Essential fatty acid deficiency in congenital biliary atresia: successful treatment to reverse deficiency.
Supplementation of lipid intake by infusion of solutions of essential fatty acid (EFA)-rich powder through Suruga II enterostomy was carried out for the treatment of EFA deficiency in nine children with postoperative congenital biliary atresia (CBA). Infusion of EFA-rich powder dissolved in excreted bile was effective in six patients except for a case who had a total bile acid concentration in the excreted bile that was less than critical micellar level. Administration of EFA-rich powder dissolved in a mixture of the patient's own bile and 1 to 2 mmol/L taurocholate (TC) solution corrected EFA deficiency in three children with total bile acid concentration lower than the critical level. Our results therefore show that infusion of EEA-rich lipid through Suruga II enterostomy after dissolving in the excreted bile is an effective treatment for EFA deficiency in postoperative patients with CBA, and that 1 to 2 mmol/L TC solution used as artificial bile facilitates lipid absorption in cases with total bile acid in the bile less than the critical micellar level.
Miyano
T
T
Yamashiro
Y
Y
Shimizu
T
T
Arai
T
T
Suruga
T
T
Hayasawa
H
H
D001647:Bile Acids and Salts / Q000378:metabolism; D001652:Bile Ducts / Q000002:abnormalities*; D002675:Child, Preschool; D004750:Enteral Nutrition; D005228:Fatty Acids, Essential / Q000008:administration & dosage / Q000172:deficiency*; D005260:Female; D005527:Food, Fortified; D006801:Humans; D007223:Infant; D008286:Malabsorption Syndromes / Q000628:therapy*; D008297:Male; D011183:Postoperative Complications / Q000628:therapy*
D001647:Bile Acids and Salts; D005228:Fatty Acids, Essential