A role for total alloplastic temporomandibular joint replacement in Gardner syndrome.

Gardner syndrome (GS) is a rare autosomal dominant disorder that can present with craniomaxillofacial abnormalities. The identification of osteomas or craniomaxillofacial abnormalities can therefore serve as a marker of this condition, facilitating early referral and diagnosis. A 17-year-old female with GS was referred for the management of severe limited mouth opening, causing a major problem for routine endoscopy to monitor the gastrointestinal alterations of GS. Clinical and radiological evaluations showed multiple osteomas in the mandibular angle, condylar and coronoid regions bilaterally and maximum mouth opening of 8 mm. The patient underwent surgery for osteoma removal and bilateral customized alloplastic total temporomandibular joint replacement (TMJ-TJR). At the 2-year follow-up, the patient showed improvements in quality of life, with a maximum mouth opening of 34 mm, allowing routine upper endoscopy to be performed. This is the first report of GS, a rare and challenging craniomaxillofacial abnormality, treated with TMJ-TJR. A comprehensive overview of the patient's clinical presentation, diagnostic assessment, treatment planning, and outcomes is provided.

The potential distribution of Bacillus anthracis suitability across Uganda using INLA.

To reduce the veterinary, public health, environmental, and economic burden associated with anthrax outbreaks, it is vital to identify the spatial distribution of areas suitable for Bacillus anthracis, the causative agent of the disease. Bayesian approaches have previously been applied to estimate uncertainty around detected areas of B. anthracis suitability. However, conventional simulation-based techniques are often computationally demanding. To solve this computational problem, we use Integrated Nested Laplace Approximation (INLA) which can adjust for spatially structured random effects, to predict the suitability of B. anthracis across Uganda. We apply a Generalized Additive Model (GAM) within the INLA Bayesian framework to quantify the relationships between B. anthracis occurrence and the environment. We consolidate a national database of wildlife, livestock, and human anthrax case records across Uganda built across multiple sectors bridging human and animal partners using a One Health approach. The INLA framework successfully identified known areas of species suitability in Uganda, as well as suggested unknown hotspots across Northern, Eastern, and Central Uganda, which have not been previously identified by other niche models. The major risk factors for B. anthracis suitability were proximity to water bodies (0-0.3 km), increasing soil calcium (between 10 and 25 cmolc/kg), and elevation of 140-190 m. The sensitivity of the final model against the withheld evaluation dataset was 90% (181 out of 202 = 89.6%; rounded up to 90%). The prediction maps generated using this model can guide future anthrax prevention and surveillance plans by the relevant stakeholders in Uganda.

The importance of a One Health approach for prioritising zoonotic diseases to focus on capacity-building efforts in Uganda.

Zoonotic diseases constitute a significant threat to the health of humans, livestock and wildlife, as well as to livestock production, and can also have a negative impact on our shared environment and on livelihoods. Uganda is a hotspot for emerging and re-emerging zoonotic disease threats, with the main drivers of this phenomenon being identified as agricultural intensification, proximity to wildlife reservoirs and climate change. The threat of zoonotic disease outbreaks affects not only human healthcare systems but also livestock production, food security, human capital development, wildlife health, environmental health and privatesector growth. Firstly, to prevent, control and mitigate the threat from zoonotic diseases, in March 2017, Uganda prioritised zoonotic diseases using a One Health approach that focuses the limited resources available on those diseases that have the greatest impact at the national level. The prioritised zoonotic diseases in question are anthrax, zoonotic influenza viruses, viral haemorrhagic fevers, brucellosis, trypanosomiosis (African sleeping sickness), plague and rabies. Secondly, in January 2018, Uganda published a National One Health Strategic Plan, which was developed after these zoonotic diseases had been prioritised. In addition, the Ugandan Government and its partners are currently collaboratively addressing several of these previously neglected, endemic zoonotic diseases, such as anthrax, brucellosis and rabies. As a result of these One Health efforts, capacity has increased for early detection of, reporting on and response to zoonotic diseases at all levels. To consolidate the achievements of the One Health approach, the Ugandan Government and its partners should continue to support capacity building for the prevention and control of zoonotic diseases.

