Genetic and molecular studies in humans mice indicate that Runx2 (Cbfa1) is a critical transcriptional regulator of bone tooth formation. Heterozygous mutations cause cleidocranial dysplasia (CCD), an inherited disorder characterized by skeletal defects, supernumerary teeth, delayed eruption. Mice lacking the gene die at birth lack development. Our extended phenotypic mutants showed developing teeth fail to advance beyond bud stage mandibular molar organs were more severely affected than...

Abstract Pathology in the craniocervical junction is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze prevalence and natural course anomalies patients with OI during growth. In one-center retrospective study, we analyzed lateral skull radiographs midsagittal magnetic resonance images 76 either type I, III, or IV OI. The material included longitudinal series 31 patients. total, 150 patient taken at ages 0 39 years were compared age-matched control data....

Abstract Osteogenesis imperfecta (OI) is a heterogeneous group of connective tissue diseases that mainly manifest as bone fragility and skeletal deformity. In most families it segregates dominant trait results from mutations in type I collagen genes. this study we analyzed the size form bony structures heads 59 consecutive patients with OI types I, III, or IV (Sillence classification), using lateral radiographs. Paired controls were matched for gender age. The purpose was to obtain baseline...

Object Osteogenesis imperfecta (OI), which usually results from mutations in type I collagen genes, causes bone fragility and deformities. The head is often abnormally shaped, changes skull base anatomy the form of basilar impression invagination have been reported. authors analyzed on standardized lateral cephalograms 54 patients with OI (Types I, III, IV) 108 control volunteers. They were surprised to find that previously used diagnostic measures for abnormality exceeded 6.5 7.4% controls,...

Abstract Cranial base abnormalities are an important complication of osteogenesis imperfecta (OI), a hereditary bone fragility disorder that in most patients is caused by mutations affecting collagen type I. To elucidate which clinical characteristics associated with the occurrence cranial OI, we compared cephalometric results 187 OI (median age 12.0 years, range 3.4 to 47 years; 96 female) those 191 healthy subjects and related findings descriptors disease. Overall, 41 (22%) had at least...

Joint hypermobility is a common clinical characteristic of patients with Osteogenesis imperfecta (OI), disorder serious comorbidities scoliosis and cranial base anomalies. This study aimed at evaluating how prevalent joint in paediatric OI patients, to find out whether it serves as potential predictive marker the different spinal complications; craniovertebral anomalies (basilar impression basilar invagination). In this cross-sectional one-center we analysed retrospectively patient records...

Abstract We describe results from a mutational analysis of the region dentin sialophosphoprotein (DSPP) gene encoding phosphoprotein (DPP) in 12 families with dominantly inherited diseases. In eight (five mutations N-terminal third DPP), clinical and radiologic features were uniform compatible dysplasia type II (DD-II) major signs deciduous dentition. other (four more C-terminal part), permanent teeth also affected, diseases could be classified as variants dentinogenesis imperfecta....

The aim of this study was to investigate if a developmental enamel defect known as Molar-Incisor Hypomineralization (MIH) is associated with dental caries. Socioeconomic status (SES) examined confounding factor between caries and MIH. In cross-sectional study, 636 children, aged 8 13 years, from three towns (two rural areas one urban area) in Finland were for MIH line the criteria European Academy Paediatric Dentistry. Caries permanent teeth recorded decayed, missing filled (DMFT)....

Persisting fatigue has been reported to be a common complaint by individuals with connective tissue disorders, including Osteogenesis imperfecta (OI). This controlled study evaluated in an adult OI population the subjective experience of fatigue, affecting daily life. Sleep disturbances and chronic pain were examined as hypothesized underlying factors. cross-sectional analyzed answers 56 patients matched healthy controls questionnaire, designed evaluate levels experienced bodily pain, well...

Cranial base pathology is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze whether bisphosphonate treatment, used improve bone strength, could also prevent the development craniocervical junction (basilar impression, basilar invagination, or platybasia) in children with OI.In this single-center retrospective study authors analyzed skull morphology from lateral radiographs and midsagittal MR images (total 94 images), obtained between ages 0 25 years 39...

