{"id":1055,"date":"2025-12-18T12:09:32","date_gmt":"2025-12-18T03:09:32","guid":{"rendered":"https:\/\/www.ipsen.com\/japan\/?post_type=press_release&#038;p=1055"},"modified":"2025-12-18T12:09:34","modified_gmt":"2025-12-18T03:09:34","slug":"%e3%82%a4%e3%83%97%e3%82%bb%e3%83%b3%e3%80%81%e5%b8%8c%e5%b0%91%e7%96%be%e6%82%a3%e3%80%8c%e9%80%b2%e8%a1%8c%e6%80%a7%e5%ae%b6%e6%97%8f%e6%80%a7%e8%82%9d%e5%86%85%e8%83%86%e6%b1%81%e3%81%86%e3%81%a3","status":"publish","type":"press_release","link":"https:\/\/www.ipsen.com\/japan\/press-releases\/%e3%82%a4%e3%83%97%e3%82%bb%e3%83%b3%e3%80%81%e5%b8%8c%e5%b0%91%e7%96%be%e6%82%a3%e3%80%8c%e9%80%b2%e8%a1%8c%e6%80%a7%e5%ae%b6%e6%97%8f%e6%80%a7%e8%82%9d%e5%86%85%e8%83%86%e6%b1%81%e3%81%86%e3%81%a3\/","title":{"rendered":"\u30a4\u30d7\u30bb\u30f3\u3001\u5e0c\u5c11\u75be\u60a3\u300c\u9032\u884c\u6027\u5bb6\u65cf\u6027\u809d\u5185\u80c6\u6c41\u3046\u3063\u6ede\u75c7\u306b\u4f34\u3046\u305d\u3046\u75d2\u300d\u6cbb\u7642\u85ac\u30d3\u30eb\u30d9\u30a4\u00ae\uff08\u30aa\u30c7\u30d3\u30ad\u30b7\u30d0\u30c3\u30c8\uff09\u306e\u65e5\u672c\u306b\u304a\u3051\u308b\u8ca9\u58f2\u958b\u59cb\u3092\u767a\u8868"},"content":{"rendered":"\n<p><\/p>\n\n\n\n<p>\u30d3\u30eb\u30d9\u30a4<sup>\u00ae<\/sup>\u306f\u3001\u9032\u884c\u6027\u5bb6\u65cf\u6027\u809d\u5185\u80c6\u6c41\u3046\u3063\u6ede\u75c7\uff08Progressive Familial Intrahepatic Cholestasis :PFIC\uff09\u306b\u4f34\u3046\u305d\u3046\u75d2\u306e\u6cbb\u7642\u85ac\u3068\u3057\u3066\u627f\u8a8d\u3055\u308c\u305fIBAT\u963b\u5bb3\u85ac\u3067\u3042\u308a\u3001\u4f53\u91cd\uff15\u338f\u4ee5\u4e0a\u306e\u4e73\u5e7c\u5150\u304a\u3088\u3073\u5c0f\u5150\u3001\u6210\u4eba\u306b\u3068\u3063\u3066\u306e\u65b0\u305f\u306a\u6cbb\u7642\u9078\u629e\u80a2\u3092\u63d0\u4f9b\u3057\u307e\u3059\u3002<\/p>\n\n\n\n<p class=\"has-vivid-cyan-blue-color has-text-color has-link-color wp-elements-4e988a4d278ef9db2d516d8c8a1f1d1e\"><strong>\u65e5\u672c\u3001\u6771\u4eac\u20152025 \u5e7412 \u670818 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class=\"has-vivid-cyan-blue-color has-text-color has-link-color wp-elements-0ada247f9a29643d8883ca1a3c67f7a6\"><strong>PFIC<\/strong><strong>\u306b\u3064\u3044\u3066<\/strong><\/p>\n\n\n\n<p>\u9032\u884c\u6027\u5bb6\u65cf\u6027\u809d\u5185\u80c6\u6c41\u3046\u3063\u6ede\u75c7\uff08PFIC\uff09\u306f\u3001\u809d\u81d3\u5185\u306b\u80c6\u6c41\u9178\u304c\u84c4\u7a4d\u3057\u3001\u809d\u969c\u5bb3\u3092\u5f15\u304d\u8d77\u3053\u3057\u3001\u6700\u7d42\u7684\u306b\u809d\u4e0d\u5168\u306b\u81f3\u308b\u53ef\u80fd\u6027\u306e\u3042\u308b\u7a00\u306a\u907a\u4f1d\u6027\u75be\u60a3\u306e\u7dcf\u79f0\u3067\u3059\u3002PFIC\u306f\u4e3b\u8