Clinical practice guidelines for the diagnosis and management of Charcot-Marie-Tooth disease.

INTRODUCTION: Charcot-Marie-Tooth disease (CMT) is classified according to neurophysiological and histological findings, the inheritance pattern, and the underlying genetic defect. The objective of these guidelines is to offer recommendations for the diagnosis, prognosis, follow-up, and treatment of this disease in Spain. MATERIAL AND METHODS: These consensus guidelines were developed through collaboration by a multidisciplinary panel encompassing a broad group of experts on the subject, including neurologists, paediatric neurologists, geneticists, physiatrists, and orthopaedic surgeons. RECOMMENDATIONS: The diagnosis of CMT is clinical, with patients usually presenting a common or classical phenotype. Clinical assessment should be followed by an appropriate neurophysiological study; specific recommendations are established for the parameters that should be included. Genetic diagnosis should be approached sequentially; once PMP22 duplication has been ruled out, if appropriate, a next-generation sequencing study should be considered, taking into account the limitations of the available techniques. To date, no pharmacological disease-modifying treatment is available, but symptomatic management, guided by a multidiciplinary team, is important, as is proper rehabilitation and orthopaedic management. The latter should be initiated early to identify and improve the patient's functional deficits, and should include individualised exercise guidelines, orthotic adaptation, and assessment of conservative surgeries such as tendon transfer. The follow-up of patients with CMT is exclusively clinical, and ancillary testing is not necessary in routine clinical practice.

Indicación, realización y resultado del cateterismo de venas adrenales en pacientes con hiperaldosteronismo primario / Indication, performance and outcomes of adrenal vein sampling in patients with primary hyperaldosteronism

El hiperaldosteronismo primario (HAP) es una causa importante de hipertensión arterial (HTA) secundaria. El estudio del mismo precisa de una alta sospecha clínica, además de un estudio hormonal que confirme la hipersecreción hormonal. Es importante iniciar el tratamiento adecuado una vez se confirma el diagnóstico, y para ello es preciso demostrar si la hipersecreción hormonal es unilateral (pacientes que podrían ser tributarios a tratamiento quirúrgico) o bilateral (pacientes que son tributarios a tratamiento únicamente farmacológico). En el Hospital del Mar desde el año 2016 existe un equipo de trabajo multidisciplinar en el que participan nefrólogos, endocrinólogos, radiólogos y cirujanos para evaluar los casos con sospecha de hiperaldosteronismo y consensuar el mejor abordaje diagnóstico-terapéutico de estos pacientes, incluyendo la necesidad de cateterismo venoso adrenal, que es una técnica que en los últimos años se ha erigido como gold standard para el estudio del HAP. En el presente estudio recogemos la experiencia de nuestro centro en la realización de cateterismo venoso adrenal y en la utilidad de este para el manejo de tales pacientes (AU)

Primary hyperaldosteronism (PAH) is an important cause of secondary hypertension (HTN). The study of the same requires a high clinical suspicion in addition to a hormonal study that confirms hormonal hypersecretion. It is important to start the appropriate treatment once the diagnosis is confirmed, and for this is necessary to demonstrate whether the hormonal hypersecretion is unilateral (patients who could be candidates for surgical treatment) or bilateral (patients who are candidates for pharmacological treatment only). At the Hospital del Mar since 2016 there has been a multidisciplinary work team in which Nephrologists, Endocrinologists, Radiologists and Surgeons participate to evaluate cases with suspected hyperaldosteronism and agree on the best diagnostic-therapeutic approach for these patients, including the need for adrenal vein sampling, which is a technique that in recent years has become the gold standard for the study of PAH. In the present study we collect the experience of our center in performing adrenal vein catheterization and its usefulness for the management of these patients (AU)

Indication, performance and outcomes of adrenal vein sampling in patients with primary hyperaldosteronism.

