Manejo odontológico de pacientes con el síndrome de Ehlers-Danlos: revisión narrativa / Dental management in patients with Ehlers-Danlos syndrome: a narrative review

El síndrome de Ehlers-Danlos es una enfermedad heredita- ria, producida por mutaciones cromosómicas que pueden llegar a tener un comportamiento autosómico dominante, recesivo o ligado al cromosoma X. Se caracteriza por defectos en las enzi- mas encargadas de la estructura y síntesis de colágeno. En vista de los 20 tipos de colágeno que existen, este síndrome es extre- madamente heterogéneo tanto en su presentación clínica como en su progresión y evolución. Dentro de los signos y síntomas habituales encontramos la hiperlaxitud articular, hiperelastici- dad de la piel e hiperequimosis de los vasos sanguíneos. Con relación a las complicaciones que pueden presentar es- tos pacientes, encontramos dislocaciones articulares, fragilidad en la piel, dolor articular, ruptura de grandes vasos sanguíneos, dificultad en la cicatrización y, en consecuencia, mayor inci- dencia de procesos infecciosos y de cicatrices poco estéticas. Presenta una incidencia de 1 caso cada 2.500-5.000 na- cidos vivos. Por ello, es fundamental que el odontólogo se encuentre familiarizado con el manejo médico-dental de estos pacientes, a fin de estar preparado para brindarles un trata- miento adecuado y responder ante las posibles complicacio- nes que se pueden presentar. En esta revisión se emplearon resultados extraídos manual- mente de artículos, indexados en las bases de datos PUBMED y EBSCO, que respondían a la búsqueda de los términos Ehlers-Danlos syndrome, dental management y oral surgery. El objetivo fue describir el manejo médico-odontológico del paciente con síndrome de Ehlers-Danlos hasta la fecha (AU)

Ehlers-Danlos syndrome is a hereditary disease, produced by chromosomal mutations that can have an autosomal behavior, which can be dominant, recessive or X-linked. It is characterized by defects in the enzymes responsible for the structure and syn- thesis of collagen. In view of the 20 existent types of collagen, this syndrome is extremely heterogeneous in its clinical presentation, as well as in its progression and evolution. Within the usual signs and symptoms, we find joint hyperlaxity, skin hyperelasticity and hyper-ecchymosis of the blood vessels. Regarding the complications that these patients can pres- ent, we find joint dislocations, skin fragility, joint pain, rupture of large blood vessels, difficulty in healing and, consequently, a higher incidence of infectious processes and unsightly scars. It presents an incidence of 1 case every 2.500-5.000 live births. Therefore, it is essential that the dentist is familiar with the medical-dental management of these patients, in order to be prepared to provide them with adequate treatment and re- spond to possible complications that may arise. In this review, results were manually extracted from ar- ticles, indexed in the PUBMED and EBSCO databases, that respond to the search for the terms Ehlers-Danlos syndrome, dental management and oral surgery. The aim was describing the medical-dental management of patients with Ehlers-Dan- los syndrome to date (AU)

Manejo odontológico de pacientes con el síndrome de Ehlers-Danlos. Revisión narrativa / Dental management in patients with Ehlers-Danlos syndrome. Narrative review

Resumen El síndrome de Ehlers-Danlos es una enfermedad hereditaria, producida por mutaciones cromosómicas que pueden llegar a tener un comportamiento autosómico dominante, recesivo o ligado al cromosoma X. Se caracteriza por defectos en las enzimas encargadas de la estructura y síntesis de colágeno. En vista de los 20 tipos de colágeno que existen, este síndrome es extremadamente heterogéneo tanto en su presentación clínica como en su progresión y evolución. Dentro de los signos y síntomas habituales encontramos la hiperlaxitud articular, hiperelasticidad de la piel e hiperequimosis de los vasos sanguíneos. Con relación a las complicaciones que pueden presentar estos pacientes, encontramos dislocaciones articulares, fragilidad en la piel, dolor articular, ruptura de grandes vasos sanguíneos, dificultad en la cicatrización y, en consecuencia, mayor incidencia de procesos infecciosos y de cicatrices poco estéticas. Presenta una incidencia de 1 caso cada 2.500-5.000 nacidos vivos. Por ello, es fundamental que el odontólogo se encuentre familiarizado con el manejo médico-dental de estos pacientes, a fin de estar preparado para brindarles un tratamiento adecuado y responder ante las posibles complicaciones que se pueden presentar. En esta revisión se emplearon resultados extraídos manualmente de artículos, indexados en las bases de datos PUBMED y EBSCO, que respondían a la búsqueda de los términos Ehlers-Danlos syndrome, dental management y oral surgery. El objetivo fue describir el manejo médico-odontológico del paciente con síndrome de Ehlers-Danlos hasta la fecha.

Abstract Ehlers-Danlos syndrome is a hereditary disease, produced by chromosomal mutations that can have an autosomal behavior, which can be dominant, recessive or X-linked. It is characterized by defects in the enzymes responsible for the structure and synthesis of collagen. In view of the 20 existent types of collagen, this syndrome is extremely heterogeneous in its clinical presentation, as well as in its progression and evolution. Within the usual signs and symptoms, we find joint hyperlaxity, skin hyperelasticity and hyper-ecchymosis of the blood vessels. Regarding the complications that these patients can present, we find joint dislocations, skin fragility, joint pain, rupture of large blood vessels, difficulty in healing and, consequently, a higher incidence of infectious processes and unsightly scars. It presents an incidence of 1 case every 2.500-5.000 live births. Therefore, it is essential that the dentist is familiar with the medical-dental management of these patients, in order to be prepared to provide them with adequate treatment and respond to possible complications that may arise. In this review, results were manually extracted from articles, indexed in the PUBMED and EBSCO databases, that respond to the search for the terms Ehlers-Danlos syndrome, dental management and oral surgery. The aim was describing the medical-dental management of patients with Ehlers-Danlos syndrome to date.

Repercusiones ocupacionales en personas con enfermedad renal crónica terminal que asisten a hemodiálisis: un marco de justicia ocupacional / Repercussões ocupacionais em pessoas com doença renal crônica terminal que fazem hemodiálise: um marco na justiça ocupacional / Occupational repercussions in people with end-stage chronic kidney disease who attend hemodialysis: an occupational justice framework

Resumen Objetivo Conocer las repercusiones ocupacionales que provoca el tratamiento de hemodiálisis en personas que padecen Enfermedad Renal Crónica Terminal (ERC) y de qué manera las políticas públicas en Chile, específicamente, Garantías Explícitas de Salud (GES) abordan las necesidades de esta población. Método Se utilizaron pautas de observación ambiental, notas de campo, entrevistas semiestructuradas a usuarios, usuarias y profesionales que se desempeñan en la Unidad de Hemodiálisis del Hospital Base de Valdivia, Chile. Además, se realizó un análisis documental que incluyó guías clínicas y normas técnicas de la Unidad de Hemodiálisis. Resultados Los datos se organizaron en 5 tópicos: Unidad de hemodiálisis Hospital Base Valdivia, Proceso de cambio y adaptación, Rutinas y hábitos, Roles y Suficiencia de políticas públicas. Se evidencian quiebres en las historias ocupacionales, dificultades en el proceso de adaptación y la necesidad de un abordaje integral que no es considerado por las políticas públicas. Conclusion Se propone la necesidad de un tratamiento multidisciplinario que considere un acompañamiento en el proceso de adaptación ocupacional, en especial en etapas tempranas después del diagnóstico, a fin de prevenir el impacto en la calidad de vida de esta población, así como la necesidad de desarrollar más estudios acerca del tema.

Resumo Objetivo Conhecer as repercussões ocupacionais do tratamento hemodialítico em pessoas com Doença Renal Crônica Terminal e como as políticas públicas no Chile, especificamente as Garantias Explícitas de Saúde atendem às necessidades dessa população. Método Foram utilizadas diretrizes de observação ambiental, notas de campo, entrevistas semiestruturadas com usuários, usuários e profissionais que atuam na Unidade de Hemodiálise do Hospital Base de Valdívia, Chile. Além disso, foi realizada uma análise documental que incluiu diretrizes clínicas e normas técnicas da Unidade de Hemodiálise. Resultados Os dados foram organizados em 5 tópicos: Unidade de hemodiálise da Base Hospitalar Valdívia, Processo de mudança e adaptação, Rotinas e hábitos, Papéis e Suficiência das políticas públicas. São evidenciadas quebras nas histórias ocupacionais, dificuldades no processo de adaptação e a necessidade de uma abordagem abrangente que não é contemplada pelas políticas públicas. Conclusão Propõe-se a necessidade de um tratamento multiprofissional que considere um acompanhamento no processo de adaptação ocupacional, principalmente nas fases iniciais após o diagnóstico, a fim de prevenir o impacto na qualidade de vida desta população, bem como a necessidade de desenvolver mais estudos sobre o assunto.

Abstract Objective To know the occupational repercussions caused by hemodialysis treatment in people suffering from Terminal Chronic Kidney Disease and how public policies in Chile, specifically, Explicit Health Guarantees address the needs of this population. Method Environmental observation guidelines, field notes, and semi-structured interviews with users and professionals who work in the Hemodialysis Unit of the Hospital Base de Valdivia, Chile were used. In addition, a documentary analysis was carried out that included clinical guidelines and technical standards of the Hemodialysis Unit. Results The data were organized into 5 topics: Hospital Base Valdivia hemodialysis unit, Process of change and adaptation, Routines and habits, Roles and Sufficiency of public policies. Breaks in occupational histories, difficulties in the adaptation process and the need for a comprehensive approach that is not considered by public policies are evidenced. Conclusion The need for a multidisciplinary treatment is proposed that considers an accompaniment in the process of occupational adaptation, especially in the early stages after diagnosis in order to prevent the impact on the quality of life of this population, as well as the need to develop more studies on the subject.

Evaluation of a telemedicine program in oral pathology and preventive oral examination in Hualqui Municipality, Chile

ABSTRACT: Introduction: A telemedicine program in oral pathology (TOP) and a preventive oral examination (POE) to identify oral lesions was implemented in Hualqui, Chile. The objective of this study is to describe the results of the TOP plus POE strategy at CESFAM Hualqui, Chile, during the period of 2016 to 2019. Materials and methods: A prospective cohort study was conducted with 152 Hualqui patients between 2016 and 2019. The following variables were recorded: diagnosis; injury diagnosis dates; date of teleconsultation; date and response of the specialist and subsequent control; waiting time for response (WTR) and waiting time for attention (WTA). Results: The WTR was 19.7 ± 12.8 days and WTA presented a median of 20 days (1−125) days. A total of 45 different oral pathologies were identified, the most frequent being stomatitis 8.6% (13), fibroma 8.6% (13) and mucocele 7.9% (12), and among the least frequent highlights three cases of oral cancer. Conclusions: The waiting periods until a response from the specialist and then until the face-to-face control were around 3 weeks each, most of the cases were identified through POE and correspond mostly to benign pathologies.

Estratificación de riesgo de muerte a mediano plazo de pacientes evaluados por enfermedad coronaria con SPECT de perfusión miocárdica bajo efecto de dipiridamol / Predictive value of dipyridamole myocardial perfusion tomography on survival

Background: Dipyridamole (DIP) is the most commonly employed pharmacological stressor for myocardial perfusion tomography (SPECT) in patients unable to reach an adequate work load. Aim: To assess the predictive capacity of DIP SPECT on survival. Material and Methods: We included 985 adults aged 66 ±11 years (45% women) with rest and DIP-SPECT. The main indications for the procedure were coronary artery disease (CAD) screening in 66% and known CAD in 33%. Participants were followed up for a median of 65 months (interquartile range 54 to 86 months). During the follow up, 261 deaths were recorded and 98% had a specified cause in their death certificate. Results: Myocardial SPECT was abnormal in 44% of participants. Transient ischemic defects were observed in 34%, fixed defects concordant with infarction in 27% and post-stress systolic dysfunction in 23%. Twenty five percent of deaths were attributable to cardiac or ischemic cause and 22% to cancer. In a bivariate analysis, the hazard ratio (HR) of death of any cause was lower in females and higher in the presence of CAD. The multivariate analysis showed that being older than 46 years increased the HR of death of any cause. In a bivariate analysis, the HR for cardiac death was higher when the myocardial SPECT showed ischemia, necrosis or left ventricular dilation. In the multivariate analysis, post-stress left ventricular systolic function was associated with a lower risk of cardiac death. Conclusions: An abnormal myocardial SPECT, perfusion abnormalities, left ventricular systolic function or dilation are independent predictors of cardiac death in these participants.

Carcinoma vesicular de células en anillo de sello: diseminación poco frecuente: reporte de un caso con tomografía computada por emisión de positrones con fluor 18-deoxiglucosa / Bone metastases of a gallbladder carcinoma detected by positron emission tomography/computed tomography: case report

Signet ring gallbladder carcinoma is a rare aggressive variant of mucinous adenocarcinoma with poor prognosis. Positron emission tomography/computed tomography (PET/CT) with Fluor18 deoxyglucose (F18-FDG) is a useful tool in the staging of gallbladder cancer. We report a 68 years old man with a surgically resected acute cholecystitis, whose biopsy was positive for signet ring cell gallbladder carcinoma. During surgery, locoregional lymph nodes, liver or peritoneal involvement were not detected. A PET/CT was performed for staging, finding multiple hypermetabolic lytic bone lesions. Percutaneous biopsy of a pelvis bone lesion, confirmed a metastasis of the tumor. In this case, the staging with PET/CT allowed the diagnosis of unsuspected bone metastases and was a useful tool for deciding the best site of biopsy for histologic confirmation.

Linfonodo centinela en cáncer de mama: correlación entre detección isotópica y quirúrgica / Pre and intraoperative scintigraphic detection of sentinel nodes in breast cancer

Background: Sentinel node detection localizes the first node that drains a malignant lesion aiming to detect tumor dissemination. Aim: To assess the yield of sentinel node detection in breast cancer, using pre or intraoperative scintigraphy. Material and Methods: Review of medical records of patients with breast cancer who had a scintigraphic detection of sentinel nodes. Lymph node scintigraphy and surgery were performed in the same day. Results: We studied 174 women aged 53 ± 13 years, operated with a diagnosis of breast cancer, including six highly suspicious lesions in the contralateral breast (totaling 180 studied breasts). Preoperative scintigraphy showed a sentinel node in 174 of 180 breasts (97%). Intraoperative gamma probe confirmed the presence of the sentinel node in the same 174 breasts and detected an additional one reaching a detection yield of 97%. Four patients in whom a sentinel node was not detected in the preoperative scintigraphy, had macrometastases. Frozen section biopsies were available in 177 of 180 breasts. Metastases were informed in 45 patients who underwent axillary lymph node dissection, plus one additional patient with a suspicious lesion. Conclusions: A high rate of sentinel node detection in the preoperative scintigraphy was observed. Most sentinel nodes not detected with nuclear medicine had macrometastases. In 71% of patients, the detection of sentinel node avoided axillary lymph node dissection.

Seguimiento clínico en pacientes con buena capacidad metabólica en la prueba de esfuerzo con SPECT miocárdico / Follow-up of patients with good exercise capacity in stress test with myocardial single-photon emission computed tomography (SPECT)

Background: The evaluation of coronary artery disease (CAD) can be performed with stress test and myocardial SPECT tomography. Aim: To assess the predictive value of myocardial SPECT using stress test for cardiovascular events in patients with good exercise capacity. Material and Methods: We included 102 males aged 56 ± 10 years and 19 females aged 52 ± 10 years, all able to achieve 10 METs and ≥ 85% of the theoretical maximum heart rate and at least 8 min in their stress test with gated 99mTc-sestamibi SPECT. Eighty two percent of patients were followed clinically for 33 ± 17 months. Results: Sixty seven percent of patients were studied for CAD screening and the rest for known disease assessment. Treadmill stress test was negative in 75.4%; 37% of patients with moderate to severe Duke Score presented ischemia. Normal myocardial perfusion SPECT was observed in 70.2%. Reversible defects appeared in 24.8% of cases, which were of moderate or severe degree (> 10% left ventricular extension) in 56.6%. Only seven cases had coronary events after the SPECT. Two major (myocardial infarction and emergency coronary revascularization) and 5 minor events (elective revascularization) ere observed in the follow-up. In a multivariate analysis, SPECT ischemia was the only statistically significant parameter that increased the probability of having a major or minor event. Conclusions: Nearly a quarter of our patients with good exercise capacity demonstrated reversible defects in their myocardial perfusion SPECT. In the intermediate-term follow-up, a low rate of cardiac events was observed, being the isotopic ischemia the only significant predictive parameter.

Are there any differences in the assessment of quality of life when children score the importance of what is asked to them?

Introduction. There are no studies that enable to explore the impact that might have the scoring of the importance that children assign to each dimension that encompass the assessment of their quality of life (QoL). Objective. To analyze the influence of the importance assigned by children to the different spheres of their lives when having to assess their quality of life. Material and Methods. Correlational, crosssectional design. The modified KIDSCREEN-27 questionnaire was used to evaluate the importance assigned by the child to each dimension. Results. A total of 300 schoolboys and 300 schoolgirls, with an average age of 12 years old, were evaluated. The dimension with the highest score was Peers, and the one with the lowest was Psychological well-being, both in the raw evaluation as in the weighted by importance ranking. In girls, the Physical well-being dimension scored the lowest. The dimensions Parent relations and School environment scored the highest in younger students. In girls, differences were found in the dimension that scored the lowest when they weighted the importance of the dimension; shifting the dimension with the lowest score from Psychological well-being to Physical well-being. Conclusion. There are differences in the perception of quality of life between boys and girls, the types of schools they attend and the different ages, which do not change when being weighted by importance.