生体腎移植後に不完全型中枢性尿崩症と診断された1例

Abstract

31歳男。腎不全末期の状態で緊急入院し, 同日血液透析導入となり, 腎生検よりnephrosclerosisであった。約8ヵ月後に父をドナーとする生体腎移植を施行し, 急性尿細管壊死は認めず, 血流直後より初尿を認め, 免疫抑制剤に拒絶反応もなく経過した。術後3週間頃より10000ml前後の1日尿量が続き, 体重減少, 皮膚乾燥, 血清クレアチニン値の上昇を認め, 尿崩症を疑った。MRIでは下垂体の形態異常は認めず, T1強調画像で下垂体後葉の高信号は保たれ, 尿検査で低張尿は認めなかった。高濃度食塩水テストを施行してのバソプレシン分泌の変化の検討では, 血漿浸透圧は上昇したが血漿バソプレシン濃度の上昇は鈍く, 正常バソプレシン分泌範囲を下回り, 不完全型中枢性尿崩症と診断した。デスモプレシン点鼻の開始により尿量は減少し, 体重, 血清クレアニチン値も安定した。現在もデスモプレシンの点鼻投与を継続し, 経過順調であった。

A 31-year-old man was sent to hospital for urgent treatment. He was in the terminal state of chronic renal failure, and was placed under hemodialysis immediately. Proteinuria and hypertension had been notified since adolescence, had been left untreated, and there was no record of his conditions, was. Living kidney transplantation was conducted 8 months later. The donor was his father. After the operation, rejection was not recognized, but urine volume per day was not reduced and maintained the level around 10.000 ml. At the same time, the decrease of body weight and the rise in the serum creatinine concentration were noted. The results of magnetic resonance imaging and the hypertonic saline test (Hickey Hare Test) have formed diagnosis of incomplete diabetes insipidus. Immediately after the administration of desmopressin (rhinenchysis), the decrease of urine volume was recognized, and the body weight and serum creatinine concentration became stable.