Primary hyperaldosteronism (PAH) is an important cause of secondary hypertension (HTN). The study of the same requires a high clinical suspicion in addition to a hormonal study that confirms hormonal hypersecretion. It is important to start the appropriate treatment once the diagnosis is confirmed, and for this is necessary to demonstrate whether the hormonal hypersecretion is unilateral (patients who could be candidates for surgical treatment) or bilateral (patients who are candidates for pharmacological treatment only). At the Hospital del Mar since 2016 there has been a multidisciplinary work team in which Nephrologists, Endocrinologists, Radiologists and Surgeons participate to evaluate cases with suspected hyperaldosteronism and agree on the best diagnostic-therapeutic approach for these patients, including the need for adrenal vein sampling, which is a technique that in recent years has become the gold standard for the study of PAH. In the present study we collect the experience of our centre in performing AVC and its usefulness for the management of these patients.

Diagnosis and management of isthmocele (Cesarean scar defect): a SWOT analysis.

The purpose of this State-of-the-Art Review was to provide a strategic analysis, in terms of strengths, weaknesses, opportunities and threats (SWOT analysis), of the current evidence regarding the management of uterine isthmocele (Cesarean scar defect). Strengths include the fact that isthmocele can be diagnosed on two-dimensional transvaginal ultrasound, and that surgical repair may restore natural fertility potential and prevent secondary infertility, as well as reduce the risk of miscarriage and other obstetric complications. However, there is a lack of high-quality evidence regarding the best diagnostic method and criteria, as well as the potential benefits of surgical repair with respect to fertility. There is a need for experienced surgeons skilled in the various isthmocele repair techniques. Isthmocele repair does not prevent the need for Cesarean delivery in subsequent pregnancies. There is increasing awareness regarding the accuracy of transvaginal ultrasound in diagnosing isthmocele. This may lead to surgical correction and prevention of obstetric and perinatal complications in subsequent pregnancies, including Cesarean scar pregnancy. Regarding threats, the existence of different surgical techniques means that there is a risk of selecting an inadequate approach if the type of isthmocele and the patient's characteristics are not considered. There is a risk of overtreatment when asymptomatic defects are repaired surgically. Finally, there is an absence of cost-effectiveness analyses to justify routine repair. Thus, while there are many data suggesting that isthmocele has an adverse effect on both natural fertility and the outcome of assisted reproduction techniques, high-quality evidence to support surgical isthmocele repair in all asymptomatic patients desiring future fertility are lacking. There is increasing agreement to recommend hysteroscopic repair of isthmocele as a first-line approach as long as the residual myometrial thickness is at least 2.5-3.0 mm. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Mejora del conocimiento acerca del manejo de inhaladores tras el taller impartido en las Jornadas SEFAC 2021 / Improved knowledge of inhaler management following the workshop at the SEFAC 2021 conference

JUSTIFICACIÓN: el correcto uso de los inhaladores es imprescindible para alcanzar la adherencia terapéutica (saber usar correctamente los inhaladores no siempre implica que se usen con la frecuencia óptima).OBJETIVOS: realizar mediante un taller práctico una revisión de los dispositivos de inhalación más frecuentes y comparar la preparación del farmacéutico comunitario en cuanto a su manejo para valorar su aprendizaje.MATERIAL Y MÉTODOS: el taller se impartió de forma presencial en quince ciudades. Hubo dos sesiones a elegir, mañana o tarde, con una duración de 50 minutos. Como apoyo para resolver los casos prácticos, se ha elaborado una parte teórica, a la que pudieron acceder los asistentes a través de la App de las Jornadas. Durante el taller se resolvieron 5 casos prácticos de forma amena y participativa, aclarando las posibles dudas.Los asistentes respondieron a cinco preguntas de impacto antes y después y se compararon las respuestas. Tamaño muestral: Para potencia del 90,0%, significación del 5%, y una mejora de conocimientos esperada del 30% fue necesario incluir 109 sujetos. Análisis de los resultados: Mediante programa informático G-Stat®. Test Chi-cuadrado.RESULTADOS: se celebraron 30 talleres, dos en cada ciudad de las Jornadas SEFAC 2021, en los que participaron 308 FC. (AU)

Farmacovigilancia de las vacunas frente a la COVID-19 en farmacias comunitarias II. Reacciones adversas en farmacéuticos y personal auxiliar / Pharmacovigilance of COVID-19 vaccines in community pharmacies II. Adverse reactions in pharmacists and auxiliary personnel

