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Proteolytic enzymes serve important functions during dental enamel formation, and mutations in the kallikrein 4 (KLK4) and enamelysin (MMP20) genes cause autosomal-recessive amelogenesis imperfecta (ARAI). So far, only 1 KLK4 and 3 MMP20 mutations have been reported in ARAI kindreds. To determine whether ARAI in a family with a hypomaturation-type enamel defect is caused by mutations in the genes encoding enamel proteolytic enzymes, we performed mutational analysis on candidate genes. Mutational and haplotype analyses revealed an ARAI-causing point mutation (c.910G>A, p.A304T) in exon 6 of MMP20 that results in a single amino acid substitution in the hemopexin domain. Western blot analysis showed decreased expression of the mutant protein, but zymogram analysis demonstrated that this mutant was a functional protein. The proband and an affected brother were homozygous for the mutation, and both unaffected parents were carriers. The enamel of newly erupted teeth had normal thickness, but was chalky white and became darker with age. [ABSTRACT FROM AUTHOR]

Background: An ideal aesthetic restorative material should be attached to the tooth tissues by adhesion, have a smooth surface as possible, should not cause toxic reactions in the pulp and discoloration and microleakage. This study aims at comparatively assess the cytotoxicity of current adhesive systems on human dental pulp cells., Materials and Methods: The adequate density of human pulp cells was observed from the ready cell line. The passaging was performed and the 3rd passage cells were selected. Adhesive systems and MTA were used on the cultures. Trypan blue staining was conducted on the cells at the 1st, 2nd, 3rd days and a count of live and dead cells using a light microscope. The dead cells whose membrane integrity was impaired by staining with trypan blue and the viability rate was determined using live and dead cell numbers. Data analysis was performed using IBM SPSS Statistics 22., Results: A significant difference in vialibity rates between adhesive systems was observed on the first day. No significant statistical differences were observed on the 2nd and 3rd days (p < 0.05)., Conclusion: Futurabond M showed similar biocompatibility with MTA on human pulp cells and it can be applied in cavities with 1-1.5 mm hard tissue between pulp and dentine., (© 2024. The Author(s).)

This study evaluated the influence of socio-demographic, clinical, and parental psychological factors on oral health-related quality of life (OHRQoL) and determined their predictors in preschool-aged children with traumatic dental injury (TDI). The study sample consisted of 324 dyads of children and their parents attending the Clinics of Pediatric Dentistry at Istanbul University. After clinical examination, the Sense of Coherence (SOC), Generalized Self-Efficacy Scale, Early Childhood Oral Health Impact Scale, and Multidimensional Health Locus of Control Scale-MHLC questionnaires were administered to the parents. The statistical analyses included Spearman correlation coefficients, Mann-Whitney U tests, Kruskal-Wallis tests, multiple linear regression, and confirmatory factor analysis. Having mixed TDI, a non-nuclear family, fewer children, and weak parental SOC were important predictors of worse overall OHRQoL and its sections of child and family. Lower internal health locus of control and dental pain due to TDI were predictors of worse overall OHRQoL and child impact, respectively. Consideration of these predictors may help oral health professionals to develop prevention and treatment programs for TDI and oral health literacy programs for families.

