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    Chir99021 and Valproic acid reduce the proliferative advantage of Apc mutant cells.
    Cell Death Dis 2018 Feb 15;9(3):255. Epub 2018 Feb 15.
    Cell & Developmental Biology, School of Life Sciences, University of Dundee, Dundee, DD1 5EH, Scotland.
    More than 90% of colorectal cancers carry mutations in Apc that drive tumourigenesis. A 'just-right' signalling model proposes that Apc mutations stimulate optimal, but not excessive Wnt signalling, resulting in a growth advantage of Apc mutant over wild-type cells. Reversal of this growth advantage constitutes a potential therapeutic approach. Read More

    Interaction of the Wnt/β-catenin and RAS-ERK pathways involving co-stabilization of both β-catenin and RAS plays important roles in the colorectal tumorigenesis.
    Adv Biol Regul 2018 Jan 10. Epub 2018 Jan 10.
    Translational Research Center for Protein Function Control, Yonsei University, Seoul, South Korea; Department of Biotechnology, College of Life Science and Biotechnology, Yonsei University, Seoul, South Korea. Electronic address:
    Cancer development is usually driven by multiple genetic and molecular alterations rather than by a single defect. In the human colorectal cancer (CRC), series of mutations of genes are involved in the different stages of tumorigenesis. For example, adenomatous polyposis coli (APC) and KRAS mutations have been known to play roles in the initiation and progression of the tumorigenesis, respectively. Read More

    IKKα is required in the intestinal epithelial cells for tumour stemness.
    Br J Cancer 2018 Feb 13. Epub 2018 Feb 13.
    Cancer Research Program, Institut Mar d'Investigacions Mèdiques, CIBERONC, Hospital del Mar, Doctor Aiguader 88, 08003 Barcelona, Spain.
    Background: Colorectal cancer is a common cause of death in developed countries. Progression from adenoma to invasive carcinoma requires accumulation of mutations starting with the Adenomatous Polyposis Coli (Apc) gene. NF-κB signalling is a key element in cancer, mainly related to the activity of IKKβ. Read More

    Anti-inflammatory drug resistance selects putative cancer stem cells in a cellular model for genetically predisposed colon cancer.
    Oncol Lett 2018 Jan 6;15(1):642-648. Epub 2017 Oct 6.
    Cancer Prevention Research Program, Palindrome Liaisons Consultants, Montvale, NJ 07645-1559, USA.
    Mutations in the adenomatous polyposis coli (Apc) tumor suppressor gene represent the primary genetic defect in colon carcinogenesis. Apcmouse models exhibit pre-invasive small intestinal adenomas. Cell culture models exhibiting Apc defects in the colon and quantifiable cancer risk provide a novel clinically relevant approach. Read More

    Mantle Cell Lymphoma with a Single Protruding Lesion as the Cause of Intussusception.
    Intern Med 2018 Feb 9. Epub 2018 Feb 9.
    Department of Gastroenterology, National Hospital Organization Fukuyama Medical Center, Japan.
    Mantle cell lymphoma (MCL) is a malignant lymphoma of the gastrointestinal tract that mostly presents as multiple lymphomatous polyposis (MLP); however, MLP with intussusception is rarely reported in MCL. Furthermore, a single protruding lesion with intussusception has never been reported in primary small intestinal MCL. A 70-year-old man presented with pain in the right lower abdomen. Read More

    DKK2 imparts tumor immunity evasion through β-catenin-independent suppression of cytotoxic immune-cell activation.
    Nat Med 2018 Feb 12. Epub 2018 Feb 12.
    Vascular Biology and Therapeutic Program and Department of Pharmacology, Yale School of Medicine, New Haven, Connecticut, USA.
    Immunotherapy offers new options for cancer treatment, but efficacy varies across cancer types. Colorectal cancers (CRCs) are largely refractory to immune-checkpoint blockade, which suggests the presence of yet uncharacterized immune-suppressive mechanisms. Here we report that the loss of adenomatosis polyposis coli (APC) in intestinal tumor cells or of the tumor suppressor PTEN in melanoma cells upregulates the expression of Dickkopf-related protein 2 (DKK2), which, together with its receptor LRP5, provides an unconventional mechanism for tumor immune evasion. Read More

