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1型神经纤维瘤病患儿的25-羟维生素D浓度

Pediatric 25-hydroxyvitamin D concentrations in neurofibromatosis type 1.

作者信息

Stevenson David A, Viskochil David H, Carey John C, Sheng Xiaoming, Murray Mary, Moyer-Mileur Laurie, Shelton Judd, Roberts William L, Bunker Ashley M, Hanson Heather, Bauer Stephanie, D'Astous Jacques L

机构信息

Department of Pediatrics, University of Utah, Salt Lake City, UT 84132, USA.

出版信息

J Pediatr Endocrinol Metab. 2011;24(3-4):169-74. doi: 10.1515/jpem.2011.092.

Abstract

OBJECTIVE

Low 25-hydroxyvitamin D (25OHD) concentrations have been associated with tumors and osteopenia or fractures in adults with neurofibromatosis type 1 (NF1). We report 25OHD concentrations in 109 children with NF1 and 218 controls matched for age, sex, geographic location, and time of year.

METHODS

Children with NF1 were recruited (n=109; 2-17 years), and clinical data and dual-energy X-ray absorptiometry measurements were obtained. 25OHD concentrations were measured in subjects and controls.

RESULTS

More NF1 individuals (50%) were in the 25OHD insufficient or deficient range (<30 ng/mL) (1 ng/mL = 2.496 nmol/L) compared to controls (36%) (p = 0.0129). 25OHD concentrations were higher in individuals with neurofibromas after controlling for age (p = 0.0393), and were negatively associated with whole-body subtotal bone mineral density (BMD) z-scores (p = 0.0385).

CONCLUSIONS

More children with NF1 had 25OHD concentrations <30 ng/mL, potentially because of increased pigmentation and/or decreased sunlight exposure. In contrast to adults, decreased 25OHD concentrations were not associated with neurofibromas, and there was no positive association between 25OHD and BMD.

摘要

目的

低25-羟基维生素D(25OHD)浓度与1型神经纤维瘤病(NF1)成人患者的肿瘤以及骨质减少或骨折有关。我们报告了109例NF1儿童和218例年龄、性别、地理位置及季节相匹配的对照儿童的25OHD浓度。

方法

招募了NF1儿童(n = 109;2 - 17岁),获取了临床数据和双能X线吸收测定结果。对受试者和对照者测量了25OHD浓度。

结果

与对照组(36%)相比,更多的NF1个体(50%)处于25OHD不足或缺乏范围(<30 ng/mL)(1 ng/mL = 2.496 nmol/L)(p = 0.0129)。在控制年龄后,患有神经纤维瘤的个体25OHD浓度更高(p = 0.0393),且与全身骨密度(BMD)z评分呈负相关(p = 0.0385)。

结论

更多NF1儿童的25OHD浓度<30 ng/mL,可能是由于色素沉着增加和/或阳光照射减少。与成人不同,25OHD浓度降低与神经纤维瘤无关,且25OHD与BMD之间无正相关。

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