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KMID : 0367419970400070974
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Journal of Korean Pediatric Society
1997 Volume.40 No. 7 p.974 ~ p.980
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Thin Glomerular Basement Membrane Disease Without Glomerular IgA Deposition in Children
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손일주
박용훈/안영호/김용진/정우영
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Abstract
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Purpose
: Thin glomerular basement membrane (TGBM) disease is now recognized as one of leading causes of isolated microscopic hematuria, regarded as a benign condition and characterized by the presence of familial benign hematuria. But there are few studies of TGBM in Korea. The purpose of this study was to evaluate the clinical and pathological findings in TGBM according to the presence of hematuria.
Methods
: Kidney biopsies were taken in 112 patients to evaluate recurrent benign hematuna with or without intermittent gross hematuria. Among them, clinical and pathological findings were assessed in 28 patients whose main abnormality was an essentially TGBM.
Results
: The subjects were aged 9.5¡¾3.0 (2-13) years, and 11 males and 17 females: most were referred because of recurrent microscopic hematuria for 20.9 (2-69) months. A family history of benign hematuria was found in 10 patients (35.7%). There were no significant differences in serum creatinine, BUN, creatinine clearance, 24 hour urine protein and basement membrane thickness whether patients had a family history of hematuria or not. Kidney biopsy showed few changes by light microscopy. C3 and fibrinogen deposit in mesangium was found in only 3 patients. Electron microscopic findings have revealed thinning of the GBM varied from 144 to 250nm without splitting and layering of the GBM.
Conclusion
: TGBM might be a common cause ot recurrent benign hematuria with familial microscopic : hematuria in Korea. The presence of family history of hematuria did not influence the progression of the disease.
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KEYWORD
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Thin glomerular basement disease, Hematuria, Family history
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