Background: Diet plays an integral role in the maintenance of oral health, but dietary modifications due to medical problems such as phenylketonuria (PKU) can have adverse effects on oral health. This descriptive study was performed to evaluate the oral health status of children with PKU. Methods: One hundred and ninety-seven patients with PKU aged between 1 and 22 years were evaluated. Clinical evaluations were performed by one experienced dentist regarding dental caries, gingival health and dental erosion. Categorical variables were assessed with descriptive statistics. Differences in feeding frequencies and sociodemographic characteristics were compared regarding dental caries using chi-square (χ²) tests. Results: One hundred and thirty-two patients (67%) had dental caries. The mean plaque index (PI) and gingival index (GI) values were 1.37 ± 0.58 and 1.40 ± 0.64, respectively, which shows moderate plaque accumulation and moderate gingival inflammation. Of the patients, 85.3% did not brush their teeth regularly and 90.4% had never visited a dentist before. No statistically significant differences were found in dental caries according to feeding frequencies (p = 0.448). Conclusions: Despite the high prevalence of caries in patients with PKU, most had never seen a dentist. Physicians must encourage patients with PKU and their parents to have regular dental visits to maintain an optimal general and oral health.

Objective: To evaluate the oral health status and treatment needs of children with congenital and acquired heart disease. Methods: This descriptive study included 301 children aged 5–14 from June 2022 to June 2023. Heart conditions were classified by congenital/acquired status and severity. The children’s sociodemographic characteristics, medical and dental history, tooth brushing habits, and non-nutritional habits (bruxism, nail-biting, thumb-sucking, etc.) were evaluated. Oral health assessments including caries, oral hygiene, enamel defects, and dental treatment needs-related indices were recorded. Results: The mean age was 8.95 ± 2.91 years, and 271 (90%) of the children had congenital heart disease. The children with moderate and severe heart disease had significantly higher decayed/missing/filled surfaces (dmfs) (p = 0.038) and pulp exposure (p = 0.015) compared to the children with mild heart disease. According to the International Caries Detection and Assessment System II (ICDAS II) index, which included initial caries lesions, there were no caries-free children and 75.7% had extensive caries. The mean plaque index and gingival index were found to be 1.18 ± 0.38 and 0.69 ± 0.53, respectively. Enamel defects were observed in 15.9%. The Treatment Needs Index (TNI) was 85.8% for the primary teeth and 88.9% for the permanent teeth. The Care Index (CI) was 12.4% for the primary teeth and 10.8% for the permanent teeth. Conclusions: Children with congenital and acquired heart disease exhibit a high prevalence of untreated dental caries, gingivitis, and plaque accumulation, with a high need for dental treatments. Dentists should prioritize addressing these issues to prevent the risk of infective endocarditis (IE) and improve oral health outcomes in this population.

Background: Maple syrup urine disease (MSUD) is an inherited disorder clinically characterized by ketoacidosis, seizures, coma, psychomotor delay, and intellectual disability. The treatment requires a life-long protein-restricted diet, rich in carbohydrates and fats, supplemented with a medical amino acid formula. Diet, oral health and general health influence each other in a vicious cycle. The aim of this study was to investigate the oral health status of children and young adults with MSUD in Turkey. Methods: A descriptive study was conducted on patients with MSUD who applied for routine follow-up to the pediatric metabolic diseases clinic at Hacettepe University, Children's Hospital in Ankara, Turkey in a 12-month period. Patients with any other concomitant genetic diseases and acute infection were excluded. A total of twenty-five patients were enrolled and underwent oral examination including DMFT/S, dmft/s (decayed/missing/filled teeth/surfaces for deciduous and primary teeth, respectively), plaque and gingival indices. Panoramic radiographs were obtained in 12 cooperative patients. Results: Mean age was 9.88 ± 5.68 s.d years. More than half of the parents had only primary school level education, and low income. Fourteen patients consumed medical formula during or right before sleep. Fourteen patients reported caries-associated pain. Gingival inflammation was present in all 15 patients who cooperated for evaluation. Seven out of twelve patients had at least one dental anomaly or alterations in mandibular morphology. Five patients had previously been treated for caries under general anesthesia. To our knowledge, this is the first study to document oral clinical and radiologic findings in patients with MSUD. Conclusions: Impaired oral health was observed in this rare disease population. Regular dental referral by physicians, preventive measures and dental treatments should be included in multidisciplinary management of maple syrup urine disease to promote oral health.