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Article: Phenotype and management of patients with familial adenomatous polyposis in Hong Kong: perspective of the Hereditary Gastrointestinal Cancer Registry.

TitlePhenotype and management of patients with familial adenomatous polyposis in Hong Kong: perspective of the Hereditary Gastrointestinal Cancer Registry.
Authors
Issue Date2002
PublisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.html
Citation
Hong Kong Medical Journal = Xianggang Yi Xue Za Zhi / Hong Kong Academy Of Medicine, 2002, v. 8 n. 5, p. 342-347 How to Cite?
AbstractOBJECTIVES: To report on the phenotypic spectrum and clinical management of Chinese patients suffering from the rare autosomal dominant colorectal cancer syndrome of familial adenomatous polyposis. DESIGN: Analysis of prospectively collected data from the database of a regional registry. SETTING: The Hereditary Gastrointestinal Cancer Registry, Hong Kong. PARTICIPANTS: One hundred and eight patients with proven familial adenomatous polyposis from 36 local Chinese families with the condition recruited to the Registry from 1995 to 2001. INTERVENTIONS: Screening programme for at-risk family members, prophylactic surgery at presymptomatic diagnosis, and surveillance programme for extracolonic lesions in affected individuals. MAIN OUTCOME MEASURES: Rate of colorectal cancer, type of surgical treatment, spectrum of extracolonic lesions, and management of the syndrome. RESULTS: Fifty patients suffered from colorectal cancer with a mortality rate of 78.0%. The strategy of presymptomatic diagnosis by screening and appropriate prophylactic surgery reduced the incidence of colorectal cancer. Affected individuals were prone to develop potentially serious extracolonic lesions including thyroid cancer (5.7%), desmoid tumour (15.7%), gastroduodenal adenomas (7.1%), duodenal microadenoma (17.1%), and pouch polyposis (17.4%). CONCLUSIONS: Screening and prophylactic surgery are effective ways to prevent colorectal cancer for patients with familial adenomatous polyposis. Lifelong regular surveillance is necessary to detect and manage extracolonic lesions. A dedicated registry is essential to coordinate clinical management and to compile data for furthering knowledge of this rare but complex syndrome.
Persistent Identifierhttp://hdl.handle.net/10722/45413
ISSN
2015 Impact Factor: 0.887
2015 SCImago Journal Rankings: 0.279

 

DC FieldValueLanguage
dc.contributor.authorHo, JWen_HK
dc.contributor.authorChu, KMen_HK
dc.contributor.authorTse, CWen_HK
dc.contributor.authorYuen, STen_HK
dc.date.accessioned2007-10-30T06:24:56Z-
dc.date.available2007-10-30T06:24:56Z-
dc.date.issued2002en_HK
dc.identifier.citationHong Kong Medical Journal = Xianggang Yi Xue Za Zhi / Hong Kong Academy Of Medicine, 2002, v. 8 n. 5, p. 342-347en_HK
dc.identifier.issn1024-2708en_HK
dc.identifier.urihttp://hdl.handle.net/10722/45413-
dc.description.abstractOBJECTIVES: To report on the phenotypic spectrum and clinical management of Chinese patients suffering from the rare autosomal dominant colorectal cancer syndrome of familial adenomatous polyposis. DESIGN: Analysis of prospectively collected data from the database of a regional registry. SETTING: The Hereditary Gastrointestinal Cancer Registry, Hong Kong. PARTICIPANTS: One hundred and eight patients with proven familial adenomatous polyposis from 36 local Chinese families with the condition recruited to the Registry from 1995 to 2001. INTERVENTIONS: Screening programme for at-risk family members, prophylactic surgery at presymptomatic diagnosis, and surveillance programme for extracolonic lesions in affected individuals. MAIN OUTCOME MEASURES: Rate of colorectal cancer, type of surgical treatment, spectrum of extracolonic lesions, and management of the syndrome. RESULTS: Fifty patients suffered from colorectal cancer with a mortality rate of 78.0%. The strategy of presymptomatic diagnosis by screening and appropriate prophylactic surgery reduced the incidence of colorectal cancer. Affected individuals were prone to develop potentially serious extracolonic lesions including thyroid cancer (5.7%), desmoid tumour (15.7%), gastroduodenal adenomas (7.1%), duodenal microadenoma (17.1%), and pouch polyposis (17.4%). CONCLUSIONS: Screening and prophylactic surgery are effective ways to prevent colorectal cancer for patients with familial adenomatous polyposis. Lifelong regular surveillance is necessary to detect and manage extracolonic lesions. A dedicated registry is essential to coordinate clinical management and to compile data for furthering knowledge of this rare but complex syndrome.en_HK
dc.format.extent289601 bytes-
dc.format.extent395336 bytes-
dc.format.extent3246 bytes-
dc.format.mimetypeapplication/pdf-
dc.format.mimetypeapplication/pdf-
dc.format.mimetypetext/plain-
dc.languageengen_HK
dc.publisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.htmlen_HK
dc.relation.ispartofHong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicineen_HK
dc.rightsCreative Commons: Attribution 3.0 Hong Kong License-
dc.subject.meshAdenomatous Polyposis Coli - epidemiology - genetics - therapyen_HK
dc.subject.meshColorectal Neoplasms - epidemiology - genetics - therapyen_HK
dc.subject.meshRegistriesen_HK
dc.subject.meshAdolescenten_HK
dc.subject.meshHong Kong - epidemiologyen_HK
dc.titlePhenotype and management of patients with familial adenomatous polyposis in Hong Kong: perspective of the Hereditary Gastrointestinal Cancer Registry.en_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=1024-2708&volume=8&issue=5&spage=342&epage=347&date=2002&atitle=Phenotype+and+management+of+patients+with+familial+adenomatous+polyposis+in+Hong+Kong:+perspective+of+the+Hereditary+Gastrointestinal+Cancer+Registryen_HK
dc.identifier.emailChu, KM: chukm@hkucc.hku.hken_HK
dc.identifier.authorityChu, KM=rp00435en_HK
dc.description.naturepublished_or_final_versionen_HK
dc.identifier.pmid12376711-
dc.identifier.scopuseid_2-s2.0-0036780370en_HK
dc.identifier.hkuros74933-
dc.identifier.volume8en_HK
dc.identifier.issue5en_HK
dc.identifier.spage342en_HK
dc.identifier.epage347en_HK
dc.publisher.placeHong Kongen_HK
dc.identifier.scopusauthoridHo, JW=7402649983en_HK
dc.identifier.scopusauthoridChu, KM=7402453538en_HK
dc.identifier.scopusauthoridTse, CW=36958765600en_HK
dc.identifier.scopusauthoridYuen, ST=7103160927en_HK

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