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囊性纤维化继发胎粪性肠梗阻。东伦敦的经验。

Meconium ileus secondary to cystic fibrosis. The East London experience.

作者信息

Mushtaq I, Wright V M, Drake D P, Mearns M B, Wood C B

机构信息

Department of Paediatric Surgery, Queen Elizabeth Hospital for Children, Hackney Road, London, UK.

出版信息

Pediatr Surg Int. 1998 Jul;13(5-6):365-9. doi: 10.1007/s003830050341.

Abstract

Meconium ileus (MI) affects 15% of neonates with cystic fibrosis (CF). The authors reviewed the management and outcome of 51 neonates presenting to a single institution between 1976 and 1995 with MI secondary to CF. Clinical presentation included abdominal distension (96%), bilious vomiting (49%), and delayed passage of meconium (36%). A family history of CF was present in 4 cases (8%). Twenty-three neonates presented with MI and evidence of volvulus, atresia, or perforation (complicated MI). Of these, 16 underwent stoma formation, 1 appendicectomy, and 6 resection with primary anastomosis. Twenty-eight neonates presented with uncomplicated MI. Of these, 11 were managed non-operatively by Gastrografin enema (10) or enteral N-acetylcysteine (1). The remainder required stoma formation (15) or bowel resection with primary anastomosis (2). Early postoperative complications occurred in 2 neonates (4%). In this hospital the 1-year survival for this condition has increased from 49% (1953-1970) to 98% (1976-1995) irrespective of the surgical procedure performed or the presence of volvulus, atresia, or perforation. In our experience, bowel resection with primary anastomosis is as safe as stoma formation and is associated with a reduced length of initial hospital stay.

摘要

胎粪性肠梗阻(MI)影响15%的囊性纤维化(CF)新生儿。作者回顾了1976年至1995年间在单一机构就诊的51例继发于CF的MI新生儿的治疗及预后情况。临床表现包括腹胀(96%)、胆汁性呕吐(49%)和胎粪排出延迟(36%)。4例(8%)有CF家族史。23例新生儿表现为MI并有肠扭转、闭锁或穿孔的证据(复杂性MI)。其中,16例行造口术,1例行阑尾切除术,6例行切除并一期吻合术。28例新生儿表现为非复杂性MI。其中,11例通过泛影葡胺灌肠(10例)或肠内给予N-乙酰半胱氨酸(1例)进行非手术治疗。其余患儿需要行造口术(15例)或肠切除并一期吻合术(2例)。2例新生儿(4%)发生早期术后并发症。在本院,无论采取何种手术方式或是否存在肠扭转、闭锁或穿孔,这种疾病的1年生存率已从49%(1953 - 1970年)提高到98%(1976 - 1995年)。根据我们的经验,肠切除并一期吻合术与造口术一样安全,且初始住院时间缩短。

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