How to reduce the complication rate of multiple burr holes surgery in moyamoya angiopathy.

PURPOSE: This study is aimed at analyzing clinical outcome, absence of stroke recurrence, revascularization, and complications and long-term follow-up in the surgical treatment of moyamoya angiopathy (MMA) using the multiple burr holes (MBH) technique with dura opening and arachnoid preservation as a single procedure. To the best of our knowledge, this is the first to describe an MBH technique with arachnoid preservation. METHOD: We retrospectively reviewed all patients operated from June 2001 to March 2021, for a symptomatic and progressive MMA operated with opening of the dura but arachnoid preservation. Clinical examinations were obtained in all patients, and radiological monitoring was performed by cerebral 3D-magnetic resonance angiography (MRA) with perfusion or single-photon emission computed tomography (SPECT) with acetazolamide. RESULTS: In total, 21 consecutive patients (6 children and 15 adults) were included with a mean age of 7.4 years in the pediatric group and 36.9 years in the adult group. Initial presentation was permanent ischemic stroke in 15 cases, transient ischemic attack (TIA) in 5 cases, and cerebral hemorrhage in one case. The MBH with dura opening and arachnoid preservation was performed bilaterally in 9 cases (43%) and unilaterally in 12 cases (57%). One patient died due to intraoperative bilateral ischemic stroke. Of the 20 other patients, 30% demonstrated clinical stability and 70% showed partial or complete recovery. Although one patient experienced a perioperative stroke, we did not observe any pseudomeningocele or postoperative ischemic stroke (IS) recurrence in all surviving cases during the average follow-up period of 55.5 months (range: 1-195). These outcomes emphasize the importance of preoperative monitoring to ensure the effectiveness and safety of the intervention. Postoperative angiography studies showed revascularization in 96.3% of treated hemispheres (100% in the adult group vs 80% in the pediatric group). CONCLUSIONS: Our results on this small cohort suggest that the MBH technique with opening of the dura and arachnoids preservation can prevent recurrent strokes and reduce the risk of pseudomeningocele.

Measurement of the Cosmic Ray Helium Energy Spectrum from 70 GeV to 80 TeV with the DAMPE Space Mission.

The measurement of the energy spectrum of cosmic ray helium nuclei from 70 GeV to 80 TeV using 4.5 years of data recorded by the Dark Matter Particle Explorer (DAMPE) is reported in this work. A hardening of the spectrum is observed at an energy of about 1.3 TeV, similar to previous observations. In addition, a spectral softening at about 34 TeV is revealed for the first time with large statistics and well controlled systematic uncertainties, with an overall significance of 4.3σ. The DAMPE spectral measurements of both cosmic protons and helium nuclei suggest a particle charge dependent softening energy, although with current uncertainties a dependence on the number of nucleons cannot be ruled out.

Pseudomonas stutzeri MJL19, a rhizosphere-colonizing bacterium that promotes plant growth under saline stress.

AIMS: The aim of this study was to find and use rhizobacteria able to confer plants advantages to deal with saline conditions. METHODS AND RESULTS: We isolated 24 different bacterial species from the rhizosphere of halophyte plants growing in Santiago del Estero, Argentina salt flat. Four strains were selected upon their ability to grow in salinity and their biochemical traits associated with plant growth promotion. Next, we tested the adhesion on soybean seeds surface and root colonization with the four selected isolates. Isolate 19 stood out from the rest and was selected for further experiments. This strain showed positive chemotaxis towards soybean root exudates and a remarkable ability to form biofilm both in vitro conditions and on soybean roots. Interestingly, this trait was enhanced in high saline conditions, indicating the extremely adapted nature of the bacterium to high salinity. In addition, this strain positively impacted on seed germination, plant growth and general plant health status also under saline stress. CONCLUSIONS: A bacterium isolate with outstanding ability to promote seed germination and plant growth under saline conditions was found. SIGNIFICANCE AND IMPACT OF THE STUDY: The experimental approach allowed us to find a suitable bacterial candidate for a biofertilizer intended to alleviate saline stress on crops. This would allow the use of soil now considered inadequate for agriculture and thus prevent further advancement of agriculture frontiers into areas of environmental value.

Measurement of the cosmic ray proton spectrum from 40 GeV to 100 TeV with the DAMPE satellite.

The precise measurement of the spectrum of protons, the most abundant component of the cosmic radiation, is necessary to understand the source and acceleration of cosmic rays in the Milky Way. This work reports the measurement of the cosmic ray proton fluxes with kinetic energies from 40 GeV to 100 TeV, with 2 1/2 years of data recorded by the DArk Matter Particle Explorer (DAMPE). This is the first time that an experiment directly measures the cosmic ray protons up to ~100 TeV with high statistics. The measured spectrum confirms the spectral hardening at ~300 GeV found by previous experiments and reveals a softening at ~13.6 TeV, with the spectral index changing from ~2.60 to ~2.85. Our result suggests the existence of a new spectral feature of cosmic rays at energies lower than the so-called knee and sheds new light on the origin of Galactic cosmic rays.

