[Bilateral vocal cord paralysis with acute airway obstruction and urgent tracheotomy in a patient with an acute vertebrobasilar stroke]. / Parálisis bilateral de las cuerdas vocales con obstrucción aguda de la vía aérea y traqueotomía urgente en una paciente con ictus vertebrobasilar agudo.

INTRODUCTION: Vertebrobasilar stroke can be a diagnostic challenge. Bilateral vocal cord paralysis is very rare as a manifestation of acute stroke, yet it is potentially life-threatening because of the possibility of acute airway obstruction. No cases of bilateral vocal cord paralysis have been reported as a presenting symptom of acute stroke of mixed central and peripheral neurological aetiology. CASE REPORT: An 88-year-old woman with dysphonia resulting from paralysis of the right vocal cord following a thyroidectomy presented with sudden onset of vertigo, dysmetria and mild dysarthria (National Institutes of Health Stroke Scale: 2) associated with arterial hypertension. An urgent brain computed tomography (CT) scan evidenced distal occlusive thrombosis of the left vertebral artery without established ischaemia. Due to the improvement of symptoms achieved with control of her blood pressure, revascularisation therapy was not performed. Four hours later, the patient suddenly developed inspiratory stridor and severe respiratory failure due to bilateral vocal cord paralysis with complete airway obstruction. An urgent tracheotomy was performed, which resulted in an improvement in her breathing. A control brain CT scan performed at 24 hours showed established infarction in the left hemicerebellum and lateral medullary region, consistent with the territory of the left posterior inferior cerebellar artery. CONCLUSION: Our case illustrates the possibility of the rare occurrence of acute bilateral vocal cord paralysis in the context of acute stroke in conjunction with chronic peripheral involvement of the recurrent laryngeal nerve. Although exceptional, it exemplifies the potential risk associated with vertebrobasilar strokes. A more aggressive reperfusion therapy may be appropriate in these cases, despite an initially mild deficit, because of the possibility of progression to life-threatening complications.

TITLE: Parálisis bilateral de las cuerdas vocales con obstrucción aguda de la vía aérea y traqueotomía urgente en una paciente con ictus vertebrobasilar agudo.Introducción. El ictus vertebrobasilar puede suponer un reto diagnóstico. La parálisis bilateral de las cuerdas vocales como manifestación de ictus agudo es muy rara, pero potencialmente mortal, por la posibilidad de obstrucción aguda de la vía aérea. No hay casos descritos de parálisis bilateral de las cuerdas vocales como presentación de ictus agudo de etiología neurológica mixta central y periférica. Caso clínico. Mujer de 88 años con disfonía secuelar a parálisis de la cuerda vocal derecha postiroidectomía que presentó un cuadro brusco de vértigo, dismetría y disartria leve (National Institutes of Health Stroke Scale: 2) asociado a hipertensión arterial. La tomografía computarizada cerebral urgente demostró trombosis distal oclusiva de la arteria vertebral izquierda sin isquemia establecida. Por mejoría sintomática con control tensional, no se realizó terapia de revascularización. Cuatro horas después, la paciente desarrolló de forma brusca estridor inspiratorio e insuficiencia respiratoria grave por parálisis bilateral de las cuerdas vocales con obstrucción completa de la vía aérea. Se realizó una traqueotomía urgente con mejoría respiratoria. Una tomografía computarizada cerebral de control a las 24 horas mostró un infarto establecido en el hemicerebelo izquierdo y la región bulbar lateral, congruente con territorio de la arteria cerebelosa posterior inferior izquierda. Conclusión. Nuestro caso ilustra la posibilidad de la rara aparición de una parálisis bilateral aguda de las cuerdas vocales en el contexto de un ictus agudo junto con una afectación periférica crónica del nervio recurrente laríngeo. Aunque es excepcional, ejemplifica el potencial riesgo asociado a los ictus vertebrobasilares. Un tratamiento más agresivo de reperfusión podría ser adecuado en estos casos, pese a un déficit inicialmente leve, por la posibilidad de progresar a complicaciones vitales.

