[Treatment of emotion regulation problems in people with neurofibromatosis type 1]. / Behandeling van emotieregulatieproblemen bij mensen met neurofibromatose type 1.

Individuals with the genetic disorder neurofibromatosis type 1 (NF1) are typically diagnosed in a medical hospital setting strongly relying on the presence of well-defined physical symptoms such as neurofibromas or pigmentary spots (known as café-au-lait spots). In mental health care settings, however, aside from a few highly specialized centres, the diagnosis and treatment of individuals with NF1 receives little attention, while the need for psychological treatment is increasingly identified, both in clinical practice and in the scientific literature. Occasional referrals of individuals with NF1 to the mental health services are often only targeted at psychological assessment. Subsequent treatment, however, is usually lacking. We describe two individuals with NF1 for whom by means of specialized clinical neuropsychological assessment, participation in a tailored dialectical behavior therapy (DBT) skills training was indicated. We exposit how they were able to develop their skills and how they themselves and their significant others experienced the treatment.

The effectiveness and safety of dialectical behavior therapy for suicidal ideation and behavior in autistic adults: a pragmatic randomized controlled trial.

BACKGROUNDS: Many autistic people in mental health are suicidal. This study evaluated the effectiveness of dialectical behavior therapy (DBT) v. treatment as usual (TAU) in reducing suicidal ideation and suicide attempts. METHODS: At six Dutch mental health centers, 123 outpatients (18-65 years) with DSM-5 diagnosed autism spectrum disorder (ASD) and suicidal behavior were randomly assigned to the DBT intervention group (n = 63) or TAU control group (n = 60). Assessments were conducted at baseline, post-treatment at 6 months and 12-month follow-up. The primary outcomes were severity of suicidal ideation and frequency of suicide attempts. The severity of depression and social anxiety were secondary outcomes. RESULTS: At end-of-treatment, DBT significantly reduced both suicidal ideation (z = -2.24; p = 0.025; b = -4.41; s.e. = 197.0) and suicide attempts (z = -3.15; p = 0.002; IRR = 0.046; s.e. = 0.045) compared to TAU, but lost statistical significance at the 12-month follow-up. Depression severity significantly decreased with DBT (z = -1.99; p = 0.046: b = -2.74; s.e. = 1.37) remaining so at 12 months (z = -2.46; p = 0.014; b = -3.37; s.e. = 1.37). No effects were observed on social anxiety. Severe adverse events included two suicides in the TAU condition. CONCLUSIONS: DBT is an acceptable, safe, and short-term effective intervention to reduce suicidal ideation and suicide attempts in autistic adults with suicidal behavior.

Presumptive spontaneous hemothorax associated to thymic involution in a dog with steroid responsive meningitis-arteritis (SRMA).

OBJECTIVE: To describe an unusual case of spontaneous hemothorax resulting from thymic involution in a dog with suspected acquired bleeding dyscrasia associated with steroid-responsive meningitis-arteritis (SRMA). CASE DESCRIPTION: A 6-month-old spayed female Golden Retriever was referred due to the sudden onset of lethargy, fever (pyrexia), loss of appetite (anorexia), and moderate neck pain. These symptoms emerged six days after an ovariohysterectomy performed by the primary veterinarian. Upon admission, the patient exhibited pale mucous membranes, tachycardia (180 bpm), bilateral muffled heart sounds and tachypnea. Abdominal and thoracic point-of-care ultrasound (POCUS) were performed and revealed bilateral pleural effusion. Due to the patient's unstable condition, emergent thoracocentesis and transfusion of packed red blood cells was required. The initial work-up performed included a complete blood cell count (CBC), biochemistry profile, venous blood gas and coagulation panel (PT, APTT, fibrinogen). Pleural effusion analysis was compatible with hemothorax. Bloodwork was unremarkable including the initial coagulation panel. Further coagulation test was performed including buccal mucosal bleeding time, viscoelastic-based clot detection tests (TEG) and Von Willebrand factor antigen measurement. TEG revealed marked hyperfibrinolysis. Angiostrongylus vasorum and 4DX snap test were performed and yielded a negative result. Thoracic CT scan revealed the presence of a soft tissue-attenuating mass in the ventral mediastinum, thymic involution, and enlargement of the sternal and mediastinal lymph nodes. Therapy with tranexamic acid and corticosteroids at anti-inflammatory doses was initiated. Marked clinical improvement was observed within 24 hours, and after three days of hospitalization the patient was discharged. One month later, the dog was referred again for acute pyrexia, hyporexia, and neck pain which progressed to non-ambulatory tetraparesis. Neurological examination was compatible with C6-T2 lesion. MRI and cerebrospinal fluid analysis were performed and revealed a final diagnosis of steroid-responsive meningitis-arteritis (SRMA) with associated intramedullary hemorrhage. Corticosteroids were started again, and the patient showed a dramatic improvement over the next 24 hours. Three weeks after the diagnosis, the dog returned to a clinically normal state. The treatment was gradually tapered over the following months, guided by regular neurological and clinical examinations and CRP measurements, without any relapses. NEW OR UNIQUE INFORMATION: To the best of the author's knowledge, this is the first documented case of a dog experiencing spontaneous hemothorax as a result of thymic hemorrhage/involution which, in the absence of other identifiable diseases, was attributed to a hyperfibrinolytic state induced by a severe inflammatory disease such as SRMA.

