The Pivotal Role of Echocardiography in the Diagnosis of Stress-Induced Cardiomyopathy Presenting with Atypical Pattern in Critically Ill Children. An Illustrative Case Report.

BACKGROUND: Takotsubo cardiomyopathy (TCM) has some distinctive features like greater proportion of reverse-TCM and central nervous system disease as a prevalent triggering cause. We expose the case of a child with cardiogenic shock presenting an atypical echocardiographic TCM pattern on an echocardiography, after an acute neurologic trigger. We also include a systematic literature review of previously described cases of atypical-TCM in children. CASE REPORT: A previously healthy 9 year-old boy with status epilepticus presented abrupt cardiogenic shock. The EKG showed signs of myocardial ischemia, cardiac biomarkers NT-proBNP (2756 pg/mL ) and Troponin I (1707 pg/mL ) , and the echocardiography exposed a dilated LV with severely reduced systolic function (LVEF 28%) along with hypokinetic mid-basal segments (circumferential ballooning), and preserved hypercontractile apical segments, with the normal origin of both coronary arterial systems. A presumptive diagnosis of "reverse", "inverse" or atypical Takotsubo cardiomyopathy was built based on the echocardiographic findings, apart from the ACS-like EKG findings, the raised cardiac biomarkers, and the neurological trigger of the hyper catecholaminergic state. Despite cardiovascular improvement with supportive treatment, the patient eventually expired on day 2 after PICU admission due to neurological complications. As shown in our systematic review, only 19 similar cases have been reported to date. CONCLUSION: With the report of this unusual case, we aim to point out the fundamental role of bedside echocardiography as a diagnostic test for critically ill children presenting with ACS-like in the context of neurosurgical emergencies, where bedside echocardiography itself can accurately establish a presumptive diagnosis of TCM.

Síndrome del abdomen en ciruela pasa / Prune belly syndrome

No disponible

The FXII c.-4T>C Polymorphism as a Disease Modifier in Patients With Hereditary Angioedema Due to the FXII p.Thr328Lys Variant.

BACKGROUND: Hereditary angioedema due to the Thr328Lys variant in the coagulation factor XII (HAE-FXII) affects mainly women in whom the symptomatology is dependent on high estrogen levels. Clinical variability and incomplete penetrance are challenging features that hinder the diagnosis and management of HAE-FXII. The c.-4T>C Kozak polymorphism is the only common variation accounting for FXII plasma levels and was previously shown to modify the course of HAE due to C1-Inhibitor deficiency. OBJECTIVES: To assess the influence of the c.-4T>C polymorphism on disease expression in 39 Spanish HAE-FXII index patients. METHODS: The c.-4T>C polymorphism was sequenced by the standard Sanger method, and HAE severity was calculated according to the score by Cumming et al. (2003) The activation of the contact system was quantified by the kallikrein-like activity of plasma in chromogenic assays upon activation with high-molecular-weight dextran sulfate. RESULTS: The c.-4CC genotype was overrepresented in the studied cohort: 82% were CC-homozygous (expected frequency = 59%) and 18% were CT-heterozygous (expected frequency = 39%) (p = 0.001). Patients with a c.-4CC genotype exhibited higher kallikrein-like activity (0.9659 ± 0.1136) than those with a c.-4TC genotype (0.7645 ± 0.1235) (p = 0.024) or healthy donors. Moreover, the polymorphism influenced HAE-FXII severity score (c.-4CC = 4.43 ± 2.28 vs c.-4TC = 2.0 ± 1.15; p = 0.006) but not the degree of estrogen dependence or time until remission. CONCLUSION: The c.-4T>C polymorphism is overrepresented in a Spanish HAE-FXII cohort and significantly influences the degree of contact system activation and the clinical severity of the disease.

Intradermal Phleum pratense allergoid immunotherapy. Double-blind, randomized, placebo-controlled trial.