Parálisis bilateral de las cuerdas vocales con obstrucción aguda de la vía aérea y traqueotomía urgente en una paciente con ictus vertebrobasilar agudo / Bilateral vocal cord paralysis with acute airway obstruction and urgent tracheotomy in a patient with an acute vertebrobasilar stroke

Introducción El ictus vertebrobasilar puede suponer un reto diagnóstico. La parálisis bilateral de las cuerdas vocales como manifestación de ictus agudo es muy rara, pero potencialmente mortal, por la posibilidad de obstrucción aguda de la vía aérea. No hay casos descritos de parálisis bilateral de las cuerdas vocales como presentación de ictus agudo de etiología neurológica mixta central y periférica. Caso clínico Mujer de 88 años con disfonía secuelar a parálisis de la cuerda vocal derecha postiroidectomía que presentó un cuadro brusco de vértigo, dismetría y disartria leve (National Institutes of Health Stroke Scale: 2) asociado a hipertensión arterial. La tomografía computarizada cerebral urgente demostró trombosis distal oclusiva de la arteria vertebral izquierda sin isquemia establecida. Por mejoría sintomática con control tensional, no se realizó terapia de revascularización. Cuatro horas después, la paciente desarrolló de forma brusca estridor inspiratorio e insuficiencia respiratoria grave por parálisis bilateral de las cuerdas vocales con obstrucción completa de la vía aérea. Se realizó una traqueotomía urgente con mejoría respiratoria. Una tomografía computarizada cerebral de control a las 24 horas mostró un infarto establecido en el hemicerebelo izquierdo y la región bulbar lateral, congruente con territorio de la arteria cerebelosa posterior inferior izquierda. Conclusión Nuestro caso ilustra la posibilidad de la rara aparición de una parálisis bilateral aguda de las cuerdas vocales en el contexto de un ictus agudo junto con una afectación periférica crónica del nervio recurrente laríngeo. Aunque es excepcional, ejemplifica el potencial riesgo asociado a los ictus vertebrobasilares. Un tratamiento más agresivo de reperfusión podría ser adecuado en estos casos, pese a un déficit inicialmente leve, por la posibilidad de progresar a complicaciones vitales. (AU)

INTRODUCTION Vertebrobasilar stroke can be a diagnostic challenge. Bilateral vocal cord paralysis is very rare as a manifestation of acute stroke, yet it is potentially life-threatening because of the possibility of acute airway obstruction. No cases of bilateral vocal cord paralysis have been reported as a presenting symptom of acute stroke of mixed central and peripheral neurological aetiology. CASE REPORT An 88-year-old woman with dysphonia resulting from paralysis of the right vocal cord following a thyroidectomy presented with sudden onset of vertigo, dysmetria and mild dysarthria (National Institutes of Health Stroke Scale: 2) associated with arterial hypertension. An urgent brain computed tomography (CT) scan evidenced distal occlusive thrombosis of the left vertebral artery without established ischaemia. Due to the improvement of symptoms achieved with control of her blood pressure, revascularisation therapy was not performed. Four hours later, the patient suddenly developed inspiratory stridor and severe respiratory failure due to bilateral vocal cord paralysis with complete airway obstruction. An urgent tracheotomy was performed, which resulted in an improvement in her breathing. A control brain CT scan performed at 24 hours showed established infarction in the left hemicerebellum and lateral medullary region, consistent with the territory of the left posterior inferior cerebellar artery. CONCLUSION. Our case illustrates the possibility of the rare occurrence of acute bilateral vocal cord paralysis in the context of acute stroke in conjunction with chronic peripheral involvement of the recurrent laryngeal nerve. Although exceptional, it exemplifies the potential risk associated with vertebrobasilar strokes. A more aggressive reperfusion therapy may be appropriate in these cases, despite an initially mild deficit, because of the possibility of progression to life-threatening complications. (AU)

Mechanical characterisation of hydrophobic and hydrophilic acrylates used in intraocular lenses through depth sensing indentation.

In this work, the mechanical behaviour of hydrophilic and hydrophobic acrylates has been characterised by depth sensing indentation. Time-dependent behaviour has been studied using load-relaxation tests. Experiments have been simulated with a finite element software using a visco-hyperelastic material model. The parameters of this model have been determined using deep learning techniques. The developed material models have been used to mechanically simulate a standard compression test of a prototype intraocular lens.

