Advertisement

Inherited deficiency of the second component of complement (C2) with membranoproliferative glomerulonephritis

      This paper is only available as a PDF. To read, Please Download here.

      Abstract

      The occurrence of membranoproliferative glomerulonephritis in a 13 year old boy with inherited complete deficiency of the second component of complement (C2) is described here for the first time. Results of the complement studies and the associations of glomerulonephritis with complement deficiencies are discussed.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to The American Journal of Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Stroud RM
        Genetic abnormalities of the complement system of man associated with disease.
        Transplant Proc. 1974; 6: 59
        • Alper CA
        • Rosen FS
        The role of complement in vivo as revealed by genetic defects.
        in: Progress in Immunology II. vol 1. North-Holland Publishing Company, Amsterdam1974: 201
        • Friend PS
        • Handwerger BS
        • Kim Y
        • et al.
        C2 deficiency in man. Genetic relationship to a mixed lymphocyte reaction determinant (7a∗).
        Immunogenetics. 1975; 2: 569
        • Nelson Jr, RA
        • Jensen J
        • Gigli I
        • et al.
        Methods for the separation, purification and measurement of nine components of hemolytic complement in guinea pig serum.
        Immunochemistry. 1966; 3: 111
        • Borsos T
        • Rapp HJ
        Immune hemolysis: a simplified method for the preparation of EAC4 with guinea pig or with human complement.
        J Immunol. 1967; 99: 263
        • Gewurz H
        • Page AR
        • Pickering RJ
        • et al.
        Complement activity and inflammatory neutrophil exudation in man. Studies in patients with glomerulonephritis, essential hypocomplementemia and agammaglobulinemia.
        Int Arch Allergy Appl Immunol. 1967; 32: 64
        • McLean RH
        • Geiger H
        • Burke B
        • et al.
        Recurrence of membranoproliferative glomerulonephritis following kidney transplantation. Serum complement component studies.
        Am J Med. 1976; 60: 60
        • McLean RH
        • Michael AF
        Properdin and C3 proactivator: alternate pathway components in human glomerulonephritis.
        J Clin Invest. 1973; 52: 634
        • Morse JH
        • Christian CL
        Immunological studies of the 11S protein component of the human complement system.
        J Exp Med. 1964; 119: 195
        • Müller-Eberhard HJ
        • Nilsson U
        • Aronsson T
        Isolation and characterization of two β1-glycoproteins of human serum.
        J Exp Med. 1960; 111: 201
        • Götze O
        • Müller-Eberhard HJ
        The C3-activator system: an alternate pathway of complement activation.
        J Exp Med. 1971; 134: 90S
        • Pensky J
        • Hinz Jr, CF
        • Todd EW
        • et al.
        Properties of highly purified human properdin.
        J Immunol. 1968; 100: 142
        • Vallota EH
        • Götze O
        • Spiegelberg HL
        • et al.
        A serum factor in chronic hypocomplementemic nephritis distinct from immunoglobulins and activating the alternate pathway of complement.
        J Exp Med. 1974; 139: 1249
        • Michael AF
        • Drummond KN
        • Good RA
        • et al.
        Acute post-streptococcal glomerulonephritis: immune deposit disease.
        J Clin Invest. 1966; 45: 237
        • Wold RT
        • Young FE
        • Tan EM
        • et al.
        Deoxyribonucleic acid antibody: a method to detect its primary interaction with deoxyribonucleic acid.
        Science. 1968; 161: 806
        • Pickering RJ
        • Michael AF
        • Herdman RC
        • et al.
        The complement system in chronic glomerulonephritis: three newly associated aberrations.
        J Pediatr. 1971; 78: 30
        • Mancado B
        • Day NKB
        • Good RA
        • et al.
        Lupus-erythematosus-like syndrome with a familial defect of complement.
        N Engl J Med. 1972; 286: 689
        • Day NK
        • Geiger H
        • Stroud R
        • et al.
        C1r deficiency: an inborn error associated with cutaneous and renal disease.
        J Clin Invest. 1972; 51: 1102
        • Hauptmann G
        • Grosshans E
        • Held E
        • et al.
        Lupus erythemateaux aigu avec deficit complet de la fraction C4 du complement.
        Nouv Presse Med. 1974; 3: 881
        • Gelfand EW
