両側腎に多数の腎囊胞を認めるGitelman症候群に腹膜透析を導入した1例  [in Japanese] A case of Gitelman syndrome involving bilateral progressive renal cysts that necessitated the initiation of peritoneal dialysis  [in Japanese]

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Abstract

<p>45歳女性. 見当識障害を主訴に当院を受診した. 38歳時にGitelman症候群と診断されている. 38歳時はCr 1.68mg/dLであったが徐々に腎機能が悪化し, 入院時はCr 5.59mg/dLに上昇していた. 入院後, 意識レベルが低下し, 全身痙攣も出現したため, CHDFを開始した. CHDF後に意識レベルがすみやかに改善したことから, 腎不全による代謝性脳症と診断した. 血圧低値であり, 就労継続も考慮し, 腹膜透析を導入した. また, 38歳時よりCTで両側腎に囊胞を認め, 年々, 囊胞数, 腎容積とも増大した. これまでGitelman症候群に腎囊胞が多発したとする報告はなく, 本症例はGitelman症候群に囊胞性腎疾患を合併した最初の報告である. 腎障害, 囊胞形成に関して低K血症の関与が疑われた.</p>

<p>A 45-year-old female was admitted to our hospital with disorientation. She had been diagnosed with Gitelman syndrome at the age of 38, when her serum creatinine level was 1.68 mg/dL. However, her renal function gradually declined, and her creatinine level had increased to 5.59 mg/dL by the time of her admission. As her consciousness was impaired, and she began to have systemic convulsions after admission, continuous hemodiafiltration (CHDF) was started. She rapidly regained consciousness after the initiation of CHDF and was finally diagnosed with metabolic encephalopathy. Her renal replacement therapy was changed from CHDF to nocturnal tidal peritoneal dialysis because of sustained hypotension and the patient's wish to continue working. At the age of 38, bilateral renal cysts had been detected on computed tomography, and the number and volume of renal cysts gradually increased. To the best of our knowledge, this is the first report about a case of Gitelman syndrome involving progressive bilateral renal cysts. Chronic hypokalemia might contribute to the progression of renal cysts and renal insufficiency.</p>

Journal

  • Nihon Toseki Igakkai Zasshi

    Nihon Toseki Igakkai Zasshi 50(9), 581-586, 2017

    The Japanese Society for Dialysis Therapy

Codes

  • NII Article ID (NAID)
    130006105810
  • NII NACSIS-CAT ID (NCID)
    AN10432053
  • Text Lang
    JPN
  • ISSN
    1340-3451
  • NDL Article ID
    028576389
  • NDL Call No.
    Z19-1413
  • Data Source
    NDL  J-STAGE 
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