OBJETIVO: conocer tipo y frecuencia de las sospechas de reacciones adversas (RA) experimentadas entre los farmacéuticos comunitarios (FC) y personal auxiliar de las farmacias de Pontevedra con la primera dosis de la vacuna y su repercusión laboral y personal.MÉTODOS . Diseño: estudio observacional transversal, febrero-abril de 2021.Sujetos: farmacéuticos, técnicos y auxiliares de la provincia de Pontevedra que ejercen en contacto con los pacientes, vacunados con la primera dosis frente al SARS-CoV-2, que dieron su consentimiento explícito. Variables: número, tipo y frecuencia de RA, repercusión. Procedimiento: se habilitó un formulario en la web del Colegio de Farmacéuticos de Pontevedra. Se anunció a los FC colegiados de la provincia su existencia y la conveniencia de cumplimentarlo. Tamaño muestral: teniendo en cuenta la metodología de incorporación al estudio, mediante cumplimentación voluntaria del cuestionario, no se consideró procedente. Análisis de resultados: test de chi- cuadrado para el análisis de variables cualitativas, t de Student y Mann-Whitney para cuantitativas. La normalidad comprobada mediante Kolmogorov-Smirnov con corrección de Lilliefors. Significación estadística p<0,05.Resultados 153 participantes manifestaron síntomas compatibles con reactividad a la vacuna, 122 (93,1 %) mujeres y 31 (86,1 %) hombres. 146 (95,4 %) que recibieron Vaxzevria® (VZ), 116 (79,4 %) mujeres y 30 (20,6 %) hombres, y 7 (4,6 %) Comirnaty® Pfizer-BioNtech (CO), 6 (85,7 %) mujeres y 1 (14,3 %) hombres. Se comunicaron 823 RA, 811 (98,5 %) con VZ y 12 (1,5 %) con CO. Reacciones más frecuentes: dolor en punto de inyección, 128 (87,7 %); escalofríos, 107 (73,3 %); dolor muscular, 106 (72,6 %). El número máximo de RA manifestadas por un participante fue 12. (AU)

The Mars Environmental Dynamics Analyzer, MEDA. A Suite of Environmental Sensors for the Mars 2020 Mission.

NASA's Mars 2020 (M2020) rover mission includes a suite of sensors to monitor current environmental conditions near the surface of Mars and to constrain bulk aerosol properties from changes in atmospheric radiation at the surface. The Mars Environmental Dynamics Analyzer (MEDA) consists of a set of meteorological sensors including wind sensor, a barometer, a relative humidity sensor, a set of 5 thermocouples to measure atmospheric temperature at ∼1.5 m and ∼0.5 m above the surface, a set of thermopiles to characterize the thermal IR brightness temperatures of the surface and the lower atmosphere. MEDA adds a radiation and dust sensor to monitor the optical atmospheric properties that can be used to infer bulk aerosol physical properties such as particle size distribution, non-sphericity, and concentration. The MEDA package and its scientific purpose are described in this document as well as how it responded to the calibration tests and how it helps prepare for the human exploration of Mars. A comparison is also presented to previous environmental monitoring payloads landed on Mars on the Viking, Pathfinder, Phoenix, MSL, and InSight spacecraft.

Analysis of the diagnostic pathway and delay in patients with amyotrophic lateral sclerosis in the Valencian Community.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is an insidious, clinically heterogeneous neurodegenerative disease associated with a diagnostic delay of approximately 12 months. No study conducted to date has analysed the diagnostic pathway in Spain. METHODS: We gathered data on variables related to the diagnostic pathway and delay for patients diagnosed with ALS between October 2013 and July 2017. RESULTS: The study included 143 patients with ALS (57% men; 68% spinal onset). Patients were diagnosed in public centres in 86% of cases and in private centres in 14%. The mean diagnostic delay was 13.1 months (median 11.7). Patients were examined by neurologists a mean time of 7.9 months after symptom onset, with diagnosis being made 5.2 months later. Half of all patients underwent unnecessary diagnostic tests and multiple electrophysiological studies before diagnosis was established. Diagnostic delay was longer in cases of spinal onset (P=.008) due to onset of the disease in the lower limbs. No differences were found between the public and private healthcare systems (P=.897). CONCLUSIONS: The diagnostic delay in ALS in Spain is similar to that of neighbouring countries and seems to depend on disease-related factors, not on the healthcare system. Patients with lower-limb onset ALS constitute the greatest diagnostic challenge. Misdiagnosis is frequent, and partly attributable to an incorrect approach or erroneous interpretation of electrophysiological studies. Specific training programmes for neurologists and general neurophysiologists and early referral to reference centres may help to reduce diagnostic delay.

