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Adenomatous Polyposis Coli (Familial Adenomatous Polyposis)

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A polyposis syndrome due to an autosomal dominant mutation of the APC genes (GENES, APC) on CHROMOSOME 5. The syndrome is characterized by the development of hundreds of ADENOMATOUS POLYPS in the COLON and RECTUM of affected individuals by early adulthood. The lifetime risk of colorectal cancer in these patients reaches 100 percent by age 60.

Also Known As:

Familial Adenomatous Polyposis; Adenomatous Polyposis of the Colon; Adenomatous Polyposis Coli, Familial; Familial Adenomatous Polyposis Coli; Familial Adenomatous Polyposis of the Colon; Familial Intestinal Polyposis; Familial Multiple Polyposi; Familial Polyposis Coli; Familial Polyposis of the Colon; Hereditary Polyposis Coli; Polyposis, Adenomatous Intestinal; Adenomatous Intestinal Polyposes; Adenomatous Intestinal Polyposis; Adenomatous Polyposes, Familial; Adenomatous Polyposis Colus; Adenomatous Polyposis, Familial; Coli, Adenomatous Polyposis; Coli, Familial Polyposis; Coli, Hereditary Polyposis; Colus, Adenomatous Polyposis; Colus, Familial Polyposis; Colus, Hereditary Polyposis; Familial Adenomatous Polyposes; Familial Intestinal Polyposes; Familial Multiple Polyposus; Familial Polyposis Colus; Familial Polyposis Syndrome; Familial Polyposis Syndromes; Hereditary Polyposis Colus; Intestinal Polyposes, Familial; Intestinal Polyposis, Adenomatous; Intestinal Polyposis, Familial; Multiple Polyposi, Familial; Multiple Polyposus, Familial; Polyposes, Familial Adenomatous; Polyposi, Familial Multiple; Polyposis Coli, Adenomatous; Polyposis Coli, Hereditary; Polyposis Colus, Adenomatous; Polyposis Colus, Familial; Polyposis Colus, Hereditary; Polyposis, Familial Adenomatous; Polyposus, Familial Multiple; Polyposis Coli, Familial; Polyposis Syndrome, Familial

Networked: 2205 relevant articles (46 outcomes, 223 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Polyps
2.	Adenoma (Adenomas)
3.	Ulcerative Colitis
4.	Neoplasms (Cancer)
5.	Colonic Neoplasms (Colon Cancer)

Experts

1.	Giardiello, Francis M: 16 articles (02/2015 - 03/2002)
2.	Akiyama, Tetsu: 14 articles (04/2015 - 06/2002)
3.	Yang, Vincent W: 11 articles (09/2012 - 04/2002)
4.	Phillips, Robin K S: 11 articles (05/2011 - 01/2004)
5.	Nagengast, Fokko M: 10 articles (01/2013 - 11/2006)
6.	Phillips, R K S: 10 articles (04/2012 - 05/2002)
7.	Hylind, Linda M: 10 articles (06/2011 - 03/2002)
8.	Jones, David A: 10 articles (08/2009 - 08/2004)
9.	Behrens, Jürgen: 9 articles (02/2014 - 01/2004)
10.	Nakamura, Y: 9 articles (01/2014 - 01/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Adenomatous Polyposis Coli:

1.	celecoxib (Celebrex)FDA Link 01/01/2002 - "More recently, the selective cyclo-oxygenase-2 inhibitor Celebrex was also shown to be effective in familial adenomatous polyposis and was approved by the Food and Drug Administration as a adjuct to usual care in these patients. " 06/01/2010 - "The safety and efficacy of celecoxib in children with familial adenomatous polyposis." 04/01/2012 - "We aimed to address this issue by performing a clinical study with celecoxib in familial adenomatous polyposis (FAP). " 06/01/2006 - "Celecoxib (Cx), a chemopreventative drug for familial adenomatous polyposis (FAP) and under trial for reducing post surgical colorectal malignancies, was selected as a model drug for this topical system because of the contraindications that are associated with its systemic administration. " 06/01/2011 - "Clinical characteristics and outcomes in familial adenomatous polyposis patients with a long-term treatment of celecoxib: a matched cohort study."
2.	Sulindac (Copal)FDA LinkGeneric 03/15/2001 - "Sulindac, a nonsteroidal antiinflammatory drug effective against colorectal tumorigenesis in familial adenomatous polyposis syndrome, suppressed the formation of foci. " 01/01/2006 - "The aim of the present study was to elucidate the effect of sulindac on uncolectomized familial adenomatous polyposis (FAP). " 01/01/1990 - "The aim of this study was to assess the effectiveness of sulindac therapy for eradication of micropolyps in 8 patients with familial polyposis coli. " 12/01/2015 - "Sulindac is chemopreventive and has utility in patients with familial adenomatous polyposis; however, side effects preclude its long-term use. " 04/01/2007 - "The NSAID (non-steroidal anti-inflammatory drug) sulindac is a potent inhibitor of intestinal carcinogenesis in the C57BL/6J-Apc(Min)/J mouse model and is used to treat humans with FAP (familial adenomatous polyposis). "
3.	Non-Steroidal Anti-Inflammatory Agents (NSAIDs)IBA 02/01/2005 - "NSAIDs were first shown to be effective in patients with familial adenomatous polyposis (FAP). " 11/01/2002 - "Furthermore, epidemiological studies and clinical trials for familial adenomatous polyposis (FAP) patients supported the possibility that NSAIDs can be used as chemopreventive agents. " 01/01/2002 - "Furthermore, epidemiological studies and clinical trials for familial adenomatous polyposis (FAP) patients supported the possibility that NSAIDs can be used as chemopreventive agents. " 01/15/2002 - "Nonsteroidal anti-inflammatory drugs (NSAIDs) are antitumorigenic in humans as well as in animal models of intestinal neoplasia, such as the adenomatous polyposis coli (Min/+) (Apc(Min/+)) mouse. " 03/01/1997 - "In addition, clinical studies of the human disease familial adenomatous polyposis have demonstrated the efficacy of NSAIDs in mediating regression of colorectal adenomas. "
4.	Cyclooxygenase 2 Inhibitors (COX-2 Inhibitors)IBA 05/01/2004 - "In animals study Cox2 inhibitors was shown to be effective and in humans Cox2 inhibitors are approved by the Food and Drug Administration as an adjunct to endoscopic surveillance and surgery in patients with Familial Adenomatous Polyposis (FAP). " 05/01/2004 - "In animals study Cox2 inhibitors was shown to be effective and in humans Cox2 inhibitors are approved by the Food and Drug Administration as an adjunct to endoscopic surveillance and surgery in patients with Familial Adenomatous Polyposis (FAP). " 01/01/2013 - "Coxibs might still be reserved for younger patients with familial adenomatous polyposis (FAP). " 08/01/2008 - "In conclusion, COXIBs have a niche role for patients with familial adenomatous polyposis. " 05/01/2004 - "[Cyclooxygenase 2 inhibitors and adenomatous polyposis coli]."
5.	Indomethacin (Indometacin)FDA LinkGeneric 09/01/2002 - "A total of seven colectomized patients with familial adenomatous polyposis were treated with peroral sustained-release indomethacin at the usual clinical doses. " 09/01/2002 - "The efficacy of peroral sustained-release indomethacin on rectal adenomas in colectomized patients with familial adenomatous polyposis and its toxicity were evaluated preliminarily. " 09/01/1994 - "Indomethacin suppository treatment was effective in controlling rectal remnant polyps in the two patients with familial adenomatous polyposis. " 09/01/2002 - "Peroral sustained-release indomethacin treatment for rectal adenomas in familial adenomatous polyposis: a pilot study." 09/01/1994 - "Regression of rectal polyps by indomethacin suppository in familial adenomatous polyposis. "
