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| 1 | Expanding etiology of progressive familial intrahepatic cholestasis显示文摘BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)refers to a disparate group of autosomal recessive disorders that are linked by the inability to appropriately form and excrete bile from hepatocytes,resulting in a hepatocellular form of cholestasis.While the diagnosis of such disorders had historically been based on pattern recognition of unremitting cholestasis without other identified molecular or anatomic cause,recent scientific advancements have uncovered multiple specific responsible proteins.The variety of identified defects has resulted in an ever-broadening phenotypic spectrum,ranging from traditional benign recurrent jaundice to progressive cholestasis and end-stage liver disease.AIM To review current data on defects in bile acid homeostasis,explore the expanding knowledge base of genetic based diseases in this field,and report disease characteristics and management.METHODS We conducted a systemic review according to PRISMA guidelines.We performed a Medline/PubMed search in February-March 2019 for relevant articles relating to the understanding,diagnosis,and management of bile acid homeostasis with a focus on the family of diseases collectively known as PFIC.English only articles were accessed in full.The manual search included references of retrieved articles.We extracted data on disease characteristics,associations with other diseases,and treatment.Data was summarized and presented in text,figure,and table format.RESULTS Genetic-based liver disease resulting in the inability to properly form and secrete bile constitute an important cause of morbidity and mortality in children and increasingly in adults.A growing number of PFIC have been described based on an expanded understanding of biliary transport mechanism defects and the development of a common phenotype.CONCLUSION We present a summary of current advances made in a number of areas relevant to both the classically described FIC1(ATP8B1),BSEP(ABCB11),and MDR3(ABCB4)transporter deficiencies,as well as more recently described gene mutations--TJP2(TJP2),FXR(NR1H4),MYO5B(MYO5B),and others which expand the etiology and understanding of PFIC-related cholestatic diseases and bile transport. | Sarah AF Henkel Judy H Squires Mary Ayers Armando Ganoza Patrick Mckiernan James E Squires | 2019 | World Journal of Hepatology2019，11，5： | 13 |
| 2 | Differential mutagenic,antimutagenic and cytotoxic responses induced by apomorphine and its oxidation product, 8-oxo-apomorphine-semiquinone, in bacteria and yeast显示文摘 | PICADA JN MARIS AF CKLESS K | 2003 | Mutat Res2003，539，12： | 1 |
| 3 | High dose bicalutamide for androgen independent prostate cancer: effect of prior hormonal therapy显示文摘 | Robin J Mary AF Paola R | 1998 | Urology1998，159，1： | 1 |
| 4 | Sympa- thetic neural hyperactivity and its normalization following unstable angi- na andac -ute myocardial infarction 显示文摘 | Graham LN Smith PA Stoker JB Mackintosh AF Mary DA | 2004 | C lin Sci (Lond)2004，106，： | 1 |
| 5 | Workforce conflict: What' s the problem? 显示文摘 | Dewitty VP Osborne JW Mary AF | 2009 | Nurs Manage2009，3，： | 1 |
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| 7 | Antiinflammatory effects of salmeterol after inhalation of lipopolysaccharide by healthy volunteers显示文摘 | Maris NA de Vos AF Dessing MC | 2005 | Am J Respir Crit Care Med2005，172，7： | 1 |
| 8 | Annual summary of vital statistics trends in the health of americans during the 20th century显示文摘 | Bernard G Mary AF Donna MS | 2000 | Pediatrics2000，106，6： | 1 |
| 9 | High dose bicalutamide for androgen independent prostate cancer:effect of prior hormonal therapy显示文摘 | Mary AF Paola R | 1998 | Urology1998，159，1： | 1 |
| 10 | Studies on the carcinogenis activity of protein-denaturing agents:hepatocarcino-genicity of dioxane显示文摘 | Mary AF Arcos JC Cornelia HL | 1965 | Hepatocarcinogenicity of Dioxane1965，35，： | 1 |
| 11 | Subcutaneous administration of recombinant glycosylated interleukin 6 in patients with cancer: pharmacokinetics, pharmacodynamics and immunomodu- latory effects显示文摘 | Rosamonde EB Mary AF Poulam MP | 2000 | Cytokine2000，12，4： | 1 |
| 12 | Canonical transient receptor poten- tiall plays a role in basic fibroblast growth factor(bFGF)/FGF re- ceptor- 1 -induced Ca2 + entry and embryonic rat neural stem cell proliferation显示文摘 | Pla AF Marie D Brazer SC | 2008 | J Neurosci2008，25，： | 1 |
| 13 | Utilization and transport of L-Arabinose by non-Saccharomyces yeasts 显示文摘 | Eric PK Mary AF Boris US | 2009 | Cellulose2009，16，4： | 1 |
| 14 | Workforce conflict:what's the problem?显示文摘 | Dewitty VP Osborne JW Mary AF | 2009 | Nurs Manag2009，40，5： | 1 |
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