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Major histocompatibility complex antigens in steroid-responsive nephrotic syndrome

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Abstract

An increased frequency of specific major histocompatibility complex (MHC) class I, II and III antigens in children with steroid-responsive nephrotic syndrome (SRNS) has been reported. This frequency distortion, in some cases, is thought to affect the outcome of the disorder. We studied the phenotypic frequency of HLA antigens-A,-B, and-DR, as well as complement proteins Bf and C4 in an unrelated population of 25 SRNS children. Complete MHC haplotypes were also derived for four families in which 8 individuals developed SRNS. HLA-DR8, with a relative risk of 4.8, showed the strongest association with SRNS. Nonetheless, the 95% confidence intervals of this and the relative risks for all other antigens fell below 1.0. No common haplotype was found in SRNS patients in whom complete family studies were available, and disease and inheritance of the MHC were discordant in two of these families. In this study of well-characterized SRNS patients we were unable to discover a clear association between this disorder and the MHC.

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References

  1. International Study of Kidney Disease in Childhood (1981) The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr 98: 561–564

    Google Scholar 

  2. Trompeter RS, Barratt TM, Kay R, Turner MW, Soothill JF (1980) HLA, atopy, and cyclophosphamide in steroidresponsive childhood nephrotic syndrome. Kidney Int 17: 113–117

    Google Scholar 

  3. Giangiacomo J, Cleary TG, Cole BR, Hoffsten P, Robson AM (1975) Serum immunoglobulins in the nephrotic syndrome. N Engl J Med 293: 8–12

    Google Scholar 

  4. Schnaper HW, Aune TM (1987) Steroid-sensitive mechanism of soluble immune response suppressor production in steroid-responsive nephrotic syndrome. J Clin Invest 79: 257–264

    Google Scholar 

  5. Lagrue G, Sobel A, Branellec A, Blanc C (1978) Etude d'une lymphokine agissant sur la permeabilite vasulaire. Ann Med Interne (Paris) 129: 57–60

    Google Scholar 

  6. McLean RH, Ruley EJ, Tina L, Medani D, Chesapeake Pediatric Nephrology Study Group (1986) Increased frequency of silent C4A allele (C4A*QO) of the fourth component of complement (C4) in the idiopathic nephrotic syndrome. Pediatr Res 20: 454A

    Google Scholar 

  7. McLean RH (1983) Increased frequency of factor B fast variant (BF*F) in the idiopathic nephrotic syndrome of childhood. Dis Markers 1: 25–32

    Google Scholar 

  8. McEnery PT, Strife CF (1982) Nephrotic syndrome in childhood: management and treatment in patients with minimal change disease, mesangial proliferation or focal glomerulosclerosis. Pediatr Clin North Am 89: 875–894

    Google Scholar 

  9. Mittal KK, Mickey MR, Singal DP, Terasaki P (1968) Serotyping for hemotransplantation. XVIII. Refinement of microdroplet lymphocyte cytotoxicity kit. Transplantation 6: 913–927

    Google Scholar 

  10. Welch TR, Beischel L, Balakrishnan K, Quinlan M, West CD (1986) Major-histocompatibility complex extended haplotypes in membranoproliferative glomerulonephritis. N Engl J Med 314: 1476–1481

    Google Scholar 

  11. Welch TR, Beischel L, Berry A, Forristal J, West CD (1985) The effect of null C4 alleles on complement function. Clin Immunol Immopathol 34: 316–325

    Google Scholar 

  12. Tiwari JL, Terasaki PI (1985)HLA and disease association. Springer, Berlin Heidelberg New York, pp 18–27

    Google Scholar 

  13. Miettinen OS (1974) Simple interval-estimation of risk ratio. Am J Epidemiol 100: 515

    Google Scholar 

  14. McDevitt HO, Bodmer WF (1972) Histocompatibility antigens, immune responsiveness, and susceptibility to disease. Am J Med 52: 1–8

    Google Scholar 

  15. Bell JI, Denny D, Joster L, Lee BSM, Hardy D, McDevitt HO (1986) Molecular biology of the class II response of the human major histocompatibility complex. Cold Spring Harbor Symp Quant Biol 51: 75–82

