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| 1 | Aggressive juvenile polyposis in children with chromosome 10q23 deletion显示文摘Juvenile polyps are relatively common findings in children,while juvenile polyposis syndrome(JPS) is a rare hereditary syndrome entailing an increased risk of colorectal cancer.Mutations in BMPR1A or SMAD4 are found in roughly half of patients diagnosed with JPS.Mutations in PTEN gene are also found in patients with juvenile polyps and in Bannayan-Riley-Ruvalcaba syndrome and Cowden syndrome.Several previous reports have described microdeletions in chromosome 10q23 encompassing both PTEN and BMPR1A causing aggressive polyposis and malignancy in childhood.These reports have also described extra-intestinal findings in most cases including cardiac anomalies,developmental delay and macrocephaly.In this report we describe a boy with a 5.75 Mb deletion of chromosome 10q23 and a 1.03 Mb deletion within chromosome band 1p31.3who displayed aggressive juvenile polyposis and multiple extra-intestinal anomalies including macrocephaly,developmental delay,short stature,hypothyroidism,atrial septal defect,ventricular septal defect and hypospadias.He required colectomy at six years of age,and early colectomy was a common outcome in other children with similar deletions.Due to the aggressive polyposis and reports of dysplasia and even malignancy at a young age,we propose aggressive gastrointestinal surveillance in children with 10q23 microdeletions encompassing the BMPR1A and PTEN genes to include both the upper and lower gastrointestinal tracts,and also include a flowchart for an effective genetic testing strategy in children with juvenile polyposis. | Seth Septer Lei Zhang Caitlin E Lawson Jose Cocjin Thomas Attard Holly H Ardinger | 2013 | World Journal of Gastroenterology2013，19，14： | 4 |
| 2 | Local immunotherapy with interleuk in-2 delivered from biodegradable p olymer microspheres combined with interstitial chemotherapy:a novel treatme nt for experimental malignant glioma显示文摘OBJECTIVE:Local delivery of carmustine(BCNU)from biodegradable polymers prolon gs survival against experimental brain tumors.Moreover,paracrine administration of inte rleukin-2(IL-2)has been shown to elicit a potent antitumor immune response and to improve survival in animal brain tumor models.We r eport the use of a novel polymeric mic rosphere delivery vehicle to release IL-2.We demonstr ate both in vitro release of cytokine from the microspheres and histological evidence of the inflammatory response elicited by I L-2released from the microspheres i n the rat brain.These microspheres a re used to deliver IL-2,and biodegradable polymer wafers are used to deliver BCNU,directly at the site of an intracranially implanted glioma in the rat.The two agents administered locally show a s ynergistic effect.METHODS:Fischer 344rats challenged intracranially with 9L gliosarcoma received an intracranial implant of either empty microspheres or microspheres containing IL-2(IL-2MS ).Five days later,animals in each group were randomized to receiv e polymer implants loaded with 0,3.8,or 10%BCNU at the tumor site.RESULTS:Animals that received the combination of IL-2MS a nd 3.8%BCNU polymer(median survival,28.5d )or IL-2MS and 10%BCNU polymer(median survival,45.5d )showed significantly improved surv ival compared with animals that rece ived monotherapy with IL-2microsph eres(median survival,24d ),3.8%BCNU polymer(median survival,24d ),or 10%BCNU polymer(median survival,32.5d ).Control animals had a median survival of 18days.The combination of either 3.8or 10%BCNU polymer with IL-2MS resulted in 7and 25%long-term survivors,respectively.CONCLUSION:By showing synergy of IL-2and BCNU in an animal glioma model and using a reproducible synthetic delivery sy stem for each agent (i.e.,one that did not rely on genetic ally engineered cells or viruses),we hope that the combination of local immunotherapy and chemotherapy can take an important step closer to clinical applic ation in patients with malignant brain | Rhines LD DiMeco F Lawson HC Tyler BM Hanes J Olivi A Brem H | 2003 | 癌症2003，22，7： | 2 |