BACKGROUND: In allergology, the intradermal approach is generally used to establish an aetiological diagnosis, with limited experience in specific allergen immunotherapy. OBJECTIVE: To evaluate the efficacy and safety of immunotherapy with an allergen extract of glutaraldehyde-polymerized Phleum pratense, administered intradermally, in patients with rhinoconjunctivitis sensitized to grass pollen. METHODS: Multicentre, randomized, double-blind, placebo-controlled clinical trial in patients from 12 to 65 years of age with rhinitis or rhinoconjunctivitis, with or without asthma, due to grass pollen allergy. Patients were divided into three groups and received a total of six doses in a weekly interval, of either placebo; 0.03 or 0.06 µg of protein per dose of P pratense allergoid. The primary objective was to evaluate the combined symptoms and medication consumption score (CSMS). The secondary objectives were symptoms and medication, tolerance to the conjunctival provocation test, specific IgE and IgG4 antibodies and the safety profile according to the WAO scale. RESULTS: The dose of 0.06 µg of protein proved to be effective versus the placebo by significantly reducing CSMS and increasing tolerance to the allergenic extract in the conjunctival provocation test, after the first pollen season. This group showed a significant reduction in specific IgE after the second pollen season relative to the baseline. There were no variations in IgG4 levels. Only one grade 2 systemic reaction was recorded. CONCLUSION & CLINICAL RELEVANCE: Intradermal immunotherapy with P pratense allergoid has been shown to be effective and safe, reducing CSMS, increasing tolerance to the conjunctival provocation test and reducing IgE levels.

Lentil sprouts: a nutraceutical alternative for the elaboration of bread.

The pro-health action of germinated lentils could be useful to be added with wheat flour in the production of box bread. In this work, we spectroscopically evaluate the germinated and non-germinated lentils, and use them at the concentrations of 5 and 10% for the production of box bread. The chemical and physical tests of the bread and its determination of phenolic acids and flavonoids (by HPLC) were also performed. As well as the evaluation of the quality of flour and dough used to produce the bread and the acceptance of the germinated lentil bread with a population of 20 people with diabetes or with diabetic relatives It is shown that: (1) The amplitude of photoacoustic signal obtained by photoacoustic spectroscopy is modified as a function of the percentage of germinated lentil (GL) flour (0, 5 or 10%) add to the bread; being higher the photoacoustic amplitude to higher concentration of GL in the absorption band of 300-425 nm, which is related to higher content of phenols and flavonoids. (2) The contents of phenolic acids (Sinapinic, ß- resorcylic, Chlorogenic and Ferulic) and flavonoids (Quercetin and Isorhamnetin) tended to increase in the germinated lentil bread with 10% concentration of germinated lentil flour with respect to the control bread (0% GL). (3) The addition of germinated lentils flour to 5 and 10% into wheat flour to produce bread with higher hardness and less cohesiveness than bread based on wheat flour only. The Falling number indicate that there is no significant difference between the control sample and the 5% GL flour, while in the 10% GL flour there was a reduction of 21 s, with respect to the control. The effect of the germinated lentil flour percentage on the pasting properties of the flours was significant between the control and 10% GL flour. In general, the quality of the dough and flour are modified due to the addition of germinates lentils, and this affectation increases with the increase in the concentration of GL. (4) The bread added with germinated lentil has sensory acceptance with a group of people with diabetes and/or diabetic relatives in their attributes in general. The obtained results thus support the production of wheat bread with mixed germinated lentils flour, as a nutraceutical option for human consumption.

