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Introduction and Objective: Dyskeratosis congenita (DC) also known as Zinsser-Engman- Cole Syndrome is a rare inherited disorder with a prevalence of less than one per million. Zinsser et al. described an inherited variant of ectodermal dysplasia that affected skin, nails and mucous membranes in early 1900s.The syndrome eventually came to be known as DC and is classified as one of the inherited bone marrow failure syndromes (IBMFS). DC is the association of three clinical features: dystrophic nails, oral leukoplakia (white spots on the tongue and oral mucosa) and abnormal skin pigmentation. Case report and Conclusion: This case report describes a dental management of a case of DC. Fluctuating vital and blood parameters and deteriorating overall health status were major challenges delivering dental treatment. Dental extractions of this patient were done while maintaining blood parameters by blood and platelet transfusion.

Aim Physical properties of obturating materials have a direct effect on the quality of obturation; less viscous material will tend to extrude beyond the apex. We hypothesize that the use of absorbable gelatin sponge (AGS) can prevent the extrusion of these materials. This study aimed to evaluate the quality of obturation in root canals filled with iodoform‐calcium hydroxide (Metapex) premixed paste and Metapex with AGS (MAGS) in primary molars. Methods This in vivo study comprised 60 primary mandibular molars which after instrumentation were divided into 2 groups according to the obturation material used, Metapex and MAGS, followed by radiographic evaluation to assess the quality of the obturation. The χ2‐test was applied for statistical analysis. Results There was a statistically significant difference between Metapex and MAGS groups in achieving optimum obturation (P < .001). Metapex and MAGS exhibited 86.65% and 38.88% optimum obturation respectively. The overfilling of root canals effectively reduced from 38.88% to 4.44%. Conclusion The addition of AGS to Metapex causes a “tamponade effect”, which reduces the tendency of Metapex to extrude beyond the root apex. Root canal obturation using a modified filling paste like MAGS is effective in obtaining optimum obturation in primary teeth.

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