Endocarditis infecciosa nativa en pediatría: enfoque clínico cardiológico / Native valve infective endocarditis in children: From the focus of clinical cardiology

Objetivos: Analizar las características demográficas, clínicas, microbiológicas, evolución y factores de riesgo de morbimortalidad asociados a la endocarditis de válvula nativa (EIN) en pacientes (p) pediátricos. Población y métodos: Se evaluaron 176 p con EIN, divididos en grupo I: <3meses (27p) y grupo II: >3meses (149p). Resultados: Grupo I: el 66% tenía corazón sano. El microorganismo más frecuente fue Staphylococcus aureus (44,4%). Afectación derecha (77,8%-p<0,0001). Evento principal: infección no controlada (INC) (52%, p=0,0009) y asociada a Candida (p<0,00001). Se indicó tratamiento quirúrgico a 10 p (37%). Mortalidad 29,6%(8p). Grupo II: el 57% presentaba cardiopatía previa. Microorganismos prevalentes: S.aureus (49,1%) y estreptococos del grupo viridans (22,5%). Hubo compromiso predominantemente izquierdo (p=0,001). Eventos: embolias sistémicas (36,2%-p=0,01), perforación valvular (51%-p=0,0005), insuficiencia cardíaca (26%-p=0,03) e INC (21,5%). La embolia sistémica se asoció a S.aureus (p=0,01). El 36,2% requirió cirugía. Mortalidad 6,7% (10p). En el análisis univariado la mortalidad se asoció a edad <3meses (p=0,0003), INC (p=0,002) y S.aureus (p=0,03). En el multivariado la mortalidad se relacionó a edad < 3meses (OR:7,50 ­IC95%:1,77­31,69) y a INC (OR:4,2-IC95%:1,16­15,29). Conclusiones: La EN se presentó en pacientes con corazón sano en el 50% de los casos. El microorganismo más frecuente fue S.aureus. En los <3 meses la infección no controlada fue la complicación más frecuente asociada a Candida, con afectación predominante de cavidades derechas. Los >3meses tuvieron mayor prevalencia de perforación valvular izquierda asociada a embolias sistémicas e insuficiencia cardíaca. El 35% de los p requirió cirugía. Los predictores de mortalidad fueron la edad <3meses y la INC.

Objectives: To analyze demographic, clinical, and microbiological, outcome, and morbidity and mortality risk factors associated with native valve endocarditis (NVE) in pediatric patients (p). Population and methods: 176 p with NVE were evaluated and divided into group I: <3 months (27p) and group II: >3 months (149p). Results: Group I: 66% had a healthy heart. The most common microorganism was Staphylococcus aureus (44.4%). Right-sided involvement (77.8%-p<0.0001). Main event: Uncontrolled infection (UCI) (52%, p-0.0009) and association with Candida (p<0.00001). Surgical treatment was indicated in 10 p (37%). Mortality was 29.6% (8p). Group II: 57% had previous heart disease. Prevalent microorganisms: S. aureus (49.1%) and viridans group streptococci ( (22.5%). Left-sided involvement predominated (p-0.001). Events: systemic embolism (36.2%-p-0.01), valve perforation (51%-p-0.0005), heart failure (26%-p-0.03), and UCI (21.5%). Systemic embolism was associated with S. aureus infection (p-0.01). 36.2% required surgery. Mortality was 6.7% (10p). In univariate analysis, mortality was associated with age <3 months (p-0.0003), UCI (p-0.002), and S. aureus infection (p-0.03). In multivariate analysis, mortality was related to age <3 months (OR:7.50 ­ 95% CI:1.77­31.69) and UCI (OR:4.2 -95% CI:1.16­15.29). Conclusions: NVE was observed in patients with a healthy heart in 50% of cases. The most common microorganism found was S. aureus. In the <3 months group, uncontrolled infection was the most common complication associated with Candida, predominantly affecting the right side. The >3 months group had a higher prevalence of left-valve perforation associated with systemic embolism and heart failure. 35% of p required surgery. Predictors of mortality were age <3 months and UC (AU)

Cardiopatías congénitas asociadas a la microdeleción 22q11. 2, en un hospital pediátrico / Congenital heart defects associated with a 22q11. 2 microdeletion at a pediatric hospital