981\u306a\u75c5\u578b\u3068\u3057\u3066PFIC1\u578b\u30012\u578b\u30013\u578b\u304c\u77e5\u3089\u308c\u3066\u3044\u307e\u3059\u3002PFIC1\u578b\u304a\u3088\u3073PFIC2\u578b\u306f\u901a\u5e38\u3001\u4e73\u5150\u671f\u307e\u305f\u306f\u5e7c\u5150\u671f\u3068\u3044\u3063\u305f\u751f\u5f8c\u65e9\u671f\u306b\u767a\u75c7\u3057\u307e\u3059\u3002\u4e00\u65b9\u3001PFIC3\u578b\u306f\u4e73\u5150\u671f\u304b\u3089\u601d\u6625\u671f\u307e\u3067\u306e\u9593\u306b\u767a\u75c7\u3059\u308b\u53ef\u80fd\u6027\u304c\u3042\u308a\u307e\u3059\u3002<sup>2 <\/sup>PFIC \u306e\u767a\u75c7\u306b\u7537\u5973\u5dee\u306f\u306a\u304f\u3001\u51fa\u751f5 \u4e07\u4ef6\u306b1 \u4f8b\u304b\u308910 \u4e07\u4ef6\u306b1 \u4f8b\u306e\u5272\u5408\u3067\u767a\u751f\u3057\u307e\u3059\u3002<sup>3<\/sup> PFIC\u306f\u6210\u4eba\u671f\u306b\u767a\u75c7\u3059\u308b\u3053\u3068\u3082\u3042\u308a\u307e\u3059\u304c\u3001\u901a\u5e38\u306f\u4e73\u5e7c\u5150\u671f\u306b\u767a\u75c7\u3057\u3066\u6700\u3082\u9032\u884c\u304c\u901f\u304f\u91cd\u7be4\u306a\u7d4c\u904e\u3092\u305f\u3069\u308a\u307e\u3059\u3002PFIC\u306f\u3001\u91cd\u5ea6\u306e\u304b\u3086\u307f\uff08\u305d\u3046\u75d2\uff09\u3092\u306f\u3058\u3081\u3068\u3059\u308b\u8870\u5f31\u6027\u306e\u75c7\u72b6\u3092\u4f34\u3044\u3001\u63bb\u304d\u3080\u3057\u308a\u306b\u3088\u308b\u76ae\u819a\u640d\u50b7\u3001\u7761\u7720\u969c\u5bb3\u3001\u795e\u7d4c\u904e\u654f\u3001\u6ce8\u610f\u529b\u4f4e\u4e0b\u3001\u5b66\u696d\u6210\u7e3e\u306e\u4e0d\u632f\u306a\u3069\u3001\u60a3\u8005\u3055\u3093\u306e\u65e5\u5e38\u751f\u6d3b\u306b\u5f71\u97ff\u3092\u53ca\u307c\u3057\u307e\u3059\u3002PFIC1\u578b\u30012\u578b\u3001\u304a\u3088\u30733\u578b\u306f\u3001PFIC\u306e\u4e2d\u3067\u3088\u304f\u77e5\u3089\u308c\u3066\u304a\u308a\u3001\u6700\u3082\u7814\u7a76\u304c\u9032\u3093\u3067\u3044\u308b\u75c5\u578b\u3067\u3059\u304c\u3001PFIC\u907a\u4f1d\u5b50\u306f\u3055\u307e\u3056\u307e\u306a\u809d\u75be\u60a3\u306e\u767a\u75c7\u306b\u95a2\u4e0e\u3057\u3066\u3044\u308b\u53ef\u80fd\u6027\u304c\u3042\u308a\u3001\u8fd1\u5e74\u3067\u306fPFIC\u306e\u75c5\u578b\u306f13\u578b\u307e\u3067\u5831\u544a\u3055\u308c\u3066\u304a\u308a\u307e\u3059\u3002<sup>4,5<\/sup><\/p>\n\n\n\n<p 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2024\u5e74\u306b\u306f\u3001Kayfanda\uff08\u30aa\u30c7\u30d3\u30ad\u30b7\u30d0\u30c3\u30c8\uff09\u306e\u88fd\u54c1\u540d\u3067\u3001\u751f\u5f8c6\u304b\u6708\u4ee5\u4e0a\u306e\u30a2\u30e9\u30b8\u30fc\u30eb\u75c7\u5019\u7fa4\uff08ALGS\uff09\u60a3\u8005\u3055\u3093\u306b\u304a\u3051\u308b\u80c6\u6c41\u3046\u3063\u6ede\u6027\u305d\u3046\u75d2\u306e\u6cbb\u7642\u85ac\u3068\u3057\u3066\u3001\u4f8b\u5916\u7684\u306a\u72b6\u6cc1\u4e0b\u3067\u306e\u627f\u8a8d\u304c\u4ed8\u4e0e\u3055\u308c\u307e\u3057\u305f\u3002<\/p>\n\n\n\n<p>\u7c73\u56fd\u6dfb\u4ed8\u6587\u66f8\u306e\u8a73\u7d30\u306f\u3053\u3061\u3089\u3092\u3054\u89a7\u304f\u3060\u3055\u3044\uff1a<a