| 3 | Activation of caspase 3 (CPP32)-like proteases is essential for TNF-alpha-induced hepatic parenchymal cell apoptosis and neutrophil-mediated necrosis in a murine endotoxin shock model显示文摘 | Jaeschke H Fisher MA Lawson JA | 1998 | J Immunol1998，160，7： | 1 |
| 4 | Intestinal adenomatosis in the pig:Immunofluorescent and electron microscopic studies显示文摘 | Rowland A C Lawson G H K | 1974 | Res Vet Sci1974，17，： | 1 |
| 5 | Pregnancy outcomes after laparoscopic myomectomy with ultrasonic energy and laparoscopic suturing of the endometrial cavity显示文摘 | Stringer N H Strassner H T Lawson L | 2001 | J Am Assoc Gynecol Lap2001，8，1： | 1 |
| 6 | Pulse sensitivity of the lu- teinizing hormone beta promoter is determined by a negative feed- back loop involving early growth response - 1 and Ngfi - A binding protein 1 and 2 显示文摘 | Lawson MA Tsutsumi R Zhang H | 2007 | Mol Endocrino12007，21，5： | 1 |
| 7 | Knockdownmortality,repellency,and residual effects of insecticides for controlof adult Bactericera cockerelli (Hemiptera:Psyllidae)显示文摘 | Gharalari A H Nansen C Lawson D S | 2009 | Journalof Economic Entomology2009，102，3： | 1 |
| 8 | Proliferative enteropathy 显示文摘 | Lawson G H K Gebhart C J | 2000 | J Comp Path2000，122，： | 1 |
| 9 | Activation of caspase 3 (CPP32)-like proteases is essential for TNF-alpha-induced hepatic parenehymal cell apoptosis and neutrophil-mediated necrosis in a murine endotoxin shock model 显示文摘 | Jaeschke H Fisher MA Lawson JA | 1998 | J Immunol1998，160，7： | 1 |
| 10 | Maturity of fresh market sweet corn with direct-seeded plants,transplants,clear plastic mulch,and rowcover combinations显示文摘 | Aguyoh J Taber H G Lawson V | 1999 | HortTechnology1999，9，3： | 1 |
| 11 | The development of new brain tumor therapy utilizing the local and sustained delivery of chemotherapeutic agents from biodegradable polymers显示文摘 | Brem H Lawson HC | 1999 | Cancer1999，86，2： | 1 |
| 12 | Experimental and modeling study of hydrogen/syngas production and particulate emissions from a natural gas-fueled partial oxidation engine显示文摘 | Michael H McMillian Seth A Lawson | 2006 | J Hydrogen Energy2006，31，7： | 1 |
| 13 | Activation of resting human primary T cells with chimeric receptors: costimulation from CD28, inducible costimulator, CD134, and CD137 in series with signals from the TCR zeta chain 显示文摘 | Finney H M Akbar A N Lawson A D | 2004 | J Immunol2004，172，1： | 1 |
| 14 | Endoplasmic reticulum stress as a pro-fibrotic stimulus显示文摘 | Tanjore H Lawson WE Blackwell TS | 2013 | Biochimica et biophysica acta2013，1832，7： | 1 |
| 15 | Slimflor and slimdek construction: European developments 显示文摘 | Lawson R M Bode H Brekelmans J W P M | 1999 | The Structural Engineer1999，77，8： | 1 |
| 16 | Improved cell therapy protocols for Parkinson's disease based on diffe- rentiation efficiency and safety of hESC-, hiPSC-, and non-human primate iPSC-derived dopaminergic neurons 显示文摘 | Sundberg M Bogetofte H Lawson T | 2013 | Stein Cells2013，31，8： | 1 |
| 17 | Transverse infrahyoid approach to bilateral glottic tumors显示文摘 | REINO A J LAWSON W BILLER H F | 1999 | Ann Otol Rhinol Laryngol1999，108，： | 1 |
| 18 | On stable currents and their application to global problems in real and complex ge ometry显示文摘 | Lawson H B Simons J | 1973 | Ann of Math1973，，98： | 1 |
| 19 | The development of new brain tumor therapy utilizing the local and sustained delivery of chemotherapeutic agents from biodegradable polymers 显示文摘 | Brem H Lawson H C | 1999 | Cancer1999，86，2： | 1 |
| 20 | Coordinated induction of plasminogen activator inhibitor-1 (PAT-1) and inhibition of plasminogen activator gene expression by hypoxia promotes pulmonary vascular fibrin deposition 显示文摘 | Pinsky DJ Liao H Lawson CA | 1998 | J Clin Invest1998，102，5： | 1 |
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