Introducción: El síndrome de deleción 22q11.2, también llamado síndrome Velo-Cardio-Facial (VCFS/del22q11.2) o síndrome de DiGeorge, es una entidad causada por una anomalía cromosómica, deleción en la región q11.2 (brazo largo) del cromosoma 22. Se trata de una enfermedad multisistémica de expresión variable que afecta el aparato cardiovascular, la inmunidad, las funciones endocrinológicas, la cavidad oral, el desarrollo neurocognitivo, con una expresión facial particular. La prevalencia estimada es de 1:2000/4000. Objetivos: Identificar y describir las cardiopatías congénitas más frecuentemente asociadas a pacientes con síndrome de microdeleción 22q11.2. Materiales y métodos: Estudio descriptivo, transversal y retrospectivo que analiza los pacientes con diagnóstico de microdeleción 22q11.2 atendidos en el Hospital Garrahan desde Octubre de 1998 hasta Febrero 2018. El criterio diagnóstico fueron signos clínicos compatibles y la presencia de la microdeleción 22q11.2 por técnica de FISH o MLPA. Resultados: Población: 321 pacientes, 151 Femeninos (47%) 170 Masculinos (53%). Rango etario: 0 a 197 meses (1 día a 16,4 años). Mediana de edad al diagnóstico clínico: 31 meses. El 74,4% (239/321) de los pacientes evaluados con microdeleción 22q11.2 tuvieron cardiopatías congénitas asociadas a facies peculiar. Las cardiopatías congénitas más frecuentemente asociadas fueron conotroncales. De los pacientes con cardiopatías congénitas el 68,6% requirió cirugía cardiovascular. Fallecieron 24 pacientes (10%) con cardiopatías congénitas asociadas y en el 93% la causa de muerte estuvo relacionada a la afección cardiológica. Conclusiones: Los pacientes con microdeleción 22q11.2 se asocian con un alto porcentaje de cardiopatías congénitas, la gran mayoría son complejas (conotroncales) y requieren resolución quirúrgica en los primeros años de vida. Es de vital importancia la evaluación multidisciplinaria de este grupo especial de pacientes con cardiopatía asociada a otras alteraciones extra cardíacas para el diagnóstico precoz y tratamiento oportuno (AU)

Introduction: 22q11.2 deletion syndrome, also called velocardiofacial syndrome (VCFS/del22q11.2) or DiGeorge syndrome, is a condition caused by chromosomal abnormality, a deletion in the q11.2 region (long arm) of chromosome 22. VCFS is a multisystem disease of variable expression that affects the cardiovascular, immune, and endocrine systems, the oral cavity, neurocognitive development, and is associated with specific facial features. The estimated prevalence is 1:2000/4000. Objectives: To identify and describe the most common congenital heart defects associated with 22q11.2 micro-deletion syndrome. Materials and methods: Descriptive, cross-sectional, and retrospective study analyzing patients diagnosed with a 22q11.2 microdeletion seen at Garrahan Hospital from October 1998 to February 2018. Diagnostic criteria were compatible clinical signs and the presence of a 22q11.2 microdeletion identified by FISH or MLPA. Results: Population: 321 patients, 151 female (47%) and 170 Male (53%). Age range: 0 to 197 months (1 day to 16.4 years). Median age at clinical diagnosis: 31 months. Overall, 74.4% (239/321) of patients with a 22q11.2 microdeletion had congenital heart defects associated with a peculiar facies. The most commonly associated congenital heart defects were conotruncal. Of the patients with congenital heart defects, 68.6% required cardiovascular surgery. Of the patients with congenital heart defects 24 patients died (10%) and in 93% the cause of death was related to the heart disease (p 0.002). Conclusions: A high percentage of patients with a 22q11.2 microdeletion have congenital heart defects, which are complex (conotruncal) in the majority, requiring surgical treatment in the first years of life. Multidisciplinary evaluation of this special group of patients with heart defects associated with other extracardiac disorders is essential for an early diagnosis and timely treatment (AU)

Consultorio de orientaciòn de cardilogía: epidemiología y perfil de la población / Cardiology guidance clinic: Epidemiology and profile of the population