href=\"https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2021\/215498s000lbl.pdf\">label (fda.gov)<\/a><\/p>\n\n\n\n<p>\u6b27\u5dde\u88fd\u54c1\u6982\u8981\uff08SmPC\uff09\u306e\u8a73\u7d30\u306f\u3053\u3061\u3089\u3092\u3054\u89a7\u304f\u3060\u3055\u3044\uff1a<a href=\"https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/bylvay-epar-product-information_en.pdf\">Bylvay, INN-odevixibat (europa.eu)<\/a><\/p>\n\n\n\n<p class=\"has-vivid-cyan-blue-color has-text-color has-link-color wp-elements-369e01ec94ebb88ab82861998f03c8c0\"><strong>PEDFIC<\/strong><strong>\u8a66\u9a13\u306b\u3064\u3044\u3066<\/strong><\/p>\n\n\n\n<p>PEDFIC\u306f\u3001PFIC\u3092\u5bfe\u8c61\u3068\u3057\u305f\u56fd\u969b\u7684\u306a\u7b2cIII\u76f8\u8a66\u9a13\u3067\u3059\u3002PEDFIC1\u306f\u3001\u30e9\u30f3\u30c0\u30e0\u5316\uff081:1:1\uff09\u3001\u4e8c\u91cd\u76f2\u691c\u3001\u30d7\u30e9\u30bb\u30dc\u5bfe\u7167\u306e24\u9031\u9593\u8a66\u9a13\u3067\u3042\u308a\u3001PFIC \u306e\u5c0f\u5150\u60a3\u8005\u3055\u3093\u306b\u304a\u3051\u308b2\u7a2e\u985e\u306e\u30d3\u30eb\u30d9\u30a4\u6295\u4e0e\u91cf\uff0840\u03bcg\/kg\u307e\u305f\u306f120\u03bcg\/kg\uff09\u306e\u305d\u3046\u75d2\u306a\u3089\u3073\u306b\u8840\u6e05\u80c6\u6c41\u9178\u5024\u306e\u4f4e\u6e1b\u52b9\u679c\u304a\u3088\u3073\u5fcd\u5bb9\u6027\u3092\u8a55\u4fa1\u3057\u307e\u3057\u305f\u3002\u8a66\u9a13\u7d50\u679c\u306fThe Lancet Gastroenterology &amp; Hepatology\u8a8c\u306b\u63b2\u8f09\u3055\u308c\u307e\u3057\u305f\u3002<sup>6<\/sup><\/p>\n\n\n\n<p>\u7b2cIII\u76f8PEDFIC1\u8a66\u9a13\u306e\u30c7\u30fc\u30bf\u306f\u3001\u30d3\u30eb\u30d9\u30a4\u304cPFIC\u306b\u5bfe\u3059\u308b\u52b9\u679c\u7684\u3067\u65b0\u3057\u3044\u6cbb\u7642\u9078\u629e\u80a2\u3068\u306a\u308a\u5f97\u308b\u53ef\u80fd\u6027\u3092\u793a\u3057\u307e\u3057\u305f\u3002<\/p>\n\n\n\n<ul 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\u30921\u65e51\u56de\u6295\u4e0e\u3057\u305f\u969b\u306e\u6709\u52b9\u6027\u304a\u3088\u3073\u5fcd\u5bb9\u6027\u3092\u8a55\u4fa1\u3059\u308b\u3053\u3068\u3092\u76ee\u7684\u3068\u3057\u305f\u300172\u9031\u9593\u8a66\u9a13\u3067\u3059\u3002 \u4e2d\u9593\u7d50\u679c\u306fThe Journal of Hepatology \u8a8c\u306b\u63b2\u8f09\u3055\u308c\u307e\u3057\u305f\u3002<sup>7<\/sup><\/p>\n\n\n\n<p class=\"has-vivid-cyan-blue-color has-text-color has-link-color wp-elements-b357f73b02a2b5b4e0d85a3ce231ae37\"><strong>\u30a4\u30d7\u30bb\u30f3\u306b\u3064\u3044\u3066<\/strong><strong><\/strong><\/p>\n\n\n\n<div