Orthodontically Relevant Manifestations in People with Rare Diseases.

BACKGROUND: Approximately 15% of all rare diseases occur with orofacial manifestations. Symptoms and manifestations of relevance to orthodontists represent a considerable proportion of these diseases and require appropriate strategies for their treatment. This article provides an overview of the orthodontically relevant manifestations of rare diseases. MATERIAL AND METHODS: Overall, 3,639 rare diseases listed at the Orphanet, OMIM or Pubmed database were evaluated for orofacial manifestations. All rare diseases which were indicated with at least one orofacial manifestation were recorded in a database for rare diseases with orofacial manifestations called "ROMSE," which was developed by the authors. All the rare diseases were analysed with regard to orthodontically relevant orofacial manifestations, such as dysgnathia, changes in the number of teeth, failures of eruption, pathologies of bone metabolism or orofacial clefts. For all rare diseases with orthodontic relevance, an exact analysis was undertaken. RESULTS: The orthodontically relevant orofacial manifestation termed dysgnathia is described in 151 of 535 identified rare diseases (28.2%). In these 151 rare diseases, 15 different subforms of dysgnathia, in the sense of skeletal misdevelopments of the jaws but without dental abnormalities, were described. Also changes in the number of teeth (17.9%), orofacial clefts (27.6%), failures of eruption (8.4%) and pathologies of the bone (2.1%) were described. CONCLUSIONS: Orthodontics play an important role in the diagnosis and treatment of orofacial manifestations in rare diseases. Databases such as ROMSE are a first step toward providing valid information in publicly accessible databases.

Perception and knowledge of dental interns toward interdepartmental coordination for successful prosthodontic treatment: A pilot study.

BACKGROUND: The graduating dentist should be trained in providing treatment utilizing the interdisciplinary approach because of the rapid advancements and increase in patient expectations, which demands for collaboration between the different specialists. PURPOSE: A pilot study was undertaken with an aim to assess the perception and knowledge of dental interns toward interdepartmental coordination for successful prosthodontic treatment. SUBJECTS AND METHODS: Dental interns from two dental colleges in Sangli (India) participated in the study. A 24-item self-administered, structured closed-ended questionnaire was used to collect the data. Four questions assessed the perception and 20 questions assessed the knowledge, which were based on the four domains viz.: General, Endodontics, Orthodontics, and Prosthodontics. They were framed from case scenarios reporting to the Department of Prosthodontics, which required interdepartmental consultation. The questionnaire was validated before its application and reliability were also assessed. The final score for each question was calculated based on the correct responses. Descriptive analysis was calculated using the frequencies, percentages, and mean values by using SPSS 16 software. RESULTS: Among 117 interns who participated in the study, 79.5% reported that they lacked training in an interdisciplinary approach. Approximately, 96% reported that the curriculum should be designed to include interdisciplinary training. Nearly, 88% reported that specialist from different specialty should be posted in one interdisciplinary department. Around 60% reported that they did not have the confidence of treating the patient as a whole. When the overall mean scores were considered, the highest scores were obtained for the general domain (95.3) and the lowest for the prosthodontic domain (83.6). CONCLUSION: The dental interns perceived that they lacked training in interdisciplinary approach, and the curriculum should include interdisciplinary training.

Interdisciplinary Approach in Periodontal Management of Orthodontic Patient: Long-Term Follow-up.

Orthodontic therapy is becoming a mainstay for the correction of esthetic and functional disturbances in both young and older adults. Orthodontic forces act on the periodontium and can have wide-ranging effects, from beneficial to harmful, from being a hindrance to oral hygiene maintenance to causing irreversible periodontal breakdown. In some patients, the presence of periodontal conditions like gingival recession, before start of orthodontic therapy, raises many questions. Much controversy and confusion exist regarding the need for periodontal therapy, its sequencing, especially in case of recession in a young patient who needs orthodontic therapy. An interactive, interdisciplinary approach is essential in such cases for success. This case report illustrates the interdisciplinary management of a young patient with gingival recession with an eight-year follow-up of the case.

