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：

囊性纖維化; 支氣管擴張; 肝硬化; 遺傳性疾病

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

目的　提高對囊性纖維化的認識。方法　2011年11月收治1例自幼有臨床表現的囊性纖維化患者，回顧其診斷及治療經過，復習相關文獻總結其臨床特征、診療進展及預后評價。結果　囊性纖維化起病年齡較早，患者自幼年起即反復出現肺、消化道、肝臟等多系統病變，最終導致多器官功能衰竭。結論　應提高對囊性纖維化的識別度，對于發病年齡過早、反復發作的嚴重支氣管擴張，伴隨生長發育延遲、肝硬化等臨床征象應注意對囊性纖維化的篩查。

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

1.	于寧, 李漢忠,李宏軍. 囊性纖維化病相關基因突變及其檢測的研究進展[J]. 中國男科學雜志, 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.	柴棟, 張梅, 王睿.肺囊性纖維化感染與細菌生物被膜的免疫逃逸[J].藥物與臨床, 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 insuf?ciency: 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 ?brosis[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 ?brosis 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. 　于寧, 李漢忠,李宏軍. 囊性纖維化病相關基因突變及其檢測的研究進展[J]. 中國男科學雜志, 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. 　柴棟, 張梅, 王睿.肺囊性纖維化感染與細菌生物被膜的免疫逃逸[J].藥物與臨床, 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 insuf?ciency: 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 ?brosis[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 ?brosis 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

Abstract Full text Figures/Tables Video References Cited by

Format

Content