88 resultados para osteogenesis imperfecta

em Repositório Institucional UNESP - Universidade Estadual Paulista "Julio de Mesquita Filho"


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The fixation and the bone ingrowth at the interface of porous cylindrical implants (total porosity of 37% and average pores diameter of 480 mu m) were compared in vivo to rough cylindrical implants (R-a = 5.3 mu m), both of commercially pure titanium, made by powder metallurgy. The implants were inserted into the tibias of 20 rabbits and the animals were sacrificed 4 and 8 weeks after surgery. The percentage of bone-implant contact observed in porous implant was significantly larger than in the rough ones for all of sacrifice periods, respectively, 57% vs. 46% after 4 weeks, and 59% vs. 50% after 8 weeks. The mechanical tests showed a significant increase in the shear strength of the porous implants for the two analyzed periods, 4 and 8 weeks (14 and 20 MPa), when compared with rough ones (4 and 13 MPa). These results suggest that porous implants improve the contact at the implant-bone interface and increase the fixation to the bone, improving the osseointegration. Thus, the porous implant might be an alternative to dental implant in less favorable conditions, and appear to be better fixed to bone, offering promising alternatives.

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Amelogenesis imperfecta (AI) is a collective term used to describe phenotypically diverse forms of defective tooth enamel development. AI has been reported to exhibit a variety of inheritance patterns, and several loci have been identified that are associated with AI. We have performed a genome-wide scan in a large Brazilian family segregating an autosomal dominant form of AI and mapped a novel locus to 8q24.3. A maximum multipoint LOD score of 7.5 was obtained at marker D8S2334 (146,101,309 bp). The disease locus lies in a 1.9 cM (2.1 Mb) region according to the Rutgers Combined Linkage-Physical map, between a VNTR marker (at 143,988,705 bp) and the telomere (146,274,826 bp). Ten candidate genes were identified based on gene ontology and microarray-facilitated gene selection using the expression of murine orthologues in dental tissue, and examined for the presence of a mutation. However, no causative mutation was identified.

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Aim: To evaluate the healing at implants with a moderately rough surface placed and stabilized in recipient sites of dimensions deeper and larger than that of the implants to avoid any contact between parent bone and the implant.Material & methods: In six Labrador dogs, premolars and first molars were extracted bilaterally in the mandible. After 3 months of healing, mucoperiosteal full-thickness flaps were elevated and the premolar area of the alveolar bony crest was selected. Three recipient sites were prepared to place three implants. One implant was used as control. The other two were placed in recipient sites which left a circumferentially and periapical prepared defect of 0.7 mm (small) and 1.2 mm (large), respectively. All implants were stabilized with passive fixation plates to maintain the implants stable and without any contact with the implant bed. After 3 months of submerged healing, the animals were sacrificed. Ground sections were prepared and analyzed histomorphometrically.Results: The BIC% was 5.3% and 0.3% for implants placed in small and large defect sites, respectively, whereas it was 46.1% for control implants. The differences were statistically significant. The width of the residual defects was 0.4 and 0.5 mm at the small and large defects, respectively. An approximately 0.09 mm layer of dense connective tissue (DCT) rich in fibers and fibroblast-like cells was observed adherent to the implant surfaces. The percentage of implant surface covered by DCT was 92.8% and 95.6% at the small and large defects, respectively.Conclusion: Osseointegration was observed at the test sites, and the dimensions of the defects influenced the outcomes. However, the degree of osseointegration at both small and large defects was very low compared with the control sites.