Introducción: Las cardiopatías congénitas (CC) son las anomalías congénitas más frecuentes. Representan el 0,8-1,2% de todos los defectos del nacimiento y tienen una prevalencia de alrededor de 5,8 por cada 1000 personas. El Servicio de Cardiología del Hospital Garrahan es un centro de referencia nacional y de países limítrofes donde se realizan 18000 consultas anuales. Los pacientes que concurren por primera vez se atienden en el consultorio de orientación. Objetivo: Describir la epidemiologia y perfil de los pacientes que asisten diariamente al consultorio de orientación de cardiología infantil en un hospital pediátrico de tercer nivel de Buenos Aires. Métodos: Entre septiembre de 2017 y febrero de 2018 se recolectaron los datos de 1000 pacientes atendidos en forma consecutiva en el consultorio de orientación de cardiología. A la totalidad de los pacientes se les realizó anamnesis, examen físico cardiovascular, electrocardiograma, y en los casos en los que se consideró necesario, saturometría, radiografía de tórax y/o ecocardiograma. Las variables a considerar fueron edad, procedencia, presencia o ausencia de cardiopatías congénitas o adquiridas, soplo, cianosis, insuficiencia cardíaca, estado nutricional, síndromes genéticos asociados, métodos diagnósticos e indicaciones terapéuticas implementadas. Se subdividió la población en cinco grupos: Grupo A (pacientes con cardiopatía congénita), Grupo B (cardiopatías operadas), Grupo C (miocardiopatías), Grupo D (arritmias), Grupo E (corazón sano). Resultados: La edad mediana fue 4.86 años (0.03 a 18.9 años). El 64% de los pacientes procedían de la provincia de Buenos Aires. Los motivos de consulta fueron: interconsultas internas 29.5%, derivación por cardiopatía 27.2%, soplo 17.6%, síncope 7%, segunda opinión 5.1%, arritmias 4.8%, precordialgia 3.1%, palpitaciones 2.6%, episodio paroxístico 1.4%, cardiomegalia 0.7%, disnea 0.5%, mal progreso de peso 0.3%. El 10.6% tenían un síndrome genético. Grupo A: 252 pacientes con una edad mediana de 1.9 años. Las cardiopatías acianóticas con hiperflujo pulmonar fueron las más frecuentes (66.66%, 168/252). Grupo B: 51 pacientes, 23.52%(12/51) fueron Fallot reparados en otra institución. Grupo C: 22 pacientes, siendo la miocardiopatía hipertrófica la más frecuente. Grupo D: 47 pacientes, la preexcitación ventricular fue el hallazgo más frecuente (34,04%, 16/47). Grupo E: 628 pacientes, 45.70% (287/628) derivados por pediatras del área ambulatoria, principalmente para valoración de pacientes con enfermedades sistémicas o síndromes genéticos. Conclusión: Los motivos de derivación al consultorio de orientación de cardiología fueron muy diversos. La mayoría de los pacientes provenían de provincia de Buenos Aires. Solamente el 37.2% presentó algún problema cardiológico de base. El 91% de los pacientes que consultaron por soplo, no tuvieron cardiopatía. El grupo correspondiente a los pacientes con cardiopatías no operadas (grupo A) fue el de menor edad (mediana de 1.9 años) y las cardiopatías simples no cianóticas con hiperflujo pulmonar representaron el 66.66% de las cardiopatías. La implementación del ecocardiograma portátil en el consultorio de orientación permitió confirmar el diagnóstico y definir la conducta terapéutica en el 29.4% de los pacientes durante la primer consulta (AU)

Introduction: Congenital heart defects (CHD) are the most common congenital abnormalities. They account for 0.8-1.2% of all birth defects and have a prevalence of around 5.8 per 1000 people. The Department of Cardiology of Garrahan Hospital is a national and bordering-country reference center, receiving 18000 consultations annually. Patients seen for the first time are assessed at the cardiology guidance clinic. Objective: To describe the epidemiology and profile of patients who seen daily at the child cardiology guidance clinic of a third-level pediatric hospital in Buenos Aires. Methods: Between September 2017 and February 2018, data from 1000 patients consecutively seen at the cardiology guidance clinic were collected. All patients underwent anamnesis, cardiovascular physical examination, electrocardiogram and, if considered necessary, pulse oximetry, chest x-ray, and/or echocardiogram. The variables considered were age, place of origin, presence or absence of congenital or acquired heart disease, murmur, cyanosis, heart failure, nutritional status, associated genetic syndromes, diagnostic methods, and treatment. The population was divided into five groups: Group A (patients with congenital heart defects), Group B (operated cardiopathies), Group C (myocardiopathies), Group D (arrhythmias), Group E (healthy heart). Results: Median age was 4.86 years (0.03 to 18.9 years). Overall, 64% of patients came from the province of Buenos Aires. The reasons for consultation were: internal consultations 29.5%, cardiac shunt 27.2%, murmur 17.6%, syncope 7%, second opinion 5.1%, arrhythmias 4.8%, precordialgia 3.1%, palpitations 2.6%, paroxysmal episode 1.4%, cardiomegaly 0.7%, dyspnea 0.5%, 0.3% poor weight gain. A genetic syndrome was identified in 10.6%. Group A: 252 patients with a median age of 1.9 years. Acyanotic congenital heart defect with pulmonary hyperflow was the most common (66.66%, 168/252). Group B: 51 patients, 23.52% (12/51) had tetralogy of Fallot repaired at another institution. Group C: 22 patients, in whom hypertrophic cardiomyopathy was the most common. Group D: 47 patients, in whom ventricular preexcitation was the most common finding (34.04%, 16/47). Group E: 628 patients, 45.70% (287/628) referred by pediatricians from the outpatient clinics, mainly for the assessment of systemic diseases or genetic syndromes. Conclusion: The reasons for referral to the cardiology guidance clinic were varied. Most of the patients came from the province of Buenos Aires. Only 37.2% had an underlying heart disease. Of the patients who consulted because of a murmur, 91% did not suffer from heart disease. The group of patients with congenital heart disease who had not undergone surgery (group A) was the youngest (median 1.9 years) and simple non-cyanotic heart disease with pulmonary hyperflow accounted for 66.66% of heart diseases. The implementation of the portable echocardiography in the guidance clinic confirmed the diagnosis and defined the management in 29.4% of patients during the first consultation (AU)

