title: Use of rifampin for severe pruritus in children with chronic cholestasis.
abstract: BACKGROUND
  Rifampin has been proposed to reduce pruritus in children and adults with chronic cholestasis; however, there is a paucity of published data regarding the use of rifampin in children.
  
  
  METHODS
  In an open trial, 24 children were evaluated during a 6-year period. Diagnoses included 13 patients with extrahepatic biliary atresia (54%), six with Alagille's syndrome, three with Byler's disease, and one each with primary sclerosing cholangitis and alpha1-antitrypsin deficiency. All patients had severe pruritus that had not responded adequately to at least 2 months of therapy with ursodeoxycholic acid, diphenhydramine, or phenobarbital and local skin care measures. Treatment was initiated with rifampin, 10 mg/kg per day in two divided doses for 18+/-20 months, and the effect on the severity of pruritus was assessed by a clinical scoring system.
  
  
  RESULTS
  Ten patients showed a complete response, 12 a partial response, and 2 no response. Complete response was more common in extrahepatic cholestasis (64% vs. 10%), whereas partial response was more common in intrahepatic cholestasis (80% vs. 29%). Treatment was associated with reduction of gamma-glutamyl transpeptidase. No clinical or biochemical toxicity of rifampin was observed.
  
  
  CONCLUSIONS
  We conclude that for more than 90% of children with chronic cholestasis and severe pruritus unresponsive to other treatments, rifampin appears to be a safe and effective therapy.
authors.affiliation: Pediatric Liver Center, Department of Pediatrics, University of Colorado School of Medicine and The Children's Hospital, Denver 80218, USA.
authors.forename: B
authors.initials: B
authors.lastname: Yerushalmi
authors.forename: R J
authors.initials: RJ
authors.lastname: Sokol
authors.forename: M R
authors.initials: MR
authors.lastname: Narkewicz
authors.forename: D
authors.initials: D
authors.lastname: Smith
authors.forename: F M
authors.initials: FM
authors.lastname: Karrer
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000188:drug therapy*; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011537:Pruritus / Q000188:drug therapy*; D012293:Rifampin / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D012293:Rifampin