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      Nephrotic syndrome associated with Kimura disease.

      Pediatric Nephrology (Berlin, Germany)
      Adolescent, Angiolymphoid Hyperplasia with Eosinophilia, complications, drug therapy, pathology, Anti-Inflammatory Agents, therapeutic use, Antineoplastic Agents, Alkylating, Chlorambucil, Drug Resistance, Fatal Outcome, Glomerulonephritis, Membranoproliferative, Humans, Male, Nephrotic Syndrome, Prednisone, Steroids

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          Abstract

          Kimura disease presents as benign subcutaneous swelling predominantly around the head and neck region. It has a high incidence of renal involvement. However, the pathogenesis of this association remains elusive. Only 2 pediatric cases and 11 adult cases of Kimura disease with renal involvement have been reported in the literature. In recent years many immunopathogenetic features suggesting an underlying T-cell and related cytokine defect have been noted in Kimura disease. We describe a unique case of an Asian boy who presented with nephrotic syndrome resistant to steroid and cytotoxic therapy, and 5 years later developed cervical lymphadenopathy consistent with Kimura disease. We also review the literature, summarizing the presentation, differential diagnosis, incidence of renal disease, prognosis, immunopathogenetic features, and therapy.

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