    Google Scholar 

  16. Fielder AHL, Walport MJ, Batchelor JR, Ryner RI, Black CM, Dodi IA, Hughes GRV (1983) Family study of the major histocompatibility complex in patients with systemic lupus erythematosus. Br Med J 186: 425–428

    Google Scholar 

  17. Raum D, Audeh Z, Glau D, Alper CA (1981) MHC-linked complement haplotypes (complotypes) in juvenile rheumatoid arthritis. Clin Res 29: 559A

    Google Scholar 

  18. Howell MD, Austin RK, Kelleher D, Nepom GT, Kagnoff MF (1986) An HLA-D region restricted fragment length polymorphism associated with celiac disease. J Exp Med 164: 333–338

    Google Scholar 

  19. Raum D, Awdeh Z, Gung EJ, Alper CA, Gabbay KH (1984) Extended major histocompatibility complex haplotypes in type I diabetes mellitus. J Clin Invest 74: 449–454

    Google Scholar 

  20. Klouda PT, Acheson EJ, Goldby FS, Lawler W, Manos J, Dyer PA, Harris R, Mallick NP, Williams G (1979) Strong association between idiopathic membranous nephropathy and HLA-DRW3. Lancet 2: 770–771

    Google Scholar 

  21. Kashiwabara H, Shishido H, Tomura S, Tuchida H, Miyajima T (1982) Strong association between IgA nephropathy and HLA-DR4 antigen. Kidney Int 22: 377–382

    Google Scholar 

  22. Berthoux FC, Gagne A, Sabatier JC, Ducret F, LePetit SC, Marcellin M, Mercier B, Brizard CP (1978) HLA-Bw35 and mesangial IgA glomerulonephritis. N Engl J Med 298: 1034–1035

    Google Scholar 

  23. O'Regan D, O'Callaghan U, Dunden S, Reen DJ (1980) HLA antigens and steroid responsive nephrotic syndrome of childhood. Tissue Antigens 16: 147–151

    Google Scholar 

  24. Thomson PD, Barratt TM, Stokes CR, Turner MW, Soothill JF (1976) HLA antigens and atopic features in steroid responsive nephrotic syndrome of childhood. Lancet II: 765–768

    Google Scholar 

  25. Alfiler CA, Roy LP, Doran T, Sheldon A, Baylir H (1980) HLA-Drw7 and steroid responsive nephrotic syndrome of childhood. Clin Nephrol 14: 71–74

    Google Scholar 

  26. Kobayashi Y, Chen X-M, Hiki Y, Fujii K, Kashiwagi N (1985) Association of HLA-DRw8 and DQw3 with minimal change nephrotic syndrome in Japanese adults. Kidney Int 28: 193–197

    Google Scholar 

  27. Noel LH, Descamps B, Jungers P, Bach JF, Busson M, Suet C, Hors J, Dausset J (1978) HLA antigens in three types of glomerulonephritis. Clin Immunol Immunopathol 10: 19–23

    Google Scholar 

  28. Chandra M, Mouradian MD, Hoyer JR, Lewy JE (1981) Familial nephrotic syndrome and focal segmental glomerulosclerosis. J Pediatr 98: 55–560

    Google Scholar 

  29. Welch TR, Berry A, Beischel LS (1987) C4 isotype deficiency in IgA nephropathy. Pediatr Nephrol 1: 136–139

    Google Scholar 

  30. McLean RH, Wyatt RJ, Julian BA (1984) Complement phenotypes in glomerulonephritis: increased frequency of homozygous null C4 phenotypes in IgA nephropathy and Henoch-Schönlein purpura. Kidney Int 26: 855–860

    Google Scholar 

  31. Tiwari JL, Terasaki PI (1984)

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McEnery, P.T., Welch, T.R. Major histocompatibility complex antigens in steroid-responsive nephrotic syndrome. Pediatr Nephrol 3, 33–36 (1989). https://doi.org/10.1007/BF00859622

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  • DOI: https://doi.org/10.1007/BF00859622

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