Department of Organization and Management in Health Care, Poznan University of Medical Sciences, Poznan, Poland.
Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznan, Poland.
Adv Med Sci. 2019 Sep;64(2):409-414. doi: 10.1016/j.advms.2019.08.001. Epub 2019 Aug 29.
This cohort study aimed to determine the frequency of overweight and obesity in classical phenylketonuria children and to identify the possible influence of metabolic control on the BMI of the studied patients.
The study group included 63 classical phenylketonuria patients (40 girls and 23 boys; aged 5-16 years). Their z-score BMI, metabolic control, educational level of parents and socioeconomic status were determined.
Twenty children were overweight or obese and only three were underweight. The percentages of overweight and obese children were 31.7% for the whole group, 21.7% (5 out of 23) for boys and 37.5% (15 out of 40) for girls. Overweight and obesity in these phenylketonuria patients was statistically significantly more frequent when compared to national reference studies (p = 0.0031). The five-year index of dietary control and the percentage of spikes exceeding 6 and 12 mg/dl (Spikes 6 and 12) indicated better metabolic control in the case of normal weight children than those who were overweight and obese (p < 0.049, p < 0.041 and p < 0.011, respectively). The odds ratio of being overweight or obese for those having poorer metabolic control (values higher vs lower than mean) was statistically significantly higher than for the remaining patients (for Spikes 12: 6.926 < 95%CI: 2.011-23.854 > ; p < 0.002). These results strongly suggest a link between overweight and diet non-compliance.
Children with classical phenylketonuria presented higher odds of being overweight or obese as compared with reference national studies, with girls only having a higher frequency of overweight.
本队列研究旨在确定经典苯丙酮尿症患儿超重和肥胖的频率，并确定代谢控制对研究患者 BMI 的可能影响。
研究组包括 63 例经典苯丙酮尿症患儿（40 名女孩和 23 名男孩；年龄 5-16 岁）。确定了他们的 z 评分 BMI、代谢控制、父母的教育水平和社会经济地位。
20 名儿童超重或肥胖，只有 3 名儿童体重不足。超重和肥胖儿童的百分比为：全组 31.7%（23 名男孩中有 5 名，40 名女孩中有 15 名）。与全国参考研究相比，这些苯丙酮尿症患儿超重和肥胖的比例具有统计学意义（p=0.0031）。五年饮食控制指数和超过 6 和 12mg/dl 的尖峰百分比（尖峰 6 和 12）表明，正常体重儿童的代谢控制优于超重和肥胖儿童（p<0.049、p<0.041 和 p<0.011）。代谢控制较差（高于或低于平均值）的儿童超重或肥胖的优势比与其余患者相比具有统计学意义更高（尖峰 12：6.926<95%CI：2.011-23.854>；p<0.002）。这些结果强烈表明超重与饮食不依从之间存在关联。
与全国参考研究相比，经典苯丙酮尿症患儿超重或肥胖的可能性更高，只有女孩超重的频率更高。