Rationale and Methodology of the SARAH Trial: Long-Term Cardiovascular Outcomes in Patients With Resistant Hypertension and Obstructive Sleep Apnea / Justificación y metodología del ensayo clínico SARAH: evolución cardiovascular a largo plazo en pacientes con hipertensión resistente y apnea obstructiva del sueño

Introduction: Patients with resistant hypertension (RH) have a high risk of developing cardiovascular events; therefore, new therapeutic approaches to better control blood pressure may be useful in improving cardiovascular outcomes. The prevalence of obstructive sleep apnea (OSA) is very high among patients with RH. Continuous positive airway pressure (CPAP) has been shown to be an effective treatment for reducing blood pressure in patients with RH. Nevertheless, the long-term effect of CPAP treatment on cardiovascular outcomes has not been explored. The main objective of the SARAH study is to assess the impact of OSA and its treatment on cardiovascular outcomes (morbidity and mortality) in patients with RH. Methods: This study is a multi-center, prospective, observational cohort study. A total of 1371 patients with RH will be enrolled in the study and followed once a year for five years. At inclusion, ambulatory blood pressure monitoring (ABPM) and a sleep study will be performed in all subjects. Socio-demographic, clinical and cardiovascular variables will be collected at baseline and follow-up. Subsequently, subjects with OSA will be managed according to local standard practice. Based on the OSA diagnosis and its treatment, three cohorts of subjects with RH will be defined: non-OSA, treated OSA and non-treated OSA. Conclusions: This study will contribute to elucidating the long-term impact of OSA treatments on blood pressure control and cardiovascular outcomes in patients with RH. These results will contribute to improve the cardiovascular prognosis of patients with RH

Introducción: Los pacientes con hipertensión resistente (HR) tienen un elevado riesgo de desarrollar eventos cardiovasculares; así, las nuevas estrategias terapéuticas para el control de la presión arterial podrían ser útiles para mejorar los resultados cardiovasculares. La prevalencia de la apnea obstructiva del sueño (AOS) es muy elevada entre los pacientes con HR. Se ha demostrado que la presión positiva continua en la vía aérea (CPAP) constituye un tratamiento efectivo para reducir la presión arterial en pacientes con HR. Sin embargo, todavía no se han estudiado los efectos a largo plazo del tratamiento con CPAP sobre los resultados cardiovasculares. El principal objetivo del estudio SARAH es evaluar el impacto de la AOS y su tratamiento en los resultados cardiovasculares (morbilidad y mortalidad) en pacientes con HR. Métodos: Este es un estudio observacional prospectivo multicéntrico en el que se incluirán 1.371 pacientes con HR, a los cuales se les realizará seguimiento una vez al año durante 5 años. En el momento de la inclusión se realizará a todos los pacientes monitorización ambulatoria de la presión arterial (MAPA), así como un estudio de sueño. Se recogerán las variables sociodemográficas, clínicas y cardiovasculares tanto al inicio del estudio como durante el seguimiento. Posteriormente, los individuos con AOS serán tratados de acuerdo con la práctica clínica estándar de cada centro. Se definirán 3 grupos en función del diagnóstico de AOS y su tratamiento: sin AOS, con AOS en tratamiento, con AOS sin tratar. Conclusiones: Este estudio contribuirá a averiguar el impacto a largo plazo del tratamiento de la AOS en el control de la presión arterial y los resultados cardiovasculares en pacientes con HR. Asimismo, estos resultados contribuirán a mejorar el pronóstico cardiovascular de los pacientes con HR

Rationale and Methodology of the SARAH Trial: Long-Term Cardiovascular Outcomes in Patients With Resistant Hypertension and Obstructive Sleep Apnea.