Systematic review of oral and craniofacial findings in patients with Fabry disease or Pompe disease.

Fabry disease and Pompe disease are rare lysosomal storage disorders that belong to a heterogeneous group of more than 200 distinct inborn metabolic diseases. Mutations followed by loss of function of enzymes or transporters that are localised in the acidic environment of the lysosome may result in degradation of many substrates, such as glycosaminoglycans, glycosphingolipids, glycogen, cholesterol, oligosaccharides, glycoproteins, and peptides, or the excretion of the products degraded by the lysosome. Our aim was to identify the oral signs and symptoms of Fabry disease and Pompe disease from a systematic review made using MEDLINE/PubMed, and a hand search for relevant articles, following the PRISMA guidelines. Both diseases show various craniofacial and oral changes, including supernumerary teeth, dental agenesis, angiokeratoma, and telangiectases in Fabry disease; and macroglossia, teeth fusion, and taurodontism in Pompe disease. Common clinical signs of Fabry disease include hyposalivation, hypohidrosis, and xerophthalmia, and a generally reduced physical resilience was apparent in patients with Pompe disease. Oral and craniofacial changes in patients with both diseases extend over their entire lifetime and can be detected even in an infant. Lysosomal storage diseases should be taken into consideration in the differential diagnosis of relevant diverse symptoms, because treatment, when available, is most effective when started early. The main therapeutic concepts are enzymatic replacement for Pompe disease, whereas patients with Fabry disease require additional oral chaperone treatment or enzyme replacement.

Development of a database to record orofacial manifestations in patients with rare diseases: a status report from the ROMSE (recording of orofacial manifestations in people with rare diseases) database.

The aim of this working group was to establish a ROMSE (recording of orofacial manifestations in people with rare diseases) database to provide clinicians, patients, and their families with better information about these diseases. In 2011, we began to search the databases Orphanet, OMIM® (Online Mendelian Inheritance in Man®), and PubMed, for rare diseases with orofacial symptoms, and since 2013, the collected information has been incorporated into a web-based, freely accessible database. To date, 471 rare diseases with orofacial signs have been listed on ROMSE, and 10 main categories with 99 subcategories of signs such as different types of dental anomalies, changes in the oral mucosa, dysgnathia, and orofacial clefts, have been defined. The database provides a platform for general clinicians, orthodontists, and oral and maxillofacial surgeons to work on the best treatments.

Cardiopatía isquémica. Consideraciones para la atención odontológica / Ischemic heart disease. Considerations for dental treatment

La cardiopatía isquémica es un padecimiento que se caracteriza por la falta de oxígeno del músculo cardiaco y es la principal causa de infarto de miocardio. Existen múltiples factores que predisponen al desarrollo de ésta como la obesidad, la hiperlipidemia, el sedenta- rismo, tabaquismo, diabetes e hipertensión. Dadas las características que configuran la fisiopatología de la cardiopatía isquémica, existen diversas consideraciones que deben ser tomadas en cuenta toda vez que el estomatólogo brinde atención a un paciente con este padecimiento. El objetivo del presente artículo es conocer todo lo relacionado con la fisiopatología de la cardiopatía isquémica, sus manifestaciones clínicas, su tratamiento médico y lo más importante, las consideraciones que deben tomarse en el consultorio dental cuando se atienda a un paciente que padezca esta condición (AU)

Ischemic heart disease is a condition characterized by a lack of oxygen in the heart muscle and is the main cause of myocardial infarction. There are multiple factors that predispose to the development of this, such as obesity, hiyperlipidemia, sedentary lifestyle, smoking, diabetes and hypertension. Given the characteristics that make up the pathophysiology of ischemic heart disease, there are various considerations that must be taken into account whenever the stomatologist provides care to a patient with this condition. The objective of this article is to know everything related to the pathophysiology of ischemic heart disease, its clinical manifestation, its medical treatment and most importantly, the considerations that must be taken in the dental office when caring for a patient with this condition (AU)

Orthodontic management of dentition in patients with periodontally compromised dentition.

