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Metabolic handling of 13C labelled tripalmitin inhealthy controls and patients with cystic fibrosis

机译:代谢处理13C标记的Tripalmitin健康对照和囊性纤维化患者

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摘要

AIM—To examine the gastrointestinal handling and metabolic disposal of emulsified [1-13C]palmitic acid esterified into a triglyceride in nine healthy children and seven patients with cystic fibrosis on enzyme replacement treatment.
METHODS—After an overnight fast, each child was given 10 mg/kg body weight
[1,1,1-13C]tripalmitin with a standardised test meal of low natural 13C abundance. The total enrichment of 13C was measured using isotope ratio mass spectrometry in stool collected for a period of up to five days and in breath samples collected over a 24 hour period.
RESULTS—The mean proportion of administered 13C label excreted in stool was 6% (range, 1-12.7%) in healthy children and 24.6% (range, 0-64%) in patients with cystic fibrosis. Healthy children excreted 31.3% of the administered label on their breath (range, 14.2-42.9%). Correcting the excretion of administered 13C label on the breath for differences in digestion and absorption in patients with cystic fibrosis increased the difference between individuals from 0-31.3% of administered dose (mean, 17.9%) to 0-49.1% of absorbeddose (mean, 23.2%) and was poorly related to the amount of13C label in stool.
CONCLUSION—Measurementsof breath 13CO2 do not consistently reflect thegastrointestinal handling of emulsified 13C labelledtripalmitin because of differences in digestion and absorption incystic fibrosis. Further studies need to examine whether "breathtests" alone can predict with confidence the gastrointestinal handling of other 13C labelled triglycerides and fatty acids.

机译:目的:探讨9名健康儿童和7名囊性纤维化患者经酶替代治疗后乳化的[1- 13 C]棕榈酸酯化为甘油三酸酯的胃肠道处理和代谢处理。
方法-禁食过夜后,给每个孩子10 mg / kg体重
[1,1,1- 13 C]曲帕米汀和低天然的标准测试餐13 C丰度。使用同位素比质谱法测量了长达五天的粪便和经过24小时的呼吸样本中的 13 C总富集。
结果—平均值健康儿童的粪便中排泄的 13 C标签比例为6%(范围为1-12.7%),囊性纤维化患者的比例为24.6%(范围为0-64%)。健康的孩子在呼吸时排泄了所服用标签的31.3%(范围14.2-42.9%)。纠正囊性纤维化患者消化中吸收的 13 C标记在呼吸中的排泄,将个体之间的差异从给药剂量的0-31.3%(平均17.9%)增加到0吸收量的-49.1%剂量(平均为23.2%),并且与粪便中的 13 C标签。
结论—测量呼吸 13 CO2不能始终如一地反映出乳化的 13 C标记的胃肠道处理Tripalmitin是因为在消化和吸收方面存在差异囊性纤维化。进一步的研究需要检查“呼吸检测”可以单独预测其他 13 C标记的甘油三酸酯和脂肪酸的胃肠道处理。

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