Unravelling the immunopathological mechanisms of heavy chain deposition disease with implications for clinical management.

@article{Bridoux2017UnravellingTI,
  title={Unravelling the immunopathological mechanisms of heavy chain deposition disease with implications for clinical management.},
  author={Frank Bridoux and Vincent Javaugue and S{\'e}bastien Bender and Fannie Leroy and P. Aucouturier and C{\'e}line Debiais-Delpech and J M Goujon and Nathalie Quellard and Am{\'e}lie Bonaud and Marie Clavel and Patrick Trouillas and Florent Di Meo and J Gombert and J P Fermand and Arnaud Jaccard and Michel Cogn{\'e} and Guy Touchard and Christophe Sirac},
  journal={Kidney international},
  year={2017},
  volume={91 2},
  pages={423-434}
}
Randall-type heavy chain deposition disease (HCDD) is a rare disorder characterized by tissue deposition of a truncated monoclonal immunoglobulin heavy chain lacking the first constant domain. Pathophysiological mechanisms are unclear and management remains to be defined. Here we retrospectively studied 15 patients with biopsy-proven HCDD of whom 14 presented with stage 3 or higher chronic kidney disease, with nephrotic syndrome in 9. Renal lesions were characterized by nodular… CONTINUE READING
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