Les maladies zoonotiques représentent une menace importante pour la santé publique, pour la santé du bétail et de la faune sauvage et pour la productivité du secteur de l'élevage ; en outre, elles ont un impact négatif sur notre environnement commun et sur les moyens de subsistance des populations. L'Ouganda est particulièrement exposé au risque d'émergence et de réémergence des menaces zoonotiques, les principaux facteurs de ce phénomène étant l'intensification agricole, la proximité des espèces sauvages faisant office de réservoirs et le changement climatique. Les menaces de foyers de maladie zoonotique n'affectent pas seulement les systèmes de santé publique mais aussi la production animale, la sécurité alimentaire, le développement du capital humain, la santé de la faune sauvage, la santé environnementale et la croissance du secteur privé. Afin de maîtriser et d'atténuer les menaces liées aux zoonoses, l'Ouganda a d'abord entrepris en mars 2017 de prioriser ces maladies au moyen d'une méthode Une seule santé destinée à recentrer les ressources disponibles, qui sont limitées, sur les zoonoses présentant le plus fort impact au niveau national. Les maladies ainsi définies comme prioritaires sont la fièvre charbonneuse, les virus influenza zoonotiques, les fièvres hémorragiques virales, la brucellose, la trypanosomose humaine africaine (maladie du sommeil), la peste et la rage. Dans un deuxième temps, l'Ouganda a publié en janvier 2018 son Plan stratégique national Une seule santé, en tenant compte des maladies zoonotiques définies comme prioritaires. En outre, le gouvernement ougandais et ses partenaires ont mis en place un cadre de collaboration en vue de traiter un certain nombre de maladies zoonotiques endémiques précédemment négligées telles que la fièvre charbonneuse, la brucellose et la rage. Grâce à ces efforts Une seule santé, l'Ouganda à pu renforcer ses capacités en matière de détection précoce des maladies zoonotiques, de notification et de réponse, et ce à tous les niveaux. Afin de consolider les résultats obtenus par l'approche Une seule santé, le gouvernement ougandais et ses partenaires devraient continuer à soutenir les efforts de renforcement des capacités destinées à la prévention et au contrôle des zoonoses.

Las enfermedades zoonóticas constituyen una importante amenaza no solo para la salud de personas, animales domésticos y fauna silvestre, sino también para la producción pecuaria, sin olvidar que pueden repercutir negativamente en el entorno natural que todos compartimos y en nuestros medios de sustento. Por lo que respecta al peligro que representan las enfermedades zoonóticas emergentes y reemergentes, cabe decir que Uganda es un «avispero¼, en el que convergen varios de los principales factores que alimentan el fenómeno: la intensificación de la agricultura, la cercanía de animales salvajes que ejercen de reservorio y el cambio climático. La amenaza de brotes de enfermedades zoonóticas afecta no solo a los sistemas de atención sanitaria sino también a la producción pecuaria, la seguridad alimentaria, el desarrollo del capital humano, la sanidad de los animales silvestres, la salud ambiental y el crecimiento del sector privado. En primer lugar, con el fin de prevenir, controlar y reducir la amenaza derivada de enfermedades zoonóticas, en marzo de 2017 Uganda definió un orden de prioridades entre esta clase de enfermedades, aplicando para ello la lógica de Una sola salud para concentrar los escasos recursos en aquellas enfermedades que tienen la mayor repercusión a escala nacional. Las enfermedades zoonóticas consideradas así prioritarias son: el carbunco bacteridiano, los virus gripales zoonóticos, las fiebres hemorrágicas víricas, la brucelosis, la tripanosomosis (enfermedad del sueño africana), la peste y la rabia. En segundo lugar, en enero de 2018 Uganda hizo público un Plan Estratégico Nacional de Una sola salud, que se elaboró tras haber definido como prioritarias las mencionadas enfermedades zoonóticas. Además, el Gobierno ugandés y sus asociados colaboran ahora en la lucha contra varias de estas enfermedades zoonóticas endémicas, hasta ahora desatendidas, como el carbunco, la brucelosis o la rabia. Gracias a estas actividades en clave de Una sola salud, el país se ha dotado de mayor capacidad para detectar rápidamente enfermedades zoonóticas, notificarlas y afrontarlas a todos los niveles. Ahora, para afianzar los resultados obtenidos, es preciso que el Gobierno del país y sus asociados sigan respaldando la labor de creación de capacidad para la prevención y el control de enfermedades zoonóticas.

Flotillin-1 interacts with the serotonin transporter and modulates chronic corticosterone response.

