Most visitors come to visit museums; in reality, few immersive solutions support the senses experience. Virtual reality (VR) technology attaches the virtual information from the real environment. Applying the VR technology in the 3D relic information display and visualization in the museum field is a hot research issue. However, most current solutions of relics are one-sided, only focusing on the virtual exhibition, lack of associations with actual function, and senses experience, especially the large artistic cultural relics. The scenario-based virtual exhibition solution is an available approach to allow visitors to imitate ancient artist and provide relatively experience in the form of content and sense organ of ancient art. It converts large relics into “digital large relics” and enables experiencing performance of ancient civilization in person. The solution presents relics to the visitors in a more direct and vivid manner and with innovative forms, strong interaction, and intelligence, thereby improving the interests and satisfaction among visitors in this type of relic exhibition. Besides, it also provides visitors with a convenient way to experience and learn ritual and culture. Evaluation and conclusion can be drawn that most participants appreciated this solution in clear interface and completion aspects. The history museum is considered as a privileged means for communication and provide a physical means to connect us to past cultures . Museum'To make it possible for visitors to gain more display information about cultural relics and related historical and cultural knowledge when visiting museums, a new concept of digital exhibition has been put forward, that is, the “expanded spatial display” in the physical space of the museum . The conThe VR technology utilizes the 3D graphics generation technology, multisensor interaction technology, and high-resolution display technology to generate a 3D lifelike virtual environment. The VR system, which contains abundant perceptual information and models, generates the same feedback information as in the real world with the help of the 3D interaction technology and complex sensor devices, such as the digital helmets and gamepads, to provide people with the same feeling as in the real world. Characterized by immersion, interaction, and imaginative, the VR system emphasizes the leading role played by people, which enables the information processing system to satisfy the demands of people and match the senses of people.With more attention focused on Chinese traditional culture, finding the suitable approach to display the relic information and transmit the historical knowledge to visitors are becoming more necessary. 3D virtual exhibition expands the museum's display space and provides a full range of information about the exhibits, which is a great opportunity for developing the display methods of physical museums. As one of the representatives of large cultural relics, chime bells   Displaying basic text descriptions of cultural relics: the information functions of digital cultural relics mainly include text introduction and historical allusions.  (G.2) Giving the user a basic understanding of the structural function analysis of Chime bells: displaying immersive virtual models of cultural relics and allowing visitors operate these models through various user interactions. Visitors observe the 3D relic, rapidly and firstly realizing the exterior design, structure, and material of the current relic.  (G.3) Giving the user a basic understanding of how the researches for realistic musical Instruments performance experience processes occur: VR-chime bells have the consistent performance function as the real instrument. There are feelings of beating chime bells while applying feedback, auditory feedback, and visual feedback to experiencers and experience of simulating how gavel knock chime bells play and acoustical principle of “a two-tone bell” of chime bells.System goal analysis is an important part in application research. The visualization system developed in this paper addressed three major design goals:Virtual cultural relic chime experience system is a combination of VR and music archeology. The subject took the first chime bells  to be unThe design of this subject in the VR environment uses HTC VIVE handle as the hit tool to hit the chimes for playing. Based on the acoustic theories of chimes, combining the immersive, interactive, and conceptual features of VR technology, we made some attempts on the chime music playing rules and experiencing methods in virtual environments. It is a novel VR application system and a combination of software and hardware design.As shown in HTC VIVE allows users to move within a certain space, which is the chime virtual playing space. The VR development platform invokes and controls the two handheld controllers to complete the events of the virtual chimes and commands from human, as well as the interaction and feedback between human and chimes in the VR environment.The positioning system tracks the monitor and two handle controllers within this space. The immersive application uses the Lighthouse optical positioning framework. Such accurate optical tracking technology can offer better experience, which has advantages, such as small amount of required computing resources, unapparent delay, and a large number of simultaneously tracked objects.The engine layer includes image engine, logic engine, sound engine, and support for the development of VR applets.The message processing layer consists of the script interpreter and message pool. If users input and output instructions in the system, corresponding messages will be released. Both script and instruction messages will enter the message pool, and the message processor will call each module in the engine to process these messages.The business logic layer includes system space mapping and digital object interaction, which constitutes the important business logic in the system.The control layer mainly receives requests from users, invokes the corresponding business processing object to execute the business module according to different requests, obtains the execution results, and returns appropriate view components to users based on the current state data and the processing results of the business logic.The view layer accepts the users' requests and forward these requests to the control layer.Boring defined the object of human-computer interaction as behavior, namely, people's behavior created and supported by media role of products; he pointed out creating and supporting human activities through the mediating influence of products . Its proIn terms of conceptual guidance, the Info Detail View displays the detailed text description and animation. The Info View and Info Detail View show the basic text information of relics, and a 360-degree display of virtual chimes to make users enter into an unconscious state, completing the G.1 in the goal analysis.This system enables users to obtain knowledge of cultural relics by directly clicking on digital cultural relics. By dividing the structure of the digital cultural relic model, this system adds interactive commands for each component so that users can “disassemble” the cultural relics to learn about the detailed information of each component. In this way, users can directly interact with digital cultural relics, thereby completing G.2 in goal analysis.To enhance user experience, G.3 should be completed. Visitors can play the chime bells to experience the acoustic principle of “two-tone bell” and get the haptic feedback during the hitting process. This design enables the visitors to experience real chime bells playing in both the form and content and also provides a convenient way to experience and learn chime ritual and musical culture for museum visitors, music learners, and cultural heritage professionals. Besides, the method is also an exploration on how to experience and learn Chinese ancient music art in virtual environments and how to develop the art as a kind of digital entertainment.The system is comprised of three primary components: (1) user interface (UI) and relevant events, which include the initial interface, help interface, and all UI elements in system; (2) virtual scene system, which used 3DS Max and Unity3D to create the virtual model and Virtual Multisensory Immersion Environment of the virtual chime bells; visualization renders the effect of real-time interaction in a virtual scene to the screen via the HTC VIVE head-mounted display; (3) interactive control, where the function goal of an operational control is to be able to meet the requirements of interactive visualization, including two aspects: it enables a user to directly select and accurately manipulate a model in real time, and it analyzes the incoming message through the virtual interaction scenario control interface and then gives the corresponding interactive control instruction to implement the performance of virtual relic chime and observe the structure in a multidimensional viewpoint. It will provide immersive 3D visualization of chime bells and an overall good user experience.The scene-based virtual chime system ultimately serves users, so the interactive advantages of VR are highlighted during the design process. The interactive design follows the basic principles of being easy to understand and operate. The design is simplified as much as possible, and the most natural reactions of most users are regarded as an important reference for the design.First of all, the majority of users are completely unfamiliar with VIVE devices. During operation, users may encounter two kinds of confusion: one is they do not know how to operate a certain function; the other is they do not know the existence of some functions. So, the system provides some simple and efficient operational guidance to encourage users to actively explore and learn, and the pavilion uses functional guidance to let experiencers quickly know what a virtual chime pavilion is, what functions it has, how to achieve these functions, and the specific steps. Before the users use this system, the operators should briefly teach the users so that they can rapidly adapt to the system. To save the teaching cost, it is more convenient to add animations showing users how to use the system. Therefore, a guidance interface of this VR system is designed for users to help them master the experiencing system faster and feel more pleasure from the experience. It can tell the users what this system is, what functions it has, and how to use it. When users first approach the virtual chime pavilion, it needs to quickly convey the concept of the system to them and make them get a good impression and more knowledge about the system so that the system can present itself fully when users use it.Graphical user interface is such an interactive design that places the clicking button near the digital cultural relics in the form of a spatial exhibition board. Users can check information about the cultural relics by clicking the button event in the user interface. The user gazes at the button event in the user interface through the cursor to perform the click behavior, and after that, knowledge about the digital cultural relics will be introduced. Users can perform personalized operations on the digital cultural relics based on their interests and hobbies, thereby gaining insight into related knowledge of cultural relics.Natural user interface is an interactive design that enables users to directly manipulate digital cultural relics. By directly clicking on the digital cultural relics to obtain relevant knowledge, users can learn about each component of the cultural relics in detail, which is similar to the effect of disambiguation and explaining. To make users more familiar with the system, cursor feedback is added to each structural component, for example, “highlight the material.” When users move the gamepad to a specific component, the material of that part will be highlighted as a reminder telling users about the interactive design.The user-centered interactive design considers the actual needs and using habits of users of all ages to the greatest extent. To help users conveniently gain knowledge about cultural relics, the system presents users with visualized knowledge of cultural relics in the following two ways:Human beings sense the environment through visual, auditory, olfactory, haptic, and gustatory senses. The mixture of these can influence our sense about the environment and provides a richer feeling than the feeling when each one of those senses is independently experienced. Compared with traditional VR, immersive VR brings high-fidelity multisensory virtual experiences. As an instrument of cultural relics, a chime involves three senses: sight, hearing, and touch in its virtualization process. In designing the virtual chime playing environment, we leveraged stimulation of visual, auditory, and haptic senses to make the playing experience more natural and realistic.Visual Element Simulation. The virtual chime pavilion has a virtual exhibition hall similar to an