INTRODUCTION: Patients with resistant hypertension (RH) have a high risk of developing cardiovascular events; therefore, new therapeutic approaches to better control blood pressure may be useful in improving cardiovascular outcomes. The prevalence of obstructive sleep apnea (OSA) is very high among patients with RH. Continuous positive airway pressure (CPAP) has been shown to be an effective treatment for reducing blood pressure in patients with RH. Nevertheless, the long-term effect of CPAP treatment on cardiovascular outcomes has not been explored. The main objective of the SARAH study is to assess the impact of OSA and its treatment on cardiovascular outcomes (morbidity and mortality) in patients with RH. METHODS: This study is a multi-center, prospective, observational cohort study. A total of 1371 patients with RH will be enrolled in the study and followed once a year for five years. At inclusion, ambulatory blood pressure monitoring (ABPM) and a sleep study will be performed in all subjects. Socio-demographic, clinical and cardiovascular variables will be collected at baseline and follow-up. Subsequently, subjects with OSA will be managed according to local standard practice. Based on the OSA diagnosis and its treatment, three cohorts of subjects with RH will be defined: non-OSA, treated OSA and non-treated OSA. CONCLUSIONS: This study will contribute to elucidating the long-term impact of OSA treatments on blood pressure control and cardiovascular outcomes in patients with RH. These results will contribute to improve the cardiovascular prognosis of patients with RH.

[Algorithm for diagnosis and treatment of hereditary angioedema as a tool for management]. / Algoritmo de diagnóstico y tratamiento del angioedema hereditario como herramienta para su manejo.

INTRODUCTION: Hereditary angioedema is a disease with low prevalence and high heterogeneity with regards to the severity of the clinical picture, which makes the diagnosis difficult and requires the need for early start of specific treatment in order to prevent complications. OBJECTIVE: To propose a decision algorithm for hereditary angioedema (HAE), based on the evidence available on the diagnosis, clinical assessment, and treatment. The aim is to present the available therapeutic options as well as a decision algorithm to select the most efficient therapy at each time. MATERIAL AND METHODS: Literature search by means of PubMed and other relevant sources. RESULTS: four decision algorithms have been developed for HAE; diagnosis of bradikinin-mediated angioedema, treatment of acute attacks and short and long-term prophylaxis for HAE due to C1 inhibitor deficiency. CONCLUSIONS: The application of a decision algorithm based on the clinical variables helps to select the most efficient therapeutic option at each time and may be a useful tool for the therapeutic approach.

Introducción: El angioedema hereditario es una enfermedad rara de baja prevalencia y gran heterogeneidad en la gravedad del cuadro clínico, lo que dificulta su diagnóstico, y establece la necesidad de iniciar un tratamiento precoz y específico con el fin de evitar complicaciones. Objetivo: Proponer un algoritmo de decisión en el angioedema hereditario (AEH), basado en la evidencia disponible, sobre el diagnóstico, valoración clínica y tratamiento. Se trata de presentar opciones terapéuticas disponibles, así como un algoritmo de decisión para seleccionar el tratamiento más eficiente en cada momento. Material y Métodos: Revisión bibliográfica mediante una búsqueda a través de PubMed y otras fuentes de interés. Resultados: Se han desarrollado cuatro algoritmos de decisión para el AEH; diagnóstico de angioedema mediado por bradicinina, tratamiento del ataque agudo y profilaxis a corto y largo plazo del AEH por déficit del inhibidor C1. Conclusiones: La aplicación de un algoritmo de decisión, en función de unas variables clínicas, ayuda a la selección de la opción terapéutica más eficiente en cada momento y puede ser un instrumento de utilidad en el abordaje terapéutico.

Evaluación de la satisfacción de los adultos mayores en relación al uso de sobredentaduras mandibulares retenidas por implantes dentales intermentonianos / Evaluation of the satisfaction of older adults related to the use of mandibular dentures held by interforaminal dental implants

