大腸腺腫症を除く大腸ポリポーシスの中,とくにCronkhite-Canada症候群についてのべた.本症候群は1985年末までに,世界で154例の報告があり,その中,本邦報告例は110例で,本邦に多発している.発症の誘因として,精神的,肉体的ストレスがあり,初発症状には味覚異常がもっとも多く,次いで下痢,脱毛,爪甲萎縮皮膚色素沈着をきたす症例が多い.本症候群の消化管ポリープは若年性ポリープに類似し,その可逆性であることより,成因に炎症性機序が推定される.予後については死亡例は発症後2年以内に死亡しており,報告例の半数は現在,生存中で,10年以上の生存例もある.治療はステロイド,抗プラスミン剤の著効例が多いが,対症療法のみで軽快した症例もある。癌の合併は19例(17.2%)であり,偶発的発症が考慮される.Peutz-Jeghers症候群および若年性ポリポーシスについてはポリープの悪性化,消化管病変以外の病変および治療についてのべた.
Colon polyposis excluding colon adenomatosis were reviewed especially about Cronkhite-Canada syndrome in Japanese cases.
One hundred and ten cases of Cronkhite- Canada syndorome were reported in Japan from 1958 to 1986. Two third of all cases of Cronkhite-Canada syndrome reported in world literature were reported from Japan. Hypogeusia, the most frequent initial syndrome, is usually followed by diarrhea, nail dystrophia and skin pigmentation. The gastrointestinal polyps of Cronkhite-Canada syndrome resemble juvenile polyps and are frequnetly reversible. The etilology of poyps are suggested an inflammatory process. The most of all death cases died within two years. About of all reported cases are alive in good condition. Steroid and antiplasmin drugs are most effective in majority cases. The complication with cancer were reported in 19 cases (17.2%) and incidential associations are suspected.
Concerning about Peutz-Jeghers syndrome and Juvenile polyposis, malignant chagnes, lesions of excluding gastrointestinal tract and treatment were discussed with recent studies.
