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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 | New agents for bacillus Calmette–Guérin-refractory nonmuscle invasive bladder cancer显示文摘 | Jennifer J. Ahn Rashed A. Ghandour James M. McKiernan | 2014 | Current Opinion in Urology2014，，5： | 2 |
| 3 | Long-Term Survival Outcomes with Intravesical Docetaxel in the Management of Recurrent Nonmuscle Invasive Bladder Cancer After Previous Bacillus Calmette-Guérin Therapy显示文摘 | LaMont J. Barlow James M. McKiernan Mitchell C. Benson | 2013 | The Journal of Urology2013，，： | 2 |
| 4 | Early embryonic development by co-culture with oviduct epithelial cells in pigs显示文摘 | White K L Ando H Mckiernan S H | 1989 | Biol Reprod1989，41，： | 1 |
| 5 | A parametric manipulation of factors affecting task-induced deactivation in functional neuroimaging 显示文摘 | McKiernan K A Kaufman J N Kucera-Thompson J | 2003 | J Cogn Neurosci2003，15，3： | 1 |
| 6 | The detection of renal carcinoma cells in the peripheral blood with an enhanced reverse transcriptase-polymerase chain reaction assay for MN/CA9 显示文摘 | McKiernan JM Buttyan R Bander NH | 1999 | Cancer1999，86，3： | 1 |
| 7 | Quality of lifefo llowing vertebroplasty显示文摘 | McKiernan F Faciszewski T Jensen R | 2004 | J Bone Joint Surg Am2004，86，12： | 1 |
| 8 | Latent mobility of osteoporotic bertebral compression fractures 显示文摘 | Mckiernan F Faciszewski T Jensen R | 2006 | J Vasc Interv Radiol2006，17，9： | 1 |
| 9 | Intravertebral clefts in osteoporotic vertebral compression fractures显示文摘 | McKiernan F Faciszewski T | | 0，，05： | 1 |
| 10 | Dysregulation of circulating microRNAs and prediction of aggressive prostate cancer显示文摘 | Jing Shen Gregory W. Hruby James M. McKiernan Irina Gurvich Michael Jeffrey Lipsky Mitchell C. Benson Regina M. Santella | 2012 | Prostate2012，，13： | 1 |
| 11 | The cost of treatment of skeletal-related events in patients with bone metastases from lung cancer显示文摘 | Delea T Langer C McKiernan J | 2004 | Oncology2004，67，56： | 1 |
| 12 | Calling all vertebral fractures-classification of vertebral compression fractures: a consensus for comparison of treatment and outcome 显示文摘 | Faciszewski T McKiernan F | 2002 | J Bone Miner Res2002，17，2： | 1 |
| 13 | Aberrant 'default mode' functional connectivity in schizophrenia显示文摘 | Garrity A G Pearlson G D McKiernan K | 2007 | Am J Psychiatry2007，164，3： | 1 |
| 14 | Calling all vertebral fractures classification of vertebral compression fractures:a consensus for comparison of treatment and outcome显示文摘 | Faciszewski T Mckiernan F | | 0，，： | 1 |
| 15 | Intravertebral clefts in osteoporotic vertebral compression fractures 显示文摘 | McKiernan F Faciszewski T | 2003 | Arthritis Rheum2003，48，5： | 1 |
| 16 | A controlled trial of group cognitive behaviortherapy for Irish breast cancer patients显示文摘 | McKiernan A | 2010 | J Psychosoc On-col2010，28，2： | 1 |
| 17 | Expression of the tumor-associated gene MN:a potential biomarker for human renal cell carcinoma显示文摘 | McKiernan JM Buttyan R Bander NH | 1997 | Cancer Res1997，57，12： | 1 |
| 18 | Quality of life following vertebroplasty 显示文摘 | McKiernan F Faciszewski T Jensen R | 2004 | J Bone Joint Surg Am2004，86，： | 1 |
| 19 | The fischer black hypothe- sis:some time-series evidence显示文摘 | Caporale T B McKiernan | 1998 | Southern Econom- ic Journal1998，64，： | 1 |
| 20 | Intravertebral clefts in osteoporotic vertebral compression fractures显示文摘 | McKiernan F Faciszewski T | 2003 | Arthritis Rheum2003，48，5： | 1 |
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