Enamel and dental anomalies in latent‐transforming growth factor beta‐binding protein 3 mutant mice

Latent‐transforming growth factor beta‐binding protein 3 (LTBP‐3) is important for craniofacial morphogenesis and hard tissue mineralization, as it is essential for activation of transforming growth factor‐β (TGF‐β). To investigate the role of LTBP‐3 in tooth formation we performed micro‐computed tomography (micro‐CT), histology, and scanning electron microscopy analyses of adult Ltbp3‐/‐ mice. The Ltbp3‐/‐ mutants presented with unique craniofacial malformations and reductions in enamel formation that began at the matrix formation stage. Organization of maturation‐stage ameloblasts was severely disrupted. The lateral side of the incisor was affected most. Reduced enamel mineralization, modification of the enamel prism pattern, and enamel nodules were observed throughout the incisors, as revealed by scanning electron microscopy. Molar roots had internal irregular bulbous‐like formations. The cementum thickness was reduced, and microscopic dentinal tubules showed minor nanostructural changes. Thus, LTBP‐3 is required for ameloblast differentiation and for the formation of decussating enamel prisms, to prevent enamel nodule formation, and for proper root morphogenesis. Also, and consistent with the role of TGF‐β signaling during mineralization, almost all craniofacial bone components were affected in Ltbp3‐/‐ mice, especially those involving the upper jaw and snout. This mouse model demonstrates phenotypic overlap with Verloes Bourguignon syndrome, also caused by mutation of LTBP3, which is hallmarked by craniofacial anomalies and amelogenesis imperfecta phenotypes.     

Growth of Ceria Nano-Islands on a Stepped Au(788) Surface

The growth morphology and structure of ceria nano-islands on a stepped Au(788) surface has been investigated by scanning tunneling microscopy (STM) and low-energy electron diffraction (LEED). Within the concept of physical vapor deposition, different kinetic routes have been employed to design ceria-Au inverse model catalysts with different ceria nanoparticle shapes and arrangements. A two-dimensional superlattice of ceria nano-islands with a relatively narrow size distribution (5 ± 2 nm²) has been generated on the Au(788) surface by the postoxidation method. This reflects the periodic anisotropy of the template surface and has been ascribed to the pinning of ceria clusters and thus nucleation on the fcc domains of the herringbone reconstruction on the Au terraces. In contrast, the reactive evaporation method yields ceria islands elongated in [01-1] direction, i.e., parallel to the step edges, with high aspect ratios (~6). Diffusion along the Au step edges of ceria clusters and their limited step crossing in conjunction with a growth front perpendicular to the step edges is tentatively proposed to control the ceria growth under reactive evaporation conditions. Both deposition recipes generate two-dimensional islands of CeO₂(111)-type O–Ce–O single and double trilayer structures for submonolayer coverages.     

State-of-the-art nanopathological diagnostics

The nanopathological diagnostics (ND) is an ultra-specialized branch of pathological anatomy aimed to identify the nanoparticles of metallic, semimetallic, or nonmetallic elements in the inorganic particulate matter present inside pathological tissues, even on the nanometer scale. ND exploits an environmental scanning electron microscope, connected to an X-ray microprobe mounted on an energy-dispersive spectrometer. The searching of nanoparticles can be performed on paraffin-embedded material, omitting emissions of black overlay and plating procedures. The technique is highly sensitive and specific, reproducible and rapid, covering an entire operating cycle in few hours. Nowadays, ND finds many applications: (I) intratumor detection of heavy metals and endocrine metal disruptors; (II) identification of pathogenic nanoparticles in medical or veterinary drugs and devices, cosmetics, household products, and foodstuffs; (III) differential diagnosis of sarcoid-type granulomas (berylliosis, baritosis) and foreign body granulomas (prosthetic, iatrogenic); (IV) attestation of occupational disease correlating the datum with the occupational risk (anthracosis, asbestosis, bauxite fibrosis, byssinosis, chalicosis, siderosis, silicosis, stannosis, talcosis); and (V) forensic investigations to ascertain a causal link between disease and environmental, military, or work exposure. In addition to filling a knowledge gap, ND offers to the pathologist a current research field, with particular reference to the impact of occupational and environmental pollution on the human health and cancer.     

Confocal Laser Scanning Microscopy Analysis of 10 Cases of Craniofacial Fibrous Dysplasia

Fibro-osseous lesions (FOL) represent a heterogeneous group of lesions that exhibit a variety of clinic-pathological features. Recently, based on the new World Health Organization classification system, these lesions were reclassified as follows: (1) fibrous dysplasia (FD), (2) osseous dysplasia, and (3) ossifying fibroma. Nevertheless, the nosologic placement of FOL may be problematic because of substantial overlap in the histopathological findings. In this study, we analyzed 10 cases of FD by both optical and confocal laser scanning microscopy, a research technique based on the laser light microscopic analysis of stained biological samples that allows improved tissue imaging and bidimensional pictures with high resolution at the cellular level to provide a better understanding of the diagnosis of this disease.     

