Hyper-IgE syndrome with recurrent infections--an autosomal dominant multisystem disorder.
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BACKGROUND: The hyper-IgE syndrome with recurrent infections is a rare immunodeficiency characterized by recurrent skin and pulmonary abscesses and extremely elevated levels of IgE in serum. Associated facial and skeletal features have been recognized, but their frequency is unknown, and the genetic basis of the hyper-IgE syndrome is poorly understood. METHODS: We studied 30 patients with the hyper-IgE syndrome and 70 of their relatives. We took histories, reviewed records, performed physical and dental examinations, took anthropometric measurements, and conducted laboratory studies. RESULTS: Nonimmunologic features of the hyper-IgE syndrome were present in all patients older than eight years. Seventy-two percent had the previously unrecognized feature of failure or delay of shedding of the primary teeth owing to lack of root resorption. Common findings among patients were recurrent fractures (in 57 percent of patients), hyperextensible joints (in 68 percent), and scoliosis (in 76 percent of patients 16 years of age or older). The classic triad of abscesses, pneumonia, and an elevated IgE level was identified in 77 percent of all patients and in 85 percent of those older than eight. In 6 of 23 adults (26 percent), IgE levels declined over time and came closer to or fell within the normal range. Autosomal dominant transmission of the hyper-IgE syndrome was found, but with variable expressivity. Of the 27 relatives at risk for inheriting the hyper-IgE syndrome, 10 were fully affected, 11 were unaffected, and 6 had combinations of mild immunologic, dental, and skeletal features of the hyper-IgE syndrome. CONCLUSIONS: The hyper-IgE syndrome is a multisystem disorder that affects the dentition, the skeleton, connective tissue, and the immune system. It is inherited as a single-locus autosomal dominant trait with variable expressivity. (+info)
Immunolocalization of vacuolar-type H+-ATPase, cathepsin K, matrix metalloproteinase-9, and receptor activator of NFkappaB ligand in odontoclasts during physiological root resorption of human deciduous teeth.
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To investigate the cellular mechanisms of physiological root resorption in human deciduous teeth, the authors examined the immunocytochemical localization of vacuolar-type H+-ATPase, a lysosomal cysteine proteinase, cathepsin K, matrix metalloproteinase-9 (MMP-9), and receptor activator of NFKB ligand (RANKL) in odontoclasts. H+-ATPase, cathepsin K, and MMP-9 are the most important enzymes for decalcification of apatite crystals and degradation of type-I collagen. In addition, RANKL is one of the key regulatory molecules in osteoclast formation and functions. Odontoclasts developed extensive ruffled borders and clear zones apposed to the resorbing root dentine surfaces. On immunoelectron microscopy, the expression of vacuolar-type H+-ATPase was detected along the limiting membranes of pale vacuoles and the ruffled border membranes of odontoclasts. Cathepsin K in odontoclasts was localized within pale vacuoles, lysosomes, the extracellular canals of ruffled borders, and the underlying resorbing dentine surfaces. MMP-9 localization in odontoclasts was similar to those of cathepsin K. RANKL was detected in both mononuclear stromal cells and odontoclasts located on resorbing dentine surfaces. These results suggest that (1) odontoclasts are directly involved in decalcification of apatite crystals by active extrusion of proton ions mediated by H+-ATPase and (2) extracellular degradation of dentine type-I collagen by both cathepsin K and MMP-9, and (3) odontoclast differentiation and activity are regulated, at least in part, by RANKL, possibly produced by mononuclear stromal cells and odontoclasts themselves in the resorbing tissues. Thus, the cellular mechanisms of physiological root resorption appear to be quite similar to those of osteoclastic bone resorption. (+info)
In vivo killing of Porphyromonas gingivalis by toluidine blue-mediated photosensitization in an animal model.
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Porphyromonas gingivalis is one of the major causative organisms of periodontitis and has been shown to be susceptible to toluidine blue-mediated photosensitization in vitro. The aims of the present study were to determine whether this technique could be used to kill the organism in the oral cavities of rats and whether this would result in a reduction in the alveolar bone loss characteristic of periodontitis. The maxillary molars of rats were inoculated with P. gingivalis and exposed to up to 48 J of 630-nm laser light in the presence of toluidine blue. The number of surviving bacteria was then determined, and the periodontal structures were examined for evidence of any damage. When toluidine blue was used together with laser light there was a significant reduction in the number of viable P. gingivalis organisms. No viable bacteria could be detected when 1 mg of toluidine blue per ml was used in conjunction with all light doses used. On histological examination, no adverse effect of photosensitization on the adjacent tissues was observed. In a further group of animals, after time was allowed for the disease to develop in controls, the rats were killed and the level of maxillary molar alveolar bone was assessed. The bone loss in the animals treated with light and toluidine blue was found to be significantly less than that in the control groups. The results of this study show that toluidine blue-mediated lethal photosensitization of P. gingivalis is possible in vivo and that this results in decreased bone loss. These findings suggest that photodynamic therapy may be useful as an alternative approach for the antimicrobial treatment of periodontitis. (+info)
Cementum-like tissue deposition on the resorbed enamel surface of human deciduous teeth prior to shedding.