Aberrant serotonergic neurotransmission in the brain is considered at the core of the pathophysiological mechanisms involved in neuropsychiatric disorders. Gene by environment interactions contribute to the development of depression and involve modulation of the availability and functional activity of the serotonin transporter (SERT). Using behavioral and in vivo electrophysiological approaches together with biochemical, molecular-biological and molecular imaging tools we establish Flotillin-1 (Flot1) as a novel protein interacting with SERT and demonstrate its involvement in the response to chronic corticosterone (CORT) treatment. We show that genetic Flot1 depletion augments chronic CORT-induced behavioral despair and describe concomitant alterations in the expression of SERT, activity of serotonergic neurons and alterations of the glucocorticoid receptor transport machinery. Hence, we propose a role for Flot1 as modulatory factor for the depressogenic consequences of chronic CORT exposure and suggest Flotillin-1-dependent regulation of SERT expression and activity of serotonergic neurotransmission at the core of the molecular mechanisms involved.

Análisis de la calidad de vida en los pacientes afectos de escoliosis vertebral / Analysis of the quality of life in patients affected by scoliosis

Objetivos: Analizar la calidad de vida mediante el test SRS 22 en los pacientes con escoliosis de 20 o más grados Cobb. Material y método: Estudio descriptivo prospectivo realizado a pacientes con escoliosis de al menos 20 grados Cobb y con edad comprendida entre 10 y 20 años. Fue realizado entre abril y mayo de 2016.Se les registró peso, talla, índice de masa corporal y cuestionario SR 22, que es un cuestionario específico de calidad de vida para pacientes con escoliosis. Los pacientes se dividieron en 2 grupos para su análisis: a) escoliosis entre 20 y 29 grados Cobb (n=44); y b) escoliosis de 30 grados Cobb o superior (n=32). Resultados: Existen diferencias significativas en las dimensiones que evalúan el dolor, la autopercepción de la imagen y la satisfacción del tratamiento, siendo peor valoradas cuando el grado de escoliosis es de 30 grados Cobb o superior. No hay diferencias significativas en la función/actividad ni en la salud mental. La puntuación global del cuestionario también fue peor en el grupo con mayor grado de escoliosis. El peso, talla e índice de masa corporal no han mostrado diferencias significativas según el mayor o menor grado de escoliosis. Conclusiones: La escoliosis afecta de forma importante a la calidad de vida de las personas que la padecen, existiendo una correlación negativa entre la gravedad de la escoliosis medida mediante grados Cobb y la calidad de vida

Objectives: To assess the quality of life using the SRS 22 test in patients with scoliosis of 20 or more degrees Cobb. Material and methods: A prospective descriptive study was conducted between April and May 2016 on patients with scoliosis of at least 20 degrees Cobb and aged between 10 and 20 years. A record was made of weight, height, body mass index, and the SR 22 specific quality of life questionnaire for patients with scoliosis was completed. Patients were divided into two groups for analysis: a) scoliosis between 20 and 29 degrees Cobb (n=44); and b) scoliosis with a Cobb of 30 degrees or greater (n=32). Results: There were significant differences in the dimensions that assess pain, image self-perception, and satisfaction with treatment, being valued worse when the degree of scoliosis Cobb is 30 degrees or higher. There were no significant differences in function/activity or mental health. The overall score of the questionnaire was also worse in the group with the highest degree of scoliosis. The weight, height, and BMI showed no significant differences due to the varying degrees of scoliosis. Conclusions: Scoliosis significantly affects the quality of life of people who suffer it, and there is a negative correlation between the severity of scoliosis measured by degrees Cobb and quality of life

[Analysis of the quality of life in patients affected by scoliosis]. / Análisis de la calidad de vida en los pacientes afectos de escoliosis vertebral.

