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For: van der Woerd WL, Houwen RH, van de Graaf SF. Current and future therapies for inherited cholestatic liver diseases. World J Gastroenterol 2017; 23(5): 763-775 [PMID: 28223721 DOI: 10.3748/wjg.v23.i5.763]
URL: https://www.wjgnet.com/1007-9327/full/v23/i5/763.htm
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Number Citing Articles
1
Hao Bing, Yi-Ling Li, Dan Li, Chen Zhang, Bing Chang. Case Report: A Rare Heterozygous ATP8B1 Mutation in a BRIC1 Patient: Haploinsufficiency?. Frontiers in Medicine 2022; 9 doi: 10.3389/fmed.2022.897108
2
Hadeer Mohamed Nasr El-Din, Noha Adel Yassin, Nehal M EL Koofy, Alaa Obeida, Sherif Nabhan Kaddah, Gamal Hassan Eltagy, Wesam Mohamed. Evaluation of Clinical Outcomes in Children with Intrahepatic Cholestasis Postpartial External Biliary Diversion. Journal of Indian Association of Pediatric Surgeons 2023; 28(4) doi: 10.4103/jiaps.jiaps_49_23
3
Naser-Aldin Lashgari, Danial Khayatan, Nazanin Momeni Roudsari, Saeideh Momtaz, Ahmad Reza Dehpour, Amir Hossein Abdolghaffari. Therapeutic approaches for cholestatic liver diseases: the role of nitric oxide pathway. Naunyn-Schmiedeberg's Archives of Pharmacology 2024; 397(3) doi: 10.1007/s00210-023-02684-2
4
Beatriz Mínguez Rodríguez, Cristina Molera Busoms, Loreto Martorell Sampol, Ruth García Romero, Gemma Colomé Rivero, Javier Martín de Carpi. Heterozygous mutations of ATP8B1, ABCB11 and ABCB4 cause mild forms of Progressive Familial Intrahepatic Cholestasis in a pediatric cohort. Gastroenterología y Hepatología 2022; 45(8) doi: 10.1016/j.gastrohep.2021.12.005
5
Yi-Gui Zou, Huan Wang, Wen-Wen Li, Dong-Ling Dai. Challenges in pediatric inherited/metabolic liver disease: Focus on the disease spectrum, diagnosis and management of relatively common disorders. World Journal of Gastroenterology 2023; 29(14): 2114-2126 doi: 10.3748/wjg.v29.i14.2114
6
Rania Sakka, Hela Abroug, Sabrine Ben Youssef, Mongi Mekki, Ridha M’rad, Alessandro Granito. Types of genotypes in progressive familial intrahepatic cholestasis and liver transplantation: A meta-analysis of observational studies. PLOS One 2026; 21(6) doi: 10.1371/journal.pone.0350508
7
Alastair Baker, Nanda Kerkar, Lora Todorova, Binita M. Kamath, Roderick H.J. Houwen. Systematic review of progressive familial intrahepatic cholestasis. Clinics and Research in Hepatology and Gastroenterology 2019; 43(1) doi: 10.1016/j.clinre.2018.07.010
8
H. Eickhölter, C. Krüger, S. Kathemann, M. Endmann. Die intrahepatische, familiäre Cholestase – Manifestation bei einer Jugendlichen mit Pruritus und Ikterus nach Beginn oraler Kontrazeption. Monatsschrift Kinderheilkunde 2023;  doi: 10.1007/s00112-023-01806-7
9
Eva Sticova, Milan Jirsa, Joanna Pawłowska. New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications. Canadian Journal of Gastroenterology and Hepatology 2018; 2018 doi: 10.1155/2018/2313675
10
Yuji Jiang, Weiyuan Fang, Mingzi Cen, Jihu Li, Jianshe Wang, Jinxi Ding, Ye Chen. The disease burden and health-related quality of life in Chinese children with genetic cholestatic liver diseases: a cross-sectional study of progressive familial intrahepatic cholestasis and Alagille syndrome. Orphanet Journal of Rare Diseases 2026; 21(1) doi: 10.1186/s13023-026-04365-8