class=\"wp-block-group is-vertical is-layout-flex wp-container-core-group-is-layout-8cf370e7 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wp-block-group-is-layout-flex\">\n<p class=\"has-small-font-size\">1 <a href=\"https:\/\/www.nanbyou.or.jp\/entry\/22457\">https:\/\/www.nanbyou.or.jp\/entry\/22457<\/a>\u3000\uff082025\u5e749\u6708\u30a2\u30af\u30bb\u30b9\uff09<\/p>\n\n\n\n<p class=\"has-small-font-size\">2 Agarwal S, et al. Progressive Familial Intrahepatic Cholestasis (PFIC) in Indian Children: Clinical Spectrum and Outcome. J Clin Exp Hepatol. 2016. v.6(3).<\/p>\n\n\n\n<p class=\"has-small-font-size\">3 Davit-Spraul A, et al. Progressive familial intrahepatic cholestasis. Orphanet J Rare Dis. 2009. 4(1):1-12.<\/p>\n\n\n\n<p class=\"has-small-font-size\">4 Nayagam J, et al. Hepatol Commun. 2022;6(10):2654-2664.<\/p>\n\n\n\n<p class=\"has-small-font-size\">5 Online Mendelian Inheritance in Man. <a href=\"https:\/\/www.omim.org\/clinicalSynopsis\/table?mimNumber=601847,619484,619658,602347,615878,619662,617049,619849,619874,620010,619868,211600,620962\">https:\/\/www.omim.org\/clinicalSynopsis\/table?mimNumber=601847,619484,619658,602347,615878,619662,617049,619849,619874,620010,619868,211600,620962<\/a> \uff082025\u5e7411 \u6708\u30a2\u30af\u30bb\u30b9\uff09<\/p>\n\n\n\n<p class=\"has-small-font-size\">6 Thompson RJ, et al. Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomized, placebo-controlled, phase 3 trial. Lancet Gastroenterol Hepatol 2022;7:830-842.<\/p>\n\n\n\n<p class=\"has-small-font-size\">7 Thompson RJ, et al. Interim results from an ongoing, open-label, single-arm trial of odevixibat in progressive familial intrahepatic cholestasis 2023. JHEP Rep. 5(8):100782.<\/p>\n<\/div>\n\n\n\n<p><\/p>\n","protected":false},"excerpt":{"rendered":"","protected":false},"author":102,"template":"","categories":[31],"tags":[],"class_list":["post-1055","press_release","type-press_release","status-publish","hentry","category-31","entry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v26.8 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>\u30a4\u30d7\u30bb\u30f3\u3001\u5e0c\u5c11\u75be\u60a3\u300c\u9032\u884c\u6027\u5bb6\u65cf\u6027\u809d\u5185\u80c6\u6c41\u3046\u3063\u6ede\u75c7\u306b\u4f34\u3046\u305d\u3046\u75d2\u300d\u6cbb\u7642\u85ac\u30d3\u30eb\u30d9\u30a4\u00ae\uff08\u30aa\u30c7\u30d3\u30ad\u30b7\u30d0\u30c3\u30c8\uff09\u306e\u65e5\u672c\u306b\u304a\u3051\u308b\u8ca9\u58f2\u958b\u59cb\u3092\u767a\u8868 - Japan<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" 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