臨牀透析 Vol.30 No.9(5)


特集名 原疾患と合併症に合わせた透析導入と透析維持
題名 多発性囊胞腎
発刊年月 2014年 08月
著者 諏訪部 達也 虎の門病院腎センター
著者 乳原 善文 虎の門病院腎センター
【 要旨 】 常染色体優性多発性囊胞腎（ADPKD）は，もっとも多い遺伝性腎疾患である．一般的に30～50歳にかけて囊胞の拡大を認め，60歳までに約半数が末期腎不全に移行する．透析導入後にも腎腫大が進行し，著明な腹部膨満をきたすことがある．また，肝囊胞，脳動脈瘤などを合併することもある．本邦でのADPKDを原疾患とする透析患者は，全体の3.4％，透析導入平均年齢は62.5歳である．腎動脈塞栓術（TAE）は，ADPKD患者の著明な腫大腎の容積縮小に有効であると考えられる．肝TAEは，腎TAEに比べ縮小効果は限定的である．囊胞感染症は，しばしば発生する合併症で，難治化し再発を繰り返すことがある．脳動脈瘤は致死的な合併症であり，定期的なスクリーニング検査が重要である．
Theme The initiation and maintenance of dialysis in terms of original kidney diseases and complications
Title Polycystic kidney disease
Author Tatsuya Suwabe Nephrology Center, Toranomon Hospital
Author Yoshifumi Ubara Nephrology Center, Toranomon Hospital
[ Summary ] Autosomal dominant polycystic kidney disease (ADPKD) is a very common renal disorder. Generally speaking, renal cysts in ADPKD patints continue to enlarge from their thirties to their fifties. Approximately half advance to renal failure by 60 years of age. These renal cysts continue to enlarge even after initiation of dialysis. Some patients advance to have marked abdominal distention. Hepatic cysts are aother frequent complication associated with ADPKD. Dialysis patients with conditions caused by ADPKD account for 3.4 % of all dialysis patients in Japan and average age at initial dialysis is 62.5 years. Renal transcatheter arterial embolization (TAE) may be effective for reducing enlarged kidney volume. Compared to renal TAE, the effectiveness of hepatic TAE for reducing liver volume is limited. Cyst induced infections are a frequent and serious complication associated with ADPKD and are often refractory. Cerebral aneurythms are one fatal complication connected with ADPKD, so regular brain MRAs are important.
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