Epidemiology and burden of progressive familial intrahepatic cholestasis: a systematic review

被引：0
|
作者：
Tracey Jones-Hughes
Jo Campbell
Louise Crathorne
机构：
来源：
Orphanet Journal of Rare Diseases | / 16卷
关键词：
Pruritus; Bile acid; and ;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
引用
收藏
相关论文
共 50 条
  • [31] Genotypes and different clinical variants between children and adults in progressive familial intrahepatic cholestasis: a state-of-the-art review
    Vitale, Giovanni
    Sciveres, Marco
    Mandato, Claudia
    d'Adamo, Adamo Pio
    Di Giorgio, Angelo
    ORPHANET JOURNAL OF RARE DISEASES, 2025, 20 (01)
  • [32] Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II
    Giulia Jannone
    Xavier Stephenne
    Isabelle Scheers
    Françoise Smets
    Catherine de Magnée
    Raymond Reding
    Etienne M. Sokal
    European Journal of Pediatrics, 2020, 179 : 1547 - 1552
  • [33] Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II
    Jannone, Giulia
    Stephenne, Xavier
    Scheers, Isabelle
    Smets, Francoise
    de Magnee, Catherine
    Reding, Raymond
    Sokal, Etienne M.
    EUROPEAN JOURNAL OF PEDIATRICS, 2020, 179 (10) : 1547 - 1552
  • [34] Long-term outcome following cholecystocolostomy in 41 patients with progressive familial intrahepatic cholestasis
    Qianqing Li
    Clara Chong
    Rui Sun
    Tong Yin
    Ting Huang
    Mei Diao
    Long Li
    Pediatric Surgery International, 2021, 37 : 723 - 730
  • [35] Long-term outcome following cholecystocolostomy in 41 patients with progressive familial intrahepatic cholestasis
    Li, Qianqing
    Chong, Clara
    Sun, Rui
    Yin, Tong
    Huang, Ting
    Diao, Mei
    Li, Long
    PEDIATRIC SURGERY INTERNATIONAL, 2021, 37 (06) : 723 - 730
  • [36] Effect of food on the pharmacokinetics and therapeutic efficacy of 4-phenylbutyrate in progressive familial intrahepatic cholestasis
    Nakano, Satoshi
    Osaka, Shuhei
    Sabu, Yusuke
    Minowa, Kei
    Hirai, Saeko
    Kondou, Hiroki
    Kimura, Takeshi
    Azuma, Yoshihiro
    Watanabe, Satoshi
    Inui, Ayano
    Bessho, Kazuhiko
    Nakamura, Hidefumi
    Kusano, Hironori
    Nakazawa, Atsuko
    Tanikawa, Ken
    Kage, Masayoshi
    Shimizu, Toshiaki
    Kusuhara, Hiroyuki
    Zen, Yoh
    Suzuki, Mitsuyoshi
    Hayashi, Hisamitsu
    SCIENTIFIC REPORTS, 2019, 9 (1)
  • [37] Food and Drug Administration Approval Summary: Odevixibat (Bylvay) for the Treatment of Pruritus With Progressive Familial Intrahepatic Cholestasis
    Yi, Sojeong
    Kim, Insook
    Hager, Rebecca
    Strazzeri, Marian M.
    Garrard, Lili
    Matsubayashi, Toru
    Mehta, Ruby
    GASTRO HEP ADVANCES, 2025, 4 (04):
  • [38] A homozygous R148W mutation in Semaphorin 7A causes progressive familial intrahepatic cholestasis
    Pan, Qiong
    Luo, Gang
    Qu, Jiaquan
    Chen, Sheng
    Zhang, Xiaoxun
    Zhao, Nan
    Ding, Jingjing
    Yang, Hong
    Li, Mingqiao
    Li, Ling
    Cheng, Ying
    Li, Xuan
    Xie, Qiaoling
    Li, Qiao
    Zhou, Xueqian
    Zou, Huiling
    Fan, Shijun
    Zou, Lingyun
    Liu, Wei
    Deng, Guohong
    Cai, Shi-Ying
    Boyer, James L.
    Chai, Jin
    EMBO MOLECULAR MEDICINE, 2021, 13 (11)
  • [39] Potential of ileal bile acid transporter inhibition as a therapeutic target in Alagille syndrome and progressive familial intrahepatic cholestasis
    Kamath, Binita M.
    Stein, Philip
    Houwen, Roderick H. J.
    Verkade, Henkjan J.
    LIVER INTERNATIONAL, 2020, 40 (08) : 1812 - 1822
  • [40] Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1
    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
    ORPHANET JOURNAL OF RARE DISEASES, 2014, 9
12345