No abstract

We encountered a patient with autosomal dominant polycystic kidney disease (ADPKD) complicated with emphysematous polycystic renal infection (EPRI). A 44-year-old woman visited our hospital for a fever, appetite loss, and gross hematuria. Because the patient was suffering from end-stage renal disease (ESRD), she was immediately hospitalized for hemodialysis. Multiple emphysematous infected cysts were noted in the right kidney, and antibiotic therapy and three rounds of cystic drainage were performed. However, the patient did not respond to treatment. Therefore, laparoscopic right nephrectomy was performed. ADPKD with comorbid EPRI is unresponsive to conservative treatment, and we believe that nephrectomy should be considered.

キーワード： 血液透析，免疫グロブリン遊離軽鎖κ/λ比，多発性骨髄腫 〈要旨〉 症例は 47 歳男性．200X 年に初めて腎機能障害 Cr 1.2 mg/dL(尿蛋白陰性)を指摘され，2 年後には Cr 6.9 mg/dL と急速に進行し，翌年原因不明の慢性腎不全にて血液透析を導入した．導入時より心肥大を認め，2 か月後より肝 機能障害，肝左葉腫大，脾腫を認め，透析導入 1 年 6 か月後に免疫グロブリン軽鎖κ/λ比 68.23 と高値であったこ とから，骨髄穿刺を施行し多発性骨髄腫と診断した．原因不明の透析患者において，原因疾患を探す姿勢は大切で あり，今回，免疫グロブリン軽鎖κ/λ比の上昇から軽鎖型(Bence-Jones 型疑い)の多発性骨髄腫の診断に至った 症例を経験したので報告する．A case of light-chain-type multiple myeloma in a hemodialysis patient that was diagnosed based on an elevated free light chain κ/λ ratio alone

We describe a 17-year-old female with juvenile rheumatoid arthritis accompanied by Guillain-Barré syndrome (GBS) during the course of central nervous system (CNS) lupus. She initially developed CNS lupus, including headache and convulsion. A high-signal area in a magnetic resonance scan of her brain with T2-weighted images was noted, and her cerebrospinal fluid exhibited increased levels of IgG and interleukin-6. Eighteen days after the onset of CNS lupus, polyneuropathy in the lower extremities developed, and a diagnosis of GBS was made. No obvious preceding infections in the upper respiratory or gastrointestinal systems were noted before the onset of GBS, indicating that GBS might be part of the symptoms of CNS lupus.