    [Concurrent APC and MLH1mutations identified in a family affected with familial adenomatous polyposis].
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2018 Feb;35(1):84-88
    Department of Obstetrics and Gynecology, Department of Pathology, Peking University First Hospital, Beijing 100034, China. Email:
    OBJECTIVE To report on concurrent mutations of APC and MLH1 genes identified in a family affected with familial adenomatous polyposis(FAP). METHODS The proband was diagnosed with FAP based on her clinical manifestation, family history and histopathology examination. She developed endometrial epithelial neoplasia(EIN) two years later. Read More

    Adenocarcinoma in a Blind Loop of the Ileum 53 Years After an Ileotransversostomy Procedure.
    Am J Case Rep 2018 Feb 6;19:133-136. Epub 2018 Feb 6.
    Department of Surgery, National Hospital Organization Kanazawa Medical Center, Kanazawa, Ishikawa, Japan.
    BACKGROUND Primary small bowel cancer is a rare malignancy; the common histopathological types are carcinoid and adenocarcinoma. Inflammatory bowel diseases and familial adenomatous polyposis are known risk factors for small bowel cancer. Additionally, cases of surgery-induced small bowel adenocarcinoma are sometimes reported after ileostomy. Read More

    Insights into insulin resistance, lifestyle, and anthropometric measures of patients with prior colorectal cancer compared to controls: A National Health and Nutrition Examination Survey (NHANES) Study.
    Curr Probl Cancer 2018 Jan 5. Epub 2018 Jan 5.
    Section of Intestinal Neoplasia and Hereditary Polyposis (INHP), Division of Gastroenterology, Hepatology and Nutrition, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH; Comprehensive Cancer Center, The Ohio State University, Columbus, OH. Electronic address:
    Background: Insulin resistance (IR) increases the risk of index colorectal cancer (CRC) development. Limited data exist on IR values, lifestyle, and anthropometric alterations of patients after CRC diagnosis, a population at high risk for CRC recurrence.

    Methods: This is a retrospective cohort study using the National Health and Nutrition Examination Survey (NHANES), 1999-2010. Read More

    Mast Cell-Dependent CD8T-cell Recruitment Mediates Immune Surveillance of Intestinal Tumors in ApcMice.
    Cancer Immunol Res 2018 Jan 30. Epub 2018 Jan 30.
    James Graham Brown Cancer Center, University of Louisville Health Sciences Center, Louisville, Kentucky.
    The presence of mast cells in some human colorectal cancers is a positive prognostic factor, but the basis for this association is incompletely understood. Here, we found that mice with a heterozygous mutation in thedisplayed reduced intestinal tumor burdens and increased survival in a chemokine decoy receptor, ACKR2-null background, which led to discovery of a critical role for mast cells in tumor defense. ACKR2Apctumors showed increased infiltration of mast cells, their survival advantage was lost in mast cell-deficient ACKR2SAApcmice as the tumors grew rapidly, and adoptive transfer of mast cells restored control of tumor growth. Read More

    The Yin and Yang of Invariant Natural Killer T Cells in Tumor Immunity-Suppression of Tumor Immunity in the Intestine.
    Front Immunol 2017 10;8:1945. Epub 2018 Jan 10.
    Department of Microbiology and Immunology, University of Gothenburg, Gothenburg, Sweden.
    CD1d-restricted invariant natural killer T (iNKT) cells are known as early responding, potent regulatory cells of immune responses. Besides their established role in the regulation of inflammation and autoimmune disease, numerous studies have shown that iNKT cells have important functions in tumor immunosurveillance and control of tumor metastasis. Tumor-infiltrating T helper 1 (TH1)/cytotoxic T lymphocytes have been associated with a positive prognosis. Read More

    Gamma-glutamyltransferase, possible novel biomarker in colon diverticulosis: a case-control study.
    J Enzyme Inhib Med Chem 2018 Dec;33(1):428-432
    c Faculty of Medecine , Titu Maiorescu University , Bucharest , Romania.
    The gamma-glutamyltransferase (GGT) is recognized in medical practice as a useful indicator for the detection of liver lesions, especially those induced by the excessive consumption of alcoholic or cholesterol-associated drinks. The present study, although it includes a very small number of cases diagnosed with colon diverticulosis-diverticulitis associated with polyposis at the same intestinal level, identifies the presence of increased circulating concentrations of this enzyme in the serum. Its serum levels are tracked "dynamically" throughout a year after the diagnosis and start of the therapy. Read More

