Miocardiopatía hipertrófica en la edad pediátrica. Revisión a propósito de una serie de casos

Introducción: La miocardiopatía hipertrófica (MCH) constituye una enfermedad compleja y variable en cuanto a morfología, fisiopatología, pronóstico y sobrevida. Se caracteriza por una hipertrofia del ventrículo izquierdo, aunque en ocasiones puede ser biventricular o ventricular derecha aislada. En la edad pediátrica predominan las causas no sarcoméricas. La ecocardiografía es el método diagnóstico más utilizado para caracterizar la morfología y repercusión hemodinámica, sin embargo, la resonancia magnética cardíaca (RMC) es de elección por aportar mayores ventajas morfológicas y funcionales. Las pruebas genéticas son de gran importancia en la determinación del diagnóstico etiológico, manejo terapéutico y pronóstico. El tratamiento tiene como objetivo principal mejorar la capacidad funcional y aliviar los síntomas. Los betabloqueantes no vasodilatadores son considerados de primera líneay en algunos casos se puede recurrir a terapias avanzadas como disopiramida o terapia de reducción septal (TRS), uso del cardiodesfibrilador implantable (CDI), y en ocasiones se debe considerar trasplante cardiaco en pacientes con síntomas refractarios al tratamiento médico y en MCH del tipo obstructivo. Materiales y métodos: Estudio observacional, descriptivo, corte transversal. Resultados: Se estudiaron 12 pacientes, desde enero de 2020 a diciembre de 2022, con edades comprendidas desde 5 meses hasta 16 años, diagnosticados con MCH de presentación clínica variable y de distintas etiologías. Conclusión: La MCH de causas no sarcoméricas como los síndromes malformativos y errores innatos del metabolismo son las que tienen peor pronóstico y sobrevida. La finalidad de detectar precozmente la MCH es el inicio temprano de la terapia específica, para así retrasar el compromiso cardiovascular y mejorar la sobrevida.

Introduction: Hypertrophic cardiomyopathy (HCM) is a complex and variable disease in terms of morphology, pathophysiology, prognosis and survival. It is characterized by left ventricular hypertrophy, although it can sometimes be biventricular or isolated right ventricular. In the pediatric population, non-sarcomeric causes predominate. Echocardiography is the most widely used diagnostic method to characterize morphology and hemodynamic repercussions; however, cardiac magnetic resonance imaging (CMR) is preferred because it provides greater morphological and functional advantages. Genetic tests are of great importance in determining the etiological diagnosis, therapeutic management and prognosis. The main objective of the treatment is to improve functional capacity and alleviate symptoms. Non-vasodilator beta-blockers are considered first-line treatment and in some cases advanced therapies such as disopyramide or septal reduction therapy (SRT), use of implantable cardioverter-defibrillator (ICD) can be used; heart transplantation should be considered in patients with symptoms refractory to medical treatment and in HCM of the obstructive type. Materials and methods: This was an observational, descriptive, cross-sectional study. Results: 12 patients were studied, from January 2020 to December 2022, with ages ranging from 5 months to 16 years, diagnosed with HCM of variable clinical presentation and of different etiologies. Conclusion: HCM from non-sarcomeric causes such as malformation syndromes and inborn errors of metabolism have the worst prognosis and survival. The purpose of early detection of HCM is the early initiation of specific therapy, in order to delay cardiovascular compromise and improve survival.

Bone Health in Metastatic Cancer.

OBJECTIVE: This article reviews the epidemiology and treatment of bone metastases. Also, it revisits the mechanism of action, indications, benefits, and complications of antiresorptive agents; discusses the adverse event profile of these agents; and addresses the prevention and management of these side effects. DATA SOURCES: For this review, peer-reviewed articles, research publications, and relevant clinical guidelines identified from PubMed were used. CONCLUSION: Bone metastases are common in advanced cancer and much more frequently than in primary bone cancer, especially in adults. Bisphosphonates and Denosumab are potent antiresorptives and significantly reduce the risk of skeletal-related events. These complications are related to poor quality of life, bone pain, functional disability, and decreased survival. Prolonged therapy with these antiresorptive agents in patients with advanced malignancy is generally well tolerated, but some side effects are potentially serious and require periodic monitoring. Furthermore, some of them can be avoided. IMPLICATIONS FOR NURSING PRACTICE: The role of health care providers and specially nurses on identifying patients at risk of complications from these antiresorptive agents, and providing information on how to prevent them, is essential for health and quality of life maintenance in these patients.

