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    Evaluation of the effect of dental caries, oral hygiene, and treatment need on oral health-related quality of life among Turkish orphan children and adolescents
    (Bmc, 2025) Akbeyaz Sivet, Ecem; Sezer, Berkant; Atmaca, Neslihan; Huseynova, Narmin; Kargül, Betül
    Background The oral and dental health and related quality of life of orphan children and adolescents-who are considered a vulnerable population-are critical for both individual and public health. Objectives This study aimed to investigate the status of the dental caries, oral hygiene, and dental treatment needs among a group of Turkish male orphan children and adolescents, as well as their impact on oral health-related quality of life. Methods A total of 112 orphan children and adolescents aged between 9 and 17 years living in a residential care facility in Istanbul, T & uuml;rkiye, were involved in this cross-sectional study. A comprehensive examination was conducted on each individual, encompassing the assessment of dental caries using the decayed, missing, and filled teeth (DMFT/dft) index, oral hygiene using the debris (DI-S), calculus (CI-S), and simplified oral hygiene (OHI-S) indices, and dental treatment need using the treatment need index (TNI). Oral health-related quality of life was evaluated using the validated Turkish version of the Child Oral Health Impact Profile-Short Form (COHIP-SF-19). The relationship between oral health-related quality of life and explanatory variables was evaluated using a multivariable linear regression model. Results The mean age of the participants was 12 +/- 1.79 years. The mean DMFT score was 4.57 +/- 3.42, and the mean OHI-S score was 1.4 +/- 0.52. Participants had a mean COHIP-SF-19 score of 57.79 +/- 14.11, with 25% requiring at least one tooth to undergo radical treatment. Multivariable linear regression analysis indicated that the DMFT score (p = 0.009) and the need for radical treatment (TNI code 6) (p < 0.001) had a statistically significant impact on oral health-related quality of life among orphan children and adolescents aged 13-17. Conclusions This study underscores that dental caries and the need for radical treatment significantly impact the oral health-related quality of life of orphan children and adolescents.
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    Kohlschütter-Tönz Syndrome: A Rare Clinical Entity with Amelogenesis Imperfecta in Two Siblings, Dental Management and Scoping Review
    (Turkish Pediatric Association, 2025) Akbeyaz Sivet, Ecem; Akbeyaz, İsmail Hakkı; Berkel, Gülcan; Yeşilyurt, Ahmet; Sezer, Berkant; Menteş, Ali
    Kohlschütter-Tönz syndrome (KTS) (OMIM#226750) is a rare autosomal recessive disorder characterized by epileptic encephalopathy, developmental delay, and amelogenesis imperfecta. Early diagnosis and management are crucial, but the complexity of symptoms, particularly dental and neurological impairments, poses significant challenges. The aim of this report is to describe the clinical findings of 2 siblings and their dental management, whose dental examination led to genetic referral and subsequent diagnosis of KTS. Dental examinations revealed enamel defects consistent with amelogenesis imperfecta, including yellow-brown discoloration, soft enamel, and diastemas in both siblings. The younger sibling, a 9-year-old boy, exhibited early-onset seizures, intellectual disability, spasticity, and a history of kidney stones. The older sibling, a 13-year-old boy, presented with more severe neurodevelopmental delay, early-onset seizures, and drug-resistant epilepsy. Genetic testing confirmed homozygous deletions in the ROGDI gene, leading to the diagnosis of KTS in both siblings. The younger sibling received successful restorative treatment under general anesthesia, while the older sibling’s oral care was managed conservatively due to contraindications for general anesthesia. These cases underscore the importance of pediatric dentists in the early identification of rare genetic disorders such as KTS, especially when dental anomalies like amelogenesis imperfecta are present. Timely referral for genetic evaluation can facilitate accurate diagnosis and appropriate care planning. Moreover, sharing clinical experiences and treatment outcomes contributes to a better understanding of this rare syndrome and helps guide future diagnostic and therapeutic strategies.