6.	Cyclooxygenase 2 (Cyclooxygenase-2)IBA 10/01/2002 - "Immunohistochemical study for cyclooxygenase-2 and genetic analysis in the adenomatous polyposis coli gene was also performed. " 01/01/2009 - "Cyclooxygenase-2 (COX-2) expression is increased in gastrointestinal tumorigenesis and familial adenomatous polyposis. " 06/01/2005 - "Increased cyclooxygenase-2 expression in duodenal compared with colonic tissues in familial adenomatous polyposis and relationship to the -765G -> C COX-2 polymorphism." 02/01/2004 - "Indeed, cyclooxygenase-2 (COX-2) inhibitors recently have been approved for use for prophylaxis in individuals with familial adenomatous polyposis. " 08/01/2001 - "Enhanced cyclooxygenase-2 expression in sporadic and familial adenomatous polyposis of the human colon."
7.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 02/01/2014 - "Herein, we compared the efficacy of AKBA with that of aspirin in an adenomatous polyposis coli intestinal neoplasia consecutive weeks. " 02/01/2013 - "However, only one double-blind trial of aspirin in patients with familial adenomatous polyposis (FAP) has been reported to date. " 05/01/2011 - "Aspirin and familial adenomatous polyposis: coming full circle." 05/01/1998 - "This study was designed to determine if aspirin would significantly inhibit gastrointestinal tumor formation in a mouse model of familial adenomatous polyposis. " 02/01/2013 - "Preventive effects of low-dose aspirin on colorectal adenoma growth in patients with familial adenomatous polyposis: double-blind, randomized clinical trial."
8.	DNA (Deoxyribonucleic Acid)IBA 08/17/1996 - "Since the course of familial adenomatous polyposis is influenced by the site of mutation in the polyposis gene, DNA analysis might be helpful in treatment decisions. " 12/01/1993 - "Improved predictive carrier testing for familial adenomatous polyposis using DNA from a single archival specimen and polymorphic markers with multiple alleles." 07/01/2000 - "During the course of genome studies in a rural community in the South Indian state of Karnataka, DNA-based investigations and counselling for familial adenomatous polyposis (FAP) were requested via the community physician. " 03/22/2011 - "Assembly of the base excision repair complex on abasic DNA and role of adenomatous polyposis coli on its functional activity." 06/01/2010 - "Methylation alterations at the IGF2 locus are more extensive than previously reported and DMR2 hypomethylation in lymphocyte DNA might be a specific epigenetic biomarker for familial adenomatous polyposis patients."
9.	beta CateninIBA 06/01/2013 - "In this study, we examined the role of canonical Wnt signaling by targeting the gene for Adenomatous Polyposis Coli (Apc), which controls Wnt signaling activity via mediating phosphorylation of beta-catenin (Ctnnb). " 05/01/1998 - "The second aim of this study was to investigate whether differences in the phenotypic variability in FAP (familial adenomatous polyposis coli) might be due to inherited alterations in the beta-catenin gene. " 04/18/2014 - "Downregulation of adenomatous polyposis coli by microRNA-663 promotes odontogenic differentiation through activation of Wnt/beta-catenin signaling." 01/01/2014 - "Short-term induction of mutations in Adenomatous polyposis coli (Apc) and beta-catenin (Ctnnbl), which increase and decrease Wnt signaling levels, were generated in AhCre Apc(fl/fl) and AhCre Ctnnb1(fl/fl) mice, respectively. " 01/01/2013 - "Deregulated WNT/catenin pathway, usually resulting from mutations in the adenomatous polyposis coli and beta-catenin genes, drives colorectal tumorigenesis. "
10.	Codon (Codons)IBA 09/08/2000 - "In order to continue the previous morphological studies of eyes from mice with adenomatous polyposis coli (APC) gene mutation at codon 1638, we determined the ultrastructural and electrophysiologic characteristics of these eyes. " 06/01/1994 - "This study evaluated the phenotype of 74 patients with familial adenomatous polyposis from 11 unrelated families with an identical 5-base pair deletion at codon 1309 of the APC gene. " 07/01/2015 - "Novel Missense Mutation at Codon 2774 (C.8321 G>A) p.S2774N of APC Gene in a Denovo Case of Familial Adenomatous Polyposis." 09/01/2014 - "Two Chinese pedigrees for adenomatous polyposis coli: new mutations at codon 1309 and predisposition to phenotypic variations." 10/01/2013 - "A new germline stop codon mutation in exon 15 of the APC gene predisposing to familial adenomatous polyposis."

