Early assessment of cardiomyopathy in Duchenne patients by means of longitudinal strain echocardiography

Introduction: The diagnosis of Duchenne-linked cardiomyopathy may be challenging. Conventional echocardiographic measurements typically show deterioration beyond the second decade. Global longitudinal strain has been proposed as an earlier marker than left ventricular ejection fraction. Material and methods: A prospective, observational, cross-sectional, case-control study was carried out. Both Duchenne patients and control subjects underwent transthoracic echocardiogram in order to assess left ventricle function. Bayesian linear regression was the main tool for inference. Age effects were parameterised by means of a spline function because of its simplicity to characterise continuous variables and smooth contributions. The posterior distribution of the marginal age effects was used to assess the earliest age of deterioration of each marker. Results: Sixteen Duchenne patients and twenty-two healthy male subjects were enrolled. On overage, cardiac function measures were found for ejection fraction and longitudinal strain among different groups. Age effects on global longitudinal strain are more reliably found at ages of 6 and above, while ejection fraction starts to deteriorate at an older age. Progressive left ventricular dysfunction in Duchenne patients is one of the key issues and starts at an early age with subtle symptoms. Conclusion: This cross-sectional study provides supporting evidence that global longitudinal strain is an earlier marker of disease progression than ejection fraction in Duchenne patients

Propéptido natriurético cerebral como marcador de evolución digestiva en el recién nacido prematuro

Introducción: Antecedentes y objetivo: el ductus arterioso persistente hemodinámicamente significativo (DAP-HS) se asocia a mayor riesgo de enterocolitis necrotizante (ECN) y peor tolerancia enteral en los recién nacidos prematuros (RNPT). Se ha demostrado asociación entre el propéptido natriurético cerebral (proBNP) y el DAP-HS. Nuestro objetivo fue analizar la relación entre los niveles de proBNP y la tolerancia enteral, el riesgo de ECN y la ganancia ponderal en el RNPT. Material y métodos: estudio retrospectivo observacional, que incluyó a RNPT menores de 32 semanas de gestación y/o 1.500 g, con estudio ecocardiográfico y determinación de niveles de proBNP a las 48-72 horas de vida. Resultados: de 117 pacientes incluidos, el 65, 8% tuvo un DAPHS y el 9, 4% presentó ECN confirmada. El DAP-HS se asoció a mayor duración de la nutrición parenteral (p < 0, 001), a ECN confirmada (p = 0, 006) y a peor ganancia ponderal durante el ingreso (p < 0, 001). Los valores de proBNP se relacionaron con la ECN (no ECN 12.189, 5 pg/ml, rango 654-247.986; ECN 41.445 pg/ml, rango 15.275-166.172; p < 0, 001), sin encontrar asociación con el resto de variables de evolución digestiva. En el análisis multivariante de regresión logística, las variables relacionadas de forma independiente con el desarrollo de ECN fueron la edad gestacional y el proBNP superior a 22.400 pg/ml (OR 13, 386; IC 95% 1, 541-116, 262; p = 0, 019). Conclusiones: el proBNP podría ser un marcador precoz de patología digestiva grave en el RNPT. Los niveles elevados podrían relacionarse con mayor riesgo de ECN en los neonatos más inmaduros. Introduction: Background and objective: hemodynamically significant patent ductus arteriosus (HS-PDA) is associated with an increased risk of necrotizing enterocolitis (NEC) and worse enteral tolerance in preterm newborns (PN). An association has been demonstrated between brain natriuretic propeptide (proBNP) and HS-PDA. Our objective was to analyze the relationship between proBNP levels and enteral tolerance, NEC risk and weight gain in PN. Material and methods: a retrospective study was performed in neonates born before 32 weeks' gestation or with birth weight below 1500 grams, in whom proBNP determination and echocardiography were performed at 48 to 72 h of life. Results: 117 patients were included. 65.8% had a HS-PDA and 9.4% had an outcome of NEC. HS-PDA was associated with longer duration of parenteral nutrition (p < 0.001), a confirmed NEC (p = 0.006) and worse weight gain during admission (p < 0.001). ProBNP levels were associated to NEC (no NEC 12189.5 pg / mL, range 654-247986; NEC 41445 pg/mL, range 15275-166172, p < 0.001). No association was found with the rest of gastrointestinal outcomes. Multivariate logistic regression analysis showed a significant association of NEC with gestational age and proBNP above 22, 400 pg/mL (OR 13, 386, 95% CI 1, 541-116, 262, p = 0.019). Conclusions: proBNP could be an early marker of severe digestive pathology in PN. Increased proBNP levels could be associated with a significant increased risk of NEC in very immature newborns