Incidence of contrast-induced nephropathy after a computed tomography scan.

BACKGROUND AND AIMS: The term contrast-induced nephropathy is used to describe acute deterioration of renal function after the intravenous administration of iodinated contrast material. We aimed to estimate the incidence of contrast-induced nephropathy and to analyze the evolution of different biomarkers of renal function in patients who underwent computed tomography with intravenous contrast administration after premedication with oral hydration and N-acetylcysteine. MATERIAL AND METHODS: This prospective observational study included 112 patients with chronic renal failure (glomerular filtration rate (GFR) 30ml-60ml/min/1.73m2) scheduled for computed tomography with intravenous iodinated contrast material. We recorded demographic variables, dose of contrast material, diabetes mellitus, hypertension, and serum hemoglobin. We measured serum creatinine and GFR after premedication and after the CT examination. We summarized variables as means, standard deviations, and percentages. We used the Wilcoxon and Mann-Whitney tests to compare pre- and post-CT values and Pearson's r to analyze correlations. RESULTS: Incidence acute kidney injury: 0.9%; 95%CI: 0.36-1.4. Mean difference between pre- and post-CT creatinine: 0.04; 95%CI: 0.002-0.09, p<0.004. Mean difference between pre- and post-CT GFR: -3.06; 95%CI: -4.66 to -1.47), p<0.001. CONCLUSIONS: The incidence of contrast-induced nephropathy in patients with chronic renal failure and GFR 30ml-60ml/min/1.73m2 is low. The biomarkers of renal function analyzed improve in patients who receive premedication and the minimum dose of contrast material.

Leucoencefalopatía multifocal progresiva en paciente con lupus eritematoso sistémico: ¿podría ser la linfocitopenia-CD4+ el principal factor de riesgo? / Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus: Could CD4+ lymphopenia be the main risk factor?

No disponible

Humanización. Cuidados centrados en la familia / No disponible

No disponible

Valor de la ecografía doppler en la disfunción de los accesos vasculares periféricos para hemodiálisis / Value of doppler ultrasonography in the study of hemodialysis peripheral vascular access dysfunction

Objetivo . El objetivo principal del estudio es evaluar la sensibilidad y especificidad de la ecografía dúplex-Doppler para estudiar la disfunción de los accesos vasculares periféricos para hemodiálisis, y analizar el índice de resistencia y el flujo en la arteria aferente. Material y métodos. Se estudiaron prospectivamente 178 pacientes con 178 accesos vasculares periféricos disfuncionantes durante al menos 3 sesiones de hemodiálisis seguidas. Se realizaron ecografía dúplex-Doppler, angiografía y seguimiento clínico y analítico durante 3 meses (si la angiografía fue negativa). Se calcularon los valores de sensibilidad, especificidad, valores predictivos y cocientes de probabilidad. Se estudiaron morfológicamente la arteria aferente, la anastomosis arteriovenosa y la vena eferente, y se midieron el índice de resistencia y el flujo de la arteria aferente, el diámetro de la anastomosis, y el flujo y velocidad picosistólica en la vena eferente. Resultados. La muestra final la constituyeron 159 pacientes. Los valores de sensibilidad, especificidad, valor predictivo positivo y negativo y cociente de probabilidad positivo y negativo, fueron 0,98 (IC 95% 0,88-1), 0,74 (IC 95% 0,66-0,81), 0,96, 0,82, 3,7 y 0,03 respectivamente. El índice de resistencia fue < 0,5 en el 78,5% de los accesos vasculares periféricos normofuncionantes y > 0,5 en el 86,1% de los disfuncionantes. Se encontraron aneurismas en 19 de los accesos vasculares periféricos nativos y seudoaneurismas en 7 de los protésicos. El flujo invertido apareció en 57 accesos vasculares periféricos. Conclusión. La ecografía doppler dúplex es un método eficaz de detección y caracterización de estenosis y trombosis del accesos vasculares periféricos y aporta información morfológica y hemodinámica (AU)