    Autophagic flux is essential for the downregulation of D-dopachrome tautomerase by atractylenolide I to ameliorate intestinal adenoma formation.
    J Cell Commun Signal 2018 Jan 24. Epub 2018 Jan 24.
    School of Traditional Chinese Medicine, Southern Medical University, Guangzhou, 510515, China.
    Colorectal cancer is generally believed to progress through an adenoma - carcinoma sequence. Adenomatous polyposis coli (APC) mutations serve as the initiating event in adenoma formation. The Apcmouse harbors a mutation in the APC gene, which is similar or identical to the mutation found in individuals with familial adenomatous polyposis and 70% of all sporadic CRC cases. Read More

    [Consensus on clinical diagnosis, treatment and pedigree management of hereditary colorectal cancer in China].
    • Authors:
    Zhonghua Zhong Liu Za Zhi 2018 Jan;40(1):64-77
    Hereditary colorectal cancer can be divded into two categories based on the presence or absence of polyps. The first category is characterized by the development of polyposis, which includes familial adenomatous polyposis (FAP); The second category is nonpolyposis colorectal cancer, which is represented by Lynch syndrome. "Consensus on clinical diagnosis, treatment and pedigree management of hereditary colorectal cancer in China" developed by the Genetics Group of the Committee of Colorectal Cancer, Chinese Anti-cancer Association, is composed of three sections, including hereditary nonpolyposis syndrome, polyposis syndrome as well as genetic evaluation of hereditary colorectal cancer. Read More

    Cyclic-GMP-Elevating Agents Suppress Polyposis inmice by Targeting the Preneoplastic Epithelium.
    Cancer Prev Res (Phila) 2018 Feb 4;11(2):81-92. Epub 2018 Jan 4.
    Department of Biochemistry and Molecular Biology, Augusta University, Augusta, Georgia.
    The cGMP signaling axis has been implicated in the suppression of intestinal cancers, but the inhibitory mechanism and the extent to which this pathway can be targeted remains poorly understood. This study has tested the effect of cGMP-elevating agents on tumorigenesis in themouse model of intestinal cancer. Treatment ofmice with the receptor guanylyl-cyclase C (GCC) agonist linaclotide, or the phosphodiesterase-5 (PDE5) inhibitor sildenafil, significantly reduced the number of polyps per mouse (67% and 50%, respectively). Read More

    Multivisceral transplant is a viable treatment option for patients with non-resectable intra-abdominal fibromatosis.
    Clin Transplant 2017 Dec 30. Epub 2017 Dec 30.
    Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
    Background: Intra-abdominal fibromatosis often involves the mesentery root which is non-resectable by conventional surgery. Multivisceral transplant (MVT), as a potential cure to non-resectable fibromatosis, has rarely been reported and the prognosis is unknown.

    Methods: Six patients who underwent MVT for intra-abdominal fibromatosis were reviewed. Read More

    Germlinevariants are enriched in-wildtype Bannayan-Riley-Ruvalcaba syndrome.
    NPJ Genom Med 2017 18;2:37. Epub 2017 Dec 18.
    Genomic Medicine Institute, Cleveland Clinic, Cleveland, OH 44195 USA.
    Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare congenital disorder classically characterized by macrocephaly in combination with intestinal hamartomatous polyposis, vascular malformations, lipomas, and genital lentiginosis. Germlinemutations have been reported in up to 60% of BRRS patients. The remaining cases are of unknown genetic etiology. Read More

    A novel germline mutation (c.A527G) in STK11 gene causes Peutz-Jeghers syndrome in a Chinese girl: A case report.
    Medicine (Baltimore) 2017 Dec;96(49):e8591
    aDepartment of Medical Genetics, Naval Medical University, Shanghai 200433, ChinabHebei North University, Zhangjiakou, Hebei Province, ChinacDepartment of Gastroenterology, Airforce General Hospital of PLA, Beijing, China.
    Rationale: Peutz-Jeghers syndrome (PJS) is a Mendelian autosomal dominant disease caused by mutations in the tumor suppressor gene, serine/threonine kinase 11 (STK11). The features of this syndrome include gastrointestinal (GI) hamartomas, melanin spots on the lips and the extremities, and an increased risk of developing cancer. Early onset of disease is often characterized by mucocutaneous pigmentation and intussusception due to GI polyps in childhood. Read More

