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| 1 | Molecular overview of progressive familial intrahepatic cholestasis显示文摘Cholestasis is a clinical condition resulting from the imapairment of bile flow.This condition could be caused by defects of the hepatocytes,which are responsible for the complex process of bile formation and secretion,and/or caused by defects in the secretory machinery of cholangiocytes.Several mutations and pathways that lead to cholestasis have been described.Progressive familial intrahepatic cholestasis(PFIC)is a group of rare diseases caused by autosomal recessive mutations in the genes that encode proteins expressed mainly in the apical membrane of the hepatocytes.PFIC 1,also known as Byler’s disease,is caused by mutations of the ATP8B1 gene,which encodes the familial intrahepatic cholestasis 1 protein.PFIC 2 is characterized by the downregulation or absence of functional bile salt export pump(BSEP)expression via variations in the ABCB11 gene.Mutations of the ABCB4 gene result in lower expression of the multidrug resistance class 3 glycoprotein,leading to the third type of PFIC.Newer variations of this disease have been described.Loss of function of the tight junction protein 2 protein results in PFIC 4,while mutations of the NR1H4 gene,which encodes farnesoid X receptor,an important transcription factor for bile formation,cause PFIC 5.A recently described type of PFIC is associated with a mutation in the MYO5B gene,important for the trafficking of BSEP and hepatocyte membrane polarization.In this review,we provide a brief overview of the molecular mechanisms and clinical features associated with each type of PFIC based on peer reviewed journals published between 1993 and 2020. | Sriram Amirneni Nils Haep Mohammad A Gad Alejandro Soto-Gutierrez James E Squires Rodrigo MFlorentino | 2020 | World Journal of Gastroenterology2020，26，47： | 17 |
| 2 | Trends and outcomes of pancreaticoduodenectomy for periampullary tumors: A 25-year single-center study of 1000 consecutive cases显示文摘AIM To evaluate the evolution, trends in surgical approaches a n d r e c o n s t r u c t i o n t e c h n i q u e s, a n d i m p o r t a n t lessons learned from performing 1000 consecutive pancreaticoduodenectomies(PDs) for periampullary tumors.METHODS This is a retrospective review of the data of all patients who underwent PD for periampullary tumor during the period from January 1993 to April 2017. The data were categorized into three periods, including early period(1993-2002), middle period(2003-2012), and late period(2013-2017).RESULTS The frequency showed PD was increasingly performed after the year 2000. With time, elderly, cirrhotic and obese patients, as well as patients with uncinate process carcinoma and borderline tumor were increasingly selected for PD. The median operative time and postoperative hospital stay decreased significantly over the periods. Hospital mortality declined significantly, from 6.6% to 3.1%. Postoperative complications significantly decreased, from 40% to 27.9%. There was significant decrease in postoperative pancreatic fistula in the second 10 years, from 15% to 12.7%. There was a significant improvement in median survival and overall survival among the periods.CONCLUSION Surgical results of PD significantly improved, with mortality rate nearly reaching 3%. Pancreatic reconstruction following PD is still debatable. The survival rate was also improved but the rate of recurrence is still high, at 36.9%. | ayman el nakeeb waleed askar ehab atef ehab el hanafy ahmad m sultan tarek salah ahmed shehta mohamed el sorogy emad hamdy mohamed el hemly ahmed a el-geidi tharwat kandil mohamed el shobari talaat abd allah amgad fouad mostafa abu zeid ahmed abu el eneen nabil gad el-hak gamal el ebidy omar fathy ahmed sultan mohamed abdel wahab | 2017 | World Journal of Gastroenterology2017，23，38： | 15 |
