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| 1 | Current and future therapies for inherited cholestatic liver diseases显示文摘Familial intrahepatic cholestasis(FIC) comprises a group of rare cholestatic liver diseases associated with canalicular transport defects resulting predominantly from mutations in ATP8B1, ABCB11 and ABCB4. Phe-notypes range from benign recurrent intrahepatic cholestasis(BRIC), associated with recurrent cholestatic attacks, to progressive FIC(PFIC). Patients often suffer from severe pruritus and eventually progressive cholestasis results in liver failure. Currently, first-line treatment includes ursodeoxycholic acid in patients with ABCB4 deficiency(PFIC3) and partial biliary diversion in patients with ATP8B1 or ABCB11 deficiency(PFIC1 and PFIC2). When treatment fails, liver transplantation is needed which is associated with complications like rejection, post-transplant hepatic steatosis and recurrence of disease. Therefore, the need for more and better therapies for this group of chronic diseases remains. Here, we discuss new symptomatic treatment options like total biliary diversion, pharmacological diversion of bile acids and hepatocyte transplantation. Furthermore, we focus on emerging mutation-targeted therapeutic strategies, providing an outlook for future personalized treatment for inherited cholestatic liver diseases. | Wendy L van der Woerd Roderick HJ Houwen Stan FJ van de Graaf | 2017 | World Journal of Gastroenterology2017，23，5： | 11 |
| 2 | Colonic manifestations of PTEN hamartoma tumor syndrome: Case series and systematic review显示文摘AIM:To investigate our clinical experience with the colonic manifestations of phosphatase and tensin homolog on chromosome ten(PTEN)hamartoma tumor syndrome(PHTS)and to perform a systematic literature review regarding the same.METHODS:This study was approved by the appropriate institutional review board prior to initiation.A clinical genetics database was searched for patients with PHTS or a component syndrome that received gastrointestinal endoscopy or pathology interpretation at our center.These patient’s records were retrospectively reviewed for clinical characteristics(including family history and genetic testing),endoscopy results and pathology findings.We also performed a systematic review of the literature for case series of PHTS or component syndromes that reported gastrointestinal manifestations and investigations published after consensus diagnostic criteria were established in 1996.These results were compiled and reported.RESULTS:Eight patients from our institution met initial inclusion criteria.Of these,5 patients underwent4.2 colonoscopies at mean age 45.8±10.8 years.All were found to have colon polyps during their clinical course and polyp histology included adenoma,hyperplastic,ganglioneuroma and juvenile.No malignant lesions were identified.Two had multiple histologic types.One patient underwent colectomy due to innumerable polyps and concern for future malignant potential.Systematic literature review of PHTS patients undergoing endoscopy revealed 107 patients receiving colonoscopy at mean age 37.4 years.Colon polyps were noted in92.5%and multiple colon polyp histologies were reported in 53.6%.Common polyp histologies included hyperplastic(43.6%),adenoma(40.4%),hamartoma(38.3%),ganglioneuroma(33%)and inflammatory(24.5%)polyps.Twelve(11.2%)patients had colorectal cancer at mean age 46.7 years(range 35-62).Clinical outcomes secondary to colon polyposis and malignancy were not commonly reported.CONCLUSION:PHTS has a high prevalence of colon polyposis with multiple histologic types.It should be considered a mixed polyposis syndrome.Systematic review found an increased prevalence of colorectal cancer and we recommend initiating colonoscopy for colorectal cancer surveillance at age 35 years. | Peter P Stanich Robert Pilarski Jonathan Rock Wendy L Frankel Samer El-Dika Marty M Meyer | 2014 | World Journal of Gastroenterology2014，20，7： | 5 |
| 3 | Antibiotic Resistance and Heavy Metals Tolerance in Gram-Negative Bacteria from Diseased American Bullfrog (Rana catesbeiana) Cultured in Malaysia显示文摘A total of 140 bacterial isolates have been successfully isolated from various organs of diseased American bullfrog (Rana catesbeiana) cultured in Malaysia. The most frequently isolated bacteria was Edwardsiella spp. (46 isolates) followed by Aeromonas spp. (33 isolates), Flavobacterium spp. (31 isolates), and Vibrio spp. (30 isolates). Majority of the bacterial isolates were found sensitive to furazolidone (85.0%), chloramphenicol (85.0%), oxolinic acid (90.0%), florfenicol (95.0%), and flumequine (97.5%). On the other hand, most of the bacterial isolates were resistant to oleandomycin (77.5%) and lincomycin (87.5%). Nitrofurantoin and flumequine can be inhibited the growth of all of Vibrio spp. whereas all isolates of Edwardsiella spp. were found sensitive to florfenicol and flumequine. Multiple antibiotic resistance (MAR) index were in range of 0.30-0.40, indicating that bacterial isolates from cultured bullfrogs may have received high risk exposure to the tested antibiotics. In addition, 90-100% of the isolates were resistant to copper, cadmium, and chromium. These results provided insight information on tolerance level of bacterial isolates from cultured bullfrogs to 21 antibiotics as well as heavy metals. | M Najiah S W Lee W Wendy L W Tee M Nadirah S H Faizah | 2009 | Agricultural Sciences in China2009，8，10： | 4 |
