Linking the Mechanics of Chewing to Biology of the Junctional Epithelium.

The capacity of a tissue to continuously alter its phenotype lies at the heart of how an animal is able to quickly adapt to changes in environmental stimuli. Within tissues, differentiated cells are rigid and play a limited role in adapting to new environments; however, differentiated cells are replenished by stem cells that are defined by their phenotypic plasticity. Here we demonstrate that a Wnt-responsive stem cell niche in the junctional epithelium is responsible for the capability of this tissue to quickly adapt to changes in the physical consistency of a diet. Mechanical input from chewing is required to both establish and maintain this niche. Since the junctional epithelium directly attaches to the tooth surface via hemidesmosomes, a soft diet requires minimal mastication, and consequently, lower distortional strains are produced in the tissue. This reduced strain state is accompanied by reduced mitotic activity in both stem cells and their progeny, leading to tissue atrophy. The atrophied junctional epithelium exhibits suboptimal barrier functions, allowing the ingression of bacteria into the underlying connective tissues, which in turn trigger inflammation and mild alveolar bone loss. These data link the mechanics of chewing to the biology of tooth-supporting tissues, revealing how a stem cell niche is responsible for the remarkable adaptability of the junctional epithelium to different diets.

Wnt/ß-catenin Signaling Controls Maxillofacial Hyperostosis.

The roles of Wnt/ß-catenin signaling in regulating the morphology and microstructure of craniomaxillofacial (CMF) bones was explored using mice carrying a constitutively active form of ß-catenin in activating Dmp1-expressing cells (e.g., daßcatOt mice). By postnatal day 24, daßcatOt mice exhibited midfacial truncations coupled with maxillary and mandibular hyperostosis that progressively worsened with age. Mechanistic insights into the basis for the hyperostotic facial phenotype were gained through molecular and cellular analyses, which revealed that constitutively activated ß-catenin in Dmp1-expressing cells resulted in an increase in osteoblast number and an increased rate of mineral apposition. An increase in osteoblasts was accompanied by an increase in osteocytes, but they failed to mature. The resulting CMF bone matrix also had an abundance of osteoid, and in locations where compact lamellar bone typically forms, it was replaced by porous, woven bone. The hyperostotic facial phenotype was progressive. These findings identify for the first time a ligand-independent positive feedback loop whereby unrestrained Wnt/ß-catenin signaling results in a CMF phenotype of progressive hyperostosis combined with architecturally abnormal, poorly mineralized matrix that is reminiscent of craniotubular disorders in humans.

Accelerating Socket Repair via WNT3A Curtails Alveolar Ridge Resorption.

Tooth extraction triggers alveolar ridge resorption, and when this resorption is extensive, it can complicate subsequent reconstructive procedures that use dental implants. Clinical data demonstrate that the most significant dimensional changes in the ridge occur soon after tooth extraction. Here, we sought to understand whether a correlation existed between the rate at which an extraction socket heals and the extent of alveolar ridge resorption. Maxillary molars were extracted from young and osteoporotic rodents, and quantitative micro-computed tomographic imaging, histology, and immunohistochemistry were used to simultaneously follow socket repair and alveolar ridge resorption. Extraction sockets rapidly filled with new bone via the proliferation and differentiation of Wnt-responsive osteoprogenitor cells and their progeny. At the same time that new bone was being deposited in the socket, tartrate-resistant acid phosphatase-expressing osteoclasts were resorbing the ridge. Significantly faster socket repair in young animals was associated with significantly more Wnt-responsive osteoprogenitor cells and their progeny as compared with osteoporotic animals. Delivery of WNT3A to the extraction sockets of osteoporotic animals restored the number of Wnt-responsive cells and their progeny back to levels seen in young healthy animals and accelerated socket repair in osteoporotic animals back to rates seen in the young. In cases where the extraction socket was treated with WNT3A, alveolar ridge resorption was significantly reduced. These data demonstrate a causal link between enhancing socket repair via WNT3A and preserving alveolar ridge dimensions following tooth extraction.

Utilidad del sistema Myo Plus en pacientes amputados transradiales / Use of the Myo Plus system in transradial amputation patients

Las amputaciones de miembro superior son infrecuentes pero provocan una gran discapacidad. Suelen ser de etiología traumática y en varones jóvenes menores de 40 años y activos. La frecuencia de la amputación de nivel transradial es del 35% de los casos, siendo la prótesis mioeléctrica la que aporta mayor funcionalidad. Un 25% de los pacientes abandona el uso de la prótesis, siendo la complejidad de su uso, mediante 2 electrodos convencionales, una de las causas más frecuentes del abandono. El sistema Myo Plus, con 8 canales de electromiografía, facilita al paciente el control protésico utilizando el miembro fantasma y consiguiendo los patrones de cierre, apertura y pronosupinación de la mano en un tiempo menor al convencional y más fácilmente. Además, conlleva un menor tiempo de rehabilitación, disminuyendo los costes y los desplazamientos innecesarios, sin incrementar el tiempo de fabricación del encaje (AU)

Upper limb amputations are uncommon but cause severe disability. It is usually of traumatic origin and in young males less than 40 years-old, and active. The frequency of amputation at transradial level is around 35% of cases, with the myoelectric prosthesis achieving greater functionality. Around 25% of the patients give up using of the prosthesis, due to its complexity of use by means of 2 conventional electrodes, one of the most common causes for giving up. The Myo Plus system with an 8-channel electromyograph helps the patient to control the prosthesis using the phantom limb and achieving the closing, opening, and pronation-supination patterns of the hand in a time less than the conventional one and more easily. It also leads to less rehabilitation time, decreasing costs and unnecessary travel, without increasing the time for the manufacture of the socket (AU)

[Use of the Myo Plus system in transradial amputation patients]. / Utilidad del sistema Myo Plus en pacientes amputados transradiales.

Upper limb amputations are uncommon but cause severe disability. It is usually of traumatic origin and in young males less than 40 years-old, and active. The frequency of amputation at transradial level is around 35% of cases, with the myoelectric prosthesis achieving greater functionality. Around 25% of the patients give up using of the prosthesis, due to its complexity of use by means of 2 conventional electrodes, one of the most common causes for giving up. The Myo Plus system with an 8-channel electromyograph helps the patient to control the prosthesis using the phantom limb and achieving the closing, opening, and pronation-supination patterns of the hand in a time less than the conventional one and more easily. It also leads to less rehabilitation time, decreasing costs and unnecessary travel, without increasing the time for the manufacture of the socket.

Is mitotic rate still useful in the management of patients with thin melanoma?

BACKGROUND: T1 melanoma substaging was recently modified by the American Joint Committee on Cancer (AJCC). Although sentinel lymph node (SLN) positivity is the most important prognostic factor in melanoma, there is a lack of consensus on whether SLN biopsy should be performed in patients with thin melanoma (≤1 mm). OBJECTIVE: The main aim of this study was to investigate predictors of SLN positivity in patients with thin melanoma, with a special emphasis on mitotic rate. A secondary aim was to evaluate survival in this group of patients. MATERIALS AND METHODS: Retrospective multicenter observational study with analysis of age, sex, tumour location, thickness, mitotic rate, regression and microscopic satellites. Predictive factors were identified using a classification and regression tree (CART) approach. Melanoma-specific survival according to SLN status was estimated using Kaplan-Meier curves. RESULTS: We analysed 203 patients with a melanoma ≤1 mm. Using the new AJCC staging criteria, the CART algorithm identified a 7.5% likelihood of SLN positivity in T1a patients. In the case of T1b melanoma, there was a 14.3% likelihood of SLN positivity in patients with a mitotic rate >1 mitosis/mm2 and a 3.2% likelihood in those with ≤1 mitoses/mm2 . None of the patients with T1b disease who had ≤1 mitoses/mm2 and regression had SLN positivity. In T1b patients, 5-year melanoma-specific survival was 98.7% in the SLN-negative group and 75% in the SLN-positive group (P = 0.05). When stratified by mitotic rate, survival was 100% for patients with a mitotic rate of ≤1 mitoses/mm2 and 91.4% for those with >1 mitosis/mm2 (P = 0.022). There were no deaths in the T1a subgroup. CONCLUSIONS: Sentinel lymph node metastasis was less common in patients with T1b melanoma who had a mitotic rate of ≤1 mitoses/mm2 . Performance of SLN biopsy should be carefully considered in this subgroup of patients, particularly considering the good prognosis.

[Obstetric hysterectomy in the General Hospital Dr. Aurelio Valdivieso: three-year review]. / Histerectomía obstétrica en el Hospital General Dr. Aurelio Valdivieso: revisión de tres años.

BACKGROUND: Post-cesarean hysterectomy is the most extensive procedure used during the postpartum stage. This is an indicator of quality substantially associated with extreme obstetric morbidity. OBJECTIVE: To determine the incidence, indications, and complications associated with obstetric hysterectomy in a hospital, after three years to implement the program of prevention and management of massive obstetric hemorrhage. MATERIALS AND METHODS: Observational, transversal, retrospective and descriptive study conducted from January 2011 to November 2013. We records patients who underwent obstetric hysterectomy, of any age and at any time during pregnancy were reviewed. The results are expressed as frequencies, percentages and central tendency measures. RESULTS: 38 patients were recorded with obstetric hysterectomy. We found prevalence of 18.4 per 10,000, and incidence per year of 1.7, 1.4 and 2.6 per 1,000 births for 2011, 2012 and 2013. The prevalence of post-cesarean hysterectomy was 63.05 per 10,000 while postpartum was 9.05 per 10,000 births. The only difference between scheduled and emergency surgery was operating time. The procedure is associated with anemia in postpartum 13 times and the main indications for the procedures were hypo/atony and placenta accrete. CONCLUSIONS: The prenatal diagnoses of placenta accrete and improvement in the use of blood products and surgical technique has eliminated maternal mortality by massive obstetric hemorrhage in the last three years at the General Hospital "Dr. Aurelio Valdivieso".

Repercusiones físicas, mentales y sociales del cuidador primario de adultos mayores con enfermedad vascular cerebral en el Hospital Juárez de México / Physical, mental and social repercussions of the primary caregiver of older adults with CVD at the Juárez de México hospital

El adulto mayor hospitalizado con dependencia total a causa de la Enfermedad Vascular Cerebral (EVC), que se determina como crónica y degenerativa, está incapacitado para la realización de las actividades básicas de la vida diaria, por lo cual requiere la atención continua de un familiar denominado cuidador primario quien le brinda apoyo en diferentes aspectos. De ahí, que el cuidador primario se afecte en los ámbitos físico, mental y social. En este sentido, se visualiza la figura de cuidador primario como el sujeto-objeto de investigación. OBJETIVO. Determinar las repercusiones en los ámbitos personal, familiar y social del cuidador primario que brinda atención a un adulto mayor con EVC. MÉTODO. Se realizó un estudio usando la técnica de investigación cualitativa, fenomenológica, exploratoria, descriptiva. Se eligió a 6 cuidadores primarios cuyo familiar adulto mayor (AM) presentara dependencia total a causa de EVC. Se les aplicó la Entrevista en Profundidad. Los resultados se analizaron de acuerdo con el instrumento: Análisis de contenido de tipo temático. RESULTADOS. Se encontró, a través de la codificación libre en el texto de las entrevistas, 3 categorías y 9 subcategorías. 1. Cambio en la vida del cuidador: a) Disminuyen sus ingresos económicos, debido a que el cuidador primario dedica el mayor tiempo al cuidado del AM, por ello abandona parcial o totalmente su actividad remunerada; b) Deja de cuidar su salud, al priorizar la atención del AM en detrimento de la propia, presenta alteraciones del sueño, fatiga, alimentación inadecuada lo cual impacta negativamente en su salud; c) Deja de socializar con familiares y amigos, la permanencia en el hospital hasta por 36 horas continuas aísla al cuidador primario de su contexto familiar. 2. Enseñanza y aprendizaje: a) Fuentes de adquisición de nuevos conocimientos, el cuidador primario aun cuando cuente con conocimientos previos para el cuidado, no está capacitado de manera formal para el desempeño de las actividades que debe desarrollar en el hospital, aprende observando, preguntando a otros cuidadores o profesionales y por ensayo y error, lo cual le genera angustia. No existe un programa que capacite al cuidador primario para brindar la atención adecuada al AM; b) Cuidando en el hospital, el cuidado en el hogar difiere en gran medida del cuidado en el hospital, sus actividades son de apoyo al personal de salud debido a que el cuidador primario no recibe una enseñanza formal para el cuidado, lo cual le genera estrés, además de que pudiera representar un riesgo para el AM; 3) La vida en el hospital: a) Percepción del cuidado, el cuidador primario reconoce que el personal médico brinda atención de calidad al AM, no así lo refiere del personal de enfermería, ya que en sus discursos evidencia que no le capacitan para el desempeño de sus actividades, además de percibir maltrato o tardanza para la atención, por otro lado, justifica lo anterior debido a que el personal es insuficiente y los pacientes numerosos; b) Vigilancia en el hospital, con base en los discursos se observó que la relación cuidador primario-personal de vigilancia es complicada porque éstos se apegan rigurosamente a la normatividad del hospital descuidando las normas morales y el trato digno a que tienen derecho los cuidadores primarios; c) Actividades del cuidador primario en el hospital, son las acciones físicas de cuidado, acompañamiento y organización de los relevos, sin embargo, la principal es la de acompañar al AM, aunque el cuidador primario puede ser un colaborador del equipo de salud; d) Espacio de socialización, la permanencia constante del cuidador primario en el hospital, convierte este sitio en el espacio de socialización, en el que convive con otros pacientes y sus familiares, personal de salud y otras personas creando nuevos vínculos para compartir experiencias y ayuda mutua. CONCLUSIÓN. Los resultados obtenidos sugieren que el cuidador primario es un colaborador fundamental para la atención del AM hospitalizado, de ahí que es necesario implementar en los planes de estudio de la Licenciatura en Enfermería un área de formación en la cual se contemple al cuidador primario como persona vulnerable que debe recibir atención al igual que su paciente y la enseñanza formal que le capacite para atender las necesidades del AM, por otro lado, es preciso establecer programas para incluirle en un plan de atención y salud básica.

The older adult hospitalized with total dependence because of Cerebral Vascular Disease (CVD) , which is determined as chronic and degenerative, is unable to perform basic activities of daily life, which requires continuous care of a family called primary caregiver who provides support in different aspects. Hence, the primary caregiver affect on the physical, mental and social fields. In this sense, the figure of primary caregiver as the subject-object research is displayed. OBJECTIVE. Determine the impact on personal, family and social areas of the primary caregiver who provides care to an older adult with CVD. METHOD. A study was conducted using qualitative research technique, phenomenological, exploratory, descriptive study was conducted. 6 primary caregivers whose family AM present total dependence because of CVD was chosen. It was applied in-depth interview. The results were analyzed according to the instrument: analysis of thematic content. RESULTS. It was found through the free coding in the text of the interviews, 3 and 9 sub-categories. 1. Change in the caregiver's life: a) reduce their income, because the primary caregiver spends more time taking care of AM, thus partially or totally abandon their remunerated activity; b) Stop health care, prioritizing the attention of AM detriment of their own, presents sleep disturbances, fatigue, inadequate food which negatively impacts on their health; c) Stop socializing with family and friends, stay in the hospital for up to 36 continuous hours isolates the primary caregiver of the family context. 2. Teaching and learning: a) Sources of acquiring new knowledge, the primary caregiver even when it has prior knowledge care is not formally trained to carry out the activities to be developed in the hospital, learns by watching, asking other caregivers or professionals and by trial and error, which generates anxiety. There is a program that enables the primary caregiver to provide adequate attention to AM; b) Taking care in hospital, home care differs greatly from hospital care, their activities are supportive health personnel because the primary caregiver does not receive a formal education for care, which generates stress, and that could pose a risk to the AM; 3) Life in the hospital: a) Perception of care, the primary caregiver recognizes that medical personnel providing quality care to AM, not so concerned nursing staff, since in his evidence speeches that do not train you for the performance of its activities, in addition to perceived abuse or delay for attention, on the other hand, justifies the above because the staff is insufficient and many patients; b) Monitoring in the hospital, based on the speeches was observed that the primary-care staff monitoring relationship is complicated because they are strictly adhere to the regulations of the hospital neglecting moral standards and fair treatment they are entitled to primary caregivers ; c) Activities of the primary caregiver in the hospital, are the physical actions of care, support and organization of the relays, however, the principal is to accompany the AM, although the primary caregiver can be a partner of the health team; d) Space socialization, constant permanence of the primary caregiver in the hospital, makes this site in the space of socialization, which coexists with other patients and families, health workers and others creating new links to share experiences and help mutual. CONCLUSION. The results suggest that the primary caregiver is a key partner for the attention of AM hospitalized, hence need to be implemented in the curricula of Nursing a training area in which the primary caregiver as a vulnerable person is contemplated which should receive attention as patient education and formal training enabling them to meet the needs of AM, on the other hand, we must establish programs to include him in a care plan and basic health.

Distonía del músico: fenomenología y desencadenantes vinculados a la ejecución musical / Focal dystonia in musicians: Phenomenology and musical triggering factors

Las distonías se definen como una contracción conjunta, sostenida e involuntaria de músculos agonistas y antagonistas, que puede causar torsión, movimientos involuntarios anormales repetitivos y/o posturas anormales. Un grupo especial de distonías son las conocidas como ocupacionales, que incluyen trastornos distónicos desencadenados por una actividad motora repetitiva, relacionada con la actividad profesional o tarea específica. Los músicos son una población especialmente vulnerable a este tipo de distonías que se presentan como una pérdida de coordinación y control motor voluntario de movimientos altamente entrenados en la interpretación musical. Nuestro objetivo es describir una serie clínica de distonías focales en músicos evaluados y tratados en nuestro centro. Pacientes y métodos: Se presentan los datos de una serie clínica de 12 músicos con distonía ocupacional; se describen sus antecedentes y fenomenología, así como su evolución después de de la terapia. Resultados: Antecedentes demográficos: edad promedio 34,8 ± 11,8 años, 10 hombres (83,3%) y 2 mujeres (16,7%). Antecedentes médicos: antecedentes traumáticos en segmento distónico, 6 pacientes (50%); antecedentes familiares de enfermedades neurológicas en parientes de primer grado, 6 pacientes (50%); antecedentes laborales según categoría musical, 8 pacientes (66,6%) eran músicos clásicos y 4 pacientes (33,3%) eran músicos populares. Fenomenología: El cuadro distónico se caracterizó por presentarse a una edad promedio de inicio de 28,2 ± 11,3 años (rango 18-57 años). En 11 pacientes el segmento afectado fue la mano (91,7%). De todos los músicos consultados, un total de 9 (75%) recibió terapia. En la mayoría de los pacientes se describen desencadenantes específicos de la ejecución musical, asociados a requerimientos de control motor fino. Cabe mencionar que el 50% de los músicos tratados mantuvo su actividad laboral o puesto en la orquesta a la que pertenecía. Conclusiones: La mayoría de nuestros hallazgos fenomenológicos son coherentes con la literatura actualmente disponible. Sin embargo, nos parece destacable la presencia de desencadenantes atribuibles a requerimientos específicos de la ejecución musical, ligados a la participación del control motor fino

Dystonias are defined as a joint sustained and involuntary contraction of agonist and antagonist muscles, which can cause torsion, repetitive abnormal involuntary movements, and/or abnormal postures. One special group of dystonias are those known as occupational, which include dystonia disorders triggered by a repetitive motor activity associated with a specific professional activity or task. Musicians are a population particularly vulnerable to these types of dystonia, which are presented as a loss of coordination and voluntary motor control movements highly trained in musical interpretation. Our aim is to describe a clinical series of focal dystonias in musicians evaluated and treated in our centre. Patients and methods: Data is presented on a clinical series of 12 musicians with occupational dystonia. Their history and phenomenology are described, as well as well as their outcome after therapy. Results: Demographic details: Mean age 34.8 ± 11.8 years, 10 males (83.3%) and 2 females (16.7%). Clinical history: History of trauma in dystonic segment, 6 patients (50%); family history of neurological diseases in first-degree relatives, 6 patients (50%); occupational history according to music category, 8 patients (66.6%) were classical musicians and 4 patients (33.3%) were popular musicians. Phenomenology: The dystonia syndrome was characterised by having a mean age of onset of 28.2 ± 11.3 years (range 18-57 years). The segment affected was the hand (91.7%) in 11patients. Of all the musicians seen in the clinic, 9 of them (75%) received therapy. The majority of patients appeared to have triggering factors specific to musical execution and linked to the requirement of fine motor control. It should be mentioned that 50% of the musicians treated maintained their professional activity or position in the orchestra to which they belonged. Conclusions: The majority of our phenomenological findings are consistent with those reported in the current literature. However, it is worth mentioning the presence of triggering factors attributed to the specific requirements of performing music, linked to the participation of fine motor control

Factores predictivos del estado del ganglio centinela en el melanoma cutáneo: análisis mediante un árbol de clasificación y regresión / Predictors of Sentinel Lymph Node Status in Cutaneous Melanoma: A Classification and Regression Tree Analysis

OBJETIVO: El objetivo principal de este estudio es identificar factores predictivos de la afectación metastásica del ganglio centinela (GC). Pacientes y método: Se trata de un estudio de cohortes retrospectivo realizado en 2 centros hospitalarios de tercer nivel. Se incluyeron un total de 818 pacientes. La medida de resultado principal fue la afectación del GC. La valoración de predictores independientes de esta afectación se realizó mediante una regresión logística múltiple y un árbol de clasificación y regresión (CART). RESULTADOS: El análisis de regresión logística múltiple mostró que la ulceración, el grosor tumoral y un alto índice mitótico (IM) (≥ 6 mitosis/mm2) se relacionaron con la afectación metastásica del GC de forma independiente. El CART mostró que el grosor de Breslow fue el factor más importante como predictor de la afectación linfática. Para los melanomas gruesos (> 2 mm) las variables predictoras fueron la ausencia de infiltrado inflamatorio, la edad y la localización. Para los melanomas menores de 2 mm las variables predictoras fueron el IM (> 6 mitosis/mm2), la ulceración y el grosor. El grosor tumoral, la edad, la localización y el IM fueron predictores de la supervivencia de estos pacientes. CONCLUSIÓN: Un alto IM se asocia con una mayor afectación metastásica del GC y una peor supervivencia. Con la metodología CART es posible una mejor predicción de la afectación metastásica regional con vistas al mejor manejo clínico de estos pacientes o su inclusión en ensayos clínicos. © 2014 Elsevier España, S.L.U. y AEDV. Todos los derechos reservados

OBJECTIVE: The main aim of this study was to identify predictors of sentinel lymph node (SN) metastasis in cutaneous melanoma. PATIENTS AND METHODS: This was a retrospective cohort study of 818 patients in 2 tertiarylevel hospitals. The primary outcome variable was SN involvement. Independent predictors were identified using multiple logistic regression and a classification and regression tree (CART) analysis. RESULTS: Ulceration, tumor thickness, and a high mitotic rate (≥6 mitoses/mm2) were independently associated with SN metastasis in the multiple regression analysis. The most important predictor in the CART analysis was Breslow thickness. Absence of an inflammatory infiltrate, patient age, and tumor location were predictive of SN metastasis in patients with tumors thicker than 2 mm. In the case of thinner melanomas, the predictors were mitotic rate (> 6 mitoses/mm2), presence of ulceration, and tumor thickness. Patient age, mitotic rate, and tumor thickness and location were predictive of survival. CONCLUSIONS: A high mitotic rate predicts a higher risk of SN involvement and worse survival. CART analysis improves the prediction of regional metastasis, resulting in better clinical management of melanoma patients. It may also help select suitable candidates for inclusion in clinical trials

Predictors of sentinel lymph node status in cutaneous melanoma: a classification and regression tree analysis.

OBJECTIVE: The main aim of this study was to identify predictors of sentinel lymph node (SN) metastasis in cutaneous melanoma. PATIENTS AND METHODS: This was a retrospective cohort study of 818 patients in 2 tertiary-level hospitals. The primary outcome variable was SN involvement. Independent predictors were identified using multiple logistic regression and a classification and regression tree (CART) analysis. RESULTS: Ulceration, tumor thickness, and a high mitotic rate (≥6 mitoses/mm(2)) were independently associated with SN metastasis in the multiple regression analysis. The most important predictor in the CART analysis was Breslow thickness. Absence of an inflammatory infiltrate, patient age, and tumor location were predictive of SN metastasis in patients with tumors thicker than 2mm. In the case of thinner melanomas, the predictors were mitotic rate (>6 mitoses/mm(2)), presence of ulceration, and tumor thickness. Patient age, mitotic rate, and tumor thickness and location were predictive of survival. CONCLUSIONS: A high mitotic rate predicts a higher risk of SN involvement and worse survival. CART analysis improves the prediction of regional metastasis, resulting in better clinical management of melanoma patients. It may also help select suitable candidates for inclusion in clinical trials.

[Focal dystonia in musicians: Phenomenology and musical triggering factors]. / Distonía del músico: fenomenología y desencadenantes vinculados a la ejecución musical.

Dystonias are defined as a joint sustained and involuntary contraction of agonist and antagonist muscles, which can cause torsion, repetitive abnormal involuntary movements, and/or abnormal postures. One special group of dystonias are those known as occupational, which include dystonia disorders triggered by a repetitive motor activity associated with a specific professional activity or task. Musicians are a population particularly vulnerable to these types of dystonia, which are presented as a loss of coordination and voluntary motor control movements highly trained in musical interpretation. Our aim is to describe a clinical series of focal dystonias in musicians evaluated and treated in our centre. PATIENTS AND METHODS: Data is presented on a clinical series of 12 musicians with occupational dystonia. Their history and phenomenology are described, as well as well as their outcome after therapy. RESULTS: Demographic details: Mean age 34.8 ± 11.8 years, 10 males (83.3%) and 2 females (16.7%). CLINICAL HISTORY: History of trauma in dystonic segment, 6 patients (50%); family history of neurological diseases in first-degree relatives, 6 patients (50%); occupational history according to music category, 8 patients (66.6%) were classical musicians and 4 patients (33.3%) were popular musicians. PHENOMENOLOGY: The dystonia syndrome was characterised by having a mean age of onset of 28.2 ± 11.3 years (range 18-57 years). The segment affected was the hand (91.7%) in 11 patients. Of all the musicians seen in the clinic, 9 of them (75%) received therapy. The majority of patients appeared to have triggering factors specific to musical execution and linked to the requirement of fine motor control. It should be mentioned that 50% of the musicians treated maintained their professional activity or position in the orchestra to which they belonged. CONCLUSIONS: The majority of our phenomenological findings are consistent with those reported in the current literature. However, it is worth mentioning the presence of triggering factors attributed to the specific requirements of performing music, linked to the participation of fine motor control.

Durable recovery of the macular architecture and functionality of a diagnosed age-related macular degeneration 1 year after a single intravitreal injection of dobesilate.

Among the age-related diseases that affect vision, age-related macular degeneration is the most frequent cause of blindness in patients older than 60 years. In this communication, we report the full anatomical and functional recovery of a patient diagnosed with wet age-related macular degeneration 1 year after a single intravitreal injection of dobesilate.

A pilot study of a telehealth intervention for persons with spinal cord dysfunction.

STUDY DESIGN: Single-blind randomized controlled trial of 6 months' duration. OBJECTIVES: To evaluate the efficacy of a novel telehealth intervention, 'CareCall', on reducing pressure ulcers and depression and enhancing the use of appropriate health care. SETTING: General community, Massachusetts and Connecticut, United States METHODS: 'CareCall' is an automated, interactive voice response system that combines patient education, cognitive behavioral interventions, screening and referrals, with alerts to a nurse telerehabilitation coordinator for direct non-emergent phone follow up. Participants consisted of a convenience sample of 142 persons with multiple sclerosis or spinal cord injury using a wheelchair >6 h per day. The intervention group received CareCall (n=71) The control group received usual care (n=71). The main outcome measures were: The pressure ulcer scale for healing tool, Patient Health Questionnaire-9 depression scale, Cornell Services Index and Craig Hospital Inventory of Environmental Factors-Short Form Question 5. RESULTS: CareCall achieved a reduction in presence of pressure ulcers at 6 months in women (P<0.0001). Among those with baseline depression, CareCall reduced 6-month severity of depression, adjusting for age and gender (P<0.047). CareCall did not have a significant impact on health-care utilization (OR=1.8, P=0.07), but did significantly improve participants' report of health-care availability (OR=2.03, P<0.04). CONCLUSION: This is the first study to demonstrate the efficacy of a largely automated telehealth intervention for adults with spinal cord dysfunction. Future research needs to replicate this study in a larger, multisite trial.

Ca2+ -activated K+ channel (KCa) stimulation improves relaxant capacity of PDE5 inhibitors in human penile arteries and recovers the reduced efficacy of PDE5 inhibition in diabetic erectile dysfunction.

BACKGROUND AND PURPOSE: We have evaluated the influence of calcium-activated potassium channels (KCa ) activation on cGMP-mediated relaxation in human penile tissues from non-diabetic and diabetic patients, and on the effects of PDE5 inhibitors on erectile responses in control and diabetic rats. EXPERIMENTAL APPROACH: Cavernosal tissues were collected from organ donors and from patients with erectile dysfunction (ED). Relaxations of corpus cavernosum strips (HCC) and penile resistance arteries (HPRA) obtained from these specimens were evaluated. Intracavernosal pressure (ICP) increases to cavernosal nerve electrical stimulation were determined in anaesthetized diabetic and non-diabetic rats. KEY RESULTS: Concentration-dependent vasodilation to the PDE5 inhibitor, sildenafil, in HPRA was sensitive to endothelium removal, NO/cGMP pathway inhibition and KCa blockade. Accordingly, activation of KCa with NS-8 (10 µM) significantly potentiated sildenafil-induced relaxations in HPRA (EC50 0.49 ± 0.22 vs. 5.21 ± 0.63 µM). In HCC, sildenafil-induced relaxation was unaffected by KCa blockade or activation. Potentiating effects in HPRA were reproduced with an alternative PDE5 inhibitor (tadalafil) and KCa activator (NS1619) and prevented by removing the endothelium. Large-conductance KCa (BK) and intermediate-conductance KCa (IK) contribute to NS-8-induced effects and were immunodetected in human and rat penile arteries. NS-8 potentiated sildenafil-induced enhancement of erectile responses in rats. Activation of KCa recovered the impaired relaxation to sildenafil in diabetic HPRA while sildenafil completely reversed diabetes-induced ED in rats only when combined with KCa activation. CONCLUSIONS AND IMPLICATIONS: Activation of KCa improves vasodilatory capacity of PDE5 inhibitors in diabetic and non-diabetic HPRA, resulting in the recovery of erectile function in diabetic rats. These results suggest a therapeutic potential for KCa activation in diabetic ED.

Treatment of dry age-related macular degeneration with dobesilate.

The authors present anatomical and functional evidences of dry age-macular degeneration improvement, after intravitreal treatment with dobesilate. Main outcomes measures were normalisation of retinal structure and function, assessed by optical coherence tomography, fundus-monitored microperimetry, electrophysiology and visual acuity. The effect might be related to the normalisation of the outer retinal architecture.

Causa infrecuente de hipertensión arterial en lactantes: neuroblastoma congénito quístico suprarrenal: caso clínico / A rare cause of hypertension in childhood: congenital cystic adrenal neuroblastoma: clinical case

The frequency of hypertension (HBP) in children has increased significantly over the past decade. The younger the patient the greater the likelihood of having secondary HBP. Thus, the main causes of hypertension in new-borns are of renovascular or parenchymatous origin. objective: To present the case of an infant with hypertension caused by a congenital cystic neuroblastoma (NB). Case History: Newborn with prenatal diagnosis of adrenal cyst, who evolved with significant hypertension unresponsive to medical therapy. Neuroblastoma was suspected on the basis of magnetic resonance imaging findings and resection of the lesion was able to resolve the hypertension and to confirm the diagnosis by anatomo-pathological study. Conclusion: Most cases of neonatal hypertension are of renal origin, with the 2 largest categories being renovascular and renal parenchymal diseases. NB is the most common neonatal malignancy. It usually presents as an abdominal mass of antenatal diagnosis, being the hypertension an unusual form of presentation.

La frecuencia de hipertensión arterial (HTA) en niños ha aumentado significativamente en la última década. A menor edad del paciente mayor es la probabilidad de que la HTA sea secundaria. Así, las principales causas de HTA en recién nacidos son de origen renovascular o parenquimatoso. objetivo: Presentar el caso de un lactante hipertenso por neuroblastoma (NB) congénito quístico. Caso Clínico: Recién nacido con diagnóstico prenatal de quiste suprarrenal, quien evolucionó con HTA por sobre el percentil 99 para edad, sexo y talla, sin respuesta a terapia farmacológica. La resonancia magnética permitió realizar el diagnóstico presuntivo de neuroblastoma congénito y la resección de la lesión permitió resolver la HTA y confirmar el diagnóstico. Conclusión: La HTA en recién nacidos generalmente se debe a causas secundarias. El NB es el tumor maligno neonatal más frecuente que se puede presentar como una masa abdominal de diagnóstico antenatal, siendo la HTA una forma infrecuente de presentación.

Distonía focales en los músicos / Focal dystonia in musicians

Introducción: Un grupo especial de distonía focal son las conocidas como ocupacionales, las que incluyen trastornos distónicos desencadenados por una actividad motora repetitiva, íntimamente relacionada con la actividad profesional o tarea específica que realiza el afectado. En este sentido los músicos son una población especialmente vulnerable a esta patología, que se presenta durante la ejecución de movimientos altamente entrenados. Objetivo: En este manuscrito se revisa la fisiopatología y sus implicancias terapéuticas.Desarrollo: Las bases fisiopatológicas de la distonía focal del músico aún no se conocen completamente. Sin embargo, gracias al aporte de estudios neurofisiológicos y de neuroimágenes funcionales, existe creciente evidencia de alteraciones en el procesamiento de información sensorial, integración sensorio-motora, procesos corticales y subcorticales de inhibición, que subyacen a esta patología.Desarrollo: Clínicamente, se caracteriza por aparición de contracción muscular involuntaria y se asocia a pérdida del control motor durante la ejecución musical. Es de aparición gradual y ocasionalmente pueden existir antecedentes de lesiones musculoesqueléticas o posturas no fisiológicas que anteceden la aparición de los síntomas. El examen neurológico es usualmente normal, aunque pueden desarrollarse posturas distónicas sutiles espontáneamente o con movimientos que involucran los segmentos afectados. La distonía permanece focal y no se generaliza.Conclusiones: El tratamiento se basa en la utilización de múltiples estrategias para el manejo de la distonía, con resultado variables. Si bien no se ha definido una terapia especifica, existen principios generales que se combinan en cada situación buscando obtener resultados. Esto incluye intervenciones desde una perspectiva farmacológica, manejo con toxina botulínica, técnicas de reentrenamiento sensorial, entre otras (AU)

Introduction: A special group of focal dystonia is that known as occupational, which include dystonic disorders triggered by repetitive motor activity, closely associated with the professional activity of a specific task that the affected person performs. In this sense, musicians are a population particularly vulnerable to this disorder, which is presented during the execution of highly trained movements. Objective: This article reviews the pathophysiology of focal dystonia and its therapeutic implications. Development: The pathophysiological basis of focal dystonia in the musician is still not well established. However, due to the contribution of neurophysiological studies and functional neuroimaging, there is growing evidence of anomalies in the processing of sensory information, sensory-motor integration, cortical and subcortical inhibitory processes, which underline this disease. Development: Clinically, it is characterised by the appearance of involuntary muscle contractions, and is associated with loss of motor control while practicing music. It is a gradual appearance and sometimes there may be a history of musculoskeletal injuries or non-physiological postures preceding the appearance of the symptoms. The neurological examination is usually normal, although subtle dystonic postures can develop spontaneously or with movements that involve the affected segments. The dystonia remains focal and is not generalised. Conclussions: Treatment is based on using multiple strategies for the management of the dystonia, with variable results. Although a specific therapy has not been defined, there are general principles that are combined in each situation looking for results. This includes, among others, pharmacological interventions, management with botulinum toxin, and sensory re-training techniques (AU)

Focal dystonia in musicians.

INTRODUCTION: A special group of focal dystonia is that known as occupational, which include dystonic disorders triggered by repetitive motor activity, closely associated with the professional activity of a specific task that the affected person performs. In this sense, musicians are a population particularly vulnerable to this disorder, which is presented during the execution of highly trained movements. OBJECTIVE: This article reviews the pathophysiology of focal dystonia and its therapeutic implications. DEVELOPMENT: The pathophysiological basis of focal dystonia in the musician is still not well established. However, due to the contribution of neurophysiological studies and functional neuroimaging, there is growing evidence of anomalies in the processing of sensory information, sensory-motor integration, cortical and subcortical inhibitory processes, which underline this disease. Clinically, it is characterised by the appearance of involuntary muscle contractions, and is associated with loss of motor control while practicing music. It is a gradual appearance and sometimes there may be a history of musculoskeletal injuries or non-physiological postures preceding the appearance of the symptoms. The neurological examination is usually normal, although subtle dystonic postures can develop spontaneously or with movements that involve the affected segments. The dystonia remains focal and is not generalised. CONCLUSIONS: Treatment is based on using multiple strategies for the management of the dystonia, with variable results. Although a specific therapy has not been defined, there are general principles that are combined in each situation looking for results. This includes, among others, pharmacological interventions, management with botulinum toxin, and sensory re-training techniques.

Life without a wall or division machine in Bacillus subtilis.

The cell wall is an essential structure for virtually all bacteria, forming a tough outer shell that protects the cell from damage and osmotic lysis. It is the target of our best antibiotics. L-form strains are wall-deficient derivatives of common bacteria that have been studied for decades. However, they are difficult to generate and typically require growth for many generations on osmotically protective media with antibiotics or enzymes that kill walled forms. Despite their potential importance for understanding antibiotic resistance and pathogenesis, little is known about their basic cell biology or their means of propagation. We have developed a controllable system for generating L-forms in the highly tractable model bacterium Bacillus subtilis. Here, using genome sequencing, we identify a single point mutation that predisposes cells to grow without a wall. We show that propagation of L-forms does not require the normal FtsZ-dependent division machine but occurs by a remarkable extrusion-resolution mechanism. This novel form of propagation provides insights into how early forms of cellular life may have proliferated.