Reduction of Recurrence Risk of Pancreatitis in Cystic Fibrosis With Ivacaftor: Case Series

被引：57

作者：

Carrion, Andres ^{[1
]}

Borowitz, Drucy S. ^{[2
]}

Freedman, Steven D. ^{[3
]}

Siracusa, Christopher M. ^{[4
]}

Goralski, Jennifer L. ^{[5
]}

Hadjiliadis, Denis ^{[6
]}

Srinivasan, Saumini ^{[1
]}

Stokes, Dennis C. ^{[1
]}

机构：

[1] Univ Tennessee, Le Bonheur Childrens Hosp, Hlth Sci Ctr, Memphis, TN USA

[2] Univ Buffalo Jacobs Sch Med & Biomed Sci, Women & Childrens Hosp Buffalo, Buffalo, NY USA

[3] Harvard Med Sch, Beth Israel Deaconess Med Ctr, Boston, MA USA

[4] Cincinnati Childrens Med Ctr, Cincinnati, OH USA

[5] Univ N Carolina, Chapel Hill, NC USA

[6] Univ Penn, Philadelphia, PA 19104 USA

来源：

JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION | 2018年 / 66卷 / 03期

关键词：

cystic fibrosis; ivacaftor; recurrent pancreatitis; TRANSMEMBRANE CONDUCTANCE REGULATOR; G551D MUTATION; LOCALIZATION; POTENTIATOR;

D O I：

10.1097/MPG.0000000000001788

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

The effect of ivacaftor in patients with cystic fibrosis (CF) with recurrent pancreatitis is unknown. We conducted a multicenter retrospective study of patients with CF taking ivacaftor who had a history of recurrent pancreatitis. During the first 3 months of therapy, only 1 of the 6 patients had an episode of pancreatitis, which was managed on an outpatient basis. Between 3 and 12 months on ivacaftor therapy, none of the patients had recurrence of pancreatitis or required hospitalization. The use of ivacaftor was associated with a reduced frequency and recurrence rate of pancreatitis in patients with CF.

引用

页码：451 / 454

页数：4

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