Respuesta a la carta: Contestación a la carta «Actuación ante un caso de extubación accidental con paciente en posición de decúbito prono» / Reply to letter: Response to the letter «Anaesthesia management of accidental extubation in the prone position»

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Actuación ante un caso de extubación accidental con paciente en posición de decúbito prono / Anesthesia management of accidental extubation in the prone position

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Influencia de la anestesia general sobre el tronco encefálico / Influence of general anaesthesia on the brainstem

El papel que desempeña el tronco encefálico en el control del funcionamiento basal del organismo y los detalles sobre cómo la anestesia general puede influir sobre este aún no está completamente definido. Sin embargo, en cada anestesia general el anestesiólogo debe ser consciente de la interacción de los fármacos anestésicos y la función del tronco encefálico en relación con la homeostasis del organismo. Como resultado de esta interacción habrá cambios en el nivel de consciencia, los reflejos protectores del organismo, el ritmo respiratorio, la frecuencia cardíaca, la temperatura o la presión arterial entre otros. La función del tronco encefálico puede ser explorada usando 3 enfoques diferentes: a través de la exploración clínica, analizando los cambios en la actividad eléctrica del cerebro o mediante el uso de técnicas de neuroimagen. El presente artículo de formación continuada trata de la influencia de los efectos de los fármacos anestésicos sobre la función del tronco encefálico. Para ello se estudia la exploración clínica de los nervios craneales y de diversos arcos reflejos afectados, el análisis de las señales eléctricas, tales como los cambios electroencefalográficos, y lo que se sabe acerca del tronco encefálico a través del uso de técnicas de imagen, más concretamente a través de imágenes obtenidas por resonancia magnética funcional. El objetivo es proporcionar al anestesiólogo clínico una visión global de la interacción entre los cambios inducidos por los anestésicos relacionados con la función del tronco encefálico (AU)

The exact role of the brainstem in the control of body functions is not yet well known and the same applies to the influence of general anaesthesia on brainstem functions. Nevertheless in all general anaesthesia the anaesthesiologist should be aware of the interaction of anaesthetic drugs and brainstem function in relation to whole body homeostasis. As a result of this interaction there will be changes in consciousness, protective reflexes, breathing pattern, heart rate, temperature or arterial blood pressure to name a few. Brainstem function can be explored using three different approaches: clinically, analyzing changes in brain electric activity or using neuroimaging techniques. With the aim of providing the clinician anaesthesiologist with a global view of the interaction between the anaesthetic state and homeostatic changes related to brainstem function, the present review article addresses the influence of anaesthetic drug effects on brainstem function through clinical exploration of cranial nerves and reflexes, analysis of electric signals such as electroencephalographic changes and what it is known about brainstem through the use of imaging techniques, more specifically functional magnetic resonance imaging (AU)

Femoral and sciatic nerve block for knee arthroscopy in a patient with acute intermittent porphyria / Bloqueo nervioso femoral y ciático para una artroscopia de rodilla en paciente con porfiria aguda intermitente

Acute intermittent porphyria is an autosomal dominant disorder that results from a partial deficiency of porphobilinogen deaminase and that causes very severe symptoms. Attacks may be triggered by a series of drugs and by other factors that the anesthesiologist should be aware of in order to reduce morbidity and mortality. Our objective is to review anesthetic considerations in acute intermittent porphyria. We present the case of a patient diagnosed with acute intermittent porphyria who was scheduled for knee arthroscopy. The anesthetic technique used was a femoral and sciatic nerve block under sedation with an infusion of remifentanil. The surgery proceeded without incident and the patient was discharged home after 24h. We consider the use of a peripheral plexus block of the lower limb to have been the safest anesthetic technique for this patient (AU)

La porfiria aguda intermitente es una enfermedad autosómica dominante que resulta de un déficit de porfobilinógeno deaminasa y que causa síntomas muy severos. Los ataques se pueden desencadenar por fármacos y otros factores que el anestesiólogo debe conocer para reducir la morbilidad y la mortalidad. Nuestro objetivo es revisar las consideraciones anestésicas en la porfiria aguda intermitente. Presentamos el caso de una paciente diagnosticada de que porfiria aguda intermitente programada para una artroscopia de rodilla. La técnica anestésica realizada fue bloqueo nervioso femoral y ciático bajo sedación con perfusión de remifentanilo. La cirugía transcurrió sin incidencias y la paciente fue dada de alta a domicilio a las 24 h. Consideramos que el bloqueo nervioso periférico de la extremidad inferior es la técnica anestésica más segura para esta paciente (AU)

