만성 신부전으로 진행된 비만 연관 사구체병증 1례
Obesity Related Glomerulopathy Progressed to Chronic Renal Failure
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Obesity-related glomerulopathy (ORG)는 단백뇨를 보이는 비만아에서 신장 조직학적 소견상 사구체 비대, 국소 분절성 사구체 경화 소견이 관찰되고 기저막 비후, 국소 간질조직 증식, 중등도의 발 돌기 소실 등을 보이는 질환으로 정의된다. 특발성 국소분절 사구체경화증과 비교하여 신증후군이 적으며 콜레스테롤 상승이 적고, 병의 진행이 느리다는 점에서 차이가 있다고 알려져 있으며, 체중감소를 통해 신장 기능이 회복될 수 있다고 알려져 있다. 본 증례의 9세된 비만아는 임상증상과 신장 조직검사에서 ORG로 진단되어 치료하였으나, 지속적인 체중 증가와 단백뇨로 말기 신부전으로 진행하여 소아 ORG 환아에서는 드문 경과를 취하여 이를 보고하는 바이다.
Abstract
Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index 35 kg/m2) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin- converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.
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