日本新生児成育医学会雑誌　38(2)：295-302；2026　

日本新生児成育医学会雑誌　第38巻　第2号　103 ～ 110 頁（2026年）

受付日：2026．01．22

受理日：2026．03．30

先天性高インスリン血症におけるジアゾキシド治療の臨床的検討 ─導入時期および長期投与例の解析─

Diazoxide Therapy for Congenital Hyperinsulinism：Clinical Analysis of Early Initiation and Long-term Treatment

＊ 1 京都第一赤十字病院　新生児科，＊ 2 同　小児科

1 Department of Neonatology, Japanese Red Cross Kyoto Daiichi Hospital， ＊ 2 Department of Pediatrics, Japanese Red Cross Kyoto Daiichi Hospital

林　藍＊ 1・木下大介＊ 1・西村　陽＊ 1 ＊ 2

Ai HAYASHI ＊ 1，Daisuke KINOSHITA ＊ 1，Akira NISHIMURA ＊ 1 ＊ 2

Key Words：congenital hyperinsulinism，neonate，diazoxide

先天性高インスリン血症（congenital hyperinsulinism：CHI）に対してジアゾキシド（以下，DZX）は第一選択 薬であるが，水分貯留，心不全，動脈管開存症などの副作用が知られている。DZX の適応や投与開始，中止時期 の決定の一助とするため，当院に入院したCHI 症例について後方視的に検討した。CHI 104 例中20 例（19％）で DZX 投与を要した。DZX 投与群は非投与群と比較し，男児，胎児機能不全，出生体重SD が小さい例が多く，診 断時および入院中の糖投与量（glucose infusion rate：GIR）が高値で，GIR 最大値到達日齢も遅かった。DZX 投与 20 例中9 例（45％）に副作用を認めたが重篤例はなかった。1 歳以降まで投与継続を要した長期投与群は5 例（25 ％）で，長期投与群ではCHI 診断日の日齢が遅かったが，長期投与を早期に予測可能な因子は明らかでなかった。

Diazoxide is the first-line pharmacologic treatment for congenital hyperinsulinism （CHI）. However, several adverse effects have been reported in association with its use, including fluid retention, heart failure, and patent ductus arteriosus. To clarify the indications for diazoxide and the appropriate timing of initiation and discontinuation, we retrospectively reviewed the records of patients with CHI. Of the 104 patients diagnosed with CHI, 20 （19％） required diazoxide treatment. Compared with the other patients, those receiving diazoxide were more often male, had a greater incidence of fetal distress, and lower birth weights relative to gestational age. These patients also exhibited higher glucose infusion rates （GIR） at diagnosis and during hospitalization, with the maximum GIR occurring at a later postnatal age. Adverse effects were observed in nine patients （45％）, although none were severe. Five patients （25％） required long-term treatment extending beyond 1 year of age. In these five patients, CHI was diagnosed at a later age than in the other diazoxide recipients. However, we did not identify any early factors predictive of prolonged diazoxide therapy.