Literature DB >> 2792129

Familial deficiency of the seventh component of complement associated with recurrent meningococcal infections.

W Nürnberger1, H Pietsch, R Seger, T Bufon, V Wahn.   

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 half-normal levels of both immunochemically and functionally determined C7, indicating a heterozygous state for C7 deficiency.

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Year:  1989        PMID: 2792129     DOI: 10.1007/bf00443104

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  13 in total

1.  Immunoblot analysis of the eighth component of human complement. Demonstration of subunits and detection of C8 alpha-gamma double and triple bands.

Authors:  W Nürnberger; R Seger; P Kobler; I Mons; F Tedesco; V Wahn
Journal:  J Immunol Methods       Date:  1988-05-09       Impact factor: 2.303

2.  [Preventive long-term intravenous immunoglobulin infusion in children with acute lymphatic leukemia. I. Anticomplementary activity of gamma globulin preparations is a function of the preparation and IgG concentration prior to infusions].

Authors:  W Nürnberger; R Willers; D Körholz; U Göbel; V Wahn
Journal:  Monatsschr Kinderheilkd       Date:  1988-03       Impact factor: 0.323

3.  Deficiency of C7 with systemic lupus erythematosus: solubilization of immune complexes in complement-deficient sera.

Authors:  H J Zeitz; G W Miller; T F Lint; M A Ali; H Gewurz
Journal:  Arthritis Rheum       Date:  1981-01

4.  A simple method for quantitative measurement of C3d in human plasma.

Authors:  S Bhakdi; M Roth; W Nürnberger
Journal:  J Immunol Methods       Date:  1984-11-16       Impact factor: 2.303

5.  Anti-C4 in the serum of a transfused C4-deficient patient with systemic lupus erythematosus.

Authors:  C M Giles; J L Swanson
Journal:  Vox Sang       Date:  1984       Impact factor: 2.144

6.  Neutrophil function in a patient with meningococcal meningitis and C7 deficiency.

Authors:  C A Davis; S B Shurin; K Pate; B L Congeni
Journal:  Am J Dis Child       Date:  1983-04

7.  Monoclonal antibodies recognizing a neoantigen of poly(C9) detect the human terminal complement complex in tissue and plasma.

Authors:  T E Mollnes; T Lea; M Harboe; J Tschopp
Journal:  Scand J Immunol       Date:  1985-08       Impact factor: 3.487

8.  Inherited deficiency of the seventh component of complement associated with nephritis. Propensity to formation of C56 and related C7-consuming activity.

Authors:  G R Nemerow; H Gewurz; S G Osofsky; T F Lint
Journal:  J Clin Invest       Date:  1978-06       Impact factor: 14.808

9.  Hereditary C7 deficiency. Diagnosis and HLA studies in a French-Canadian family.

Authors:  J M Delâge; P Bergeron; J Simard; G Lehner-Netsch; E Prochazka
Journal:  J Clin Invest       Date:  1977-11       Impact factor: 14.808

10.  [Meningococcal meningitis in isolated familial deficiency of the 7th complement component, C7].

Authors:  P W Straub; P Späth
Journal:  Schweiz Med Wochenschr       Date:  1986-05-24
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  6 in total

Review 1.  Epidemiology, etiology, pathogenesis, and diagnosis of recurrent bacterial meningitis.

Authors:  Marc Tebruegge; Nigel Curtis
Journal:  Clin Microbiol Rev       Date:  2008-07       Impact factor: 26.132

2.  Complement components 2 and 7 (C2 and C7) gene polymorphisms are not major risk factors for SLE susceptibility in the Malaysian population.

Authors:  Lay-Hoong Lian; Ai-Sze Ching; Zheng-Yi Chong; Kek-Heng Chua
Journal:  Rheumatol Int       Date:  2011-09-01       Impact factor: 2.631

3.  Deficiency of late complement components in patients with severe and recurrent meningococcal infections.

Authors:  R Cremer; V Wahn
Journal:  Eur J Pediatr       Date:  1996-08       Impact factor: 3.183

4.  Recurrent bacterial meningitis.

Authors:  G Lieb; J Krauss; H Collmann; L Schrod; N Sörensen
Journal:  Eur J Pediatr       Date:  1996-01       Impact factor: 3.183

Review 5.  Infectious diseases associated with complement deficiencies.

Authors:  J E Figueroa; P Densen
Journal:  Clin Microbiol Rev       Date:  1991-07       Impact factor: 26.132

6.  Complement component deficiencies and infection: C5, C8 and C3 deficiencies in three families.

Authors:  O Sanal; M Loos; F Ersoy; G Kanra; G Seçmeer; I Tezcan
Journal:  Eur J Pediatr       Date:  1992-09       Impact factor: 3.183

  6 in total

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