11,332 results match your criteria Cronkhite-Canada Syndrome


Microencapsulated and in Combination with Quercetin Inhibit Colorectal Cancer Development in Apc Mice.

Int J Mol Sci 2021 May 5;22(9). Epub 2021 May 5.

Laboratory of Animal Physiology and Nutrition, School of Agronomy, Public University of Navarre, Campus Arrosadia, 31006 Pamplona, Spain.

Recent studies have suggested that flavonoids such as quercetin and probiotics such as () and () could play a relevant role in inhibiting colon cancer cell growth. Our study investigated the role of dietary supplementation with microencapsulated probiotics ( and ) along with quercetin in the development of mouse colorectal cancer (CRC). Methods: Adenomatous polyposis coli/multiple intestinal neoplasia (Apc) mice were fed a standard diet or the same diet supplemented with microencapsulated probiotics ( and strains, 10 CFU/100 g food) or both probiotics strains plus microencapsulated quercetin (15 mg/100 g food) for 73 days. Read More

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Activity of Arhgef4 is modulated through Staufen1 in neurons.

Neurosci Lett 2021 May 19;756:135962. Epub 2021 May 19.

Department of Medicine and Microbiology, Graduate Program in Neuroscience, College of Medicine, Chungbuk National University, Cheongju 28644, Republic of Korea. Electronic address:

The role of Arhgef4, also known as adenomatous polyposis coli (APC)-stimulated guanine nucleotide exchange factor 1 (Asef1), has been identified in colorectal cancers. Interestingly, Arhgef4 is more highly expressed in brain regions than intestinal regions, suggesting a role in neurons. In our previous study, we reported that Arhgef4 negatively regulates the level of PSD-95 in excitatory post-synaptic regions by binding with Staufen1. Read More

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The Transcriptomic Landscape of Mismatch Repair-Deficient Intestinal Stem Cells.

Cancer Res 2021 May 18;81(10):2760-2773. Epub 2021 Mar 18.

Department of Clinical Cancer Prevention, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Lynch syndrome is the most common cause of hereditary colorectal cancer and is secondary to germline alterations in one of four DNA mismatch repair (MMR) genes. Here we aimed to provide novel insights into the initiation of MMR-deficient (MMRd) colorectal carcinogenesis by characterizing the expression profile of MMRd intestinal stem cells (ISC). A tissue-specific MMRd mouse model (Villin-Cre;Msh2 ) was crossed with a reporter mouse () to trace and isolate ISCs (Lgr5+) using flow cytometry. Read More

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Cronkhite-Canada syndrome with steroid dependency: A case report.

World J Clin Cases 2021 May;9(14):3466-3471

Department of Gastroenterology, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China.

Background: Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disease characterized by chronic diarrhoea, diffuse gastrointestinal polyposis and ectodermal manifestations. The lethality of CCS can be up to 50% if it is untreated or if treatment is delayed or inadequate. More than 35% of the patients do not achieve long-term clinical remission after corticosteroid administration, with relapse occurring during or after the cessation of glucocorticoid use. Read More

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Retention of an endoscopic videocapsule on inflammatory polyposis of the small bowel.

Ann Med Surg (Lond) 2021 May 16;65:102323. Epub 2021 Apr 16.

Department of General Surgery, Ibn Rochd University Hospital Center, Casablanca, Morocco.

The Video Capsule Diagnostic Imaging is a technique for exploring the digestive tract, particularly the small bowel. It is indicated for any unexplained digestive bleeding or as a means of monitoring intestinal polyposis or inflammatory diseases. This videocapsule is not digestible, and the risk of its retention, symptomatic or not, is not negligible following an inflammatory, anastomatous or tumoral stenosis. Read More

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Aplastic Anemia in a Patient with Cronkhite-Canada Syndrome.

Intern Med 2021 15;60(10):1601-1605. Epub 2021 May 15.

Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, Japan.

Cronkhite-Canada syndrome (CCS) is a rare polyposis disorder accompanied by alopecia and onychodystrophy. A 63-year-old man with a history of CCS and repeated embolism developed progressive thrombocytopenia and mild anemia. Laboratory testing, a bone marrow examination, and magnetic resonance imaging of the spine resulted in a diagnosis of concurrent aplastic anemia (AA). Read More

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Endoplasmic reticulum stress regulates the intestinal stem cell state through CtBP2.

Sci Rep 2021 May 10;11(1):9892. Epub 2021 May 10.

Department of Gastroenterology and Hepatology, Amsterdam Gastroenterology and Metabolism, Tytgat Institute for Liver and Intestinal Research, Amsterdam UMC, University of Amsterdam, Meibergdreef 69-71, Amsterdam, The Netherlands.

