사구체 기저막 비박화를 보인 우리 나라 소아들의 조직학적 및 임상적 고찰
18, ii p.
비박화 사구체 신증 혈뇨;
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Purpose: Thin glomerular basement membrane nephropathy is found in a patient who has a family history of hematuria. TGBM is autosomal dominant and is known one of the commonest causes of asymptomatic hematuria in children and adults. This study was conducted to see histological and clinical features of the patients with thin glomerular basement disease, diagnosed by renal biopsy. Patients and Study Method: 150 cases were taken as study patients who were diagnosed as TGBM by renal biopsy while admitted at the department of Pediatrics of Kyungpook National University Hospital between January, 1999 and December, 2003. The followings were retrospectively observed: onset age, hematuria pattern, existence of proteinuria, the period between onset of hematuria and renal biopsy, process of diagnosis, blood pressure, laboratory findings, thickness and character of basement membrane in renal biopsy and family history. Result: The mean age at the time of diagnosis was 7.9 years. Female outnumbered male by Male vs Female of 69:77. All patients had hematuria, of whom 97 cases or 66% visited the hospital with a chief complaint of persistent microscopic hematuria. Gross hematuria accounted for 13 cases or 9% with the same number of cases accompanied by protenuria. 80 cases,55%, were found to have hematuria for the first time from a group urine test of students for screening. The average time between the onset of hematuria and renal biopsy was two years. The renal biopsy showed the thickness of basement membrane to be 186±36nm. Eight cases showed IgA deposit on immuno-fluorescent microscopy. Focal lamellation of basement membrane was found in eight cases. In family history, hematuria was shown 12 cases on father's side, 13 on mother's 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. During the study, no case was found in which renal failure or complications including hypertension developed. Conclusion: Thin glomerular basement membrane disease is one of the major causes of asymptomatic hematuria in children, which was found in an increasing number since school urinary mass screening test started in 1998. In case of progressive renal disease in family history or focal duplication in basement membrane, doubt should be raised on whether or not it is the case of Alport syndrome. Follow?ups should continue.