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老年囊性纤维化患者的胎粪性肠梗阻等效征

Meconium ileus equivalent in older patients with cystic fibrosis.

作者信息

Hanly J G, Fitzgerald M X

出版信息

Br Med J (Clin Res Ed). 1983 Apr 30;286(6375):1411-3. doi: 10.1136/bmj.286.6375.1411.

Abstract

Meconium ileus equivalent is one of the lesser known manifestations of cystic fibrosis, and occurs most often in older patients. With the improved overall survival of patients with cystic fibrosis, one would expect to see this condition more often in the future. Of 53 patients attending our cystic fibrosis clinic for adolescents and adults, eight had experienced a total of 25 episodes of meconium ileus equivalent. Recurrent attacks occurred in seven patients, of whom four had at least four separate, well documented episodes: these episodes were associated with obvious recognised precipitating factors in only three patients. All patients responded to appropriate medical treatment with acetylcysteine or sodium diatrizoate, including three who had previously undergone surgery for meconium ileus equivalent elsewhere. Controversies remain, however, concerning the role of prophylactic medical management with acetylcysteine, sodium diatrizoate, and pancreatic supplements.

摘要

胎粪性肠梗阻等效症是囊性纤维化较少见的表现之一,多见于老年患者。随着囊性纤维化患者总体生存率的提高,预计未来这种情况会更常见。在我们诊所就诊的53例青少年及成人囊性纤维化患者中,8例共经历了25次胎粪性肠梗阻等效症发作。7例患者有复发发作,其中4例至少有4次独立且记录良好的发作:这些发作仅在3例患者中有明显公认的诱发因素。所有患者对乙酰半胱氨酸或泛影葡胺的适当药物治疗均有反应,包括3例之前在其他地方因胎粪性肠梗阻等效症接受过手术的患者。然而,关于乙酰半胱氨酸、泛影葡胺和胰腺补充剂的预防性药物治疗的作用仍存在争议。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/53d4/1547848/e633c69e6421/bmjcred00551-0038-a.jpg

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