ancient environment. As shown in Acoustic Simulation of the Two-Tone Bronze Chimes. The chime bell body is of an “involute-tile shape,” [ shape,”  namely,  shape,” . This prHaptic Sense Simulation. The word “haptic” is derived from the Greek “haptesthai,” whose meaning is haptic sense or haptic sense induced. Webster points out that contact sensing is the contacts with the environment felt by the high-bandwidth (50 Hz) sensors near the skin, including the senses about the geometry of a fine surface, the temperature of a wrinkled surface, and sliding [ sliding . MeanwhiPosition Recognition. When users turn around or walk around wearing the headset, the renderer dynamically calculates the MVP composite matrix of each object through the vision-projection matrix at each frame and finally draws the vertices and image elements. Meanwhile, the 3D scene should be aware of the existence of the user head. Sensor event: the sensor physically tracks the gamepadButton event: interactive behavior generated by clicking a buttonControl unit event: generated by the input of thumbstick and touchpadGamepad events can be divided into the following three categories:By interacting with the virtual scene through holding the gamepad, users can directly obtain the direction, position, velocity, and acceleration of the gamepad, thereby tracking the direction and position and mapping the physical gamepad to the 3D VR world. Moreover, more diversified physical behaviors can be conducted. For instance, the force on the object can be calculated by multiplying acceleration by mass. Therefore, the movements of the arms can be well simulated.Simulation of Reality Physical Engine. The immersive virtual chime system simulates Newtonian mechanics through installing the PhysX physics engine developed by NVIDIA in unity and sets the sound effect of the gavel striking the chime by means of parameters, such as mass, gravity, friction, and speed, so as to realistically simulate physical effects, such as collision and gravity, making the virtual chime more real and vivid.In physical simulation, the rigidbody component enables the object to move under the control of the physical system, and the rigid body accepts external force and torque force to make sure that the object moves as if it were in the real world. By setting the attribute parameters of the rigidbody component, the mass, drag, angular drag, and gravity of the object are set, and the interactive operation between the NVIDIA physical engine and the other objects is completed. The collides component triggers the collision simulation to prevent rigid bodies without colliders from passing through each other.User evaluation is an important part to evaluate this application. The field observation and questionnaire survey are carried out to acknowledge visitors' behaviors; the previous visualization research about visitors' behaviors in the museum is also a great help . The evaAdapting to the Immersive Device. Multisensory virtual chime music experience pavilion is a virtual chime music immersive experience environment built on the VR helmet system (HTC VIVE), jointly developed by HTC and Valve. The VR environment uses head-mounted displays and handle as the hit tool to hit the chimes for playing. Therefore, it is necessary to evaluate the comfortableness immersion of users of different ages in using virtual wearable devices, the wearing habits of users from different backgrounds, whether those without experience of using virtual devices will accept wearing such devices, and their abilities of learning to use new devices.Experiencing the Application. For an immersive virtual instrument display system used for museum exhibition, it is necessary to evaluate the user's understanding of the system, the professional levels of virtual cultural relic instruments, and scientific nature of the system.Virtual chime bells' interactive experience should be able to provide its necessary functions. First, the experience test scale to obtain the experiment data and comprehensively judge the experience of the instrument simulation of the virtual chimes. Second, evaluation explored the role of virtual reality in experiencing the performance of ancient musical instrument and verified the effectiveness of VR method in studying the sound characteristics and performance skills of ancient music instrument chime bells. Finally, the comparison test method is to compare the experience with existing chime music playing experiences and understand the pros and cons of the VR-based interactive design, so as to offer data support for functional improvement.Effects of the Psychophysics. Human psychophysics is the quantitative measurement of perceptions. In essence, it is simply a more sophisticated version of what humans have done since time immemorial: noticed and reflected upon what humans can see, hear, and feel. As a virtual instrument relic, a chime has the following sensory feedbacks on three aspects.Visual sense is the first channel to obtain the information from the virtual chime bells exhibition hall. Among all senses, visual sense provides the largest proportion of sensory experience. Visual information includes interactive environment information , virtual scene information , and other elements.As it is the virtual simulation of an ancient musical instrument, auditory experience is an important indicator for the virtual chime bells exhibition hall's sensory experience. Chime bells, with its unique tone, charm, and ability to produce two tones with each bell, bring unique stimulation to auditory sense, which is the core part of virtual chime bells' art experience.Chime Bells Are Played Using a Mallet. The tactile feedback from this small tool after striking is an indispensable sense. In the experiment, the tactile sense from tactile feedback interface was measured. At the same time, the subject evaluated the effect of virtual tactile sense, which was generated by the sensory experience and psychological perception caused by visual and auditory senses during the interaction with chime bells.The experience in artistic activities is often a comprehensive experience, where the immediate physiological experience of the environment generated from multiple surface sensory organs goes into a higher level to become psychological and emotional experience.P is the perception of the environment as a whole, which is assessed from four aspects, visual sense (V), auditory sense (A), tactile sense (T), and participant's experience (E), and ω  denotes the weight of each sense, and the weight of each index is processed with Kano model, ∑ωi=1.Winkler and Faller found that high-quality sound, together with visual elements, could suggest visually perceived quality . CharactThe maximum threshold of each perceptual weight would not exceed the extent sensed in the real environment. According to signal detection theory, perception depends on knowledge and experience. The human consciousness comes from the primitive message of the external world and is conditioned by the past experience. For this reason, the new formula better corresponds with the psychophysics theory in the cognitive psychology. The relation between perception and prior knowledge is manifested in the interpretation of scientific data. The research holds the opinion that if participants are with the familiar bell culture, then the feeling of freshness lowers, and there will be fewer perception effects.The evaluation adopted anonymous questionnaire form. Questionnaire survey as a research method collects data based on written questions. It reflects the investigation targets and level, and it relates to whether the collected data are real and comprehensive and whether the results of the study are scientific and effective.The design of questionnaire is the most critical and important core link in the process of questionnaire survey because it directly affects the accuracy of the survey results and the usefulness of the entire study. Specific to a virtual chime interactive system for multisensory experience, a cognition-environment-emotion assessment system is proposed to form an assessment scale for cultural relics of musical instruments.In a human-computer interaction system, behavior is influenced by user characteristics, namely, the internal cognitive attributes of the user, and the external environment. Internal cognitions include user's knowledge background, cultural emotion, and others. The external environment includes the scene and media. These internal and external uncertainties exert influence on the user experience. Thus, the questionnaire was organized into two parts. In the first part, indicators refer to the interaction design theory of Lanir et al. , which iCognitive science reveals the psychological cognitive activities of the user, so as to evaluate user's cognition and behavior. Professor Glovanna in the field of cognitive neuroscience believes that cognitive models do not directly seek its physiological support, but they should not contradict neurophysiological findings . A cogniCognition Scale Indicators. In the cognition scale, “role” means the subject of the cognitive task, and it represents the target users group. Research on this aspect includes work like user characteristic analysis. Due to the artistic professionalism of instrumental music relics, it is emphasized in this paper that user characteristic is one of the key factors affecting the user experience rating of the interaction system. “Behavior” means research on behaviors, such as user behavioral habits. It is an important means to get tasks feedback from users' products usability testing and evaluation. The perceptual feedback of the interaction process is the foundation of the system, which involves the user's understanding of the object and the special emotional elements of art and culture. All task designs must comply with requirements on this dimension.Environment Scale Indicators. The environmental module contains three index dimensions. “Scene” is an important part of environment, and it includes all nonfunctionality. However, it is a media, which directly contacts the user and affects the user media to some extent. “Task” is a dimension expressing the user's goal and showing the user's intent, and it is the direct purpose for establishing the interactive system of virtual musical instrument relics, and the users may achieve their goals by performing tasks. “Technology” is the guarantee that the system is able to interact with users.Emotion Scale Indicators. Emotion is the ultimate expression of the experience system, including the comprehensive physiological and the psychological perception generated by users' experience of virtual musical instrument relics. Emotional expression can be divided into three categories: pleasure of experience mode, music emotion, and cultural emotion. So, evaluation assessed the expression emotion in aspects including experience's pleasure, music emotion, and cultural identity. In addition, evaluation found out the enhancing effect of virtual tactile sense brought by the visual and auditory experience and psychological perception during human-computer interaction on tactile sense feedback.Pleasure. The pleasure of experience mode is the key factor that determines whether the users are willing to experience virtual relic musical instrument. The pleasure includes the attraction of immersive display of cultural relics, satisfaction for experiencing virtual musical instrument relics, and user's expectation for the same type of works after using the virtual instrument cultural relics display system.Music Emotion. Music emotion refers to the user's knowledge and understanding of instrumental music. As the subject of ritual and music culture unique to China, chime bells are an important testimony to the developmental history of ancient Chinese music, and their form and acoustics features reflect aesthetic consciousness and performance modes of China in different stages. Only when the user has certain instrumental music cognitive foundation and appreciation ability can user's corresponding aesthetic emotion be formed. Otherwise, instrumental music will be regarded as gratuitous noise. In order to enhance the emotional experience of music, multisensory chime bells playing experience system designs of two different modes, free experience mode and professional learning mode, to meet different needs of visitors and ancient instrument learners. In the learning mode, the bells are highlighted according to the choice of music step by step All the conditions of the participants were in line with the objective of this experiment. Participants were asked to follow the following steps:The assessment results of virtual bell performance experience are shown in Besides, gender and immersive device using experience can also influence the cognition result, the average score of male participants is larger than the score of female participants, and the average score of participants who have contacted immersive devices is lower than the score of those have not contacted immersive devices. It can be inferred from the text comments