上腹部 CT 多期扫描与超声造影检查对肝血管瘤患者的诊断价值分析

目的探讨肝血管瘤患者应用 CT 检查与超声造影（CEUS）检查的诊断价值。方法回顾性分析2011年3月至2013年7月收治的48例肝血管瘤患者的临床资料。比较两种检查方法对不同大小的肝血管瘤、是否合并脂肪肝等因素下的检查效率。结果 CT 检查检出42例,检出率为87.50％;超声造影检出43例,检出率89.58％;两种方法的检出
　　率比较无显著差异（ P >0.05）。CT 检查>3 cm 的肝血管里发现率显著高于≤3 cm 的肝血管瘤（ P <0.05）。对不同大小的肝血管瘤超声造影检查发现率无显著差异（ P >0.05）。对不同大小的肝血管瘤超声造影表现为整体信号的增强变化均不显著（ P >0.05）;但是>3 cm 肝血管瘤的斑片状增强型信号显著高于≤3 cm 肝血管瘤组（ P <0.05）。CT 对是否合并脂肪肝的肝血管瘤患者的检出率无显著差异（ P >0.05）;超声造影检查,合并脂肪肝的肝血管瘤患者检出率为58.33％显著低于不合并脂肪肝的肝血管瘤患者的100％（ P <0.05）。结论对于合并脂肪肝的肝血管瘤患者 CT 检出更具优势,超声造影检出结果受肝血管瘤大小的影响较小,两种方法可以结合应用。     

The expanding spectrum of low-penetrance TNFRSF1A gene variants in adults presenting with recurrent inflammatory attacks: Clinical manifestations and long-term follow-up

ObjectiveTo analyze the clinical manifestations and response to treatment in a cohort of adult patients presenting with recurrent inflammatory attacks and carrying low-penetrance TNFRSF1A variants, as well as to provide data on their long-term follow-up.MethodsWe performed a retrospective chart review of 36 patients carrying low-penetrance TNFRSF1A variants. Moreover, 60 genetically negative patients treated for recurrent inflammatory attacks and 13 patients with structural TNFRSF1A mutations were also analyzed. Detailed demographic and clinical data were collected at the time of molecular screening and at each follow-up visit. Treatments and markers of inflammation were also assessed.ResultsIndividuals with low-penetrance TNFRSF1A variants have a lower family history for inflammatory attacks and present with a later disease onset compared with patients with structural mutations, but do not differ, in this respect, with genetically negative individuals. Moreover, low-penetrance variants are less frequently associated with a chronic disease course, with clinical manifestations such as abdominal pain and myalgia, and with amyloidosis. A distinctive clinical feature is a higher rate of pericarditis. Interestingly, mutation-negative patients were found to present with a significant history of recurrent pharyngitis during childhood. Patients with low-penetrance variants are mostly managed with short courses of steroids or non-steroidal anti-inflammatory drugs on attacks. Although the need for a biological treatment is significantly lower compared with patients with structural mutations, still approximately 20% of individuals with recurrent inflammatory attacks carrying low-penetrance variants ultimately require these therapies.ConclusionsOur study confirms that low-penetrance TNFRSF1A variants can be associated with an autoinflammatory phenotype. Although a chronic disease course is rarely observed, some patients ultimately benefit from a biological treatment.     

A three-dimensional soft tissue analysis of Class III malocclusion: a case-controlled cross-sectional study

Objective: The present study used the optical surface laser scanning technique to compare the facial features of patients aged 8–18 years presenting with Class I and Class III incisor relationship in a case-control design.

Materials and methods: Subjects with a Class III incisor relationship, aged 8–18 years, were age and gender matched with Class I control and underwent a 3-dimensional (3-D) optical surface scan of the facial soft tissues.

Results: Landmark analysis revealed Class III subjects displayed greater mean dimensions compared to the control group most notably between the ages of 8–10 and 17–18 years in both males and females, in respect of antero-posterior (P?=?0.01) and vertical (P?=?0.006) facial dimensions. Surface-based analysis, revealed the greatest difference in the lower facial region, followed by the mid-face, whilst the upper face remained fairly consistent.

Conclusion: Significant detectable differences were found in the surface facial features of developing Class III subjects.     

Wear of polyetherketoneketones — Influence of titanium dioxide content and antagonistic material

Objective

The aim of this laboratory study was to analyze the influence of titanium dioxide (TiO₂) content and antagonistic material on the wear of polyetherketoneketones (PEKKs).

Volumetric analysis of keratocystic odontogenic tumors and non-neoplastic jaw cysts – Comparison and its clinical relevance

The keratocystic odontogenic tumor (KCOT) is capable of causing vast osseous destruction. Histopathological examination is pivotal for diagnosis. The diagnostic process can sometimes be hindered by tissue inflammation of KCOTs with loss of defining criteria, resulting in misdiagnosis as an odontogenic jaw cyst. We discuss the possible merits of volumetric analysis when facing this particular diagnostic dilemma and for pathophysiological characterization of KCOTs. We included 114 patients, of whom 27 were histopathologically diagnosed with a KCOT and 87 with dentigerous (n = 41) and periapical cyst (n = 46). Semiautomatic segmentation and radiological analysis of preoperative cone beam computed tomography (CBCT) image data was carried out using ITK-SNAP. The mean volumetric extent of KCOTs is significantly higher compared to non-neoplastic odontogenic jaw cysts (p = 0.001). The mean volume and standard deviation for KCOTs and non-neoplastic odontogenic jaw cysts was 10381 mm³ ± 6410 and 5813 mm³ ± 4425, respectively. Volumetric analysis reveals that KCOTs significantly exceed the mean size of non-neoplastic odontogenic jaw cysts, adding an argument in favor of the neoplastic nature of KCOTs. In the case of difficult histopathological examination, lesions with a size exceeding a value of about 3000 mm³ could be considered for close clinico-radiologic follow-up.