全内脏转位、支气管扩张、副鼻窦病变三联征又称 Kartagener 氏综合征。本病较罕见,尤其伴蝶鞍畸形国内未见报道。我们最近发现1例。杜×,女,18岁,学生.头胀、头痛、鼻塞、流脓涕,反复咳嗽吐脓痰及血丝痰10余年,1977年发现“右位心”,因患“肺炎”曾3次注院并2次退学达16个月之久。其父母非近亲结婚,祖父母及2个弟弟无类似表现。 Total visceral transposition, bronchiectasis, sinus paranasal triad also known as Kartagener’s syndrome. The disease is rare, especially with no domestic report of sella deformity. We have recently found one case. Du ×, female, 18 years old, students .Total swelling, headache, stuffy nose, purulent tears, repeated cough spit pus and bloody sputum for more than 10 years, 1977 found “right centroid”, suffering from “pneumonia” Hospital and withdraw two times for 16 months. His parents were married non-relatives, grandparents and two younger brothers had no similar performance.