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- Familial_adenomatous_polyposis abstract "Familial adenomatous polyposis (FAP) is an inherited condition in which numerous adenomatous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon cancer occurs when left untreated. Three variants are known to exist, FAP and attenuated FAP (originally called “hereditary flat adenoma syndrome) are caused by APC gene defects and autosomal recessive FAP (or MYH-associated polyposis) is caused by MUTYH gene defects. Of the three, FAP itself is the most severe and most common, although for all three, the resulting colonic polyps and cancers are confined to the colon wall and will not spread for many years, during which they can be completely cured by surgery.The root cause of FAP is understood to be a genetic mutation - a flaw in the body's tumour suppressor genes that prevent development of tumours. The flaw allows numerous cells of the intestinal wall to develop into potentially cancerous polyps when they would usually reach the end of their life; inevitably one or more will eventually progress and give rise to cancer (7% risk by age 21, rising to 87% by age 45 and 93% by age 50). The flawed genes do not trigger cancer, but rather, they reduce the body's ability to protect against the risk of aged cells becoming cancerous. Even with the flawed gene, it may still take time before a cell actually does develop that is cancerous as a result, and the gene may in some cases still partially operate to control tumours, therefore cancer from FAP takes many years to develop and is almost always an adult-onset disease.The second form of FAP, known as attenuated familial adenomatous polyposis has the APC gene functional but slightly impaired. It is therefore somewhat able to operate as usual. Attenuated FAP still presents a high 70% lifetime risk of cancer (as estimated), but typically presents with far fewer polyps (typically 30) rather than the hundreds or thousands usually found in FAP, and arises at an age when FAP is usually no longer considered likely - typically between 40 and 70 years old (average 55) rather than the more usual 30's upward. Because it has far fewer polyps, options for management may be different.The third variant, autosomal recessive familial adenomatous polyposis or MYH-associated polyposis, is also milder and, as its name suggests, requires both parents to be 'carriers' to manifest the condition.In some cases FAP can manifest higher in the colon than usual (for example, the ascending colon,[citation needed] or proximal to the splenic flexure, or in the gastric or duodenal tracts) where they show no symptoms until cancer is present and greatly advanced. APC mutations have been linked to certain other cancers such as thyroid cancer. As the mutation causing FAP is genetic, it can be inherited hereditarily from either parent, and passed to children. A genetic blood test of the APC gene exists that can determine whether it is deficient, and therefore can predict the possibility of FAP. Individuals at risk (due to family links or genetic testing) are usually offered routine monitoring of the intestinal tract every 1 – 5 years for life, from early adulthood, to detect the slow-forming polyps and act if found, before they can pose a threat. International polyposis registries exists that track known cases of FAP or APC gene defects, for research and clinical purposes. Mutation of APC also occurs commonly in incident cases of colorectal carcinoma, emphasizing its importance in this form of cancer.".
- Familial_adenomatous_polyposis diseasesdb "4678".
- Familial_adenomatous_polyposis emedicineSubject "med".
- Familial_adenomatous_polyposis emedicineTopic "769".
- Familial_adenomatous_polyposis icd10 "C18, D12".
- Familial_adenomatous_polyposis icd9 "211.3".
- Familial_adenomatous_polyposis icdo "8220/0".
- Familial_adenomatous_polyposis meshId "D011125".
- Familial_adenomatous_polyposis omim "175100".
- Familial_adenomatous_polyposis thumbnail FAP.jpg?width=300.
- Familial_adenomatous_polyposis wikiPageExternalLink 175377-overview.
- Familial_adenomatous_polyposis wikiPageExternalLink condition=familialadenomatouspolyposis.
- Familial_adenomatous_polyposis wikiPageExternalLink healthprofessional.
- Familial_adenomatous_polyposis wikiPageExternalLink attenuated-familial-adenomatous-polyposis.
- Familial_adenomatous_polyposis wikiPageExternalLink familial-adenomatous-polyposis.
- Familial_adenomatous_polyposis wikiPageExternalLink topic567.htm.
- Familial_adenomatous_polyposis wikiPageExternalLink www.fapgene.com.
- Familial_adenomatous_polyposis wikiPageExternalLink NBK1345.
- Familial_adenomatous_polyposis wikiPageExternalLink br.fcgi?book=gene&part=fap.
- Familial_adenomatous_polyposis wikiPageExternalLink www.polyposisregistry.org.uk.
- Familial_adenomatous_polyposis wikiPageExternalLink index.htm.
- Familial_adenomatous_polyposis wikiPageID "1106793".
- Familial_adenomatous_polyposis wikiPageRevisionID "604296471".
- Familial_adenomatous_polyposis caption "Endoscopic image of sigmoid colon of patient with familial adenomatous polyposis.".
- Familial_adenomatous_polyposis diseasesdb "4678".
- Familial_adenomatous_polyposis emedicinesubj "med".
- Familial_adenomatous_polyposis emedicinetopic "769".
