Refractory infantile diarrhea acid malabsorption due to primary bile

A boy is described who had severe, refractory diarrhea beginning soon after birth. Prolonged parenteral alimentation was required to support life. Investigations of bile acid metabolism showed impaired intestinal absorption of bile acids. Contracted bile acid pool sizes resulted, with low intraluminal bile acid concentrations and severe malabsorption of water and fat. Bile acid malabsorption is a cause of refractory infantile diarrhea.