    A giant and extensive solitary Peutz-Jeghers-type polyp in the antrum of stomach: Case report.
    Medicine (Baltimore) 2017 Dec;96(49):e8466
    aDepartment of Gastroenterology, the Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, ChinabDepartment of Gastroenterology, Xi'an Children's Hospital, Xi'an, Shaanxi, China.
    Rationale: A solitary Peutz-Jeghers-type polyp is a hamartomatous polyp which without either mucocutaneous pigmentation or a family history of Peutz-Jeghers syndrome (PJS). It can occur in all of the gastrointestinal tract, but it is extremely rare in the stomach.

    Patient Concerns: A 53-year-old man was admitted to the local hospital with left upper abdominal pain lasting 2 weeks. Read More

    Duodenal Adenomas in Patients With Multiple Colorectal Adenomas Without Germline APC or MUTYH Mutations.
    Dis Colon Rectum 2018 Jan;61(1):58-66
    Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
    Background: Patients with genetic adenomatous polyposis syndromes have an increased risk for duodenal cancer, and clear surveillance recommendations exist for this group. However, limited data are available on the duodenal phenotype of patients with multiple colorectal adenomas (10-99) without a germline APC or MUTYH mutation.

    Objective: We aimed to assess the frequency, extent, and progression of duodenal adenomas in patients with multiple colorectal adenomas without a germline APC or MUTYH mutation. Read More

    A novel bioactive derivative of eicosapentaenoic acid (EPA) suppresses intestinal tumor development in ApcΔ14/+ mice.
    Carcinogenesis 2017 Dec 1. Epub 2017 Dec 1.
    University of Connecticut Health, Farmington, CT.
    Familial adenomatous polyposis (FAP) is a genetic disorder characterized by the development of hundreds of polyps throughout the colon. Without prophylactic colectomy, most individuals with FAP develop colorectal cancer at an early age. Treatment with eicosapentaenoic acid (EPA) in the free fatty acid form (EPA-FFA) has been shown to reduce polyp burden in FAP patients. Read More

    Cronkhite-Canada syndrome causing pouch outlet obstruction 5 years after roux-en-y gastric bypass.
    Acta Chir Belg 2017 Dec 5:1-3. Epub 2017 Dec 5.
    a Dienst Abdominale, Kinder- en Reconstructieve Heelkunde , UZA , Antwerpen , Belgium.
    Objective: We present the case of a 57-year-old man with a history of Roux-en-Y gastric bypass (RYGB) and colonic polyps who presented with an upper gastrointestinal obstruction based on massive stomach polyposis in the pouch.

    Methods: Two months prior to this acute admission, he had undergone resection of the gastric remnant due to massive refractory intraluminal bleeding from a polypoid mass. Ten years earlier, right colectomy was performed due to hypertrophic polyposis unsuitable for endoscopic polypectomy. Read More

    [Advance in research on the correlation between genotypes of susceptible mutations and clinical phenotype of familial adenomatous polyposis].
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2017 Dec;34(6):919-923
    Department of Colorectal Surgery, Fudan University Shanghai Cancer Center, Shanghai 200032, China. Email:
    Familial adenomatous polyposis (FAP) is one of the most common hereditary colorectal cancers. Its intestinal and extra-intestinal manifestations are correlated with mutation sties of the APC gene. Potential gene modulation sites in patients who have typical clinical manifestations but with unidentified APC mutations are also discussed, which included MUTYH gene, AXIN gene and certain epigenetic changes. Read More

    [Current status of hereditary gastrointestinal neoplasms].
    Zhonghua Wei Chang Wai Ke Za Zhi 2017 Nov;20(11):1222-1226
    Gastrointestinal Surgery Center, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China.
    Gastric cancer and colorectal cancer are two common malignant tumors in digestive tract. 1% to 3% of gastric cancer and 5% to 15% of colorectal cancer are definitely hereditary cancer caused by germline gene mutation. Hereditary gastric cancer includes hereditary diffuse gastric cancer(HDGC) and hereditary intestinal gastric cancer (HIGC). Read More