- Familial_adenomatous_polyposis hasPhotoCollection Familial_adenomatous_polyposis.
- Familial_adenomatous_polyposis icd "211.3".
- Familial_adenomatous_polyposis icd "C18, D12".
- Familial_adenomatous_polyposis icdo "8220".
- Familial_adenomatous_polyposis meshid "D011125".
- Familial_adenomatous_polyposis name "Familial adenomatous polyposis".
- Familial_adenomatous_polyposis omim "175100".
- Familial_adenomatous_polyposis wordnet_type synset-disease-noun-1.
- Familial_adenomatous_polyposis subject Category:Benign_neoplasms.
- Familial_adenomatous_polyposis subject Category:Gastrointestinal_cancer.
- Familial_adenomatous_polyposis subject Category:Hereditary_cancers.
- Familial_adenomatous_polyposis type Abstraction100002137.
- Familial_adenomatous_polyposis type Attribute100024264.
- Familial_adenomatous_polyposis type BenignNeoplasms.
- Familial_adenomatous_polyposis type Cancer114239918.
- Familial_adenomatous_polyposis type Condition113920835.
- Familial_adenomatous_polyposis type Disease114070360.
- Familial_adenomatous_polyposis type Growth114234074.
- Familial_adenomatous_polyposis type HereditaryCancers.
- Familial_adenomatous_polyposis type IllHealth114052046.
- Familial_adenomatous_polyposis type Illness114061805.
- Familial_adenomatous_polyposis type Malignancy114237561.
- Familial_adenomatous_polyposis type MalignantTumor114239425.
- Familial_adenomatous_polyposis type PathologicalState114051917.
- Familial_adenomatous_polyposis type PhysicalCondition114034177.
- Familial_adenomatous_polyposis type State100024720.
- Familial_adenomatous_polyposis type Tumor114235200.
- Familial_adenomatous_polyposis type Disease.
- Familial_adenomatous_polyposis type AilmentCondition.
- Familial_adenomatous_polyposis type Situation.
- Familial_adenomatous_polyposis comment "Familial adenomatous polyposis (FAP) is an inherited condition in which numerous adenomatous polyps form mainly in the epithelium of the large intestine. While these polyps start out benign, malignant transformation into colon cancer occurs when left untreated. Three variants are known to exist, FAP and attenuated FAP (originally called “hereditary flat adenoma syndrome) are caused by APC gene defects and autosomal recessive FAP (or MYH-associated polyposis) is caused by MUTYH gene defects.".
- Familial_adenomatous_polyposis label "Familiaire adenomateuze polypose".
- Familial_adenomatous_polyposis label "Familial adenomatous polyposis".
- Familial_adenomatous_polyposis label "Familiäre adenomatöse Polyposis".
- Familial_adenomatous_polyposis label "Polipose adenomatosa familiar".
- Familial_adenomatous_polyposis label "Poliposi adenomatosa familiare".
- Familial_adenomatous_polyposis label "Poliposis adenomatosa familiar".
- Familial_adenomatous_polyposis label "Polypose recto-colique familiale".
- Familial_adenomatous_polyposis label "Rodzinna polipowatość gruczolakowata".
- Familial_adenomatous_polyposis label "家族性大腸腺腫症".
- Familial_adenomatous_polyposis sameAs Familiární_adenomatózní_polypóza.
- Familial_adenomatous_polyposis sameAs Familiäre_adenomatöse_Polyposis.
- Familial_adenomatous_polyposis sameAs Poliposis_adenomatosa_familiar.
- Familial_adenomatous_polyposis sameAs Polypose_recto-colique_familiale.
- Familial_adenomatous_polyposis sameAs Poliposi_adenomatosa_familiare.
- Familial_adenomatous_polyposis sameAs 家族性大腸腺腫症.
- Familial_adenomatous_polyposis sameAs 가족성_용종증.
- Familial_adenomatous_polyposis sameAs Familiaire_adenomateuze_polypose.
- Familial_adenomatous_polyposis sameAs Rodzinna_polipowatość_gruczolakowata.
- Familial_adenomatous_polyposis sameAs Polipose_adenomatosa_familiar.
- Familial_adenomatous_polyposis sameAs m.046k0y.
- Familial_adenomatous_polyposis sameAs Q1369011.
- Familial_adenomatous_polyposis sameAs Q1369011.
- Familial_adenomatous_polyposis sameAs 1333.
- Familial_adenomatous_polyposis sameAs C0032580.
- Familial_adenomatous_polyposis sameAs Familial_adenomatous_polyposis.
- Familial_adenomatous_polyposis wasDerivedFrom Familial_adenomatous_polyposis?oldid=604296471.
- Familial_adenomatous_polyposis depiction FAP.jpg.
- Familial_adenomatous_polyposis isPrimaryTopicOf Familial_adenomatous_polyposis.
- Familial_adenomatous_polyposis name "Familial adenomatous polyposis".