in the questionnaire that female participants have less interest in immersive devices than male participants, and participants who have not contacted immersive devices are more interested in those who have contacted immersive devices before, so in the opinion of these participants more interested in immersive devices, each aspect in the application is more excellent, and it may lead to score difference to a certain degree.The study also explored the psychological implications of virtual tactile sense brought by the visual and auditory experience. To explore the influence of tactile feedback of virtual reality devices for participants' haptic perception, we set the four different versions of the experience, what was asked to assess the effect of visual factor , auditory factor (striking sound effect), tactile feedback, and multisensory experience on the tactile sense of the virtual chime bells' performance, respectively. In the evaluation, two evaluation parameters were set, namely, the impact on user experience  and the impact on virtual playing music . The psychological perception scores of user experience  were obtained by summing the two scores of each user. According to the Lewis magic number 5 theory , most orImmersive technologies provide cultural heritage scholars and musicologists with a novel research way on 3D digital equivalents of ancient instrument. Based on the comparative human perception in chime bells' several different performance modes, the assessment scores show that watching real chime bells exhibits brought the highest satisfaction in terms of visual experience and provided low pleasure level due to a lack of auditory and tactile experience. Multitouch technology for chime bells' performance had limitations on cognitive experience, having a lower pleasure score than that of watching actors' performance. Virtual chime bells exhibition hall obtained a higher score for tactile experience. Though it had a lower score for auditory experience than that of actor performance, its score for pleasure level was the highest. The average experience with different methods is shown in Human perceives the environment through visual, auditory, tactile, and other sensory organs. The integrated experience of them can affect our psychological perception of the environment. Multisensory perception is richer than the sum of each sensory experience. Compared with traditional virtual chime bells' performance, immersive virtual reality can bring high-fidelity and multisensory experience with more natural sensory stimulation.This research showed that sense organs will interact with each other, and the cooperation between high-quality sound and visual elements can increase tactile perceived quality, as high-level perception and interactive feedback are closely related to satisfaction. Virtual chime bells exhibition hall allows experiencers to feel the connotations of ritual and music culture of ancient chime bells and boost their interest and pleasure.Virtual chime bells exhibition can bring this large cultural relic back to the public. It not only solves the limitations of large cultural relics in time and space dimensions but also improves digital research experience of ancient Chinese music and art. This attempt to develop new digital experiencing method in virtual environment has promoted the development of chime bells as a kind of digital entertainment.Virtual chime bells can also be deployed in colleges and universities that make a specialty of music theory about chime bells and chime bells-playing skills. For musicology researchers, it is appealing to further study and research chime bells culture with this virtual simulation system as a tool.


During professional hygiene procedures, different instruments used may cause various damage to dental prostheses. Deplaquing and scaling with curettes and ultrasonic instruments may inadvertently increase the surface roughness of the material and the risk of future bacterial adhesion and/or also compromise the marginal seal of the prosthesis. Hence, the aim of this study was to assess the qualitative effects of two types of curettes and one piezoelectric instrument with a stainless-steel tip on three types of metal-free samples. After treating the samples with different instrumentations, they were analyzed using the scanning electron microscope and then underwent a qualitative microanalysis by using a spectroscopy machine. All the materials tested in this study have undergone significant changes of their superficial structure after instrumentation both with mechanical and manual instruments. Plastic curettes appeared to be less aggressive than the other instruments. Disilicate samples show a significantly lower degree of surface glazing erosion compared to the zirconia sample with all the instruments used. Today, several metal-free prostheses have become available and popular in prosthetic dentistry; they allow a better reproduction of the natural tooth, a higher aesthetics and require a lower preparation than metal-ceramic restorations avoiding some of their shortcomings . Metal-fOral hygiene procedures are necessary to reduce risks of gingival and periodontal diseases and then to promote the long-term success of prosthetic restorations ,12,13. DSeveral types of curettes and scalers have been developed for professional oral hygiene procedures from universal to specific scaler, curettes and piezoelectric instruments fabricated with different materials. Several studies were performed to analyze the surface roughness of these materials in combination with different instruments ,21. The aim of this in vitro study was to assess the qualitative effects of professional hygiene maintenance of two types of curettes and one piezoelectric instrument with a stainless-steel tip on metal-free samples using the scanning electron microscope (SEM). In addition, qualitative micro-analyzes were carried out to highlight potential exchanges of material between the instruments used and the samples.Three cylindrical metal-free samples  of 1 cm For each sample four distinct areas were marked and named A, B, C and D. In “A zones”, no instrumentation was performed, representing the “untreated control” surface that will serve as a reference. In “B zones”, the samples were treated with plastic curettes . In “C zones”, the samples were treated with a steel curette . In “D zones”, the samples received a piezoelectric ultrasonic scaler treatment with a steel tip . A new instrument for each sample was used to avoid dullness problems.The instruments were used as recommended by the manufacturers. The samples were oriented horizontally in relation to the operator and kept still during scaling procedure . The untreated surface of each specimen was placed at the opposite side of scaling. Subsequential horizontal and oblique scaling motions were performed for each surface treated with curettes. The blades were oriented at a 70-degree angle to the ceramic surfaces with pressure that simulated clinical procedures , while tThe time of treatment was established based on the study of Torfason and Badersten ,15, who The ultrasonic scalers were used for 30 s with a water-cooling system, using short and constant movements in the same direction, as described in previous studies . All instrumentation was performed by the same experienced dental operator and in standard conditions.After treatment, the samples were sent to the Center for Electron Microscopy of the University of Ferrara. To visualize surface scraping areas, a scanning electron microscope  was used, while to identify the chemical elements on the surfaces, a qualitative microanalysis by using a spectroscopy machine  was carried out. EDS sensors allow a quick acquisition of the complete emission spectrum and the identification of the different components of the sample and their proportions in a relatively short time. Before the scan, the specimens were mounted on specific fixed supports and coated with a thin layer of graphite, a conductive material able to allow the scanning electron microscopy (SEM) without interfering with the qualitative analysis. For all the areas of each sample, SEM scans were performed at two different magnification levels  and a qualitative microanalysis was performed.All the samples of the study were analyzed under a scanning microscope and then with the spectroscopy. The images of the scans at differentiated magnification are shown below . ResultsIn general, comparing the different images at SEM by visual inspection, both the steel curette (Group C) and ultrasound steel tips (Group D) have visibly furrowed and/or scratched the structural surface of these materials, while plastic curettes (Group B) appeared to be less aggressive than the other instruments. Steel curettes (Group C) ere demonstrated to be more aggressive than the sonic driven (Group D) across all the samples. These findings could be justified by the different composition of the instruments (plastic vs steel) and from the sharpness, which is more pronounced for curettes than for the tips of piezoelectric instrument. Further observations provided evidence that steel curettes (Group C) caused superficial structure loss on all the materials tested e,f, withBy comparing the qualitative spectroscopy analyses relating to the zirconia sample, it was possible to see how in zone A (untreated control surface), the presence of the Zr element is below detection level, while the most common element is silicon oxide. This is due to the glazing process carried out by the dental technician after the fabrication of the ceramic restorations. This layer was clearly compromised after instrumentation  showing a reduced percentage of silicon oxide and, on the other hand, a considerable increase in zirconium percentage. With steel curettes instrumentation (Group C), the presence of Zr rose to 33%, which is quite significative of the erosion of the surface glaze layer of the sample. The result is a rougher and more porous surface, which increases the risk of plaque retention.However, the release of metallic components (Iron e Chromium) from steel instruments to the sample surface was higher for mechanical instrumentation (Group D). Only for the zirconia sample, group C showed a higher release of iron and chromium than piezoelectric. This result could be explained by thinking that, during instrumentation, the roughness of zirconia considerably increased. Then, a major wear of steel curettes may be possible also because of the wider contact surface of the instrument itself, the more maintained contact and the greater and continued force applied than piezoelectric tips.Conversely, both the disilicate samples show a comparable and significantly lower degree of surface glazing erosion compared to the zirconia sample with all the instruments used, as demonstrated by the constant presence of surface silicon oxide also after instrumentation. This could be attributed to the different bonding of the silicon oxide of the glaze with zirconia and disilicate materials. Probably, the absence of a clear chemical adhesion between glaze and zirconium ,25, and There are several limitations to this study: the in vitro conditions such as the absence of saliva, blood, gingiva, and the fact that instrumentation is performed directly on the surface of the material with a constant inclination, almost impossible with in vivo conditions, obviously could have influenced the results. In addition, the round shape of the samples is different than the shape of the dental crown or other indirect restorations and on this surface, instrumentations could have been performed in an unrealistic way.Other limits are represented by the restricted number of samples and the limited types of materials tested; it is possible that testing other types of restoration materials but also other types of zirconium and lithium disilicate may lead to different results. Furthermore, an evaluation of roughness profile values of these materials is lacking, and, in future, it could be interesting to associate it with a micro-qualitative analysis.The acquisition of images and qualitative analysis have shown that all the materials tested in this study have undergone significant changes of their superficial structure after instrumentation both with sonic and manual instruments.Steel curettes demonstrated a more aggressive behavior than the sonic one across all the samples, while plastic curettes revealed a low aggressive behavior.The disilicate samples show a comparable and significantly lower degree of surface glazing erosion compared to zirconia sample with all the instruments used.However, more studies with more samples, and possibly in vivo conditions are required to evaluate the superficial change in new restoration materials after routine professional hygiene procedures. The continuous evolution in the field of dental materials is generating a lot of new materials for dental restorations. It is, therefore, important to evaluate their behaviors in the mouth of the patient, especially after hygiene procedures, to understand the possible advantages or disadvantages of prosthetic treatment.