Objective. The main objectives of this study were to evaluate the sensitivity and specificity of duplex Doppler ultrasonography in the study of hemodialysis peripheral vascular access dysfunction and to analyze the resistance index and flow in the afferent artery. Material and methods. We prospectively studied 178 patients with 178 peripheral vascular accesses that were dysfunctional in at least three consecutive hemodialysis sessions. Patients underwent duplex Doppler ultrasonography and clinical and laboratory follow-up for three months (provided angiography findings were negative). We calculated the sensitivity, specificity, predictive values, and coefficients of probability. We studied the morphology of the afferent artery, the arteriovenous anastomosis, and the efferent vein, and we measured the resistance index and the flow of the afferent artery, the diameter of the anastomosis, and the flow and peak systolic velocity in the efferent vein. Results. The final sample consisted of 159 patients. The sensitivity, specificity, positive and negative predictive values, and positive and negative coefficients of probability were 0,98 (95% CI: 0,88-1.00), 0,74 (95% CI: 0,66-0,81), 0,96, 0,82, 3.7, and 0,03, respectively. The resistance index was less than 0,5 in 78.5% of the peripheral vascular accesses with normal function and greater than 0,5 in 86.1% of the dysfunctional peripheral vascular accesses. We found aneurysms in 19 of the native peripheral vascular accesses and pseudoaneurysms in 7 of the prosthetic grafts. Inverted flow was seen in 57 peripheral vascular accesses. Conclusion. Duplex Doppler ultrasonography is an efficacious method for detecting and characterizing stenosis and thrombosis in peripheral vascular accesses, and it provides information about the morphology and hemodynamics (AU)

Value of doppler ultrasonography in the study of hemodialysis peripheral vascular access dysfunction.

OBJECTIVE: The main objectives of this study were to evaluate the sensitivity and specificity of duplex Doppler ultrasonography in the study of hemodialysis peripheral vascular access dysfunction and to analyze the resistance index and flow in the afferent artery. MATERIAL AND METHODS: We prospectively studied 178 patients with 178 peripheral vascular accesses that were dysfunctional in at least three consecutive hemodialysis sessions. Patients underwent duplex Doppler ultrasonography and clinical and laboratory follow-up for three months (provided angiography findings were negative). We calculated the sensitivity, specificity, predictive values, and coefficients of probability. We studied the morphology of the afferent artery, the arteriovenous anastomosis, and the efferent vein, and we measured the resistance index and the flow of the afferent artery, the diameter of the anastomosis, and the flow and peak systolic velocity in the efferent vein. RESULTS: The final sample consisted of 159 patients. The sensitivity, specificity, positive and negative predictive values, and positive and negative coefficients of probability were 0,98 (95% CI: 0,88-1.00), 0,74 (95% CI: 0,66-0,81), 0,96, 0,82, 3.7, and 0,03, respectively. The resistance index was less than 0,5 in 78.5% of the peripheral vascular accesses with normal function and greater than 0,5 in 86.1% of the dysfunctional peripheral vascular accesses. We found aneurysms in 19 of the native peripheral vascular accesses and pseudoaneurysms in 7 of the prosthetic grafts. Inverted flow was seen in 57 peripheral vascular accesses. CONCLUSION: Duplex Doppler ultrasonography is an efficacious method for detecting and characterizing stenosis and thrombosis in peripheral vascular accesses, and it provides information about the morphology and hemodynamics.

Ventilación mecánica domiciliaria en niños: estudio multicéntrico español / Domiciliary mechanical ventilation in children: a spanish multicentre study