| 3 | 显示文摘 | Hamed A M El-Sharkawy I I | 2001 | Rene wable Energy2001，22，4： | 1 |
| 4 | The use of wood pulp and radiation modified starch in wastewater treatment显示文摘 | Abdel Aal S E Gad Y Dessouki A M | 2006 | J Appl Polym Sci2006，99，5： | 1 |
| 5 | Serelaxin, recombinant human relaxin-2, for treatment of acute heart failure (RELAX-AHF): a randomised, placebo-controlled trial显示文摘 | John R Teerlink Gad Cotter Beth A Davison G Michael Felker Gerasimos Filippatos Barry H Greenberg Piotr Ponikowski Elaine Unemori Adriaan A Voors Kirkwood F Adams Maria I Dorobantu Liliana R Grinfeld Guillaume Jondeau Alon Marmor Josep Masip Peter S Pang | 2013 | The Lancet . 2013 (9860)2013，，9860： | 1 |
| 6 | Study on desertification of irrigated arable lands in Egypt 显示文摘 | GAD A ABDEL S | 2000 | Egyptian Journal of Soil Science2000，40，3： | 1 |
| 7 | Analysis of longitudinal data with intermittent missing values using the stochastic EM algorithm显示文摘 | Gad A M Ahmed A S | 2005 | Computational Statistics and Data Analysis2005，50，10： | 1 |
| 8 | Open preperitoneal ver- sus anterior approach for recurrent inguinal hernia:a randomized study显示文摘 | Saber A Ellabban GM Gad MA | 2012 | BMC Surg2012，12，： | 1 |
| 9 | Toxoplasmosis and pregnancy显示文摘 | Chaudhry S A Gad N Koren G | 2014 | Can Fam Physician2014，60，4： | 1 |
| 10 | Thermal and thermooxidative degradation of poly(ethylene oxide)-metal salt complexes显示文摘 | Costa L Gad A M Camino G | 1992 | Macromolecules1992，25，： | 1 |
| 11 | Preoperative segmental localization of hepatic metastases:utility of three-dimensional CT during arterial portography显示文摘 | Soyer P Roche A Gad M | 1991 | Radiology1991，180，3： | 1 |
| 12 | Early conformation change and activity moclulation induced by guanidinium chloride on intestinal alkalinephosphatase显示文摘 | Miggiano GAD Mordente A Pischiutta MG at el | 1987 | Biochem J1987，284，： | 1 |
| 13 | Assessment of causes and patterns of recurrent varicose veins after surgery显示文摘 | Gad MA Saber A Hokkam EN | 2012 | N Am J Med Sci2012，4，1： | 1 |
| 14 | Radiologically assessed testicular changes in infertile males with varicocele 显示文摘 | Hassan A Gad H M Mostafa T | 2011 | Andrologia2011，43，5： | 1 |
| 15 | Modal analysis of steelframed residential structures for application to seismic design 显示文摘 | Gad E F Chandler A M | 2001 | Journal of Vibration and Control2001，17，1： | 1 |
| 16 | Oxidative stress biomarkers in the digestive gland of Theba pisana exposed to heavy metals 显示文摘 | R_adwan M A EI-Gendy K S Gad A F | 2010 | Arch Environ Contarn Toxicol2010，58，： | 1 |
| 17 | Detection of icaA, icaD genes and biofilm production by Staphylococcus aureus and Staphylococcus epidermidis isolated from urinary tract catheterized patients 显示文摘 | Gad G F E1-Feky M A E1-Rehewy M S | 2009 | J lnfect Dev Ctries2009，3，5： | 1 |
| 18 | Hepatocellular carcinoma:recent trends in Japan显示文摘 | Kiyosawa K Umemura T Ichijo T Matsumoto A Yoshizawa K Gad A | | Gastroenterology0，，： | 1 |
| 19 | In- creased prevalence of asthma in Saudi Arabia 显示文摘 | A1 Frayh A R Shakoor Z Gad E1 Rab M O | 2001 | Ann Allergy Asthma Immunol2001，86，3： | 1 |
| 20 | Lateral Behavior of Plasterboard-clad Residential Steel Frames显示文摘 | Gad E F Chandeler A M Duffield C F | 1999 | Journal of Structural Engineering1999，125，1： | 1 |
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