Análisis del trayecto y retraso diagnóstico de los pacientes con esclerosis lateral amiotrófica en la Comunidad Valenciana / Analysis of the diagnostic pathway and delay in patients with amyotrophic lateral sclerosis in the Valencian Community

Introducción: La esclerosis lateral amiotrófica (ELA) es una enfermedad insidiosa y clínicamente heterogénea, lo que resulta en un retraso diagnóstico de unos 12 meses. En España el trayecto diagnóstico no ha sido analizado.MétodosSe recogieron variables relativas al trayecto y retraso diagnóstico de pacientes diagnosticados de ELA entre octubre del 2013 y julio del 2017.ResultadosSe incluyó a 143 pacientes con ELA (57% varones, 68% de inicio espinal). El 86% de ellos fueron estudiados en centros públicos y un 14% en privados. El retraso diagnóstico medio fue de 13,1 meses (mediana 11.7). El paciente tardó de media 7,9 meses en llegar al neurólogo y este, 5,2 meses más en diagnosticarlo. En la mitad de los pacientes se realizaron pruebas innecesarias y más de un estudio electrofisiológico para llegar al diagnóstico. El retraso diagnóstico fue mayor en los casos espinales (p = 0,008), atribuible a los pacientes cuyos síntomas se iniciaron en miembros inferiores, pero sin diferencias entre el sistema público y privado (p = 0,897).ConclusionesEl retraso diagnóstico de la ELA en nuestro medio es similar al de países de nuestro entorno y parece determinado por factores propios de la enfermedad e independiente del sistema sanitario. Las formas de inicio en miembros inferiores constituyen el mayor reto. Los errores diagnósticos del neurólogo son frecuentes y en parte atribuibles a una mala orientación o interpretación del estudio electrofisiológico. La formación específica del neurólogo y neurofisiólogo general y la derivación precoz a centros de referencia podrían ayudar a reducir la demora. (AU)

Introduction: Amyotrophic lateral sclerosis (ALS) is an insidious, clinically heterogeneous neurodegenerative disease associated with a diagnostic delay of approximately 12 months. No study conducted to date has analysed the diagnostic pathway in Spain.MethodsWe gathered data on variables related to the diagnostic pathway and delay for patients diagnosed with ALS between October 2013 and July 2017.ResultsThe study included 143 patients with ALS (57% men; 68% spinal onset). Patients were diagnosed in public centres in 86% of cases and in private centres in 14%.The mean diagnostic delay was 13.1 months (median 11.7). Patients were examined by neurologists a mean time of 7.9 months after symptom onset, with diagnosis being made 5.2 months later. Half of all patients underwent unnecessary diagnostic tests and multiple electrophysiological studies before diagnosis was established. Diagnostic delay was longer in cases of spinal onset (P = .008) due to onset of the disease in the lower limbs. No differences were found between the public and private healthcare systems (P = .897).ConclusionsThe diagnostic delay in ALS in Spain is similar to that of neighboring countries and seems to depend on disease-related factors, not on the healthcare system. Patients with lower-limb onset ALS constitute the greatest diagnostic challenge. Misdiagnosis is frequent, and partly attributable to an incorrect approach or erroneous interpretation of electrophysiological studies. Specific training programmes for neurologists and general neurophysiologists and early referral to reference centers may help to reduce diagnostic delay. (AU)

