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  • Kenneth D. R. Setchell, M Schwarz, +7 authors D. W. Russell
  • Biology, Medicine
  • The Journal of clinical investigation
  • 1998 (First Publication: 1 November 1998)
  • We describe a metabolic defect in bile acid synthesis involving a deficiency in 7alpha-hydroxylation due to a mutation in the gene for the microsomal oxysterol 7alpha-hydroxylase enzyme, active inExpand
  • Michael Joseph Krowka, Michael B. Fallon, +5 authors Ronald J. Sokol
  • Medicine
  • Transplantation
  • 2016 (First Publication: 21 June 2016)
  • Two distinct pulmonary vascular disorders, hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) may occur as a consequence of hepatic parenchymal or vascular abnormalities. HPS andExpand
  • Edward J. Hoffenberg, Michael R. Narkewicz, Judith M. Sondheimer, David J. Smith, Arnold R. Silverman, Ronald J. Sokol
  • Medicine
  • The Journal of pediatrics
  • 1995 (First Publication: 1 August 1995)
  • OBJECTIVE To determine the outcome, in index patients followed at an American Center, of syndromic paucity of interlobular bile ducts (sPILBD; Alagille syndrome), with onset of cholestasis inExpand