El tratamiento de los pacientes adultos mayores y edéntulos totales mandibulares mediante sobre dentaduras implanto retenidas se ha convertido en una terapia viable reconocida mundialmente. Evaluar la satisfacción de los pacientes de edad avanzada, portadores de sobredentaduras implanto-retenidas mandibulares en cuanto a su estabilidad, estética, habilidad para masticar los alimentos y reír. Investigación de carácter descriptivo evaluativo con diseño no experimental tipo transeccional. Se seleccionó una muestra a conveniencia de 9 pacientes a los que se les colocó dos implantes dentales intermentonianos para posteriormente se rehabilita dos con sobre dentaduras implanto retenidas mediante un sistema de bola y anillo. Se elaboró un cuestionario de tipo cerrado que fue llenado por cada paciente después de insertadas las sobredentaduras, y transcurrido más de 3 meses de uso. El 67 por ciento de los pacientes refirieron no presentar movilidad de la prótesis y el 78 por ciento contestó que se atrevían a comer alimentos de cualquier consistencia, inclusive dura. 89 por ciento respondió que siempre les resultaba fácil colocar la prótesis en los implantes. Ningún paciente refirió morderse la lengua, mejilla, y/o labios con la sobre dentadura. Los implantes dentales oseointegrados son una opción válida en la rehabilitación del paciente edéntulo adulto mayor.

Lipid and lipoprotein concentrations at age 4. Association with neonatal and parental levels.

BACKGROUND AND OBJECTIVE: To study the influence of diet, anthropometrical measurements and neonatal and parental lipoprotein variables on lipoprotein concentrations at age 4. SUBJECTS AND METHOD: 18 neonates with normal serum lipoprotein values (group 1), 19 neonates with high total cholesterol (TC) levels (group 2) and 21 neonates with normal TC but altered levels in other lipids, apolipoproteins, lipoproteins or ratios (group 3) were selected for a follow-up study. Body weight, body mass index (BMI) and the suitability of diet at age 4 for coronary heart disease prevention were evaluated. Multivariable stepwise linear regression analyses were performed for each lipid or lipoprotein parameter at age 4 considering group at birth, diet, neonatal and parental BMI, lipid or lipoprotein parameters. RESULTS: A large percentage of 4 year-olds had high low density lipoproteins-cholesterol (LDLc) and low high density lipoproteins-cholesterol (HDLc) and followed an unsuitable diet. Prevalence of altered lipoprotein variables, except for TC/HDLc, was similar in the 3 groups. Correlations of birth versus 4 year levels were significant (p = 0.021-0.0001) for all parameters except triglycerides, TC and LDLc. However, all tracking correlations were not significant in group 3. In the multiple regression study, parental and neonatal parameters were retained as explicative variables in many of the models but diet was not retained in any of them. Maternal concentrations were always more explicative than paternal or neonatal ones. Models for TC/HDLc and LDLc/HDLc were the most explicative (both, R2 > 0.578; p < 0.0001). CONCLUSIONS: Lipoprotein variables at age 4 were more closely associated with progenitors' and neonatal lipoprotein values than BMI or diet.

Lipid and lipoprotein concentrations at age 4. Association with neonatal and parental levels

Fundamento y objetivo: Estudiar la influencia de la dieta, de los parámetros antropométricos y de las características lipoproteínicas al nacimiento y de los padres sobre la concentración de lipoproteínas a los 4 años de edad. Sujetos y método: Se seleccionó para participar en un estudio de seguimiento a los 4 años a 18 niños con un perfil lipoproteico normal al nacer (grupo 1), a otros 19 con concentraciones elevadas de colesterol total (CT) al nacer (grupo 2) y a 21 con CT normal pero valores anormales al nacer de triglicéridos, apolipoproteínas, lipoproteínas y cocientes lipoproteínicos (grupo 3). Se evaluaron el peso corporal, el índice de masa corporal (IMC) y la adecuación de la dieta a los 4 años desde el punto de vista cardiovascular. Se realizó un análisis de regresión lineal múltiple por pasos para explicar la concentración a los 4 años, considerando la dieta, el IMC y los parámetros lipoproteínicos de ambos padres y de los recién nacidos. Resultados: A los 4 años un gran porcentaje de niños tenía concentraciones elevadas de colesterol unido a lipoproteínas de baja densidad (cLDL), cifras bajas de colesterol unido a lipoproteínas de alta densidad (cHDL) y una dieta incorrecta, rica en colesterol y ácidos grasos saturados. La prevalencia de concentraciones lipoproteínicas alteradas fue similar en los 3 grupos, excepto para el cociente CT/cHDL. Las correlaciones entre las variables al nacimiento y a los 4 años fueron todas significativas (p = 0,021-0,0001), salvo para los triglicéridos, el CT y el cLDL. En el grupo 3 se perdía la significación en todas las variables. En las regresiones múltiples las variables maternas, paternas y de los recién nacidos aparecieron como variables explicativas en un gran número de modelos. Las concentraciones maternas fueron más explicativas que las paternas y las de los neonatos. Los modelos más explicativos fueron para CT/cHDL y cLDL/cHDL (ambos, R2 > 0,578; p < 0,0001). Conclusiones: A los 4 años, las variables lipoproteínicas, más que el IMC o la dieta, se asocian a los valores lipoproteínicos de los progenitores y los de los niños al nacer