OBJECTIVES: To assess the quality of life using the SRS 22 test in patients with scoliosis of 20 or more degrees Cobb. MATERIAL AND METHODS: A prospective descriptive study was conducted between April and May 2016 on patients with scoliosis of at least 20 degrees Cobb and aged between 10 and 20 years. A record was made of weight, height, body mass index, and the SR 22 specific quality of life questionnaire for patients with scoliosis was completed. Patients were divided into two groups for analysis: a) scoliosis between 20 and 29 degrees Cobb (n=44); and b) scoliosis with a Cobb of 30 degrees or greater (n=32). RESULTS: There were significant differences in the dimensions that assess pain, image self-perception, and satisfaction with treatment, being valued worse when the degree of scoliosis Cobb is 30 degrees or higher. There were no significant differences in function/activity or mental health. The overall score of the questionnaire was also worse in the group with the highest degree of scoliosis. The weight, height, and BMI showed no significant differences due to the varying degrees of scoliosis. CONCLUSIONS: Scoliosis significantly affects the quality of life of people who suffer it, and there is a negative correlation between the severity of scoliosis measured by degrees Cobb and quality of life.

Rare primary leiomyosarcoma of the internal jugular vein with cervical extravascular extension.

Leiomysarcoma of intravascular origin is a rare malignant tumour of the soft tissue. We present what is, to our knowledge, the first example of one that has arisen from the wall of the internal jugular vein.

Choosing sheep (Ovis aries) as animal model for temporomandibular joint research: Morphological, histological and biomechanical characterization of the joint disc.

Preclinical trials are essential to the development of scientific technologies. Remarkable molecular and cellular research has been done using small animal models. However, significant differences exist regarding the articular behavior between these models and humans. Thus, large animal models may be more appropriate to perform trials involving the temporomandibular joint (TMJ). The aim of this work was to make a morphological (anatomic dissection and white light 3D scanning system), histological (TMJ in bloc was removed for histologic analysis) and biomechanical characterization (tension and compression tests) of sheep TMJ comparing the obtained results with human data. Results showed that sheep processus condylaris and fossa mandibularis are anatomically similar to the same human structures. TMJ disc has an elliptical perimeter, thinner in the center than in periphery. Peripheral area acts as a ring structure supporting the central zone. The disc cells display both fibroblast and chondrocyte-like morphology. Marginal area is formed by loose connective tissue, with some chondrocyte-like cells and collagen fibers in diverse orientations. Discs obtained a tensile modulus of 3.97±0.73MPa and 9.39±1.67MPa, for anteroposterior and mediolateral assessment. The TMJ discs presented a compressive modulus (E) of 446.41±5.16MPa and their maximum stress value (σmax) was 18.87±1.33MPa. Obtained results suggest that these animals should be considered as a prime model for TMJ research and procedural training. Further investigations in the field of oromaxillofacial surgery involving TMJ should consider sheep as a good animal model due to its resemblance of the same joint in humans.

Fenestrated internal jugular vein: a rare finding in neck dissection.

Fenestration of the internal jugular vein (IJV) is an extremely rare phenomenon. An 85-year-old woman was referred to our department with T2N0M0 squamous cell carcinoma of the right floor of the mouth. The patient underwent local resection, an ipsilateral modified type 3 radical neck dissection, and microsurgical reconstruction. During neck dissection, a fenestration was observed in the middle of the IJV. No structure passed through the fenestration. Such anatomical variations are rare, but clinicians and surgeons should be aware of them in order to avoid damage to the important vascular structures, especially if a neck dissection is performed.

Papel del médico de familia en la cumplimentación y registro del documento de instrucciones previas / Role of the family doctor in the completion and registration of advance directives documents

La actitud de los profesionales sobre el testamento vital no es homogénea y varía en función de la especialidad, la experiencia y de las propias creencias; todavía muchos médicos siguen teniendo miedo de incomodar a los pacientes. No es igual la situación que se plantea para el profesional en un hospital de agudos con un paciente relativamente desconocido que lo que pueda surgir en una unidad de cuidados paliativos o en la consulta del médico de familia que es el que atiende de forma integral al paciente. En atención primaria se dispone de una situación privilegiada para acercarnos a la vida y los valores de nuestros pacientes y sus familiares y no solo a la enfermedad, lo que hace que sea el lugar adecuado para orientar y asesorar al paciente sobre la preparación y registro del documento de últimas voluntades (AU)

The attitude of professionals about living wills (advance directives) is not homogenous and varies depending on the specialty, experience and beliefs. Many doctors are still afraid of inconveniencing patients. The situation confronting the professional in an acute care hospital with a relatively unknown patient in a palliative care unit is not the same as consulting a family doctor who is caring for the patient holistically. Primary care has a unique position to approach the life and values of our patients and their families and not just the disease, which makes it the right place to guide and advise the patient on the preparation and registration of living wills (AU)

[Role of the family doctor in the completion and registration of advance directives documents]. / Papel del médico de familia en la cumplimentación y registro del documento de instrucciones previas.

