DOCOSAHEXAENOIC ACID STATUS OF PATIENTS WITH EXTRAHEPATIC BILIARY ATRESIA

Docosahexaenoic acid (DHA) is believed to be an important long-chain p olyunsaturated fatty acid (LCPUFA), which may be essential for neurofu nction in infants. Patients with extrahepatic biliary atresia (EBA) ma y have DHA deficiency secondary to fat malabsorption. The authors inve stigated DHA and other LCPUFA levels in plasma and red blood cell (RBC ) phospholipids of patients after the Kasai portoenterostomy and after supplementation with essential fatty acids. Ten children aged 8 to 17 months (mean, 12.6 months) comprised the study group. Five were jaund iced and five had a normal bilirubin level. The patients received 1 mL /kg of fat emulsions (10% Intralipid, containing 50% linoleic acid and 9% alpha-linolenic acid) in addition to an age-appropriate diet. Addi tional supplements were urodeoxycholic acid (UDCA) (15 mg/kg/d) and ta urine (100 mg/kg/d). The percentages of DHA in both plasma and RBC pho spholipids of patients in the jaundiced group were significantly lower than those of normal children. Patients in the jaundice-free group ha d significantly lower levels of DHA and higher levels of linoleic acid in both plasma and RBC phospholipids in comparison to the normal grou p. This study shows that postoperative EBA patient become DHA-deficien t even when supplemented with fat emulsions (largely composed of linol eic acid) that contain DHA's precursor, alpha-linolenic acid. This dem onstrates a deficiency in the long-chain acid desaturase activity of t hese patients. It is recommended that excessive/inoleic acid intake be avoided and that all EBA patients have small amounts of DHA added to their lipid supplementation. Copyright (C) 1994 by W.B. Saunders Compa ny