The appraisal of appropriate levels of investment for devising flooding mitigation and to support recovery interventions is a complex and challenging task. Evaluation must account for social, political, environmental and other conditions, such as flood state expectations and local priorities. The evaluation method should be able to quickly identify evolving investment needs as the incidence and magnitude of flood events continue to grow. Quantification is essential and must consider multiple direct and indirect effects on flood related outcomes. The method proposed is this study is a Bayesian network, which may be used ex-post for evaluation, but also ex-ante for future assessment, and near real-time for the reallocation of investment into interventions. The particular case we study is the effect of flood interventions upon mental health, which is a gap in current investment analyses. Natural events such as floods expose people to negative mental health disorders including anxiety, distress and post-traumatic stress disorder. Such outcomes can be mitigated or exacerbated not only by state funded interventions, but by individual and community skills and experience. Success is also dampened when vulnerable and previously exposed victims are affected. Current measures evaluate solely the effectiveness of interventions to reduce physical damage to people and assets. This paper contributes a design for a Bayesian network that exposes causal pathways and conditional probabilities between interventions and mental health outcomes as well as providing a tool that can readily indicate the level of investment needed in alternative interventions based on desired mental health outcomes. Natural hazards can have large societal impacts. It is estimated that they caused 7700 human fatalities and $110 billion loss of infrastructural assets worldwide just in 2014 .Similar to other natural disasters, when flooding occurs, it creates significant damage to homes, communities, businesses, public services, and so forth. Residential properties usually suffer the greatest proportion of flood damage, with 25% of total damage, for example, costs of £320 million incurred by 10,465 properties due to flooding [It has been argued that flood risk management is usually measured as direct property and infrastructure losses, since these are the most important input for cost–benefit analysis to guide the government bodies to invest in flood risk management strategies . HoweverThe most apparent intangible impact of flooding is on human health. Direct intangible damage is a primary loss, which manifests as physical injury or even loss of life. Indirect health impacts are mental health disorders, which are caused by the experience of being flooded, or being impacted during the restoration process. Estimating flooding impacts will provide valuable insights for decision making and risk mitigation, policy-making, civil protection, emergency alertness and response, insurance and reinsurance, damage estimation practice/research, and so forth .On account of this, a comprehensive societal cost–benefit assessment must take into account the intangible losses caused by floods, such as psychological disorders or anxiety , as wellIn this paper, the primary focus is on the evaluation of flood impacts on human health, particularly mental health . Flood iIt is thus crucial to embrace social, political, environmental and other conditions, such as flood likelihood and local priorities, in the comprehensive evaluation. The evaluation method must be also able to swiftly determine changed investment requirements as the incidence and magnitude of flood events continue to grow. This quantification is essential and must examine various direct and indirect flood impacts on flood related outcomes in a probabilistic manner.In this paper, we illustrate how the Bayesian network (BN) probabilistic method can be used efficiently for ex-post economic evaluation, as well as ex-ante for future assessment, and indeed near real-time for reallocation of investment into interventions.In general, BNs provide a robust and flexible analytic approach to the challenge of complex health datasets, which pose specific computational challenges because of missing data, large or small size of data, complexity , changing populations, and nonlinear relationships between exposures and outcomes . Unlike In order to construct the proposed probabilistic methods, we need to have a holistic overview of the relationship between flood events, their aftermath and population well-being, and risk factors causing psychological disorders. The psychological health impacts of flooding, and their relationship with flooding and other risk factors will be briefly discussed in the next section. Estimating the cost of flooding on human health, in particular on human mental health, is very challenging but essential so that investment into interventions can be evaluated against reduced mental health impacts. It is essential to use metrics/methods to monetize mental health impacts.In order to reduce the damage to the community and people caused by flood events, environmental agencies are using various interventions each with different outcomes, efficiencies and costs. Any combination of interventions results in different value for money, with multiple conditional dependencies between interventions, choices of implementation and their contexts. This study provides an efficient construction of a probabilistic BN that displays causal pathways and their probabilities between interventions and mental health outcomes, as well as providing a tool that can readily indicate the level of investment needed for alternative interventions based on anticipated mental health outcomes.The psychological impacts of flooding can be very significant and long-lasting. Difficulties in evaluating the mental health impacts of flooding arise because accurate diagnosis of any condition is not straightforward, and mental health impacts are often under-reported and can be overlooked in comparison to the physical health impacts.There are some studies that evaluate the impact of flooding on mental health. In one of the earlier studies, Reference  conducteThe UK and England in particular are prone to flooding. In 2005, a severe flood hit Carlisle, UK, and many homes were affected. Carroll et al.  conducteResearch in other regions provide similar findings. Vietnam is also susceptible to natural disasters and specifically to flooding. Bich et al.  highlighZhong et al.  provide Estimating the cost of flooding on human health, including mental health, is extremely challenging. The study by  reports Most studies focused on direct impacts. The common types of health metrics used are: death; hospital admissions and out-patient visits; cases of acute morbidity or injuries; and mental disorders or reduction in well-being .However, loss of life or the number of injured are commonly used to measure the health burden associated with any natural disaster and, therefore, the impact of flooding on individuals’ mental health is often overlooked. In order to monetize health impacts in the flooding context, the following should be considered: Healthcare resource use; Productivity loss; Dis-utility from suffering or life-shortening.The monetary value of the latter component is typically evaluated by wealth–health trade-offs that the affected people reveal in surrogate markets, or which can be implemented through multiple choice experiments. The monetary value of dis-utility associated with an adverse health outcome is thus attributed to the willingness to pay (WTP) to avert outcomes or, when considering mortality risk, the value of a statistical life that is derived from individuals’ aggregated WTP for a small change in survival probabilities . In the Matsushima et al.  valued WThe UK Environment Agency (EA) has recently studied the new economic costs for the mental health impacts of flooding by analysing the data provided by Public Health England (PHE). It was illustrated that the mental health prevalence of people disrupted or affected by flooding is considerably higher than the unaffected groups, over 12 and 24 month periods. The findings of the study are comparable to the results from the flooding occurred in 2007. It was also reported that the chance of any type of mental health outcomes among the affected population will increase with the flood severity (or depth of flood). The same study confirms that WTP could be a very useful metric to evaluate the social cost of the flood impact; however, it cannot be used to include the actual cost of the mental health outcomes to the economy.Knowledge of each outcome (or condition);Prevalence of these outcomes;Presence of known treatment plans;Duration of any treatment;Likely impact of the outcome over the short term in terms of days of work lost.A study commissioned by Defra suggested that households were, on average, willing to pay £200 per year (2004 prices) to avoid the negative health impacts of flooding  . Defra’sThe uncertainty of flood occurrence has noticeably increased due to intensified climate change; andThe consequences of flooding considerably depend on the behaviour of the affected people and their capability to adapt.The quantification of the benefits of flood risk prevention measures is still an unresolved challenge in disaster management research. In particular, there is no clear flood risk management to quantify the effect of interventions in reducing the flooding impacts on people including the effects on the affected population’s mental health. The most widely adopted framework in flood risk reduction is represented as the calculation of the expected damages as a function of flood hazard, physical vulnerability and exposure . AccordiThere are various interventions that reduce the damages caused by flood events on communities, local environmental agencies, with different outcomes, efficiencies and costs. For instance, an intervention can be to use an early warning system (EWS) to reduce the amount of direct tangible costs . The aim of a flood warning system is to provide useful information, for instance, by issuing alerts or activating the required protection measures with a view to improve decision making and action. The connections and feedback between hydrological and social spheres of early warning systems are key elements of successful flood mitigation.The behaviour of the public and first responders during flood situations is determined by their preparedness, and is heavily influenced by numerous