Objectives:  Precocious puberty indicates quick growth inception and delayed puberty indicates retardation in growth. This study aimed to investigate whether dental development is synchronous with somatic development., Materials and Methods:  In this study, 62 girls and 34 boys with precocious puberty aged 5 to 9, 29 girls with delayed puberty aged 13 to 16, and 43 boys with delayed puberty aged 14 to 17; 169 children (91 girls and 78 boys) with normal development were compared about their dental ages through their panoramic radiographs by using the Demirjian method and skeletal ages from hand-wrist radiographs by using Greulich-Pyle atlas., Results:  The findings showed that, in all cases, the dental age values were higher than chronologic and skeletal age values to a statistically significant degree. In the precocious puberty group, the dental age values were higher than chronologic age values to a statistically significant degree. In the delayed puberty group, the difference determined between the chronological age and the dental age was not found to be statistically significant., Conclusion:  Given that the Demirjian method is inclined to make calculations that are higher than the chronological age, our findings suggest that the dental development was faster in the precocious puberty group and retarded in the delayed puberty group., Competing Interests: None declared.This study was presented in Turkish Dental Association,23rd International Dental Congress at Istanbul CongressCenter, Istanbul, Turkey 21-24 September 2017 (Oralpresentation “Comparative Study of Dental Age inPrecocious and Delayed Puberty Periods in Children” byD.T., R.B., and K.G., (European Journal of Dentistry. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

There are recent studies which aimed to detect the inheritance on the etiology of dental caries exploring oral composition. We present data on the oral microbiota and its relation with dental caries and other factors in monozygotic (MZ) and dizygotic (DZ) twin children. Following clinical investigation, DNA samples were collected and isolated from saliva of 198 patients (49 MZ and 50 DZ twins) with an average age of 9.7 ± 2.7 years. Salivary bacterial microbiota analysis was performed using high throughput amplicon sequencing method targeting V3-V4 region of the 16S rRNA gene. A total of 8,297,859 raw reads corresponding to 41,908 reads per sample were obtained on average. The QIIME2-deblur workflow was used for 16S rRNA amplicon analysis. Microbiome similarity analyses between twins (based on Bray-Curtis dissimilarity, weighted and unweighted Unifrac distances) showed that monozygotic twins share more bacterial microbial content compared to dizygotic twins. This is a large microbial community dataset of MZ and DZ twins with or without dental findings which can be further used for children oral microbiome profile explorations.

Objective: The goal of the present work was to use dental conditions that have been independently associated with cleft lip and palate (CL/P) as a tool to identify a broader collection of individuals to be used for gene identification that lead to clefts., Study Design: We studied 1573 DNA samples combining individuals that were born with CL/P or had tooth agenesis, supernumerary teeth, molar incisor hypomineralization, or dental caries with the goal to identify genetic associations. We tested 2 single-nucleotide polymorphisms that were located in the vicinity of regions suggested to contribute to supernumerary teeth. Overrepresentation of alleles were determined for combinations of individuals as well as for each individual phenotypic group with an α of .05., Results: We determined that the allele C of rs622260 was overrepresented in all individuals studied compared with a group of unrelated individuals who did not present any of the conditions described earlier. When subgroups were tested, associations were found for individuals with hypomineralization., Conclusions: Although we did not test this hypothesis directly in the present study, based on associations reported previously, we believe that CL/P is actually a syndrome of alterations of the dentition, and considering it that way may allow for the identification of genotype-phenotype correlations that may be useful for clinical care., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background/aims: Enamel-renal syndrome is characterized by nephrocalcinosis, enamel defects, gingival hyperplasia and eruption failures. It has been recently identified that recessive mutations in the FAM20A gene result in amelogenesis imperfecta (AI)-gingival fibromatosis. The aim of this research to determine whether AI patients with known -FAM20A mutations also have nephrocalcinosis., Methods: Complete oral and radiological examinations were performed for all participating family members. Renal examinations were performed using ultrasound., Results: The teeth were evaluated for severe loss, and multiple eruption failures were evident from the clinical and radiological examinations. Unexpected extensive and fast crown resorption was found by radiological examination. Renal ultrasound revealed bilateral nephrocalcinosis in both affected individuals. Recessive FAM20A mutations can cause nephrocalcinosis in addition to the oral phenotype., Conclusion: AI patients with similar clinical phenotypes and FAM20A mutations should be examined for nephropathy even if they lack pertinent symptoms. Nephrology referral is warranted for patients who have clinical phenotypes related to AI-gingival fibromatosis even if they are not symptomatic., (© 2018 S. Karger AG, Basel.)

