28 AN INTERNATIONAL STUDY EVALUATING THE FARNESOID X RECEPTOR AGONIST OBETICHOLIC ACID AS MONOTHERAPY IN PBC
2011; Elsevier BV; Volume: 54; Linguagem: Inglês
10.1016/s0168-8278(11)60030-5
ISSN1600-0641
AutoresKris V. Kowdley, David Jones, Velimir A. Luketic, Robert Chapman, A.K. Burroughs, Gideon M. Hirschfield, Raoul Poupon, Christoph Schramm, C. Vincent, C Rust, Albert Parés, Andrew L. Mason, Cathi Sciacca, T. Beecher-Jones, O. Böhm, Erin Castelloe, Mark Pruzanski, Д. А. Шапиро,
Tópico(s)Inflammatory mediators and NSAID effects
ResumoConclusions: Individuals with ALGS due to JAG1-or NOTCH2-related disease have a similar pattern of hepatic involvement, while the prevalence of a renal phenotype is higher among NOTCH2 mutationpositive ALGS individuals.Facial features and vertebral defects are less penetrant, supporting the notion that the diagnostic criteria for ALGS require revision.Individuals with partial ALGS features should still be considered for this diagnosis and should undergo sequential JAG1 and then NOTCH2 mutation screening.
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