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A case of congenital nephrotic syndrome associated with positive C1q immunofluorescence

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Abstract

We present a 1-month-old girl with a congenital nephrotic syndrome and unusual histological findings. Immunofluorescence microscopy demonstrated granular mesangial deposition of C1q and electron microscopy revealed electron-dense mesangial deposits. Her heavy proteinuria gradually decreased and the steroid therapy did not have a significant effect. Her renal function was normal throughout the entire period of observation. The clinical evidence and histopathological features of this patient were compatible with C1q nephropathy.

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References

  1. Huttunen NP (1976) Congenital nephrotic syndrome of Finnish type. Study of 75 patients. Arch Dis Child 51: 344–348

    Google Scholar 

  2. Sibley RK, Mahan J, Mauer SM, Vernier RL (1985) A clinicopathologic study of forty-eight infants with nephrotic syndrome. Kidney Int 27: 544–552

    Google Scholar 

  3. Mahan JD, Mauer SM, Sibley RK, Vernier RL (1984) Congenital nephrotic syndrome: evolution of medical management and results of renal transplantation. J Pediatr 105: 549–557

    Google Scholar 

  4. Jennette JC, Hipp CG (1985) C1q nephropathy: a distinct pathologic entity usually causing nephrotic syndrome. Am J Kidney Dis 6: 103–110

    Google Scholar 

  5. Shindo S, Yoshimoto M, Kuriya N, Bernstein J (1988) Glomerular basement membrane thickness in recurrent and persistent hematuria and nephrotic syndrome: correlation with sex and age. Pediatr Nephrol 2: 196–199

    Google Scholar 

  6. Habib R, Bois E (1973) Hétérogénéité des syndromes néphrotiques á début précoce du nourrisson (syndrome néphrotique “infantile”). Helv Paediatr Acta 28: 91–107

    Google Scholar 

  7. Evans DGRE, Lyon AJ (1991) Fatal congenital cytomegalovirus infection acquired by an intra-uterine transfusion. Eur J Pediatr 150: 780–781

    Google Scholar 

  8. George CRP, Hickmann RO, Stricker GE (1976) Infantile nephrotic syndrome. Clin Nephrol 5: 20–24

    Google Scholar 

  9. Hoyer JR, Michael AF, Good RA, Vernier RL (1967) The nephrotic syndrome of infancy: clinical morphologic and immunologic studies of four infants. Pediatrics 40: 233–246

    Google Scholar 

  10. Iskandar SS, Browning MC, Lorentz WB (1991) C1q nephropathy: a pediatric clinicopathologic study. Am J Kidney Dis 18: 459–465

    Google Scholar 

  11. Habib R, Girardin E, Gagnadoux MF, Hinglais N, Levy M, Broyer M (1988) Immunopathological findings in idiopathic nephrosis: clinical significance of glomerular “immune deposits” Pediatr Nephrol 2: 402–408

    Google Scholar 

  12. Shingu M, Yoshioka K, Nobunaga M, Motomatu T (1989) C1q binding to human vascular smooth muscle cells mediates immune complex deposition and superoxide generation. Inflammation 13: 561–569

    Google Scholar 

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Kuwano, M., Ito, Y., Amamoto, Y. et al. A case of congenital nephrotic syndrome associated with positive C1q immunofluorescence. Pediatr Nephrol 7, 452–454 (1993). https://doi.org/10.1007/BF00857571

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  • DOI: https://doi.org/10.1007/BF00857571

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