Amelogenesis imperfecta is a group of rare inherited disorders that affect tooth enamel formation, quantitatively and/or qualitatively. The aim of this study was to identify the genetic etiologies of two families presenting with hypomaturation amelogenesis imperfecta. DNA was isolated from peripheral blood samples obtained from participating family members. Whole exome sequencing was performed using DNA samples from the two probands. Sequencing data was aligned to the NCBI human reference genome (NCBI build 37.2, hg19) and sequence variations were annotated with the dbSNP build 138. Mutations in MMP20 were identified in both probands. A homozygous missense mutation (c.678T>A; p.His226Gln) was identified in the consanguineous Family 1. Compound heterozygous MMP20 mutations (c.540T>A, p.Tyr180 * and c.389C>T, p.Thr130Ile) were identified in the non-consanguineous Family 2. Affected persons in Family 1 showed hypomaturation AI with dark brown discoloration, which is similar to the clinical phenotype in a previous report with the same mutation. However, the dentition of the Family 2 proband exhibited slight yellowish discoloration with reduced transparency. Functional analysis showed that the p.Thr130Ile mutant protein had reduced activity of MMP20, while there was no functional MMP20 in the Family 1 proband. These results expand the mutational spectrum of the MMP20 and broaden our understanding of genotype-phenotype correlations in amelogenesis imperfecta.

Amelogenesis imperfecta (AI) is a heterogeneous group of genetic disorders affecting tooth enamel. The affected enamel can be hypoplastic and/or hypomineralized. In this study, we identified ACPT (testicular acid phosphatase) biallelic mutations causing non-syndromic, generalized hypoplastic autosomal-recessive amelogenesis imperfecta (AI) in individuals from six apparently unrelated Turkish families. Families 1, 4, and 5 were affected by the homozygous ACPT mutation c.713C>T (p.Ser238Leu), family 2 by the homozygous ACPT mutation c.331C>T (p.Arg111Cys), family 3 by the homozygous ACPT mutation c.226C>T (p.Arg76Cys), and family 6 by the compound heterozygous ACPT mutations c.382G>C (p.Ala128Pro) and 397G>A (p.Glu133Lys). Analysis of the ACPT crystal structure suggests that these mutations damaged the activity of ACPT by altering the sizes and charges of key amino acid side chains, limiting accessibility of the catalytic core, and interfering with homodimerization. Immunohistochemical analysis confirmed localization of ACPT in secretory-stage ameloblasts. The study results provide evidence for the crucial function of ACPT during amelogenesis., (Copyright © 2016 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Background: Talon cusp is a rare dental anomaly characterized by a cusp-like projection, often including the palatal surface of the affected tooth. The aim of the present study was to investigate the prevalence and characteristics of talon cusps in a group of Turkish children., Materials and Methods: The study population consisted of 14,400 subjects who attended the clinics of the Department of Pediatric Dentistry at the Istanbul University, Istanbul, Turkey. Subjects ranged in age from 1 to 14 years with a mean age of 10.5 ± 2.55 years. Talon cusps were mainly categorized by visual examination according to the classification of Hattab et al. The distribution and frequency of talon cusps were calculated with respect to dentition type, tooth type, talon type, the affected surface, associated dental anomalies, and clinical complications. Statistical analysis included descriptive statistics, frequencies, and crosstabs with Chi-square analysis., Results: Talon cusps were detected in 49 subjects (26 males and 23 females) of 14,400 (0.34%). A total of 108 teeth showed talon cusps. Distribution of talon cusps according to gender showed no statistically significant differences. The incidence of talon cusps was found to be greater in maxillary lateral incisors (53.7%) than central incisors (29.62%). Regarding the type of talon cusp, 47.22% of teeth showed a Type III talon cusp, whereas 30.55% of teeth demonstrated a Type II talon and 22.22% of teeth demonstrated a Type I talon cusp. Nine patients (18.36%) with talon cups also exhibited other developmental dental anomalies. Clinical complications associated with talon cusps were detected as caries formation and occlusal interference., Conclusion: This is the most comprehensive study of the prevalence of talon cusps in Turkish population using the largest sample size to date. Also, taurodontism associated with a talon cusp has been reported here for the 1(st) time. Clinical complications associated with talon cusps need more investigations.

