Surrogate markers of cholesterol metabolism in children with native liver after successful portoenterostomy for biliary atresia.
OBJECTIVE
Cholestasis gradually ensues after portoenterostomy for biliary atresia (BA) and may deteriorate liver function. Cholesterol metabolism and its relationships with serum markers of liver function were evaluated in children living with native liver after successful portoenterostomy for BA.


METHODS
Serum lipids, noncholesterol sterol ratios to cholesterol, that is, surrogate markers of cholesterol metabolism, and liver function were cross-sectionally studied in 17 consecutive children after successful (postoperative bilirubin <20 micromol/L) portoenterostomy for BA with native liver and a mean age of 5.2 years. The results were compared with healthy age-matched controls.


RESULTS
Mean serum total and low-density lipoprotein cholesterol and campesterol ratio were 18%, 43%, and 26% less than those of controls, respectively (P < .01 for all). Despite low serum cholesterol and campesterol (marker of cholesterol absorption) levels, serum lathosterol (marker of cholesterol synthesis) was decreased by 34% (P < .0001) from control levels and reflected serum prealbumin (r = 0.666) and cholestanol (r = -0.515, P < .05 for both). Cholestanol, twice higher than those of controls (P < .0001), reflected abnormally high serum alkaline phosphatase, glutamyl transferase, and bile acids (r = 0.558-0.711, P < .05). Serum campesterol was inversely related with lathosterol (r = -0.238, P < .05) in controls, but not in patients (r = -0.039).


CONCLUSIONS
Children living with native liver after portoenterostomy for BA are inclined to low serum concentration and absorption of cholesterol. Cholesterol homeostasis was disturbed so that low cholesterol absorption was not associated with compensatory increase in cholesterol synthesis that decreased together with worsening of cholestasis.
Pakarinen
Mikko P
MP
Section of Pediatric Surgery, Hospital for Children and Adolescents, University of Helsinki, Helsinki 00029-HUS, Finland. mikko.pakarinen@hus.fi
Lampela
Hanna
H
Gylling
Helena
H
Miettinen
Tatu A
TA
Koivusalo
Antti I
AI
Nissinen
Markku J
MJ
D000469:Alkaline Phosphatase / Q000097:blood / Q000378:metabolism; D001656:Biliary Atresia / Q000378:metabolism / Q000601:surgery*; D001663:Bilirubin / Q000097:blood / Q000378:metabolism; D015415:Biomarkers / Q000097:blood / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D004083:Cholestanol / Q000097:blood / Q000378:metabolism; D002784:Cholesterol / Q000097:blood / Q000378:metabolism*; D005260:Female; D006706:Homeostasis; D006801:Humans; D007408:Intestinal Absorption; D007536:Isomerism; D050356:Lipid Metabolism / Q000502:physiology; D008055:Lipids / Q000097:blood; D008099:Liver / Q000378:metabolism; D008105:Liver Cirrhosis, Biliary / Q000601:surgery; D008111:Liver Function Tests; D008297:Male; D011171:Portoenterostomy, Hepatic / Q000379:methods*; D011228:Prealbumin / Q000032:analysis / Q000378:metabolism
D015415:Biomarkers; D008055:Lipids; D011228:Prealbumin; C001521:lathosterol; D004083:Cholestanol; D002784:Cholesterol; D000469:Alkaline Phosphatase; D001663:Bilirubin