Enforcing differentiation of cancer stem cells is considered as a potential strategy to sensitize colorectal cancer cells to irradiation and chemotherapy. Activation of the unfolded protein response, due to endoplasmic reticulum (ER) stress, causes rapid stem cell differentiation in normal intestinal and colon cancer cells. We previously found that stem cell differentiation was mediated by a Protein kinase R-like ER kinase (PERK) dependent arrest of mRNA translation, resulting in rapid protein depletion of WNT-dependent transcription factor c-MYC. Read More

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Peutz-Jeghers syndrome: an unusual autopsy finding in pregnancy.

Autops Case Rep 2021 Apr 20;11:e2021279. Epub 2021 Apr 20.

Universidade Estadual Paulista (UNESP), Faculdade de Medicina de Botucatu, Departamento de Patologia, Botucatu, SP, Brasil.

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant polyposis entity that often remains undiagnosed. The major problems associated with PJS are acute complications due to (i) polyp-related intestinal obstruction, (ii) intussusception, and (iii) the risk of cancer in the long-term. We report the case of a 32-year-old female who presented at the emergency room with signs of acute abdomen and died during the clinical workup. Read More

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Inhibition of β-Catenin Activity Abolishes LKB1 Loss-Driven Pancreatic Cystadenoma in Mice.

Int J Mol Sci 2021 Apr 28;22(9). Epub 2021 Apr 28.

Institute of Biomedical Sciences, National Sun Yat-Sen University, Kaohsiung 804, Taiwan.

Pancreatic cancer (PC) is the seventh leading cause of cancer death worldwide, and remains one of our most recalcitrant and dismal diseases. In contrast to many other malignancies, there has not been a significant improvement in patient survival over the past decade. Despite advances in our understanding of the genetic alterations associated with this disease, an incomplete understanding of the underlying biology and lack of suitable animal models have hampered efforts to develop more effective therapies. Read More

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Small Bowel Epithelial Precursor Lesions: A Focus on Molecular Alterations.

Int J Mol Sci 2021 Apr 22;22(9). Epub 2021 Apr 22.

Department of Internal Medicine, University of Pavia and Fondazione IRCCS San Matteo Hospital, 27100 Pavia, Lombardy, Italy.

The wider use of gastrointestinal endoscopic procedures has led to an increased detection of small intestinal preneoplastic and neoplastic epithelial lesions, most of which are identified in the duodenum and ampullary region. Like their malignant counterparts, small intestinal glandular precursor lesions, which include adenomas and hamartomas, may arise sporadically or be associated with hereditary tumor syndromes, such as familial adenomatous polyposis, -associated polyposis, Lynch syndrome, Peutz-Jeghers syndrome, juvenile polyposis syndrome, and Cowden syndrome. In addition, dysplastic, preinvasive lesions have been observed adjacent to small bowel adenocarcinomas complicating immune-related disorders, such as celiac or Crohn's disease. Read More

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Combining the Fecal Immunochemical Test with a Logistic Regression Model for Screening Colorectal Neoplasia.

Front Pharmacol 2021 17;12:635481. Epub 2021 Mar 17.

Department of Health Management, The First Affiliated Hospital, Southern University of Science and Technology, Shenzhen People's Hospital, Shenzhen, China.

The fecal immunochemical test (FIT) is a widely used strategy for colorectal cancer (CRC) screening with moderate sensitivity. To further increase the sensitivity of FIT in identifying colorectal neoplasia, in this study, we established a classifier model by combining FIT result and other demographic and clinical features. A total of 4,477 participants were examined with FIT and those who tested positive (over 100 ng/ml) were followed up by a colonoscopy examination. Read More

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Transition state analogue of MTAP extends lifespan of APC mice.

Sci Rep 2021 Apr 23;11(1):8844. Epub 2021 Apr 23.

Department of Biochemistry, Albert Einstein College of Medicine, Bronx, NY, 10461, USA.

A mouse model of human Familial Adenomatous Polyposis responds favorably to pharmacological inhibition of 5'-methylthioadenosine phosphorylase (MTAP). Methylthio-DADMe-Immucillin-A (MTDIA) is an orally available, transition state analogue inhibitor of MTAP. 5'-Methylthioadenosine (MTA), the substrate for MTAP, is formed in polyamine synthesis and is recycled by MTAP to S-adenosyl-L-methionine (SAM) via salvage pathways. Read More

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Author commentary on Mariko Sekiya et al.

Authors:

Endoscopy 2021 05 22;53(5):v23. Epub 2021 Apr 22.

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Endoscopic papillectomy in patients with familial adenomatous polyposis: Does one size fit all?

Gastrointest Endosc 2021 05;93(5):1202

Department of Gastroenterology and Hepatology, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio, USA; Sanford R. Weiss MD Center for Hereditary Gastrointestinal Neoplasia, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio, USA.

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Response.