11
Wenkang Gao, Zhonglin Li, Huikuan Chu, Hang Yuan, Lilin Hu, Lin Yao, Li Zhang, Weijun Wang, Rong Lin, Ling Yang. Pharmacotherapy for Liver Cirrhosis and Its Complications. 2022;  doi: 10.1007/978-981-19-2615-0_6
12
Verena Keitel-Anselmino, Carola Dröge, Jan Stindt. Therapie-Handbuch - Gastroenterologie und Hepatologie. 2024;  doi: 10.1016/B978-3-437-21005-1.00053-6
13
Christoph Slavetinsky, Ekkehard Sturm. Odevixibat and partial external biliary diversion showed equal improvement of cholestasis in a patient with progressive familial intrahepatic cholestasis. BMJ Case Reports 2020; 13(6) doi: 10.1136/bcr-2019-234185
14
Saigopala Reddy, Nathan Fleishman, Katherine Dempsey, Edwin Ferren, Michal Kamionek, Vani V. Gopalareddy. Progressive Familial Intrahepatic Cholestasis-2 Mimicking Non-accidental Injury. ACG Case Reports Journal 2024; 11(4) doi: 10.14309/crj.0000000000001312
15
Martin Prescher, Tim Kroll, Lutz Schmitt. ABCB4/MDR3 in health and disease – at the crossroads of biochemistry and medicine. Biological Chemistry 2019; 400(10) doi: 10.1515/hsz-2018-0441
16
Eva Sticova, Milan Jirsa. ABCB4 disease: Many faces of one gene deficiency. Annals of Hepatology 2020; 19(2) doi: 10.1016/j.aohep.2019.09.010
17
Lavinija Matakovic, Arend W. Overeem, Karin Klappe, Sven C. D. van IJzendoorn. Hepatocytes. Methods in Molecular Biology 2022; 2544 doi: 10.1007/978-1-0716-2557-6_4
18
Jorge Mallea, Candice Bolan, Cherise Cortese, Denise Harnois. Cystic Fibrosis–Associated Liver Disease in Lung Transplant Recipients. Liver Transplantation 2019; 25(8) doi: 10.1002/lt.25496
19
. 2019;  doi: 10.70675/5ae39dfdz2409z4c11z9be3z176c1acb5ce6
20
Tim Kroll, Sander H.J. Smits, Lutz Schmitt. Monomeric bile acids modulate the ATPase activity of detergent-solubilized ABCB4/MDR3. Journal of Lipid Research 2021; 62 doi: 10.1016/j.jlr.2021.100087
21
Min Ji Sohn, Min Hyung Woo, Moon-Woo Seong, Sung Sup Park, Gyeong Hoon Kang, Jin Soo Moon, Jae Sung Ko. Benign Recurrent Intrahepatic Cholestasis Type 2 in Siblings with Novel ABCB11 Mutations. Pediatric Gastroenterology, Hepatology & Nutrition 2019; 22(2) doi: 10.5223/pghn.2019.22.2.201
22
Patryk Lipiński, Elżbieta Ciara, Dorota Jurkiewicz, Rafał Płoski, Marta Wawrzynowicz-Syczewska, Joanna Pawłowska, Irena Jankowska. Progressive familial intrahepatic cholestasis type 3: Report of four clinical cases, novel ABCB4 variants and long-term follow-up. Annals of Hepatology 2021; 25 doi: 10.1016/j.aohep.2021.100342
23
Eleni V. Geladari, Natalia G. Vallianou, Evangelia Margellou, Dimitris Kounatidis, Vassilios Sevastianos, Alexandra Alexopoulou. Benign Recurrent Intrahepatic Cholestasis: Where Are We Now?. Gastroenterology Insights 2024; 15(1) doi: 10.3390/gastroent15010011
24
Melissa Palmer, Arie Regev, Keith Lindor, Mark I. Avigan, Lara Dimick‐Santos, William Treem, John F. Marcinak, James H. Lewis, Frank A. Anania, Daniel Seekins, Benjamin L. Shneider, Naga Chalasani. Consensus guidelines: best practices for detection, assessment and management of suspected acute drug‐induced liver injury occurring during clinical trials in adults with chronic cholestatic liver disease. Alimentary Pharmacology & Therapeutics 2020; 51(1) doi: 10.1111/apt.15579