Truncation mutations in FAM83H (family with sequence similarity 83, member H) cause autosomal dominant hypocalcified amelogenesis imperfecta (ADHCAI), but little is known about FAM83H function and the pathogenesis of ADHCAI. We recruited three ADHCAI families and identified two novel (p.Gln457*; p.Lys639*) and one previously documented (p.Q452*) disease-causing FAM83H mutations. We generated and characterized Fam83h-knockout/lacZ-knockin mice. Surprisingly, enamel thickness, density, Knoop hardness, morphology, and prism patterns were similar in Fam83h (+/+), Fam83h (+/-), and Fam83h (-/-) mice. The histology of ameloblasts in all stages of development, in both molars and incisors, was virtually identical in all three genotypes and showed no signs of pathology, although the Fam83h (-/-) mice usually died after 2 weeks and rarely survived to 7 weeks. LacZ expression in the knockin mice was used to report Fam83h expression in the epithelial tissues of many organs, notably in skin and hair follicles, which manifested a disease phenotype. Pull-down studies determined that FAM83H dimerizes through its N-terminal phospholipase D-like (PLD-like) domain and identified potential FAM83H interacting proteins. Casein kinase 1 (CK1) interacts with the FAM83H PLD-like domain via an F(270)-X-X-X-F(274)-X-X-X-F(278) motif. CK1 can phosphorylate FAM83H in vitro, and many phosphorylation sites were identified in the FAM83H C-terminus. Truncation of FAM83H alters its subcellular localization and that of CK1. Our results support the conclusion that FAM83H is not necessary for proper dental enamel formation in mice, but may act as a scaffold protein that localizes CK1. ADHCAI is likely caused by gain-of-function effects mediated by truncated FAM83H, which potentially mislocalizes CK1 as part of its pathological mechanism.

Objective: The aim of this in vitro study was to evaluate antimicrobial activities of three different pulp capping materials; Biodentine, mineral trioxide aggregate (MTA) Angelus, and Dycal against Enterococcus faecalis and their durability with time., Materials and Methods: Direct contact test was used for the assessment. Three sets of sealers were mixed and placed on microtiter plate wells: One set was used within 20 min of recommended setting time while others were used after 24-h and 1-week. E. faecalis suspension was placed directly on the materials for 1 h and then transferred to another plate with fresh media. Nine wells of bacteria without the tested cements served as the positive control. One well of the tested cements without bacteria served as the negative control. Bacterial growth was evaluated by a temperature-controlled microplate spectrophotometer for 1-h intervals among 24 h. Data were analyzed using Kruskal-Wallis test., Results: All tested materials showed less bacterial density than the control group. MTA, Biodentine, and Dycal showed significantly higher bacterial density than the control group in freshly mixed samples (P < 0.05). And MTA showed significantly higher antibacterial activity than Dycal (P < 0.05). In 24 h, materials did not show any differences (P > 0.05). MTA and Biodentine samples showed significant differences than Dycal; MTA also showed higher antibacterial activity than control in 1-week samples (P < 0.05)., Conclusion: While freshly mixed MTA showed the best antibacterial activity over time, Biodentine had shown similar antibacterial activity to MTA.

