Adjustment to disease and quality of life in people with vascular Ehlers-Danlos and Loeys-Dietz syndromes : A mixed-method study

Vascular Ehlers-Danlos (vEDS) and Loeys-Dietz syndromes (LDS) are hereditary disorders of connective tissue having severe vascular complications (HDCTv) which lead to an increased risk of premature death. Little is known about the impact of the disease in patient's daily life. Sixteen HDCTv patients (vEDS = 9 and LDS = 7), 16 age and sex-matched hypermobile Ehlers-Danlos syndrome patients (hEDS) and 18 healthy subjects (HS), responded to self-questionnaires assessing psychosocial adjustment, quality of life (QoL), anxiety, depression, pain, fatigue and sleep problems. Patients with HDCTv were also interviewed in order to explore qualitatively their experience with the disease. Compared with HS, patients with HDCTv scored significantly higher on anxiety, depression, fatigue, sleep problems, and lower on QoL. Most HDCTv patients (93.8%) have optimal psychosocial adjustment. In addition, HDCTv patients scored higher on QoL and psychosocial adjustment, but lower in pain, fatigue, sleep problems, and depressive symptoms than hEDS patients. Four main themes were identified in qualitative analyses: living with HDCTv, knowledge/ignorance of the disease, health behaviors/self-care and coping strategies. Our results suggest that despite the negative impact of HDCTv on the patients' daily lives, overall, they present an optimal disease adjustment which points to appropriate coping strategies. More research in psychosocial aspects of people with these rare diseases are needed to confirm these results and better understand their needs

Evaluación de los riesgos psicosociales con el instrumento “CTCPS-MAC” -Batería para el Estudio de la Condiciones de Trabajo de Carácter Psicosocial en el departamento de Obras Públicas de un Ayuntamiento del Sur de Jalisco.

Objetivo: Describir las condiciones de trabajo de carácter psicosocial percibidas por el personal del departamento de obras públicas de un ayuntamiento del sur de Jalisco, México. Materiales y métodos: El estudio fue descriptivo y se realizó en una muestra de 53 empleados de diferentes áreas de la organización compuesta por 115 trabajadores. Se utilizó el “CTCPS-MAC -Batería para el Estudio de la Condiciones de Trabajo de Carácter Psicosocial”. Resultados: Se obtuvieron datos sociodemográficos de los participantes así como de las dimensiones y los factores de riesgo que componen cada una de ellas. Se encontró que si bien hay una percepción de que las condiciones de trabajo de carácter psicosocial se encuentran en niveles de bueno y muy bueno. Sin embargo, se presentan puntuaciones de regular a nocivo para algunos casos. Conclusión: La dimensión  contenido de trabajo es percibido por el 41.5% como regular también en tres de sus cuatro factores con 41.5% ambientes laborales, con 45.3% equipo y agentes físicos y, con 32.1% concepción de las tareas del puesto de trabajo registrando además casos en nivel nocivo que también presenta el factor papel o rol en la organización de la dimensión contexto de trabajo y el factor síntomas subjetivos y alteraciones de la salud – estados psicológicos- respuesta fisiológica de la dimensión desgaste psíquico por lo que se necesita un proceso de intervención

Scientific approach for ozone absorption in blood during systemic indirect endovenous ozonetherapy

Introduction. Reading reference ozone books from Dra. Menendez, Dra. Viebhan, Dra. Borrelli and Dr. Bocci, proper timing for mixing ozone in blood during autohemotherapy is not calculated in a scientific way, having only an estimation of it based on changes in the blood color, more related to oxygen absorption than on ozone itself. Material and methods. We decided to reproduce a reduced model of great autohemotherapy or recently renamed as systemic indirect endovenous ozonotherapy (SIEVO) by the World Federation of Ozone Therapy ? WFOT, using syringes to simplify the experiment. Our model consisted of a 20 mL syringe filled with 10 mL of blood withdrawn from healthy volunteers and mixed it gently but in controlled way with 10 mL medical ozone at different concentrations; after 5 and 8 seconds, the remaining gas was analyzed by an spectrophotometer based ozone detector to check the amount of ozone. Data were analyzed using a linear regression model. Results. Results show that even for 60 mcgr/mL ozone concentration, 8 seconds is enough to let all ozone absorbed in blood. Discussion and Conclusions. The experiment shows how quick ozone reacts with blood and claims for a trial with real SIEVO devices to achieve a real timing