25
Eva Sticova, Magdalena Neroldova, Radana Kotalova, Iva Subhanova, Milan Jirsa. ABCB4 disease mimicking morbus Wilson: A potential diagnostic pitfall. Biomedical Papers 2020; 164(1) doi: 10.5507/bp.2019.054
26
Ming Li, Qian Wang, Yong Li, Shengtian Cao, Yingjun Zhang, Zhongqing Wang, Guozhu Liu, Jing Li, Baohua Gu. Apical sodium-dependent bile acid transporter, drug target for bile acid related diseases and delivery target for prodrugs: Current and future challenges. Pharmacology & Therapeutics 2020; 212 doi: 10.1016/j.pharmthera.2020.107539
27
Rong Chen, Feng-Xia Yang, Yan-Fang Tan, Mei Deng, Hua Li, Yi Xu, Wen-Xian Ouyang, Yuan-Zong Song. Clinical and genetic characterization of pediatric patients with progressive familial intrahepatic cholestasis type 3 (PFIC3): identification of 14 novel ABCB4 variants and review of the literatures. Orphanet Journal of Rare Diseases 2022; 17(1) doi: 10.1186/s13023-022-02597-y
28
Tim Kroll, Martin Prescher, Sander H. J. Smits, Lutz Schmitt. Structure and Function of Hepatobiliary ATP Binding Cassette Transporters. Chemical Reviews 2021; 121(9) doi: 10.1021/acs.chemrev.0c00659
29
Christoph Jüngst, Christina Justinger, Janett Fischer, Thomas Berg, Frank Lammert. Common <b><i>ABCB4</i></b> and <b><i>ABCB11</i></b> Genotypes Are Associated with Idiopathic Chronic Cholestasis in Adults. Digestive Diseases 2022; 40(4) doi: 10.1159/000518203
30
Holmfridur Helgadottir, Geir Folvik, Mette Vesterhus. Improvement of cholestatic episodes in patients with benign recurrent intrahepatic cholestasis (BRIC) treated with rifampicin. A long-term follow-up. Scandinavian Journal of Gastroenterology 2023; 58(5) doi: 10.1080/00365521.2022.2143725
31
Eric Arthur Lorio, David Valadez, Naim Alkhouri, Nicole Loo. Cholestasis in Benign Recurrent Intrahepatic Cholestasis 2. ACG Case Reports Journal 2020; 7(6) doi: 10.14309/crj.0000000000000412
32
Mounia Lakli, Julie Dumont, Virginie Vauthier, Julie Charton, Veronica Crespi, Manon Banet, Yosra Riahi, Amel Ben Saad, Elodie Mareux, Martine Lapalus, Emmanuel Gonzales, Emmanuel Jacquemin, Florent Di Meo, Benoit Deprez, Florence Leroux, Thomas Falguières. Identification of new correctors for traffic-defective ABCB4 variants by a high-content screening approach. Communications Biology 2024; 7(1) doi: 10.1038/s42003-024-06590-y
33
Kazuto Tajiri, Yukihiro Shimizu. Recent advances in the management of pruritus in chronic liver diseases. World Journal of Gastroenterology 2017; 23(19): 3418-3426 doi: 10.3748/wjg.v23.i19.3418
34
Mounia Lakli, Marion Onnée, Thomas Carrez, Frédéric Becq, Thomas Falguières, Pascale Fanen. ABC transporters involved in respiratory and cholestatic diseases: From rare to very rare monogenic diseases. Biochemical Pharmacology 2024; 229 doi: 10.1016/j.bcp.2024.116468
35
Eva-Doreen Pfister, Veronika K Jaeger, André Karch, Denys Shay, Nagoud Schukfeh, Johanna Ohlendorf, Norman Junge, Imeke Goldschmidt, Amelie Stalke, Verena Keitel-Anselmino, Ulrich Baumann. Native liver survival in bile salt export pump deficiency: results of a retrospective cohort study. Hepatology Communications 2023; 7(4) doi: 10.1097/HC9.0000000000000092
