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FAP and other polyposis syndromes are generalised disorders with ramifications throughout the body. Early diagnosis by recognition of at-risk individuals and proctoscopic surveillance is important in preventing cancer. Surgical options now available can eliminate the risk of colorectal cancer and provide a good functional outcome without a permanent ileostomy. Ongoing surveillance programmes are important because there is still a risk of dying of extra-colic manifestations of the disorder. Prophylactic colectomy of whatever type does not predict total cure of the disease. The two most frequent procedures (IRA and IAA) have been evaluated [34]. The long-term survival following either approach is similar: 87% for IRA and 83% for IAA.

Management of these families is best accom plished within the framework of a familial polyposis registry. In the Czech Republic, there are more than 80 families registered. This allows for better communication with individual patients and family members, for identification of those at risk and for education of family members. These family members must be encouraged to undergo surveillance examinations so that the condition can be diagnosed early, and surgical intervention can prevent the development of colorectal cancer.

Interesting developments in the management of desmoids with medical agents should be further pursued in a prospective fashion in an effort to determine their true role in management of the condition. The potential of similar therapeutic agents, such as Sundilac, to promote regression of colic polyps has been suggested and requires further prospective investigation. Sundilac suppositories for patients with polyps in the rectal stump after IRA also show some promise. Whether the disappearance of polyps with the use of Sundilac eliminates the risk of developing cancer or simply eliminates the polyps is still undetermined.

There is also uncertainty regarding the management of upper gastrointestinal polyps in FAP. Further investigation into the local factors that dictate the development of adenomas in the duodenum and the polyp-cancer sequence in this area is necessary. The effect of bile on that sequence of events remains an important area for further research. The possibility of medical management of GI polyps is obviously attractive, and there are some ongoing studies that are seeking to determine whether medical agents can influence the development of upper GI polyps in FAP patients. It is hoped that further research into the chromosomal abnormality and a distinct blood tests for this disease are truly forthcoming because these could eliminate the problem of the unavailability of blood samples from other family members. The growth in the number of familial polyposis registries and the addition of increasing numbers of patients to them are certainly helpful for patients, their families and their physicians. The registries give researchers more long-term follow-up information on the clinical manifestations of the disease and on the results of medical and surgical treatment.

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