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Steroid-sensitive nephrotic syndrome associated with Kimura disease
C Nakahara1, T Wada, J Kusakari
1Department of Pediatrics, Tsukuba University Hospital, University of Tsukuba, Ibaraki, Japan. Chico@md.tsukuba.ac.jp
Pediatric Nephrology (Berlin, Germany)
|June 29, 2000
Summary
Kimura disease, a rare condition, can cause swelling and nephrotic syndrome in children. Cyclosporine effectively treated a young patient's recurring symptoms when steroids failed, preventing further relapses.
Area of Science:
- Pediatric Nephrology
- Immunology
- Dermatology
Background:
- Kimura disease is a rare, benign inflammatory condition characterized by eosinophilic infiltration, typically affecting the head and neck region.
- Nephrotic syndrome is a kidney disorder characterized by heavy protein in the urine, low blood protein levels, and swelling.
Observation:
- An 11-year-old Japanese boy presented with recurrent episodes of left cheek swelling and nephrotic syndrome.
- Initial treatment with steroids showed temporary efficacy for both Kimura disease and nephrotic syndrome, but symptoms recurred upon steroid discontinuation.
Findings:
- Cyclosporine (CsA) was administered for the fourth episode of cheek swelling.
- The subcutaneous tumor associated with Kimura disease responded rapidly to CsA, resolving within days.
- Following CsA treatment, the patient has remained free of nephrotic syndrome relapse.
Implications:
- Cyclosporine may be an effective therapeutic option for managing Kimura disease with associated nephrotic syndrome, particularly in cases refractory to steroid therapy.
- This case highlights the potential link between Kimura disease and nephrotic syndrome and suggests a targeted immunomodulatory approach for treatment.
- Further research is warranted to explore the efficacy and long-term outcomes of cyclosporine in pediatric patients with Kimura disease and renal involvement.