Impacts of the traditional baited basket fishing trap "gargoor" on green sea turtles Chelonia mydas (Testudines: Cheloniidae) Linnaeus, 1758 from two case reports in the United Arab Emirates.

The United Arab Emirates fisheries consist of highly diverse fish communities, and the most popular equipment used by fishermen to harvest them is a traditional baited basket fishing trap known locally as "gargoor". Gargoors are dome-shaped traps made from galvanized steel; they have a circular supporting base and a funnel-like entrance. Unintended impacts of gargoors on marine fauna include bycatch of non-target species and, when lost, ghost fishing. However, there is very little information on sea turtle interaction with gargoors. In this paper we present two case reports from the eastern coast of the UAE of green sea turtle strandings associated with gargoor interactions. The first case report describes a turtle that was discovered trapped inside a lost or abandoned gargoor. The second case report describes another turtle that suffered from extensive perforation of the gastrointestinal tract resulting from the ingestion of 32 pieces of rusty gargoor fragments.

Marine debris ingestion of green sea turtles, Chelonia mydas, (Linnaeus, 1758) from the eastern coast of the United Arab Emirates.

The deleterious effects of marine debris ingestion on marine turtles are well documented in literature globally. In this study, the qualitative and quantitative aspects of marine debris ingested by 14 stranded green sea turtles Chelonia mydas, (Linnaeus, 1758) along the eastern coast of the United Arab Emirates were investigated. The numeric and gravimetric proportions of debris in the esophagus, stomach and intestines were documented following classification of color, presumed sources and Marine Strategy Framework Directive categories and sub-categories. The results show that 85.7% of the specimens examined consumed marine debris. On average, specimens consumed 61.9 ±â€¯17.2 items of 1.0 ±â€¯0.3 g mass. Plastics, particularly white, and transparent thread-like and sheet-like plastics, were the predominant debris ingested. The results reflect a potentially high level of interaction between green sea turtles and anthropogenic marine debris along the Gulf of Oman coast of the UAE.

Significado que otorgan las personas trans a sus experiencias en la atención en el sistema de salud chileno / The meaning That transexual individuals assign to their experiences in the Chilean health care system

En Chile, la atención de salud para usuarios/as transexuales se desarrolla en un contexto marcado por la desigualdad territorial en cuanto al acceso a servicios trans específicos del proceso de adecuación corporal. Por otro lado, las circulares sanitarias que regulan su trato en relación a la identidad de género no se cumplen a cabalidad, lo que favorece la discriminación y la transfobia. Las experiencias de salud de estos/as usuarios/as aún permanecen poco abordadas, por lo tanto también los impactos que esta tiene en su calidad de vida y bienestar. Objetivo: Conocer las experiencias de vida y el significado que un grupo de usuarios/as transexuales le otorga a sus experiencias derivadas del proceso de atención en el sistema de salud chileno, en relación al respeto que reciben por su identidad de genero. Material y método: Estudio cualitativo interpretativista de tipo exploratorio con enfoque fenomenológico. Entrevistas semi-estructuradas fueron llevadas a cabo con 8 personas transexuales, una muestra determinada por la saturación del discurso. El análisis fue hermenéutico del discurso. Resultados: Las personas transexuales en Chile suelen recibir una atención en el sistema de salud poco respetuosa de su identidad de género; sin embargo, participantes reportan que los cuidados que reciben en el Hospital Las Higueras de Talcahuano mejoran su calidad de vida. Conclusión: El modelo de atención de salud respetuoso de la identidad de género tiene un impacto positivo en la dignidad de las personas transexuales, por lo que es indispensable replicar este modelo de atención.

In Chile, health care for transsexual users is offered in a context marked by territorial inequality in terms of access to trans-specific services in the process of corporal adaptation. At the same time, sanitary norms that regulate the treatment of transsexual users in relation to their gender identity are not fully complied with, which facilitates discrimination and transphobia. These health care experiences, and their subsequent impact on the users' quality of life and well-being, still remain poorly addressed. Objective: To explore the experiences of transsexual users in the Chilean health care system and the meaning the users assign to those experiences, in relation to the respect shown for their gender identity. Materials: Exploratory, interpretative qualitative study with a phenomenological approach. Semi-structured interviews were conducted with 8 transsexual individuals; this sample size was determined by the data saturation point. The Hermeneutic Content Analysis method was used. Results: The Chilean health care system tends to not respect the gender identity of transsexual individuals; nevertheless, participants reported that the care they receive at Las Higueras Hospital of Talcahuano improves their quality of life. Conclusion: Gender-respectful health care has a positive impact on the dignity of transsexual individuals, which is why it is essential to replicate this model of care.

