常染色体優性多発性嚢胞腎の診療の取り組み

Abstract

2014 年トルバプタンが初めて治療薬として常染色体優性多発性嚢胞腎に適応承認された．これを受けて，2015 年3 月に多 発性嚢胞腎専門外来を開設したところ，他施設や他科から多数の紹介患者を得た．個々の症例ごとに，重大な合併症の一つで ある脳動脈瘤のスクリーニングを行うとともに，トルバプタンの適応を検討した．これまでに26 例にトルバプタンを導入し， 腎容積増大が抑制され，多くの例で腎機能低下抑制効果も認められた．病診連携にも取り組み，一部の患者については通院の 利便性もふまえ，トルバプタンの維持投与と毎月の血液検査を近隣の診療所に依頼している．今後，より一層の医療連携をは かり，常染色体優性多発性嚢胞腎の早期診療および治療普及につとめたい．

In 2014, tolvaptan was first approved for the treatment of autosomal dominant polycystic kidney disease. We therefore opened our Polycystic Kidney Disease Outpatient Department in March 2015 and have received many referrals from other medical facilities and departments. In each case, we screened for any cerebral aneurysm, which is one of the major complications, and examined the indication of tolvaptan. Tolvaptan was introduced in 26 cases and the increase in kidney volume was prevented. In many cases, the deterioration of kidney function was also suppressed. For the convenience of some patients, we have collaborated with several local clinics and requested maintenance administration of tolvaptan and monthly blood tests from them. We will work on further medical cooperation to promote early diagnosis and treatment of autosomal dominant polycystic kidney disease.