36
Peter Hegyi, Jozsef Maléth, Julian R. Walters, Alan F. Hofmann, Stephen J. Keely. Guts and Gall: Bile Acids in Regulation of Intestinal Epithelial Function in Health and Disease. Physiological Reviews 2018; 98(4) doi: 10.1152/physrev.00054.2017
37
Sara Hassan, Paula Hertel. Overview of Progressive Familial Intrahepatic Cholestasis. Clinics in Liver Disease 2022; 26(3) doi: 10.1016/j.cld.2022.03.003
38
David Daniel, Diana Fang, Kiyoko Oshima, Sonja O. Vozniak, Aaron A. R. Tobian, Elizabeth P. Crowe, James Peter Hamilton, Evan M. Bloch, Herleen Rai. Long‐Term Management of Recurrent Antibody‐Induced Bile Salt Export Pump Deficiency After Liver Transplantation Using Therapeutic Plasma Exchange. Journal of Clinical Apheresis 2026; 41(1) doi: 10.1002/jca.70096
39
Yue Wang, Yin-Ling Wang, Yue-Ping Zhu, Wei-Lu Xu, Jin-Long Huang, Xiao-Zhe Chai, Ming Li, Feng Qian, Chuan-Wu Zhu. Type 3 progressive familial intrahepatic cholestasis combined with autoimmune hepatitis-primary biliary cirrhosis overlap syndrome: A case report. World Chinese Journal of Digestology 2022; 30(1) doi: 10.11569/wcjd.v30.i1.56
40
Brandon Vu, Ryo Kawamoto, Priscila Villalba-Davila, Xinzhong Dong, Wikrom Karnsakul. Bile acids as therapeutic agents. Frontiers in Pharmacology 2026; 16 doi: 10.3389/fphar.2025.1732854
41
Ben-Ping Zhang, Zhi-Hua Huang, Chen Dong. Biliary atresia combined with progressive familial intrahepatic cholestasis type 3. Medicine 2019; 98(19) doi: 10.1097/MD.0000000000015593
42
Manon Banet, Veronica Crespi, Jonathan Elie, Yosra Riahi, Mounia Lakli, Elodie Mareux, Emmanuel Gonzales, Emmanuel Jacquemin, Laurent Meijer, Martine Lapalus, Florent Di Meo, Thomas Falguières. Molecular characterisation of the trafficking rescue of defective ABCB4 variants by roscovitine analogues. Scientific Reports 2026; 16(1) doi: 10.1038/s41598-026-39840-6
43
Tengqi Ren, Liwei Pang, Wanlin Dai, Shuodong Wu, Jing Kong. Regulatory mechanisms of the bile salt export pump (BSEP/ABCB11) and its role in related diseases. Clinics and Research in Hepatology and Gastroenterology 2021; 45(6) doi: 10.1016/j.clinre.2021.101641
44
Beatriz Mínguez Rodríguez, Cristina Molera Busoms, Loreto Martorell Sampol, Ruth García Romero, Gemma Colomé Rivero, Javier Martín de Carpi. Heterozygous mutations of ATP8B1, ABCB11 and ABCB4 cause mild forms of Progressive Familial Intrahepatic Cholestasis in a pediatric cohort. Gastroenterología y Hepatología (English Edition) 2022; 45(8) doi: 10.1016/j.gastre.2021.12.004
45
Adil Salyani, Linda Barasa, Allan Rajula, Sayed K. Ali. Benign Recurrent Intrahepatic Cholestasis (BRIC): An African Case Report. Case Reports in Gastrointestinal Medicine 2020; 2020 doi: 10.1155/2020/2894293
46
Antonia Felzen, Henkjan J. Verkade. The spectrum of Progressive Familial Intrahepatic Cholestasis diseases: Update on pathophysiology and emerging treatments. European Journal of Medical Genetics 2021; 64(11) doi: 10.1016/j.ejmg.2021.104317
47
Verena Keitel-Anselmino. Therapie-Handbuch - Gastroenterologie und Hepatologie. 2021;  doi: 10.1016/B978-3-437-23847-5.00050-8
48