Background and objective: To study the influence of diet, anthropometrical measurements and neonatal and parental lipoprotein variables on lipoprotein concentrations at age 4. Subjects and method: 18 neonates with normal serum lipoprotein values (group 1), 19 neonates with high total cholesterol (TC) levels (group 2) and 21 neonates with normal TC but altered levels in other lipids, apolipoproteins, lipoproteins or ratios (group 3) were selected for a follow-up study. Body weight, body mass index (BMI) and the suitability of diet at age 4 for coronary heart disease prevention were evaluated. Multivariable stepwise linear regression analyses were performed for each lipid or lipoprotein parameter at age 4 considering group at birth, diet, neonatal and parental BMI, lipid or lipoprotein parameters. Results: A large percentage of 4 year-olds had high low density lipoproteins-cholesterol (LDLc) and low high density lipoproteins-cholesterol (HDLc) and followed an unsuitable diet. Prevalence of altered lipoprotein variables, except for TC/HDLc, was similar in the 3 groups. Correlations of birth versus 4 year levels were significant (p = 0.021-0.0001) for all parameters except triglycerides, TC and LDLc. However, all tracking correlations were not significant in group 3. In the multiple regression study, parental and neonatal parameters were retained as explicative variables in many of the models but diet was not retained in any of them. Maternal concentrations were always more explicative than paternal or neonatal ones. Models for TC/HDLc and LDLc/HDLc were the most explicative (both, R2 > 0.578; p < 0.0001). Conclusions: Lipoprotein variables at age 4 were more closely associated with progenitors' and neonatal lipoprotein values than BMI or diet

Improvement of detection specificity of Plasmodium-infected murine erythrocytes by flow cytometry using autofluorescence and YOYO-1.

BACKGROUND: Microscopic analysis of blood smears is currently the most frequently used method to measure parasitemias in experiments of drug efficacy in murine models of malaria. However, it is subjective and labour intensive, which preclude its utilization in large-scale evaluation programs. Flow cytometry is an alternative method, but due to the limited specificity achieved with the currently available techniques, it has not been widely used in murine models of malaria during preclinical evaluation. We describe a new flow cytometric method based on the differences of autofluorescence and DNA content measured after staining with YOYO-1 that are observed in infected erythrocytes compared with noninfected erythrocytes. METHODS: Samples of blood from Plasmodium yoelii-infected animals were fixed with glutaraldehyde, incubated with RNAase, and stained with YOYO-1 in 96-well plate format. After acquisition, erythrocytes gated in logarithmic side/scatter plots were analyzed in bidimensional FL-2/YOYO-1 plots in comparison with unidimensional YOYO-1 analysis. RESULTS: The infected erythrocytes showed a characteristic pattern of staining different from that of noninfected erythrocytes. In routine evaluation, the limit of sensitivity was 0.01% and the measurements of parasitemia were linear at parasitemias above 0.1%. Interestingly, using this approach, infected reticulocytes could be differentiated from infected normocytes. CONCLUSIONS: The method described is robust, increases the specificity and sensitivity of detection in routine testing, and is especially well suited for detection of low parasitemias in murine models of malaria.