Rapamicina oral: una alternativa en niños con anomalías vasculares complicadas / Oral rapamycin: an alternative in children with complicated vascular abnormalities

Objetivos: El uso del inhibidor mTOR sirolimus ha supuesto unavance en el tratamiento de pacientes con anomalías vasculares complicadas. El objetivo de este estudio es presentar nuestra serie de pacientespediátricos con anomalías vasculares tratados con sirolimus oral y haceruna revisión de la literatura al respecto. Material y métodos: Se realizó un análisis retrospectivo de lospacientes con anomalías vasculares complicadas tratados con sirolimusoral en nuestro centro desde el año 2016. La dosis inicial utilizada fuede 0,8 mg/m2 cada 12 horas y el rango terapéutico de 5-15 ng/ml. Todos los pacientes recibieron profilaxis con trimetoprim-sulfametoxazol. Resultados: Se incluyeron seis niños, tres varones y tres mujeres, con una edad media al inicio del tratamiento de 9,5 años. Trespresentaban una malformación linfática en cabeza y cuello, dos unamalformación venosa en miembro inferior y la última una malformación combinada linfática-venosa a nivel toracoabdominal. Todos habíanrecibido múltiples tratamientos previos sin mejoría. Tras el inicio desirolimus, cinco pacientes mejoraron clínicamente (tiempo medio 3,6meses) y cuatro radiológicamente (tiempo medio 6,6 meses). Se registraron efectos adversos leves y transitorios en tres casos. Actualmente,cinco pacientes continúan con el tratamiento. Conclusiones: El sirolimus oral es un tratamiento eficaz y seguroen pacientes con anomalías vasculares complicadas. Nuestros resultadosapoyan su uso en malformaciones linfáticas y venosas en las que hanfracasado otros tratamientos, presentando buenas respuestas sintomáticasy, en menor medida, radiológicas.(AU)

Objective: Sirolimus mTOR inhibitor represents a major advancein the treatment of patients with complicated vascular abnormalities.The objective of this study was to present our series of pediatric patientswith vascular abnormalities treated with oral sirolimus, and to conducta review of the relevant literature. Materials and methods: A retrospective analysis of patients withcomplicated vascular abnormalities treated with oral sirolimus in ourhealthcare facility from 2016 was carried out. Initial dosage was 0.8 mg/m 2 every 12 hours, and therapeutic range was 5-15 ng/ml. All patientsreceived trimethoprim-sulfamethoxazole prophylaxis. Results: 6 children –3 boys and 3 girls– with a mean age of 9.5years at treatment initiation were included. 3 of them had head and necklymphatic malformation, 2 had lower limb venous malformation, and 1had combined lymphatic-venous malformation at the thoracoabdominal level. They all had received multiple previous treatments withoutimprovement. Following sirolimus initiation, 5 patients had clinicalimprovement (mean time: 3.6 months) and 4 had radiological improvement (mean time: 6.6 months). Mild and transitory adverse effects werenoted in the 3 cases. Today, 5 patients remain under treatment. Conclusions: Oral sirolimus is an effective and safe treatment inpatients with complicated vascular abnormalities. Our results supportsirolimus use in lymphatic and venous malformations in which previoustreatments have failed, with a good symptomatic and, to a lesser extent,radiological response.(AU)

Point-of-care lung ultrasound predicts in-hospital mortality in acute heart failure.

BACKGROUND: B-lines have been associated with adverse clinical outcomes in patients with heart failure (HF) when found at hospital discharge or during outpatient visits. Whether lung ultrasound (LUS) assessed B-lines may predict in-hospital mortality in patients with acute HF is still undetermined. AIM: To evaluate the association between B-lines on admission and in-hospital mortality among patients admitted with acute HF. METHODS: Hand-held LUS was used to examine patients with acute HF. LUS was performed in eight chest zones with a pocket ultrasound device and analyzed offline. The association between B-lines and in-hospital mortality was assessed using Cox regression models. RESULTS: We included 62 patients with median age 56 years, 69.4% men, and median left ventricle ejection fraction 25%. The sum of B-lines ranged from 0 to 53 (median 6.5). An optimal receiver operating characteristic-determined cut-off of ≥19 B-lines demonstrated a sensitivity of 57% and a specificity of 86% (area under the curve 0.788) for in-hospital mortality. The incremental prognostic value of LUS when compared with lung crackles or peripheral edema by integrated discrimination improvement was 12.96% (95% CI: 7.0-18.8, P = 0.02). Patients with ≥19 B-lines had a 4-fold higher risk of in-hospital mortality (HR 4.38; 95% CI: 1.37-13.95, P < 0.01). CONCLUSION: In patients admitted with acute HF, point-of-care LUS measurements of pulmonary congestion (B-lines) are associated with in-hospital mortality.