| 4 | Gynecologic oncologists involvement on ovarian cancer standard of care receipt and survival显示文摘AIM:To examine the influence of gynecologic oncolo-gists(GO) in the United States on surgical/chemotherapeutic standard of care(SOC), and how this translates into improved survival among women with ovarian cancer(OC).METHODS: Surveillance, Epidemiology, and End Result(SEER)-Medicare data were used to identify 11688 OC patients(1992-2006). Only Medicare recipients with an initial surgical procedure code(n = 6714) were included. Physician specialty was identified by linking SEER-Medicare to the American Medical Association Masterfile. SOC was defined by a panel of GOs. Multivariate logistic regression was used to determine predictors of receiving surgical/chemotherapeutic SOCand proportional hazards modeling to estimate the effect of SOC treatment and physician specialty on survival. RESULTS: About 34% received surgery from a GO and 25% received the overall SOC. One-third of women had a GO involved sometime during their care. Women receiving surgery from a GO vs non-GO had 2.35 times the odds of receiving the surgical SOC and 1.25 times the odds of receiving chemotherapeutic SOC(P < 0.01). Risk of mortality was greater among women not receiving surgical SOC compared to those who did [hazard ratio = 1.22(95%CI: 1.12-1.33), P < 0.01], and also was higher among women seen by non-GOs vs GOs(for surgical treatment) after adjusting for covariates. Median survival time was 14 mo longer for women receiving combined SOC. CONCLUSION: A survival advantage associated with receiving surgical SOC and overall treatment by a GO is supported. Persistent survival differences, particularly among those not receiving the SOC, require further investigation. | Sun Hee Rim Shawn Hirsch Cheryll C Thomas Wendy R Brewster Darryl Cooney Trevor D Thompson Sherri L Stewart | 2016 | World Journal of Obstetrics and Gynecology2016，5，2： | 3 |
| 5 | Human cord blood-derived cells can differentiate into hepatocytes in the mouse liver with no evidence of cellular fusion显示文摘 | Philip N Newsome Ingolfur Johannessen Shelagh Boyle Evangelos Dalakas Karen A Mcaulay Kay Samuel Frances Rae Lesley Forrester Marc L Turner Peter C Hayes David J Harrison Wendy A Bickmore John N Plevris | 2003 | Gastroenterology2003，，7： | 2 |
| 6 | Diagnosis and management of bile leaks after blunt liver injary显示文摘 | Wendy L Wahl Mary-Margaret Brandt Mark R Hetnmila | 2005 | Surgery2005，138，4： | 1 |
| 7 | Understanding and Managing the Service Supply Chain 显示文摘 | LISA M ELLRAM WENDY L T COREY B | 2004 | Journal of Supply Chain Management2004，40，4： | 1 |
| 8 | Vasopressin: a new target for the treatment of heart failure显示文摘 | Craig R Lee Michael L Watkins J.Herbert Patterson Wendy Gattis Christopher M O'Connor Mihai Gheorghiade Kirkwood F Adams | 2003 | American Heart Journal2003，，1： | 1 |
| 9 | Managemnent strategy impacts on ammonia volatilization from swine manure 显示文摘 | Daine M P Wendy J P Jeffery C L | 2005 | J Environ Qual2005，34，： | 1 |
| 10 | Coal desulfurization with sodium hypochlorite显示文摘 | WENDI L | 2005 | Energy & Fuels2005，19，2： | 1 |
| 11 | Mapping of the snuffbox and cubital vessels for percutaneous arterial venous fistula (pAVF) in dialysis patients显示文摘 | Jeffrey E Hull Wendy L Bishop | 2013 | The Journal of Vascular &ccess2013，14，3： | 1 |
| 12 | Management of general surgical problems in the pregnant patient显示文摘 | David M Melnick Wendy L Wahl Vanessa K Dalton | 2004 | The American Journal of Surgery2004，，2： | 1 |
| 13 | QoS-aware routing based on bandwidth estimation for mobile Ad Hoc networks显示文摘 | Chen L Heinzelman Wendi B | 2005 | IEEE Journal on Selected Areas in Communications2005，23，2： | 1 |
| 14 | Coping in middle childhood: Relations to competence, Type A behavior, monitoring, bluntingand locus of control显示文摘 | Liewer L Wendy J | 1991 | Developmental Psychology1991，27，4： | 1 |
| 15 | PTEN suppresses breastcancer cellgrowth by phosphatase activity dependent G1 arrest followed by cell death显示文摘 | Weng LP Wendy M Patricia L | | 0，，22： | 1 |
| 16 | Understanding and managing the service supply chain显示文摘 | Lisa M Ellram Wendy L T Comy B | 2004 | Journal of Supply Chain Management2004，，： | 1 |
| 17 | Multicentric and Multffocal Cancer: Whole-Breast US in preoperative Evaluation显示文摘 | Wendie A Berg Patrical L Glibreath | 2000 | Radiology2000，214，： | 1 |
| 18 | Understanding and managing the services supply chain显示文摘 | LISA M E WENDY L T COREY B | 2004 | Journal of Supply Chain Management2004，40，4： | 1 |
| 19 | Raw or heated cow milk consumption :Review of risks and benefits显示文摘 | Wendie L Sabine C Georges D | 2013 | Food Control2013，31，： | 1 |
| 20 | The utility of es-trogen receptor and progesterone receptor immunohisto-chemistry in the distinction of metastatic breast carcino-ma from other tumors in the liver 显示文摘 | Nash J W Morrison C Wendy L | 2003 | Arch Pathol Lab2003，127，12： | 1 |
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