    Polyposis deserves a perfect physical examination for final diagnosis: Bannayan-Riley-Ruvalcaba syndrome.
    Turk J Pediatr 2017 ;59(1):80-83
    Divisions of Pediatric Gastroenterology, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
    Hızarcıoğlu-Gülşen H, Kılıç E, Dominguez-Garrido E, Aydemir Y, Utine GE, Saltık-Temizel İN. Polyposis deserves a perfect physical examination for final diagnosis: Bannayan-Riley-Ruvalcaba syndrome. Turk J Pediatr 2017; 59: 80-83. Read More

    WNT Inhibitory Activity of Malus Pumila miller cv Annurca and Malus domestica cv Limoncella Apple Extracts on Human Colon-Rectal Cells Carrying Familial Adenomatous Polyposis Mutations.
    Nutrients 2017 Nov 18;9(11). Epub 2017 Nov 18.
    Department of Pharmacy, University of Naples Federico II, 80131 Naples, Italy.
    Inhibitors of the Wingless-related Integration site (WNT)/β-catenin pathway have recently been under consideration as potential chemopreventive agents against Familial Adenomatous Polyposis (FAP). This autosomal-dominant syndrome is caused by germline mutations in the gene coding for the protein APC and leads to hyperactivation of the WNT/β-catenin signaling pathway, uncontrolled intestinal cell proliferation and formation of adenocarcinomas. The aim of the present work was to: (i) test, on in vitro cultures of cells carrying FAP mutations and on ex vivo biopsies of FAP patients, the WNT inhibitory activity of extracts from two common southern Italian apples,cv. Read More

    Sanger sequencing in exonic regions of STK11 gene uncovers a novel de-novo germline mutation (c.962_963delCC) associated with Peutz-Jeghers syndrome and elevated cancer risk: case report of a Chinese patient.
    BMC Med Genet 2017 11 15;18(1):130. Epub 2017 Nov 15.
    Department of Medical Genetics, Naval Medical University, 800 Xiangyin Rd, Shanghai, 200433, China.
    Background: Peutz-Jeghers syndrome (PJS) is caused by mutations in the tumor suppressor gene, STK11, and is characterized by gastrointestinal hamartomas, melanin spots on the lips and the extremities, and an increased risk of developing cancer.

    Case Presentation: We reported an isolated PJS patient who died of colon cancer, whose blood sample was collected together with all the available family members'. The entire coding region of the STK11 gene was amplified by PCR and analyzed by Sanger sequencing, through which, a novel mutation, c. Read More

    Effects of a Grapevine Shoot Extract Containing Resveratrol and Resveratrol Oligomers on Intestinal Adenoma Development in Mice: In Vitro and In Vivo Studies.
    Mol Nutr Food Res 2018 Jan 9;62(2). Epub 2018 Jan 9.
    Institute for Food Toxicology, University of Veterinary Medicine Hannover, Hannover, Germany.
    Scope: Evidence suggests that the dietary consumption of plant extracts containing polyphenols might help prevent the onset of cancers of the gastrointestinal tract. In the present study, the chemopreventive and antiproliferative efficacy of a grapevine shoot extract (Vineatrol®30) containing resveratrol and resveratrol oligomers is investigated in vivo and in vitro.

    Methods And Results: The in vivo study is performed using Apcmice on a high-fat diet, which represents a model of human adenomatous polyposis, while the potential of the extract as well as some of its isolated constituents to inhibit intestinal adenoma cell proliferation in vitro is investigated using APC10. Read More

    Neoplastic Lesions of Gastric Adenocarcinoma and Proximal Polyposis Syndrome (GAPPS) Are Gastric Phenotype.
    Am J Surg Pathol 2018 Jan;42(1):1-8
    PathWest Laboratory Medicine (QE 2 Medical Centre) and University of Western Australia.
    Neoplastic lesions of gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) are gastric phenotype. GAPPS was reported in 2011 as a new autosomal dominant gastric polyposis syndrome characterized by involvement of the gastric body/fundus with sparing of the antrum by multiple polyps, reported to be primarily fundic gland polyps (FGPs), with progression to dysplasia and adenocarcinoma of intestinal type. Our series consists of 51 endoscopic biopsies and 5 gastrectomy specimens from 25 patients belonging to a previously defined GAPPS family. Read More

    Emerging Concepts in Gastric Neoplasia: Heritable Gastric Cancers and Polyposis Disorders.
    Surg Pathol Clin 2017 Dec;10(4):931-945
    Department of Pathology, Centro Hospitalar de São João, Porto, Portugal; Department of Pathology, Faculty of Medicine of the University of Porto (FMUP), Alameda Professor Hernâni Monteiro, Porto 4200-319, Portugal; Institute of Molecular Pathology and Immunology of the University of Porto (Ipatimup), Rua Júlio Amaral de Carvalho, 45, Porto 4200-135, Portugal; Institute for Research Innovation in Health (i3S), Rua Júlio Amaral de Carvalho, 45, Porto 4200-135, Portugal.
    Hereditary gastric cancer is a relatively rare disease with specific clinical and histopathologic characteristics. Hereditary gastric cancer of the diffuse type is predominantly caused by germline mutations in CDH1. The inherited cause of familial intestinal gastric cancer is unknown. Read More

    Role of GALNT12 in the genetic predisposition to attenuated adenomatous polyposis syndrome.
    PLoS One 2017 2;12(11):e0187312. Epub 2017 Nov 2.
    Laboratorio de Oncología Molecular, CIBERONC, IdISSC, Hospital Clínico San Carlos, Madrid, Spain.
    The involvement of GALNT12 in colorectal carcinogenesis has been demonstrated but it is not clear to what extent it is implicated in familial CRC susceptibility. Partially inactivating variant, NM_024642.4:c. Read More

    Cap polyposis refractory toeradication treated with endoscopic submucosal dissection.
    World J Gastrointest Endosc 2017 Oct;9(10):529-534
    Department of Gastroenterology, Shiga University of Medical Science Hospital, Otsu 520-2192, Japan.
    Cap polyposis is a rare intestinal disorder. Characteristic endoscopic findings are multiple inflammatory polypoid lesions covered by caps of fibrous purulent exudate. Although a specific treatment has not been established, some studies have suggested that eradication therapy for() is effective. Read More

    Nail Matrix Pathology in Cronkhite-Canada Syndrome: The First Case Report.
    Am J Dermatopathol 2017 Nov;39(11):860-862
    *Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand; and†Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
    Cronkhite-Canada Syndrome (CCS) presents with gastrointestinal polyposis and the triad of cutaneous abnormalities including nail dystrophy, alopecia, and hyperpigmentation of the skin. The etiology is not well understood. The histology of skin lesion in CCS has not been routinely described. Read More

    The TLR3/TICAM-1 signal constitutively controls spontaneous polyposis through suppression of c-Myc in Apcmice.
    J Biomed Sci 2017 Oct 17;24(1):79. Epub 2017 Oct 17.
    Department of Vaccine Immunology, Hokkaido University Graduate School of Medicine, Kita 15, Nishi 7, Kita-ku, Sapporo, 060-8638, Japan.
    Background: Intestinal tumorigenesis is promoted by myeloid differentiation primary response gene 88 (MyD88) activation in response to the components of microbiota in Apcmice. Microbiota also contains double-stranded RNA (dsRNA), a ligand for TLR3, which activates the toll-like receptor adaptor molecule 1 (TICAM-1, also known as TRIF) pathway.

    Methods: We established ApcTicam1mice and their survival was compared to survival of ApcMyd88and wild-type (WT) mice. Read More

    Surveillance of Duodenal Polyposis in Familial Adenomatous Polyposis: Should the Spigelman Score Be Modified?
    Dis Colon Rectum 2017 Nov;60(11):1137-1146
    1 Department of Digestive Surgery, Hôpital Saint-Antoine, Paris, France 2 Mater Misericordiae University Hospital, Dublin, Ireland 3 Laboratory of Angiogenetics and Oncogenetics, Hôpital Pitié-Salpétrière, Paris, France 4 Centre de Prise en Charge Multidisciplinaire des Personnes Predisposes Héréditairement au Cancer Colorectal, Hôpital Saint-Antoine, Paris, France 5 Department of Digestive Endoscopy, Hôpital Saint-Antoine, Université Pierre et Marie Curie, Paris, France.
    Background: Duodenal polyposis is a manifestation of adenomatous polyposis that predisposes to duodenal or ampullary adenocarcinoma. Duodenal polyposis is monitored by upper GI endoscopies and may require iterative resections and prophylactic radical surgical treatment when malignancy is threatening.

