Cystic Fibrosis Involving Multisystem: A Case Report and Literature Review_West China Medical Journal

Authors：

WANG Ben ¹ ,  YANG Li ²

1. West China Medical School; 2. Departmnt of Digestive Medicine, West China Hospital; Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

YANG Li, Email: wangben1210@126.com

Keywords：

囊性纤维化; 支气管扩张; 肝硬化; 遗传性疾病

Video：

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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。

Citation： WANG Ben,YANG Li. Cystic Fibrosis Involving Multisystem: A Case Report and Literature Review. West China Medical Journal, 2012, 27(6): 852-853. doi: Copy

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3.	柴栋, 张梅, 王睿.肺囊性纤维化感染与细菌生物被膜的免疫逃逸[J].药物与临床, 2007, 17(2): 35-37.
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5.	Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic fibrosis pulmonary guidelines treatment of pulmonary exacerbations[J]. Am J Respir Crit Care Med, 2009, 180(9): 802-808.
6.	Stallings VA, Stark LJ, Robinson KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufﬁciency: results of a systematic review[J]. J Am Diet Assoc, 2008, 108(5): 832-839.
7.	Mendizabal M, Reddy KR, Cassuto J, et al. Liver transplantation in patients with cystic fibrosis: analysis of united network for organ sharing data[J]. Liver Transpl, 2011, 17(3): 243-250.
8.	Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: aprospective study on incidence, risk factors, and outcome[J]. Hepatology, 2002, 36(6): 1374-1382.
9.	Efrati O, Barak A, Dalit MM, et al. Liver cirrhosis and portal hypertension in cystic ﬁbrosis[J]. Eur J Gastroen Hepat, 2003, 15(10): 1073-1078.
10.	Lewindon PJ, Shepherd RW, Walsh MJ, et al. Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy[J]. Hepatology, 2011, 53(1): 193-201.
11.	Costa PC, Barreto CC, Pereira L, et al. Cystic fibrosis-related liver disease: a single-center experience[J]. Pediatr Rep, 2011, 3(3): 21.
12.	Colomboa C, Ellemunter H, Houwenc R，et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic ﬁbrosis patients[J]. J Cyst Fibros, 2011, 10(Suppl 2): 24-28.
13.	Flume PA, Stenbit A. Making the diagnosis of cystic fibrosis[J]. Am J Med Sci, 2008, 335(1): 51-54.

1. 　于宁, 李汉忠,李宏军. 囊性纤维化病相关基因突变及其检测的研究进展[J]. 中国男科学杂志, 2007, 21(4): 51-53.
2. 　Davis PB. Cystic fibrosis since 1938[J]. Am J Respir Crit Care Med, 2006, 173(5): 475-82.
3. 　柴栋, 张梅, 王睿.肺囊性纤维化感染与细菌生物被膜的免疫逃逸[J].药物与临床, 2007, 17(2): 35-37.
4. 　Stenbit A, Flume PA. Pulmonary Complications in adult patients with cystic fibrosis[J]. Am J Med Sci, 2008, 335(1): 55-59.
5. 　Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic fibrosis pulmonary guidelines treatment of pulmonary exacerbations[J]. Am J Respir Crit Care Med, 2009, 180(9): 802-808.
6. 　Stallings VA, Stark LJ, Robinson KA, et al. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufﬁciency: results of a systematic review[J]. J Am Diet Assoc, 2008, 108(5): 832-839.
7. 　Mendizabal M, Reddy KR, Cassuto J, et al. Liver transplantation in patients with cystic fibrosis: analysis of united network for organ sharing data[J]. Liver Transpl, 2011, 17(3): 243-250.
8. 　Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in cystic fibrosis: aprospective study on incidence, risk factors, and outcome[J]. Hepatology, 2002, 36(6): 1374-1382.
9. 　Efrati O, Barak A, Dalit MM, et al. Liver cirrhosis and portal hypertension in cystic ﬁbrosis[J]. Eur J Gastroen Hepat, 2003, 15(10): 1073-1078.
10. 　Lewindon PJ, Shepherd RW, Walsh MJ, et al. Importance of hepatic fibrosis in cystic fibrosis and the predictive value of liver biopsy[J]. Hepatology, 2011, 53(1): 193-201.
11. 　Costa PC, Barreto CC, Pereira L, et al. Cystic fibrosis-related liver disease: a single-center experience[J]. Pediatr Rep, 2011, 3(3): 21.
12. 　Colomboa C, Ellemunter H, Houwenc R，et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic ﬁbrosis patients[J]. J Cyst Fibros, 2011, 10(Suppl 2): 24-28.
13. 　Flume PA, Stenbit A. Making the diagnosis of cystic fibrosis[J]. Am J Med Sci, 2008, 335(1): 51-54.

West China Medical Journal

Cystic Fibrosis Involving Multisystem: A Case Report and Literature Review

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