J Korean Soc Pediatr Nephrol > Volume 14(1); 2010 > Article
Journal of the Korean Society of Pediatric Nephrology 2010;14(1): 22-31. doi: https://doi.org/10.3339/jkspn.2010.14.1.22
Alport 증후군에서 사구체 기저막의 형태학적 변화와 사구체 상피세포의 구조 및 임상지표와의 관계
엄혜진, 홍성진, 이재승, 정현주, 김영기, 김기혁
1국민건강보험공단 일산병원 소아청소년과
2늘푸른 소아청소년과
3연세대학교 의과대학 소아과학교실
4연세대학교 의과대학 병리학교실
5미네소타대학교 의과대학 소아과학교실
6국민건강보험공단 일산병원 소아청소년과
Relationship of Glomerular Basement Membrane Alterations to Epithelial Cell Structure and Clinical Parameters in Alport Syndrome
Hye-Jin Eom, Seung-Jin Hong, Jae-Seung Lee, Hyeon-Joo Jeong, Young-Ki Kim, Kee-Hyuck Kim
1Department of Pediatrics, NHIC Ilsan Hospital
2Neulpurun Pediatric Clinic
3Departments of Pediatrics, Yonsei University College of Medicine
4Departments of Pathology, Yonsei University College of Medicine
5Department of Pediatrics, Minneapolis
6Department of Pediatrics, NHIC Ilsan Hospital
Received: March 16, 2010;  Revised: March 25, 2010.  Accepted: March 31, 2010.
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Purpose : This study was performed to evaluate the relationship between glomerular basement membrane (GBM) alterations to epithelial cell (EpC) structure and renal function in Alport Syndrome (AS) patients.
Methods : Fifteen patients diagnosed with AS (4-26yrs) were examined. The GBM in AS was categorized as : C1) normal, C2) minor alterations (widening of lamina rara interna or externa without lamina densa change), C3) nonspecific splitting of lamina densa, C4) basket-weaving pattern of lamina densa splitting. The length of each GBM portion along the epithelial side was measured on the systematically obtained electron microscopic photographs. Furthermore to obtain an objective assessment of the degree of glomerular EpC foot process change, the number of slit pores along $10;{mu}m$ of peripheral GBM in each category was obtained.
Results : The percentage of normal GBM portion (C1) correlated inversely with daily protein excretion (g/day/$m^2$, P<0.05) and sum of the percentage of abnormal GBM portion (C2+C3+C4) had direct correlation with daily protein excretion (g/day/$m^2$, P<0.05). There were no significant relationships between the percentages of other categories of GBM alterations and creatinine clearance or protein excretion. There were no significant relationships between of creatinine clearance in relation to normal GBM(C1) portion as well as that in relation to sum of the percentage of abnormal GBM portion (C2+C3+C4). GBM abnormality did not correlate with age at biopsy.
Conclusion : The extent of GBM structural abnormality is related to proteinuria in AS but the epithelial response is uniform even though the GBM ultrastructural lesions are not.
Key words: Alport syndrome | Glomerular basement membrane | Epithelial cell | Renal function

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