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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 | Glyphmaker: creating customized visualization of complex data 显示文摘 | RIBARSKY W AYERS E EBLE J | 1995 | IEEE Computer1995，27，7： | 1 |
| 3 | Oxidative stress in suharaehnoid haemor- rhage: significance in acute brain injury and vasospasm 显示文摘 | Ayer R E Zhang J H | 2008 | Acta Neurochir Suppl2008，104，1： | 1 |
| 4 | Pretransplant positive positron emission tomography/gallium scans predict poor outcome in patients with recurrent/refractory Hodgkin lymphoma 显示文摘 | Jabbour E Hosing C Ayers G | 2007 | Cancer2007，109，12： | 1 |
| 5 | Field evaluation of response times for a variable rate ( pressure-based and injection) liquid chemical applicator显示文摘 | Anglund E A Ayers P D | 2003 | Applied Engineering in Agriculture2003，19，3： | 1 |
| 6 | Rapid solidification processing with specific application to aluminum alloys显示文摘 | Lavemia E J Ayers J D Srivatsan T S | 1991 | International Materials Review1991，37，1： | 1 |
| 7 | Oxidative stress in subarachnoid haemorrhage:significance in acute brain injury and vasospasm显示文摘 | Ayer R E Zhang J H | 2008 | Acta Neurochir Suppl2008，104，： | 1 |
| 8 | Pretransplant positive positron emission tomography/gallium scans predict poor outcome in patients with recurrent/re- fractory Hodgkin lymphoma 显示文摘 | Jabbour E Hosing C Ayers G | 2007 | Cancer2007，109，： | 1 |
| 9 | Rapid solidification processing with specific application to aluminium alloys 显示文摘 | Lavemia E J Ayers J D Srivatsan S | 1992 | International Materials Reviews1992，37，1： | 1 |
| 10 | Oxidative Stress in Subarachnoid Haemorrhage: Significance in Acute Brain Injury and Vasospasm显示文摘 | Ayer R E Zhang J H | 2008 | Acta Neurochir2008，104，： | 1 |
| 11 | Effect of celecoxib on capecitabine induced hand-foot syndrome and antiumor显示文摘 | Lin E Morris JS Ayers GD | 2002 | Oncology2002，16，： | 1 |
| 12 | Field evaluation of response times for a variable rate liquid chemical applicator显示文摘 | Anglund E A Ayers P D | 2003 | Applied Engineering in Agriculture2003，19，3： | 1 |
| 13 | Effect of celecoxib on capecitabine-induced hand foot syndrome and antitumor activity显示文摘 | Lin E Morris JS Ayers GD | 2002 | Oncology2002，16，： | 1 |
| 14 | Effect of celecoxib on capecit abine-in- duced hand-foot syndrome and antitumor activity显示文摘 | Lin E Morris JS Ayers GD | 2002 | Oncology ( Williston Park)2002，16，1214： | 1 |
| 15 | Effect of celecoxib on capecltabine-induced hand-foot syndrome and antitumor activity显示文摘 | Lin E Morris JS Ayers GD | 2002 | Oncology(Huntingt)2002，16，1214： | 1 |
| 16 | Mad: a heterodimeric partner for Max that antagonizes Myc transcriptional activity显示文摘 | Ayer D E Kretzner L Eisenman R N | 1993 | Cell1993，72，2： | 1 |
| 17 | Me latonin decreases mortality following severe subarachnoid hem orrhage显示文摘 | Robert E Ayer Takashi Sugawara Wanqiu Chen | 2008 | J-PinealRes2008，44，2： | 1 |
| 18 | Rapid solidification processing with specific application to aluminum alloys显示文摘 | LAVERNIA E J AYERS J D SRIVATSAN T S | 1992 | International Materials Reviews1992，37，1： | 1 |
| 19 | Traumatic spinal cord injury in the United States,1993-2012显示文摘 | Jain N B Ayers G D Peterson E N | 2015 | JAMA2015，313，22： | 1 |
| 20 | Rapid solidification processing with application to aluminum alloys显示文摘 | LAVERNIA E J AYER J D SRIVATSAN T S | 1992 | International Materials Reviews1992，37，1： | 1 |
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