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A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency

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Abstract

A 14-year-old girl presented with acute glomerulonephritis. Tests revealed hypocomplementemia and elevated Antistreptolysin-O titers, and renal biopsy revealed endocapillary and mesangial proliferative glomerulonephritis with double contours of the glomerular basement membrane (GBM). Despite methylprednisolone pulse therapy and the administration of oral prednisolone, overt proteinuria and hypocomplementemia persisted. A second renal biopsy 6 months later confirmed the initial diagnosis of dense deposit disease (DDD) based on electron-dense deposits in the GBM. C3 nephritic factor (C3NeF) and a deficiency of complement factor H (CFH) were not evident. A nephritis-associated plasmin receptor (NAPlr), nephritogenic group A streptococcal antigen, and the plasmin activity by in situ zymography were been in both the first and second biopsy specimens. The patient received combined immunomodulatory therapy with prednisolone and mizoribine, and the urinary protein decreased to a mild level at 27 months after disease onset. These findings suggest that persistent glomerular NAPlr deposition may be associated with the pathogenesis of DDD in some patients without the involvement of C3NeF or CFH mutation and that DDD patients of this type may respond to immunomodulatory treatment.

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References

  1. Nasr SH, Valeri AM, Appel GB, Sherwinter J, Stokes MB, Said SM, Markowitz GS, D’Agati VD (2009) Dense deposit disease: clinicopthologic study of 32 pediatric and adult patients. Clin J Am Soc Nephrol 4:22–32

    Article  PubMed  Google Scholar 

  2. Smith RJH, Alexander J, Balow PN, Botto M, Cassavant TL, Cook HT, de Córdoba SR, Hageman GS, Jokiranta TS, Kimberling WJ, Lambris JD, Lanning LD, Levidiotis V, Licht C, Lutz HU, Meri S, Pickering MC, Quigg RJ, Rops AL, Salant DJ, Sethi S, Thurman JM, Tully HF, Tully SP, van der Vlag J, Walker PD, Würzner R, Zipfel PF (2007) New approach to the treatment of dense deposit disease. J Am Soc Nephrol 18:2447–2456

    Article  CAS  PubMed  Google Scholar 

  3. Appel GB, Cook T, Hageman G, Jannette C, Kashgarian M, Kirschfink M, Lambris JD, Lanning L, Lutz HU, Meri S, Rose NR, Salant DJ, Sethi S, Smith RJ, Smoyer W, Tully HF, Tully SP, Walker P, Welsh M, Würzner R, Zipfel PF (2005) Membranoproliferative glomerulonphritis type II (Dense Deposit Disease): an update. J Am Soc Nephrol 16:1392–1404

    Article  PubMed  Google Scholar 

  4. West CD (2008) A hemolytic method for the measurement of nephritic factor. J Immunol Methods 335:1–7

    Article  CAS  PubMed  Google Scholar 

  5. Pickering MC, Cook HT (2008) Translational mini review series on complement Factor H: Renal disease associated with complement factor H: novel insights from humans and animals. Clin Exp Immunol 151:210–230

    Article  CAS  PubMed  Google Scholar 

  6. Zhou XJ, Silva FG (2007) Membranoproliferative glomerulonephritis type II. In: Hepstinstall’s pathology of the kidney, 6th edn. Lippincott Williams & Wilkins, Philadelphia, pp 271–287

    Google Scholar 

  7. Yosizawa N, Yamakami K, Fujino M, Oda T, Tamaru K, Matsumoto K, Sugisaki T, Boyle MD (2004) Nephritis-associated plasmin receptor and acute poststreptococcal glomerulonephritis: characterization of the antigen and associated immune response. J Am Soc Nephrol 15:1785–1793

    Article  CAS  Google Scholar 

  8. Oda T, Yamakami K, Omasu F, Suzuki S, Miura S, Sugisaki T, Yoshizawa N (2005) Glomerular plasmin-like activity to nephritis-associated plasmin receptor in acute poststreptococcal glomerulonephritis. J Am Soc Nephrol 16:247–254

    Article  CAS  PubMed  Google Scholar 

  9. Sawanobori E, Umino A, Kanai H, Matsushita K, Iwasa S, Kitamura H, Oda T, Yoshizawa N, Sugita K, Higashida K (2009) A prolonged course of group a streptococcus-associated nephritis: a mild case of dense deposit disease (DDD)? Clin Nephrol 71:703–707

    CAS  PubMed  Google Scholar 

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Acknowledgments

This work was supported by Grants-in-Aid for Scientific Research (20591277, 20591278) and the Morinaga Foundation for Health and Nutrition.

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Authors and Affiliations

  1. Department of Pediatrics, The Institute of Health Bioscience, The University of Tokushima Graduate School, Tokushima University, 18-15 Kuramotocho-3 chome, Tokushima city, Tokushima, 770-8503, Japan

    Kenichi Suga, Shuji Kondo, Sato Matsuura, Yukiko Kinoshita & Shoji Kagami

  2. Department of Medical Technology, Kobe Tokiwa University, Kobe, Japan

    Etsuko Kitano & Michiyo Hatanaka

  3. Department of Nutritional Sciences for Well-being, Kansai University of Welfare Sciences, Kashiwara, Osaka, Japan

    Hajime Kitamura

  4. Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Nagano, Japan

    Yoshihiko Hidaka

  5. Department of Nephrology, National Defense Medical College, Tokorozawa, Saitama, Japan

    Takashi Oda

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  1. Kenichi Suga
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  2. Shuji Kondo
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  3. Sato Matsuura
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  4. Yukiko Kinoshita
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  5. Etsuko Kitano
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  6. Michiyo Hatanaka
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  7. Hajime Kitamura
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  8. Yoshihiko Hidaka
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  9. Takashi Oda
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  10. Shoji Kagami
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Corresponding author

Correspondence to Kenichi Suga.

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Suga, K., Kondo, S., Matsuura, S. et al. A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency. Pediatr Nephrol 25, 1547–1550 (2010). https://doi.org/10.1007/s00467-010-1479-0

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  • DOI: https://doi.org/10.1007/s00467-010-1479-0

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