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Genetic predisposition to colorectal cancer: new pieces in the pediatric puzzle.
J Pediatr Gastroenterol Nutr. 2006 Jul; 43(1):5-15.JP

Abstract

Colorectal cancer is rare in childhood. The 2 best characterized familial syndromes, hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) and familial adenomatous polyposis (FAP), are autosomal dominant inherited disorders. HNPCC is relevant to pediatric gastroenterology practice because children and adolescents with underlying colorectal cancer can have germ-line mutations of mismatch repair genes. Recent attention has focused on characterizing genetic predisposition to attenuated FAP in individuals who do not have germ-line mutations in the APC gene. The identification of a second mechanistic explanation called MYH-associated polyposis (MAP), which is an autosomal-recessive condition, has important implications for both screening and management strategies. Hereditary colorectal cancer including HNPCC, FAP, attenuated FAP and MYH-associated polyposis in children are the subject of this review.

Authors+Show Affiliations

Division of Gastroenterology/Hepatology and Clinical Nutrition, Hospital for Sick Children, University of Toronto, Mount Sinai Hospital, Toronto, Ontario, Canada. carol.durno@sickkids.caNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

16819371

Citation

Durno, Carol A., and Steven Gallinger. "Genetic Predisposition to Colorectal Cancer: New Pieces in the Pediatric Puzzle." Journal of Pediatric Gastroenterology and Nutrition, vol. 43, no. 1, 2006, pp. 5-15.
Durno CA, Gallinger S. Genetic predisposition to colorectal cancer: new pieces in the pediatric puzzle. J Pediatr Gastroenterol Nutr. 2006;43(1):5-15.
Durno, C. A., & Gallinger, S. (2006). Genetic predisposition to colorectal cancer: new pieces in the pediatric puzzle. Journal of Pediatric Gastroenterology and Nutrition, 43(1), 5-15.
Durno CA, Gallinger S. Genetic Predisposition to Colorectal Cancer: New Pieces in the Pediatric Puzzle. J Pediatr Gastroenterol Nutr. 2006;43(1):5-15. PubMed PMID: 16819371.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Genetic predisposition to colorectal cancer: new pieces in the pediatric puzzle. AU - Durno,Carol A, AU - Gallinger,Steven, PY - 2006/7/5/pubmed PY - 2007/3/14/medline PY - 2006/7/5/entrez SP - 5 EP - 15 JF - Journal of pediatric gastroenterology and nutrition JO - J. Pediatr. Gastroenterol. Nutr. VL - 43 IS - 1 N2 - Colorectal cancer is rare in childhood. The 2 best characterized familial syndromes, hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) and familial adenomatous polyposis (FAP), are autosomal dominant inherited disorders. HNPCC is relevant to pediatric gastroenterology practice because children and adolescents with underlying colorectal cancer can have germ-line mutations of mismatch repair genes. Recent attention has focused on characterizing genetic predisposition to attenuated FAP in individuals who do not have germ-line mutations in the APC gene. The identification of a second mechanistic explanation called MYH-associated polyposis (MAP), which is an autosomal-recessive condition, has important implications for both screening and management strategies. Hereditary colorectal cancer including HNPCC, FAP, attenuated FAP and MYH-associated polyposis in children are the subject of this review. SN - 0277-2116 UR - https://www.unboundmedicine.com/medline/citation/16819371/Genetic_predisposition_to_colorectal_cancer:_new_pieces_in_the_pediatric_puzzle_ L2 - http://dx.doi.org/10.1097/01.mpg.0000221889.36501.bb DB - PRIME DP - Unbound Medicine ER -