%0 Journal Article
%T Bile Acid Synthesis Defect and Hyperinsulinism
%A Francisco Sylvester
%A Michael Rogers
%A Steven Lichtman
%J Archive of "ACG Case Reports Journal".
%D 2018
%R 10.14309/crj.2018.89
%X Congenital defects of bile acid synthesis are rare disorders that cause progressive liver dysfunction. Prolonged neonatal hyperinsulism (PNH) is a separate entity that leads to persistent hypoglycemia secondary to stress. We present a 4-month-old infant who presented with liver failure secondary to a bile acid synthesis defect. The patient¡¯s liver failure resolved with oral cholic acid therapy. This patient also developed PNH, which slowly resolved over time. This case illustrates a possible relationship between cholestatic liver failure and PNH. This relationship may help define specific stressors that increase the likelihood of developing PNH
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6358580/