Oral health status of children with phenylketonuria

Ballıkaya, Elif; Yıldız, Yılmaz; Sivri, Hatice Serap; Tokatlı, Ayşegül; Dursun, Ali; Ölmez, Seval; Coşkun, Turgay; Tekçiçek, Meryem

doi:10.1515/jpem-2019-0439

Cited by 13 publications

(19 citation statements)

References 8 publications

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“…However, significantly greater number of children with PKU exhibited the signs of tooth wear compared to their healthy counterparts (33% vs. 24%). More recently, in line with our results, Ballikaya et al ( 13 ), reported higher caries prevalence among PKU patients. Nevertheless, it should be noted that their study primarily examined very young patients (1 to 5 years old) and only a small percentage (16.2%) of children was older than 11 years.…”

Section: Discussionsupporting

confidence: 93%

“…For example, Lucas et al ( 18 ), found a significantly greater mean plaque score for 41 PKU patients compared with controls. Ballikaya et al ( 13 ), found moderate plaque accumulation and gingival inflammation in PKU patients, with nearly all of them requiring professional oral hygiene procedures due to excessive plaque and calculus. The scores for several oral hygiene indices were noticeably higher for our PKU patients compared to their controls, thus indicating an increased periodontal disease risk.…”

Section: Discussionmentioning

confidence: 99%

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Dental Status and Periodontal Health of Patients with Phenylketonuria in Latvia

Abola¹,

Emulina²,

Skadiņš³

et al. 2022

Acta Stomatol Croat

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Objectives Phenylketonuria (PKU) is an autosomal recessive inherited disorder of phenylalanine metabolism resulting from a deficiency of phenylalanine hydroxylase. The aim of this study was to evaluate the dental status and periodontal health of PKU patients in Latvia. Material and Methods Forty-five PKU patients and age/sex-matched controls were recruited for this cross-sectional study. Their anamnestic data, periodontal health and dental status were assessed by one experienced dentist. Results Dental and periodontal clinical evaluation revealed that the median number of filled teeth was significantly smaller among PKU patients compared to the control group (p=0.021). PKU patients had a significantly larger median number of carious teeth than their healthy counterparts (p<0.001). Significant differences between the PKU and control groups were observed for several oral hygiene indices (p<0.001): Silness-Löe plaque index, OR=29.3 (95% CI: 3.7–232.4); CPITN index, OR=35.2 (95% CI: 4.5–278.3); Greene-Vermillion index, OR=10.2 (95% CI: 2.8–38.0); calculus removal necessity, OR=12.3 (95% CI: 3.3–45.4). Conclusion Dental status and periodontal health of PKU patients was found to be significantly inferior compared to healthy controls. This is likely due to the regular consumption of PKU formula and the difficulties which mentally and/or physically disabled PKU patients experience with their oral hygiene. To prevent tooth decay and periodontal disease, PKU patients should visit a professional oral hygienist every three to six months. Furthermore, they should adopt the habit of rinsing their mouth with water immediately after consuming PKU formula to counteract the acidity in their oral cavity.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Dental Status and Periodontal Health of Patients with Phenylketonuria in Latvia

Abola¹,

Emulina²,

Skadiņš³

et al. 2022

Acta Stomatol Croat

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“…Since there has been no other report on oral health indices of patients with MSUD, we could only compare the oral health results of MSUD with the data of patients with PKU. Studies evaluating the oral health status of children with PKU are few and have conflicting results [ 10 – 12 ]. Most studies in children with PKU have revealed a similar or lower prevalence of caries compared to healthy children.…”

Section: Discussionmentioning

confidence: 99%

“…A low rate of caries despite a highly cariogenic diet might result from high phenylalanine (the accumulating amino acid in PKU), which has been considered to possibly act as a factor that limits the growth of plaque microorganisms [ 10 , 11 ]. In a study performed at the same clinic, we have reported the prevalence of dental caries in 132 well-treated PKU patients as 67% [ 12 ]. Although dental caries in MSUD patients was higher than the PKU patients in that study; these differences might be attributed to the oral health behaviors, diet, and concomitant diseases such as epilepsy or intellectual disorders.…”

Section: Discussionmentioning

confidence: 99%

“…Phenylketonuria (PKU) is another inherited metabolic disease treated with a diet similar to MSUD: a low-protein diet supplemented with phenylalanine-free amino acid formulas consumed throughout the day and sometimes during the night [ 9 ]. Dental caries and gingival inflammation were evaluated in PKU patients in previous studies [ 10 – 12 ] because of the fact that these patients have a diet rich in carbohydrates and fats, which is considered to be risk factors for oral health. Patients with MSUD are also at high risk of dental caries, not only because they consume a similar, artificial diet, but also because they may have concomitant intellectual disability.…”

Section: Introductionmentioning

confidence: 99%

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Oral health status of children and young adults with maple syrup urine disease in Turkey

et al. 2021

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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.

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Dietary and metabolic effects on the oral status of patients with phenylketonuria: a nation-based cross-sectional study

Bingöl

Pandis

et al. 2023

Clin Oral Invest

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Objectives The aim of this study was to compare the prevalence of oral diseases (caries, periodontal disease, enamel defects) between patients with phenylketonuria (PKU), their siblings, and a matched control group. Materials and methods A total of 109 patients with PKU, 14 siblings of PKU patients, and 100 healthy individuals aged 6 to 68 years were recruited. All participants completed a questionnaire based on their health status. The patients’ decayed/missing/filled teeth index (dmft/DMFT), gingival bleeding index (GBI), plaque control record (PCR), periodontal screening and recording index (PSR), and developmental enamel defects index (DDE) were recorded. Descriptive statistics and regression modeling were used to examine potential associations between the exposure and the outcomes of interest. Results Patients with PKU had 1.6 times more caries (95% confidence interval (CI) 1.22 to 2.20; p = 0.001), seven times more enamel defects (95% CI 3.94 to 14.21; p < 0.001), and four times higher PSR values (95% CI 2.26 to 7.15; p < 0.001) than the control group. The siblings had significantly fewer enamel defects but no significant differences in caries and periodontal parameters compared to the PKU patients. Conclusions The results showed a higher risk for the development of caries, periodontitis, and enamel defects in PKU patients. Clinical relevance Implementation of preventive measures and regular dental care is necessary for patients with PKU.

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Oral health status of children with phenylketonuria

Cited by 13 publications

References 8 publications

Dental Status and Periodontal Health of Patients with Phenylketonuria in Latvia

Dental Status and Periodontal Health of Patients with Phenylketonuria in Latvia

Oral health status of children and young adults with maple syrup urine disease in Turkey

Dietary and metabolic effects on the oral status of patients with phenylketonuria: a nation-based cross-sectional study

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