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Bare lymphocyte syndrome — combined immunodeficiency and neutrophil dysfunction (1990)

Will, N. ; Seger, R. A. ; Betzler, C. ; [et al.]

Springer

European journal of pediatrics 149 (1990), S. 700-704

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ISSN: 1432-1076

Keywords: Bare lymphocyte syndrome ; HLA expression ; Severe combined immunodeficiency syndrome ; Neutrophil dysfunction

Source: Springer Online Journal Archives 1860-2000

Topics: Medicine

Notes: Abstract A 4-year-old girl presented with recurrent infections. Immunoglobulin deficiency (serum and secretory IgA, serum IgG3) neutropenia and neutrophil dysfunction (defective spontaneous migration and chemotaxis) were found. T-lymphocyte counts were normal and they responded to phytohaemagglutinin but were not stimulated by Concanavalin A, pokeweed mitogen and microbial antigens in vitro. Delayed cutaneous hypersensitivity testing to purified protein derivative and candidin was negative. Despite bacille Calmette-Guérm vaccination and candidiasis, near normal β-2-microglobulin and human leucocyte antigen (HLA) class I concentrations were detected on mononuclear cells and phytohaemagglutinin-induced lymphoblasts. HLA class II antigens (HLA-DP, −DQ, −DR) were not expressed. These observations indicated a bare lymphocyte syndrome (BLS) type II. This is the first time neutrophil dysfunction has been noted in association with BLS.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1007/BF01959526

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Eisen- und Mangankomplexe mit sauerstoff-, schwefel- und methylen-verbrückten Distibanen und mit Chlorodiphenylstiban als Liganden (1993)

Graf, N. ; Wieber, M.

Weinheim : Wiley-Blackwell

Zeitschrift für anorganische Chemie 619 (1993), S. 2061-2065

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ISSN: 0044-2313

Keywords: Dicarbonylbis(triphenylphosphite)(stibine)iron complexes ; Bis[dicarbonylbis(triphenylphosphite)iron](stibine) complexes ; Dicarbonyl(η5-methylcyclopentadienyl)(stibine)manganese complexes ; Bis[dicarbonyl(η5-methylcyclopentadienyl)manganese](stibine) complexes ; Chemistry ; Inorganic Chemistry

Source: Wiley InterScience Backfile Collection 1832-2000

Topics: Chemistry and Pharmacology

Description / Table of Contents: Complexes of Iron and Manganese with Oxygen-, Sulfur-, and Methylene-bridged Distibines and with Chlorodiphenylstibine as LigandsThe photochemical reaction of CO3Fe[P(OPh)3]2 and of MeCpMn(CO)3 with the stibines Ph2SbCl and (Ph2Sb)2X (X = O, S and CH2) yields mononuclear complexes (CO)2[P(OPh)3]2FeL and °Cp(CO)2MnL (L = Ph2SbCl, (Ph2Sb)2X) and the stibine bridged binuclear complexes °CO)2[P(OPh)3]2Fe{(Ph2Sb)2X}Fe(CO)2[P(OPh)3]2 and °Cp(CO)2Mn{(Ph2Sb)2X}Mn(CO)2MeCp.

Notes: Durch photochemische Umsetzung von (CO)3Fe[P(OPh)3]2 und von MeCpMn(CO)3 mit Anti-monliganden des Typs Ph2SbCl und (Ph2Sb)2X (X = O, S und CH2) können Einkernverbindungen (CO)2[P(OPh)3]2FeL und MeCp(CO)2MnL (L = Ph2SbCl, (Ph2Sb)2X sowie die über Distibanliganden verbrückten Zweikernverbindungen (CO)2[P(OPh)3]2Fe{(Ph2Sb)2X}Fe(CO)2[P(OPh)3]2 und MeCp(CO)2Mn{(Ph2Sb)2X}Mn(CO)2MeCp synthetisiert werden.

Additional Material: 4 Tab.