Phenotypic heterogeneity of impacted third molar tooth: family case study

Tooth impaction is a pathological condition where a tooth fails to attain its normal functional position. The present study aims to highlight the phenotypic heterogeneity of impacted third molars in a Caucasian family, to investigate the characteristics of the dental phenotype, to evidence the diversity of dental phenotype, and to identify the inheritance mode of the condition. Detailed anamnesis, clinical examination, complementary tests (panoramic radiographs), family study and pedigree analysis. Phenotypic characteristics of impacted third molar tooth in our family case report was: Severe horizontal impaction of the mandibular right third molar, Angular impaction of the maxillary right third molar and angular impaction of the maxillary left third molar, Angular impaction of the mandibular left third molar and partial eruption of the maxillary right third molar, Horizontal impaction of the mandibular left third molar and Angular impaction of the mandibular left third molar. The inheritance mode of the impacted third molar tooth in the family case report was: from mother to both daughters’ transmission, from mother to both sons’ transmission, and from mother to daughter transmission. Family study and pedigree analysis are very important for illustrate the genetic basis of impacted teeth

THE PREVALENCE OF MANDIBULAR MIDLINE DEVIATION IN CHILDREN AND ADOLESCENTS WITH DIFFERENT TYPES OF MALOCCLUSIONS

In this study, the prevalence of mandibular midline deviation in different types of malocclusions were evaluated taking into account sexual dimorphism in patients with mixed and permanent dentition. With the aforementioned aim, a cross-sectional study was carried out on a total of 214 patients (134 females and 80 males) aged between 7-15 years (with a mean age of 9.5 ± 2 years). On the study casts of each subject participating in the study, the displacement of the lower dental midline compared to the upper one was measured in different classes of malocclusion, taking into account sexual dimorphism. Three study groups were formed: first with mandibular midline deviation with values less than 2 mm, second with deviation between 2-4 mm and third with deviation more than 4 mm. Subsequently, the prevalence of the displacement of the lower dental midline was assessed taking into account the sexual dimorphism and the Angle classes malocclusions analyzed (Angle Class I, II/1, II/2, III) and UPC (unilateral posterior crossbite). Mandibular midline deviation greater than 2 mm was observed in more than three quarters (75.7%) of the patients in the studied group. In the five classes of malocclusion, a higher frequency of displacement of the lower dental midline was appreciated in the range of 2-4 mm of 59.8%. In this interval, the highest percentage of displacement was observed in Angle Class II/1 (18.2%), followed by unilateral posterior crossbite UPC (15%) and Angle Class II/2 (13.1%). Most large displacement of the lower dental midline (with a value more 4 mm) was recorded in unilateral posterior crossbite UPC (5.6%). Although the displacement of the mandibular midline was recorded in a slightly increased percentage in males (61.25%) compared to the group of females (58.95%), from a statistical point of view, no notable differences were reported between the two genders. Dental midline deviation changes should be noted and analyzed from the beginning of orthodontic treatment in order not to cause later functional or aesthetic disorder

Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

Objective Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. Methods A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. Results Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. Conclusion Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis

Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study.

Objective To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice. Methods We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. Results 254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47-5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55-1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56-3.53], p<0.0001). Patients treated concomitantly with mycophenolate mofetil had a trend for better outcomes as compared with patients receiving rituximab alone (delta FVC: 5.22 [0.83-9.62]; p=0.019 as compared with controls vs 3 [0.66-5.35]; p=0.012). Conclusion Rituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial

Phenotypes determined by cluster analysis and their survival in the prospective european scleroderma trials and research cohort of patients with systemic sclerosis

Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained

Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice