English / The Danish Polyposis Register

The Danish Polyposis Register

The Danish Polyposis Register is a clinical database including all Danish patients with familial adenomatous polyposis (FAP).

The aim of the register is to improve the prognosis in FAP by:

1. Registration of FAP patients living in Denmark
2. Construction of the pedigrees of FAP patients
3. Identification of first degree relatives of affected family members
4. DNA analysis of affected family members
5. Prophylactic molecular genetic or endoscopical examination of all biological first     degree relatives
6. Prophylactic colectomy in newly diagnosed FAP patients
7. Postoperative surveillance
8. Information to medical colleagues about examination, treatment and surveillance
9. Research
10. Counselling of patients and family members
11. Information to health care personnel, the authorities and the public

The history of the Danish Polyposis Register

The Danish Polyposis Register was established in 1961 by Mogens Sprechler as a local register of patients in 10 families treated in Bispebjerg Hospital in Copenhagen. In a leading article in Ugeskrift for Læger in 1971 he invited to the establishment of a national polyposis register according to the British and Swedish model, and in 1976 Steffen Bülow was handed over the task of extending the register to include the entire country. On the background of data from the Danish Cancer Register the medical journals were examined in 951 patients with colorectal cancer below the age of 40 years in 1943-67, and this resulted in the identification of several new cases of FAP.

During the following years the pedigrees of the patients were constructed, information was obtained from the Central Office of Civil Registration, local population registers, parish registers and death certificates. Family members at risk were identified, and through their general practitioner informed of their risk of having inherited the disease and advised to have a prophylactic sigmoidoscopy. In 1980 the Polyposis Register was approved by the Danish Data Protecting Agency. In 1986 Steffen Bülow defended his thesis about FAP on the background of 319 patients, including 168 probands and 79 call-up cases. The completeness of registration was 97% in 1992.

In the beginning of the 1990’s Helle Højen was engaged as the secretary in the register. In cooperation with Flemming Moesgaard the register became computerised, and later on Hanne Dinesen, HD Support, transferred the data into the present ”WinPoly”-database. The pedigrees are constructed in the pedigree software Cyrillic.

At the end of 2017 the register included 226 FAP families with 717 affected members. Colorectal cancer was found at the diagnosis of FAP in 62% of the probands, but only in 2% of call-up cases. 

Status in 2018

Niels Jespersen (consultant in the Department of Surgical Gastroenterology) is director of the registry, and in charge of treatment and control of polyposis patients in the department. The former director Steffen Bülow is advisor to the register concerning continuous updating and validation of the register. John Gásdal Karstensen is research fellow.

Helle Højen is secretary of the Polyposis Register.

Previously the update of data depended on information from the surgical departments about a new development in their polyposis patients, and in addition the register asked for copies of the medical record at intervals. 

Over the last years admission to electronic medical records has been improved. Concerning patients in the Copenhagen Region the Polyposis Register is able to collect data directly, and the surgical departments in Aarhus and Odense inform the Polyposis Register about new developments in their polyposis patients, e.g. the first endoscopy in a gene carrier, colectomy, development of desmoid or advanced duodenal adenomatosis. Other surgical departments are asked to do the same.

Data on 1st degree relatives are continuously updated to ensure that they are offered DNA analysis or, in families without a known mutation, a regular sigmoideoscopic screening.

Staff
Niels Jespersen, MD. Director 
Steffen Bülow, MD, DMSc: Consultant and webmaster
John Gásdal Karstensen, MD, PhD. Research fellow 
Helle Højen: Secretary
 
Contact
The Danish Polyposis Register
Department of Surgical Gastroenterology, Section 360
Hvidovre University Hospital
DK-2650 Hvidovre
Denmark
Tel.: +45 3862 2236
Fax: +45 3862 3760
Send mail 
Steffen Bülow | Tjørnekrogen 4, Gentofte - Danmark | Tlf.: 21472454