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Med Clin (Barc). 2007 Jun 9;129(2):51-2.

[Impact of a registry on survival in familial adenomatous polyposis].

[Article in Spanish]

Author information

1
Servicio de Aparato Digestivo, Hospital Universitario Son Dureta, Palma de Mallorca, Islas Baleares, España. jreyes@hsd.es

Abstract

BACKGROUND AND OBJECTIVE:

Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease characterized by hundreds of adenomatous polyps in the large intestine. Almost all affected untreated patients will die from colorectal cancer (CRC) at the age of 40-50 years. The Balearic Islands Polyposis Registry was established in 1988. The aim of the present study was to evaluate changes in the prevalence of CRC and the prognosis of FAP, before and after the establishment of the registry.

PATIENTS AND METHOD:

The diagnosis of FAP was defined by history, clinical examination, histopathological assessment and/or genetic testing. In this study we compare the data of probands and call-up patients. To evaluate the impact of the registry, selected variables were calculated for the periods 1970-1987 and 1988-2005.

RESULTS:

At the end of 2005 the registry included information of 19 families with 52 affected members (19 probands, 33 call-up). Thirty-six patients were alive and 16 had died. The cumulative 15 years survival was 82% in call-up cases compared with 47% in probands (p < 0.05). The cumulative 15 years survival was 87% for the period 1988-2005, compared with 33% for the period 1970-1987 (p < 0.001). The frequency of CRC was 72% for the period 1970-1987 and 21% for the period 1988-2005 (p < 0.005).

CONCLUSIONS:

The survival of the call-up patients was significantly improved as compared to the probands. Since the establishment of the registry, the frequency of CRC has decreased considerably, and the prognosis has improved substantially in FAP patients.

PMID:
17588361
[Indexed for MEDLINE]
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