behavioural traits such as the perceived benefits of protection measures, risk awareness, or even denial of the effects that might occur. In the UK, the Environment Agency (EA) has an important role in warning citizens about the risk of flooding with a view to reducing the impact of flooding from rivers and the sea as well as pluvial floods.In November 2009, Cumbria experienced devastating flooding in its different regions due to the heaviest rainfall ever recorded in the UK . FollowiAnother intervention to reduce mental health harm is to relocate people away from the affected regions as soon as possible, and to support them during and after a flood. This also has rebound effects.A probabilistic method is needed to consider all sources of uncertainty that may influence an intervention in a particular context in order to evaluate its value for money There are complex relationships between flood events, their aftermath, population well-being and risk factors causing people’s health deterioration and/or psychological disorders .To understand the potential benefits or drawbacks of any intervention for reducing the damaging impacts on people’s health, and particularly their mental health, the nature of the hazard and the vulnerability of the community and its exposure need to be taken into consideration. There are currently several statistical methods to explore the relationship between flooding and the health risk factors discussed above. For instance, a multivariable logistic regression model was proposed by  to modelAlternatively, probabilistic methods, particularly BNs, have become an increasingly popular method for modelling uncertain and complex systems  and are Bayesian network (BN) is a mathematical model that graphically and numerically represents the probabilistic relationships between random variables through the Bayes theorem. This technique is becoming popular for aiding decision-making in several domains due to the evolution of computational capacity, which makes possible the calculation of complex BN . ApplicaE representing the direct dependencies between these variables. In the BN, as a probabilistic graphical model which is used to represent knowledge about an uncertain domain , each raables X=X,X2,…,Xn.A simple example of a BN is illustrated in X: Lunge cancer in As can be observed, a conditional probability table (CPT) is attached to each node. The CPT on each node is associated with the conditional probability distribution, as given in Equation . The CPTp, grows exponentially with p [BN learning consists of two general steps: (i) Finding DAG, which illustrates the interdependency between the variables/nodes and (ii) Finding CPT for each node given the values of its parents on the learned DAG. Finding the best DAG is the crucial step in BN design. The construction of a graph to describe a BN is commonly achieved based on probabilistic methods, which utilise databases of records  such as y with p .In this paper, BN will be used to evaluate the effect of flood interventions upon mental health to explore and display causal and complex relationships between key factors and final outcomes in a straightforward and understandable manner. The proposed BN is also used to calculate the effectiveness of the interventions and the uncertainties associated with these causal relationships, which will be discussed in the next section. Due to the lack of data, the proposed BN in this study was learned based on expert judgments ), and narrative in the relevant literature (as discussed in the next section). However, this approach will effectively work with data from a variety of sources, and handles a mix of subjective and objective data that can be incorporated with variables from different contexts ,48. MoreBN structure learning: There are a number of risk factors related to the flood interventions upon mental health including healthcare resources, flood management practices, existing mental disorders and many more, which will be considered as input and mediate nodes in the proposed BN model. The level of effectiveness of these nodes and the causal relationships between them are presented by edges, which can be elicited from the domain experts and the available data to construct the BN structure.Parameter learning: prior probabilities assigned to root nodes and conditional probabilities for dependent (leaf) nodes are elicited from the experts’ domain and existing information. In the BN, the state of some nodes could be influenced by their prior states, or affect other nodes. The probabilities of these nodes are determined before propagating evidence to the model ,51.Outcomes of BN (Posterior probability learning): The final step in BN is to run the model at agreed intervals. As new information is added to the model, the current priors/states will be updated using the Bayesian paradigm in a very efficient way.To construct a BN for the evaluation of the effect of flood interventions upon mental health, the following steps need to be performed:It is also straightforward to use the BN to identify which variables have the largest influence on the final outcomes of the network. A unique feature of BNs is the ability to back propagate the model’s conditional probabilities through the model structure. This means that we can test how to achieve desired outcomes by identifying the most likely combination of risk factors.The BN model can be used to develop an effective and efficient decision support tool. In the next section, the BN-based decision support tool will be developed to evaluate the cost-effectiveness  of various flood interventions upon mental health in the presence of different uncertainties and under certain constraints.In this section, we evaluate the impact of the flood interventions on the mental health of people affected by flooding using a BN trained by a combination of the data extracted from a narrative in the relevant literature from the published reports and expert judgments (including experts from EA and PHE). However, it is very straightforward to train a BN based on a combination of the heterogeneous data collected from surveys and other methods .We first need to learn the BN for a subset of the risk factors selected in relation to the flood intervention’s impact upon mental health, including the prevalence of probable depression in people who have been flooded (Flood), loss of sentimental items (LSOI), prevalence of loss of sentimental item as secondary stressor in those exposed to flooding (PPD), less severe depression (Lsever), and more severe depression (Msever).It is usually recommended that the BN structure and model parameters should be learned from the combination of data and expert judgments . Howeverhttps://bit.ly/3eXiKwt . The probability of PPD was determined to be In the next step, we need to estimate or determine the CPTs. Due to the lack of data, the conditional probabilities of each node of the BN shown in be 20.1%  from thebe 18.6% . The proFollowing , the thrmeasures ,56, whicNext, the proposed BN presented in For Msever: CQALY = 0.055;For Lsever: CQALY = 0.062.Let us assume that before taking an intervention  that could lead to an individual losing their sentimental item, the changes in QALY for two mental health states  are computed from the learned BN illustrated in The above CQALYs are computed based on the mean values suggested for the mental states as given in For Msever: CQALY = 0.033;For Lsever: CQALY = 0.038.The QALY values, if the intervention was decided to be taken by the local EA prior to the flooding, will be computed (from the BN) as follows:The differences that the intervention will make to the mentioned mental health states are given byFor Msever: 0.055 − 0.033 = 0.022;For Lsever: 0.062 − 0.038 = 0.024.For Msever: For Lsever: Finally, by multiplying these changes in QALY by the lowest point of NICE’s QALY cost-effectiveness threshold , we can evaluate the cost-effectiveness of the suggested intervention on reducing the mental health impact due to the loss of sentimental items, as:This suggests that using a flood early warning system from the local EA to inform people could save at least It should be noted that the early warning system could itself create further stress. An alternative way would be to relocate people away from the affected regions as soon as possible, and to support them during and after a flood. However, any of these strategies or their combinations could affect the flooded people’s mental health, with each strategy imposing varying benefits and costs. The method proposed above can provide us with an effective cost–benefit analysis approach in comparing the suggested interventions, by taking into account the complex relationships between flood events, their aftermath, population wellbeing and risk factors causing people’s health deterioration and/or psychological disorders, and the costs and benefits of the interventions.BNs have been written to evaluate (ex-post) the effect of different factors on outcomes, in contexts other than flooding. For instance, a BN has represented the interactions of indoor climate factors on the mental performance of office workers, to demonstrate that investment in improved thermal conditions is economically justified in most cases with different building designs . This paA BN used for evaluation, based on retrospective data and expert opinion, would need to change to represent the future scenario being assessed. The scenario may introduce new variables (and associated changes to prior probabilities), for example, to represent how the future complexity of the system will work. The scenario may require changes to the values of existing variables, for example, to reflect future efficiencies or the effects of different ways of organising. In fact, a number of BNs may be developed to examine alternative viable futures. Every BN will have a set of unique conditional probabilities which will assess the future conditions or scenarios. Across all BNs, a range of potential outcomes will ensue and will provide an indication of future outcomes. However, scenarios are speculative and indeed deterministic , but if The health economic evaluation methodology proposed in this study, to explore the level of investment required for alternative interventions based on desired mental health outcomes, could be developed further by computing the value of information (VoI) analysis   after removing all uncertainty from such an analysis, more research and data are required to investigate the effectiveness of the proposed method in this regard.


