OBJECTIVE: To establish age-related reference values for 7-alpha-hydroxy-4-cholesten-3-one (C4) in a pediatric population and to investigate bile acid malabsorption in children with short bowel syndrome (SBS). STUDY DESIGN: Serum was obtained between 8:00 a.m. and 11:00 a.m. from 100 healthy children (52% males, 9 months to 18 years of age) after 10 hours of fasting. Pediatric patients with SBS served as disease controls (n = 12). Following solid-phase extraction and purification, C4 was determined by high-performance liquid chromatography using a ultraviolet detector at a wavelength of 241 nm. The upper limit of normal for C4 concentrations was defined as the mean plus 2 SD of the log-normal distribution. RESULTS: The mean concentration and SD of C4 in healthy children was 22.8 ± 15.8 ng/mL with no relation to age or sex and an upper limit of normal of 66.5 ng/mL. Normal C4 values were found in 97 of 100 healthy children, and all 12 patients with SBS had C4 concentrations above 100 ng/mL (mean 299.6 ± 167.8 ng/mL; range 105.7-562.1 ng/mL, P < .0001 compared with controls). CONCLUSIONS: The determined upper limit of normal for C4 concentration in healthy children corresponds to previously published levels in healthy adults and is independent of age and sex. The consistently elevated C4 concentrations in our patients with SBS confirm the reliability of this noninvasive, nonisotopic method to assess bile acid malabsorption in children.
OBJECTIVE: To establish age-related reference values for 7-alpha-hydroxy-4-cholesten-3-one (C4) in a pediatric population and to investigate bile acid malabsorption in children with short bowel syndrome (SBS). STUDY DESIGN: Serum was obtained between 8:00 a.m. and 11:00 a.m. from 100 healthy children (52% males, 9 months to 18 years of age) after 10 hours of fasting. Pediatric patients with SBS served as disease controls (n = 12). Following solid-phase extraction and purification, C4 was determined by high-performance liquid chromatography using a ultraviolet detector at a wavelength of 241 nm. The upper limit of normal for C4 concentrations was defined as the mean plus 2 SD of the log-normal distribution. RESULTS: The mean concentration and SD of C4 in healthy children was 22.8 ± 15.8 ng/mL with no relation to age or sex and an upper limit of normal of 66.5 ng/mL. Normal C4 values were found in 97 of 100 healthy children, and all 12 patients with SBS had C4 concentrations above 100 ng/mL (mean 299.6 ± 167.8 ng/mL; range 105.7-562.1 ng/mL, P < .0001 compared with controls). CONCLUSIONS: The determined upper limit of normal for C4 concentration in healthy children corresponds to previously published levels in healthy adults and is independent of age and sex. The consistently elevated C4 concentrations in our patients with SBS confirm the reliability of this noninvasive, nonisotopic method to assess bile acid malabsorption in children.
Authors: Davor Slijepcevic; Reinout L P Roscam Abbing; Takeshi Katafuchi; Antje Blank; Joanne M Donkers; Stéphanie van Hoppe; Dirk R de Waart; Dagmar Tolenaars; Jonathan H M van der Meer; Manon Wildenberg; Ulrich Beuers; Ronald P J Oude Elferink; Alfred H Schinkel; Stan F J van de Graaf Journal: Hepatology Date: 2017-09-29 Impact factor: 17.425
Authors: Natalia Gomez-Ospina; Carol J Potter; Rui Xiao; Kandamurugu Manickam; Mi-Sun Kim; Kang Ho Kim; Benjamin L Shneider; Jennifer L Picarsic; Theodora A Jacobson; Jing Zhang; Weimin He; Pengfei Liu; A S Knisely; Milton J Finegold; Donna M Muzny; Eric Boerwinkle; James R Lupski; Sharon E Plon; Richard A Gibbs; Christine M Eng; Yaping Yang; Gabriel C Washington; Matthew H Porteus; William E Berquist; Neeraja Kambham; Ravinder J Singh; Fan Xia; Gregory M Enns; David D Moore Journal: Nat Commun Date: 2016-02-18 Impact factor: 14.919