    Objective: The purpose of this study was to evaluate severity scoring for surveillance and treatment in a large series of duodenal polyposis. Read More

    A 23-Nucleotide Deletion in STK11 Gene Causes Peutz-Jeghers Syndrome and Malignancy in a Chinese Patient Without a Positive Family History.
    Dig Dis Sci 2017 11 6;62(11):3014-3020. Epub 2017 Oct 6.
    Department of Medical Genetics, Naval Medical University, 800 Xiangyin Rd., Shanghai, 200433, China.
    Background And Aims: Peutz-Jeghers syndrome (PJS) is an autosomal-dominant genetic disease caused by mutations in the tumor suppressor gene, STK11, which is characterized by gastrointestinal hamartomas, melanin spots on the lips and the extremities, and an increased risk of developing both gastrointestinal and extraintestinal malignancies.

    Methods And Results: We treated a PJS patient without a positive family history, who possessed typical clinical manifestations including polyp canceration. In order to explore the genotype of this patient, blood samples were collected from all the available family members. Read More

    Interkinetic nuclear migration and basal tethering facilitates post-mitotic daughter separation in intestinal organoids.
    J Cell Sci 2017 Nov 5;130(22):3862-3877. Epub 2017 Oct 5.
    Cell & Developmental Biology, University of Dundee, Dundee DD1 5EH, UK
    Homeostasis of renewing tissues requires balanced proliferation, differentiation and movement. This is particularly important in the intestinal epithelium where lineage tracing suggests that stochastic differentiation choices are intricately coupled to the position of a cell relative to a niche. To determine how position is achieved, we followed proliferating cells in intestinal organoids and discovered that the behaviour of mitotic sisters predicted long-term positioning. Read More

    Adenomatous Polyposis Coli Defines Treg Differentiation and Anti-inflammatory Function through Microtubule-Mediated NFAT Localization.
    Cell Rep 2017 Oct;21(1):181-194
    Institut Pasteur, Department of Immunology, Lymphocyte Cell Biology Unit, 75015 Paris, France; CNRS URA1961, 75015 Paris, France; INSERM U1221, 75015 Paris, France. Electronic address:
    Adenomatous polyposis coli (APC) is a polarity regulator and tumor suppressor associated with familial adenomatous polyposis and colorectal cancer development. Although extensively studied in epithelial transformation, the effect of APC on T lymphocyte activation remains poorly defined. We found that APC ensures T cell receptor-triggered activation through Nuclear Factor of Activated T cells (NFAT), since APC is necessary for NFAT's nuclear localization in a microtubule-dependent fashion and for NFAT-driven transcription leading to cytokine gene expression. Read More

    Magnetic Compression Anastomosis (Magnamosis) for Functional Undiversion of Ileostomy in Pediatric Patients.
    J Laparoendosc Adv Surg Tech A 2017 Dec 4;27(12):1314-1317. Epub 2017 Oct 4.
    2 Department of Pediatric Surgery, University of California , San Francisco, San Francisco, California.
    Introduction: Magnamosis forms a compression anastomosis using self-aligning magnetic Harrison rings. The device has been approved by the Food and Drug Administration for first-in-human testing and has been applied in adults for intestinal anastomosis during urologic reconstructions. We now report the first cases of magnamosis to functionally undivert the fecal stream from a previously created loop ileostomy in pediatric patients. Read More

    Requisite role of vasohibin-2 in spontaneous gastric cancer formation and accumulation of cancer-associated fibroblasts.
    Cancer Sci 2017 Dec 21;108(12):2342-2351. Epub 2017 Oct 21.
    Department of Vascular Biology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Miyagi, Japan.
    The vasohibin (VASH) family consists of two genes, VASH1 and VASH2. VASH1 is mainly expressed in vascular endothelial cells and suppresses angiogenesis in an autocrine manner, whereas VASH2 is mainly expressed in cancer cells and exhibits pro-angiogenic activity. Employing adenomatous polyposis coli gene mutant mice, we recently reported on the role of Vash2 in the spontaneous formation of intestinal tumors. Read More