Therapies and Procedures

1.	Restorative Proctocolectomy 07/01/2007 - "This study was designed to assess the impact of technical factors on functional outcomes and complications in patients undergoing restorative proctocolectomy for familial adenomatous polyposis. " 10/01/2003 - "All patients with familial adenomatous polyposis treated with restorative proctocolectomy and ileal pouch-anal anastomosis in The Cleveland Clinic were included in the study. " 05/01/1998 - "The objective of this study was to determine the frequency of pelvic pouch neoplasia in familial adenomatous polyposis patients after restorative proctocolectomy. " 02/01/2015 - "Restorative proctocolectomy has become the standard surgical procedure for familial adenomatous polyposis (FAP) patients. " 01/01/2015 - "Australian Clinical Practice Guidelines suggest six to twelve-monthly endoscopic pouch surveillance in patients after restorative proctocolectomy for Familial Adenomatous Polyposis (FAP). "
2.	Colectomy 04/01/2012 - "The aim of this study was to investigate the outcome in patients with familial adenomatous polyposis who had colectomy at ≤14 years. " 11/01/2002 - "11 patients with juvenile polyposis and 4 patients with familial adenomatous polyposis after total colectomy entered the study. " 06/01/1997 - "This study was undertaken to describe our results in a series of patients undergoing total abdominal colectomy with ileorectal anastomosis (TAC/IRA) using laparoscopic techniques in patients with familial adenomatous polyposis (FAP) and rectal-sparing. " 05/06/1993 - "We conducted a randomized, double-blind, placebo-controlled study of 22 patients with familial adenomatous polyposis, including 18 who had not undergone colectomy. " 01/01/2016 - "French comment on article: Endoscopic management of familial adenomatous polyposis in patients refusing colectomy."
3.	Chemoprevention 08/01/2004 - "This report reviews the studies on chemoprevention in familial adenomatous polyposis. " 09/01/1997 - "This review focuses on the three genetically altered mouse models that have been the most widely used in chemoprevention studies: Min mice, which carry a mutation in the adenomatous polyposis coli (APC) gene; APC-knockout mice; and p53-knockout mice. " 06/01/1989 - "Subject-reported compliance in a chemoprevention trial for familial adenomatous polyposis." 06/01/2015 - "[Chemoprevention for Familial Adenomatous Polyposis]." 04/15/2012 - "Chemoprevention would be a desirable strategy to avoid duodenectomy in patients with familial adenomatous polyposis (FAP) suffering from duodenal adenomatosis. "
4.	Ileostomy 09/01/2008 - "Twenty-one consecutive patients with familial adenomatous polyposis were examined before proctocolectomy (T0), on ileostomy reversal (T1), and 6 (T2) and 12 months (T3) after surgery by means of standardized questionnaires and interviews. " 04/01/2004 - "Endoscopically assisted wireless capsule endoscopy in a patient with familial adenomatous polyposis after total proctocolectomy and continent ileostomy." 09/01/2003 - "These estimates are based largely on surgeries performed at a time when the only alternative option to ileorectal anastomosis for patients with severe familial adenomatous polyposis was proctocolectomy and ileostomy. " 06/01/1999 - "The ileostomy had been fashioned 21 years earlier after proctocolectomy for familial adenomatous polyposis (FAP). " 04/01/1991 - "One case of malignant degeneration on an ileostomy following proctocolectomy for familial intestinal polyposis is reported. "
5.	Drug Therapy (Chemotherapy) 10/15/2004 - "We additionally explored its expression and activity using models of adenomatous polyposis coli (APC)- and chemotherapy-induced apoptosis. " 06/01/1996 - "Cytotoxic chemotherapy is an effective treatment for desmoid tumours associated with familial adenomatous polyposis. " 06/01/1996 - "To determine the efficacy of chemotherapy for inoperable desmoid tumours associated with familial adenomatous polyposis. " 12/01/2012 - "Macroscopic and histologic regression of duodenal polyposis with FOLFOX4 chemotherapy for an ileal pouch adenocarcinoma in a patient with familial adenomatous polyposis." 09/01/2005 - "Regression of colorectal adenomas with intravenous cytotoxic chemotherapy in a patient with familial adenomatous polyposis."