Congenital stromal corneal dystrophy in a Spanish family: Clinical, genetic and histological analysis.

PURPOSE: To present the clinical, genetic, and histopathological features of the ninth family affected by congenital stromal corneal dystrophy (CSCD) to date. METHODS: Twelve cases of a Spanish family affected by CSCD were analyzed regarding history, visual acuity (VA, decimal scale), an ophthalmologic exam and specular microscopy. Five eyes were treated by deep anterior lamellar keratoplasty (DALK), and thirteen eyes by penetrating keratoplasty (PK). In the two last generations, a genetic study was performed. RESULTS: Most of the patients affected were born with opaque corneas except for three, whose corneas were clear at birth. Biomicroscopy showed a whitish diffuse stromal opacity with an unaltered epithelium, causing poor VA (from hand motions to 0.4). Patients treated with PK presented mean postoperative VA of 0.19±0.20 over a follow-up time of 235.3±101.4months with 38% recurrences. Patients who underwent DALK experienced VA improvement to 0.17±0.11 over a follow-up time of 10.8±2.6months without signs of recurrence. In the latter, the big bubble technique was not achieved, so a manual technique was performed. The genetic study showed heterozygosis for a 1-bp deletion at nucleotide 962 in exon 8 of the decorin gene. CONCLUSIONS: CSCD is a rare entity, which should be treated by DALK whenever possible, obtaining better results than PK. Close monitoring of children of affected individuals is important, because CSCD can progress during the early years of life.

Detección y caracterización de defectos del campo visual mediante perimetría Octopus en glaucoma congénito primario / Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma

Detectar y caracterizar los defectos del campo visual (CV) mediante perimetría Octopus en pacientes con glaucoma congénito primario (GCP) y determinar la calidad y duración del CV. Material y métodos: Se incluyeron 88 ojos de 70 pacientes diagnosticados de GCP. Las evaluaciones se realizaron con un Octopus 900 y cada ojo se evaluó con el algoritmo de perimetría orientada por tendencias (G-TOP). Se recogieron datos cuantitativos de CV: datos de calidad (respuestas falsa positiva y negativa, y duración del tiempo) y resultados de desviación media (DM) y raíz cuadrada de la varianza de pérdida (sLV). También se recogieron datos cualitativos: presencia de defectos difusos y localizados, hemicampo afectado y grado de defectos utilizando la clasificación de Aulhorn y Karmeyer. Se analizaron las correlaciones entre los resultados perimétricos y las variables clínicas. Resultados: La mediana de edad fue de 11 (8-17) años. El 65,9% (58/88) de los ojos con GCP presentaban defectos de CV. Se observaron defectos difusos en 10/58 ojos (16,94%) (DM media=23,92 [DE: 2,52]) dB) y defectos localizados en 48/58 ojos (82,75%). El defecto más frecuente fue el escotoma incipiente paracentral (n=15), el escalón nasal (n=8), el defecto arciforme añadido (n=2), el semianular (n=13) y el defecto concéntrico con isla central (n=9). El hemicampo visual afectado con mayor frecuencia fue el inferior. La duración media de la prueba fue de 2min 12s (DE: 21,6s). Los valores MD y sLV se correlacionaron con la agudeza visual mejor corregida, la relación excavación/disco y el número de cirugías de glaucoma (todas p<0,001). Conclusión: Se identificó un alto número de defectos difusos y localizados utilizando la perimetría Octopus en pacientes con GCP. El defecto más frecuente fue el escotoma paracentral, y el hemicampo inferior fue el más afectado.(AU)

Purpose: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. Material and methods: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. ResultsMedian age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD=23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n=15), nasal step (n=8), adding arcuate defect (n=2), half ring-shaped (n=13) and concentric defect with a central island (n=9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2min 12s (SD: 21.6s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P<.001). Conclusion: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.(AU)

Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients.

INTRODUCTION: PRP is a rare entity of unknown etiopathogenesis. Lack of management guidelines makes it a challenge for clinicians. OBJECTIVE: To add our experience to increase evidence about PRP. METHODS: We performed a retrospective, descriptive and multicentric study of 65 patients with PRP, being the largest European case series of patients with PRP. RESULTS: PRP was more frequent in male patients with an average age of 51 years, but erythrodermic forms presented in older patients (average age 61 years). Six (75%) paediatric patients and ten (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. On the contrary, 26 (68%) erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6.5 months to achieve complete response. CONCLUSION: Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma.

Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma.

PURPOSE: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. MATERIAL AND METHODS: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. RESULTS: Median age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD = 23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n = 15), nasal step (n = 8), adding arcuate defect (n = 2), half ring-shaped (n = 13) and concentric defect with a central island (n = 9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2 min 12 s (SD: 21.6 s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P < .001). CONCLUSION: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.

Corneal endothelial validation in the eye bank: differences in automated methods and repeatability.

PURPOSE: To evaluate reproducibility of endothelial cell density (ECD) measurements using the Konan Cell Check D in donor corneas by two different ophthalmologists and to compare the two automated cell count methods (center and flex-center) available in the software of this specular microscope. METHODS: ECD values were quantified in 54 donor corneas by two independent investigators using the Cell Check D (Konan Medical USA Inc) with both automated cell count methods. In the center method, at least 30 contiguous cells are marked. For the flex-center method, an area is delineated and only the cells within the designated area are counted. RESULTS: The mean ECD was 2473.81±378.22 cells/mm2. Good ECD intergrader reproducibility for the center (ICC=0.821) and the flex-center method (ICC=0.784) were noted. Poor reliability was observed for coefficient of variation and hexagonality (ICC≤0.265). When both methods for ECD analysis were compared, a moderate correlation for the two independent graders using the two manual (center and flex-center) methods was detected (correlation coefficient of 0.678 and 0.745 for each of the investigators). Comparison between methods yielded significantly higher ECD with the flex-center method (P=0.013). When corneas were divided by ECD, those under 2200 cells/mm2 and those between 2200 and 2700 cells/mm2 also had significantly higher ECD with the flex-center method (P<0.022). CONCLUSIONS: ECD values are reproducible with both methods, although the flex-center method ECDs tend to be higher, particularly in cases of low ECD. Eye banks and surgeons should exercise caution in making decisions based only on small differences in ECD.

Method for quantifying arousal and consciousness in healthy states and severe brain injury via EEG-based measures of corticothalamic physiology.

BACKGROUND: Characterization of normal arousal states has been achieved by fitting predictions of corticothalamic neural field theory (NFT) to electroencephalographic (EEG) spectra to yield relevant physiological parameters. NEW METHOD: A prior fitting method is extended to distinguish conscious and unconscious states in healthy and brain injured subjects by identifying additional parameters and clusters in parameter space. RESULTS: Fits of NFT predictions to EEG spectra are used to estimate neurophysiological parameters in healthy and brain injured subjects. Spectra are used from healthy subjects in wake and sleep and from patients with unresponsive wakefulness syndrome, in a minimally conscious state (MCS), and emerged from MCS. Subjects cluster into three groups in parameter space: conscious healthy (wake and REM), sleep, and brain injured. These are distinguished by the difference X-Y between corticocortical (X) and corticothalamic (Y) feedbacks, and by mean neural response rates α and ß to incoming spikes. X-Y tracks consciousness in healthy individuals, with smaller values in wake/REM than sleep, but cannot distinguish between brain injuries. Parameters α and ß differentiate deep sleep from wake/REM and brain injury. COMPARISON WITH EXISTING METHODS: Other methods typically rely on laborious clinical assessment, manual EEG scoring, or evaluation of measures like Φ from integrated information theory, for which no efficient method exists. In contrast, the present method can be automated on a personal computer. CONCLUSION: The method provides a means to quantify consciousness and arousal in healthy and brain injured subjects, but does not distinguish subtypes of brain injury.

Patients with severe polytrauma: management and imaging protocols.

Traumatic injuries can be severe and complex, requiring the coordinated efforts of a multidisciplinary team. Imaging tests play a fundamental role in rapid and accurate diagnosis. In particular, whole-body computed tomography (CT) has become a key tool. There are different CT protocols depending on the patient's condition; whereas dose-optimized protocols can be used in stable patients, time/precision protocols prioritizing speed at the cost of delivering higher doses of radiation should be used in more severe patients. In unstable patients who cannot be examined by CT, X-rays of the chest and pelvis and FAST or e-FAST ultrasound studies, although less sensitive than CT, enable the detection of situations that require immediate treatment. This article reviews the imaging techniques and CT protocols for the initial hospital workup for patients with multiple trauma.

