PURPOSE: 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. SUBJECTS AND 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). CONCLUSION: 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. Copyright 2010 Elsevier Inc. All rights reserved.
PURPOSE: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. SUBJECTS AND 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). CONCLUSION: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. Copyright 2010 Elsevier Inc. All rights reserved.