This paper describes the case of a 54-year-old woman diagnosed with Hajdu–Cheney syndrome, who presents with characteristic craniofacial dysmorphia, short stature, premature loss of teeth, developmental skeletal disorders, fibrocystic mastopathy, bilateral hearing loss and an intermittent mild neutropenia. The patient received treatment with bisphosphonates and was awaiting evaluation for surgical arthroplasty of both hips when she suffered a motor vehicle accident, which led to a rapid progression in her disease by increasing her degree of dependence for most activities of daily living. The clinical presentation and radiologic findings seen in this case confirm the three main features of the syndrome: phenotypic variability, an age-dependent progression and the presence of generalized osteoporosis and acroosteolysis of distal phalanges. The main objective of the manuscript is to describe a new case of a patient diagnosed with Hajdu–Cheney syndrome. Due to the low prevalence of the syndrome and the small number of cases reported in the scientific literature, obtaining a complete description and a global perspective of the disease is complex. Hajdu-Cheney syndrome (HCS) is a rare genetic disease. It is registered in the database of the OMIM project with reference number 102500 and in ORPHANET under the reference ORPHA955. This disease mainly affects the connective tissue and belongs to the osteolysis syndromes group , althougN. Hajdu first described the disease in 1948 , and theThe phenotypic variability  is the cThis disease has a degenerative nature ; therefoThe osteolysis of distal phalanges and generalized osteoporosis  found inThese differences include cranial alterations  such as The definitive diagnosis is reached by genetic sequencing , althougCurrently, there is no definitive or effective pharmacological treatment for HCS, although there are projects underway studying this aspect . At presThere are currently more than 7000 rare diseases in the world, of which only 800 have minimal scientific knowledge. In general, rare diseases and in particular, Hajdu–Cheney syndrome, have a low prevalence and few registered cases. The study population sample is dispersed, with a variable phenotype, little-described clinical symptoms and different evolution.Considering the basic characteristics of this disease, a field of study with many aspects to be explored, can be seen, highlighting aspects such as the complete definition of the phenotype and clinical symptoms through the study of existing cases.The patient was a 54-year-old Caucasian woman . Her motThe patient was diagnosed with congenital acroosteolysis or Hajdu–Cheney syndrome, osteoporosis, disabling coxarthrosis with acetabular protrusion, musculoskeletal alterations, flat feet, short stature, dysplastic facies, dorsal hyperkyphosis, vitamin D deficiency, low bone density in the trabecular bone, fibrocystic mastopathy, bilateral hearing loss, intermittent mild neutropenia, dysphonia with mild sleep apnea without oximetric repercussions, chronic gastritis and constipation, premature loss of teeth, and adjustment disorder with a longstanding depressive disorder.The patient was born in 1967. The first clinical registry was obtained at the age of six years after a visit to the Nuestra Señora del Prado Hospital in Talavera de la Reina . After clinical examination, the patient was found to have a moderate psychomotor delay, dysplastic facies, alterations of the extremities and delay in stature closure. The first diagnostic suspicion was pycnodysostosis.At the age of 9 years and 11 months, at a follow-up consultation, the following data were compiled from the physical examination: good nutritional state, stature 134 cm, weight 26 kg, presence of dysplastic facies, underdeveloped dentition, a systolic murmur identified at cardiac auscultation, pubic hair beginning to develop on the major labia and upper zone, observation of symmetric upper extremities with a proportionate shortening of the arm over the forearm during the examination of the musculoskeletal apparatus, prominence on the internal face of both elbows affecting pronation–supination movements, swelling of terminal phalanges of hands with watch-glass nails without local pain, and examination of lower extremities found bilateral genus valgus with widening of the distal feet and toenails of the same characteristics as those of the hands .An X-ray of the skull showed dolichocephaly, elongated sella turcica and alterations of the sphenoid fissure. X-rays of the arms revealed bilateral dislocation of the radioulnar joint and acroosteolysis of the hands.In 1977, the patient was diagnosed with idiopathic progressive acroosteolysis. The description of the case was performed by analyzing, in detail, the different clinical manifestations within their physiologic context and in a chronological order with the aim of evaluating the progressive nature of the syndrome.In 1978, the patient was referred to the traumatology service of the Nuestra Señora del Prado Hospital for the assessment of valgus flat foot that did not improve with orthopedic insoles. Finally, a surgical procedure was performed and the use of orthopedic footwear was recommended.In 1987, due to the natural progression of the disease of her feet, the patient developed third degree pes cavus with irreducible claw toes and a marked equinus position of her left foot. She also presented with a persistence of recurvatum of the left knee and later developed the same problem on her right knee as well as bilateral genu valgum. Then, cephalic necrosis of the left hip appeared causing painful limitations with movements of the hip joint.In 1993, the diagnosis worsened as the diseases progressed over time and deformities of the hands, feet, knees, hips and spine were present, accompanied by a considerable loss of strength .In 1996, the patient visited the 12 de Octubre Hospital. During these visits, alterations of facial bones and the jaw were identified, and many teeth were found to be missing alongside alterations at the point of implantation. Other findings include early onset of hip arthritis, a complete disappearance of the third and second phalanges of the first and second fingers of her left hand, severe deformities of both feet that, alongside her knee deformities and a lumbar hyperlordosis, practically impeded walking. The patient walked with difficulty using crutches, although she began to use a wheelchair for longer distances.In 1998, the patient underwent a bone biopsy, the results of which showed findings consistent with osteoporosis.In 1999, new severe lesions of the distal phalanges of both feet were registered and a progressive destruction of areas of the tarsus, metatarsus and toes with spontaneous anquilosis of the left ankle in equinus position was found. The patient also had other congenital alterations such as a posterior dislocation of the radius of both elbows with functional limitations and a spondylosis of L5 that was identified during imaging controls. Later evaluations found a worsening of mobility and positioning of both feet, including the ankles, as well as a progressive destruction of both hips and spontaneous pain and contracture in flexion and internal rotation of the left hip. At this stage, both feet had lost all anatomical structure. Due to the auto aggressive nature of the syndrome, the patient was considered for surgical intervention for the insertion of hip prosthesis.In 2008, the patient was diagnosed with bilateral collapse of femoral heads.By 2012, the patient was limited to a wheelchair due to a complete inability to walk. There was also a progressive bone decalcification due to the lack of deambulation, further deformities in both hands, a left acetabular protrusion and bilateral coxarthrosis.In 2013, a pelvis CT was obtained, which found a marked deepening of the femoral heads within the acetabular and bilateral protrusion; a diagnosis of Otto pelvis was made. The patient required evaluation for hip replacement. Her clinical condition was poor, with considerable disability requiring continuous assistance for everyday activities and transition to a motorized wheelchair to enable mobility. The patient complained of pain in the feet and edemas and of the inability to remain in a standing position due to the extensive destructuration of her coxofemoral joints.In 2016, the clinical condition of her pelvis required referral to the Ramón y Cajal Hospital for evaluation of a possible bilateral hip replacement arthroplasty. Whilst the ongoing evaluation, the patient suffered a motor vehicle accident that has had a significant impact on her condition, notably worsening her general condition and her prospects of recovering any deambulation.The accident suffered in 2017 marks a before and after in the progression of her disease. The patient was run over by a vehicle, taking a fall to the ground, which caused multiple lesions, mainly to her legs.After the accident, the patient was referred to the pain management unit due to a severe worsening of the pain in her hips, ankle and left foot, although no fractures were identified.By 2018, there was a slight improvement of the pain and the department of rehabilitation carried out a new evaluation of the possible impacts of a hip replacement but resolved to not move forward with the arthroplasty, based on the premise that the patient was unlikely to regain the ability to walk unassisted despite the surgical intervention.In 2019, scleroderma was identified in association with the already present acroosteolysis.Over the years of follow-up, bone density studies were carried out every 2–3 years by the department of rheumatology to study the progression of the disease regarding osteoporosis in order to adapt the established pharmacological treatment. Bone densitometry of the spine and the hip were performed. In the densitometry control of 2012, there was an improvement in the parameters of bone density with regards to the 2005–2009 studies.2 and 0.885 gr/cm2 in the left hip. Therefore, the diagnosis for the lumbar spine is compatible with osteoporosis and with osteopenia for the left hip.The last densitometry was performed in 2021. The results found that the bone density in the lumbar spine (L1–L4) was 0.673 gr/cmIn 2019, the patient was referred to the department of respiratory medicine for the study of dysphonia and a sensation of shortness of breath when speaking. It was decided to continue the study of her dyspnea in the sleep disorders’ unit to rule out nocturnal hypoventilation. A respiratory polysomnography study was carried out to establish baseline conditions.Results showed a saturation tracing with several dips constituting a desaturation index of 15.1 per hour or registry, with a time of saturation below 90% (TC90) of 0.4%, and initial saturation of 95%, minimum saturation registered 80% and a mean saturation of 93.4%.The mean heart rate was 83.2 bpm, with a maximum of 119 bpm and a minimum of 69 bpm. Regarding the respiratory