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Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1

BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1), an inherited liver disease caused by mutations in ATP8B1, progresses to severe cholestasis with a sustained intractable itch. Currently, no effective therapy has been established for PFIC1. Decreased function of the bile salt...

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Detalles Bibliográficos
Autores principales: Hasegawa, Yasuhiro, Hayashi, Hisamitsu, Naoi, Sotaro, Kondou, Hiroki, Bessho, Kazuhiko, Igarashi, Koji, Hanada, Kentaro, Nakao, Kie, Kimura, Takeshi, Konishi, Akiko, Nagasaka, Hironori, Miyoshi, Yoko, Ozono, Keiichi, Kusuhara, Hiroyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4105841/
https://www.ncbi.nlm.nih.gov/pubmed/25022842
http://dx.doi.org/10.1186/1750-1172-9-89