Lander Heyerick, Annemieke Dhondt, Hans Van Vlierberghe, Xavier Verhelst, Sarah Raevens, Anja Geerts. Early plasmapheresis in type 2 benign recurrent intrahepatic cholestasis: A case report and review of literature. World Journal of Hepatology 2025; 17(2): 102375 doi: 10.4254/wjh.v17.i2.102375
49
Jawaher Abdullah Alamoudi, Wenkuan Li, Nagsen Gautam, Marco Olivera, Jane Meza, Sandeep Mukherjee, Yazen Alnouti. Bile acid indices as biomarkers for liver diseases I: Diagnostic markers. World Journal of Hepatology 2021; 13(4): 433-455 doi: 10.4254/wjh.v13.i4.433
50
Shuying Xie, Shizhang Wei, Xiao Ma, Ruilin Wang, Tingting He, Zhao Zhang, Ju Yang, Jiawei Wang, Lei Chang, Manyi Jing, Haotian Li, Xuelin Zhou, Yanling Zhao. Genetic alterations and molecular mechanisms underlying hereditary intrahepatic cholestasis. Frontiers in Pharmacology 2023; 14 doi: 10.3389/fphar.2023.1173542
51
Samiksha Gupta, Ijlal Akbar Ali, Eleanor Abreo, Veena Gujju, Maham Hayat. The Mystery of Episodic Recurrent Jaundice in a Young Male: Cholestasis With a Normal Gamma-Glutamyl Transferase. Cureus 2021;  doi: 10.7759/cureus.13834
52
Jonathan M. Moore, Eric L. Bell, Robert O. Hughes, Alastair S. Garfield. ABC transporters: human disease and pharmacotherapeutic potential. Trends in Molecular Medicine 2023; 29(2) doi: 10.1016/j.molmed.2022.11.001
53
Davor Slijepcevic, Reinout L.P. Roscam Abbing, Claudia D. Fuchs, Lizette C.M. Haazen, Ulrich Beuers, Michael Trauner, Ronald P.J. Oude Elferink, Stan F.J. van de Graaf. Na+‐taurocholate cotransporting polypeptide inhibition has hepatoprotective effects in cholestasis in mice. Hepatology 2018; 68(3) doi: 10.1002/hep.29888
54
James Neuberger. Liver Transplantation. 2021;  doi: 10.1002/9781119634010.ch49
55
Amil Sinha, Meha Bhuva, Claire Grant, Alexander E. Gimson, Edward Thompson, Adam Duckworth, Susan E. Davies, Guruprasad Aithal, William J. Griffiths. ABCB4 Mutations in Adults Cause a Spectrum Cholestatic Disorder Histologically Distinct from Other Biliary Disease. Digestive Diseases and Sciences 2022; 67(12) doi: 10.1007/s10620-022-07416-9
56
Willemien F.J. Hof, Jan Freark de Boer, Henkjan J. Verkade. Emerging drugs for the treatment of progressive familial intrahepatic cholestasis: a focus on phase II and III trials. Expert Opinion on Emerging Drugs 2024; 29(3) doi: 10.1080/14728214.2024.2336986
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58
Binita M. Kamath, Philip Stein, Roderick H. J. Houwen, Henkjan J. Verkade. Potential of ileal bile acid transporter inhibition as a therapeutic target in Alagille syndrome and progressive familial intrahepatic cholestasis. Liver International 2020; 40(8) doi: 10.1111/liv.14553
59
Haibing Gao, Shenglong Lin, Xujiang Lv, Huaxi Ma, Xiangmei Wang, Jiankai Fang, Wenjun Wu, Jiahuang Lin, Xiumin Chen, Minghua Lin. The Identification of Two New ABCB11 Gene Mutations and the Treatment Outcome in a Young Adult with Benign Recurrent Intrahepatic Cholestasis: A Case Report. Hepatitis Monthly 2017; 17(10) doi: 10.5812/hepatmon.55087
60
Roderick Houwen. Chapter 6.4. Diagnostic Progress in Cholestasis. Journal of Pediatric Gastroenterology and Nutrition 2018; 66(S1) doi: 10.1097/MPG.0000000000001916
61