El adolescente con cardiopatía congénita: transición y transferencia / The adolescent with congenital heart defects: transition and transfer

Introducción: Los grandes avances en el diagnóstico y tratamiento de los pacientes con cardiopatías congénitas en las últimas décadas han permitido que mas del 90% lleguen a la adolescencia y edad adulta. Sin embargo, muchos de ellos requirieran seguimiento e intervenciones de por vida, por lo que necesitaran ser transferidos desde el hospital pediátrico al de adultos. Material y Métodos: Se incluyeron los pacientes mayores de 15 años que consultaron en el área ambulatoria del servicio de cardiología del Hospital Garrahan durante el periodo agosto 2017 - agosto 2018. Las variables analizadas fueron: procedencia, cardiopatía de base y variedad pronostica ,procedimientos intervencionistas factores asociados como síndrome genéticos y otras comorbilidades, cobertura social, nivel educativo, terapéutica medicamentosa, clase funcional, embarazos, prevalencia de cardiopatías en la descendencia y transición-transferencia al hospital de adultos. Resultados: Registramos 704 consultas de 309 pacientes con una edad media de 19,17 años (DS +- 4,62; (rango 15- 49,4 años). Fueron 112 mujeres y 197 varones. El 51,1 % provenían de Buenos Aires,40 % de las provincias del interior y 8,1% CABA. El 92% de los pacientes tenía cardiopatías de moderada y severa complejidad, y el 93,5% eran operadas. El 13,2 % eran síndromes genéticos. El 48.5% tenían comorbilidades, siendo los trastornos electrofisiológicos los más frecuentes en el 72,66% de los casos. El 63% tenía cobertura social pero solo el 2,6% prepagos con cobertura en centros alta complejidad. El 23.6% recibía terapia combinada con 2 o más drogas. El 48,78% ya presentaban antecedente de algún tipo de reintervención, 98,5% de estas se vincularon a las cardiopatías moderadas a complejas. Registramos 15 embarazos con 14 recién nacidos vivos, 1 con cardiopatía congénita. El proceso de transición ­ transferencia en el 55% (170 p) se había iniciado, siendo efectiva (8p), frustra (9p), compartida (49 p), y en proceso (103 p). Hubo un solo fallecimiento durante el periodo de estudio, vinculado a cardiopatía compleja, múltiples reintervenciones y endocarditis. Conclusiones: El 92% de los pacientes en nuestro estudio, tienen cardiopatías operadas de moderada y severa complejidad. Los trastornos electrofisiológicos y la necesidad de reintervenciones durante el seguimiento alejado han sido las complicaciones más frecuentes de esta población. El proceso de transición y transferencia desde el hospital pediátrico al de adultos es deficitario, principalmente por falta de cobertura y experiencia sobre todo para la atención continua de las cardiopatías moderadas y complejas (AU)

Introduction: In recent decades, important advances in the diagnosis and treatment of patients with congenital heart defects have allowed more than 90% of them to reach adolescence and adulthood. However, many patients required lifelong follow-up and interventions, and therefore the need to be transitioned from pediatric to adult care. Material and Methods: Patients older than 15 years who consulted at the outpatient clinic of the department of cardiology at Garrahan Hospital from August 2017 to August 2018 were included. The variables analyzed were place of origin, underlying heart disease, and diagnosis, interventions, associated factors, such as genetic syndromes and other comorbidities, insurance coverage, educational level, pharmacological treatment, functional class, pregnancies, prevalence of heart disease in offspring, and transition-transfer to adult hospital. Results: We recorded 704 consultations from 309 patients with an average age of 19.17 years (SD +- 4.62; range 15-49.4 years); 112 patients were female and 197 male. Overall, 51.1% came from the province of Buenos Aires, 40% from the other provinces, and 8.1% from the city of Buenos Aires. Of the patients, 92% had moderate and severe heart disease, and 93.5% had undergone surgery. Genetic syndromes were identified in 13.2%. Overall, 48.5% had comorbidities, of which electrophysiological disorders were the most common in 72.66% of cases. 63% had social insurance coverage but only 2.6% had a prepaid insurance with coverage in tertiary-level centers. Overall, 23.6% received combination therapy with 2 or more drugs. 48.78% had undergone some type of previous reintervention, 98.5% of whom had moderate-to-severe heart disease. We recorded 15 pregnancies with 14 live neonates, one of whom had congenital heart defects. The transition - transfer had been initiated in 55% (170 p) and was effective (8p), frustrated (9p), shared (49 p), or in progress (103 p). There was only one death during the study period, related to severe heart disease, multiple reinterventions, and endocarditis. Conclusions: 92% of patients in our study have moderate or severe operated heart disease. Electrophysiological disorders and the need for reintervention during the long-term follow-up were the most common complications of this population. The process of transition and transfer from pediatric to adult care is deficient, mainly due to lack of insurance coverage and experience especially for the ongoing care of moderate-to-severe heart disease

