法国成年人的骨矿物质密度在早期治疗的基尿症
Elisa Dybal1, François Maillot2, François Feillet3
1Fédération d'Endocrinologie, Maladies Métaboliques, Diabète, et Nutrition, Hôpital Louis Pradel, Hospices Civils de Lyon, 69677 Bron Cedex, France.
Molecular genetics and metabolism
|February 7, 2025
概括
基尿症 (PKU) 患者在终身低氨饮食可能面临低骨矿物质密度 (BMD). 这项研究发现,长期坚持饮食,特别是在年轻人中,与 BMD 降低有关,突出显示了潜在的长期健康问题.
科学领域:
- 代谢障碍 代谢障碍 代谢障碍
- 营养科学 营养科学
- 骨健康 骨健康 骨健康
背景情况:
- 基尿症 (PKU) 管理需要终身低氨饮食,限制天然蛋白质,依靠专门的医疗食品和氨基酸补充剂.
- 虽然对神经心理结果有效,但这种限制性饮食对PKU成年人骨健康的长期影响仍然不太清楚.
研究的目的:
- 为了确定患有PKU的成年人骨矿物质密度 (BMD) 低的患病率.
- 在这个人群中确定与低BMD相关的风险因素.
主要方法:
- 分析了法国ECOPHEN队列,包括171名成年PKU患者 (67.3%为女性,中位数年龄为27岁).
- 骨矿物质密度 (BMD) 通过在股骨和脊椎部位的Z分数进行评估. 研究了包括BMI,饮食坚持和蛋白质摄入量在内的危险因素.
主要成果:
- 11.4%的成年PKU患者表现出低BMD (Z分数≤-2).
- 低的BMD与较低的BMI有关,有史以来从未停止饮食,以及较高的总蛋白质摄入量,特别是来自氨基酸补充剂.
- 年轻的患者 (1990年以后出生) 始终保持饮食,患有低BMD的风险增加了4.7倍.
结论:
- 成年PKU患者的一个子集经历了低BMD.
- 长期坚持低天然蛋白质饮食,特别是在年轻人中,是PKU患者骨矿物质密度降低的重要危险因素.
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