Oral Bacteria of Children with Turner Syndrome

Aim:Turner syndrome (TS) is a genetic disorder caused by a numerical or structural aberration of the X chromosome, which is associated with a female phenotype. Concerning oral status, several studies have revealed that girls with TS have dental anomalies and periodontal problems. The aim of this study was to evaluate the effects of oral bacteria on caries prevalence and periodontal status in pediatric patients with TS.Materials and Methods:Twenty TS patients and 17 healthy girls were examined for cariological and periodontal status. The levels of mutans streptococci (MS), lactobacilli (LB), yeast and 10 different periodontal bacteria were determined by using culture and microarray techniques in children’s stimulated saliva samples.Results:There was no difference in salivary flow rate and buffering capacity, decayed-missing-filled teeth, MS, LB, or yeast levels between the groups. Plaque index and gingival index levels were significantly higher in the Turner group and dft was significantly higher in the control group (p<0.05). As a result, microarray analysis, Prevotella intermedia, Fusobacterium nucleatum, Eikenella corrodens, Aggregatibacter actinomycetemcomitans, Actinomyces viscosus were detected at high levels in the Turner group (p<0.05).Conclusion:Besides dental and craniofacial anomalies, clinicians should be alert to the early diagnosis and treatment of periodontal problems in patients with TS

The frequency of Duchenne muscular dystrophy/Becker muscular dystrophy and Pompe disease in children with isolated transaminase elevation: results from the observational VICTORIA study

IntroductionElevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia.MethodsThis multi-center, prospective study enrolled patients aged 3–216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels &gt;2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients were analyzed.ResultsOverall, 589 patients [66.8% male, mean age of 63.4 months (standard deviation: 60.5)] were included. In total, 251 patients (188 male and 63 female) had CPK levels above the ULN. Of the patients assessed, 47% (85/182) of male patients were diagnosed with DMD/BMD and 1% (3/228) of male and female patients were diagnosed with PD. The median ALT, AST, and CPK levels were statistically significantly higher, and the questioned neurological symptoms and previously unnoticed examination findings were more common in DMD/BMD patients than those without DMD/BMD or PD (p &lt; 0.001).DiscussionQuestioning neurological symptoms, conducting a complete physical examination, and testing for CPK levels in patients with isolated hypertransaminasemia will prevent costly and time-consuming investigations for liver diseases and will lead to the diagnosis of occult neuromuscular diseases. Trial RegistrationClinicaltrials.gov NCT04120168

EVALUATION OF MALOCCLUSIONS IN PRIMARY DENTITION

It is a known fact that the primary teeth have a significant effect on the development of the permanent occlusion. Malocclusions can be detected early in the primary dentition and if they are left untreated they may be transferred to the permanent dentition. Therefore, knowing aetiology of malocclusions, detecting malocclusions in all three planes of space, deciding when or even whether to treat an orthodontic problem in primary dentition and the treatment options are very important for the general and pediatric dentists