Faculty of Medicine, University of Valladolid, Avenida Ramón y Cajal, 7, 47005 Valladolid, Spain.
Castilla y León Cystic Fibrosis Unit, University Clinical Hospital of Valladolid, Avenida Ramón y Cajal, 3, 47005 Valladolid, Spain.
Int J Mol Sci. 2024 Feb 5;25(3):1900. doi: 10.3390/ijms25031900.
Cystic fibrosis (CF) is a monogenic disease with different types of mutations that mainly affect the respiratory-digestive system. Calcium (Ca), phosphorus (P), and vitamin D (Vit-D) are essential nutrients for maintaining adequate growth and development, as well as key components in crucial metabolic pathways. Proper diagnosis, treatment, and response are decisive components of precision medicine. Therefore, we conducted a cross-sectional study to evaluate Ca, P, and Vit-D levels along with health and nutritional indicators, regarding their non-skeletal functions, in a series of CF patients. Anthropometric and clinical evaluation, biochemical analysis, dietary survey, and respiratory and pancreatic status were performed. Even though the results showed that all patients had normal dietary and serum Ca levels, 47% of patients had deficient Vit-D intake, 53% of patients had hypovitaminosis D, 35% had insufficient Vit-D levels, 18% had hypophosphatemia, 76% had elevated alkaline phosphate levels, 29% had hypercalciuria, and 65% had hyperphosphaturia. There were no significant differences between homozygous and compound heterozygous patients. Ca, P, and Vit-D levels were associated with body mass index; body composition; physical activity; diet; growth hormones; and the immune, liver, and kidney systems. We suggest a periodically evaluation of Ca and P losses.
囊性纤维化 (CF) 是一种单基因疾病，有多种类型的突变，主要影响呼吸-消化系统。钙 (Ca)、磷 (P) 和维生素 D (Vit-D) 是维持充分生长和发育所必需的营养物质，也是关键代谢途径的重要组成部分。适当的诊断、治疗和反应是精准医学的决定性组成部分。因此，我们进行了一项横断面研究，以评估一系列 CF 患者的 Ca、P 和 Vit-D 水平以及健康和营养指标，这些指标与非骨骼功能有关。进行了人体测量和临床评估、生化分析、饮食调查以及呼吸和胰腺功能评估。尽管结果表明所有患者的膳食和血清 Ca 水平正常，但 47%的患者 Vit-D 摄入不足，53%的患者存在 Vit-D 缺乏症，35%的患者 Vit-D 水平不足，18%的患者存在低磷血症，76%的患者碱性磷酸酶水平升高，29%的患者出现高钙尿症，65%的患者出现高磷尿症。纯合子和复合杂合子患者之间没有显著差异。Ca、P 和 Vit-D 水平与体重指数、身体成分、身体活动、饮食、生长激素以及免疫、肝脏和肾脏系统有关。我们建议定期评估 Ca 和 P 的流失情况。
