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囊性纤维化累及多系统一例
引用本文:王犇,杨丽.囊性纤维化累及多系统一例[J].华西医学,2012(6):852-854.
作者姓名:王犇  杨丽
作者单位:四川大学华西临床医学院;四川大学华西医院消化内科
摘    要:目的提高对囊性纤维化的认识。方法 2011年11月收治1例自幼有临床表现的囊性纤维化患者,回顾其诊断及治疗经过,复习相关文献总结其临床特征、诊疗进展及预后评价。结果囊性纤维化起病年龄较早,患者自幼年起即反复出现肺、消化道、肝脏等多系统病变,最终导致多器官功能衰竭。结论应提高对囊性纤维化的识别度,对于发病年龄过早、反复发作的严重支气管扩张,伴随生长发育延迟、肝硬化等临床征象应注意对囊性纤维化的筛查。

关 键 词:囊性纤维化  支气管扩张  肝硬化  遗传性疾病

Cystic Fibrosis Involving Multisystem: A Case Report and Literature Review
WANG Ben,YANG Li.Cystic Fibrosis Involving Multisystem: A Case Report and Literature Review[J].West China Medical Journal,2012(6):852-854.
Authors:WANG Ben  YANG Li
Institution:1.West China Medical School;2.Departmnt of Digestive Medicine,West China Hospital;Sichuan University,Chengdu,Sichuan 610041, P.R.China
Abstract:Objective To know more about cystic fibrosis.Methods A patient with cystic fibrosis with typical clinical features was diagnosed and treated.We retrospectively analyzed and evaluate the clinical characteristics,diagnosis and treatment,and the prognosis.Results The onset of cystic fibrosis was early.This patient had repeating multisystem diseases including lung,pancreas,digestive system,liver,etc from a child,and she finally had multiple organ failure.Conclusion We should know more about cystic fibrosis,and should screen the patients with early onset and repeating severe bronchiectasis with delaying growing development and hepatocirrhosis.
Keywords:Cystic fibrosis  Bronchiectasis  Bepatocirrhosis  Hereditary diseases
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