1Departments of Pediatrics, and The Institute of Kidney Disease, Yonsei University College of Medicine 2Departments of Pediatrics, and The Institute of Kidney Disease, Yonsei University College of Medicine 3Departments of Pediatrics, and The Institute of Kidney Disease, Yonsei University College of Medicine 4Departments of Pathology, and The Institute of Kidney Disease, Yonsei University College of Medicine 5Departments of Radiology, and The Institute of Kidney Disease, Yonsei University College of Medicine 6Departments of Pediatrics, and The Institute of Kidney Disease, Yonsei University College of Medicine
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ABSTRACT
Glomerulocystic kidney disease(GCKD) is a rare form of renal cystic disease defined histopathologically by containing dilated Bowman's space with variable atrophy of glomerular tufts, which may occur as sporadically or as familial cases and can be presented as a major component of heritable syndromes. It has not been recognized in Korean children but only one report of adult case has been reported having GCKD. We experienced a case of GCKD in a 10-year-10-month-old boy, who was admitted for hypertension. Abdominal ultrasonography and computed tomography revealed clustered numerous small cysts in left kidney and renal biopsy findings was consistent with the GCKD showing cystic dilatation of Bowman's space with intact glomerular structure.