INTRODUCTION Prader-Willi Syndrome (PWS) is a genetic disorder caused by the lack of expression of certain paternal genes located on chromosome 15q11-q13. This anomaly causes cognitive, neurological and endocrine abnormalities, among which one of the most important is hyperphagia. The aim of this study was to assess the oral health of children with PWA and to establish preventive criteria. RESULTS Thirty patients with PWS (mean age 10.2 years) and 30 age- and gender-matched controls were included in the study. Twenty-six patients with PWS(86.6%) followed dietary treatment prescribed by their endocrinologist. Individuals with PWS had a mean caries index of 53.3% and Decayed Missing Filled teeth (DMFT) index 2.5, and 53.3% had gingivitis, in the control group the respective figures were 43.3%, 0.93, and 60%. Only the DMFT index (p 0.017) presented significant differences. Regarding stimulated salivary secretion, patients with PWS presented a mean of 0.475 ml/min with a pH of 6.15, while controls presented a mean of 0.848 ml/min with a pH of 7.53; the differences between the groups were statistically significant in both cases (p 0.032 and p 0.0001 respectively). The population with PWS presented a higher plaque index (> 2) than their healthy peers, but the differences were not significant. CONCLUSION Pediatric patients with Prader-Willi syndrome have an increased risk of caries and gingivitis. The children with this syndrome have a decreased salivary flow and a more acidic salivary pH. In these patients, dental care is an essential part of their multidisciplinary medical treatment.

中文翻译：

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普瑞德威利综合征儿童的口腔疾病：病例对照研究。

简介 普瑞德威利综合征 (PWS) 是一种遗传性疾病，由位于染色体 15q11-q13 上的某些父系基因缺乏表达引起。这种异常会导致认知、神经和内分泌异常，其中最重要的异常之一是食欲过多。本研究的目的是评估患有 PWA 的儿童的口腔健康并建立预防标准。结果 该研究纳入了 30 名 PWS 患者（平均年龄 10.2 岁）和 30 名年龄和性别匹配的对照者。26 名 PWS 患者（86.6%）遵循内分泌科医生规定的饮食治疗。PWS 患者的平均龋齿指数为 53.3%，龋齿缺失补牙 (DMFT) 指数为 2.5，53.3% 患有牙龈炎，而对照组的数字分别为 43.3%、0.93 和 60%。只有 DMFT 指数 (p < 0.017) 存在显着差异。在刺激唾液分泌方面，PWS 患者的平均唾液分泌量为 0.475 毫升/分钟，pH 为 6.15，而对照组的平均唾液分泌量为 0.848 毫升/分钟，pH 为 7.53；在这两种情况下，组间差异均具有统计学显着性（分别为 p < 0.032 和 p < 0.0001）。PWS 人群的菌斑指数 (> 2) 高于健康人群，但差异并不显着。结论 患有普瑞德威利综合征的儿科患者患龋齿和牙龈炎的风险增加。患有这种综合征的儿童唾液流量减少，唾液 pH 值呈酸性。对于这些患者来说，牙科护理是多学科治疗的重要组成部分。