signal, there was a tracing with apneas and hypopneas accompanied with oxygen desaturation that constituted an apnea–hypopnea index (AHI) of 15.9 events per hour of registry , with an index of central apneas of 0.3 per hour of registry, an index of obstructive apneas of 2.1 per hour or registry, an index of mixed apneas of 0 per hour of registry and an index of hypopneas of 13.5 per hour of registry. These results determine the existence of a mild to moderate sleep apnea–hypopnea syndrome without oximetric repercussions.The patient has been followed-up with since 2019 for dysphonia and hypoacusia. During the physical exam, a small hyperemic polyp was identified on the anterior third of the right vocal cord. The patient was found to have a narrow nasal valve and a deep voice. An audiogram was performed, which detected signs of hypoacusia .In 2011, an upper endoscopy identified signs of gastritis and a peptic-like lower esophageal ring.In 2018, the patient was referred to the department of gastroenterology for the study of her dyspepsia. The patient complained of a longstanding intermittent diffuse abdominal discomfort and chronic constipation. Several lab and imaging studies did not identify any findings of interest.The patient has been followed by the department of gynecology since 1994 for discomfort in both breasts.In 1994, a bilateral mammogram report described very dense breasts with a predominant fibroglandular component compared to fatty tissue. Several cysts were seen in both breasts, the largest in the right breast of 18 × 14 mm. The patient was diagnosed as having bilateral fibrocystic mastopathy.In 2001, a new bilateral mammogram detected several 2 cm nodules in the left breast, disperse bilateral calcifications that were more evident in the left breast in relation to sclerotic adenosis. A fine needle aspiration of the left breast allows for the pathology diagnosis of fibroadenoma.In 2003, several cysts that resemble fibroadenomas were detected in both breasts.In 2006, several new benign nodules were detected. Biopsies of both breasts were obtained, and the results confirmed the diagnosis of fibrocystic mastopathy.In 2007, multiple bilateral cysts were observed, some with echogenic contents that gave them a solid aspect. A biopsy was taken for confirmation. The biopsy report stated duct ectasia and fibrocystic mastopathy.In 2011, multiple simple cysts were found in both breasts, some of them with a less characteristic liquid content, that were most likely fibromas.In 2016, a 2 cm well-defined solid nodule was found in the inferior quadrant of the left breast. The decision was made to carry out an ultrasound-guided core needle biopsy. The diagnosis was fibroadenoma.In 2011, the patient herself sought treatment as she had a complete loss of teeth and an extreme maxillary atrophy. She requested surgical intervention for the implant of teeth. However, an orthopantomography confirmed the high degree of atrophy, describing very fine alveolar edges and type IV maxillomandibular atrophy. Due to the state of the jaw bones, the surgical intervention was not considered .In 2018, during a routine lab analysis, a neutropenia of 900 was detected, leading to referral to the department of hematology. The patient did not have a history of fever or recurrent infections.She was diagnosed with a mild–moderate intermittent cyclic neutropenia of an unknown etiology, although this was possibly idiopathic, autoimmune or drug-induced.After the accident that took place in 2017, the patient required evaluation by the psychiatric team. She suffered from anxiety that negatively impacted her daily life. Her extreme dependency on other people for all activities made her feel sad and the appearance of nightmares about the accident caused her sleep disturbances. She described feelings of apathy regarding the impossibility of recovering her former life. The patient required medication in order to sleep and expressed difficulties for paying attention and concentrating and a certain hopelessness about her current situation. She presented no sensory perceptual alterations, and her judgement of reality was preserved.The patient was diagnosed with a prolonged adjustment disorder with clinical manifestations of depression and anxiety secondary to the pain and loss of autonomy after her traffic accident.The patient experienced significant changes in her lifestyle and the psychosocial spheres of her life after the 2007 accident. The functional limitations of her lower extremities did not allow her to carry out everyday activities independently or with any degree of autonomy. Activities such as bathing, cooking, getting dressed, and driving were activities she once carried out daily but were no longer possible. She could not drive and wore nighttime diapers to avoid unnecessary painful bathroom visits. She used a transportation crane for most of her mobilization. The levels of depression and anxiety that arose from these situations were high.To evaluate the degree of depression, we used the Patient Health Questionnaire (PHQ-9) , obtainiWhen it comes to rare diseases, one of the main problems that often arises is the late diagnosis. Approximately 80% of rare diseases have a genetic origin, thus, most of the time, the definitive diagnosis is only obtained after genetic sequencing. A prior diagnostic suspicion and a careful consideration of the observed clinical findings are essential. In the case of this specific disease, there is a useful clinical tool that was designed by Brennam et al.  that estPycnodysostosis  is also As one can see, there are clinical links between the two syndromes, which complicates making the diagnosis. The study of the complete phenotype of the disease, the observation of the clinical findings and, finally, genetic sequencing, are the key elements needed to arrive at the definitive diagnosis.The prognosis of patients affected by HCS depends on the severity of the disease, clinical complications and the degenerative progression of each patient. The generalized osteoporosis and the development of acroosteolysis will likely cause fractures, limit deambulation and lead to dependency for basic everyday activities. The most frequent complications in this disease include basilar invagination, which causes neurological alterations, or chest wall deformities that give way to ventilation restriction.The case we have described presents the three main features of Hajdu–Cheney syndrome.The phenotypic variability ,27,28, aThe degenerative nature  is evideSimilar cases of the acroosteolysis of the distal phalanges and the generalized osteoporosis that this patient presented are described in the majority of cases diagnosed with HCS that are published to date. The cases reported by Rosenmann et al. , Elias eDiagnostic reasoning is guided by observation and the radiological findings . BrennamDuring the first medical visits, the suspected possible diagnosis was pycnodysostosis  due to tOnce a suspected diagnosis is established, the definitive diagnostic confirmation must be determined by Gibofsky genetic sequencing . In thisHajdu–Cheney syndrome generally follows an autosomal-dominant inheritance pattern, as reported by Majewski et al. ; howeverOver the course of the follow-up of this patient, a series of findings comparable to previously described cases of this disease have reinforced the available knowledge on this disorder. At the same time, new aspects arise that may serve as a guide for future lines of research.The musculoskeletal alterations that are presented in this case, such as the generalized osteoporosis and the acroosteolysis of the distal phalanges, are two of the main manifestations of this syndrome that are also present in the majority of cases that have been studied, as Letchumanan et al. , StathopSiklar et al.  describeThe bilateral genu valgum that our patient presented has previously been described in different occasions by Willians  and WeleA frequent finding in Hajdu–Cheney syndrome is watch-glass nails, an element which is present in this case and that has also been described in the literature by Rosenmann et al. , among oThe disease progresses over time, a fact which is most noticeable in the deterioration of the musculoskeletal apparatus. This patient presented deformities of the hands, such as those described by Jiménez et al.  ShurtlefThe presence of hyperlordosis and hyperkyphosis in this case made walking progressively difficult. Initially, the patient was able to manage with the help of crutches, but this became no longer possible and she progressed to requiring a wheelchair for any kind of mobilization. In the case described by Rosenmann et al. , the samThe disabling coxarthrosis with acetabular protrusion is another sign of the degenerative nature of the disease as it can be seen how it is worsening as time passes. Otto pelvis is a sign detected in this case that has not been previously reported in any of the studied cases to date.Reviewing the complete radiological study that is available in the patient’s clinical record, there is a finding that is worth noting, although it was not referenced in any of the clinical reports that we reviewed: the presence of serpentine fibula. This finding gives the case certain peculiarities that should be mentioned.For some time, patients who presented serpentine fibula were diagnosed with serpentine fibula and polycystic kidney syndrome. Considering their phenotypes and the evident differences between those patients and patients diagnosed with HCS, the two syndromes we considered were completely independent diseases. Currently, owing to the advances in genetics, it has been demonstrated that the gene responsible for Hajdu–Cheney syndrome and the gene responsible for serpentine fibula and polycystic kidneys syndrome are one and the same. Therefore, the two entities constitute a single disease. Studies such as those by Fryns et al. , Ramos eBiopsies are a frequent method used for the analysis of bone alterations in this syndrome. Elias et al.  and IwayIn their study, Sargin et al.  mention The sensation of dysphonia and shortness of breath when talking leads to the diagnosis of a mild to moderate sleep apnea–hypopnea syndrome without oximetric repercussions. In the literature, there are cases that present with dyspnea as a consequence of chest wall deformities that limits ventilation capacity and increases the likelihood of infections. The cases described by Williams  and SasaHearing loss is another frequent sign of this syndrome, and it is present in this case and also in cases as described by Herrman et al. ).At a gastrointestinal level, intestinal malrotation and dysphagia are elements that are present in cases such as those described by Williams  and N. HFibrocystic mastopathy and fibroadenomas have not been reported in any of the cases in the literature to date. One of the few descriptions of gynecological issues in this syndrome was carried out by Nozaki , who preThe premature loss of teeth is another typical