Department of Nutritional Sciences, University of Wisconsin-Madison, Madison, Wisconsin, USA.
Department of Pediatrics, University of Wisconsin-Madison, Madison, Wisconsin, USA.
Pediatr Pulmonol. 2022 Apr;57(4):935-944. doi: 10.1002/ppul.25825. Epub 2022 Jan 20.
The variable response to fat-soluble vitamin supplementation in young children with cystic fibrosis (CF), and factors contributing to this variability, remain under-investigated.
To determine if recommended supplement doses normalize serum vitamins A (retinol), D (25-hydroxy-vitamin D, 25OHD), and E (α-tocopherol), and identify factors predictive of achieving sufficiency, in children with CF in the first 3 years of life.
We studied 144 infants born during 2012-2017 and diagnosed with CF through newborn screening. Serum retinol, 25OHD, α-tocopherol and plasma cytokines interleukin (IL)-6, IL-8, IL-10, and tumor necrosis factor (TNF)-α were measured in early infancy and yearly thereafter. Vitamin supplement intakes and respiratory microbiology were assessed every 1-2 months in infancy and quarterly thereafter.
The prevalence of vitamin D insufficiency (<30 ng/ml) at all ages combined was significantly higher (22%) compared to vitamin A (<200 ng/ml, 3%) and vitamin E (<5 µg/ml, 5%). All children were vitamin A sufficient by age 2 years. Vitamin E insufficiency was rare. Only 42% were early responders of vitamin D and 17% remain insufficient despite high supplement intakes. IL-6 was positively correlated, while IL-8, IL-10, and TNF-α were negatively correlated, with retinol and 25OHD. Multiple regression analysis revealed that supplement dose, season, α-tocopherol, pancreatic insufficiency, respiratory infections and IL-10 were significant predictors of 25OHD.
Diagnosis through newborn screening coupled with supplementation normalized serum retinol and α-tocopherol in almost all infants with CF by age 3 years. However, response to vitamin D supplements in young children with CF occurred later and variably despite early and sustained supplementation.
囊性纤维化（CF）患儿对脂溶性维生素补充的反应存在差异，而导致这种差异的因素仍未得到充分研究。
确定在 CF 患儿生命的头 3 年内，推荐的补充剂量是否能使血清维生素 A（视黄醇）、D（25-羟维生素 D，25OHD）和 E（α-生育酚）正常化，并确定达到充足水平的预测因素。
我们研究了 2012-2017 年间出生并通过新生儿筛查诊断为 CF 的 144 名婴儿。在婴儿早期和此后每年测量血清视黄醇、25OHD、α-生育酚和血浆细胞因子白细胞介素（IL）-6、IL-8、IL-10 和肿瘤坏死因子（TNF）-α。在婴儿期每 1-2 个月评估一次维生素补充剂摄入量和呼吸微生物学，此后每季度评估一次。
所有年龄段维生素 D 不足（<30ng/ml）的患病率均显著高于维生素 A（<200ng/ml，3%）和维生素 E（<5μg/ml，5%）。所有儿童在 2 岁时均达到维生素 A 充足。维生素 E 不足的情况很少见。只有 42%的儿童对维生素 D 有早期反应，尽管补充剂摄入量高，但仍有 17%的儿童缺乏维生素 D。IL-6 与视黄醇和 25OHD 呈正相关，而 IL-8、IL-10 和 TNF-α则呈负相关。多元回归分析显示，补充剂量、季节、α-生育酚、胰腺功能不全、呼吸道感染和 IL-10 是 25OHD 的显著预测因素。
通过新生儿筛查诊断并辅以补充剂治疗，使几乎所有 CF 患儿在 3 岁时血清视黄醇和α-生育酚恢复正常。然而，尽管早期和持续补充，CF 幼儿对维生素 D 补充剂的反应较晚且各不相同。
