Abola Iveta, Intlere Nikola Anna, Brinkmane Anda, Laktina Sabine, Zarina Agnese, Vasilevska Lauma, Skadins Ingus, Moisejevs Georgijs, Gailite Linda, Auzenbaha Madara
Department of Conservative Dentistry and Oral Health, Rīga Stradiņš University, LV-1007 Rīga, Latvia.
Scientific Laboratory of Molecular Genetics, Rīga Stradiņš University, LV-1007 Rīga, Latvia.
Mol Genet Metab Rep. 2024 Nov 27;41:101167. doi: 10.1016/j.ymgmr.2024.101167. eCollection 2024 Dec.
Phenylketonuria (PKU) is an autosomal recessive inherited disorder of phenylalanine (Phe) metabolism that results from a deficiency of phenylalanine hydroxylase (PAH). Patients with PKU rely on amino acid mixtures and low-protein diets, which often exhibit an acidic nature and pose various challenges to oral health. The objective of the study was to evaluate oral care habits of PKU patients in Latvia and the impact of the recommendations developed on improving oral care.
In this study, during a one-month interval before and after the implementation of oral hygiene recommendations, questionnaires were distributed to all patients with PKU diagnosed in Latvia, with a response rate of 78 % (79 of 101).
The group older and 18 years of age showed a poorer understanding of oral care even after receiving recommendations, 82 % brushing their teeth twice a day (92 % in the group <18 years of age), continuing 57 % rinsing their mouth after using amino acid formula (75 % in the younger group). Significant improvements were observed only in the respondent group younger than 18 years of age - including increases in toothbrushing twice a day by 25 % ( = 0.001), dental flossing by 23 % ( = 0.001), mouth rinsing after amino acid-based formula by 13 % ( = 0.020).
This study concludes that PKU patients older and 18 years of age have a poor understanding of maintaining oral hygiene and the use of the necessary supplements to improve it. Activities are needed in the future that would regularly remind and motivate PKU patients to take care of their oral health.
苯丙酮尿症（PKU）是一种常染色体隐性遗传的苯丙氨酸（Phe）代谢紊乱疾病，由苯丙氨酸羟化酶（PAH）缺乏引起。PKU患者依赖氨基酸混合物和低蛋白饮食，这些饮食通常呈酸性，给口腔健康带来各种挑战。本研究的目的是评估拉脱维亚PKU患者的口腔护理习惯，以及所制定的建议对改善口腔护理的影响。
在本研究中，在实施口腔卫生建议前后的一个月时间内，向拉脱维亚所有确诊的PKU患者发放问卷，回复率为78%（101人中79人）。
18岁及以上的患者即使在收到建议后对口腔护理的理解仍较差，82%的人每天刷牙两次（18岁以下组为92%），57%的人在使用氨基酸配方后继续漱口（较年轻组为75%）。仅在18岁以下的受访者组中观察到显著改善，包括每天刷牙两次的比例增加25%（P = 0.001），使用牙线的比例增加23%（P = 0.001），在使用氨基酸配方后漱口的比例增加13%（P = 0.020）。
本研究得出结论，18岁及以上的PKU患者对保持口腔卫生以及使用必要补充剂来改善口腔卫生的理解较差。未来需要开展活动，定期提醒和激励PKU患者关注口腔健康。
