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Title of Journal: Pediatr Nephrol

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Abbravation: Pediatric Nephrology

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Springer-Verlag

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DOI

10.1002/btpr.1566

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1432-198X

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A case of dense deposit disease associated with a

Authors: Kenichi Suga Shuji Kondo Sato Matsuura Yukiko Kinoshita Etsuko Kitano Michiyo Hatanaka Hajime Kitamura Yoshihiko Hidaka Takashi Oda Shoji Kagami
Publish Date: 2010/03/11
Volume: 25, Issue: 8, Pages: 1547-1550
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Abstract

A 14yearold girl presented with acute glomerulonephritis Tests revealed hypocomplementemia and elevated AntistreptolysinO 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 electrondense deposits in the GBM C3 nephritic factor C3NeF and a deficiency of complement factor H CFH were not evident A nephritisassociated 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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