CAJ | 학술논문

目的：探讨成人型青少年消耗病-髓质囊性综合征（JN-MCD）的临床特征、病理特点和治疗方法。方法回顾性分析1例临床资料并进行相关文献复习。结果患者行右肾根治性切除术，术后病理为间质性肾炎伴局灶性囊肿。结论成人型JN-MCD是一组起病隐匿、以肾髓质囊肿形成及慢性肾功能不全为特征的遗传性囊性肾病，该病临床表现多样，有时临床表现不典型，加强对该病的认识，可减少误诊、漏诊、误治。
목적：탐토성인형청소년소모병-수질낭성종합정（JN-MCD）적림상특정、병리특점화치료방법。방법회고성분석1례림상자료병진행상관문헌복습。결과환자행우신근치성절제술，술후병리위간질성신염반국조성낭종。결론성인형JN-MCD시일조기병은닉、이신수질낭종형성급만성신공능불전위특정적유전성낭성신병，해병림상표현다양，유시림상표현불전형，가강대해병적인식，가감소오진、루진、오치。
Objective To evaluate the clinical presentations, pathologic characteristics and the diagnosis and treatment of medullary cystic disease. Methods One case of medullary cystic disease was analyzed and the relevant literature was reviewed. A 38-year-old male with a history of no manifest clinical presentations.CT scan demonstrated a large mass about 7.6 cm×7.1 cm×8.4 cm arising from medial upper pole of right renal, asymmetrical enhancement at nephrogram. Results Right renal radical ectomy was performed, pathology showed interstitial nephritis with focal cystis.Conclusion Medullary cystic disease is an intriguing complex, its clinical onset and course are so insidious and involve such a paucity of signs and symptoms, it is easily misdiagnosed and missed diagnosing.