Background: Periodontal ligament (PDL) healing and long term prognosis of replanted avulsed teeth should rely on several factors including length of extra-oral dry time and type of the storage medium. The status of periodontal ligament is critical for the healing of replanted teeth. Different substances have been used for root surface treatment to promote formation of PDL and increase the survival of avulsed teeth submitted to replantation., Aim: The purpose of this study was to assess the effect of recombinant basic fibroblast growth factor 2 (bFGF) and enamel matrix derivative (EMD) on root resorption after delayed replantation., Design: 18 freshly extracted single-rooted incisor and premolar teeth were extracted from the beagle dogs and immersed in whole bovine milk for 45 and 60 min (n = 3 each). Following storage period, sockets washed and teeth were treated with bFGF and EMD and replanted into the sockets. After 8 weeks, dogs were sacrificed, specimens processed to 4-μm thick serial sections for histopathologic examination and morphometric assessments. Thus, the proportions of the roots that exhibited signs of surface resorption, inflammatory resorption, and replacement resorption, that is, ankylosis and normal PDL were noted., Results: The percentage of root resorption was in the following order: EMD>milk>bFGF for 45 min and milk>EMD>bFGF for 60 min. For all groups, teeth stored 60 min showed significantly higher incidence of PDL resorption than those stored for 45 min (P < 0.01). The highest incidence of replacement resorption was observed in teeth treated with EMD for 60 min. After 8 weeks, the least resorption was found in bFGF-treated group (P < 0.01)., Conclusions: The findings of this study suggest that use of bFGF favored the formation of new periodontal ligament; prevent ankylosis and resorption process following delayed replantation of teeth while EMD shows replacement resorption, which may turn to ankylosis., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

The principles of management of mandibular fractures differ in children when compared to adults and depend on the specific age-related status of the growing mandible and the developing dentition. This paper presents a case report with a complex facial trauma affecting the mandibular body and condyle region and dentoalveolar complex. Clinical examination revealed soft tissue injuries, limited mouth opening, lateral deviation of the mandible, an avulsed incisor, a subluxated incisor, and a fractured crown. CBCT examination revealed a nondisplaced fracture and an oblique greenstick fracture of the mandibular body and unilateral fracture of the condyle. Closed reduction technique was chosen to manage fractures of the mandible. Favorable healing outcomes on multiple fractures of the mandible throughout the 6-year follow-up period proved the success of the conservative treatment. This case report is important since it presents a variety of pathological sequelae to trauma within one case.

Aims: The purpose of this study was to investigate the relationship between vertical asymmetries of the mandibular condyle with different occlusion types, including Angle Cl I, Cl II, Cl III malocclusions and unilateral posterior crossbite (UPC) in adolescent patients., Methodology: A total number of 120 patients (60 girls, 60 boys with a mean age of 13.64 ± 1.58 years) with no signs and symptoms of temporomandibular disorders were included in the study [n = 30 for each group; Group I: normal occlusion, Group II: Angle Class II malocclusion, Group III: Angle Class III malocclusion and Group IV: UPC]. The asymmetry index for each patient was measured using panoramic radiographs. The results were analyzed using Kruskal-Wallis and Mann-Whitney U test at the 95% confidence level., Results: The results of the analyses showed no statistically significant differences between the gender and the age of the patients for condylar height asymmetry (P>0.05). No statistically significant difference was found between the occlusion types, according to condylar asymmetry level. The patients with UPC showed a significantly different level of condylar height asymmetry compared to the Class I, II and III occlusion types (P<0.05; P<0.01)., Conclusions: Patients with UPC have asymmetric condylar heights. These patients might be at risk for developing skeletal mandibular asymmetries in the future. Early correction of posterior crossbite can help practitioners prevent skeletal asymmetries.

Dentigerous cysts (DCs) are benign odontogenic cysts that are associated with the crowns of permanent teeth. The purpose of this study is to describe the management of DCs in four children. Four boys aged between 7 and 9 years were referred to our clinics with the complaints of intraoral alveolar swelling or facial asymmetry on the affected area. The panoramic radiographies showed large, well-defined radiolucent lesions associated with the deciduous teeth and displaced tooth buds. The treatment consisted of the extraction of the involved deciduous tooth and marsupialization of the cyst to allow eruption of the permanent tooth. Permanent teeth displaced by the DCs in three cases erupted spontaneously within one-year period. The case with horizontally displaced permanent tooth was managed by replantation. This is the first time that underlying permanent tooth in a DC case was intentionally replanted.