Oral rapamycin: an alternative in children with complicated vascular abnormalities. / Rapamicina oral: una alternativa en niños con anomalías vasculares complicadas.

OBJECTIVE: Sirolimus mTOR inhibitor represents a major advance in the treatment of patients with complicated vascular abnormalities. The objective of this study was to present our series of pediatric patients with vascular abnormalities treated with oral sirolimus, and to conduct a review of the relevant literature. MATERIAL AND METHODS: A retrospective analysis of patients with complicated vascular abnormalities treated with oral sirolimus in our healthcare facility from 2016 was carried out. Initial dosage was 0.8 mg/m2 every 12 hours, and therapeutic range was 5-15 ng/ml. All patients received trimethoprim-sulfamethoxazole prophylaxis. RESULTS: 6 children -3 boys and 3 girls- with a mean age of 9.5 years at treatment initiation were included. 3 of them had head and neck lymphatic malformation, 2 had lower limb venous malformation, and 1 had combined lymphatic-venous malformation at the thoracoabdominal level. They all had received multiple previous treatments without improvement. Following sirolimus initiation, 5 patients had clinical improvement (mean time: 3.6 months) and 4 had radiological improvement (mean time: 6.6 months). Mild and transitory adverse effects were noted in the 3 cases. Today, 5 patients remain under treatment. CONCLUSIONS: Oral sirolimus is an effective and safe treatment in patients with complicated vascular abnormalities. Our results support sirolimus use in lymphatic and venous malformations in which previous treatments have failed, with a good symptomatic and, to a lesser extent, radiological response.

OBJETIVOS: El uso del inhibidor mTOR sirolimus ha supuesto un avance en el tratamiento de pacientes con anomalías vasculares complicadas. El objetivo de este estudio es presentar nuestra serie de pacientes pediátricos con anomalías vasculares tratados con sirolimus oral y hacer una revisión de la literatura al respecto. MATERIAL Y METODOS: Se realizó un análisis retrospectivo de los pacientes con anomalías vasculares complicadas tratados con sirolimus oral en nuestro centro desde el año 2016. La dosis inicial utilizada fue de 0,8 mg/m2 cada 12 horas y el rango terapéutico de 5-15 ng/ml. Todos los pacientes recibieron profilaxis con trimetoprim-sulfametoxazol. RESULTADOS: Se incluyeron seis niños, tres varones y tres mujeres, con una edad media al inicio del tratamiento de 9,5 años. Tres presentaban una malformación linfática en cabeza y cuello, dos una malformación venosa en miembro inferior y la última una malformación combinada linfática-venosa a nivel toracoabdominal. Todos habían recibido múltiples tratamientos previos sin mejoría. Tras el inicio de sirolimus, cinco pacientes mejoraron clínicamente (tiempo medio 3,6 meses) y cuatro radiológicamente (tiempo medio 6,6 meses). Se registraron efectos adversos leves y transitorios en tres casos. Actualmente, cinco pacientes continúan con el tratamiento. CONCLUSIONES: El sirolimus oral es un tratamiento eficaz y seguro en pacientes con anomalías vasculares complicadas. Nuestros resultados apoyan su uso en malformaciones linfáticas y venosas en las que han fracasado otros tratamientos, presentando buenas respuestas sintomáticas y, en menor medida, radiológicas.

Strong opioids and non-cancer chronic pain in Catalonia. An analysis of the family physicians prescription patterns. / Opioides fuertes y dolor crónico no oncológico en Cataluña. Análisis del patrón de prescripción por parte de los médicos de familia.

