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양성 가족성 혈뇨의 일가족 2명 |
장병식 , 김병길 , 성순희 |
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Abstract |
Benign familial hematuria is a hereditary disease of autosomal dominant which is a menifested by continu- ous hematuria of unknown origin and normal renal function found in more than two member in a family. In 1963, Roger et al discovered a familial tendenay in some patients of benign familial hematuria. In 1978, Yoshikawa et al discovered that the glomerular base ment membrane is thinner than normal seen by electron microscope of renal biopsy specimens in 50 children of benign familial hematuria. He reported that hematuria was from the disruption of the glomerular basement membrane. We are reporting cases of benign familial hematuria experienced in two children of the same family whose renal function was normal and had a continuous hematuria for several months. Their renal biopsy speci- mens showed no abnormalities in light microscopy and immunofluorescence microscopy but a specific finding was seen in electron microscopy. |
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