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Scandinavian Journal of Infectious Diseases Volume 21, 1989 - Issue 3
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Original Article

Another Swedish Family with Complete Properdin Deficiency: Association with Fulminant Meningococcal Disease in one Male Family Member

Claes Söderström Department of Infectious Diseases, University Hospital, Lund, Sweden
,
Anders G. Sjöholm Department of Medical Microbiology, University Hospital, Lund, Sweden
,
Rune Svensson Department of Infectious Diseases, County Hospital, Uddevalla, Sweden
&
Sten Östenson Department of Medical Microbiology, University Hospital, Lund, Sweden
Pages 259-265 | Published online: 08 Jul 2009

Citations (16)

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Read on this site (2)

Konrad Kolble & Kenneth B. M. Reid. (1993) Genetic Deficiencies of the Complement System and Association with Disease-Early Components. International Reviews of Immunology 10:1, pages 17-36.
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Stefan Berg, Birger Trollfors, Kjell Alestig & Ulf Jodal. (1992) Incidence, Serogroups and Case-Fatality Rate of Invasive Meningococcal Infections in a Swedish Region 1975–1989. Scandinavian Journal of Infectious Diseases 24:3, pages 333-338.
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Articles from other publishers (14)

Ala-Eddine Deghmane & Muhamed-Kheir Taha. (2021) Invasive Bacterial Infections in Subjects with Genetic and Acquired Susceptibility and Impacts on Recommendations for Vaccination: A Narrative Review. Microorganisms 9:3, pages 467.
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Lisa A. Lewis & Sanjay Ram. (2020) Complement interactions with the pathogenic Neisseriae: clinical features, deficiency states, and evasion mechanisms. FEBS Letters 594:16, pages 2670-2694.
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Marieta M. Ruseva, Katherine A. Vernon, Allison M. Lesher, Wilhelm J. Schwaeble, Youssif M. Ali, Marina Botto, Terence Cook, Wenchao Song, Cordula M. Stover & Matthew Caleb Pickering. (2013) Loss of Properdin Exacerbates C3 Glomerulopathy Resulting from Factor H Deficiency. Journal of the American Society of Nephrology 24:1, pages 43-52.
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Sanjay Ram, Lisa A. Lewis & Sarika Agarwal. (2011) Meningococcal Group W-135 and Y Capsular Polysaccharides Paradoxically Enhance Activation of the Alternative Pathway of Complement. Journal of Biological Chemistry 286:10, pages 8297-8307.
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Lone Schejbel, Vibeke Rosenfeldt, Hanne Marquart, Niels Henrik Valerius & Peter Garred. (2009) Properdin deficiency associated with recurrent otitis media and pneumonia, and identification of male carrier with Klinefelter syndrome. Clinical Immunology 131:3, pages 456-462.
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Ralph D. Feigin & William B. Cutrer. 2009. Feigin and Cherry's Textbook of Pediatric Infectious Diseases. Feigin and Cherry's Textbook of Pediatric Infectious Diseases 439 471 .
Jo Anne Welsch & Sanjay Ram. (2008) Factor H and Neisserial pathogenesis. Vaccine 26, pages I40-I45.
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P J Späth, A G Sjöholm, G Fredrikson, G Misiano, R Scherz, U B Schaad, B Uhring-Lambert, G Hauptmann, J Westberg, M Uhlén, C Wadelius & L Truedsson. (1999) Properdin deficiency in a large Swiss family: identification of a stop codon in the properdin gene, and association of meningococcal disease with lack of the IgG2 allotype marker G2m(n). Clinical and Experimental Immunology 118:2, pages 278-284.
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S Ram, F.G Mackinnon, S Gulati, D.P McQuillen, U Vogel, M Frosch, C Elkins, H.-K Guttormsen, L.M Wetzler, M Oppermann, M.K Pangburn & P.A Rice. (1999) The contrasting mechanisms of serum resistance of Neisseria gonorrhoeae and group B Neisseria meningitidis. Molecular Immunology 36:13-14, pages 915-928.
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. 1999. Complement Regulatory Proteins. Complement Regulatory Proteins 261 370 .
L. Truedsson, J. Westberg, G. Nordin Fredrikson, A.G. Sjöholm, E.J. Kuijper, C.A.P. Fijen, P.J. Späth & M. Uhlén. (1997) Human properdin deficiency has a heterogeneous genetic background. Immunopharmacology 38:1-2, pages 203-206.
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Claes Wadelius, Maritta Pigg, Mats Sundvall, Anders G. Sjöholm, Ponmani Goonewardena, Ed J. Kuijper, Cees C. Tijssen, Anton Jansz, Peter J. Späth, Ulf B. Schaad, Lisbeth Tranebjaerg, Hans E. Nielsen, Claos Söderström, Göran Annerén & Ulf Pettersson. (2008) Linkage analysis in properdin deficiency families: refined location in proximal Xp. Clinical Genetics 42:1, pages 8-12.
Crossref
J E FigueroaP Densen. (1991) Infectious diseases associated with complement deficiencies. Clinical Microbiology Reviews 4:3, pages 359-395.
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ANDERS G. SjÖHolm. (1990) Inherited complement deficiency states: implications for immunity and immunological disease. APMIS 98:7-12, pages 861-874.
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