Introducción: La ventilación mecánica domiciliaria (VMD) es una técnica cada vez más frecuente en el niño. Existen pocos estudios que hayan analizado las características y necesidades de los niños sometidos a esta técnica. Material y métodos: Estudio descriptivo observacional transversal multicéntrico de pacientes entre un mes y 16 años dependientes de ventilación mecánica domiciliaria. Resultados: Se estudiaron 163 pacientes de 17 hospitales españoles con una edad media de 7,6 años. La causa más frecuente de VMD fueron los trastornos neuromusculares. El inicio de la VMD fue a una edad media de 4,6 años. Un 71,3% recibieron ventilación no invasiva. Los pacientes con ventilación invasiva tenían menor edad, menor edad de inicio de la VMD y mayor tiempo de uso diario. El 80,9% precisaban VM solo durante el sueño, y un 11,7% durante todo el día. Únicamente un 3,4% de los pacientes tiene asistencia sanitaria externa como ayuda a la familia. Un 48,2% es controlado en consultas específicas de VMD o consultas multidisciplinares. Un 72,1% de los pacientes está escolarizado (recibiendo enseñanza adaptada un 42,3%). Solo un 47,8% de los pacientes escolarizados cuentan con cuidadores específicos en su centro escolar. Conclusiones: La VMD en niños se utiliza en un grupo muy heterogéneo de pacientes iniciándose en un importante porcentaje en los primeros 3 años de vida. A pesar de que un significativo porcentaje de pacientes tiene una gran dependencia de la VMD pocas familias cuentan con ayudas específicas tanto a nivel escolar como en el domicilio, y el seguimiento sanitario es heterogéneo y poco coordinado(AU)

Introduction: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. Materials and methods: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. Results: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. Conclusions: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated(AU)

Angiotomografía computerizada, colgajos de perforante, cirujano y OsiriX / Computed tomography angiography, perforator flaps, surgeon and OsiriX

Desde la introducción de la transferencia cutánea basada en perforantes, la angiotomografía computerizada ha emergido como una técnica de gran valor en la planificación preoperatoria de los colgajos de perforante. Sin embargo, parece probable que el aprovechamiento de la técnica sea menor del deseable. Evaluamos, a través de la experiencia en 144 pacientes, la utilidad del visor gratuito de imágenes DICOM OsiriX para Mac en la planificación preoperatoria de los colgajos de perforante con tres objetivos: 1) ampliarlos conocimientos actuales relacionados con la aplicación de la angiotomografía computerizada en la planificación preoperatoria de los colgajos de perforante, 2) evaluar la aplicación OsiriX en el post-procesamiento de imágenes en la planificación preoperatoria de colgajos de perforante y3) evaluar el rendimiento obtenible de la angiotomografía considerando que el postprocesamiento es realizado por un cirujano (no especialista en Radiodiagnóstico).La experiencia permite afirmar que el postprocesamiento de las imágenes DICOM por el cirujano con la aplicación Osirix permite habitualmente evaluar de manera adecuada diversas estructuras y parámetros de gran interés en la cirugía de colgajos de perforante: 1) arteria principal, origen de la perforante, 2) diámetro de arteria y vena/s en el hipotético sitio de anastomosis microquirúrgica, 3) recorrido y patrón de ramificación del pedículo del colgajo, 4) disposición de la perforante en la grasa subcutánea(teórico eje de diseño del colgajo), 5) medición del grosor cutáneo en el punto de perforación de la fascia profunda por la rama perforante (teórico grosor de colgajo), 6) medición de la distancia entre el punto de perforación de la fascia profunda por la rama perforante (..) (AU)

With the advent of perforator-based skin transfer, computed tomography angiography has emerged as an invaluable tool in the preoperative planning of perforator flaps. But most likely, the exploitation of the technique is less than desirable. Through our experience with 144 patients, we evaluate the use of the free DICOM viewer OsiriX for Mac in the preoperative planning of perforator flaps with three objectives: 1) increase the present knowledge related with the preoperative planning of perforator flaps with computed tomography angiography, 2) evaluate the OsiriX application in the image post-processing of perforator flaps and 3) evaluate the performance of the procedure when performed by a surgeon (not specialized in Radiology).The experience has shown that the image post-processing performed by the surgeon with the OsiriX application usually allows an adequate evaluation of different structures and parameters of great preoperative interest in perforator flap surgery: 1) source artery, 2) diameter of artery and vein/s at the hypothetical site of microsurgical anastomoses, 3) course and branching pattern of the flap pedicle, 4) perforator course in the subcutaneous fat (theoretical flap axis), 5) measurement of the skin and fat where the perforator pierces the deep fascia (theoretical flap thickness ), 6) measurement of the (..)(AU)

Biometeorological and autoregressive indices for predicting olive pollen intensity.

