Periodontal Ehlers-Danlos syndrome (pEDS) is an autosomal-dominant disorder characterized by early-onset periodontitis leading to premature loss of teeth, joint hypermobility, and mild skin findings. A locus was mapped to an approximately 5.8 Mb region at 12p13.1 but no candidate gene was identified. In an international consortium we recruited 19 independent families comprising 107 individuals with pEDS to identify the locus, characterize the clinical details in those with defined genetic causes, and try to understand the physiological basis of the condition. In 17 of these families, we identified heterozygous missense or in-frame insertion/deletion mutations in C1R (15 families) or C1S (2 families), contiguous genes in the mapped locus that encode subunits C1r and C1s of the first component of the classical complement pathway. These two proteins form a heterotetramer that then combines with six C1q subunits. Pathogenic variants involve the subunit interfaces or inter-domain hinges of C1r and C1s and are associated with intracellular retention and mild endoplasmic reticulum enlargement. Clinical features of affected individuals in these families include rapidly progressing periodontitis with onset in the teens or childhood, a previously unrecognized lack of attached gingiva, pretibial hyperpigmentation, skin and vascular fragility, easy bruising, and variable musculoskeletal symptoms. Our findings open a connection between the inflammatory classical complement pathway and connective tissue homeostasis.

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ObjectivesComputer-aided design (CAD)/computer-aided manufacturing (CAM) dentures are assumed to have more favourable material properties than conventionally fabricated dentures, among them a lower methacrylate monomer release. The aim of this study was to test this hypothesis.Materials and methodsCAD/CAM dentures were generated from ten different master casts by using four different CAD/CAM systems. Conventional, heat-polymerised dentures served as control group. Denture weight and volume were measured; the density was calculated, and the denture surface area was assessed digitally. The monomer release after 7 days of water storage was measured by high-performance liquid chromatography.ResultsWhole You Nexteeth and Wieland Digital Dentures had significantly lower mean volume and weight than conventional dentures. Baltic Denture System and Whole You Nexteeth had a significantly increased density. Baltic Denture System had a significantly smaller surface area. None of the CAD/CAM dentures released significantly less monomer than the control group.ConclusionsAll tested dentures released very low amounts of methacrylate monomer, but not significantly less than conventional dentures. A statistically significant difference might nevertheless exist in comparison to other, less recommendable denture base materials, such as the frequently used autopolymerising resins.Clinical relevanceCAD/CAM denture fabrication has numerous advantages. It enables the fabrication of dentures with lower resin volume and lower denture weight. Both could increase the patient comfort. Dentures with higher density might exhibit more favourable mechanical properties. The hypothesis that CAD/CAM dentures release less monomer than conventional dentures could, however, not be verified.

Objectives Resin polymerisation shrinkage reduces the congruence of the denture base with denture-bearing tissues and thereby decreases the retention of conventionally fabricated dentures. CAD/CAM denture manufacturing is a subtractive process, and polymerisation shrinkage is not an issue anymore. Therefore, CAD/CAM dentures are assumed to show a higher denture base congruence than conventionally fabricated dentures. It has been the aim of this study to test this hypothesis. Materials and methods CAD/CAM dentures provided by four different manufacturers (AvaDent, Merz Dental, Whole You, Wieland/Ivoclar) were generated from ten different master casts. Ten conventional dentures (pack and press, long-term heat polymerisation) made from the same master casts served as control group. The master casts and all denture bases were scanned and matched digitally. The absolute incongruences were measured using a 2-mm mesh. Results Conventionally fabricated dentures showed a mean deviation of 0.105 mm, SD = 0.019 from the master cast. All CAD/ CAM dentures showed lower mean incongruences. From all CAD/CAM dentures, AvaDent Digital Dentures showed the highest congruence with the master cast surface with a mean deviation of 0.058 mm, SD = 0.005. Wieland Digital Dentures showed a mean deviation of 0.068 mm, SD = 0.005, Whole You Nexteeth prostheses showed a mean deviation of 0.074 mm, SD = 0.011 and Baltic Denture System prostheses showed a mean deviation of 0.086 mm, SD = 0.012. Conclusions CAD/CAM produces dentures with better fit than conventional dentures. Clinical Relevance The present study explains the clinically observed enhanced retention and lower traumatic ulcer-frequency in CAD/CAM dentures.

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