Literature DB >> 3497744

Combined familial C7 and C4B deficiency in an adult with meningococcal disease.

H M Chapel, T E Peto, G A Luzzi, R A Thompson, A H Fielder, J R Batchelor.   

Abstract

A case of meningococcal septicaemia is reported in an adult with a deficiency of the seventh component of complement combined with a deficiency of the B locus product of C4. A family study demonstrated that the two deficiencies were not linked. This is the first time that the individual alleles of C4 were determined in a patient with a deficiency of one component of the terminal pathway. It is possible that the heterogeneous clinical picture of a terminal pathway deficiency may, in part, be explained by the co-existence of other subtle complement defects.

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Year:  1987        PMID: 3497744      PMCID: PMC1542554     

Source DB:  PubMed          Journal:  Clin Exp Immunol        ISSN: 0009-9104            Impact factor:   4.330


  11 in total

1.  Combined genetic deficiency of C6 and C7 in man.

Authors:  P J Lachmann; M J Hobart; P Woo
Journal:  Clin Exp Immunol       Date:  1978-08       Impact factor: 4.330

Review 2.  Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency.

Authors:  S C Ross; P Densen
Journal:  Medicine (Baltimore)       Date:  1984-09       Impact factor: 1.889

3.  Covalent binding efficiency of the third and fourth complement proteins in relation to pH, nucleophilicity, and availability of hydroxyl groups.

Authors:  S K Law; T M Minich; R P Levine
Journal:  Biochemistry       Date:  1984-07-03       Impact factor: 3.162

4.  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

5.  The molecular basis for the difference in immune hemolysis activity of the Chido and Rodgers isotypes of human complement component C4.

Authors:  D E Isenman; J R Young
Journal:  J Immunol       Date:  1984-06       Impact factor: 5.422

6.  Familial late complement component (C6, C7) deficiency with chronic meningococcemia.

Authors:  J D Clough; M L Clough; A Weinstein; L H Calabrese; L R Mansfield; P Gulick; T Gavan; W E Braun
Journal:  Arch Intern Med       Date:  1980-07

7.  Prevalence of congenital or acquired complement deficiency in patients with sporadic meningococcal disease.

Authors:  R T Ellison; P F Kohler; J G Curd; F N Judson; L B Reller
Journal:  N Engl J Med       Date:  1983-04-21       Impact factor: 91.245

8.  Family study of the major histocompatibility complex in patients with systemic lupus erythematosus: importance of null alleles of C4A and C4B in determining disease susceptibility.

Authors:  A H Fielder; M J Walport; J R Batchelor; R I Rynes; C M Black; I A Dodi; G R Hughes
Journal:  Br Med J (Clin Res Ed)       Date:  1983-02-05

9.  Lack of linkage between gene(s) controlling the synthesis of the seventh component of complement and the HLA region on chromosome No. 6 in man.

Authors:  C Rittner; W Opferkuch; B Wellek; H Grosse-Wilde; P Wernet
Journal:  Hum Genet       Date:  1976-10-28       Impact factor: 4.132

10.  Reactive lysis: the complement-mediated lysis of unsensitized cells. I. The characterization of the indicator factor and its identification as C7.

Authors:  R A Thompson; P J Lachmann
Journal:  J Exp Med       Date:  1970-04-01       Impact factor: 14.307

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  6 in total

1.  Functionally active complement proteins C6 and C7 detected in C6- and C7-deficient individuals.

Authors:  R Würzner; A Orren; P Potter; B P Morgan; D Ponard; P Späth; M Brai; M Schulze; L Happe; O Götze
Journal:  Clin Exp Immunol       Date:  1991-03       Impact factor: 4.330

Review 2.  Infectious diseases associated with complement deficiencies.

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

3.  Combined total deficiency of C7 and C4B with systemic lupus erythematosus (SLE).

Authors:  O G Segurado; A A Arnaiz-Villena; P Iglesias-Casarrubios; J Martinez-Laso; J L Vicario; G Fontan; M Lopez-Trascasa
Journal:  Clin Exp Immunol       Date:  1992-03       Impact factor: 4.330

4.  The population sizes and global extinction risk of reef-building coral species at biogeographic scales.

Authors:  Andreas Dietzel; Michael Bode; Sean R Connolly; Terry P Hughes
Journal:  Nat Ecol Evol       Date:  2021-03-01       Impact factor: 15.460

5.  Real-time PCR quantification of human complement C4A and C4B genes.

Authors:  Agnes Szilagyi; Bernadett Blasko; Denes Szilassy; George Fust; Maria Sasvari-Szekely; Zsolt Ronai
Journal:  BMC Genet       Date:  2006-01-10       Impact factor: 2.797

6.  Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients.

Authors:  A Audemard-Verger; E Descloux; D Ponard; A Deroux; B Fantin; C Fieschi; M John; A Bouldouyre; L Karkowsi; G Moulis; H Auvinet; F Valla; C Lechiche; B Davido; M Martinot; C Biron; F Lucht; N Asseray; A Froissart; R Buzelé; A Perlat; D Boutboul; V Fremeaux-Bacchi; S Isnard; B Bienvenu
Journal:  Medicine (Baltimore)       Date:  2016-05       Impact factor: 1.889

  6 in total

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