Aradhana Aneja, Elizabeth Scott, Rohit Kohli. Advances in management of end stage liver disease in children. Medical Journal Armed Forces India 2021; 77(2) doi: 10.1016/j.mjafi.2021.03.001
62
Xinyu Li, Shuzhen Li, Weipu Zhang, Qi Wang, Wei Zou. Impacts of P4‐ATPase Deletion on Membrane Asymmetry and Disease Development. Cell Biochemistry and Function 2024; 42(7) doi: 10.1002/cbf.70004
63
Patryk Lipiński, Joanna Pawłowska, Irena Jankowska. Łagodna nawracająca cholestaza wewnątrzwątrobowa. Pediatria Polska 2017; 92(5) doi: 10.1016/j.pepo.2017.03.010
64
Joanna Cielecka-Kuszyk, Patryk Lipiński, Sylwia Szymańska, Hor Ismail, Irena Jankowska. Long-term follow-up in children with progressive familial intrahepatic cholestasis type 2 after partial external biliary diversion with focus on histopathological features. Polish Journal of Pathology 2019; 70(2) doi: 10.5114/pjp.2019.87099
65
Wafaa Ahmed, Rebecca Jeyaraj, David Reffitt, John Devlin, Abid Suddle, John Hunt, Michael A Heneghan, Phillip Harrison, Deepak Joshi. Nasobiliary drainage: an effective treatment for pruritus in cholestatic liver disease. Frontline Gastroenterology 2022; 13(5) doi: 10.1136/flgastro-2021-102025
66
Huayu Chen, Dongbo Wu, Wei Jiang, Ting Lei, Changli Lu, Taoyou Zhou. Case Report: A Novel Homozygous Variant Identified in a Chinese Patient With Benign Recurrent Intrahepatic Cholestasis-Type 1. Frontiers in Medicine 2021; 8 doi: 10.3389/fmed.2021.705489
67
Hamid Reza Foroutan, Ali Bahador, Sultan Mohsin Ghanim, Seyed Mohsen Dehghani, Mohammad Hossein Anbardar, Mohammad Reza Fattahi, Mehdi Forooghi, Omidreza Azh, Ali Tadayon, Alireza Sherafat, Amir Arsalan Yaghoobi, Mohammad Ali Ashraf. Effects of partial internal biliary diversion on long-term outcomes in patients with progressive familial intrahepatic cholestasis: experience in 44 patients. Pediatric Surgery International 2020; 36(5) doi: 10.1007/s00383-020-04641-z
68
Philipp Schreiner, Bruno Stieger, Valérie McLin, Anne‐Laure Rougemont, Verena Keitel, Carola Dröge, Beat Müllhaupt. A rare cause of a cholestatic jaundice in a North African teenager. Liver International 2019; 39(11) doi: 10.1111/liv.14122
69
Henkjan J. Verkade, Richard J. Thompson, Henrik Arnell, Björn Fischler, Per‐Göran Gillberg, Jan P. Mattsson, Kristina Torfgård, Erik Lindström. Systematic Review and Meta‐analysis. Journal of Pediatric Gastroenterology and Nutrition 2020; 71(2) doi: 10.1097/MPG.0000000000002789
70
Milena Marx, Steffen Hartleif, Johannes Hilberath, Christoph P. Berg, Ilias Tsiflikas, Stephan Singer, Ekkehard Sturm. Practical Considerations for Odevixibat Treatment in Patients with Progressive Familial Intrahepatic Cholestasis: A Single-Center Case Series. Journal of Clinical Medicine 2024; 13(24) doi: 10.3390/jcm13247508
71
Hui Lin, Xiao-Rong Peng, Xiao-Mei Qin, Hai-Ming Zhang, Xiang Pan, Wei-Xia Lin, Rong Chen, Shi-Shu Zhu, Fu Xiong, Xiao-Ling Huang, Zhi-Jun Zhu, Wen-Xian Ouyang, Hong-Mei Xu, Yuan-Zong Song. Genetic and clinical spectrum of progressive familial intrahepatic cholestasis type 1: novel ATP8B1 variants and genotype–phenotype correlations. European Journal of Pediatrics 2026; 185(10) doi: 10.1007/s00431-026-07403-6
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