Familial adenomatous polyposis epidemiology and demographics

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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

Familial adenomatous polyposis is a rare disease that affects individuals worldwide. The incidence and prevalence of familial adenomatous polyposis is approximately 3 to 20 and 12 per 100,000 individuals, respectively. Surgical mortality rate is approximately 4400 per 100,000 individuals (4.4%) in Sweden. Patients usually develop familial adenomatous polyposis in their first and second decades of life. Familial adenomatous polyposis affects men and women equally and there is no racial predilection. Up to 100% of patients with familial adenomatous polyposis without treatment, will develop colorectal cancer by age of 39.

Epidemiology and Demographics

The epidemiology and demographicsIncidence

  • The incidence of familial adenomatous polyposis is approximately 12 per 100,000 births in the Europe.[1]

Prevalence

  • The prevalence of familial adenomatous polyposis is approximately 10 to 20 per 100,000 individuals.[2]
  • The prevalence of familial adenomatous polyposis is approximately 3 to10 per 100,000 individuals in the Europe.[1]

Case-fatality rate/Mortality rate

  • In 1975, the incidence of familial adenomatous polyposis is approximately 13 per 100,000 individuals with a surgical mortality rate of 4400 per 100,000 individuals (4.4%) in Sweden.[3]

Age

  • The mean age for developing colon polyps in patients with familial adenomatous polyposis is 13 years old.[4]
  • Patients in their first and second decades usually develop familial adenomatous polyposis.
  • Up to 100% of patients with familial adenomatous polyposis without treatment will develop colorectal cancer by age of 39.[5]

Race

  • There is no racial predilection to familial adenomatous polyposis.

Gender

  • Familial adenomatous polyposis affects men and women equally.[1]

Region

  • Familial adenomatous polyposis is a rare disease that tends to affect individuals worldwide.[6]

References

  1. 1.0 1.1 1.2 Half, Elizabeth; Bercovich, Dani; Rozen, Paul (2009). "Familial adenomatous polyposis". Orphanet Journal of Rare Diseases. 4 (1): 22. doi:10.1186/1750-1172-4-22. ISSN 1750-1172.
  2. Nieuwenhuis, M.H.; Vasen, H.F.A. (2007). "Correlations between mutation site in APC and phenotype of familial adenomatous polyposis (FAP): A review of the literature". Critical Reviews in Oncology/Hematology. 61 (2): 153–161. doi:10.1016/j.critrevonc.2006.07.004. ISSN 1040-8428.
  3. Alm T (1975). "Surgical treatment of hereditary adenomatosis of the colon and rectum in Sweden during the last 20 years. Part II. Patients with prophylactic operations, primary and late results. Discussion and summary". Acta Chir Scand. 141 (3): 228–37.
  4. 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.
  5. 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.
  6. Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean L, Stephens K, Amemiya A, Jasperson KW, Patel SG, Ahnen DJ. "APC-Associated Polyposis Conditions". PMID 20301519. Vancouver style error: initials (help)

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