Update on acute appendicitis: Typical and untypical findings.

Acute appendicitis is the most common indication for emergency abdominal surgery throughout the world and a common reason for consultation in emergency departments. In recent decades, diagnostic imaging has played a fundamental role in identifying acute appendicitis, helping to reduce the rate of blind laparotomies and hospital costs. Given the results of clinical trials supporting the use of antibiotic therapy over surgical treatment, radiologists need to know the diagnostic criteria for complicated acute appendicitis to be able to recommend the best treatment option. This review aims not only to define the diagnostic criteria for appendicitis in different imaging modalities (ultrasonography, computed tomography, and magnetic resonance imaging), but also to explain the diagnostic protocols, atypical presentations, and other conditions that can mimic appendicitis.

Recommendations on the use of computed tomography in the stroke code: Consensus document SENR, SERAU, GEECV-SEN, SERAM.

The Spanish Society of Emergency Radiology (SERAU), the Spanish Society of Neuroradiology (SENR), the Spanish Society of Neurology through its Cerebrovascular Diseases Study Group (GEECV-SEN) and the Spanish Society of Medical Radiology (SERAM) have met to draft this consensus document that will review the use of computed tomography in the stroke code patients, focusing on its indications, the technique for its correct acquisition and the possible interpretation mistakes.

Recomendaciones sobre el uso de la tomografía computarizada en el código ictus: Documento de consenso SENR, SERAU, GEECV-SEN, SERAM / Recommendations on the use of computed tomography in the stroke code: Consensus document SENR, SERAU, GEECV-SEN, SERAM

La Sociedad Española de Radiología de Urgencias (SERAU), la Sociedad Española de Neurorradiología (SENR), la Sociedad Española de Neurología a través de su Grupo de Estudio de Enfermedades Cerebrovasculares (GEECV-SEN) y la Sociedad Española de Radiología Médica (SERAM) se han reunido para redactar este documento de consenso que repasará el uso de la tomografía computarizada en el código ictus, centrándose en sus indicaciones, la técnica para su correcta adquisición y las posibles causas de error en su interpretación (AU)

The Spanish Society of Emergency Radiology (SERAU), the Spanish Society of Neuroradiology (SENR), the Spanish Society of Neurology through its Cerebrovascular Diseases Study Group (GEECV-SEN) and the Spanish Society of Medical Radiology (SERAM) have met to draft this consensus document that will review the use of computed tomography in the stroke code patients, focusing on its indications, the technique for its correct acquisition and the possible interpretation mistakes (AU)

Vaginal neutrophil infiltration is contingent on ovarian cycle phase and independent of pathogen infection.

The mucosa of the female reproductive tract must reconcile the presence of commensal microbiota and the transit of exogenous spermatozoa with the elimination of sexually transmitted pathogens. In the vagina, neutrophils are the principal cellular arm of innate immunity and constitute the first line of protection in response to infections or injury. Neutrophils are absent from the vaginal lumen during the ovulatory phase, probably to allow sperm to fertilize; however, the mechanisms that regulate neutrophil influx to the vagina in response to aggressions remain controversial. We have used mouse inseminations and infections of Neisseria gonorrhoeae, Candida albicans, Trichomonas vaginalis, and HSV-2 models. We demonstrate that neutrophil infiltration of the vaginal mucosa is distinctively contingent on the ovarian cycle phase and independent of the sperm and pathogen challenge, probably to prevent sperm from being attacked by neutrophils. Neutrophils extravasation is a multi-step cascade of events, which includes their adhesion through selectins (E, P and L) and integrins of the endothelial cells. We have discovered that cervical endothelial cells expressed selectin-E (SELE, CD62E) to favor neutrophils recruitment and estradiol down-regulated SELE expression during ovulation, which impaired neutrophil transendothelial migration and orchestrated sperm tolerance. Progesterone up-regulated SELE to restore surveillance after ovulation.

The usefulness and positive predictive value of ultrasonography and computed tomography in the diagnosis of acute appendicitis in adults: A retrospective study.

OBJECTIVE: To analyze the evolution of the use of imaging tests (ultrasonography and computed tomography (CT)) in the diagnosis of acute appendicitis. To determine the positive predictive value of these tests and the percentage of negative and complicated appendectomies. MATERIAL AND METHODS: This retrospective study compared adults who underwent appendectomy for suspected acute appendicitis at our tertiary hospital during 2015 versus similar patients at our center during 2007. RESULTS: A total of 278patients were included. The rate of negative appendectomies descended to 5%. The positive predictive value of ultrasonography increased to 97.4% in 2015, and the positive predictive value of CT and combined CT and ultrasonography was 100%. The rate of complicated appendicitis increased (23% in 2015). CONCLUSIONS: The use of imaging tests increased, and the rate of "blind" laparotomies decreased. Nevertheless, the rate of complicated appendicitis increased.

Estudio retrospectivo y comparativo de la utilidad y del valor predictivo positivo de la ecografía y la tomografía computarizada en el diagnóstico de apendicitis aguda en adultos / The usefulness and positive predictive value of ultrasonography vs. computed tomography in the diagnosis of acute appendicitis in adults: a retrospective study

Objetivo: Analizar la evolución del uso de pruebas de imagen (ecografía y tomografía computarizada [TC]) en el diagnóstico de apendicitis aguda, el valor predictivo positivo (VPP) y determinar el porcentaje de apendicectomías negativas y complicadas. Material y métodos: Estudio retrospectivo que incluye a los pacientes adultos con apendicectomía por sospecha de apendicitis aguda en 2015 en un hospital terciario. Se compara con los del 2007 publicados previamente. Resultados: La muestra incluye 278 pacientes. La tasa de apendicectomías negativas descendió a un 5%. El VPP de la ecografía aumentó a 97,4% en 2015 y el VPP de la TC y del uso combinado de la ecografía y la TC fue del 100%. El porcentaje de apendicitis complicadas se incrementó (23% en 2015). Conclusiones: Se observó un aumento en el empleo de pruebas de imagen y una disminución de laparotomías en blanco. No obstante, las apendicitis complicadas se han incrementado.(AU)

Objective: To analyze the evolution of the use of imaging tests (ultrasonography and computed tomography (CT)) in the diagnosis of acute appendicitis. To determine the positive predictive value of these tests and the percentage of negative and complicated appendectomies. Material and methods: This retrospective study compared adults who underwent appendectomy for suspected acute appendicitis at our tertiary hospital during 2015 versus similar patients at our center during 2007. Results: A total of 278 patients were included. The rate of negative appendectomies descended to 5%. The positive predictive value of ultrasonography increased to 97.4% in 2015, and the positive predictive value of CT and combined CT and ultrasonography was 100%. The rate of complicated appendicitis increased (23% in 2015). Conclusions: The use of imaging tests increased, and the rate of “blind” laparotomies decreased. Nevertheless, the rate of complicated appendicitis increased.(AU)

Rescuers' characteristics associated with the correct chest compression during cardiopulmonary resuscitation.

AIM: To analyse the caregivers' physical, anthropometrical and educational characteristics associated with adequate chest compression and full chest recoil during cardiopulmonary resuscitation (CPR). METHODS: An observational prospective research study was conducted. Emergency and critical care health professionals and students performed two minutes of chest compressions on a dummy. Depth and residual leaning after the compressions were assessed and their association with several variables (physical, anthropometrical, and educational) was analysed using logistic regression models. RESULTS: Two hundred thirty-eight volunteers participated. Previous experience of the rescuer in less than six CPRs (OR = 3.03; 95% CI 1.2-7.63) was related to a higher probability of not achieving an adequate depth of compressions. Greater height (OR: .93; 95% CI .87-.99) and grip strength (OR: .94; 95% CI .89-.99) were associated with correct performance of chest compression. We did not find any characteristic related to chest recoil. CONCLUSIONS: The caregiver's previous experience with CPR was the strongest factor associated with adequate performance of chest compressions. To a lesser extent, the professional's height and upper body muscle strength also have an influence. No factors associated with the adequacy of full chest recoil were identified.

