Increased whole beody hydroxyproline production as assessed by a new stable isotope technique is associated with hip and spine bone mineral loss in cystic fibrosis
Increased whole beody hydroxyproline production as assessed by a new stable isotope technique is associated with hip and spine bone mineral loss in cystic fibrosis
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DOI：
10.1016/j.clnu.2013.12.008
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发表时间：
2014-12-01
影响因子：
6.3
通讯作者：
Deutz, N. E. P.
中科院分区：
文献类型：
--
作者：
Engelen, M. P. K. J.;Com, G.;Deutz, N. E. P.
背景与目的：囊性纤维化（CF）患儿常出现骨矿物质丢失、肺功能降低和营养状况受损。血药浓度和尿羟脯氨酸（OH-PRO）排泄量已被用作CF患者骨矿物质状态和肺功能的标志物。目的：用一种新的稳定同位素方法评估CF儿童患者全身羟脯氨酸生成是否增加，并与骨矿物质丢失、肺功能下降和营养状况受损有关。设计：在15名CF儿童患者和17名健康年轻对照者的横断研究中，通过启动常数和连续输注稳定同位素2-D-OH-PRO 3小时，评估吸收后状态下全身羟脯氨酸生成（Wb OH-PRO）。通过双能x线吸收仪（DXA）测定全身、髋部和旋转的骨矿物质密度（BMD）和身体组成（脂肪量和无脂肪量）。采用LC/MS/MS法测定血浆同位素富集度和OH-PRO浓度。结果：儿童CF患者的WbOH-PRO生成和血浆OH-PRO浓度高于健康青年受试者（p < 0.001）。在CF中，WbOH-PRO的产生与血浆OH-PRO浓度显著相关（r: 0.70, p = 0.007），但在健康组中无显著相关性。CF中WbOH-PRO的产生与髋部（r = -0.61, p = 0.02）和脊柱（r = -0.59, p = 0.02）的低骨密度值相关，但与全身骨密度、肺功能或身体组成无关。结论：一种新的稳定同位素方法显示CF患儿全身羟脯氨酸生成率水平增强，表明全身胶原分解增强。CF患者全身羟脯氨酸生成水平升高与髋部和脊柱严重骨矿物质流失有关，但与肺功能下降或营养状况受损无关。(C) 2013爱思唯尔有限公司和欧洲临床营养与代谢学会。版权所有。
Background & aims: Bone mineral loss, reduced lung function and impaired nutritional status are frequently present in children with Cystic Fibrosis (CF). Blood concentrations and urinary excretion of hydroxyproline (OH-PRO) have been used as markers of bone mineral status and lung function in CF.Objective: To examine whether whole body hydroxyproline production, as assessed by a new stable isotope methodology, is increased in pediatric patients with CF and associated with bone mineral loss, lung function decline and impaired nutritional status.Design: In a cross-sectional study in 15 pediatric patients with CF and 17 healthy young control subjects, whole body hydroxyproline production (Wb OH-PRO) was assessed in the postabsorptive state by primed-constant and continuous infusion of the stable isotope 2-D-OH-PRO for 3 h. Bone mineral density (BMD) of whole body, hip and spin, and body composition (fat mass and fat-free mass) were determined by dual-energy X-ray Absorptiometry (DXA). Plasma isotopic enrichments and OH-PRO concentrations were measured by LC/MS/MS.Results: Higher values for WbOH-PRO production and plasma OH-PRO concentrations were found in pediatric CF patients than in the healthy young subjects (p < 0.001). WbOH-PRO production was significantly correlated with plasma OH-PRO concentrations in the CF (r: 0.70, p = 0.007) but not in the healthy group. WbOH-PRO production in CF was associated with low BMD values in hip (r = -0.61, p = 0.02) and spine (r = -0.59, p = 0.02) but not with whole body BMD, lung function or body composition.Conclusion: A new stable isotope approach revealed enhanced levels of whole body hydroxyproline production rate in pediatric patients with CF, indicative of enhanced whole body collagen breakdown. Increased levels for whole body hydroxyproline production in CF were associated with severe bone mineral loss in hip and spine but not with lung function decline or impaired nutritional status. (C) 2013 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.