【病例学习第17期】Cystic Fibrosis（囊性纤维化）

From Pharmacy Times(www.pharmacytimes.com)
Thanks to the author Craig I. Coleman, PharmD(Dr. Coleman is an assistant professor of pharmacy practice and director of the pharmacoeconomics and outcomes studies group at the University of Connecticut School of Pharmacy.)

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Cystic Fibrosis

A mother, on behalf of her 17-year-old daughter, arrives at the pharmacy counter and presents a prescription for azithromycin 500 mg 3 times weekly to a fourth-year pharmacy student intern. The student is confused about the azithromycin dosing prescribed and, eager to make a valuable clinical recommendation, excitedly walks over to his precepting pharmacist to ask if he can call the physician to get the prescription changed to the common Z-pak dosing. The pharmacist and student look up the daughter's profile and note that she is receiving pancreatic enzymes, an ADEK vitamin supplement, inhaled tobramycin, dornase alfa, and nebulized albuterol.

How should the pharmacist respond to the pharmacy student?


ANSWER

Cystic Fibrosis

Based on the patient's prescription profile, it is clear that the patient has cystic fibrosis. Azithromycin is thought to modulate a cystic fibrosis patient's overactive inflammatory response, resulting in improvements in pulmonary function, nutritional status, health-related quality of life, and decreases in pulmonary exacerbation rates (particularly in patients chronically infected with Pseudomonas). Current cystic fibrosis treatment guidelines state that the use of azithromycin is associated with a "substantial" net benefit to the patient and recommends doses in the range of 250 to 500 mg 3 times weekly. (Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176(10):957-969.) The pharmacist should tell the student that the prescription is correct as written.

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囊性纤维化一位母亲来到药房柜台，为她十七岁的女儿咨询一张处方：阿奇霉素500毫克，每周三次，接待她的是一位药学实习生。这位实习生觉得阿奇霉素的剂量不大对劲，应该建议修正，就兴冲冲地走过去问带教药师：自己可不可以建议临床医生将阿奇霉素的剂量改为常规剂量？二人一起浏览了这张处方，发现病人目前在服用的药物有：胰酶、ADEK维生素，吸入妥布霉素，阿法链道酶和沙丁胺醇喷雾。药师该如何回答实习生的提问？

答：
从这张处方上看，显然病人患有囊性纤维病。阿奇霉素能够调整囊性纤维化病人过度的炎症应答反应，从而改善肺功能和营养状态，提高生活质量，并延缓肺病加重的速度（尤其是有假单胞菌感染的病人）。最新的囊性纤维化治疗指南指出，阿奇霉素可使病人获得“实质上的”净益处，推荐剂量为250mg－500mg,每周3次 (Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2007;176(10):957-969.) 。药师应告诉实习生，处方正确无误。

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