Effects of liver transplantation on long-chain polyunsaturated fatty acid status in infants with biliary atresia

Background: The long-chain polyunsaturated fatty acid (LC-PUFA) status of i nfants with untreated biliary atresia (BA) is known to be poor and is corre lated to the severity of the liver disease. Liver transplantation (LT) mark edly increases survival of patients with BA but the extent to which this re verses poor LC-PUFA status is not known. Methods: To explore this question, the erythrocyte (red blood cell. RBC) ph ospholipid content of eight infants with BA who underwent LT was determined 2 months after an initial portoenterostomy, immediately before LT, and 6 a nd 12 months after LT. Before LT, all infants were fed a protein hydrolysat e formula containing medium-chain triglycerides and essential fatty acids. Afterward, they were fed a normal diet for age. The RBC phospholipid conten t at each time point was compared with that of 28 age-matched control infan ts. Results: Just before LT, median RBC phospholipid content of C20:4n-6, C20:5 n-3, and C22:6n-3 was 25%, 48%, and 30% lower, respectively, than that obse rved in age-matched control infants. After LT, the RBC phospholipid content of most fatty acids reached normal values by 6 months. However, that of C2 0:4n-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 remaine d lower than in normal children, but that of C22:6n-3 did not differ. The r atio 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 w ith BA improves after LT but is not entirely reversed within a year after s urgery. They suggest that the abnormal status before LT may be secondary, i n 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.