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In this article, we describe a midsymphyseal distraction osteogenesis treatment with a novel dentally supported appliance. This approach differs from that used in previous reports because the incisors were allowed to move during the distraction procedure. This report shows that midsymphyseal distraction osteogenesis can be used to expand both arches to produce a wider smile. Borderline cases can be treated with this technique without the compromising effects commonly observed with conventional therapy. (Am J Orthod Dentofacial Orthop 2009;135:530-5)

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Amelogenesis imperfecta (AI) is a genetically heterogeneous group of diseases that result in defective development of tooth enamel. Mutations in several enamel proteins and proteinases have been associated with AI. The object of this study was to evaluate evidence of etiology for the six major candidate gene loci in two Brazilian families with AI. Genomic DMA was obtained from family members and all exons and exon-intron boundaries of the ENAM, AMBN, AMELX, MMP20, KLK4 and Amelotin gene were amplified and sequenced. Each family was also evaluated for linkage to chromosome regions known to contain genes important in enamel development. The present study indicates that the AI in these two families is not caused by any of the known loci for AI or any of the major candidate genes proposed in the literature. These findings indicate extensive genetic heterogeneity for non-syndromic AI.

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AIM: The aim of this report is to describe the restorative treatment of an 18-year-old patient diagnosed with autosomal recessive hypocalcified-hypoplastic amelogenesis imperfecta (AI). BACKGROUND: Esthetic and functional rehabilitations in AI cases are challenging and should consider individual aspects, such as age, socioeconomic status, AI type, and intraoral condition. REPORT: AI was diagnosed in an 18-year-old patient. SUMMARY: Considering the short length of crowns and roots, patient life expectancy, minimal invasiveness, amount of treatment time required, and lower costs, oral rehabilitation with overdentures provided an adequate functional and esthetic rehabilitation of the patient. CLINICAL SIGNIFICANCE: This report demonstrated overdentures to be a viable, relatively inexpensive, and non-invasive treatment choice of a patient with AI with concerns about treatment longevity, invasiveness, cost, esthetics, and long-term maintenance.

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Ankylosis of the temporomandibular joint in children is one the most difficult and complex conditions managed by oral and maxillofacial surgeons, and often leads to some facial deformity. Distraction osteogenesis of the mandible provides an excellent treatment for mandibular airway obstruction in children who do not respond to conservative measures, and allows for early removal of the tracheostomy. We report the case of a 1-year-old boy with severe micrognathia and temporomandibular ankylosis who was dependent on a tracheostomy; he was treated with piezosurgery and mandibular advancement by distraction osteogenesis. © 2010 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

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This study investigated the effects of the morphology and physicochemical properties of calcium phosphate (CaP) nanoparticles on osteogenesis. Two types of CaP nanoparticles were compared, namely amorphous calcium phosphate (ACP) nano-spheres (diameter: 9-13 nm) and poorly crystalline apatite (PCA) nano-needles (30-50 nm x 2-4 nm) that closely resemble bone apatite. CaP particles were spin-coated onto titanium discs and implants; they were evaluated in cultured mouse calvarial osteoblasts, as well as after implantation in rabbit femurs. A significant dependence of CaP coatings was observed in osteoblast-related gene expression (Runx2, Col1a1 and Spp1). Specifically, the PCA group presented an up-regulation of the osteospecific genes, while the ACP group suppressed the Runx2 and Col1a1 expression when compared to blank titanium substrates. Both the ACP and PCA groups presented a more than three-fold increase of calcium deposition, as suggested by Alizarin red staining. The removal torque results implied a slight tendency in favour of the PCA group. Different forms of CaP nanostructures presented different biologic differences; the obtained information can be used to optimize surface coatings on biomaterials. © 2013 IOP Publishing Ltd.

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Purpose: The purpose of this study was to assess restorative treatment outcomes in the mixed dentition of amelogenesis imperfecta (AI) patients and determine the postrehabilitation oral health status and satisfaction of the patients. Methods: Clinical and radiographic examinations were performed on eight AI patients, who had 74 restorations placed in permanent incisors and molars, to allow evaluation of the integrity of the restorations and periodontal status post-treatment. Subjects completed a survey regarding esthetics, function, and sensitivity. Results: Among the 74 restorations evaluated, seven were lost; of the remaining restorations, 31 were posterior, and 36 were anterior. Ten were rated clinically unacceptable. Teeth with stainless steel crowns had a moderate gingival index (mean=2.3) and plaque index (mean=2.0) scores. Widening of the periodontal ligament and pulp canal obliteration were common radiographic findings. Subject's recall of satisfaction regarding esthetics (P=.002) and sensitivity (brushing-P=.03; eating-P=.01) showed a statically significant difference before and after treatment. Conclusions: During mixed dentition, teeth with amelogenesis imperfecta may be restored with conventional treatment modalities. Direct restorations should be considered interim with multiple repairs anticipated. Post-treatment, gingival inflammation and plaque accumulation were observed. Subjects were satisfied with their appearance and reported a decrease of hypersensitivity. © 2013 Publishing Technology.

