Kimura disease: a case report and review of the Chinese literature.
BACKGROUND
Kimura disease, often accompanied by nephrotic syndrome, is a rare, chronic inflammatory disorder of unknown cause. In this report, the clinical and histopathological characteristics of 20 Chinese patients with Kimura disease-associated nephrotic syndrome were retrospectively evaluated.


METHODS
We report a case of Kimura disease that was diagnosed recently in our ward, with steroid-responsive but recurrent minimal-change nephrotic syndrome. Meanwhile, we also used three powerful Chinese journal search engines (Cqvip.com, Wanfang.data and ScienceChina) to search the cases reported in Chinese from 1984 to 2007.


RESULTS
The nephrotic syndrome of our patient occurred 20 months after the onset of Kimura disease. Renal biopsy revealed minimal-change lesions. The patient was responsive to the steroid, but proteinuria recurred. In most of the 19 other cases, the onset of nephrotic syndrome occurred after subcutaneous masses. Renal biopsy in 13 cases showed mesangial proliferative glomerulonephritis in 9, minimal change disease in 2 and membrane nephropathy in 2 cases. Serum creatinine levels were elevated in 5 patients.


CONCLUSIONS
Normally, Kimura disease-associated nephrotic syndrome patients are sensitive to prednisone therapy but are likely to relapse. In patients with recurrent nephrotic syndrome, renal insufficiency is not uncommon.
Wang
D Y
DY
The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China.
Mao
J H
JH
Zhang
Y
Y
Gu
W Z
WZ
Zhao
S A
SA
Chen
Y F
YF
Liu
A M
AM
D000293:Adolescent; D000328:Adult; D000796:Angiolymphoid Hyperplasia with Eosinophilia / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D002681:China; D003404:Creatinine / Q000097:blood; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000209:etiology / Q000473:pathology; D015433:Glomerulonephritis, Membranous / Q000209:etiology / Q000473:pathology; D006801:Humans; D007668:Kidney / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000209:etiology* / Q000473:pathology; D009404:Nephrotic Syndrome / Q000209:etiology*; D011241:Prednisone / Q000627:therapeutic use; D011507:Proteinuria / Q000209:etiology; D012008:Recurrence; D055815:Young Adult
D003404:Creatinine; D011241:Prednisone