Estimation of an individual's age has important applications in forensics. In young individuals, it often relies on separate evaluations permanent teeth (PT) and third molars (TM) development. Here, we analysed the prediction performance combined information from PT TM unusual sample healthy Somalis, born living Finland. development was staged according to Demirjian et al. (Hum Biol, 1973) Köhler (Ann Anat, 1994), using panoramic radiographs 803 subjects (397 males, 406 females) aged 3–23...

Structured Abstract Authors – Arponen H, Elf Evälahti M, Waltimo‐Sirén J Objective To explore the reliability of identification anatomic landmarks on lateral skull radiographs young unaffected individuals that has conventionally been used to diagnose pathologic relationships in craniovertebral junction. Material and Methods From Helsinki longitudinal growth study, 20 randomly selected were analyzed re‐analyzed by two examiners. Both located seven cephalometric based which five measurements...

Turner syndrome (TS) that is due to a total or partial lack of an X chromosome affects about 1 in 2000 girls. The characterized by short stature and gonadal dysgenesis. Its documented craniofacial features include retrognathic jaws, mandible, large cranial base angle. Our aim was find out whether the also has effect on pharyngeal airway space. We retrospectively analysed lateral cephalograms 35 TS subjects whose age ranged from 6.5 21 years healthy female controls matched for age. On those,...

Facial and calvarial bones form intramembranously without a cartilagenous model; however, cultured chick mesenchyme cells may differentiate into both osteoblasts chondroblasts and, in rodents, small cartilages occasionally at the sutures vivo. Therefore, we wanted to investigate what factors regulate normal differentiation of mesenchymal directly osteoblasts. In embryonic mouse heads tissue explants, analyzed expression selected transcription extracellular matrix molecules associated with...

Patients with Osteogenesis imperfecta (OI) suffer from increased bone fracture tendency generally caused by a mutation in genes coding for type I collagen. OI is also characterized numerous co-morbidities, and recent data questionnaire studies suggest that these may include risk sleep apnea, finding lacks clinical evidence cohort studies. In this cross-sectional study, 25 adults underwent otorhinolaryngology examination as well overnight polysomnography to address the question. The...

In children and adolescents, cone-beam computed tomography (CBCT) is frequently used for localization of unerupted or impacted teeth in the anterior maxilla. CBCT causes a higher radiation dose than conventional intraoral panoramic imaging. The objective was to analyze location canines three-dimensional coordinate thereby optimize field-of-view (FOV), reduction.Location 50 maxillary under 17 years retrospectively evaluated from scans. minimum maximum distances any part right- left-side three...

Objectives:Numbers of dental panoramic tomographs (DPTs) and lateral cephalometric radiographs (LCRs) outweigh other radiographic examinations in 7- to 12-year-old Finns. Orthodontists general practitioners (GPs) involved orthodontics hold therefore the highest responsibility exposure children ionising radiation with its risks. Against this background, lack reports on quality orthodontic radiography is surprising. The purpose our study was shed some light draw awareness community subject by...

Objectives: Children are especially vulnerable to harmful effects of ionizing radiation. Cutting down the dimensions X-ray beam is most effective way reduce patient dose. We evaluated appropriateness field-size in frequent radiographs, dental panoramic tomographs (DPTs) and lateral cephalometric radiographs (LCRs) among 7- 12-year-olds.

Abstract Background Cone-beam Computed Tomography (CBCT) is widely used for preoperative 3D imaging of lower third molars. Hence, this indication, the present study aimed to define minimum field-of-view (FOV) size and its optimum placement, decrease radiation exposure, highlight need computer-assisted FOV centering technique dental CBCT devices. To facilitate proper placement image field, second molar was chosen as reference. Methods The retrospective included 50 CBCT-scans 46 patients with...

Dental panoramic tomography is the most frequent examination among 7-12-year olds, according to Radiation Safety and Nuclear Authority of Finland. At those ages, dental tomographs (DPTs) are mostly obtained for orthodontic reasons. Children's dose reduction by trimming field size area interest important because their high radiosensitivity. Yet, majority DPTs in this age group still taken using an adult programme never a segmented programme. The purpose present study was raise awareness staff...