Familial adenomatous polyposis natural history, complications and prognosis: Difference between revisions
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==Natural history== | ==Natural history== | ||
* Familial adenomatous polyposis is a rare disease that usually starts during teenage years.<ref name="KennedyPotter2014">{{cite journal|last1=Kennedy|first1=Raelene D.|last2=Potter|first2=D. Dean|last3=Moir|first3=Christopher R.|last4=El-Youssef|first4=Mounif|title=The natural history of familial adenomatous polyposis syndrome: A 24year review of a single center experience in screening, diagnosis, and outcomes|journal=Journal of Pediatric Surgery|volume=49|issue=1|year=2014|pages=82–86|issn=00223468|doi=10.1016/j.jpedsurg.2013.09.033}}</ref> | * Familial adenomatous polyposis is a rare disease that usually starts during teenage years.<ref name="KennedyPotter2014">{{cite journal|last1=Kennedy|first1=Raelene D.|last2=Potter|first2=D. Dean|last3=Moir|first3=Christopher R.|last4=El-Youssef|first4=Mounif|title=The natural history of familial adenomatous polyposis syndrome: A 24year review of a single center experience in screening, diagnosis, and outcomes|journal=Journal of Pediatric Surgery|volume=49|issue=1|year=2014|pages=82–86|issn=00223468|doi=10.1016/j.jpedsurg.2013.09.033}}</ref> | ||
* Patients with familial adenomatous polyposis usually develop hundreds to thousands [[colon polyps]] till early twenties. | * Patients with familial adenomatous polyposis usually develop hundreds to thousands [[colon polyps]] till early twenties.<ref name="pmid11446392">{{cite journal |vauthors=Beech D, Pontius A, Muni N, Long WP |title=Familial adenomatous polyposis: a case report and review of the literature |journal=J Natl Med Assoc |volume=93 |issue=6 |pages=208–13 |year=2001 |pmid=11446392 |pmc=2594024 |doi= |url=}}</ref> | ||
* They might be asymptomatic till third decade of their lives or whenever they have [[malignant]] transformation to [[colorectal cancer]]. | * They might be asymptomatic till third decade of their lives or whenever they have [[malignant]] transformation to [[colorectal cancer]]. | ||
* If they left untreated, almost all of them develop [[colorectal cancer]] around 40 years of age. | * If they left untreated, almost all of them develop [[colorectal cancer]] around 40 years of age. | ||
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==Prognosis== | ==Prognosis== | ||
* The prognosis of familial adenomatous polyposis is excellent with treatment. | * The prognosis of familial adenomatous polyposis is excellent with treatment. | ||
*Without treatment, all patients with familial adenomatous polyposis develop [[Colorectal cancer|colon cancer]] at age of 40-50.<ref name="pmid11446392">{{cite journal |vauthors=Beech D, Pontius A, Muni N, Long WP |title=Familial adenomatous polyposis: a case report and review of the literature |journal=J Natl Med Assoc |volume=93 |issue=6 |pages=208–13 |year=2001 |pmid=11446392 |pmc=2594024 |doi= |url=}}</ref> | |||
*Without treatment, all patients with familial adenomatous polyposis develop [[Colorectal cancer|colon cancer]] at age of 40-50. | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2], Mohamad Alkateb, MBBCh [3]
Overview
Natural history
- Familial adenomatous polyposis is a rare disease that usually starts during teenage years.[1]
- Patients with familial adenomatous polyposis usually develop hundreds to thousands colon polyps till early twenties.[2]
- They might be asymptomatic till third decade of their lives or whenever they have malignant transformation to colorectal cancer.
- If they left untreated, almost all of them develop colorectal cancer around 40 years of age.
- They might have polyps in different organs including upper gastrointestinal tract. However, their progression to malignancy is less than colon polyps.
Complications
Complications that can develop as a result of familial adenomatous polyposis are:[3][4]
- Colon cancer which has two different pathways according to defect on APC gene:
- Molecular pathogenesis of sporadic colon cancer
- Molecular pathogenesis of colitis-associated colon cancer
- Duodenal and ampullary cancer:
- They are major causes of death after polypectomy.
- End-viewing and side-viewing duodenoscopy are conducted for surveillance.
Prognosis
- The prognosis of familial adenomatous polyposis is excellent with treatment.
- Without treatment, all patients with familial adenomatous polyposis develop colon cancer at age of 40-50.[2]
References
- ↑ Kennedy, Raelene D.; Potter, D. Dean; Moir, Christopher R.; El-Youssef, Mounif (2014). "The natural history of familial adenomatous polyposis syndrome: A 24year review of a single center experience in screening, diagnosis, and outcomes". Journal of Pediatric Surgery. 49 (1): 82–86. doi:10.1016/j.jpedsurg.2013.09.033. ISSN 0022-3468.
- ↑ 2.0 2.1 Beech D, Pontius A, Muni N, Long WP (2001). "Familial adenomatous polyposis: a case report and review of the literature". J Natl Med Assoc. 93 (6): 208–13. PMC 2594024. PMID 11446392.
- ↑ Iaquinto, Gaetano; Fornasarig, Mara; Quaia, Michele; Giardullo, Nicola; D'Onofrio, Vittorio; Iaquinto, Salvatore; Di Bella, Simone; Cannizzaro, Renato (2008). "Capsule endoscopy is useful and safe for small-bowel surveillance in familial adenomatous polyposis". Gastrointestinal Endoscopy. 67 (1): 61–67. doi:10.1016/j.gie.2007.07.048. ISSN 0016-5107.
- ↑ King, John E.; Dozois, Roger R.; Lindor, Noralane M.; Ahlquist, David A. (2000). "Care of Patients and Their Families With Familial Adenomatous Polyposis". Mayo Clinic Proceedings. 75 (1): 57–67. doi:10.4065/75.1.57. ISSN 0025-6196.