This paper reports on modelling to predict airborne olive pollen season severity, expressed as a pollen index (PI), in Córdoba province (southern Spain) several weeks prior to the pollen season start. Using a 29-year database (1982-2010), a multivariate regression model based on five indices-the index-based model-was built to enhance the efficacy of prediction models. Four of the indices used were biometeorological indices: thermal index, pre-flowering hydric index, dormancy hydric index and summer index; the fifth was an autoregressive cyclicity index based on pollen data from previous years. The extreme weather events characteristic of the Mediterranean climate were also taken into account by applying different adjustment criteria. The results obtained with this model were compared with those yielded by a traditional meteorological-based model built using multivariate regression analysis of simple meteorological-related variables. The performance of the models (confidence intervals, significance levels and standard errors) was compared, and they were also validated using the bootstrap method. The index-based model built on biometeorological and cyclicity indices was found to perform better for olive pollen forecasting purposes than the traditional meteorological-based model.

[Domiciliary mechanical ventilation in children: a Spanish multicentre study]. / Ventilación mecánica domiciliaria en niños: estudio multicéntrico español.

INTRODUCTION: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. MATERIALS AND METHODS: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. RESULTS: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. CONCLUSIONS: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated.

Distrofia torácica asfixiante, o síndrome de Jeune, asociada a una malformación espinal cervical / Asphyxiating thoracic dystrophy or Jeune syndrome associated to cervical spine malformation

La distrofia torácica asfixiante, o síndrome de Jeune, es una displasia ósea de herencia autosómica recesiva, con expresión fenotípica variable. El diagnóstico es fundamentalmente clínico y radiológico. Se caracteriza por la presencia de un tórax estrecho y acampanado, polidactilia, costillas horizontalizadas y huesos iliacos cortos, con una alteración típica del techo acetabular en tridente, y suele asociar otras complicaciones: nefrocalcinosis, hepatopatía colestásica, anomalías pancreáticas y retinianas. La distrofia torácica produce hipoplasia pulmonar secundaria e insuficiencia respiratoria restrictiva que puede ser mortal en etapas precoces de la vida. Aunque la existencia de malformaciones espinales no es un hallazgo frecuente, en este caso, el paciente presentaba una malformación de C1 que producía compresión medular. Se realizó una descompresión quirúrgica y, posteriormente, la evolución del paciente ha sido muy favorable. Concluimos que debe realizarse siempre el cribado de malformaciones espinales en pacientes afectados de este síndrome, ya que su tratamiento puede contribuir significativamente a mejorar su pronóstico y calidad de vida(AU)

Asphyxiating thoracic dystrophy-Jeune syndrome is an autosomal recessive disease. It is part of a group of skeletal dysplasias with great clinical variability. Diagnosis is based on clinical and radiographic findings: the syndrome is characterized by a small, narrow chest, variable limb shortness, postaxial polydactyl, horizontally oriented ribs, and short iliac bones with a typical trident appearance of the acetabula. Renal, hepatic, pancreatic and ocular complications may occur later in life. As result of the thoracic dystrophy a lung hypoplasia is produced and a lethal respiratory distress during neonatal and infancy period may occur as well as multiple respiratory infections. Despite spine malformations are not frequently associated, we present a patient who had C1 malformation with cord compression. Surgical cord decompression was done and probably was determinant in his course. We concluded that the screening of spinal malformation in these patients should be always done because it treatment could improve their prognosis and quality of life(AU)

The usefulness of ultrasonography in the diagnosis of carpal tunnel syndrome.

This study assessed the use of ultrasound in the diagnosis of carpal tunnel syndrome and to determine the best ultrasound criterion for diagnosis. Forty wrists of 27 patients with surgically proven moderate and severe carpal tunnel syndrome and 30 wrists of 15 controls were examined. Measurements of the cross-sectional area and the anteroposterior diameter of the median nerve at the inlet and outlet of the carpal tunnel were obtained. Patients also underwent electrophysiological evaluation. Median nerve ultrasonographic measurements were significantly higher in patients. The cross-sectional area of the median nerve at the tunnel inlet was found to be the most useful diagnostic criterion. The optimal cut-off value was 6.5 mm2 (sensitivity 89.5%, specificity 93%). Ultrasound parameters failed to correlate with the electrophysiological findings. The usefulness of ultrasonography in the diagnosis of carpal tunnel syndrome is discussed.

Searching for new mathematical growth model approaches for Listeria monocytogenes.

Different secondary modeling approaches for the estimation of Listeria monocytogenes growth rate as a function of temperature (4 to 30 degrees C), citric acid (0% to 0.4% w/v), and ascorbic acid (0% to 0.4% w/v) are presented. Response surface (RS) and square-root (SR) models are proposed together with different artificial neural networks (ANN) based on product functions units (PU), sigmoidal functions units (SU), and a novel approach based on the use of hybrid functions units (PSU), which results from a combination of PU and SU. In this study, a significantly better goodness-of-fit was obtained in the case of the ANN models presented, reflected by the lower SEP values obtained (< 24.23 for both training and generalization datasets). Among these models, the SU model provided the best generalization capacity, displaying lower RMSE and SEP values, with fewer parameters compared to the PU and PSU models. The bias factor (B(f)) and accuracy factor (A(f)) of the mathematical validation dataset were above 1 in all cases, providing fail-safe predictions. The balance between generalization properties and the ease of use is the main consideration when applying secondary modeling approaches to achieve accurate predictions about the behavior of microorganisms.

[Imprecise status allocation by users of the American Society of Anesthesiologists classification system: survey of Catalan anesthesiologists]. / Imprecisión en la aplicación de la escala de estado físico de la American Society of Anesthesiologists: encuesta realizada entre los anestesiólogos catalanes.

OBJECTIVES: To study precision in American Society of Anesthesiologists (ASA) physical status classification by Catalan anesthesiologists. METHODS: An anonymous survey carried out in Catalonia, Spain, asked medical residents, staff anesthesiologists, and chiefs of service to allocate ASA classifications in 10 hypothetical cases that were representative of usual clinicalpractice. Respondents were also asked to state how long they had been practicing. Frequency distributions, medians and interquartile ranges (IQR) were calculated for the scores for each hypothetical case. The differences between residents and staff specialists were assessed. RESULTS: Three hundred thirty-three responses were obtained: 30.4% from residents, 59.9% from staff anesthesiologists, and 9.7% from chiefs of service. The mean (SD) length of professional practice of the staff specialists was 15.13 (8) years. For 1 case the IQR was 0 points on the ASA scale; in another case it was 3, and in the remaining cases the IQR was 1. Over half of the respondents were unfamiliar with class 6 (organ donor). The responses of residents and staff specialists for 2 cases differed significantly. CONCLUSIONS: The use of the ASA physical status scale is somewhat imprecise. There are small differences between specialists and residents and the current version is not very well known.

[Mitochondrial acetoacetyl-CoA thiolase deficiency: neonatal onset]. / Déficit de acetoacetil-CoA tiolasa mitocondrial: inicio en el período neonatal.

We present the case of a 4-day-old newborn with serious dehydration, polypnea, hypertonus and lethargy. Blood analysis showed severe metabolic acidosis with ketonemia, ketonuria and elevation of the GAP anion. Urine analysis revealed increased excretion of 2-methyl-3-hydroxybutyrate acid, tiglycine, and 2-methylacetoacetate acid. Neonatal onset of mitochondrial acetoacetyl-CoA thiolase (T2) deficiency is exceptional. Most patients have no clinical symptoms in the neonatal period. This entity should be considered in patients with acute metabolic acidosis and ketosis with normal glycemia and aciduria. The urine contains large amounts of 2-methylacetoacetate and its decarboxylation products. In the neonatal period, this inherited disorder of metabolism can produce severe hydroelectrolyte disorders in the form of a gradual process or acute episodes, which can occasionally be fatal.

Déficit de acetoacetil-CoA tiolasa mitocondrial: inicio en el período neonatal / Mitochondrial acetoacetyl-CoA Thiolase deficiency: neonatal onset

