Article
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function.
The Journal of pediatrics - 1 Sept 2007
Pall Harpreet, Zielenski Julian, Jonas Maureen M, DaSilva Deborah A, Potvin Kimberly M, Yuan Xiao-Wei, Huang Qiuju, Freedman Steven D
Abstract excerpt
OBJECTIVE: To determine whether primary sclerosing cholangitis (PSC) in childhood is associated with abnormalities in cystic fibrosis transmembrane conductance regulator (CFTR). STUDY DESIGN: Subjects with PSC diagnosed in childhood (n = 20) were recruited from Children's Hospital. Subjects had testing with sweat chloride concentration, nasal transmembrane potential difference, and extensive genetic analysis of...
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