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Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

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Amelogenesis imperfecta is a hereditary condition that can alter the thickness, color, and shape of tooth enamel. Recent adhesive materials and techniques have provided less invasive treatment options. This case report presents the treatment of a patient whose anterior teeth had color alterations, white spots, pits, and shape defects. Using a more conservative technique, the mandibular and maxillary anterior teeth were restored using veneer direct composite restorations. After 6 years, the restorations demonstrated no deterioration, and no pathology was seen in association with the rehabilitation.

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Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)

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Case Report. An 8-year-old girl with amelogenesis imperfecta (AI) reported unsatisfactory aesthetics, difficulty in mastication, and dental hypersensitivity. The intraoral examination observed mixed dentition, malocclusion in anteroposterior relationships, anterior open bite, and dental asymmetry. A hypoplastic form of AI was diagnosed in the permanent dentition. A multidisciplinary planning was performed and divided into preventive, orthopedic, and rehabilitation stages. Initially, preventive treatment was implemented, with fluoride varnish applications, in order to protect the fragile enamel and reduce the dental sensitivity. In the second stage, the patient received an interceptive orthopedic treatment to improve cross-relationship of the arches during six months. Finally, the rehabilitation treatment was executed to establish the vertical dimension. In the posterior teeth, indirect composite resin crowns were performed with minimally invasive dental preparation. Direct composite resin restorations were used to improve the appearance of anterior teeth. Follow-Up. The follow-up was carried out after 3, 6, 12, and 18 months. After 18 months of follow-up, The restoration of integrity, oral hygiene, and patient satisfaction were observed . Conclusion. Successful reduction of the dental hypersensitivity and improvement of the aesthetic and functional aspects as well as quality of life were observed.

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Endochondral calcification involves the participation of matrix vesicles (MVs), but it remains unclear whether calcification ectopically induced by implants of demineralized bone matrix also proceeds via MVs. Ectopic bone formation was induced by implanting rat demineralized diaphyseal bone matrix into the dorsal subcutaneous tissue of Wistar rats and was examined histologically and biochemically. Budding of MVs from chondrocytes was observed to serve as nucleation sites for mineralization during induced ectopic osteogenesis, presenting a diameter with Gaussian distribution with a median of 306 ± 103 nm. While the role of tissue-nonspecific alkaline phosphatase (TNAP) during mineralization involves hydrolysis of inorganic pyrophosphate (PPi), it is unclear how the microenvironment of MV may affect the ability of TNAP to hydrolyze the variety of substrates present at sites of mineralization. We show that the implants contain high levels of TNAP capable of hydrolyzing p-nitrophenylphosphate (pNPP), ATP and PPi. The catalytic properties of glycosyl phosphatidylinositol-anchored, polidocanol-solubilized and phosphatidylinositol-specific phospholipase C-released TNAP were compared using pNPP, ATP and PPi as substrates. While the enzymatic efficiency (k cat/Km) remained comparable between polidocanol-solubilized and membrane-bound TNAP for all three substrates, the k cat/Km for the phosphatidylinositol-specific phospholipase C-solubilized enzyme increased approximately 108-, 56-, and 556-fold for pNPP, ATP and PPi, respectively, compared to the membrane-bound enzyme. Our data are consistent with the involvement of MVs during ectopic calcification and also suggest that the location of TNAP on the membrane of MVs may play a role in determining substrate selectivity in this micro-compartment.