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囊性纤维化跨膜电导调节因子基因突变患儿的维生素状况。

Vitamin Status in Children with Cystic Fibrosis Transmembrane Conductance Regulator Gene Mutation.

机构信息

Department of Pediatrics, Faculty of Medical Sciences, Medical University of Silesia, 40-752 Katowice, Poland.

Faculty of Health Sciences, University of Bielsko-Biala, 43-309 Bielsko-Biała, Poland.

出版信息

Nutrients. 2022 Nov 4;14(21):4661. doi: 10.3390/nu14214661.

Abstract

BACKGROUND

The issue of vitamin metabolism in children with cystic fibrosis screen positive, inconclusive diagnosis (CFSPID) is not well known. The aim of this study was to determine the status of vitamins A, D, E, and C in the blood of a group of children with CFSPID.

MATERIAL AND METHODS

A total of 89 children were enrolled in the study (Me: 3.6 years, 52.8% boys), as follows: 28 with CFSPID, 31 with CF (cystic fibrosis), and 30 HC (healthy children). Their blood concentrations of vitamins A, D, E, and C, and their dietary intake of these vitamins were analysed in the study groups on the basis of a three-day food diary.

RESULTS

The patients with CFSPID had significantly higher serum vitamin D ( = 0.01) and E ( = 0.04) concentrations, compared to the children with CF. None of the children with CFSPID revealed vitamin A or E deficiencies. Patients with CF had been consuming significantly higher vitamin D and E amounts ( = 0.01). The vitamin concentrations did not depend either on the pancreatic/liver function or on anthropometric parameters. In total, 32.14% of patients with CF did not cover the baseline recommended calorie intake, and 53.6% and 36% did not take the recommended vitamin E and vitamin A intake, respectively.

CONCLUSION

Children with CF and CFSPID did not fully cover the dietary recommendations for vitamin supply, but vitamin deficiency was found only in CF.

摘要

背景

囊性纤维化(CF)疑似阳性、不确定诊断(CFSPID)患儿的维生素代谢问题尚未得到充分认识。本研究旨在确定一组 CFSPID 患儿的血液中维生素 A、D、E 和 C 的状况。

材料与方法

共纳入 89 名儿童(中位年龄 3.6 岁,52.8%为男性)参与本研究,分为 CFSPID 组(n=28)、CF 组(n=31)和 HC 组(n=30)。根据三天食物日记,分析各组患儿的血液维生素 A、D、E 和 C 浓度及其维生素摄入量。

结果

与 CF 患儿相比,CFSPID 患儿血清维生素 D(P=0.01)和 E(P=0.04)浓度显著更高。无 CFSPID 患儿存在维生素 A 或 E 缺乏。CF 患儿摄入的维生素 D 和 E 明显更多(P=0.01)。维生素浓度既不受胰腺/肝功能也不受人体测量参数的影响。总体而言,32.14%的 CF 患儿未达到推荐的基础热量摄入,53.6%和 36%的患儿分别未达到推荐的维生素 E 和维生素 A 摄入量。

结论

CF 和 CFSPID 患儿未完全满足维生素供应的膳食推荐,但仅在 CF 患儿中发现了维生素缺乏。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/84c3/9654413/51efeb86445a/nutrients-14-04661-g001.jpg

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