BACKGROUND: An increasing number of adult patients are seeking orthodontic treatment to improve their dental appearance. However, special attention must be given to the periodontal status of the adults as periodontal disease and its sequel, such as pathologic migration of anterior teeth, result in esthetic and functional problems. In such adult patients, an interdisciplinary approach often offers the best option for achieving a predictable outcome to solve complex clinical problems. MATERIALS AND METHODS: A prospective study was carried out on 20 adult patients [mean age = 33.3 ± 4.52 (SD), 11 females and nine males] with periodontally compromised and malaligned dentition. Loe and Silness Gingival Index (GI), Ramfjord's Periodontal Disease Index (PDI) and Dental Aesthetic Index (DAI) were recorded at the start and after completion of treatment. RESULTS: Comparison of GI, PDI and DAI before and after completion of treatment showed statistically significant differences, indicating the relevance of combined orthodontic-periodontic treatment in periodontally compromised dentition (P < 0.01). CONCLUSION: The outcome of the study showed that an interdisciplinary approach is a simple solution for complex clinical problems arising as a sequel to periodontitis, such as pathological tooth migration, restoring function, esthetics and periodontal health.

Applications of stem cells in interdisciplinary dentistry and beyond: an overview.

In medicine stem cell-based treatments are being used in conditions like Parkinson's disease, neural degeneration following brain injury, cardiovascular diseases, diabetes, and autoimmune diseases. In dentistry, recent exciting discoveries have isolated dental stem cells from the pulp of the deciduous and permanent teeth, from the periodontal ligament, and an associated healthy tooth structure, to cure a number of diseases. The aim of the study was to review the applications of stem cells in various fields of dentistry, with emphasis on its banking, and to understand how dental stem cells can be used for regeneration of oral and non-oral tissues conversely. A Medline search was done including the international literature published between 1989 and 2011. It was restricted to English language articles and published work of past researchers including in vitro and in vivo studies. Google search on dental stem cell banking was also done. Our understanding of mesenchymal stem cells (MSC) in the tissue engineering of systemic, dental, oral, and craniofacial structures has advanced tremendously. Dental professionals have the opportunity to make their patients aware of these new sources of stem cells that can be stored for future use, as new therapies are developed for a range of diseases and injuries. Recent findings and scientific research articles support the use of MSC autologously within teeth and other accessible tissue harvested from oral cavity without immunorejection. A future development of the application of stem cells in interdisciplinary dentistry requires a comprehensive research program.

Clinical attachment level gain and bone regeneration around a glass ionomer restoration on root surface wall of periodontal pocket.

A case describing perio-restorative management of an accidental trauma in the mid portion of root on an upper left canine tooth following an ostectomy surgery is presented here. The traumatized root area was undergoing fast resorption and a chronic periodontal abscess had developed in relation to the lesion. The article illustrates the clinical and radiographic photo series of a periodontal flap surgery done to gain access into a subgingival region for the placement of Glass ionomer restoration on the root and its periodic follow up. The clinical condition of the area suggests 8 mm clinical attachment gain over the restoration and the review radiographs at definite intervals up to 18 months revealed evidence of consistent bone regeneration around the restoration. The article also highlights the various other possibilities, where this restorative material can be effectively used in conjunction with periodontal surgical procedures.

Amelogenesis imperfecta: A challenge to restoring esthetics and function.

Rehabilitation of complicated cases poses difficulty in clinical practice, both with respect to restoring function and with esthetics. One such clinical condition where the dentist has to give importance to proper planning of the treatment and execution of the plan is amelogenesis imperfecta (AI), a condition where both function and esthetics are accommodated. This article discusses both the functional and esthetic rehabilitation of a patient with AI. Both the esthetics and function were hampered in this patient due to the condition. As a result, the treatment was properly planned and executed. A number of treatment options are available for us today to treat such a case. There is no one technique to be followed as such. However, the aim was to properly diagnose the case and provide good function and esthetics to the patient.