title: Childhood-onset dense deposit disease: a rare cause of proteinuria.
abstract: BACKGROUND
  Dense deposit disease (DDD) is a rare renal disease related to the dysregulation of the alternative pathway of the complement cascade, caused by several factors including the presence of an autoantibody to C3 nephritic factor, mutations in factor H and autoantibodies to this protein. DDD is characterized by C3 accumulation with absent or scanty immunoglobulin deposition.
  
  
  METHODS
  Herein we report the case of a child with benign course of DDD, who presented with moderate proteinuria and lack of clinical symptoms without immunosuppressive treatment. Laboratory testing revealed moderate proteinuria, normal serum creatinine, total protein, and albumin levels, but significantly decreased serum C3 level. The results of renal biopsy were consistent with DDD. Genetic analysis revealed that the patient carried one copy of the H402 risk allele of factor H. The level of proteinuria did not change during the follow-up period and no nephrotic syndrome signs occurred. Renal function was stable.
  
  
  CONCLUSIONS
  In conclusion, a program of urine screening for asymptomatic proteinuria and hematuria to detect children with kidney disease before they experience loss of kidney functions should be considered. Children diagnosed with DDD should have the opportunity to get treatment early on and to be followed very closely.
authors: Department of Pediatrics and Nephrology, Medical University of Białystok, Waszyngtona 17, 15-274, Białystok, Poland.
authors: K
authors: K
authors: Taranta-Janusz
authors: A
authors: A
authors: Wasilewska
authors: B
authors: B
authors: Szynaka
mesh_terms: D017668:Age of Onset; D058070:Asymptomatic Diseases; D001323:Autoantibodies / Q000097:blood; D002648:Child; D003178:Complement C3 Nephritic Factor / Q000378:metabolism; D017242:Complement Factor H / Q000235:genetics*; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D007678:Kidney Glomerulus / Q000378:metabolism; D011507:Proteinuria / Q000209:etiology*
chemical_list: D001323:Autoantibodies; D003178:Complement C3 Nephritic Factor; D007166:Immunosuppressive Agents; D017242:Complement Factor H