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Mesangial proliterative glomerulonephritis with irregular intramembranous deposits

Another variant of hypocomplementemic nephritis
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      Abstract

      Two patients with a persistent mesangial proliferative glomerulonephritis and reduced serum C3 levels have been followed for six and 10 years. Both have had a mild course with normal renal function and spontaneous morphologic improvement. Levels of the fourth component (C4) and second component (C2) of complement, and properdin factor B have been normal; the third component (C3) of complement nephritic factor has not been detected. Ultrastructurally, irregular intramembranous electron-dense deposits are present, primarily within the lamina densa. These deposits are separated by varying lengths of normal-appearing glomerular basement membrane. Intramembranous and subendothelial electron-lucent areas, containing vesicles, are also seen. Subendothelial deposits, mesangial interposition, splitting of the glomerular basement membrane and ribbon-like intramembranous deposits are not found. The disease in these patients was clinically indistinguishable at onset from membranoproliferative glomerulonephritis (MPGN). The possibility of a variant with mild clinical course, no progression and spontaneous morphologic improvement is important in planning and assessing treatment programs in this disease group.
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      References

        • West CD
        • McAdams AJ
        • McConville JM
        • et al.
        Hypocomplementemic persistent (chronic) glomerulonephritis.
        J Pediatr. 1965; 67: 1089
        • Gotoff SP
        • Fellers FX
        • Vawter GF
        • et al.
        The BIC globulins in childhood nephrotic syndrome.
        N Engl J Med. 1965; 273: 524
        • Davis AE
        • Schneeberger EE
        • Grupe WE
        • et al.
        Membranoproliferative glomerulonephritis and dense deposit disease in children (abstract).
        in: Presented to American Society of Nephrology, 8th Annual Meeting, Washington, D.C.1975
        • Habib R
        • Kleinknecht C
        • Guhler MC
        • et al.
        Idopathic membranoproliferative glomerulonephritis in children. Report of 105 cases.
        Clin Nephrol. 1973; 1: 94
        • Habib R
        • Guhler MC
        • Loirat C
        • et al.
        Dense deposit disease. A variant of membranoproliferative glomerulonephritis.
        Kidney Int. 1975; 7: 204
        • Antoine B
        • Faye C
        The clinical course associated with dense deposits in the kidney basement membranes.
        Kidney Int. 1972; 1: 420
        • Bohle A
        • Gartner HV
        • Fischbach H
        • et al.
        The morphological and clinical features of membranoproliferative glomerulonephritis in adults.
        Virchows Arch A Path Anat Histol. 1974; 363: 213
        • Burkholder PM
        Atlas of Human Glomerular Pathology. Harper & Row, New York1974: 185
        • Strife CF
        • McEmery P
        • McAdams AJ
        • et al.
        A third ultrastructural variety of membranoproliferative glomerulonephritis (abstract).
        in: Presented to American Society of Nephrology, 8th Annual Meeting, Washington, D.C.1975
        • Cameron JS
        • Ogg CS
        • White RHR
        • et al.
        The clinical features and prognosis of patients with normocomplementemic mesangiocapillary glomerulonephritis.
        Clin Nephrol. 1973; 1: 8
        • Ooi YM
        • Vallota EH
        • West CD
        Classical complement pathway activation in membranoproliferative glomerulonephritis.
        Kidney Int. 1976; 9: 46
        • Cameron JS
        • Glasgow EF
        • Ogg CS
        • et al.
        Membranoproliferative glomerulonephritis and persistent hypocomplementemia.
        Br Med J. 1970; 4: 7
        • Mandalenakis N
        • Mendoza N
        • Pirani C
        • et al.
        Lobular glomerulonephritis and membranoproliferative glomerulonephritis: A clinical and pathologic study on renal biopsies.
        Medicine (Baltimore). 1971; 50: 319
        • McAdams AJ
        • McEmery PT
        • West CD
        Mesangiocapillary glomerulonephritis. Changes in glomerular morphology with long-term alternate day prednisone therapy.
        J Pediatr. 1975; 86: 23
        • Ritchie RF
        • Alper CA
        • Graves J
        • et al.
        Automated quantitation of proteins in serum and other biologic fluids.
        Am J Clin Pathol. 1973; 59: 151
        • Rapp HJ
        • Borsos T
        Molecular Basis of Complement Action.
        Appleton-Century-Crofts, New York1970
        • Goldman M
        Fluorescent Antibody Methods.
        New York, Academic Press1968
        • Karnovsky MJ
        Formaldehyde-glutaraldehyde fixative of high osmolality for use in electron microscopy (abstract).
        J Cell Biol. 1965; 27: 137A
        • Northway JD
        • McAdams AJ
        • Forristal J
        • et al.
        A “silent” phase of hypocomplementemic persistent nephritis detectable by reduced serum BIC levels.
        J Pediatr. 1969; 74: 28
        • Rubin MI
        Nephrotic syndrome.
        (chap 19)in: Rubin MI Barratt TM Pediatric Nephrology. Williams and Wilkins, Baltimore1975: 454
        • McLean RH
        • Michael AF
        Properdin and C3 proactivator alternate pathway components in human glomerulonephritis.
        J Clin Invest. 1973; 52: 634
        • Ruley EJ
        • Forristal J
        • Davis NC
        • et al.
        Hypocomplementemia of membranoproliferative nephritis' dependence of the nephritic factor reaction on properdin factor B.
        J Clin Invest. 1973; 52: 896
        • Perrin LH
        • Lambert PH
        • Nydegger VE
        • et al.
        Quantitation of C3PA (properdin factor B) and other complement components in disease associated with a low C3 level.
        Clin Immunol Immunopathol. 1973; 2: 16
        • Williams DG
        • Charlesworth JA
        • Zachmann PJ
        • et al.
        Role of C3b in breakdown of C3 in hypocomplementemic mesangiocapillary glomerulonephritis.
        Lancet. 1973; 1: 447
        • Peters DK
        • Martin A
        • Weinstein A
        • et al.
        Complement studies in membranoproliferative glomerulonephritis.
        Clin Exp Immunol. 1972; 11: 311
      1. Davis AE, Alper CA: Unpublished data.