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[成人囊性纤维化]

[Cystic fibrosis in adults].

作者信息

Polu J M, Lesur O, Delorme N

机构信息

Service des maladies respiratoires et réanimation respiratoire, CHU de Brabois, Nancy.

出版信息

Rev Prat. 1990 Jun 11;40(17):1581-6.

PMID:2363014
Abstract

While fifty years ago 20 p. 100 of cystic fibrosis patients only reached the age of one year, more than 50 p. 100 of the patients now live more than twenty years. The clinical manifestations of cystic fibrosis are more diverse in adults than in children, so that the diagnosis might concern several specialties. In actual fact, only 3 to 7 p. 100 of cystic fibroses are diagnosed after thirteen to sixteen years, and in half the cases the symptoms had been present before the age of one year. In adults, the respiratory manifestations of cystic fibrosis are predominant, whereas the gastrointestinal manifestations tend to be blurred. Radiography of the chest shows interstitial lesions (opacities, cystic images, disorders of ventilation), principally located in the right side and the apex. The most common functional defect is an obstructive syndrome corresponding to a gradual involvement of the peripheral airways. A number of complications may develop, including recurrent Pseudomonas infection of the lung, pneumothorax, heart failure, malnutrition, liver cirrhosis, episodes of intestinal occlusion, etc. The longer life span of these patients raises the problems of diabetes with its vascular complications, infertility or pregnancy, social and professional insertion, and so forth. The prognosis of cystic fibrosis in adults depends on the date the diagnosis was made, on the therapeutic follow-up and on the creation of specialized centres. The control of Pseudomonas infections and the development of lung transplantation are the main advances to be expected.

摘要

五十年前,20%的囊性纤维化患者只能活到一岁,而现在超过50%的患者能活过二十岁。囊性纤维化在成人中的临床表现比儿童更为多样,因此诊断可能涉及多个专科。实际上,只有3%至7%的囊性纤维化患者在13至16岁后才被诊断出来,且在半数病例中,症状在一岁前就已出现。在成人中,囊性纤维化的呼吸道表现最为突出,而胃肠道表现往往不明显。胸部X光显示间质性病变(模糊影、囊性影像、通气障碍),主要位于右侧和肺尖。最常见的功能缺陷是阻塞性综合征,这与外周气道的逐渐受累有关。可能会出现多种并发症,包括肺部反复感染铜绿假单胞菌、气胸、心力衰竭、营养不良、肝硬化、肠梗阻发作等。这些患者寿命的延长引发了糖尿病及其血管并发症、不孕或妊娠、社会和职业融入等问题。成人囊性纤维化的预后取决于诊断时间、治疗随访情况以及专业中心的建立。控制铜绿假单胞菌感染和开展肺移植是预期的主要进展。

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