Validation of the neuroconnective endophenotype questionnaire (NEQ): a new clinical tool for medicine and psychiatry resulting from the contribution of Ehlers–Danlos syndrome

IntroductionThe link between anxiety disorders and joint hypermobility syndrome (now under hypermobility spectrum disorders, which include hypermobile Ehlers–Danlos syndrome) has been widely replicated over the past 30 years and has grown beyond the initial nosological limits. To integrate clinical and research progress in this field, a new neuroconnective endophenotype (NE) and its corresponding instrument, the Neuroconnective Endophenotype Questionnaire (NEQ), have been developed. This new clinical construct, created with the active participation of patients, includes both somatic and psychological dimensions and symptoms and resilience items.MethodsThe NE includes five dimensions: (1) sensorial sensitivity, (2) body signs and symptoms, (3) somatic conditions, (4) polar behavioral strategies, and (5) psychological and psychopathological dimensions. The NEQ information is collected through four self-administered questionnaires (sensorial sensitivity, body signs and symptoms, polar behavioral strategies, and psychological characteristics) and a structured diagnostic part that should be completed by a trained observer. This hetero-administered part incorporates (a) psychiatric diagnoses (using structured criteria, e.g., MINI), (b) somatic disorders diagnosis, using structured criteria, and (c) assessment of joint hypermobility criteria.ResultsIn a sample of 36 anxiety cases with 36 matched controls, the NEQ obtained high scores for test–retest, inter-rater reliability, and internal consistency. As for predictive validity, cases and controls significantly differed in all five dimensions and hypermobility measurements.DiscussionWe can conclude that the NEQ has achieved acceptable reliability and validity values and, therefore, is ready to be used and tested in different samples. This original and consistent construct including somatic and mental items may improve clinical specificity, the search for more comprehensive therapies, and their genetic and neuroimaging bases

Autism, Joint Hypermobility-Related Disorders and Pain

Autism Spectrum Disorder (ASD) and Joint Hypermobility-Related Disorders are blanket terms for two etiologically and clinically heterogeneous groups of pathologies that usually appears in childhood. These conditions are seen by different medical fields, such as psychiatry in the case of ASD, and musculoskeletal disciplines and genetics in the case of hypermobility-related disorders. Thus, a link between them is rarely established in clinical setting, despite a scarce but growing body of research suggesting that both conditions co-occur more often than expected by chance. Hypermobility is a frequent sign of hereditary disorders of connective tissue (e.g., Ehlers-Danlos syndromes, Marfan syndrome), in which the main characteristic is the multisystem fragility that prone to proprioceptive and motor coordination dysfunction and hence to trauma and chronic pain. Considering the high probability that pain remains disregarded and untreated in people with ASD due to communication and methodological difficulties, increasing awareness about the interconnection between ASD and hypermobility-related disorders is relevant, since it may help identify those ASD patients susceptible to chronic pain

Joint Hypermobility, Anxiety, and Psychosomatics — The New Neuroconnective Phenotype

In this chapter, after summarizing the concept and diagnosis of the Joint Hypermobility (Hyperlaxity), we review case control studies in two directions: Anxiety in Joint Hypermobility and Joint Hypermobility in Anxiety disorders, studies in nonclinical samples, review papers, and one incidence study. Collected evidence tends to confirm the strength of the association described two and a half decades ago. Common mechanisms involved include genetics, autonomic nervous system dysfunctions, and interoceptive and exteroceptive processes. Considering clinical and nonclinical data, pathophysiological mechanisms, and present nosological status, we suggest a new Neuroconnective phenotype in which together around a common core Anxiety-Collagen hyperlaxity, it includes five dimensions: behavioral, psychopathology, somatic symptoms, somatosensory symptoms, and somatic illnesses. Somatic illnesses include irritable bowel, dysfunctional esophagus, multiple chemical sensitivity, dizziness or unsteadiness (central vestibular pattern), chronic fatigue, fibromyalgia, glossodynia, vulvodynia, hypothyroidism, asthma, migraine, temporomandibular dysfunction, and intolerances or food and drug hypersensitivity. It is envisaged that new descriptions of anxiety disorders and also of some psychosomatic conditions will emerge and different nosological approaches will be required

