Oral features of Griscelli syndrome type II: A rare case report.

Griscelli syndrome (GS) is an autosomal-recessive disorder of the vesicle transport and membrane trafficking system first identified by Griscelli et al in 1978. The three types of GS have specific genetic defects and systemic manifestations apart from classic partial pigmentary dilution, resulting in hypopigmentation of skin and silvery hair. GS-II occurs due to a defect in the Rab27a gene and is characterized by primary immune deficiency along with accelerated phases of a hemophagocytic lymphohistiocytosis (HLH) crisis. This rare disorder has been widely studied for dermatological, hematological, and neurological manifestations; however, the oral features and presentations have not been elucidated in detail. This report presents a case of a 4-year-old male with known mutation c.550C > T or p.R184X mutation (ENST00000396307) in Rab27a with oral features.

940-nm Diode Laser-Assisted Management of Tongue Abscess.

The tongue is a muscular organ with a rich blood supply and acts as an immune defence mechanism. The occurrence of a tongue abscess without immune deficiency is rare. The purpose of this report is to present the case of an eight-year-old boy with a spherical, pinkish-yellow, fluctuant, nontender swelling measuring two cm in diameter on the right anterolateral border of the tongue, causing difficulty in swallowing and speaking. Ultrasonography was performed, followed by a 940-nm diode laser-assisted incision and drainage under local anesthesia. Healing was uneventful, with no recurrence for two years now. A tongue abscess can be life-threatening due to its ability to cause respiratory obstruction. The diode laser can be a safe and effective tool for its management.

Prevalence of dental fear and its causes using three measurement scales among children in New Delhi.

INTRODUCTION: There is a great need for identifying fearful children, who often present problems in patient management, thus affecting the quality of dental care rendered to them. This study is unique in the way that dental fear was assessed through three fear scales as research has suggested the use of more than one scale because each scale has its own restrictions and is open to criticism. AIM: The aim of this study was to evaluate dental fear and anxiety (DFA) among children aged 3-14 years using three fear measurement scales. METHODS: The study was conducted on children (3-14 years) who visited the Department of Pedodontics and Preventive Dentistry at Maulana Azad Institute of Dental Sciences, New Delhi. The DFA levels were measured using three fear measurement scales, i.e., facial image scale (FIS), dental fear scale (DFS), and children's fear survey schedule-dental subscale (CFSS-DS). The dental behavior was estimated using the Frankl's behavior rating scale (FBRS). RESULTS: The prevalence of dental fear according to FIS was 14.3%, according to DFS was 22.6%, and according to CFSS-DS was 7.4%. In assessment of the behavior of children in the clinics through FBRS, it was observed that he maximum number of respondents (69.8%) showed Frankl's Rating 3 i.e. positive. In the DFS and CFSS-DS, the factor which caused most fear was "feeling the needle injected" and "injections," respectively. CONCLUSION: Assessment of dental fear is an extremely useful tool for the dental practitioner, who can use it to customize the behavioral treatment and management for child patients.

Orocraniofacial findings of a Pediatric Patient with Joubert Syndrome.

Joubert syndrome (JS) is a very rare autosomal recessive disorder, involving agenesis or dysgenesis of cerebellar vermis and brain stem. The neurological features of JS include hypotonia, ataxia, developmental delay, intellectual disability, abnormal eye movements, and neonatal breathing dysregulation. These may be associated with multiorgan involvement, mainly retinal dystrophy, nephronophthisis, hepatic fibrosis, and polydactyly. An obligatory hallmark feature associated with JS is the molar tooth sign (MTS), a complex midbrain-hindbrain malformation visible on brain imaging. This case report presents a pediatric case of JS in a 7-year-old girl. Joubert syndrome cases have been reported by various medical specialties in medical journals; however, this probably could be the first report of this rare developmental disorder in dental and oral health. HOW TO CITE THIS ARTICLE: Goswami M, Rajwar AS, Verma M. Orocraniofacial findings of a Pediatric Patient with Joubert Syndrome. Int J Clin Pediatr Dent 2016;9(4):379-383.

Evaluation of cavitated and non-cavitated carious lesions using the WHO basic methods, ICDAS-II and laser fluorescence measurements.

AIM: This study was aimed to compare the diagnostic outcome of the WHO criteria, ICDAS-II criteria and laser fluorescence measurements in measuring the caries ratings of children. SETTINGS AND DESIGN: Cross-sectional study. MATERIALS AND METHODS: The study involved 31 children between 3 and 14 years of age, attending the Department of Pedodontics at Maulana Azad College of Dental Sciences, New Delhi. The surface-related caries status was registered according to the WHO basic method criteria (1997). Additionally, the ICDAS-II visual criteria and the DIAGNOdent readings were documented. STATISTICAL ANALYSIS USED: The data were analysed with ezANOVA and Excel 2000 (Microsoft Corporation, Redmond, WA, USA). RESULTS: The mean ICDAS-II values amounted to 8.76 ± 0.72. The mean values for DMFS/def were 7.67 ± 0.91, whereas for DIAGNOdent it amounted to 4.00 ± 0.62. CONCLUSIONS: In conclusion, this study showed the diagnostic potential of the ICDAS-II criteria in comparison to the traditional WHO criteria by means of the non-cavitated caries lesions additionally detected. The DIAGNOdent use in field studies that already apply detailed visual criteria seems to bring limited additional information.