Ectodermal dysplasia is a heterogeneous group of inherited disorders, which exhibit a classic triad of hypohydrosis, hypotrichosis, and hypodontia. Hypohidrotic or anhidrotic ectodermal dysplasia or Christ Siemens Touraine syndrome is the most common condition among ectodermal dysplasia patients. This is a case report on two Christ Siemens Touraine syndrome cases and two different approaches to prosthetic management.
Aim:To evaluate and compare the efficacy of “calcium sucrose phosphate” (CaSP) toothpaste (Enafix 5%) with ordinarily used calcium, phosphate-containing toothpaste in elevating the level of calcium, phosphate ions in saliva. Secondary aims were to evaluate substantivity and plaque-reducing ability of CaSP toothpaste.Materials and Methods:Thirty study participants of age group 6–13 years were divided into two groups: Group X (Control group) was made to continue brushing with their regularly used calcium, phosphate-containing toothpaste and Group Y (Test group) was allotted CaSP toothpaste. 1 ml of unstimulated saliva was periodically collected from both groups to determine any alteration in the salivary calcium, phosphate level. Parameters such as substantivity and plaque-reducing ability of CaSP toothpaste were also evaluated. Salivary mineral's intergroup comparison was evaluated by Student's t-test while its intragroup comparison along with the plaque amount variation in Group Y was evaluated by ANOVA and Tukey's post hoc test.Results:Group Y showed an increase in the salivary calcium level though not statistically significant. The increase was pronounced in samples collected on day 1. Group Y showed a consistent level of calcium, phosphate in samples collected immediately and 6 h postbrushing, indicating its substantivity. In addition, Group Y had an impact in reducing the plaque level when the 1st-month plaque score was compared with the 12th-month score.Conclusion:CaSP leads to an increase in the salivary calcium level though it was not statistically significant. Supervised brushing and dietary habits showed a positive effect on both the groups. CaSP toothpaste also showed substantivity and plaque-reducing ability.
Hypodontia is a developmental dental anomaly defined as the absence of one or more primary or permanent teeth excluding third molars. It can be associated with syndrome or nonsyndromic condition accompanied with other developmental dental anomalies like microdontia. Bilateral occurrence of hypodontia alongside microdontia is a rare condition. This case report presents a rare occurrence of bilateral hypodontia and microdontia in a nonsyndromic patient. Owing to its nonsyndromic background, other parameters like developmental delay, height, weight and body mass index are used as the predicting factors for the occurrence of hypodontia. Developmental milestones are one of the important factors in deriving the treatment plan. Treatment options in this case were directed at delaying the treatment until the eruption of the permanent successor teeth followed by the use of mini-implants for hypodontia, porcelain jacket crown for microdontia and the orthodontic treatment for final space closure.
Stickler syndrome or hereditary progressive arthro-ophthalmopathy is an autosomal dominant condition characterized by ocular manifestations, arthritic changes, orofacial features, and deafness, in variable degrees. This is a case report of a 48-day-old infant who presented with severe feeding difficulty. It also details the clinical, diagnostic features, and management of an infant with Stickler syndrome.
Apert syndrome is another genetically inherited syndrome characterized by craniosynostosis (premature fusion of coronal sutures) resulting skull and facial deformities and syndactyly. The syndrome was first described in 1906 by French physician Eugene Apert when he described nine people with similar facial and extremity characteristics. In 1906, Eugène Apert, a French physician, described nine people sharing similar attributes and characteristics.
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