Cornelia De Lange syndrome is a relatively uncommon, multiple congenital anomaly / mental retardation disorder of unknown etiology. Its incidence has been reported to vary from 1 : 30,000 to 1 : 50,000 of live births, without any known racial predilection. However, it has been considered to be due to a new dominant mutation. Main clinical features of this syndrome include growth retardation, developmental delay, hirsutism, structural limb abnormalities, mental retardation and facial growth discrepancies. Main causes of death in such patients include pneumonia along with cardiac, respiratory and GI abnormalities. (+info)
Third molar angulation during and after treatment of adolescent orthodontic patients.
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The purpose of this study was to analyse the effect of premolar extraction therapy on third molar angulation during active treatment, and to test the significance of such changes on subsequent impaction of the third molars. Lateral cephalograms made before (T1) and after (T2) treatment and at long-term follow-up (T3) of 157 patients treated non-extraction (non-ex) or with extraction of four premolars (ex), all accurately diagnosed for impaction versus eruption of at least one third molar at T3, were evaluated. Linear regression models demonstrated that the maxillary third molars uprighted more from T1 to T2 (P < 0.05) and were less distally angulated at T2 (P < 0.01) in the ex than in the non-ex patients. No such differences were detected in the mandible (P > 0.05). The regression models also showed similar uprighting of the maxillary and mandibular third molars from T1 to T2 and similar angulation of the maxillary third molars at T2 in those patients with subsequent eruption and impaction (P > 0.05), but more mesially angulated mandibular third molars at T2 in the impaction patients (P < 0.01). Chi square testing demonstrated a higher frequency of distal tipping of the maxillary third molars from T1 to T2 in the impaction patients (P < 0.01), while mesial tipping from T1 to T2 of the mandibular third molars occurred with similar frequency in the two patient groups (P > 0.05). Chi square analysis also showed a higher frequency of greater than 30 degree distal angulation as well as an amount mesial angulation of the maxillary third molars at T2 (P < 0.01), and a higher frequency of greater than 40 degree mesial angulation of the mandibular third molars at T2 (P < 0.01) in patients with impaction than in those with eruption. (+info)
CSF-1 regulation of osteoclastogenesis for tooth eruption.
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The dental follicle regulates the alveolar bone resorption needed for tooth eruption. In the rat first mandibular molar, a decrease in the expression of osteoprotegerin (OPG) in the dental follicle at day 3 enables the osteoclastogenesis needed for eruption to occur. Because colony-stimulating factor-1 (CSF-1) is maximally expressed in the dental follicle at day 3, it was hypothesized that CSF-1 down-regulates OPG gene expression in the dental follicle in vivo. To test this, we compared the expression of OPG in osteopetrotic toothless (tl/tl) rats deficient in CSF-1 with expression in their normal littermates for given ages. OPG gene expression was found to be higher in the dental follicle of the tl/tl mutants than in normals. Transfecting short interfering RNA specific for CSF-1 mRNA into dental follicle cells resulted in an up-regulation of OPG expression. Thus, these studies support our hypothesis that the down-regulation of OPG needed for tooth eruption is mediated by CSF-1. (+info)
Inflammatory dentigerous cysts of children treated by tooth extraction and decompression--report of four cases.
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Inflammatory dentigerous cysts are only found in the mixed dentition. The four cases presented here illustrate the uncomplicated behavior of these cysts when properly treated. By extracting the infected primary teeth, opening the cyst and ensuring continuous drainage, it is possible to achieve spontaneous eruption of the involved permanent teeth into the dental arch even if they are badly dislocated. Simultaneous with the eruption of the permanent teeth, ossification of the bony defect can take place. The reparatory process is completed in one to two years. (+info)
Developmental and functional significance of the CSF-1 proteoglycan chondroitin sulfate chain.
