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Docosahexaenoic acid status of patients with extrahepatic biliary atresia
Y Yamashiro1, T Shimizu, Y Ohtsuka
1Department of Pediatrics, Juntendo University School of Medicine, Tokyo, Japan.
Insights
Infants with extrahepatic biliary atresia (EBA) may develop docosahexaenoic acid (DHA) deficiency due to fat malabsorption. Supplementation with essential fatty acids alone is insufficient, indicating a need for direct DHA addition in EBA patients.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Nutritional Science
Background:
- Docosahexaenoic acid (DHA), a crucial long-chain polyunsaturated fatty acid (LCPUFA), is vital for infant neurodevelopment.
- Extrahepatic biliary atresia (EBA) can lead to fat malabsorption, potentially causing DHA deficiency.
- Postoperative management of EBA patients often involves lipid supplementation, but its efficacy in preventing DHA deficiency is unclear.
Purpose of the Study:
- To investigate docosahexaenoic acid (DHA) and other long-chain polyunsaturated fatty acid (LCPUFA) levels in children with extrahepatic biliary atresia (EBA) after Kasai portoenterostomy and essential fatty acid supplementation.
- To assess the impact of jaundice and lipid supplementation on DHA status in EBA patients.
- To determine the efficacy of alpha-linolenic acid-rich fat emulsions in preventing DHA deficiency in EBA.
Main Methods:
- Plasma and red blood cell (RBC) phospholipid fatty acid profiles were analyzed in ten children with EBA (aged 8-17 months).
- Patients were categorized into jaundiced (n=5) and jaundice-free (n=5) groups.
- All patients received fat emulsions (containing linoleic and alpha-linolenic acid), ursodeoxycholic acid (UDCA), and taurine supplementation.
Main Results:
- Patients in the jaundiced group showed significantly lower DHA percentages in plasma and RBC phospholipids compared to normal children.
- Jaundice-free EBA patients exhibited significantly lower DHA and higher linoleic acid levels than controls.
- These findings suggest impaired long-chain fatty acid desaturase activity in EBA patients, leading to DHA deficiency despite alpha-linolenic acid supplementation.
Conclusions:
- Postoperative EBA patients are prone to DHA deficiency, even with standard essential fatty acid supplementation.
- The study highlights a potential defect in the conversion of alpha-linolenic acid to DHA in EBA patients.
- It is recommended to limit excessive linoleic acid intake and include direct DHA supplementation for all EBA patients.
Abstract:
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.