The attitude of professionals about living wills (advance directives) is not homogenous and varies depending on the specialty, experience and beliefs. Many doctors are still afraid of inconveniencing patients. The situation confronting the professional in an acute care hospital with a relatively unknown patient in a palliative care unit is not the same as consulting a family doctor who is caring for the patient holistically. Primary care has a unique position to approach the life and values of our patients and their families and not just the disease, which makes it the right place to guide and advise the patient on the preparation and registration of living wills.

Long-term effects of maternal immune activation on depression-like behavior in the mouse.

Depression is a debilitating mental disease affecting a large population worldwide, the pathophysiological mechanisms of which remain incompletely understood. Prenatal infection and associated activation of the maternal immune system (MIA) are prominently related to an increased risk for the development of several psychiatric disorders including schizophrenia and autism in the offsprings. However, the role of MIA in the etiology of depression and its neurobiological basis are insufficiently investigated. Here we induced MIA in mice by challenge with polyinosinic:polycytidylic phosphate salt-a synthetic analog of double-stranded RNA, which enhances maternal levels of the cytokine interleukin-6 (IL-6)-and demonstrate a depression-like behavioral phenotype in adult offsprings. Adult offsprings additionally show deficits in cognition and hippocampal long-term potentiation (LTP) accompanied by disturbed proliferation of newborn cells in the dentate gyrus and compromised neuronal maturation and survival. The behavioral, neurogenic and functional deficiencies observed are associated with reduced hippocampal expression of vascular endothelial growth factor (VEGF)A-VEGFR2. IL-6-STAT3-dependent aberrant VEGFA-VEGFR2 signaling is proposed as neurobiological mechanism mediating the effects of MIA on the developing fetal brain and ensuing consequences in adulthood.

Dorsalgia persistente en varón de 62 años: ¿dolor mecánico? / Persistent backache in a 62 year-old male: Mechanical pain?

Se trata de un varón de 62 años, con antecedentes de tuberculosis pulmonar, que consulta en varias ocasiones a su médico de atención primaria por dorsalgia resistente al tratamiento analgésico/antiinflamatorio, y que generalmente coincide con la actividad física. Inicialmente se diagnosticó de dolor de características mecánicas pero ante la falta de respuesta al reposo y tratamiento, se solicitó resonancia magnética de columna que informó de metástasis óseas. Posteriormente se deriva al paciente para estudio hospitalario al servicio de medicina interna. La anatomía patológica descartó la presencia de células neoplásicas, y en las pruebas de imagen se evidenciaron alteraciones radiológicas en cuerpos vertebrales (osteomielitis-discitis), asociadas a absceso epidural, compatibles con mal de Pott tuberculoso (AU)

The case is presented of a 62 year old man with a history of pulmonary tuberculosis, repeatedly consulting his primary care physician for treatment of analgesic / anti-inflammatory resistant back pain, and usually coincided with physical activity. It was initially diagnosed as mechanical pain, but in the absence of response to rest and treatment a magnetic resonance of the spine was requested, which reported bone metastases. The patient was then referred to Internal Medicine for further tests in hospital. The histopathology ruled out the presence of neoplastic cells, and imaging tests showed radiographic abnormalities in vertebral bodies (osteomyelitis-discitis) associated with epidural abscess, compatible with Pott’s disease, a kind of tuberculous arthritis of the intervertebral joints (AU)

[Persistent backache in a 62 year-old male: mechanical pain?]. / Dorsalgia persistente en varón de 62 años: ¿dolor mecánico?

The case is presented of a 62 year old man with a history of pulmonary tuberculosis, repeatedly consulting his primary care physician for treatment of analgesic / anti-inflammatory resistant back pain, and usually coincided with physical activity. It was initially diagnosed as mechanical pain, but in the absence of response to rest and treatment a magnetic resonance of the spine was requested, which reported bone metastases. The patient was then referred to Internal Medicine for further tests in hospital. The histopathology ruled out the presence of neoplastic cells, and imaging tests showed radiographic abnormalities in vertebral bodies (osteomyelitis-discitis) associated with epidural abscess, compatible with Pott's disease, a kind of tuberculous arthritis of the intervertebral joints.