Clinical and Pathologic Analysis of Thin Glomerular Basement Membrane Disease in Children

Ko, Myoung Jin; Yang, Tae Jin; Kim, Young Ju; Chung, Woo Yeong

J Korean Soc Pediatr Nephrol > Volume 5(1); 2001 > Article

J Korean Soc Pediatr Nephrol 2001;5(1): 1-8.

소아 비박형 기저막신증의 임상 및 병리학적 분석

고명진, 양태진, 김영주, 정우영

¹인제대학교 의과대학 부산백병원 소아과학교실
²인제대학교 의과대학 부산백병원 소아과학교실
³인제대학교 의과대학 부산백병원 병리학교실
⁴인제대학교 의과대학 부산백병원 소아과학교실

Clinical and Pathologic Analysis of Thin Glomerular Basement Membrane Disease in Children

Myoung Jin Ko, Tae Jin Yang, Young Ju Kim, Woo Yeong Chung

¹Department of Pediatrics, Inje University, College of Medicine, Pusan Paik Hospital
²Department of Pediatrics, Inje University, College of Medicine, Pusan Paik Hospital
³Department of Pathology, Inje University, College of Medicine, Pusan Paik Hospital
⁴Department of Pediatrics, Inje University, College of Medicine, Pusan Paik Hospital

ABSTRACT

PURPOSE : Clinical manifestations and pathologic findings of thin glomerular basement membrane disease, recognized as a common underlying disease of benign, familiar and asymptomatic hematuria has not been reported systemically in Korea. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease. METHODS : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. RESULTS : The subjects were aged 9.4+/-3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1+/-2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7%). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3cases(11.5%) of gross hematuria, 23cases(88.5%9) of microscopic hematuria, and 1case(3.8%) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9+/-35.8nm. CONCLUSION : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30%. Clinical progression was good in majorities.

Key words: Thin glomerular basement membrane disease | Microscopic hematuria | Clinical and pathologic findings | Children