Presentamos el caso de un recién nacido de cuatro días de vida con deshidratación grave, polipnea, hipertonía y letargia. Los exámenes complementarios revelaron una acidosis metabólica grave con cetonemia, cetonuria y aumento del anión GAP. El análisis de orina reveló una excreción aumentada de ácido 2-metil-3-hidroxibutirato, tiglicina y 2-metilacetoacetato. El inicio neonatal del déficit de acetoacetil-CoA tiolasa (T2) es muy infrecuente. En la mayoría de los casos, los pacientes no presentan clínica en el período neonatal. Debemos pensar en esta entidad cuando un paciente presente una acidosis metabólica aguda, cetosis con glucemia normal y aciduria. La orina presenta aumento de las concentraciones de 2-metilacetoacetato y sus productos de descarboxilación. Este error innato del metabolismo en período neonatal puede producir graves alteraciones hidroelectrolíticas en forma de un proceso gradual o agudo que ocasionalmente puede causar la muerte del paciente

We present the case of a 4-day-old newborn with serious dehydration, polypnea, hypertonus and lethargy. Blood analysis showed severe metabolic acidosis with ketonemia, ketonuria and elevation of the GAP anion. Urine analysis revealed increased excretion of 2-methyl-3-hydroxybutyrate acid, tiglycine, and 2-methylacetoacetate acid. Neonatal onset of mitochondrial acetoacetyl-CoA thiolase (T2) deficiency is exceptional. Most patients have no clinical symptoms in the neonatal period. This entity should be considered in patients with acute metabolic acidosis and ketosis with normal glycemia and aciduria. The urine contains large amounts of 2-methylacetoacetate and its decarboxylation products. In the neonatal period, this inherited disorder of metabolism can produce severe hydroelectrolyte disorders in the form of a gradual process or acute episodes, which can occasionally be fatal

Imprecisión en la aplicación de la escala de estado físico de la American Society of Anesthesiologists Encuesta realizada entre los anestesiólogos catalanes / Imprecise status allocation by users of the American Society of Anesthesiologists classification system survey of Catalan anesthesiologists

OBJETIVOS: Investigar la precisión en la aplicación dela escala de estado físico de la American Society of Anesthesiologists(ASA) por los anestesiólogos catalanes.MÉTODOS: Se realizó en 2003 una encuesta anónimaentre los anestesiólogos de Cataluña (España) que preguntabapor la categoría laboral (residentes, médicos deplantilla o jefes) y años de ejercicio profesional de losespecialistas, y por la puntuación según la escala de laASA de 10 supuestos casos clínicos representativos de lapráctica clínica habitual. Se calcularon las distribucionesde frecuencias, mediana y rango intercuartil de las puntuacionesa cada caso, y la diferencia entre residentes yespecialistas.RESULTADOS: Se obtuvieron 333 respuestas: 30,4% deresidentes, 59,9% de adjuntos y 9,7% de jefes. El ejercicioprofesional de los especialistas fue de 15,13 ± 8 años(media ± DE). En 1 caso el rango intercuartil fue de 0puntos en la escala ASA; en otro caso de 3, y en los otros8 casos de 1. Más del 50% de los encuestados desconocíanla clase 6 (donante de órganos). En 2 de los casos lasrespuestas difirieron significativamente entre residentesy especialistas.CONCLUSIONES: La escala de estado físico de la ASAse utiliza de forma relativamente imprecisa, con pequeñasdiferencias entre especialistas y residentes, y con unalto desconocimiento de la versión actual

OBJECTIVES: To study precision in American Society of Anesthesiologists (ASA) physical status classification by Catalan anesthesiologists. METHODS: An anonymous survey carried out in Catalonia, Spain, asked medical residents, staff anesthesiologists, and chiefs of service to allocate ASA classifications in 10 hypothetical cases that were representative of usual clinical practice. Respondents were also asked to state how long they had been practicing. Frequency distributions, medians and interquartile ranges (IQR) were calculated for the scores for each hypothetical case. The differences between residents and staff specialists were assessed. RESULTS: Three hundred thirty-three responses were obtained: 30.4% from residents, 59.9% from staff anesthesiologists, and 9.7% from chiefs of service. The mean (SD) length of professional practice of the staff specialists was 15.13 (8) years. For 1 case the IQR was 0 points on the ASA scale; in another case it was 3, and in the remaining cases the IQR was 1. Over half of the respondents were unfamiliar with class 6 (organ donor). The responses of residents and staff specialists for 2 cases differed significantly. CONCLUSIONS: The use of the ASA physical status scale is somewhat imprecise. There are small differences between specialists and residents and the current version is not very well known