Consultorio de condiciones crónicas complejas, seguimiento de cardiopatías congénitas críticas / Complex chronic conditions clinic, follow-up of critical congenital heart defects

Introducción: Las cardiopatías congénitas representan la segunda causa de mortalidad infantil en menores de un año. Objetivo: Describir la epidemiología, resultados y seguimiento de cardiopatías congénitas críticas (pacientes que requirieron intervención quirúrgica o cateterismo intervencionista antes del año de vida). Resultados: Se incluyeron 316 pacientes operados o intervenidos por cateterismo antes del año de vida con diagnóstico de cardiopatías congénitas, de los cuales el 30,7% eran sindromáticos y solo el 7,7% tuvieron diagnóstico prenatal. Se logró cirugía reparadora en un 86,7% de los casos siendo la CIV la cardiopatía congénita operada más frecuente en menores de un año. Los pacientes quedaron con defectos residuales significativos en un 23,73%, con una tasa de complicaciones de 39,64%. En el seguimiento post-operatorio un 19,62% requirieron reoperación, y un 5,06% cateterismo. Un 15,19% de los pacientes fallecieron. Conclusiones: El Hospital Juan P. Garrahan tiene una población numerosa y heterogénea de pacientes con cardiopatías críticas. Dado que solo un pequeño porcentaje de nuestros pacientes tuvieron diagnóstico prenatal, se debe mejo- rar en este aspecto, ya que el diagnóstico de cardiopatía en el período fetal mejora las posibilidades de sobrevida en el neona- to. Es importante el seguimiento multidisciplinario, coordinado y continuo de estos pacientes, dado que muchos niños operados tienen comorbilidades y lesiones residuales (AU)

Introduction: Congenital heart defects are the second leading cause of infant mortality in children under one year of age. Objective: To describe the epidemiology, results, and follow-up of patients with critical congenital heart defects (patients who required surgical intervention or interventional catheterization before the year of life). Results: 316 patients with congenital heart defects who underwent surgery or catheterization before the life year, of whom 30.7% were syndromic and only 7.7% were diagnosed prenatally. Repair surgery could be performed in 86.7% of cases with SVD being the most commonly operated congenital heart defect in children under one year of age. Of the patients, 23.73% had a significant residual defect with a complication rate of 39.64%. In the post-operative follow-up, 19.62% required reoperation and 5.06% catheterization. Overall, 15.19% of the patients died. Conclusions: The Juan P. Garrahan Hospital has a large and heterogeneous population of patients with critical heart disease. Since only a small percentage of our patients had a prenatal diagnosis, this aspect should be enhanced, as the diagnosis of heart disease in uterus improves the chances of survival of the neonate. Multidisciplinary, coordinated and continuous follow-up of these patients is important, as many children who undergo surgery have comorbidities and residual lesions (AU)

Pseudomonas protegens CS1 from the lemon phyllosphere as a candidate for citrus canker biocontrol agent.

Citrus canker is a worldwide-distributed disease caused by Xanthomonas citri subsp. citri. One of the most used strategies to control the disease is centred on copper-based compounds that cause environmental problems. Therefore, it is of interest to develop new strategies to manage the disease. Previously, we reported the ability of the siderophore pyochelin, produced by the opportunistic human pathogen Pseudomonas aeruginosa, to inhibit in vitro several bacterial species, including X. citri subsp. citri. The action mechanism, addressed with the model bacterium Escherichia coli, was connected to the generation of reactive oxygen species (ROS). This work aimed to find a non-pathogenic strain from the lemon phyllosphere that would produce pyochelin and therefore serve in canker biocontrol. An isolate that retained its capacity to colonise the lemon phyllosphere and inhibit X. citri subsp. citri was selected and characterised as Pseudomonas protegens CS1. From a liquid culture of this strain, the active compound was purified and identified as the pyochelin enantiomer, enantio-pyochelin. Using the producing strain and the pure compound, both in vitro and in vivo, we determined that the action mechanism of X. citri subsp. citri inhibition also involved the generation of ROS. Finally, the potential application of P. protegens CS1 was evaluated by spraying the bacterium in a model that mimics the natural X. citri subsp. citri infection. The ability of P. protegens CS1 to reduce canker formation makes this strain an interesting candidate as a biocontrol agent.

Embryos with morphokinetic abnormalities may develop into euploid blastocysts.