The CARMENES search for exoplanets around M dwarfs. Two temperate Earth-mass planet candidates around Teegarden’s Star

Context.Teegarden’s Star is the brightest and one of the nearest ultra-cool dwarfs in the solar neighbourhood. For its late spectral type (M7.0 V),the star shows relatively little activity and is a prime target for near-infrared radial velocity surveys such as CARMENES.Aims.As part of the CARMENES search for exoplanets around M dwarfs, we obtained more than 200 radial-velocity measurements of Teegarden’sStar and analysed them for planetary signals.Methods.We find periodic variability in the radial velocities of Teegarden’s Star. We also studied photometric measurements to rule out stellarbrightness variations mimicking planetary signals.Results.We find evidence for two planet candidates, each with 1.1M⊕minimum mass, orbiting at periods of 4.91 and 11.4 d, respectively. Noevidence for planetary transits could be found in archival and follow-up photometry. Small photometric variability is suggestive of slow rotationand old age.Conclusions.The two planets are among the lowest-mass planets discovered so far, and they are the first Earth-mass planets around an ultra-cooldwarf for which the masses have been determined using radial velocities.We thank the referee Rodrigo Díaz for a careful review andhelpful comments. M.Z. acknowledges support from the Deutsche Forschungs-gemeinschaft under DFG RE 1664/12-1 and Research Unit FOR2544 “BluePlanets around Red Stars”, project no. RE 1664/14-1. CARMENES isan instrument for the Centro Astronómico Hispano-Alemán de Calar Alto(CAHA, Almería, Spain). CARMENES is funded by the German Max-Planck-Gesellschaft (MPG), the Spanish Consejo Superior de InvestigacionesCientíficas (CSIC), the European Union through FEDER/ERF FICTS-2011-02 funds, and the members of the CARMENES Consortium (Max-Planck-Institut für Astronomie, Instituto de Astrofísica de Andalucía, LandessternwarteKönigstuhl, Institut de Ciències de l’Espai, Institut für Astrophysik Göttingen,Universidad Complutense de Madrid, Thüringer Landessternwarte Tautenburg,Instituto de Astrofísica de Canarias, Hamburger Sternwarte, Centro de Astro-biología and Centro Astronómico Hispano-Alemán), with additional contribu-tions by the Spanish Ministry of Economy, the German Science Foundationthrough the Major Research Instrumentation Programme and DFG ResearchUnit FOR2544 “Blue Planets around Red Stars”, the Klaus Tschira Stiftung, thestates of Baden-Württemberg and Niedersachsen, and by the Junta de Andalucía.Based on data from the CARMENES data archive at CAB (INTA-CSIC). Thisarticle is based on observations made with the MuSCAT2 instrument, devel-oped by ABC, at Telescopio Carlos Sánchez operated on the island of Tener-ife by the IAC in the Spanish Observatorio del Teide. Data were partly col-lected with the 150-cm and 90-cm telescopes at the Sierra Nevada Observa-tory (SNO) operated by the Instituto de Astrofísica de Andalucía (IAA-CSIC).Data were partly obtained with the MONET/South telescope of the MOnitoringNEtwork of Telescopes, funded by the Alfried Krupp von Bohlen und HalbachFoundation, Essen, and operated by the Georg-August-Universität Göttingen,the McDonald Observatory of the University of Texas at Austin, and the SouthAfrican Astronomical Observatory. We acknowledge financial support from theSpanish Agencia Estatal de Investigación of the Ministerio de Ciencia, Inno-vación y Universidades and the European FEDER/ERF funds through projectsAYA2015-69350-C3-2-P, AYA2016-79425-C3-1/2/3-P, AYA2018-84089, BES-2017-080769, BES-2017-082610, ESP2015-65712-C5-5-R, ESP2016-80435-C2-1/2-R, ESP2017-87143-R, ESP2017-87676-2-2, ESP2017-87676-C5-1/2/5-R, FPU15/01476, RYC-2012-09913, the Centre of Excellence ”Severo Ochoa”and ”María de Maeztu” awards to the Instituto de Astrofísica de Canarias (SEV-2015-0548), Instituto de Astrofísica de Andalucía (SEV-2017-0709), and Cen-tro de Astrobiología (MDM-2017-0737), the Generalitat de Catalunya throughCERCA programme”, the Deutsches Zentrum für Luft- und Raumfahrt throughgrants 50OW0204 and 50OO1501, the European Research Council through grant694513, the Italian Ministero dell’instruzione, dell’università de della ricerca andUniversità degli Studi di Roma Tor Vergata through FFABR 2017 and “Mis-sion: Sustainability 2016”, the UK Science and Technology Facilities Council through grant ST/P000592/1, the Israel Science Foundation through grant848/16, the Chilean CONICYT-FONDECYT through grant 3180405, the Mexi-can CONACYT through grant CVU 448248, the JSPS KAKENHI through grantsJP18H01265 and 18H05439, and the JST PRESTO through grant JPMJPR1775

