윌슨병 환아에서 D-penicillamine 사용 중 조기 발병한 미세변화형 신증후군 1례
Early Onset of Minimal change Nephrotic Syndrome during Treatment with D-penicillamine in a Patient with Wilson's Disease
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초록
윌슨병은 구리 대사 이상으로 간, 뇌, 각막, 신 및 적혈구에 구리가 침착되어 생기는 상염색체 열성 유전성 질환이다. D-penicillamine이 주된 치료로서 10% 정도의 환자에서 가역적인 부작용이 발생 할 수 있다. 저자는 3세된 여아에서 D- penicillamine치료 3주만에 미세변화형 신증후군이 발생하였고 투약 중단만으로 완전 관해된 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
Abstract
Wilsonʼs disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilsonʼs disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilsonʼs disease 3 weeks after initiation of D-penicillamine.
참고문헌 (17)
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