title: Multidisciplinary Treatment of Cystic Fibrosis-Related Recurrent Pyogenic Cholangitis (CF-RPC).
abstract: BACKGROUND
  As the survival of cystic fibrosis patients improves due to better treatment of its pulmonary manifestations, the management of hepatobiliary complications becomes increasingly vital. While focal biliary cirrhosis is common, large duct manifestations are less frequently encountered.
  
  
  METHODS
  We prospectively evaluated cases of large bile duct disease in a large adult cystic fibrosis practice at the Keck Hospital of the University of Southern California.
  
  
  RESULTS
  Over a 5-year period, six patients presented with cholangiectasia, hepatolithiasis, and strictures. Their clinical presentation and course closely resembled recurrent pyogenic cholangitis (RPC). Treatment of cholangitis and strictures was primarily by endoscopic retrograde cholangiopancreatography, but major hepatobiliary surgery following pulmonary optimization was required in 33 %.
  
  
  CONCLUSIONS
  In adult populations, CF-RPC may not be as unusual as previously reported and recognition allows optimal endoscopic, medical, and surgical management.
authors: Department of Medicine, Los Angeles County Hospital, Keck School of Medicine, University of Southern California, D & T Building Room B4H100, 1983 Marengo St., Los Angeles, CA, 90033-1370, USA, jbuxbaum@usc.edu.
authors: James
authors: J
authors: Buxbaum
authors: Nancy
authors: N
authors: Nguyen
authors: Sujit
authors: S
authors: Kulkarni
authors: Suzanne
authors: S
authors: Palmer
authors: Adupa
authors: A
authors: Rao
authors: Robert
authors: R
authors: Selby
mesh_terms: D000328:Adult; D002140:California; D002760:Cholangiopancreatography, Endoscopic Retrograde; D049448:Cholangiopancreatography, Magnetic Resonance; D002761:Cholangitis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D011446:Prospective Studies; D012008:Recurrence; D015607:Stents; D013492:Suppuration; D016896:Treatment Outcome
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