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Docosahexaenoic acid (DHA) is believed to be an important long-chain polyunsaturated fatty acid (LCPUFA), which may be essential for neurofunction in infants. Patients with extrahepatic biliary atresia (EBA) may have DHA deficiency secondary to fat malabsorption. The authors investigated 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 jaundiced 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. Additional supplements were ursodeoxycholic acid (UDCA) (15 mg/kg/d) and taurine (100 mg/kg/d). The percentages of DHA in both plasma and RBC phospholipids of patients in the jaundiced group were significantly lower than those of normal children. Patients in the jaundice-free group had significantly lower levels of DHA and higher levels of linoleic acid in both plasma and RBC phospholipids in comparison to the normal group. This study shows that postoperative EBA patient become DHA-deficient even when supplemented with fat emulsions (largely composed of linoleic acid) that contain DHA's precursor, alpha-linolenic acid. This demonstrates a deficiency in the long-chain acid desaturase activity of these patients. It is recommended that excessive/linoleic acid intake be avoided and that all EBA patients have small amounts of DHA added to their lipid supplementation.
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