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Prior to the shedding of human deciduous teeth, odontoclastic resorption takes place at the pulpal surface of the coronal dentin, and this resorption occasionally extends coronally from the dentinoenamel junction into the enamel. After the end of resorption, however, the resorbed enamel surface is repaired by the deposition of a cementum-like tissue. Using this phenomenon as an observation model, in this study we examined the sequence of cellular and extracellular/matrix events involved in the enamel resorption repair by light and electron microscopy. As the odontoclast terminated its resorption activity, it detached from the resorbed enamel surface; thereafter, numerous mononuclear cells were observed along the resorbed enamel surface. Most of these mononuclear cells made close contact with the resorbed enamel surface, and coated pits or patches were observed on their plasma membrane facing this surface. Furthermore, they frequently contained thin needle- or plate-like enamel crystals in their cytoplasmic vacuoles as well as secondary lysozomes. Following the disappearance of these monononuclear cells, the resorbed enamel surface now displayed a thin coat of organic matrix. Ultrastructurally, this organic layer was composed of a reticular and/or granular organic matrix, but contained no collagen fibrils. Energy-dispersive X-ray microanalysis of this thin organic layer in undecalcified sections revealed small spectral peaks of Ca and P. Cementum-like tissue initially formed along this thin organic layer, increased in width, and appeared to undergo mineralization as time progressed. The results of our observations demonstrate that regardless of type of matrix of dental hard tissues, tooth repair may be coupled to tooth resorption, and suggest that mononuclear cells and an organic thin layer found on the previously resorbed enamel surface may play an important role in the repair process initiated after resorption of the enamel. (+info)
Adenomatoid odontogenic tumor--a rare cause of jaw swelling.
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Adenomatoid odontogenic tumor (AOT) is an uncommon tumor of odontogenic origin, characterized histologically by the formation of ductlike structures with amyloid-like deposits. Histogenesis of AOT is still uncertain and it is often considered as a hamartomatous lesion rather than a true neoplasm. AOT has a benign behavior and conservative surgical enucleation or curettage is sufficient. We report a case of AOT in a 15-year-old female who presented with left-sided jaw swelling with tooth resorption. Histopathology revealed intraosseus follicular variant of AOT. A brief review of literature is also discussed. (+info)
Odontoclastoma.
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Case report showing classical odontoclastoma along with in vivo illustrations of the affected tooth and intral oral periapical radiograph. (+info)
Analysis of the surface characteristics and mineralization status of feline teeth using scanning electron microscopy.
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External resorption of teeth by odontoclasts is a common condition of unknown origin affecting domestic cats. Odontoclastic resorptive lesions involve the enamel cementum junction (ECJ, cervix) and root surface, leading to extensive loss of enamel, dentine and cementum. This study was undertaken in order to determine whether features of the surface anatomy and mineralization of feline teeth could explain why odontoclastic resorptive lesions are so prevalent in this species. Backscattered electron scanning electron microscopy was used to study enamel, cementum and dentine in non-resorbed, undemineralized teeth from adult cats. Analysis of the ECJ revealed thin enamel and cementum and exposed dentine at this site. Furthermore, enamel mineralization decreased from the crown tip to the ECJ, and dentine mineralization was lowest at the ECJ and cervical root. Analysis of cementum revealed variations in the organization and composition of fibres between the cervical, mid- and apical root although no significant differences in mineralization of cementum were detected between different regions of the root. Reparative patches associated with resorption of cementum by odontoclasts and repair by cementoblasts were present on the root surface. In conclusion, results suggest that the ECJ and cervical dentine could be at a greater risk of destruction by odontoclasts compared with other regions of the tooth. The relationship of these features to the development and progression of resorption now requires further examination. (+info)
Odontoclastic resorptive lesions in a dog.
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We found odontoclastic resorptive lesions on premolars and molars in a 4- year-old miniature dachshund. The teeth had been extracted because the dentin was resorbed. In some teeth, the roots had been replaced by hard tissue, and so we amputated the crowns and curetted roots and alveolar bone. Histopathological examination revealed that the dentin was resorbed by odontoclasts and was replaced with bony tissue. Ten months later we found resorptive lesions in other teeth, and we treated them along with the first treatment. At the time of writing, since this is the first report of a dog with the same lesion in other teeth after the first treatment, we hope to establish better treatment and prevention methods. (+info)