Características de los reanimadores asociadas a la correcta compresión torácica durante la reanimación cardiopulmonar / Rescuers’ characteristics associated with the correct chest compression during cardiopulmonary resuscitation

Objetivos: Analizar las características físicas, antropométricas y formativas de los reanimadores asociadas a la correcta compresión y reexpansión torácica durante la reanimación cardiopulmonar. Metodología: Estudio observacional prospectivo. Profesionales y estudiantes sanitarios de urgencias y cuidados críticos realizaron 2min de compresiones torácicas sobre un maniquí. Se evaluó la profundidad y la presión residual tras las compresiones, y se estudió su asociación a diferentes variables (físicas, antropométricas y formativas) mediante la creación de modelos de regresión logística. Resultados: Participaron 238 voluntarios. Que el reanimador tuviese una experiencia previa en menos de 6 reanimaciones cardiopulmonares (OR: 3,03; IC 95%: 1,2-7,63) se asoció a una mayor probabilidad de no lograr una profundidad adecuada en las compresiones. Una mayor estatura (OR: 0,93; IC 95%: 0,87-0,99) y fuerza de aprehensión (OR: 0,94; IC 95%: 0,89-0,99) fueron condiciones que actuaron como factores predisponentes a la ejecución de una técnica correcta. Ninguna característica se asoció a la adecuación de la reexpansión torácica. Conclusiones: La experiencia previa del reanimador es el factor más fuertemente asociado a la correcta ejecución de las compresiones torácicas. En menor medida, también influye la estatura y la fuerza del tren superior del profesional. No se han identificado factores asociados a la adecuación de la reexpansión torácica tras las compresiones.(AU)

Aim: To analyse the caregivers’ physical, anthropometrical and educational characteristics associated with adequate chest compression and full chest recoil during cardiopulmonary resuscitation (CPR). Methods: An observational prospective research study was conducted. Emergency and critical care health professionals and students performed two minutes of chest compressions on a dummy. Depth and residual leaning after the compressions were assessed and their association with several variables (physical, anthropometrical, and educational) was analysed using logistic regression models. Results: Two hundred thirty-eight volunteers participated. Previous experience of the rescuer in less than six CPRs (OR: 3.03; CI 95%: 1.2-7.63) was related to a higher probability of not achieving an adequate depth of compressions. Greater height (OR: .93; 95% CI: .87-.99) and grip strength (OR: .94; 95% CI: .89-.99) were associated with correct performance of chest compression. We did not find any characteristic related to chest recoil. Conclusions: The caregiver's previous experience with CPR was the strongest factor associated with adequate performance of chest compressions. To a lesser extent, the professional's height and upper body muscle strength also have an influence. No factors associated with the adequacy of full chest recoil were identified.(AU)

Rescuers' characteristics associated with the correct chest compression during cardiopulmonary resuscitation. / Características de los reanimadores asociadas a la correcta compresión torácica durante la reanimación cardiopulmonar.

AIM: To analyse the caregivers' physical, anthropometrical and educational characteristics associated with adequate chest compression and full chest recoil during cardiopulmonary resuscitation (CPR). METHODS: An observational prospective research study was conducted. Emergency and critical care health professionals and students performed two minutes of chest compressions on a dummy. Depth and residual leaning after the compressions were assessed and their association with several variables (physical, anthropometrical, and educational) was analysed using logistic regression models. RESULTS: Two hundred thirty-eight volunteers participated. Previous experience of the rescuer in less than six CPRs (OR: 3.03; CI 95%: 1.2-7.63) was related to a higher probability of not achieving an adequate depth of compressions. Greater height (OR: .93; 95% CI: .87-.99) and grip strength (OR: .94; 95% CI: .89-.99) were associated with correct performance of chest compression. We did not find any characteristic related to chest recoil. CONCLUSIONS: The caregiver's previous experience with CPR was the strongest factor associated with adequate performance of chest compressions. To a lesser extent, the professional's height and upper body muscle strength also have an influence. No factors associated with the adequacy of full chest recoil were identified.