OBJECTIVE: To identify family doctor prescription patterns for strong opioids for chronic, non-cancer-related pain. MATERIALS AND METHODS: Design A descriptive study based on a self-administered email questionnaire. LOCATION: All primary health care centres in Catalonia. PARTICIPANTS: 3,602 family doctors, all members of the Catalan Society of Family and Community Medicine. INTERVENTIONS: Email survey of Catalan family doctors. MAIN MEASUREMENTS: Demographic data, number of patients treated with potent opioids for chronic non-cancer pain, type of opioid used and indications, prescribing patterns and relationship with the Pain Management Unit. RESULTS: A total of 551 answers were obtained from 3,602 questionnaires sent (response rate of 15.3%), in which 480 physicians (87%) prescribed strong opioids for musculoskeletal pain, 268 (48.6%) prescribed ultra-rapid fentanyl and 434 (78.7%) reduced benzodiazepines dosage when prescribing potent opioids. The most common adverse effects were constipation and nausea. The main problems related with opioid prescription were improper use (341, 71%) and patient and/or practitioner reluctance (87, 18.1%). The assessment of the relationship with Pain Management Units was 2±1 (on a 1 to 5 scale), with communication (271, 52.2%) and accessibility (141, 27.1%) being the areas most in need of improvement. CONCLUSIONS: Opioid prescribing patterns generally follow clinical guidelines (e.g. reduction of benzodiazepine use or dose titration). However, there are some areas of improvement, such as sparse use of laxatives or use of ultra-rapid opioids for unapproved indications and in patients with no background opioid therapy. Family doctors perceive patient reluctance to adhere to the prescribed treatment, and call for specific training and better relationships with Pain Management Units.

[Surgical management of non-tuberculous mycobacterial lymphadenitis in a third level center]. / Manejo quirúrgico de la linfadenitis por micobacterias no tuberculosas en un centro de tercer nivel.

AIM OF STUDY: To describe our experience in the management of non-tuberculosis mycobacterial lymphadenitits (NTML). METHODS: Retrospective analysis of patients who underwent surgery for NTML in our centre during the period between 2010-2017. Demographic data, diagnostic tests, treatment and follow up information were recovered from medical records. RESULTS: 65 patients (26 male/39 female) with a mean age of 31 months (range 7 months-12 years) were intervened during the period of study. As diagnostic tests, chest X-ray was performed in 92.3% of patients with normal result in all cases, Mantoux test was positive in 20.3%, inconclusive in 12.5% and negative in 67.1%. Preoperative fine needle aspiration was positive for NTML (granulomatous necrotizing lymphadenitis) in 93.7% whereas culture for mycobacteria was only positive in 23.4% of cases, being Mycobacterium lentiflavum the most frequent agent found. Mean preoperative waiting time was 2.5 months with 7.7% of fistulization previous to surgery. Mean hospital stay was 1,1 days and there were no intraoperative complications. Mean follow up time was 5.5 months (range 1-24 months) during which 19 cases of temporal facial paralysis were noted, among which only 2 persisted after one year. CONCLUSIONS: NTML is a disease with a growing incidence in our country. It is important to make an early diagnosis and surgical treatment in order to avoid complications, as surgery has demonstrated to be safe and effective, with a low rate of complications. We believe the actual protocols should be revised/checked due to low effectiveness of diagnostic tests.