The aim of this clinical case series is to present a diagnosis and different treatment methods of patients in different ages with amelogenesis imperfecta (AI) as well as further treatments during a 3-6 years follow-up period. A number of 31 patients (16 female, 15 male with a mean age of 10.77 ± 2.65 years) with AI have been examined for the study group between 2007 and 2010 years. A detailed anamnesis was recorded, followed by a clinical and radiological assessment of oral health. The types of AI classified for each patient according to clinical and radiographic evaluation. The main complaints of patients, presence of dental caries and dental anomalies were noted. Necessary treatments had been planned for the individual cases of AI. A number of 19 patients had hypoplastic (HP) form, and 10 patients showed hypomaturation (HM) form of AI, while one patient showed hypocalcified form of AI and one patient had HM-HP form with taurodontism. Main complaints were chiefly related to dissatisfactory esthetics and dental sensitivity. Caries prevalence index was 93.5%. Mean decayed, missing, filling permanent teeth (DMF) and DMF surface (DMFS) were found as 2.74 ± 1.71 and 6.23 ± 3.99; df (decayed, filling primary teeth) and dfs (decayed, filling primary teeth surface) were found as 3.12 ± 2.85 and 5.24 ± 4.97, respectively. All patients received individual clinical care, including preventive, restorative, and prosthetic treatments. Patients have scheduled for regular follow-up in every 3 months. Composite restorations were used as the most common treatment (25 patients, 80.6%). The treatment plan should be based on patient's age, type of defects and individual needs of the patients. Necessary treatment plan is essential, not only due to functional and aesthetic reasons, but also for the positive psychological impact on young patients.

Genetic disturbances during dental development influence variation of number and shape of the dentition. In this study, we tested if genetic variation in enamel formation genes is associated with molar-incisor hypomineralization (MIH), also taking into consideration caries experience. DNA samples from 163 cases with MIH and 82 unaffected controls from Turkey, and 71 cases with MIH and 89 unaffected controls from Brazil were studied. Eleven markers in five genes [ameloblastin (AMBN), amelogenin (AMELX), enamelin (ENAM), tuftelin (TUFT1), and tuftelin-interacting protein 11 (TFIP11)] were genotyped by the TaqMan method. Chi-square was used to compare allele and genotype frequencies between cases with MIH and controls. In the Brazilian data, distinct caries experience within the MIH group was also tested for association with genetic variation in enamel formation genes. The ENAM rs3796704 marker was associated with MIH in both populations (Brazil: p=0.03; OR=0.28; 95% C.I.=0.06-1.0; Turkey: p=1.22e-012; OR=17.36; 95% C.I.=5.98-56.78). Associations between TFIP11 (p=0.02), ENAM (p=0.00001), and AMELX (p=0.01) could be seen with caries independent of having MIH or genomic DNA copies of Streptococcus mutans detected by real time PCR in the Brazilian sample. Several genes involved in enamel formation appear to contribute to MIH., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Aim: The aim of this study was to quantify and compare the amount of methyl methacrylate (MMA) monomer released from three different denture base acrylic material processed by different polymerization methods and storage conditions., Materials and Methods: Microwave-cured, conventional heat and injection-technique acrylic polymerized materials were stored in neutral (pH = 7) and acidic (pH = 4.5) artificial saliva for 24 hours at the room temperature, separately. The residual MMA content was determined by high performance liquid chromatography (HPLC)., Results: The MMA leaching from resins showed that microwave-cured material has lower residual MMA leach compared both conventional and injection-technique on neutral saliva storage conditions., Conclusion: The all data's exhibited higher MMA release into an acidic saliva environment than neutral artificial saliva and there were no significant differences between the materials groups on acidic saliva storage conditions., Clinical Significance: Microwave polymerization method might has some advantages on reducing release of MMA concentration and may contributory effect upon polymerization reaction on neutral pH storage condition, therefore methods of polymerization should be considered as amount of monomer release.

