title: Eculizumab in dense-deposit disease after renal transplantation.
abstract: BACKGROUND
  Dense-deposit disease (DDD) is a rare glomerulopathy characterized by electron-dense deposits in the glomerular basement membrane. About 50 % of patients with DDD progress to end-stage kidney disease and require dialysis within 10 years of diagnosis, and the disease often recurs after renal transplantation.
  
  
  METHODS
  We describe a 14-year-old girl with recurrent DDD in her transplanted kidney. Clinical onset was at 8 years of age, when steroid-resistant nephrotic syndrome was diagnosed with microhematuria, severe hypocomplementemia and normal kidney function. Although remission was initially observed after several plasma exchanges, nephrotic proteinuria returned and kidney function further declined 1 year later. The patient received a living-related kidney transplant. Initial allograft function was good, but proteinuria reappeared 3 months after transplantation, accompanied by a slight deterioration in kidney function. After histological confirmation of DDD recurrence and subsequent management with plasmapheresis, the patient was treated for 30 months with eculizumab, a humanized monoclonal antibody that binds to C5 complement protein. This intervention proved effective and resulted in complement inhibition, sustained remission of proteinuria and preservation of renal function. A graft biopsy 6 months later showed no progression of the renal lesions.
  
  
  CONCLUSIONS
  Early clinical and histological recurrence of DDD in the transplanted kidney in this 14-year-old patient was treated for 30 months with eculizumab. The patient remains asymptomatic, has no proteinuria and her kidney function is intact.
authors.affiliation: Pediatric Nephrology Unit, Hospital Universitario Virgen del Rocío, Manuel Siurot s/n, 41013, Seville, Spain, asanchezmoreno@yahoo.es.
authors.forename: Ana
authors.initials: A
authors.lastname: Sánchez-Moreno
authors.forename: Francisco
authors.initials: F
authors.lastname: De la Cerda
authors.forename: Rocío
authors.initials: R
authors.lastname: Cabrera
authors.forename: Julia
authors.initials: J
authors.lastname: Fijo
authors.forename: Margarita
authors.initials: M
authors.lastname: López-Trascasa
authors.forename: Rafael
authors.initials: R
authors.lastname: Bedoya
authors.forename: Santiago
authors.initials: S
authors.lastname: Rodríguez de Córdoba
authors.forename: Patricia
authors.initials: P
authors.lastname: Ybot-González
mesh_terms: D000293:Adolescent; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy*; D006801:Humans; D016030:Kidney Transplantation; D012008:Recurrence
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab