Mutations in CNNM4 cause Jalili syndrome, consisting of autosomal-recessive cone-rod dystrophy and amelogenesis imperfecta.

The combination of recessively inherited cone-rod dystrophy (CRD) and amelogenesis imperfecta (AI) was first reported by Jalili and Smith in 1988 in a family subsequently linked to a locus on chromosome 2q11, and it has since been reported in a second small family. We have identified five further ethnically diverse families cosegregating CRD and AI. Phenotypic characterization of teeth and visual function in the published and new families reveals a consistent syndrome in all seven families, and all link or are consistent with linkage to 2q11, confirming the existence of a genetically homogenous condition that we now propose to call Jalili syndrome. Using a positional-candidate approach, we have identified mutations in the CNNM4 gene, encoding a putative metal transporter, accounting for the condition in all seven families. Nine mutations are described in all, three missense, three terminations, two large deletions, and a single base insertion. We confirmed expression of Cnnm4 in the neural retina and in ameloblasts in the developing tooth, suggesting a hitherto unknown connection between tooth biomineralization and retinal function. The identification of CNNM4 as the causative gene for Jalili syndrome, characterized by syndromic CRD with AI, has the potential to provide new insights into the roles of metal transport in visual function and biomineralization.

Evaluation of the Cause of Dental Treatment in Children under General Anesthesia from the Perspective of Pediatric Dentists and Postgraduate Students and Parents of Children under General Anesthesia at the Dentistry School of Tehran University of Medical Science.

Background: Nowadays, dental treatment under general anesthesia is accepted as a treatment method in children in which other common methods do not allow to achieve acceptable results. The benefits of general anesthesia in pediatric dental treatment are that the treatment is completed in one session, the person is relieved of pain, and most importantly, the child does not need to cooperate. However, it is important to determine the exact dental reasons for children under general anesthesia. Objective: In this study, the cause of dental treatment under general anesthesia in children was examined from the perspective of specialists, assistants, and parents of children under general anesthesia. Methods: In this descriptive-analytical cross-sectional study, participants were selected from among the parents of children under general anesthesia for dental treatment and pediatric dentists and assistants in a purposeful manner. After the items were prepared by the professors, the prepared checklist was read to the people. After completing the checklist, the data were entered into SPSS software version 20 and analyzed using descriptive statistics such as frequency determination, mean, and statistical rests. Results: According to the consensus of parents and experts, the main reasons for general anesthesia for children to perform their dental operations were: young age, lack of cooperation during previous dental treatment, a large number of dental treatments, systemic disease of the child, unwillingness of parents to perform behavioral control procedures, higher quality work under anesthesia, mental and physical behavioral problems, and reduced number of treatment sessions. Conclusion: Therefore, reasons such as young age, lack of cooperation, and a large number of dentist's work are among the most important factors that lead to the decision to perform general anesthesia in children. In light of this decision, the consequences and disadvantages/advantages of this method are important and therefore more research should be done on this issue.

Effect of salivary contamination on microleakage of resin composites placed with a self-etch adhesive in primary teeth: an in vitro study.

PURPOSE: The purpose of this study was to investigate in vitro the effect of salivary contamination on the microleakage of resin composite restorations placed with a self-etching adhesive in primary teeth. METHODS: Fifty-six extracted primary molars received standardized Class V preparations. The teeth were assigned to 7 groups (n=8): 1-layer and 2-layer application of the self-etching primer (Clearfil SE Bond) with no contamination (groups 1 and 2, respectively); saliva, SE primer, and drying (group 3); SE primer, drying, saliva, and drying (group 4); SE primer, drying, saliva, and rinsing/drying (group 5); SE primer, drying, saliva, drying, SE primer, and drying (group 6); and SE primer, drying, saliva, rinsing/drying, SE primer, and drying (group 7). For all specimens, the bonding agent (SE adhesive) and a composite were applied. After thermocycling and immersing in basic fuchsin, the teeth were sectioned and evaluated under a stereomicroscope. Data were analyzed using Kruskal-Wallis and Mann-Whitney tests. RESULTS: The mean occlusal and gingival microleakage for groups 4 and 5 were significantly higher than that of groups 1 and 2 (P<.04). No statistically significant difference in microleakage among groups 1, 2, 6, and 7 was found (P=.09). The degree of microleakage in the gingival margins was significantly higher than that of occlusal margins (P=.027). CONCLUSIONS: The self-etching adhesive was not sensitive to saliva when contamination occurred before primer application.

Comparison of Primary Mandibular First Molar Crown Dimensions with Stainless Steel Crowns in a Sample of Iranian Children.

OBJECTIVES: Attempts to retain primary teeth have led to introduction of materials and techniques for their preservation such as stainless steel crowns (SSCs). Due to variations in tooth anatomy among different populations, this study compared the buccolingual (BL) to mesiodistal (MD) ratio of primary mandibular first molars with that of SSCs in an Iranian population. MATERIALS AND METHODS: This cross sectional study was conducted on 96 primary mandibular first molars with intact cementoenamel junction, which had been extracted for severe caries. The MD and BL dimensions of the teeth and available maxillary and mandibular SSCs (3M) were measured. Two independent examiners measured the dimensions twice at two different time points using a digital caliper. Data were analyzed by one-sample t-test and McNemar's test via SPSS 21.0 software. RESULTS: Significant differences were observed in both BL and MD dimensions between teeth and both maxillary and mandibular SSCs (P<0.001). According to McNemar's test, the dimensions of 65.6% of teeth matched those of mandibular SSCs while the dimensions of 38.5% matched those of maxillary crowns. Dimensions of 3.1% of the teeth did not match any of the crowns and in 7.4%, both maxillary and mandibular SSCs were appropriate. CONCLUSION: Comparing the variances of BL/MD ratio of teeth with SSCs revealed that the dimensions of two-thirds of the teeth matched those of mandibular SSCs, while the maxillary SSCs were appropriate for the remaining one-third. Significant differences were seen between the size of teeth and both maxillary and mandibular crowns.

Efficacy of Three Toothpastes in Iron Stain Removal from Primary Teeth.

AIM: This study aimed to assess the iron stain removal efficacy of three toothpastes in extracted primary teeth. MATERIALS AND METHODS: In this in vitro study, 60 extracted sound primary teeth were selected, decoronated at the cementoenamel junction, and their pulp chambers were filled with a composite. The teeth were then immersed in ferrous sulfate solution and brushed 3,000 times in an automatic tooth-brushing machine using Colgate, whitening Crest, and conventional Crest dentifrices. Stain removal was done in four groups using a prophylaxis paste. Digital images were obtained from the teeth before and after brushing with dentifrices and the changes in color parameters were measured using Photoshop and iColor software programs. Changes in color parameters were statistically analyzed using one-way ANOVA while multiple comparisons were done by Tukey's test. RESULTS: The greatest change in chroma was observed in the prophylaxis group and then in the whitening Crest, Colgate, and conventional Crest (mean values of -65.2, -31.07, -21.27, and -0.73, respectively). Prophylaxis completely removed the stains. The greatest reduction in value occurred in conventional Crest, Colgate, and whitening Crest (-18.07, -12.23, and -0.4, respectively). In the L*a*b* system, the least reductions were noted in the whitening Crest, Colgate, and conventional Crest (mean values of -1.96, -3.92, and -3.37, respectively). Prophylaxis significantly improved tooth brightness (a mean increase of 4.26). CONCLUSION: None of the toothpastes in this study were capable of effectively removing iron stains. Crest whitening toothpaste was slightly effective in this regard. CLINICAL SIGNIFICANCE: Iron drops are routinely prescribed for children younger than 2 years of age to prevent iron deficiency and iron deficiency anemia. However, iron stains on teeth are a common concern for many parents. Finding toothpastes with greater efficacy for iron stain removal can help in this respect. HOW TO CITE THIS ARTICLE: Heidari A, Shahrabi M, et al. Efficacy of Three Toothpastes in Iron Stain Removal from Primary Teeth. Int J Clin Pediatr Dent 2019;12(1):10-14.

Shear Bond Strength of Self-Adhesive Flowable Composite, Conventional Flowable Composite and Resin-Modified Glass Ionomer Cement to Primary Dentin.

