日本新生児成育医学会雑誌　第30巻　第1号　48～53頁（2018年）

日本新生児成育医学会雑誌　30(1)：48-53；2018　

受付日：平成29．01．13

受理日：平成29．05．27

腸切除後の腸管不全関連肝障害に対し魚油由来脂肪乳剤を使用した超低出生体重児の2 例

Two Cases of Extremely Low-Birth Weight Infants Who Received Fish-Oil-Based Fat Emulsions as Treatment for Intestinal Failure-Associated Liver Disease after Enterectomy

＊1 福井愛育病院　小児科，＊2 福井県立病院　小児科，＊3 福井大学　医学部　病態制御医学講座　小児科学

＊1 Department of Pediatrics, Fukui Aiiku Hospital，＊2 Fukui prefectural hospital，＊3 Faculty of Medical Sciences, University of Fukui

田尾克生＊1＊2・大嶋勇成＊3

Katsuo TAO＊1＊2，Yuusei OHSHIMA＊3

Key Words：extremely low-birth weight infants，intestinal failure associated liver disease（IFALD）， short bowel syndrome，fish-oil-based fat emulsions

　壊死性腸炎による腸切除後，長期間の経静脈栄養中に発症した腸管不全関連肝障害（intestinal failure associated liver disease：IFALD）に対し魚油由来脂肪乳剤（オメガベン ®）が有効であった症例を経験した。症例1 は在胎24 週で出生，壊死性腸炎のため腸切除により残存小腸は45cm となった。日齢75 より胆汁うっ滞を認め，日齢121 よりオメガベン ® を65 日間使用した。症例2 は在胎24 週で出生，壊死性腸炎の術後，残存小腸は25cm となった。術後より胆汁うっ滞，体重増加不良を認め，日齢136 よりオメガベン® を開始，87 日間使用した。2 症例ともオメガベン ® 投与開始により胆汁うっ滞と肝機能障害の改善を認め，明らかな副作用は認めなかった。超低出生体重児の IFALD にオメガベン ® は有効と考えられ，本邦での使用承認と至適投与法の確立が急務といえる。

　We present the cases of two patients who developed intestinal failure-associated liver disease（IFALD）during longterm parenteral nutrition after enterectomy due to necrotizing enterocolitis. The patients were treated with parenteral fishoil- based fat emulsions（Omegaven ®）. Case 1, born at 24 weeks of gestational age, had 45 cm of residual small intestine after the enterectomy. The infant developed cholestasis at 75 days of life and was treated with Omegaven ® from 121 to 185 days of life. Case 2, born at 24 weeks of gestational age, had 25 cm of residual small intestine；this patient presented with failure to thrive and cholestasis and was treated with Omegaven ® from 136 to 222 days of life. After initiating Omegaven ®, intractable cholestasis and liver dysfunction resolved, and apparent adverse effects were not observed in both cases. Omegaven ® can be useful for treating IFALD in extremely low-birth weight infants. The approval of Omegaven ® and the establishment of a regimen to treat IFALD are anticipated.