Kimura disease with advanced renal damage with anti-tubular basement membrane antibody

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Mehul P DixitRaymond Nagle

Abstract

Kimura disease (KD) is an autoimmune eosinophilic granulomatous disorder with generalized lymphadenopathy. A handful of pediatric patients with renal disease have been described, none of whom have been African-American (AA). We present an AA boy with KD and nephrotic syndrome (NS). Two months after stopping steroids, fever, asthma, eczema, and proteinuria recurred. His NS did not relapse but his platelet count decreased to 51,000/microl (x10(6)/l). On restarting prednisone, his platelet count normalized. A kidney biopsy revealed 23 of 37 glomeruli obsolescent and advanced damage with over 50% of cortical tissue replaced by interstitial fibrosis and chronic inflammation. Glomerular immunofluorescence was largely negative; very intense linear anti-tubular basement membrane (TBM) deposits of IgA, IgG, C3, and C4 were noted. At present, 36 months from onset, serum creatinine is 1.2 mg/dl (106 micromol/l). We present a 4-year-old AA boy with KD, NS, relapsing thrombocytopenia, and renal damage with anti-TBM antibody.

References

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Citations

Sep 20, 2007·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Mehul P DixitYihan Wang
Oct 16, 2009·Journal of Medical Case Reports·Massimiliano SorbelloMassimiliano Veroux
Mar 25, 2014·The Kaohsiung Journal of Medical Sciences·Ying-Chun HuXue-Ai Lv
Mar 23, 2011·International Journal of Nephrology·Mohamed Ashraf FoudaMohamed Sobh
Nov 4, 2009·Head & Neck·Urs D A Müller-RichterOliver Driemel
Dec 17, 2016·Internal Medicine Journal·B Alnasrallah, M Yehia
Feb 28, 2019·Diagnosis·Margaret L RushPriti Bhansali
Aug 7, 2021·Frontiers in Pediatrics·Prasanna Venkatesh RamachandranTiphanie P Vogel

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