Impacto de una intervención para mejorar el uso del sondaje uretral y reducir las infecciones del tracto urinario / Impact of an intervention to improve indwelling urinary catheter use and reduce urinary tract infections

Objetivo. Evaluar el impacto de una intervención en la adecuación del uso y en la mejora del cuidado del sondaje uretral (SU), así como en la frecuencia de infección del tracto urinario asociada al SU (ITU-SU) en pacientes hospitalizados. Material y métodos. Estudio cuasi-experimental. En el periodo preintervención se midieron indicadores basales de uso del SU. La intervención consistió en educación sobre el SU y en la implementación de recordatorios para su retirada. En el periodo postintervención se evaluaron las mismas variables. Resultados. Se incluyeron 197 pacientes durante el periodo preintervención y 194 durante el periodo postintervención. La prevalencia (17,3% versus 15,3%) y los días de SU (4,8 ± 5,8 versus 4,3 ± 4,2) experimentaron un descenso no significativo. Se incrementaron las sondas correctamente prescritas (41,1% versus 61,9%; p < 0,001) y fijadas (0% versus 38,1%; p < 0,001) y descendieron las bolsas de diuresis en el suelo (26,4% versus 6,2%; p < 0,001). El incremento en la adecuación de la indicación del SU (86,8% versus 92,3%) y el descenso en la densidad de incidencia de ITU-SU (2,1 versus 1,2 episodios/1.000 días de SU) no fueron significativos, aunque superaron los estándares. Conclusiones. Tras la intervención se incrementó de forma significativa el número de SU correctamente prescritas y fijadas, y disminuyó las bolsas de diuresis en el suelo. La mejora en la indicación del SU y en la frecuencia de ITU-SU alcanzaron los estándares de calidad. Las actividades formativas y el uso de recordatorios mejoran la seguridad del paciente ingresado con SU (AU)

Objective. To evaluate the impact of an intervention regarding the adequate use and improvement in the care of indwelling urinary catheters (IUC) and the frequency of catheter-associated urinary tract infections (CAUTI) in hospitalised patients. Material and methods. A quasi-experimental study was performed. Basic data on the use of IUC were recorded before and after the intervention, which consisted of training on IUC use and the implementation of reminders for their removal. Results. There were 197 patients in the pre-intervention period and 194 in the post-intervention period. There was a non-significant decrease in the prevalence (17.3% versus 15.3%) and days with IUC (4.8 ± 5.8 versus 4.3 ± 4.2). There was an increase in adequately prescribed (41.1% versus 61.9%; P < .001) and attached IUC (0% vs 38.1%; P < .001), and a decrease in the urine collection bags on the floor (26.4% vs 6,2%; P < .001). The increase in the appropriate indications for IUC (86.8% vs 92.3%) and the decrease in CAUTI incidence density (2.1 vs 1.2 episodes/1,000 catheter days) were not significant, although above the standards. Conclusions. After the intervention there was a significant increase in the number of adequately prescribed and attached IUC, and a decrease in the number of urine collection bags on the floor. Improvement in IUC indication and frequency of CAUTI reached the quality standards. Educational activities and the use of reminders improve safety of hospitalised patients with IUC (AU)

Tratamiento conservador en el cáncer de mama. Análisis de 451 pacientes con seguimiento mínimo de 5 años / Conservative treatment for breast cancer. Analysis of 451 patients with a minimum follow-up of 5 years

Propósito: Analizar la evolución de las primeras 451 pacientes sometidas a tratamiento conservador por cáncer de mama, con un seguimiento mínimo de 5 años en el Instituto Valenciano de Oncología (IVO).Materia y métodos: Todas tenían carcinomas infiltrantes con un tamaño máximo de 3 cm. Fueron tratadas mediante tumorectomía ampliada, linfadenectomía axilar e irradiación completa de la mama. La sobreimpresión fue dada mediante radioterapia externa o braquiterapia, y el tratamiento sistémico el correspondiente a su estadio. Resultados: A 60 meses, la supervivencia global (SG) fue del 91 por ciento, el intervalo libre de enfermedad hasta las metástasis (ILE) fue del 83 por ciento, y el intervalo libre hasta la recidiva local (IRL) fue del 93 por ciento. En el estudio multivariado, son significativos en relación a la SG, el número de ganglios axilares afectos y el grado histológico. Para el ILE, lo son el tamaño tumoral y el número de ganglios axilares infiltrados y para el IRL, el estado hormonal pre y perimenopáusico de la paciente y el componente intraductal extenso en el tumor. Conclusiones: El tratamiento conservador del cáncer de mama es la técnica de elección en los estadios iniciales. Los parámetros que influyen en la supervivencia son los mismos que en el tratamiento radical. Las recidivas locales están en relación a la situación hormonal de las pacientes y el componente intraductal extenso del tumor (AU)