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The primary macrophage growth factor, colony-stimulating factor-1 (CSF-1), is homodimeric and exists in 3 biologically active isoforms: a membrane-spanning, cell-surface glycoprotein (csCSF-1) and secreted glycoprotein (sgCSF-1) and proteoglycan (spCSF-1) isoforms. To investigate the in vivo role of the chondroitin sulfate glycosaminoglycan (GAG) chain of spCSF-1, we created mice that exclusively express, in a normal tissue-specific and developmental manner, either the secreted precursor of spCSF-1 or the corresponding precursor in which the GAG addition site was mutated. The reproductive, hematopoietic tooth eruption and tissue macrophage defects of CSF-1-deficient, osteopetrotic Csf1(op)/Csf1(op) mice were corrected by transgenic expression of the precursors of either sgCSF-1 or spCSF-1. Furthermore, in contrast to the transgene encoding csCSF-1, both failed to completely correct growth retardation, suggesting a role for csCSF-1 in the regulation of body weight. However, spCSF-1, in contrast to sgCSF-1, completely resolved the osteopetrotic phenotype. Furthermore, in transgenic lines expressing different concentrations of sgCSF-1 or spCSF-1, spCSF-1 more efficiently corrected Csf1(op)/Csf1(op) defects of tooth eruption, eyelid opening, macrophage morphology, and B-cell deficiency than sgCSF-1. These results indicate an important role of the CSF-1 chondroitin sulfate proteoglycan in in vivo signaling by secreted CSF-1. (+info)
Malnutrition and dental caries: a review of the literature.
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Protein-energy malnutrition occurs when there are deficiencies in protein, energy foods or both, relative to a body's needs. This paper reviews the association of early childhood malnutrition with: (1) dental caries, (2) enamel hypoplasia, (3) salivary gland hypofunction, and (4) delayed eruption. Studies suggest that caries of the primary dentition is associated with early childhood malnutrition, though the effect on caries of the permanent dentition has essentially not been studied. Enamel hypoplasia, salivary glandular hypofunction and saliva compositional changes may be mechanisms through which malnutrition is associated with caries, while altered eruption timing may create a challenge in the analysis of age-specific caries rates. (+info)
Three-dimensional analysis of mandibular growth and tooth eruption.
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Normal and abnormal jaw growth and tooth eruption are topics of great importance for several dental and medical disciplines. Thus far, clinical studies on these topics have used two-dimensional (2D) radiographic techniques. The purpose of the present study was to analyse normal mandibular growth and tooth eruption in three dimensions based on computer tomography (CT) scans, extending the principles of mandibular growth analysis proposed by Bjork in 1969 from two to three dimensions. As longitudinal CT data from normal children are not available (for ethical reasons), CT data from children with Apert syndrome were employed, because it has been shown that the mandible in Apert syndrome is unaffected by the malformation, and these children often have several craniofacial CT scans performed during childhood for planning of cranial and midface surgery and for follow-up after surgery. A total of 49 datasets from ten children with Apert syndrome were available for study. The number of datasets from each individual ranged from three to seven. The first CT scan in each of the ten series was carried out before 1 year of age, and the ages for the 49 scans ranged from 1 week to 14.5 years. The mandible and the teeth were segmented and iso-surfaces generated. Landmarks were placed on the surface of the mandible, along the mandibular canals, the inner contour of the cortical plate at the lower border of the symphysis menti, and on the teeth. Superimposition of the mandibles in the longitudinal series was performed using the symphysis menti and the mandibular canals as suggested by Bjork. The study supported the findings of stability of the symphysis menti and the mandibular canals as seen in profile view previously reported by Bjork & Skieller in 1983. However, the mandibular canals were, actually, relocated laterally during growth. Furthermore, the position of tooth buds remained relatively stable inside the jaw until root formation started. Eruption paths of canines and premolars were vertical, whereas molars erupted in a lingual direction. The 3D method would seem to offer new insight into jaw growth and tooth eruption, but further studies are needed. (+info)
A new design of molar distalization appliance and its fabrication are described in this article. This case report illustrates distal movement of mandibular molars with negligible loss of anterior anchorage. This new vacuum-formed Essix appliance can be a reasonable alternative to conventional appliances. (+info)