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Journal of the Korean Society of Pediatric Nephrology 2005;9(1): 31-37.
사구체 기저막 비박화를 보인 소아들의 조직학적 및 임상적 고찰
김영철, 이동원, 조민현, 곽정식, 고철우
1경북대학교 의과대학 소아과학교실
2경북대학교 의과대학 소아과학교실
3경북대학교 의과대학 소아과학교실
4경북대학교 의과대학 병리학교실
5경북대학교 의과대학 병리학교실
A Histological and Clinical Study of the Children with Thin Glomerular Basement Membrane
Young-Chol Kim, Dong-Won Lee, Min-Hyun Cho, Jung-Sik Kwak, Cheol-Woo Ko
1Departments of Pediatrics, Kyungpook National University
2Departments of Pediatrics, Kyungpook National University
3Departments of Pediatrics, Kyungpook National University
4Departments of Pathology, Kyungpook National University
5Departments of Pathology, Kyungpook National University
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ABSTRACT
Puruose : Thin glomerular basement membrane disease(TGBMD) is found in patients with family history of hematuria. TGBMD is autosomal dominant and is known to be one of the commonest causes of asymptomatic hematuria. This study was conducted to evaluate the histological and clinical features of patients with TGBMD.
Methods : 150 cases diagnosed with TGBMD by renal biopsy while admitted in the department of pediatrics, Kyungpook National University Hospital between January 1999 and December 2003 comprised the study group. The following parameters were retrospectively anaIyzed age of onset, hematuria pattern, existence of proteinuria, process of diagnosis, laboratory findings, thickness and character of basement membrane and family history.
Results : The mean age at the time of diagnosis was 7.9 years. The male to female ratio was 65:77. 94 patients or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9%. 78 cases(55%) were found to have hematuria for the first time from a routine school urinalysis screening. The renal biopsy showed the thickness of basement membrane to be 186$pm$36 nm. Focal lamellation of the basement membrane was found in eight cases. In the family history, hematuria was shown in 10 cases on the Paternal side, 13 on The maternal side and none on both sides. In seven cases, hematuria was shown among siblings. No significant differences were found among the laboratory test results which were conducted at an average interval of fifteen months.
Conclusion : TGBMD is one of the major causes of asymptomatic hematuria in children, which was diagnosed in increasing numbers since school urinary mass screening test started in 1998. In cases with familial progressive renal disease or focal duplication in the basement membrane Alport syndrome should be considered.
Key words: Thin glomerular basement membrane disease | Asymptomatic hematuria | Alport syndrome
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