry and PharmacologyMedicineNatural Sciences in GeneralPhysicsKeywords: Adolescent ; Alleles ; Child ; Cytokines/deficiency/genetics/*metabolism ; Endopeptidases/chemistry/metabolism ; Female ; Gene Expression Regulation ; Humans ; Inflammation/genetics/immunology/*prevention & control ; Interferon Type I/*immunology/metabolism ; Intracellular Space/*metabolism ; Male ; Pedigree ; S-Phase Kinase-Associated Proteins/metabolism ; Signal Transduction ; Ubiquitination ; Ubiquitins/deficiency/genetics/*metabolism ; Viruses/immunologyPublished by: -
2Sanal, O. ; Morgan, G. ; Göçmen, A. ; Novelli, V. ; Klein, N. ; Tezcan, I. ; Ersoy, F. ; Berkel, A. I. ; Yel, L.
Springer
Published 2000Staff ViewISSN: 1432-1076Keywords: Key words Bacillus-Calmette Guerin ; Disseminated infection ; Granulocyte-monocyte colony stimulating factor ; Interferon-γSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract Severe disseminated Bacillus-Calmette-Guerin (BCG) infection is very rare and has been regarded as idiopathic when no immunodeficiency is present. This entity seems to be due to several new types of inherited abnormalities in the pathways important in defence against Mycobacteria. Although improvement with interferon-γ (IFN-γ) has been reported in some patients, to our knowledge there are no reports on the effect of other cytokines in the treatment of these patients. We report here the clinical response to IFN-γ and granulocyte-monocyte colony stimulating factor (GM-CSF) treatment in a patient with idiopathic disseminated BCG infection who failed to respond to multiple antimycobacterial agents. The patient showed partial and transitory response to IFN-γ, however, GM-CSF treatment led to rapid improvement of skin lesions within 2 weeks without any effect on the progression of the disease in the other organ systems. Conclusion The response of idiopathic disseminated Bacillus-Calmette-Guerin infection to granulocyte-monocyte colony stimulating factor treatment was limited to cutaneous lesions. Granulocyte-monocyte colony stimulating factor may have acted to promote wound healing or the levels of this factor achieved in other affected organs may have been inadequate.Type of Medium: Electronic ResourceURL: -
3Staff View
ISSN: 1432-1076Keywords: C5 Deficiency ; C8 Deficiency ; C3 Deficiency ; Infection ; Recurrent meningitisSource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract Three families are described with complement component deficiencies. In one family, five children had C5 deficiency; in a second family, two children had C8 deficiency and one child in a third family had C3 deficiency. The index cases were identified during screening of patients with recurrent pyogenic infections, recurrent meningitis and meningococcaemia. Two of the five C5 deficient patients had recurrent meningitis and meningococcaemia, two had recurrent respiratory tract infections and otitis and one was healthy. One of the C8 deficient patients had meningitis, meningococcaemia and pneumonia, whereas his sibling with the same deficiency was healthy. The patient with C3 deficiency had four episodes of meningitis and recurrent otitis.Type of Medium: Electronic ResourceURL: -
4Sanal, O. ; Ersoy, F. ; Yel, L. ; Tezcan, I. ; Metin, A. ; Özyürek, H. ; Gariboglu, S. ; Fikrig, S. ; Berkel, A. I. ; Rijkers, G. T. ; Zegers, B. J. M.
Springer
Published 1999Staff ViewISSN: 1573-2592Keywords: Ataxia–telangiectasia ; pneumococcal polysaccharides ; antibody ; IgG subclass ; IgASource: Springer Online Journal Archives 1860-2000Topics: MedicineNotes: Abstract Various factors seem to be etiologic in the susceptibility to sinopulmonary infections in ataxia–telangiectasia (A-T) patients, i.e., low serum and salivary IgA, low serum IgG2, and even aspiration of saliva. S. pneumoniae is a common pathogen responsible from pulmonary infections and impaired antibody response to polysaccharide antigens is seen in patients with IgG2 and IgA deficiency as well as patients with CVID and WAS. We studied IgG-type antibody production to six pneumococcal serotypes in 29 A-T patients by ELISA before and 3–4 weeks after pneumococcal vaccine. The response was considered positive when the antibody titer was 〉10 U/ml but weak when the titer was 10–20 U/ml. Twenty-two of 29 (76%) patients did not respond to any of the serotypes, 5 (17%) showed a positive response to one serotype, 1 (3.4%) to two serotypes, and 1 (3.4%) to four serotypes. With conversion to gravimetric units (ng IgG/ml) and 〉1800 ng/ml (300 ng Ab N/ml) considered a positive response, 5 of 29 (17.2%) patients showed a positive response (300 ng ab N/ml) to two or fewer serotypes. All patients tested produced IgG antibody to tetanus toxoid. Sixteen of 27 (59.3%) patients had low IgG2 and four (14.8%) had low IgG3 levels, while 18 (62.1%) of 29 patients had low serum IgA. No correlation was found either between serum Ig isotype levels and antipolysaccharide antibody response or between susceptibility to infection and antibody production. The mechanism responsible for disturbed antipolysaccharide (TI-2 antigen) antibody production in patients with A-T needs to be investigated. It may provide additional information on the function of the ATM gene product and be helpful in clarifying the role of B cells and contribution of T cells in TI-2 responsesType of Medium: Electronic ResourceURL: