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1例伴有复发性双侧气胸和主动脉环扩张的马方综合征病例

[A case of Marfan syndrome with recurrent bilateral pneumothorax and anuloaortic ectasia].

作者信息

Yoshimura N, Asada T, Matsuda H, Nohara H, Higami T, Nishiwaki M, Mukohara N, Chibana M, Ogawa K

机构信息

Division of Cardiovascular Surgery, Hyogo Brain and Heart Center at Himeji.

出版信息

Kyobu Geka. 1990 Feb;43(2):157-9.

PMID:2308250
Abstract

A 17-year-old male patient with Marfan syndrome was admitted due to recurrent bilateral pneumothorax which had recurred totally 11 times during the past 3 years. For the treatment of obstinately continuing right pneumothorax resection of bullae in combination with pleuropexy using OK 432 was effective. Two months thereafter Bentall operation with a composite graft consisting of a woven Dacron tube and a Björk-Shiley 27 mm aortic valve prosthesis was performed for his anuloartic ectasia. Left pneumothorax recurred 2 weeks after Bentall operation. But it was treated successfully with OK 432 injection into the left pleural cavity. Now, he is doing well as a student.

摘要

一名17岁患有马凡综合征的男性患者因双侧气胸反复发作入院,在过去3年中总共复发了11次。对于顽固持续的右侧气胸,采用OK-432联合大疱切除术及胸膜固定术治疗有效。两个月后,为其主动脉瓣环扩张症施行带编织涤纶人工血管和27mm Björk-Shiley主动脉瓣假体的复合移植物的Bentall手术。Bentall手术后2周左侧气胸复发。但通过向左侧胸腔注射OK-432成功治愈。现在,他作为一名学生情况良好。

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