特集名 | 遺伝性大腸疾患の最近の話題 | |
---|---|---|
題名 | ポリポーシスの最近の話題 (5) Familial hyperplastic polyposis | |
発刊年月 | 2008年 08月 | |
著者 | 中村 眞一 | DPR株式会社 |
著者 | 及川 守康 | DPR株式会社 |
著者 | 菅井 有 | 岩手医科大学医学部臨床病理部門 |
著者 | 折居 正之 | 岩手医科大学医学部第一内科 |
【 要旨 】 | familial hyperplastic polyposis (FHPP) は現在までに7家系,20人が報告されているにすぎない.臨床病理学的特徴では,診断時年齢は26 - 77歳 (平均57.1歳),男女比11 : 9,多発するhyperplastic polyp (HP)のほかに10 mm以上のHPや鋸歯状腺腫,通常の腺腫の発生があり,大腸癌の合併も7人 (35 %) にみられた.責任遺伝子は明らかでなく,遺伝様式は常染色体優性および劣性遺伝であった.FHPPには,正確な診断と他の家族性消化器系腫瘍性疾患との鑑別が重要である.診断後は患者の大腸癌のsurveillance,患者家族の病変のスクリーニングなどが必要となる. |
Theme | Recent Topics of Hereditary Colorectal Disease | |
---|---|---|
Title | Familial Hyperplastic Polyposis | |
Author | Shin-ichi Nakamura | DPR Co., Ltd |
Author | Moriyasu Oikawa | DPR Co., Ltd |
Author | Tamotsu Sugai | Department of Clinical Pathology, Iwate Medical University School of Medicine |
Author | Seishi Orii | First Department of Internal Medicine, Iwate Medical University School of Medicine |
[ Summary ] | Familial hyperplastic polyposis (FHPP) is a rare disease which has been reported in only 7 families, with a total of 20 patients. The clinico-pathological characteristics of FHPP were as follows ; patient ages at diagnosis of FHPP, 22 - 77 (mean 57.1 years old), male / female ratio, 11 / 9 and colorectal lesions consisting of multiple hyperplastic polyps, some of which exceeded 10 mm in diameter, serrated adenomas, and tradifional adenomas. Seven (35 %) were affected by colonic carcinomas. The responsible gene for FHPP was not found. However, dominant or recessive inheritance patterns for FHPP were reported. Precise diagnosis of FHPP, including differential diagnosis from other familial digestive neoplastic diseases, is important. After a diagnosis of FHPP, surveillance for colorectal cancer and screening of diseases in all patient family members is necessary. |