sign of HCS that has been considered on several occasions by different authors, including Shaw  and Lee The case of neutropenia without fever or the presence of infections that were found in this patient have not been previously described in relation to HCS; however, the cases reported by Shurtleff et al.  and ChawIn this case, there is a clear before and after in the progression of the disease related to the traffic accident the patient suffered in 2017. The patient was run over by a vehicle, causing her to fall and suffer multiple lesions. This event had a noticeable negative effect on her day to day life, leaving her completely disabled for most daily activities, increasing her level of dependency and further developing mental disorders such as stress, anxiety and depression.At this point, it is important to note that there are studies that justify the psychological burden and the increase in mental disorders that occur in patients with rare diseases .With regard to treatment, several aspects can be analyzed: pharmacological, surgical and self-care.The studies on pharmacological treatments for this syndrome do not present clear evidence on their efficacy. Relevant studies on pharmacological treatments for HCS include that by Sakka et al. , who worThe surgical intervention of flat feet that was performed in 1978 is part of the different surgeries that are mentioned in this case. Studies on surgical interventions in HCS are those by Mattei et al.  on cerviCurrent treatment for HCS is mainly centered on the management of the complications that arise and on the treatment of the underlying problems in order to improve the patient’s quality of life and life expectancy. In this case, several self-care recommendations were made, including maintaining an active as possible lifestyle, both physically and intellectually, and avoiding overweight as a preventative method against disease progression.The limitations we have identified in the development of this case study are directly related to the low specificity of the clinical studies that were performed. The lack of a general perspective on the progressive nature of the disease and a lack of awareness with regard to the disease itself have a negative impact on the follow-up of the case.It would be of scientific interest to perform a genetic study in order to identify the exact underlying mutation and relate it to the phenotype. Confirming whether or not the patient has polycystic kidneys would provide vital information for follow-up. Regarding diagnostic testing, an updated complete radiological control to assess the possibility of basilar invagination, a typical complication in this syndrome, is recommended. Another frequent complication is ventilation restriction due to thoracic deformities, so this should also be considered. At present, the patient complains of a severe pain in the occipital area that does not resolve with her usual treatment. She also states that she experiences excessive tiredness with minimal efforts. Both signs are compatible with the most frequent complications of this syndrome, as have been previously mentioned.The study of this case, in the context of general knowledge on Hajdu–Cheney syndrome, reinforces and justifies its three main features: phenotypic variability, age-dependent progression and acroosteolysis of the distal phalanges and generalized osteoporosis, all elements considered obligatory inclusion criteria for the diagnosis of this disease.However, a complete and updated description of the phenotype of this syndrome, which includes a large sample of cases, is still needed.The study of the hematological effects of HCS may constitute a possible future line of research of this syndrome.


Introduction: It is rare to find that wide QRS tachycardia automatically changes to narrow QRS tachycardia, and it is more difficult to clarify the mechanism.Case Report: A 3-month-old infant with recurrent paroxysmal supraventricular tachycardia underwent transesophageal cardiac electrophysiological examination. The wide QRS tachycardia was induced by atrial RS2 stimulation, and it soon changed to narrow QRS tachycardia automatically. By the accurate measurement of esophageal lead, it was found that the electrocardiogram changes completely conform to Coumel law. The mechanism of wide and narrow QRS tachycardia was orthodromic atrioventricular reentrant tachycardia with or without ipsilateral functional bundle branch block, and the accessory pathway was defined as the left free wall-concealed accessory pathway.Conclusion: Transesophageal cardiac electrophysiological examination can reveal some special electrophysiological phenomena, and its non-invasive nature is especially suitable for infants. Clinically, it is rare to find that wide QRS tachycardia automatically changes to narrow QRS tachycardia, and it is more difficult to clarify the mechanism. This article reports a case of a 3-month-old infant who was found to have the above-mentioned ECG changes during the transesophageal cardiac electrophysiological examination (TECEE). Through accurate measurement of the esophageal leads, we confirmed that the changes in the electrocardiogram conform to Coumel law, an important electrophysiological phenomenon, and clarified the mechanism of tachycardia.A 3-month-old infant, with a cough for 4 days and repeated paleness for 1 day, was admitted to the hospital. In the neonatal period, the infant was hospitalized in the neonatal department for sustained paroxysmal supraventricular tachycardia (SVT) and terminated with intravenous injection of adenosine triphosphate, and then followed by oral digoxin. After discharge, digoxin was discontinued because of the inadvisable opinion of the parent. The infant had a 6-year-old healthy brother and no history of tachycardia in his family. The mother of the infant was healthy during pregnancy, had no history of drug abuse, had no negative family background or events, and delivered at the age of 32. Admission examination of the infant revealed general conditions of wellness, a heart rate of 128 beats/min (bpm) with normal heart sound, regular cardiac rhythm and no heart murmur, as well as warm extremities,no swelling of the liver, and no edema in the lower limbs, and no abnormal results in the following routine: electrocardiogram, echocardiography, myocardial injury and serum electrolyte. After the family members signed the informed consent form, the child underwent a transesophageal cardiac electrophysiological examination. Due to recurrent tachycardia, the child underwent TECEE after the informed consent was signed by the parents.After intravenous injection of Midazolam (0.2 mg/kg), the patient was sedated. The 7F esophageal electrode was inserted through the nose, and the DF-5A cardiac electrophysiological stimulation system was used for transesophageal atrial pacing.2 program. When the program stimulus negative scan to the combined rhythm interval was 130 ms, the S2-R interval was not significantly prolonged, and the wide QRS tachycardia of the left bundle branch block was induced at 259 bpm  and wide QRS complex (excitation descending with the left or right bundle branch block). 2. It was suggested that the ipsilateral block bundle branch of the accessory pathway, the prolongation of the RR interval, was mainly the prolongation of the RP interval, but the PR interval remained the same when the RR interval with the bundle branch block was longer than that of narrow QRS wave RR interval >35 ms. It was suggested that the accessory pathway was located on the opposite side of the bundle branch block if the RR interval of the bundle branch block was equal to the RR interval of the narrow QRS complex.After zooming in the figure and slowing down the paper speed to 50 mm/s, we accurately measured the ECG in the esophageal lead. It was found that the RP interval was 112 ms and the PR interval was 120 ms in the wide QRS tachycardia. In the narrow QRS tachycardia, the RP interval was 76 ms and the PR interval was 120 ms. Both wide and narrow QRS tachycardia had the characteristics of RP interval less than the PR interval and RP interval was more than 70 ms, which was consistent with atrioventricular reentrant tachycardia. Further analysis showed that from wide QRS tachycardia to narrow QRS tachycardia, RP interval was shortened by 36 ms (more than 35 ms), while PR interval was completely unchanged. The change of QRS time limit was entirely due to the change of RP interval, which was in full compliance with Coumel law. Because Coumel law was only applicable to the bypass in the free wall of the left or right ventricle, and in the wide QRS tachycardia was a left bundle branch block pattern, the mechanism of wide and narrow QRS tachycardia was orthodromic atrioventricular reentrant tachycardia with or without the ipsilateral functional bundle branch block, and the accessory pathway was defined as the left free wall-concealed accessory pathway.In this case, a non-invasive cardiac electrophysiological examination was performed for synchronous stimulation and recording. The process of inducing, changing, and terminating tachycardia and the characteristics of sudden abruptness were fully displayed, and typical figures conforming to Coumel law were collected. The RP and PR intervals of QRS complexes with different widths and narrowness were accurately measured so as to clarify the mechanism of tachycardia in the infant, locate the accessory pathway, and determine the direction for the future treatment of the infant.Cardiac electrophysiological examination has important value for the determination of electrocardiographic phenomena , 4. CardTECEE can be carried out safely and effectively in infants and even newborns , which hAs far as we know, this is the first case report of automatic conversion of wide QRS to narrow QRS tachycardia in an infant, and the pathogenesis has been confirmed by the non-invasive examination method of TECEE.Transesophageal cardiac electrophysiological examination can actively induce some special electrophysiological phenomena, which can be reasonably explained through the analysis of esophageal lead. Its non-invasive nature makes it particularly suitable for infants.The original contributions presented in the study are included in the article/supplementary materials, further inquiries can be directed to the corresponding author/s.The studies involving human participants were reviewed and approved by the Ethics Committee of West China Second University Hospital, Sichuan University. Written informed consent to participate in this study was provided by the participants' legal guardian/next of kin. Written informed consent was obtained from the minor(s)' legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.KZ and LZ were responsible for the study design and manuscript preparation. SY, TW, and KZ were responsible for the clinical data. YH contributed to data acquisition. LZ wrote this manuscript. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.