OBJECTIVES: This study aimed to compare the shear bond strength (SBS) of self-adhesive and conventional flowable composites and resin-modified glass-ionomer cement (RMGIC) to primary dentin. MATERIALS AND METHODS: In this in vitro, experimental study, the buccal surface of 48 primary canine and first molar teeth was longitudinally sectioned to expose dentin. The teeth were randomly divided into three groups (n=16) of 37.5% phosphoric acid+ OptiBond+ Premise Flow composite (group 1), Vertise Flow composite (group 2) and RMGIC (group 3). A plastic cylindrical mold was placed on the exposed dentin and filled with restorative materials. The samples were then immersed in distilled water at 37°C for 24 hours, subjected to 1000 thermal cycles between 5-55°C and underwent SBS test. The mode of failure was determined under a stereomicroscope. Data were analyzed using one-way ANOVA and Tukey's test. RESULTS: A significant difference was noted in SBS of the groups (P<0.05). The SBS of conventional flowable composite was significantly higher that of RMGIC and self-adhesive flowable composite (P<0.05). The difference in SBS of RMGIC and self-adhesive flowable composite was not significant (P>0.05). Failure at the dentin-restoration interface (adhesive failure) had the highest frequency in groups 1 and 2. The frequency of adhesive failure was 100% in group 3. CONCLUSION: Within the limitations of this study, the conventional flowable composite yielded the highest SBS to primary dentin. Self-adhesive flowable composite and RMGIC showed the lowest SBS with no significant difference with each other.

Microleakage of a Self-Adhesive Flowable Composite, a Self-Adhesive Fissure Sealant and a Conventional Fissure Sealant in Permanent Teeth with/without Saliva Contamination.

OBJECTIVES: Application of fissure sealants is a practical method for prevention of occlusal pit and fissure caries. Microleakage is an important factor affecting the success of fissure sealant treatment. This study aimed to assess the microleakage of a self-adhesive flowable composite, a self-adhesive fissure sealant and a conventional fissure sealant in permanent teeth with/without saliva contamination. MATERIALS AND METHODS: This in vitro, experimental study evaluated 108 extracted human third molars, which were randomly divided into six groups (n=18) of control, Denu-Seal conventional fissure sealant applied on etched enamel with/without saliva contamination, Vertise Flow self-adhesive flowable composite applied on rinsed and dried enamel with/without saliva contamination and Prevent Seal self-adhesive fissure sealant applied on rinsed and dried enamel with/without saliva contamination. Microleakage was assessed using the dye penetration technique and subsequent observation of samples under a stereomicroscope. Data were analyzed using the Kruskal-Wallis and Tukey's test. RESULTS: Microleakage of the conventional fissure sealant with/without saliva contamination was significantly lower than that of other groups (P<0.05). CONCLUSION: Microleakage of the conventional fissure sealant is less than that of self-adhesive fissure sealant and self-adhesive composite, irrespective of saliva contamination.

Periodontal Assessment of Permanent Molar Teeth Restored with Stainless Steel Crown in Terms of Pocket Depth, Bleeding on Probing, Gingival Color and Inflammation.

AIM: This study assessed the gingival health of permanent first molars before and 6 months after restoration with stainless steel crowns (SSCs). MATERIALS AND METHODS: This study was conducted on 23 children aged 6 to 12 years who required SSCs for their permanent first molars in one quadrant and had a contralateral first molar without SSC. Gingival color, inflammation, bleeding on probing (BOP), and probing pocket depth (PPD) were measured around both teeth. SSC was placed and, 6 months later, the same parameters were measured at both sides. RESULTS: Gingival color significantly improved and PPD, BOP, and inflammation in mesiobuccal and mesiolingual areas around the first molars restored with SSC significantly decreased after 6 months (p < 0.05). However, in distobuccal and distolingual areas, no significant change occurred in these indices 6 months after placement of SSC (p > 0.05). CONCLUSION: The results showed that SSCs with proper fit have no adverse effect on gingiva of permanent first molars given that the patient maintains a good oral hygiene. HOW TO CITE THIS ARTICLE: Heidari A, Shahrabi M, et al. Periodontal Assessment of Permanent Molar Teeth Restored with Stainless Steel Crown in Terms of Pocket Depth, Bleeding on Probing, Gingival Color and Inflammation. Int J Clin Pediatr Dent 2019;12(2):116-119.

The Effect of Disinfection with Chlorhexidine on the Shear Bond Strength of Equia Resin-Modified Glass Ionomer Cement to Dentin in Permanent Teeth after Two Thermocycling Protocols.

