Document Detail

Effects of liver transplantation on long-chain polyunsaturated fatty acid status in infants with biliary atresia.
MedLine Citation:
PMID:  10817283     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The long-chain polyunsaturated fatty acid (LC-PUFA) status of infants with untreated biliary atresia (BA) is known to be poor and is correlated to the severity of the liver disease. Liver transplantation (LT) markedly increases survival of patients with BA but the extent to which this reverses poor LC-PUFA status is not known. METHODS: To explore this question, the erythrocyte (red blood cell, RBC) phospholipid content of eight infants with BA who underwent LT was determined 2 months after an initial portoenterostomy, immediately before LT, and 6 and 12 months after LT. Before LT, all infants were fed a protein hydrolysate formula containing medium-chain triglycerides and essential fatty acids. Afterward, they were fed a normal diet for age. The RBC phospholipid content at each time point was compared with that of 28 age-matched control infants. RESULTS: Just before LT, median RBC phospholipid content of C20:4n-6, C20:5n-3, and C22:6n-3 was 25%, 48%, and 30% lower, respectively, than that observed in age-matched control infants. After LT, the RBC phospholipid content of most fatty acids reached normal values by 6 months. However, that of C20:4n-6 and C22:6n-3 contents remained 5% and 15% lower, respectively, than in normal control infants. Twelve months after LT, C20:4n-6 content remained lower than in normal children, but that of C22:6n-3 did not differ. The ratio of C20:3n-6/C20:4n-6, a reflection of delta-5 desaturase activity, was abnormal compared with normal children before LT (0.17 vs. 0.10, P < 0.009) but normalized by 6 months after LT (0.11 vs. 0.10, not significant). CONCLUSIONS: These data show that the abnormal LC-PUFA status of children with BA improves after LT but is not entirely reversed within a year after surgery. They suggest that the abnormal status before LT may be secondary, in part, to low delta-5 desaturase activity. The extent to which a different pre- and/or post-LT diet can prevent PUFA deficiency and/or hasten recovery of PUFA status remains to be determined.
A Lapillonne; C Hakme; V Mamoux; M Chambon; V Fournier; V Chirouze; A Lachaux
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of pediatric gastroenterology and nutrition     Volume:  30     ISSN:  0277-2116     ISO Abbreviation:  J. Pediatr. Gastroenterol. Nutr.     Publication Date:  2000 May 
Date Detail:
Created Date:  2000-07-31     Completed Date:  2000-07-31     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8211545     Medline TA:  J Pediatr Gastroenterol Nutr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  528-32     Citation Subset:  IM    
Department of Neonatology, and Human's Nutrition Research Centre, Lyon, France.
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MeSH Terms
Biliary Atresia / blood,  surgery*
Erythrocytes / chemistry
Fatty Acid Desaturases / metabolism
Fatty Acids, Unsaturated / blood*
Linoleic Acid / blood
Liver Transplantation*
Longitudinal Studies
Phospholipids / blood
Prospective Studies
alpha-Linolenic Acid / blood
Reg. No./Substance:
0/Fatty Acids, Unsaturated; 0/Phospholipids; 2197-37-7/Linoleic Acid; 463-40-1/alpha-Linolenic Acid; EC 1.14.19.-/Fatty Acid Desaturases; EC 1.14.99.-/delta-5 fatty acid desaturase

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