Aim of the study. To asses intestinal absorptive capacity by various serum markers in pediatric intestinal failure (IF).

 

Methods. After an ethical approval serum cholesterol precursors (cholestenol, lathosterol) and plant sterols (campesterol, stigmasterol, sitosterol), respective indicators of cholesterol synthesis and absoption were measured in 55 IF patients (median age 6.1 years, range 0.1-27) and 85 healthy matched controls. Plasma citrulline, a marker of enterocyte mass, was measured in 41 patients. Mean small bowel length was 47 cm (9-123) in the 35 short bowel patients and 188 cm (50-450) in the 20 dysmotility patients. Sixteen patients remained on PN after 69 (2.5-220) PN months, and 39 had weaned off PN 6.9 years (0.01-27) before after 26 (0.7-260) months on PN.

 

Main Results. Cholesterol precursors cholestenol [68 (11-415) vs 23 (6-58)µg/dL] and lathosterol [306 (48-1028) vs 129 (31-387)], were increased in patients compared to controls (P<0.001), reflecting compensatorily increased synthesis due to malabsorption of cholesterol. After weaning off PN plant sterols campesterol [307 (11-837) vs 448 (15-1168)], stigmasterol [19 (8-48) vs 41 (12-126)] and sitosterol [196 (19-891) vs 255 (15-742)] were decreased compared to controls (P<0.05) consistent with cholesterol malabsorption. Sitosterol (r=0.399), lathosterol (r=-0.435) and cholestenol (r=-0.439) reflected ileum length (P<0.05). Patients without ileum had higher cholestenol (84 vs 45) and lathosterol (344 vs 228) compared to patients with ileal remnant (P<0.05). Duration of PN associated with cholestenol and lathosterol (r=0.506-0.535, P<0.05). Citrulline [24 µmol/L (4-50)] was unrelated to small bowel lenght measured at initial surgery (r=-0.075, P=0.637), but associated with time after weaning off PN and duration of PN (r=0.371-0.412, P<0.05).

 

Conclusions. Increased serum cholesterol precursors and decreased plant sterol levels reflect the extent of (distal) small intestinal resection and cholesterol malabsorption in children with IF. Plasma citrulline and cholesterol precursors were related to duration of PN.