    Outcome after restorative proctocolectomy and ileal pouch-anal anastomosis in children and adults.
    Br J Surg 2017 Nov 20;104(12):1640-1647. Epub 2017 Sep 20.
    Department of Surgery, Academic Medical Centre, Amsterdam, The Netherlands.
    Background: Studies comparing the outcome of ileal pouch-anal anastomosis (IPAA) in children and adults are scarce. This complicates decision-making in young patients. The aim of this study was to compare adverse events and pouch function between children and adults who underwent IPAA. Read More

    An increased CD25-positive intestinal regulatory T lymphocyte population is dependent upon Cox-2 activity in the Apcmodel.
    Clin Exp Immunol 2018 Jan 24;191(1):32-41. Epub 2017 Oct 24.
    MRC Centre for Inflammation Research, University of Edinburgh, The Queen's Medical Research Institute, Edinburgh, UK.
    Only mismatch repair (MMR)-deficient colorectal cancer (CRC) appears to respond well to programmed death (PD)-1 inhibition at the present time. Emerging evidence suggests a role for micro-environmental factors such as CD25cells modulating response to PD-1 inhibition. In the Apcmodel of familial adenomatous polyposis (MMR-proficient CRC), increased Cyclooxygenase-2 (Cox-2) expression by cells which include alternatively activated mononuclear phagocytes promotes intestinal tumorigenesis by mechanisms which may include immune suppression. Read More

    Cronkhite-Canada syndrome associated with colon cancer metastatic to liver: A case report.
    Medicine (Baltimore) 2017 Sep;96(38):e7466
    Department of Oncology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
    Rationale: Cronkhite-Canada Syndrome (CCS) is an idiopathic, nonhereditary syndrome haracterized by gastrointestinal (GI) polyposis and ectodermal changes including alopecia, onychatrophia, and pigmentation. CCS colon polyps were previously considered to be benign neoplasms. However, serrated adenoma was reported to be associated with malignant neoplasms in some cases of gastric and colorectal carcinomas, and esophageal cancers. Read More

    Adrenal Lesions in Patients With (Attenuated) Familial Adenomatous Polyposis and MUTYH-Associated Polyposis.
    Dis Colon Rectum 2017 Oct;60(10):1057-1064
    1 Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 2 Department of Radiology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 3 Department of Endocrinology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 4 Department of Surgery, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 5 Department of Clinical Genetics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands 6 Department of Clinical Epidemiology, Biostatistics, and Bioinformatics, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.
    Background: The reported proportion of patients with familial adenomatous polyposis who have adrenal lesions varies between 7% and 13% compared with 4% in the general population; the prevalence of adrenal lesions in patients with attenuated familial adenomatous polyposis and MUTYH-associated polyposis is unknown. Data on the clinical relevance and clinical course are limited.

    Objective: We aimed to report on the frequency, characteristics, and progression of adrenal lesions in polyposis patients. Read More

    Dental anomalies in pediatric patients with familial adenomatous polyposis.
    Fam Cancer 2017 Sep 8. Epub 2017 Sep 8.
    Department of Corporate Clinical Research & Department of Research and Graduate Programs, University of Missouri - Kansas City School of Dentistry, 650 E. 25th Street, Kansas City, MO, 64108, USA.
    Familial adenomatous polyposis patients often present with non-malignant extra-intestinal manifestations which include dental anomalies that may be evident prior to the appearance of the colonic adenomas. The aims of this study were to describe the prevalence and type of dental anomalies and the relationships between gene mutations and dental anomalies in these patients. Twenty-two pediatric familial adenomatous polyposis patients and 46 controls, who were age and gender matched participated. Read More

    Berberine binds RXRα to suppress β-catenin signaling in colon cancer cells.
    Oncogene 2017 Dec 28;36(50):6906-6918. Epub 2017 Aug 28.
    Cancer Research Center, Xiamen University Medical College, Xiamen, China.
    Berberine, an isoquinoline alkaloid, is a traditional oriental medicine used to treat diarrhea and gastroenteritis. Recently, we reported that it could inhibit the growth of intestinal polyp in animals and in patients with the familial adenomatous polyposis by downregulating β-catenin signaling. However, the intracellular target mediating the effects of berberine remains elusive. Read More

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