Iatrogenic trauma can be defined as any adverse condition in a patient resulting from treatment by a physician or dentist. Orthodontic treatment carries with it the risks of tissue damage and treatment failure. The aim of this article is to present traumatic oral tissue lesions resulting from iatrogenic orthodontic origin with a 2-year follow-up period based on orthodontic intervention followed by periodontal surgery. The management of traumatic injuries is dependent on the severity of the involvement of the periodontal tissues. While, in most cases, the elimination of the offending agent and symptomatic therapy is sufficient, in severe cases, or when the injury resulted in permanent defects, periodontal/regenerative therapy may be necessary. The dentist must be aware of these risks in order to help the patient make a fully informed choice whether to proceed with orthodontic treatment. The skill, experience, and up-to-date knowledge of dentists are the main factors to prevent possible iatrogenic traumas.

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterised by a proliferation of abnormal and clonal Langerhans cells. LCH most commonly occurs as a localised solitary bone lesion and appears predominantly in paediatric patients. This case report presents clinicopathological features of this disorder in a 2-year-old boy. The presenting features of the patient included fever, bleeding gums and seborrheic dermatitis-like rash. Intraoral examination and radiographs revealed deep pockets and severe bone loss around all primary molars. Due to severe periodontal destruction, a gingival biopsy was taken. The biopsy specimen revealed heavy infiltration of Langerhans histiocytes. Chemotherapy was administered. Although no clear cause of LCH has been identified, the disease can be triggered by environmental agents and viruses, in particular Epstein-Barr virus. Oral manifestations may be the first or only signs of LCH.

Purpose: Cherubism is a rare autosomal dominant syndrome characterized by abnormal bone tissue in the lower part of the face. Mutations in the gene coding for SH3BP2 have been identified in about 80% of people with cherubism. The aim of this study was to determine whether a mutation in the SH3BP2 gene was the molecular basis of cherubism in two unrelated families., Methods: Two cases of the aggressive form of Cherubism were described in two Turkish families with extensive bilateral swelling in the mandible, typical pathological features and familial history. Genomic DNA was extracted from six affected and three unaffected individuals from two families, and mutations in the SH3BP2 were detected by PCR, and direct DNA sequencing was carried out., Results: In the first family, a missense mutation Arg415Gln was found in exon 9 of the SH3BP2 in all affected individuals. The unaffected individuals did not have this mutation. In the second family, another missense mutation Pro418Thr was identified in exon 9 of the SH3BP2 in the patient and his mother with cherubism., Conclusions: We detected the point mutations in the SH3BP2 gene in the patients with multiple affected individuals. Genotype-phenotype association studies in individuals with cherubism are necessary to provide important knowledge about molecular mechanisms of the disease.

Amelogenesis imperfecta (AI) is a genetically and clinically heterogeneous group of inherited dental enamel defects without any other nonoral symptoms. Recently, a disease-causing nonsense mutation (c.406C>T) in a novel gene, FAM20A, was identified in a large consanguineous family affected by AI with gingival hyperplasia. We performed mutational analyses on nine AI families with similar phenotypes and identified three homozygous mutations (c.34&#95;35delCT, c.813-2A>G, c.1175&#95;1179delGGCTC) in three families and a compound heterozygous mutation (c.[590-2A>G] + [c.826C>T]) in one family. An in vitro splicing assay with a minigene confirmed the mutations located in the splicing acceptor site caused the deletion of exons 3 and 6, respectively. Taking into consideration the locations of the nonsense and frameshift mutations, the mutant transcripts are most likely degraded by nonsense-mediated mRNA degradation and it results in a loss of the FAM20A protein. This study confirms the importance of the FAM20A protein in enamel biomineralization as well as tooth eruption., (© 2011 Wiley Periodicals, Inc.)