Gastrointest Endosc 2021 05;93(5):1202-1203

Amsterdam UMC, University of Amsterdam, Department of Gastroenterology and Hepatology, Cancer Center Amsterdam, Amsterdam Gastroenterology and Metabolism, Amsterdam, Netherlands.

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Underwater versus conventional endoscopic mucosal resection for colorectal polyps.

Gastrointest Endosc 2021 05;93(5):1192

Department of Thoracic Surgery, First Hospital of China Medical University, Shenyang, China.

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D-pouch: a modified ileal J-pouch for patients with ulcerative colitis and familial adenomatous polyposis.

Tech Coloproctol 2021 Apr 7. Epub 2021 Apr 7.

Department of Colorectal and Anal Surgery of Zhongnan Hospital of Wuhan University, Clinical Center of Intestinal and Colorectal Diseases of Hubei Province, Hubei Key Laboratory of Intestinal and Colorectal Diseases (Zhongnan Hospital of Wuhan University), Colorectal and Anal Disease Research Center of Medical School (Zhongnan Hospital of Wuhan University), Quality Control Center of Colorectal and Anal Surgery of Health Commission of Hubei Province, No. 169, DongHu Road, Wuhan, China.

Background: Ileal J-pouch anal anastomosis (J-IPAA) is the standard approach for patients requiring restorative proctocolectomy due to familial adenomatous polyposis (FAP) or ulcerative colitis (UC). To obviate the risk of a J-tip leak, we modified the J-pouch with a D-pouch anal anastomosis (D-IPAA) designed to eliminate the ileal stump. The aim of our study was to evaluate the feasibility, safety and medium-term functional outcomes of D-IPAA. Read More

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mTOR inhibitors reduce enteropathy, intestinal bleeding and colectomy rate in juvenile polyposis of infancy due to PTEN-BMPR1A deletion syndrome.

Hum Mol Genet 2021 Apr 2. Epub 2021 Apr 2.

Translational Gastroenterology Unit, University of Oxford, Oxford, United Kingdom.

Background: Ultrarare genetic disorders can provide proof of concept for efficacy of targeted therapeutics and reveal pathogenic mechanisms relevant to more common conditions. Juvenile polyposis of infancy (JPI) is caused by microdeletions in chromosome 10 that result in haploinsufficiency of two tumor suppressor genes: phosphatase and tensin homolog deleted on chromosome 10 (PTEN) and bone morphogenetic protein receptor, type IA (BMPR1A). Loss of PTEN and BMPR1A results in a much more severe phenotype then deletion of either gene alone, with infantile onset pan-enteric polyposis and a high mortality rate. Read More

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Histologic heterogeneity and syndromic associations of non-ampullary duodenal polyps and superficial mucosal lesions.

Dig Liver Dis 2021 Apr 1. Epub 2021 Apr 1.

First Department of Internal Medicine, University of Pavia, IRCCS San Matteo Hospital Foundation, Viale Golgi 19, 27100, Italy.

Background: Duodenal polyps and superficial mucosal lesions (DP/SMLs) are poorly characterised.

Aims: To describe a series of endoscopically-diagnosed extra-ampullary DPs/SMLs.

Methods: This is a retrospective study conducted in a tertiary referral Endoscopy Unit, including patients who had DPs or SMLs that were biopsied or removed in 2010-2019. Read More

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Cronkhite-Canada Syndrome: A Rare COVID-19 Mimicker.

Am J Gastroenterol 2021 Mar 30. Epub 2021 Mar 30.

Department of Gastroenterology, St. Luke's International Hospital, Chuo-ku, Tokyo, Japan.

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Intestinal obstruction with oral pigmentation: a clue to Peutz-Jeghers syndrome.

BMJ Case Rep 2021 Apr 1;14(4). Epub 2021 Apr 1.

General Surgery, All India Institute of Medical Sciences, Bhubaneswar, India.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder, which manifests as mucocutaneous hyperpigmentation and, intestinal and extraintestinal polyps. The classic triad of abdominal pain, mass and jam-like stools are not found commonly. On clinical examination, a strong suspicion of PJS should be made if patients below 18 years, present with mucocutaneous hyperpigmentation and intestinal obstruction. Read More

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Infection Leads to the Reprogramming of Glucose and Lipid Metabolism in the Colon of Mice.

Front Vet Sci 2021 10;8:645807. Epub 2021 Mar 10.

Jiangsu Key Laboratory of Immunity and Metabolism, Department of Pathogen Biology and Immunology, Xuzhou Medical University, Xuzhou, China.

