A longitudinal evaluation of craniofacial growth in a patient with Kabuki make-up syndrome: a case report.

The purpose of this investigation was to evaluate the craniofacial growth of a patient diagnosed with Kabuki make-up syndrome (KMS). Craniofacial growth was assessed by analysing lateral cephalometric radiographs with an interval of 12-15 months. They were taken from 6 years 9 months to 14 years 2 months. Angular and linear measurement analyses of the craniofacial complex showed a hypoplastic maxilla and a constricted maxillary basal arch width. The mandibular size was relatively large and had started to increase from 13 years 4 months. This resulted in a prognathic face caused by forward growth of the mandible and insufficient growth of the maxilla. The skeletal pattern was Class III. Open bite morphology with a steep mandibular plane (SN-MP), a relatively short ramus, and a large gonial angle were also observed. In this subject, the facial dysmorphism found in the maxilla and mandible may have been influenced by several factors. Connective tissue disorder, macroglossia, lower tongue posture, and tongue thrust swallowing have been identified as possible aetiological factors that may determine dysmorphism in the craniofacial complex in this KMS patient.

Expression and localization of MGP in rat tooth cementum.

Tooth cementum, a calcified hard tissue covering the root surfaces, is an important component connecting the teeth to the collagenous fibres of the periodontal ligament. Although the overall composition of cementum may closely resemble that of bone, each part has not been fully characterized. Here, the localization of the matrix gamma-carboxyglutamic acid (Gla) protein (MGP), one of the major Gla-containing proteins in the body, in cementum was investigated using immunohistochemistry and in situ hybridization. (1) Strong MGP antigenicity was observed in the acellular cementum, but was only moderate in the cellular cementum; (2) polygonal periodontal ligament cells facing the acellular cementum and the uncalcified cellular cementum expressed MGP mRNA, indicating that these cells produced MGP and deposited it on the cementum; (3) MGP accumulated at the junction between the uncalcified and calcified cellular cementum; and (4) the distribution pattern of MGP antigenicity resembled that of osteopontin. As one function of MGP could be as a negative regulator for mineral apposition, the expression of MGP in the cells adjacent to the cementum may be important to prevent hyperapposition of minerals.

Force-induced rapid changes in cell fate at midpalatal suture cartilage of growing rats.

The application of expansional force induces replacement of the cartilaginous tissue with bone at the midpalatal suture of growing rats. We examined the early cellular events evoked by force by analyzing the expression of proliferating cell nuclear antigen (PCNA), an operational marker of cell proliferation, and of several bone matrix proteins. A rectangular orthodontic appliance was set between the right and left upper molars of four-week-old rats, with 50 g of initial expansional force. Two days after application of the force, the pre-existing cartilage was separated laterally. Mesenchymal cells with stretched shapes were arranged parallel to the expansional force and filled the center of the suture. Only a few of these stretched cells exhibited nuclear accumulation of PCNA. In contrast, many polygonal mesenchymal cells distributed along the inner lateral side of the cartilaginous tissue exhibited strong immunoreactivity for PCNA. Localization of alkaline phosphatase activity overlapped into this proliferating cell zone. Nascent extracellular matrix under the proliferating cells was positive for osteocalcin, indicating commencement of active bone formation. These findings indicated that, among mesenchymal cells subjected to expansional forces, only cells located on the inner side of the cartilaginous tissue proliferate and differentiate into osteoblasts. In agreement with rapid bone growth progression, apoptosis was also observed in the zone of proliferating cells, as measured by TdT-mediated dUTP-biotin nick end labeling (TUNEL) assays.