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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Received: 24 June 1999 / Revised: 1 November 1999 / Accepted: 2 November 1999
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Rajpoot, D., Pahl, M. & Clark, J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 14, 486–488 (2000). https://doi.org/10.1007/s004670050799
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DOI: https://doi.org/10.1007/s004670050799