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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 | 诊断性试验和策略的证据质量和推荐强度的分级显示文摘GRADE系统能对诊断性试验或策略的证据质量和推荐强度进行分级。本文旨在阐释在此过程中如何考虑患者的重要结局, | Holger J Schünemann Andrew D Oxman Jan Brozek Paul Glasziou Roman Jaeschke Gunn E Vist John W Williams Jr Regina Kunz Jonathan Craig Victor M Montori Patrick Bossuyt Gordon H Guyatt 李晓 黄程 陈耀龙 李幼平 | 2009 | 中国循证医学杂志2009，9，5： | 7 |
| 3 | Adenoma detection with cap-assisted colonoscopy versus regular colonoscopy: a randomised controlled trial显示文摘 | Thomas R de Wijkerslooth Esther M Stoop Patrick M Bossuyt Elisabeth M H Mathus-Vliegen Jan Dees Kristien M A J Tytgat Monique E van Leerdam Paul Fockens Ernst J Kuipers Evelien Dekker | 2012 | Gut2012，，10： | 3 |
| 4 | Exiguamine A, an indoleamine 2,3-dioxygenase(IDO) inhibitor isolated from the marine sponge Neopetrosia exigua显示文摘 | BRASTIANOS H C VOTTERO E PATRICK B O | 2006 | J Am Chem Soc2006，128，16： | 1 |
| 5 | Fiber grating sensors 显示文摘 | A D Kersey M A Davis H J Patrick M LeBlanc K P Koo C G Askins M A Putnam E J Friebele | 1997 | Lightwave Technology1997，15，8： | 1 |
| 6 | Fenton-driven chemical regeneration of MTBE-spent GAC显示文摘 | Scott G H Patrick K J Wendell P E | 2005 | Water Research2005，39，10： | 1 |
| 7 | Multiple independent emer- gences of type 2 vaccine-derived polioviruses during a large outbreak in northern Nigeria显示文摘 | Burns C C Shaw J Jorba J Bukbuk D Adu F Gumede N Pate M A Abanida E A Gasasira A Iber J Chen Q Vincent A Chenoweth P Henderson E Wannemuehler K Naeem A Umarni R N Nishimura Y Shimizu H Baba M Adeniji A Williams A J Kil- patrick D R Oberste M S Wassilak S G Tomori O Pallansch M A Kew O | 2013 | J Virol2013，87，9： | 1 |
| 8 | A Dynamic Game of Transboundary Pollutant with Asymmetric Players显示文摘 | Martin E W Patrick R H Tolwinsky B | 1993 | Journal of EnvironmentalEconomics and Management1993，24，： | 1 |
| 9 | Ammonium diffusion as a factor in nitrogen loss from flooded soils 显示文摘 | Reddy K R Patrick W H J Phillips R E | 1976 | Soil Science Society1976，40，： | 1 |
| 10 | Efficacy of eplerenone versus enalapril as monotherapy in systemic hypertension显示文摘 | Gordon H Williams Ellen Burgess Rainer E Kolloch Luis Miguel Ruilope Joanna Niegowska Mark S Kipnes Barbara Roniker Jeffrey L Patrick Scott L Krause | 2004 | The American Journal of Cardiology2004，，： | 1 |
| 11 | Fiber grating sensors显示文摘 | KERSEY A D DAVIS M A PATRICK H J LeBLANC M KOO K P ASKINS C G PUTNAM M A FRIEBELE E J | 1997 | Journal Lightwave Technology1997，15，8： | 1 |
| 12 | Short in vitro half-life of thymopoietin 32-36 pentapeptide in human plasma显示文摘 | Tischio J P Patrick J E Weintraub H S | 1979 | Int JPharm1979，14，： | 1 |
| 13 | Ammonium diffusion as a factor in nitrogen loss from flooded soils显示文摘 | Reddy K R Patrick W H Phillips R E | 1976 | Soil Science Society of American Journal1976，40，： | 1 |
| 14 | Sulfate reduction in soil: effects of redox potential and pH 显示文摘 | Connell W E Patrick W H | 1968 | Science1968，159，： | 1 |
| 15 | Jasmonate andphytochrome A signaling in Arabidopsis wound and shaderesponses are integrated through JAZ1 stability 显示文摘 | Robson F Okamoto H Patrick E | 2010 | PlantCell2010，22，4： | 1 |
| 16 | Detection and quantification of mitochondfial DNA deletions in individual cells by real-time PCR显示文摘 | LANGPING H PATRICK F C STEVE E D | 2002 | Nucleic Acids Res2002，30，14： | 1 |
| 17 | Reduction and reoxidation cycles of manganese and iron in flood soil and in water solution显示文摘 | Patrick W H J R Henderson R E | 1981 | Soil Sci Soc Am J1981，45，3： | 1 |
| 18 | Human Paragonimiasis After Eating Raw or Undercooked Crayfish - Missouri, July 2006-September 2010显示文摘 | Patrick S L Turabelidze G Marx H Grim A Lane M A Weil G J Bailey T C ?nen N F Demertzis L M Tuteur P G Hayes E V Davila S Z Folk S M Mitchem R E Kammerer E Fannon L P Jones J L Wilkins P P Lo Y C | 2010 | MMWR. Morbidity and Mortality Weekly Report2010，，： | 1 |
| 19 | The place of interpretation:a new approach to the evaluation of interpretation显示文摘 | Stewart E J Brown M H Patrick J D | 1998 | Tourism Mangement1998，19，3： | 1 |
| 20 | Thoracic aorta at multi-detector row CT: motion artifact with various reconstruction windows显示文摘 | Gareth J Morgan H Patrick E | 2003 | Radiology2003，228，： | 1 |
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