Traumatic injuries may cause anatomic deficiencies in soft and hard tissues. These defects often result in the loss of attached mucosa and alveolar processes, which might reduce potential prosthesis support and require bone and skin grafting. As a result of major maxillofacial trauma, complete or partial avulsion of the palate may require extensive surgical and prosthodontic rehabilitation. The appropriate treatment for the maxillary defect demands a multidisciplinary approach by a team which consists of various fields of dentistry and medicine. The planning prostheses should replace not only missing teeth but also lost soft tissues and bone, and they should include the hard palate, residual alveolar ridges, and, in some instances, the soft palate. This paper describes the treatment procedures including plastic surgery operation procedures and prosthetic rehabilitation in a 19-year-old woman after her severe bicycle accident.

Dentigerous cysts are benign odontogenic cysts that are associated with the crowns of permanent teeth. They are usually characterized as unilocular radiolucent lesions and are rarely seen during childhood. The purpose of this article was to report the case of a 6-year-old girl with a dentigerous cyst associated with the tooth buds of premolars. The therapeutic approach included extraction of the primary molar involved and marsupialization of the lesion. After 21 months of follow-up, spontaneous eruptions of the impacted premolars were noticed. In conclusion, marsupialization might be the first treatment option for conservative management of dentigerous cysts in children.

We describe the dental and craniofacial anomalies of 2 ethnically distinct patients with Goldenhar syndrome, which is characterized by hemifacial microsomia, facial asymmetry, and ear and dental abnormalities. A 7-year-old Japanese girl and 12-year-old Turkish boy with Goldenhar syndrome were examined clinically and radiographically; both had symptoms of hemifacial microsomia. Multiple organ involvement can limit surgical correction of deformities and affect patient management. Therefore, long-term regular follow-up by a multidisciplinary team is important to monitor the growth and development of patients.

Aim: This study was designed to determine the type and amount of the monomers leached from the different particle sizes of the composite materials., Materials and Methods: Three different disk sizes (2, 4, 6 mm) prepared for each material group (Filtek Flow, Filtek A110, Filtek P60 and Filtek Supreme) were polymerised by LED and halogen light; the specimens were then placed in artificial saliva. The monomer release in 30 min and 24 hrs from the specimens was analyzed in HPLC calibrated for the monomer extracts before., Results: TEGDMA release was detected in all material groups after 30 min and after 24 hrs. BisGMA and BisEMA were not determined in any groups and UDMA was detected only in Filtek Supreme. Significant differences in release of TEGDMA and UDMA were obtained between the different sizes of discs. Significantly high amount of TEGDMA and UDMA monomer release was obtained in LED than Halogen groups. Lower amount of monomer release was obtained in species of 30 min than 24 hrs., Conclusion: Data has revealed that the monomer release could be detected significantly high from the composite materials polymerized by a lower output curing light device; and higher elution of monomers was determined as the composite thickness has increased. Therefore, the clinical applications of composite materials and the type of curing units have very important effects on the success of restorations and in the decrease of potential side effects.

A case with a dilacerated maxillary permanent central incisor, treated with forced eruption technique is illustrated and the results of the 4-year follow up are presented. After the successful eruption of the tooth, the root development was completed and the root canal was obliterated. The 4-year follow-up results revealed the tooth to be still functional and the radiographic evaluation showed that the periodontal and periapical tissues were intact and healthy. In conclusion, the impacted dilacerated incisor diagnosed in the early mixed dentition should be treated with the aid of orthodontic traction. The long-term follow up showed that once the tooth is placed in the occlusion properly, it may function well esthetically and preserve its periodontal and periapical health.