El síndrome de norte-sur, una complicación de la asistencia circulatoria con ECMO Venoarterial. Reporte de caso

We present the case of a 34-year-old male patient with a history of Marfan syndrome who was admitted to the emergency room for acute aortic regurgitation secondary to aneurysmal dilation of the ascending thoracic aorta. In the postoperative period, post-cardiotomy cardiogenic shock was documented, so circulatory support was initiated with peripheral Veno-Arterial ECMO, which developed hypoxemia due to bacterial pneumonia and data compatible with North-South syndrome. We present a review, non-conventional cannulation strategies and a diagnostic alternative for this entity.Presentamos el caso de un paciente masculino de 34 años con diagnóstico previo de síndrome de Marfan que ingresó por Insuficiencia aórtica aguda secundario a dilatación aneurismática de la aorta torácica ascendente. En el posoperatorio se documentó choque cardiogénico poscardiotomía por lo que se inició soporte circulatorio con ECMO venoarterial periférico, el cual desarrolló hipoxemia por neumonía bacteriana y datos compatibles con síndrome norte-sur. Presentamos una revisión, estrategias de canulación no convencional y una alternativa diagnóstica para esta entidad

Desarrollo y validación psicométrica de la escala de expectativas del consumo de alcohol en estudiantes hombres universitarios

El propósito de este estudio fue generar un instrumento que permita medir las expectativas del consumo de alcohol en población masculina universitaria de manera confiable y valida. Para lo cual se construyó una escala conformada por 29 reactivos con 4 opciones de respuesta; para realizar su validación psicométrica se aplicó a 189 estudiantes de licenciatura de la Ciudad de México, de entre los 18 y 24 años de edad, de los cuales todos fueron hombres estudiantes de alguna licenciatura. Se llevaron a cabo análisis para conocer el poder discriminativo de los reactivos; la confiabilidad y la estructura factorial válida para la población universitaria de la Ciudad de México. La escala final estuvo constituida por 22 reactivos, organizados en cinco factores: efectos positivos en el estado de ánimo, conducta social con otros, pasar momentos agradables con amigos, búsqueda de experiencias nuevas, reducción de la tensión.The main objective of this research was to generate a reliable and valid scale that Assessed alcohol's expectations in university's students in Mexico City. A scale was developed specifically for this purpose. The scale included 29 items with four options for answers (Totally agree, agree, disagree, and Totally disagree). The scale was applied to a sample of 189 university's students in order to obtain its psychometrical validation. The distribution of the participants was as follow: age between 18 to 24; all of them men: diverse majors and schools of university campus. A proper analysis took place in order to learn about the items discriminative power, reliability and valid factorial structure. The final scale was conformed by 22 items organized by five factors as follow: 1) positive effectts on mood, 2) social behavior with others, 3) live pleasant moments with friends, 4) looking for new experiences and 5) stress reduction, it explains 60of variance and has a reliability of Cronbach Alpha =0.89.Facultad de Humanidades y Ciencias de la Educació

Autism Spectrum Disorder and Ehlers-Danlos Syndrome - Hypermobility Type: A Case Report

Introduction: Autism Spectrum Disorder (ASD) and Ehlers-Danlos Syndrome – Hypermobility type (EDS-HT) can occur concomitantly, with the overlap of the characteristics of both conditions. Early diagnosis helps prevent suffering and injuries in patients with these conditions. Objective: To report the case of an adolescent with severe autism and EDS-HT, with musculoskeletal and extra-skeletal manifestations, and how it may affect the patient´s life of and his family. Case Report: This report shows the clinical history of the signs and symptoms of EDS-HT, mainly evidenced by generalized pain, fatigue, dislocations and motor disability, due to Joint Hypermobility (JH). These manifestations may not be noticed by the family members and health professionals, due to the communicative impairment that is part of the manifestations related to ASD. Conclusion: The early identification of signs and symptoms of EDS-HT in childhood could enable a better understanding of its manifestations in patients with ASD. It also enables the prevention of joint dislocations and the proposition of a proper ergonomic design for the home environment to minimize suffering of the patient and his family