Síndrome venolobar congénito pulmonar o síndrome de la cimitarra: descripción de 2 casos

Congenital pulmonary venolobar syndrome (CPVS) or scimitar syndrome is a rare anomaly which associates partial anomalous pulmonary venous drainage of the right pulmonary veins to the inferior vena cava or the right atrium, and right pulmonary hypoplasia. Patients are usually asymptomatic and is only when the left to right shunt becomes important, when overload of right heart can produce ventricular hypertrophy with pulmonary hypertension. Diagnosis is confirmed by echocardiography or computed tomography or magnetic resonance with contrast. Treatment will depend on the appearance of symptoms. We report 2 clinical cases of CPVS or scimitar syndrome in which diagnosis was reached by chance, following a chest radiography or echocardiography.El síndrome venolobar congénito pulmonar (SVLCP) o síndrome de la cimitarra es una rara anomalía que principalmente asocia drenaje venoso pulmonar anómalo parcial de las venas pulmonares derechas a la vena cava inferior o a la aurícula derecha e hipoplasia pulmonar derecha. Habitualmente los pacientes están asintomáticos y solo cuando el mínimo shunt izquierda a derecha se hace más importante, se puede sobrecargar el corazón derecho produciendo hipertrofia ventricular con hipertensión pulmonar. El diagnóstico se confirmará con ecocardiografía, tomografía computerizada con contraste (angio-TC) o resonancia magnética con contraste (angio-RM) y el tratamiento dependerá de la aparición de sintomatología. En este artículo se presentan 2 casos clínicos de SVLCP o síndrome de la cimitarra en los que se llegó al diagnóstico de forma casual, a raíz de una radiografía (RX) de tórax y/o ecocardiografía

Inmunizaciones en el niño cardiópata

Infants with severe congenital heart disease are considered a population at risk of potential complications in concomitant medical pathology. These patients have special needs in terms of immunization. This document defines which heart diseases need additional measures and how to manage vaccination in this high-risk population.Los niños con cardiopatías significativas se consideran un grupo de riesgo por las complicaciones que pueden presentar ante ciertas enfermedades. Es por ello, que estos pacientes tienen necesidades especiales en cuanto a la inmunización. En este documento se definen cuáles son las cardiopatías que se consideran de riesgo y qué medidas especiales hay que tener en cuenta respecto a la vacunación de estos pacientes

Hiperplasia tímica que simula masa pericárdica

Background: Mediastinal widening due to cardiomegaly requires complementary studies to guide the etiology and distinguish a benign condition from other malignant diseases. Clinical-case report: A 20 month-old infant presented cardiomegaly on casual chest X-ray examination. Echocardiography showed an extracardiac mass (pericardial mass) as the cause of the radiological cardiomegaly. This mediastinal mass agreed with an homogeneous enlargement of the thymus with a large craniocaudal extension, corresponding with a benign hyperplastic thymus in magnetic resonance imaging. Conclusions: Despite the thymus is seen as a triangular sail (thymic sail sign) in X-ray exams, its enlargement could simulate a mediastinal mass or a cardiomegaly, even a tumor or pulmonary disorders (pneumonia). Therefore, additional imaging studies should be performed.Antecedentes: El hallazgo de un ensanchamiento mediastínico secundario a cardiomegalia precisa estudios complementarios para establecer la etiología y descartar una patología potencialmente importante. Descripción del caso clínico: Se presenta el caso de una lactante de 20 meses con cardiomegalia asintomática hallada en un estudio radiológico casual. La ecocardiografía mostró la presencia de una masa extracardíaca (pericárdica) como la causante de la cardiomegalia radiológica. Dicha masa mediastínica correspondió a un timo prominente homogéneo de gran extensión cráneo-caudal, sugestivo de hiperplasia tímica en el estudio por resonancia magnética. Conclusiones: A pesar de que el timo suele presentarse en radiografía con unos hallazgos típicos («forma de vela») que permiten diferenciarlo de otras entidades, su engrosamiento puede simular una cardiomegalia o una masa mediastínica, incluso un tumor o una neumonía. Por ello, es preciso valorar si se amplía el estudio mediante el empleo de otras técnicas de imagen