Irregular cleavage divisions are expected to produce chromosomally deviant embryos. We investigated whether embryos from irregular cleavages could develop into euploid blastocysts, and, if so, whether any evidence existed of a self-correction mechanism of the embryo. We also investigated the role of different dynamic aspects of morula compaction in this process. A total of 791 embryos from 141 patients undergoing pre-implantation genetic screening were retrospectively analysed using a time-lapse imaging system, and multiple cell divisions were evaluated. A total of 276 embryos developed into blastocysts suitable for biopsy and chromosome screening through array-comparative genomic hybridization. As well as testing trophectoderm biopsy specimens for aneuploidy, excluded cells of 18 blastocysts, which developed from partially compacted morulas, were also analysed. Unique data on the developmental fate of embryos with cleavage abnormalities are presented, and a potential mechanism of 'aneuploidy rescue' is postulated through which mosaic embryos may form partially compacted morulas to exclude aneuploid cells. In addition, this process seems to be less efficient in older women. The data obtained also provide further evidence that excluded cells should not be used to infer the cytogenetic status of the embryo.

Evaluation of a clip-reinforced wrapping technique with collagen-impregnated Dacron for intracranial aneurysms inaccessible to other treatment.

OBJECTIVE: To describe our clip-reinforced wrapping technique (CRW) with collagen-impregnated Dacron and report our experience in intracranial aneurysms (IA) untreatable by coil embolization (CE) or surgical clipping (SC). METHODS: Between July 2003 and November 2010, CRW was performed on 20 IAs in 18 patients using a collagen-impregnated Dacron fabric (Hemashield(®), USA) fixed with a clip around the parent vessel and the IA. RESULTS: Two patients (11%) died of complications from their subarachnoid hemorrhage and preexisting conditions. In the remaining 16 patients, after an average follow-up of 45 months (min: 27, max: 77), 16 (89%) out of the 18 treated aneurysms were no longer visible and two were reduced and stable. CONCLUSION: Our preliminary results suggest that CRW with Hemashield could be an accurate and safe alternative technique for some IA without any other treatment option. IMPLICATIONS: Description of an alternative surgical technique for otherwise untreatable intracranial aneurysms.

A Japanese plum α-l-arabinofuranosidase/ß-D-xylosidase gene is developmentally regulated by alternative splicing.

A full-length cDNA clone named PsARF/XYL was obtained from Prunus salicina Lindl., and determined to encode a putative α-l-arabinofuranosidase/ß-d-xylosidase belonging to glycoside hydrolase (GH, EC 3.2.1.-) family 3. Two related PsARF/XYL cDNAs were amplified, one from a fully-spliced transcript (PsARF/XYLa) and another one from an intron-retained transcript (PsARF/XYLb). The protein deduced from PsARF/XYLb is a truncated peptide at C-terminus that conserves the active-site amino acid sequence. High levels of PsARF/XYLa and PsARF/XYLb transcripts are detectable in several plant tissues. PsARF/XYLb transcripts accumulate progressively during the phase of exponential fruit growth but they become barely noticeable during on-tree ripening, or after a 6-h exposure of preclimacteric full-size plums to ethylene. In contrast, PsARF/XYLa is expressed throughout fruit development, and transcript accumulation parallels the climacteric rise in ethylene production during ripening. PsARF/XYLa expression is strongly induced in preclimacteric full-size plums after a 6-h treatment with physiologically active concentrations of ethylene. These findings suggest that PsARF/XYL gene is post-transcriptionally regulated by alternative splicing during development and that ethylene may be involved in this regulation. The isolation of a partial cDNA clone, PsARF1, is also reported. It encodes a putative cell-wall α-l-arabinofuranosidase, and its transcription is rapidly inhibited by ethylene in mature green plums.

Uso del taping en el manejo clínico de sujetos con pinzamiento sub-acromial: revisión sistemática / Using the taping in the clinical management of patients with sub-acromial impingement: a systematic review

El pinzamiento sub-acromial (SPS) es el trastorno músculo- esquelético más frecuente de las patologías de hombro. El taping se ha transformado en una indicación relativamente nueva y bastante común en los últimos años. Se realizará una síntesis de la evidencia a través de una revisión sistemática (RS) de ensayos clínicos aleatorizados que hayan comparado el uso del taping para el manejo de estos pacientes. El objetivo de esta revisión es determinar el efecto del taping sobre el dolor en sujetos con pellizcamiento sub-acromial. La estrategia de búsqueda incluyó ensayos clínicos aleatorizados (ECA); las bases de datos usadas fueron: Medline, Cinahl, PEDro, Central, LILACS. Además, se realizó una búsqueda en revistas electrónicas, actas de congresos, Registro de Estudios Clínicos Aleatorizados y se contactó con expertos en el tema. Los resultados obtenidos fueron de dos artículos que cumplían con nuestros criterios de elegibilidad. Se concluye que existe evidencia de baja calidad que sugiere que la aplicación de taping sería efectiva para mejorar la funcionalidad, reducir el dolor en reposo y durante los movimientos hasta en una semana posterior a su aplicación, en sujetos que presentan pellizcamiento sub-acromial en comparación a un programa de terapia física (AU)

