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苯丙酮尿症患者及其同龄人肥胖的生长参数和患病率:这样比较合适吗?

Growth Parameters and Prevalence of Obesity in PKU Patients and Peers: Is This the Right Comparison?

作者信息

Paterno Giulia, Di Tullio Vito, Carella Rosa, De Ruvo Giada, Furioso Fabrizio, Skublewska-D'Elia Aleksandra, De Giovanni Donatella, Tummolo Albina

机构信息

Department of Metabolic Diseases, Clinical Genetics and Diabetology, Giovanni XXIII Children Hospital, Azienda Ospedaliero-Universitaria Consorziale, 70126 Bari, Italy.

Vivenda SPA, Strada Torre Tresca, 18, 70124 Bari, Italy.

出版信息

Pediatr Rep. 2024 Oct 16;16(4):892-901. doi: 10.3390/pediatric16040076.

Abstract

BACKGROUND

One of the main objectives of Phenylketonuria (PKU) management is represented by optimising the growth trend under restricted protein diet regimen. The data on long-term growth in PKU children are limited and mostly based on earlier studies.

METHODS

The data for this twelve-year longitudinal study were collected from 34 PKU children and 37 healthy peers, whose auxological parameters were taken at 7 time-points over the follow-up. The weight-for-length ratio (WLR) z-score and body mass index (BMI) z-score were considered according to age. Prevalence of overweight/obesity was evaluated at last assessment.

RESULTS

The median BMI z-score of PKU children was normal and not statistically different from that of controls on all the seven time-point assessments. Their distributions tended to be wider than those of peers, with the upper limit exceeding the normal range since 12 months old, with a peak specifically at 3 years of age. In controls, there was a tendency to approach the BMI z-score values of overweight in later childhood. The prevalence of overweight was comparable (29% vs. 25%, : 0.78) between the two groups at last assessment, and obese subjects (3/37) were only detected in the control group.

CONCLUSIONS

In this study, we report data from a long-term follow-up on growth, highlighting that the median BMI z-score of PKU children was normal and not statistically different from that of controls. Also, the prevalence of obesity at 12 years of age was overlapping. However, the high prevalence of overweight children in the general population may explain the lack of difference and does not reassure about patients' nutritional risk.

摘要

背景

苯丙酮尿症(PKU)管理的主要目标之一是在限制蛋白质饮食方案下优化生长趋势。PKU儿童长期生长的数据有限,且大多基于早期研究。

方法

这项为期12年的纵向研究数据收集自34名PKU儿童和37名健康同龄人,在随访期间的7个时间点测量了他们的体格学参数。根据年龄计算身长体重比(WLR)z评分和体重指数(BMI)z评分。在最后一次评估时评估超重/肥胖的患病率。

结果

在所有7个时间点的评估中,PKU儿童的BMI z评分中位数正常,与对照组无统计学差异。他们的分布往往比同龄人更宽,自12个月大起上限就超过正常范围,在3岁时达到峰值。在对照组中,儿童后期BMI z评分值有接近超重的趋势。在最后一次评估时,两组超重患病率相当(29%对25%,P = 0.78),肥胖受试者(3/37)仅在对照组中被检测到。

结论

在本研究中,我们报告了一项关于生长的长期随访数据,强调PKU儿童的BMI z评分中位数正常,与对照组无统计学差异。此外,12岁时肥胖患病率重叠。然而,普通人群中超重儿童的高患病率可能解释了这种差异不明显的情况,并且不能消除对患者营养风险的担忧。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d42/11503312/7b270ec5ea18/pediatrrep-16-00076-g001.jpg

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