| Thin Glomerular Basement Membrane Disease Without Glomerular IgA Deposition in Children |
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ll-Jou Sohn1, Yong-Hun Park1, Yong-Ho Ahn2, Yong-Jin Kim3, Woo-Young Chung4 |
1Department of Pediatrics, College cf medicine, Yeungnam University, Korea
2Department of Pediatrics, Catholic University of Taegu-Hyosung, Taegu, Korea
3Department of Pathology, Catholic University of Taegu-Hyosung, Taegu, Korea
4Department of Pediatrics, Inje University Pusan Paik Hospital, Pusan, Korea |
| 소아에서 사구체에 IgA 침착이 발견되지 않는 비박형 기저막 신증 |
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손일주1, 박용훈1, 안영호2, 김용진3, 정우영4 |
1영남대학교 의과대학 소아과학교실
2대구효성가톨릭대학교 의과대학 소아과학교실
3대구효성가톨릭대학교 의과대학 병리학교실
4인제의대 부산백병원 소아과학교실 |
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| Abstract |
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. |
| Key Words:
Thin glomerular basement disease, Hematuria, Family history |
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