The deposition of () eggs commonly induces inflammation, fibrosis, hyperplasia, ulceration, and polyposis in the colon, which poses a serious threat to human health. However, the underlying mechanism is largely neglected. Recently, the disorder of glucose and lipid metabolism was reported to participate in the liver fibrosis induced by the parasite, which provides a novel clue for studying the underlying mechanism of the intestinal pathology of the disease. Read More

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[Ⅰ. Evidence-Based Drug Treatment for Desmoids-Anti-Cancer Drugs, Molecular-Targeted Drugs].

Gan To Kagaku Ryoho 2021 Mar;48(3):342-345

Dept. of Rehabilitation, Nagoya University Hospital.

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Hearing Status of Children and Adolescents With Familial Adenomatous Polyposis.

Anticancer Res 2021 Mar;41(3):1439-1444

Division of Gastroenterology, Hepatology and Nutrition, Nationwide Children's Hospital and The Ohio State University College of Medicine, Columbus, OH, U.S.A.

Background/aim: Chemopreventative therapeutics may be helpful in familial adenomatous polyposis (FAP) management; however, prospective chemopreventative studies are complicated by potential ototoxicity and pre-existing hearing loss. The aim of this study was to establish and compare baseline hearing status of children and adolescents with FAP and their unaffected siblings.

Patients And Methods: Twenty FAP pediatric patients with documented mutation of the adenomatous polyposis coli (APC) gene and nine unaffected sibling controls underwent baseline hearing evaluation, including audiometry, speech perception testing, and middle and inner ear physiologic measures. Read More

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Fucoxanthin Prevents Colorectal Cancer Development in Dextran Sodium Sulfate-treated Mice.

Anticancer Res 2021 Mar;41(3):1299-1305

Department of Molecular-Targeting Prevention, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.

Background/aim: A xanthophyll of fucoxanthin (Fx) is a potential chemopreventive agent. Familial adenomatous polyposis (FAP) is an inherited disease that is associated with a high risk of developing colorectal cancer. However, it remains unclear whether Fx can modify colorectal tumorigenesis in Apc mice, a model mouse for human FAP. Read More

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Surgical results and quality of life of patients submitted to restorative proctocolectomy and ileal pouch-anal anastomosis.

Rev Col Bras Cir 2021 24;48:e20202791. Epub 2021 Mar 24.

- Hospital das Clínicas- UFMG, Instituto Alfa de Gastroenterologia - Belo Horizonte - MG - Brasil.

Purpose: restorative proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the surgical procedure of choice in some cases of familial adenomatous polyposis (FAP) and ulcerative colitis (UC). IPAA allows complete removal of the diseased colon and rectum, however, it is associated with substantial morbidity and potential consequences to patients' quality of life (QoL).

Aims: to evaluate the surgical results, functional outcomes and QoL after IPAA; and to examine the impact of surgical complications upon QoL. Read More

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Accumulated genetic mutations leading to accelerated initiation and progression of colorectal cancer in a patient with Gardner syndrome: A case report.

Medicine (Baltimore) 2021 Apr;100(13):e25247

Department of Oncology, Longhua Hospital Affiliated to Shanghai University of Traditional Chinese Medicine (TCM), Shanghai, China.

Rationale: Gardner syndrome is a rare autosomal dominant disorder with a high degree of penetrance, which is characterized by intestinal polyposis, osteomas, and dental abnormalities. Majority of patients with Gardner syndrome will develop colorectal cancer by the age of 40 to 50 years. Mutations in the adenomatous polyposis coli gene are supposed to be responsible for the initiation of Gardner syndrome. Read More

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Sexual activity in patients after proctocolectomy with ileal pouch-anal anastomosis.

Pol Przegl Chir 2020 Nov;93(1):19-24

General and Colorectal Surgery Clinic, Medical University of Lodz.

<b>Introduction:</b> Proctocolectomy with ileal pouch-anal anastomosis is the gold standard in the surgical treatment of patients with ulcerative colitis, familial adenomatous polyposis and other colorectal diseases requiring colectomy. The treatment consists in removing the large intestine and creating an intestinal reservoir from the last ileum loop and then anastomosing the intestinal reservoir with the anal canal. Like any surgical procedure, RPC-IPAA also carries the risk of complications, both early and late. Read More

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November 2020

Cronkhite-Canada syndrome associated with perianal condyloma acuminatum with malignant transformation: A case report.

Medicine (Baltimore) 2021 Mar;100(10):e25067

Binzhou Medical University Hospital, The Yellow Rive Second Road, Binzhou City, Shandong Province, China.

Rationale: Cronkhite-Canada syndrome (CCS) is a rare non-familial polyposis syndrome characterized by multiple gastrointestinal polyps with the ectodermal triad. To date, many complications of CCS have been reported in the literature, but perianal condyloma acuminatum with malignant transformation has not been included.

Patient Concerns: This report presents the case of a 52-year-old Chinese man who presented with diarrhea, loss of appetite, and weight loss. Read More

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