The sub-acromial impingement (SAI) is the most common musculoskeletal disorder of shoulder pathologies. In recent times, the taping has become a relatively new and quite common indication in recent years. There will be a synthesis of evidence through a systematic review (SR) of randomized trials that had compared the use of taping for the management of these patients. The aim of this review is to determine the effect of taping on pain in subjects with sub-acromial pinch. The research strategy included randomized clinical trials (RCTs) databases were used: Medline, Cinahl, PEDro, Central, LILACS. In addition, we searched electronic journals, conference proceedings, randomized trials registry and contacted experts in the field. The results were two articles that met our eligibility criteria. We conclude that there is low quality evidence suggesting that the application of taping, would be effective to improve functionality and reduce pain at rest and during movements up to 1 week after application, in subjects with sub-acromial pinch compared to physical therapy program (AU)

Hidatidosis cardíaca con embolismo aórtico / Cardiac echinococcosis with aortic embolism

La hidatidosis cardíaca con embolismo aórtico es una entidad extremadamente infrecuente. Se produce por una rotura o un desprendimiento total o parcial de un quiste hidatídico cardíaco. Presentamos el caso de un niño de 11 años de edad con manifestaciones de isquemia de miembros inferiores de inicio súbito y antecedentes de disnea y dolor torácico. Se describe la utilidad de la angiotomografía computada multidetector en la evaluación de este tipo de afecciones.

Cardiac echinococcosis with aortic embolism is an extremely rare entity. It is caused by the rupture or detachment of fragments of the cardiac cyst. We report the case of an 11 year-old boy with sudden onset of lower limb ischemia and a previous history of dyspnea and chest pain. We describe the usefulness of multislice computed tomography in the evaluation of this disease.

Hidatidosis cardíaca con embolismo aórtico / Cardiac echinococcosis with aortic embolism

La hidatidosis cardíaca con embolismo aórtico es una entidad extremadamente infrecuente. Se produce por una rotura o un desprendimiento total o parcial de un quiste hidatídico cardíaco. Presentamos el caso de un niño de 11 años de edad con manifestaciones de isquemia de miembros inferiores de inicio súbito y antecedentes de disnea y dolor torácico. Se describe la utilidad de la angiotomografía computada multidetector en la evaluación de este tipo de afecciones.(AU)

Cardiac echinococcosis with aortic embolism is an extremely rare entity. It is caused by the rupture or detachment of fragments of the cardiac cyst. We report the case of an 11 year-old boy with sudden onset of lower limb ischemia and a previous history of dyspnea and chest pain. We describe the usefulness of multislice computed tomography in the evaluation of this disease.(AU)

Aspectos epidemiológicos, clínicos, factores pronósticos y evolución en pacientes con miocardiopatía dilatada como potenciales candidatos para trasplante cardíacos / Epidemiological and clinical aspects and pronognostic factors and evolution in patients

El objetivo de este estudio fue analizar aspectos epidemiológicos, clínicos y evolución de pacientes con miocardiopatía dilatada. Material y Método: Fueron evaluados en forma consecutiva 90 pacientes menores de 16 años que consultaron en este hospital entre Marzo 1993 y Diciembre 2007 con diagnóstico de miocardiopatía dilatada (MD). Resultados: fueron analizados 90 pacientes con diagnóstico de MD, con edad entre 1 y 166 meses, mediana= 1 5 meses. Los menores de 1 año constituyeron el 46,8 por ciento. La distribución del sexo fue: 61 por ciento mujeres y 39 por ciento varones. Miocarditis se presumió en el 43,3 por ciento de los pacientes. Al momento del ingreso se encontraban en clase funcional I -II 74 ,5 por ciento en III-IV 25,5 por ciento; estos últimos requirieron internación en Unidad de Cuidados Intensivos (UCI). En un tiempo de seguimiento con mediana de 24m presentaron mala evolución (muerte o transplante el 31,1 por ciento. Permanecieron estables en clase funcional II el 41,1 por ciento. Recuperaron función ventricular 28,8 por ciento, la mayoría con sospecha de miocarditis. La sobrevida actuarial libre de eventos fue de 77 por ciento al año y 61 por ciento a los 5 años. En el análisis multivariado la edad y el sexo no fueron factores de riesgo para muerte o trasplante El diagnóstico disminuyó el riesgo de muerte o trasplante. conclusiones: A mediano plazo la MD puede evolucionar con recuperación de la función ventricular especialmente cuando está asociada a sospecha de miocarditis. la edad y el sexo no fueron factores de riesgo. El ingreso a UCI mejoró la sobrevida de los pacientes.(AU)