OBJETIVOS: Describir nuestra experiencia en el manejo de la linfadenitis por micobacterias no tuberculosas (LMNT). MATERIAL Y METODOS: Análisis retrospectivo de 65 pacientes (26 varones/ 39 mujeres) intervenidos en nuestro centro de LMNT durante los años 2010-2017. Se analizaron datos demográficos, pruebas complementarias realizadas y datos de seguimiento. RESULTADOS: La edad media fue de 31 meses (rango 7 meses-12 años). Para el diagnóstico la radiografía de tórax se realizó al 92,3% de pacientes, siendo en todos normal. El Mantoux fue positivo en 20,3%, dudoso en 12,5% y negativo en 67,1%. La PAAF preoperatoria fue diagnóstica (linfadenitis granulomatosa necrotizante) en 93,7% mientras que el cultivo para micobacterias fue positivo solo en 23,4%, siendo el Mycobacterium lentiflavum el patógeno más frecuentemente encontrado. El tiempo de espera preoperatorio fue de 2,5 meses con un porcentaje de fistulización previo a la cirugía de 7,7%. La estancia hospitalaria media fue 1,1 días y no hubo complicaciones perioperatorias. El tiempo medio de seguimiento fue 5,5 meses (rango 1-24 meses) observándose 19 casos de parálisis facial temporal, de los cuales 2 persistieron al cabo de 1 año. CONCLUSIONES: La LMNT es una enfermedad cada vez más frecuente en nuestro medio. Es importante realizar un diagnóstico y tratamiento precoz, puesto que el tratamiento quirúrgico ha demostrado ser seguro y eficaz asociando baja tasa de complicaciones. Creemos que los protocolos actuales se deben revisar, debido a la escasa rentabilidad de algunas pruebas complementarias.

Hipertensión en adolescentes: aproximación diagnóstica mediante monitorización ambulatoria de la presión arterial / Hypertension in adolescents: Diagnostic approach through ambulatory blood pressure monitoring

No disponible

Inesperada reagudización de enfermedad de Kawasaki / Unexpected exacerbation of Kawasaki disease

Introducción: La enfermedad de Kawasaki (EK) es la segunda vasculitis más frecuente de la infancia, después de la púrpura de Schönlein-Henoch, y la primera causa de enfermedad cardiaca adquirida en la infancia en los países desarrollados. Caso clínico: Se presenta un caso clínico de EK con reagudización y aparición de aneurismas gigantes coronarios como complicación secundaria. Discusión: El diagnóstico se basa en la combinación de criterios clínicos y analíticos, como presencia de fiebre (duración ≥ 5 días) y 4 de los siguientes: conjuntivitis bilateral no supurativa, linfadenopatía cervical, exantema polimorfo que afecta a las palmas y las plantas, cambios en las mucosas (lengua aframbuesada, enrojecimiento labial) y cambios en las extremidades (edema y eritema de las palmas, descamación del pulpejo de los dedos). El tratamiento consiste en la administración temprana de gammaglobulina intravenosa 2 g/kg y ácido acetilsalicílico 50 mg/kg/día. La secuela más importante es la afectación del sistema cardiovascular, en especial de las arterias coronarias, que puede llegar a desarrollar aneurismas coronarios que evolucionen hacia una estenosis y una posterior isquemia miocárdica. Conclusiones: Para disminuir la morbimortalidad de este proceso es importante un inicio precoz del tratamiento, lo que precisa una alta sospecha diagnóstica ante cuadros febriles prolongados, así como una correcta evaluación de los factores de riesgo de evolución tórpida, para elegir el tratamiento más adecuado

Introduction: Kawasaki disease is the second most common vasculitis of childhood and the leading cause of acquired heart disease in children in developed countries. Case report: We present the case of an infant with Kawasaki disease and acute exacerbation with coronary aneurysms as a secondary complication arises Discussion: Diagnosis is based on the combination of clinical and laboratory criteria: fever (over five days) plus 4 of the following: non-suppurative bilateral conjunctivitis, cervical lymphadenopathy, polymorphous rash affecting the palms and soles, changes in the mucous membranes (strawberry tongue, redness lip) and changes in the extremities (edema and erythema of the palms, peeling the heel of the fingers). The treatment consists of early administration of intravenous immunoglobulin 2 g/kg and acetylsalicylic acid 50 mg/kg/day. The most important consequence is the involvement of the cardiovascular system, especially the coronary arteries may develop coronary aneurysms evolve towards stenosis and subsequent myocardial ischemia. Conclusion: To decrease the morbidity and mortality of this process is important early initiation of treatment, which requires a high suspicion diagnosis before prolonged febrile illness, and an accurate assessment of risk factors torpid, to choose the most appropriate treatment