STATEMENT OF THE PROBLEM: There are some concerns regarding the effect of chlorhexidine (CHX) applied for cavity disinfection on the bond strength of adhesive restorations to dentin. PURPOSE: This study sought to assess the effect of CHX on the shear bond strength (SBS) of Equia resin-modified glass ionomer cement (RMGIC) to dentin in permanent teeth. MATERIALS AND METHOD: In this experimental study, the buccal surface of 84 freshly extracted human premolars was ground to expose the flat dentin. The samples were randomly assigned to four groups (n=21). The steps were as following in the group Ia: conditioning, Equia RMGIC, 500 thermal cycles; group Ib: conditioning, Equia RMGIC, 6000 thermal cycles; group IIa: conditioning, CHX, Equia RMGIC, 500 thermal cycles, and group IIb: conditioning, CHX, Equia RMGIC, and 6000 thermal cycles. Twenty samples from each group were subjected to SBS test and one sample was inspected under a scanning electron microscope. Data were analyzed using two-way ANOVA and t-test. RESULTS: The SBS was significantly different among the groups (p= 0.007). The groups subjected to 500 thermal cycles showed significantly higher SBS to dentin when they were treated with CHX (p= 0.000). There was no significant difference between samples thermocycled for 6000 cycles with and without application of CHX (p= 0.269). The SBS in the groups that were thermocycled for 6000 cycles (Ib and IIb) was lower than those thermocycled for 500 cycles (Ia and IIa). This difference between IIa and IIb was statistically significant (p= 0.007). CONCLUSION: Chlorhexidine can positively affect the short term SBS of Equia RMGIC to dentin.

Comparison of the Level of Substance P and Neurokinin A in Gingival Crevicular Fluid of Sound and Symptomatic Carious Primary Teeth by ELISA.

OBJECTIVES: Pulpal inflammation is often associated with odontogenic pain. Dental pulp is abundantly innervated with sensory fibers encompassing neuropeptides. Neurokinin A (NKA) and substance P (SP) are important neuropeptides in the dental pulp that can cause neurogenic inflammation. Since no previous study has assessed dental pulp neuropeptides in children, this study aimed to compare the level of NKA and SP in gingival crevicular fluid (GCF) of sound and symptomatic carious primary teeth. MATERIALS AND METHODS: Samples of GCF were obtained of 20 sound and 20 painful carious primary teeth. Enzyme-linked immunosorbent assay (ELISA) was used to quantify neuropeptides in GCF. Data were analyzed using paired t-test, ANOVA, Kolmogorov-Smirnov test and correlation coefficient test. RESULTS: A significant difference was noted in the level of NKA in GCF of painful and sound teeth (2.23 pg/ml in painful, and 1.84 pg/ml in sound teeth, P<0.05). The difference between the two groups regarding SP was not significant (2.23 pg/ml in painful, and 2.02 pg/ml in sound teeth, P>0.05). CONCLUSIONS: The results showed that the level of NKA and SP was higher in GCF of painful teeth compared to that of sound teeth. This difference was statistically significant with regard to NKA. Thus, these neuropeptides can serve as indicators for pathological activities in teeth with symptomatic irreversible pulpitis.

The Prevalence of Bruxism and Correlated Factors in Children Referred to Dental Schools of Tehran, Based on Parent's Report.

OBJECTIVE: Bruxism is defined as the habitual nonfunctional forceful contact between occlusal tooth surfaces. The aim of this study was to determine the prevalence of bruxism and correlated factors in children referred to dental schools of Tehran, based on Parent's report. METHODS: This cross-sectional descriptive study was conducted on 600 4-12 year-old children with a mean age of 7.4±2.4 years, who were referred to four dental schools in Tehran. After collecting information with questionnaire filled out by parents, χ(2), Fisher Test, Mann-Whitney and t-Test were used to analyze the data. FINDINGS: The prevalence of bruxism was 26.2%. Bruxism begun in average at the age of 4.9±2 years. Also it occurred 2.6 times more in children who had a family history of bruxism (father-mother), compared to children who didn't have such a history. 87% of children with bruxism had a history of distressing events in their life, and 13% of children with bruxism did not report any history of distressing events in their life. In this study most common oral habit was nail biting. In study of parasomnias, drooling was the most, and snoring the least reported sleep disorder. Bruxism in children with drooling was twice more than in other children. The prevalence of bruxism in children with temporomandibular disorder was 63.6% and in children without TMD was 24.7%. CONCLUSION: Based on Parent's report, 26.2% of children showed bruxism and there was a significant relation between bruxism and mother's job, family history, distressing event in life, parasomnias, especially drooling and sleep walking, TMD, hyperactivity, depression, acrophobia and lygophobia.