        • Clarkson JE
        • Minta JO
        Selective deficiency of the second component of complement in a patient with anaphylactoid purpura.
        Clin Immunol Immunopathol. 1975; 4: 269
        • Einstein LP
        • Alper CA
        • Bloch KJ
        • et al.
        Biosynthetic defect in monocytes from human beings with genetic deficiency of the second component of complement.
        N Engl J Med. 1975; 292: 1169
        • Day NK
        • Geiger H
        • McLean R
        • et al.
        C2 deficiency. Development of lupus erythematosus.
        J Clin Invest. 1973; 52: 1601
        • Osterland CK
        • Espinoza L
        • Parker LP
        • et al.
        Inherited C2 deficiency and systemic lupus erythematosus: studies on a family.
        Ann Intern Med. 1975; 82: 323
        • Kohler PF
        Inherited complement deficiencies and systemic lupus erythematosus: an immunogenetic puzzle.
        Ann Intern Med. 1975; 82: 420
        • Peters DK
        • Williams DG
        Complement and mesangiocapillary glomerulonephritis. Role of complement deficiency in the pathogenesis of nephritis.
        Nephron. 1974; 13: 189
        • Alper CA
        • Colten HR
        • Rosen FS
        • et al.
        Homozygous deficiency of C3 in a patient with repeated infections.
        Lancet. 1972; 2: 1179
        • Ballow M
        • Shira JE
        • Harden L
        • et al.
        Complete absence of the third component of complement in man.
        J Clin Invest. 1975; 56: 703
        • Verroust PJ
        • Wilson CB
        • Dixon FJ
        Lack of nephritogenicity of systemic activation of the alternate complement pathway.
        Kidney Int. 1974; 6: 157
        • Benacerraf B
        • McDevitt HO
        Histocompatibility-linked immune response genes.
        Science. 1972; 175: 273
        • Vladutin AO
        • Rose NR
        Autoimmune murine thyroiditis. Relation to histocompatibility (H-2) type.
        Science. 1971; 174: 1137
        • Williams RM
        • Moore MJ
        Linkage of susceptibility to experimental allergic encephalomyelitis to the major histocompatibility locus in the rat.
        J Exp Med. 1973; 138: 775
        • Stenglein B
        • Thoenes GH
        • Gunther E
        Genetically controlled autologous immune complex glomerulonephritis in rats.
        J Immunol. 1975; 115: 895
        • Levine BB
        • Stember RH
        • Fotino M
        Ragweed hay fever. Genetic control and linkage to HL-A haplotypes.
        Science. 1972; 178: 1201
        • Marsh DG
        • Bias WB
        • Hsu SH
        • et al.
        Association of the HL-A7 cross-reacting group with a specific reaginic antibody response in allergic man.
        Science. 1973; 179: 691
        • Blumenthal MN
        • Amos DB
        • Noreen H
        • et al.
        Genetic mapping of lr locus in man. Linkage to second locus of HL-A.
        Science. 1974; 184: 1301
        • Greenberg LJ
        • Gray ED
        • Yunis EJ
        Association of HL-A 5 and immune responsiveness in vitro to streptococcal antigens.
        J Exp Med. 1975; 141: 935
        • Fu SM
        • Kunkel HG
        • Brusman HP
        • et al.
        Evidence for linkage between HL-A histocompatibility genes and those involved in the synthesis of the second component of complement.
        J Exp Med. 1974; 140: 1108
        • Day NK
        • L'Esperance P
        • Good RA
        • et al.
        Hereditary C2 deficiency: genetic studies and association with the HL-A system.
        J Exp Med. 1975; 141: 1464
        • Shur PH
        • Gibson D
        • Glass D
        • et al.
        Association of complement deficiencies and the HL-A system (abstract).
        Clin Res. 1975; 23: 413A
        • Wolski KP
        • Schmid FR
        • Mittal KK
        Genetic linkage between the HL-A system and a deficit of the second component (C2) of complement.
        Science. 1975; 188: 1020
        • Fu SM
        • Stern R
        • Kunkel HG
        • et al.
        Mixed lymphocyte culture determinants and C2 deficiency: LD-7a associated with C2 deficiency in four families.
        J Exp Med. 1975; 142: 495
        • McDevitt HO
        • Bodmer WF
        HL-A, immune-response genes, and disease.
        Lancet. 1974; 1: 1269
        • Michael AF
        • McLean RH
        Evidence for activation of the alternate pathway in glomerulonephritis.
        in: Advances in Nephrology. vol 4. Year Book Publishers, Inc, Chicago1974: 49