Ileal bile acid transport inhibitors - A new future in the treatment of cholestatic diseases.

We present a 14-year-old male with PFIC1 who required double liver transplant at five years old. Subsequently, liver function normalized, nevertheless, bowel habits and intestinal malabsorption worsened (10-12 daily and nocturnal stools, Bristol 6-7), with severe dehydration that required multiple hospitalizations, associating malnutrition and failure to thrive that required daily home parenteral nutrition(PN). At 14 years old, Odevixibat (40 mcg/kg/day) was initiated, leading to a marked improvement in bowel habits by the second day (1-2 daily stools, Bristol 2) and progressive weight gain, which allowed after 40 days of treatment discontinue PN following seven years.