日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 33(3): 234-238 (2017)
doi:10.9794/jspccs.33.234

症例報告Case Report

Treprostinil持続皮下投与の導入と継続に成功した精神発達遅滞を有する小児特発性肺動脈性肺高血圧症例Pediatric Idiopathic Pulmonary Arterial Hypertension with Mental Retardation Successfully Treated with the Introduction and Maintenance of a Continuous Subcutaneous Infusion of Treprostinil

1東京医科歯科大学医学部附属病院小児科Department of Pediatrics, Tokyo Medical and Dental University ◇ Tokyo, Japan

2総合病院土浦協同病院小児科Department of Pediatrics, Tsuchiura Kyodo General Hospital ◇ Ibaraki, Japan

受付日:2016年11月15日Received: November 15, 2016
受理日:2017年4月10日Accepted: April 10, 2017
発行日:2017年5月1日Published: May 1, 2017
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Treprostinilは,肺動脈性肺高血圧症に対して持続静脈内投与に加えて持続皮下投与で投与が可能なプロスタグランジンI2誘導体製剤で,日本国内でも成人症例の集積がある.今回,Treprostinil持続皮下投与を国内で初めて小児に導入し成功した.患児は7歳時に特発性肺動脈性肺高血圧症(IPAH)と診断され,内服3剤の併用と在宅酸素療法が行われたが改善が不十分であった.精神発達遅滞のため中心静脈カテーテル管理が困難と判断され,Treprostinil持続皮下投与を導入し,半年の経過観察中にWHO機能分類および心臓超音波検査所見が改善した.Treprostinil持続皮下投与は小児IPAHにも有効性が示唆され,Epoprostenol持続静脈内投与の導入・継続が難しい患児やEpoprostenol持続静脈内投与からの離脱を考えている患児に良い適応があると思われる.

Treprostinil, a prostacyclin analogue, is administered subcutaneously and intravenously as a treatment for pulmonary arterial hypertension in Japanese adults. To the best of our knowledge, this is the first report to describe the successful subcutaneous infusion of treprostinil in a pediatric Japanese patient. The patient was diagnosed with idiopathic pulmonary arterial hypertension (IPAH) at the age of 7 years. Initial treatments included a combination therapy of three oral drugs and home oxygen therapy. When we determined that the treatment was insufficient, we decided to add subcutaneous treprostinil because administering intravenous epoprostenol would have been difficult due to the patient being mentally retarded. World Health Organization functional classification and echocardiographic findings showed improvement 6 months after the initiation of treprostinil. Therefore, we suggested that subcutaneous treprostinil is effective for children with IPAH and suggest that this drug is used for pediatric patients who cannot tolerate intravenous epoprostenol, or who can withdraw from epoprostenol.

Key words: treprostinil; continuous subcutaneous infusion; pediatric idiopathic pulmonary arterial hypertension

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