منابع مشابه
Dense deposit disease.
Dense deposit disease (DDD) is an orphan disease that primarily affects children and young adults without sexual predilection. Studies of its pathophysiology have shown conclusively that it is caused by fluid-phase dysregulation of the alternative pathway of complement, however the role played by genetics and autoantibodies like C3 nephritic factors must be more thoroughly defined if we are to ...
متن کاملDense deposit disease with steroid pulse therapy
Treatment of dense deposit disease (DDD) has not been established yet. Steroid therapy is considered as ineffective for DDD. We report an 8-year-old girl with DDD in whom steroid pulse therapy with subsequent oral steroid therapy led to disease remission. Serum C3 level improved in accordance with the amelioration of clinical signs. This case suggests that steroid pulse therapy with subsequent ...
متن کاملDense Deposit Disease in Korean Children: A Multicenter Clinicopathologic Study
The purpose of this study was to investigate the clinical, laboratory, and pathologic characteristics of dense deposit disease (DDD) in Korean children and to determine whether these characteristics differ between Korean and American children with DDD. In 2010, we sent a structured protocol about DDD to pediatric nephrologists throughout Korea. The data collected were compared with previously p...
متن کاملCauses of alternative pathway dysregulation in dense deposit disease.
BACKGROUND AND OBJECTIVES This study was designed to investigate the causes of alternative pathway dysregulation in a cohort of patients with dense deposit disease (DDD). DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS Thirty-two patients with biopsy-proven DDD underwent screening for C3 nephritic factors (C3Nefs), factor H autoantibodies (FHAAs), factor B autoantibodies (FBAAs), and genetic va...
متن کاملDense Deposit Disease Mimicking a Renal Small Vessel Vasculitis.
Dense deposit disease is caused by fluid-phase dysregulation of the alternative complement pathway and frequently deviates from the classic membranoproliferative pattern of injury on light microscopy. Other patterns of injury described for dense deposit disease include mesangioproliferative, acute proliferative/exudative, and crescentic GN. Regardless of the histologic pattern, C3 glomerulopath...
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ژورنال
عنوان ژورنال: Molecular Immunology
سال: 2011
ISSN: 0161-5890
DOI: 10.1016/j.molimm.2011.04.005