Синдром Пейтца-Єгерса
ORPHA:2869· ICD-10 Q85.8· Peutz-Jeghers syndrome
Визначення(English summary)
A genetic intestinal polyposis syndrome characterized by development of characteristic hamartomatous polyps throughout the gastrointestinal (GI) tract, and by mucocutaneous pigmentation. This disorder carries a considerably increased risk of GI and extra-GI malignancies.
- Поширеність
- 1-9 / 100 000
- Успадкування
- Autosomal dominant
- Вік початку
- Adolescent, Adult, Childhood