Enhanced IRES activity by the 3'UTR element determines the virulence of FMDV isolates.

A reverse genetics approach was used to identify viral genetic determinants of the differential virulence displayed by two field foot-and-mouth disease virus (FMDV) strains (A/Arg/00 and A/Arg/01) isolated in Argentina during the 2000-2001 epidemics. A molecular clone of A/Arg/01 strain and viral chimeras containing the S-fragment or the internal ribosome entry site (IRES) of A/Arg/00 in the A/Arg/01 backbone were constructed and characterized. The IRES appeared as a determining factor of the lower level of A/Arg/00 replication in cell culture. High-throughput RNA probing revealed structural differences between both IRESs. Translation experiments using either synthetic viral RNAs (in vitro) or bicistronic plasmids (in vivo) showed that these IRESs' activities differ when the viral 3' untranslated region (UTR) is present, suggesting that their function is differentially modulated by this region. This work provides experimental evidence supporting the role of the IRES-3'UTR modulation in determining the level of FMDV replication in field strains.

Baculovirus treatment fully protects mice against a lethal challenge of FMDV.

Foot-and-mouth disease virus (FMDV) causes a highly contagious and economically devastating disease that affects cattle, swine, goat and sheep among others. FMDV is able to overcome the initial host innate immune response by inhibiting the induction of antiviral molecules at both the transcriptional and the translational levels. It has been demonstrated that FMDV A/Arg/2001 causes the death of adult C57Bl/6 mice within 72h. We evaluated the capacity of Autographa californica nuclear polyhedrosis virus (AcNPV), an insect virus with potent innate immunostimulating effects, to promote early protection against FMDV A/Arg/2001 challenge in C57Bl/6 mice. Groups of 8-9 weeks old female mice were injected intravenously with AcNPV and challenged with a lethal dose of FMDV at different times post-administration. Our results showed that pretreatment of mice with a single injection of AcNPV 3h or 3 days before FMDV challenge resulted in complete abrogation of mortality and complete or partial suppression of viremia, respectively. Furthermore, no signs of disease were observed. AcNPV could be a valuable tool to improve the design of a novel vaccine that protects as an adjuvant at early times post-vaccination.

Differences in the virulence of two strains of Foot-and-Mouth Disease Virus Serotype A with the same spatiotemporal distribution.

During the 2000-2001 epidemic of Foot-and-Mouth Disease Virus (FMDV) in Argentina, two FMDV serotype A viruses were identified among others. Since different pathogenic properties between these virus strains were noticed in cattle, we evaluated several biological properties and features of FMDV A/Arg/00 and FMDV A/Arg/01 in order to compare these viruses in terms of virulence and pathogenicity. Our results indicate that FMDV A/Arg/00 grows less efficiently than FMDV A/Arg/01, exemplified by smaller sized plaques, retarded one-step growth curves and overall low viral yields. Also, FMDV A/Arg/00 displayed the lowest specific infectivity in suckling mice requiring 50-fold more infectious particles than FMDV A/Arg/01 to generate a LD50 in suckling mice. Finally, FMDV A/Arg/00 did not cause death in adult C57Bl/6 mice even at high doses (10(7)-10(6)PFU) whereas FMDV A/Arg/01 resulted lethal in doses as low as 10(2)PFU. Overall, we were able to demonstrate that these virus strains differ from each other in terms of virulence and pathogenicity.

Identification of Vibrio harveyi isolated from diseased cultured wedge sole Dicologoglossa cuneata.

We report the first isolation of Vibrio harveyi from wedge sole Dicologoglossa cuneata. The pathogen was recovered from ulcers and internal organs of ailing cultured fish, from 7 different outbreaks between 2004 and 2006. The 15 isolates found were phenotypically characterized using biochemical tests and BIOLOG GN plates, which revealed high phenotypic diversity. Diagnosis was confirmed with PCR using V harveyi specific primers and partial 16S and 23S rRNA gene sequencing. A virulence evaluation of the isolates was also performed using fry and juvenile wedge sole. Significant mortalities were recorded by intraperitoneal injection; however, no mortalities were recorded by bath immersion.

Recommendation of an appropriate medium for in vitro drug susceptibility testing of the fish pathogen Tenacibaculum maritimum.

In the present study, Anacker and Ordal agar, marine agar (MA), and Flexibacter maritimus medium (FMM) were compared with the dilute versions of Mueller-Hinton agar (DMHA) medium recommended by the National Committee for Clinical Laboratory Standards (NCCLS) for their use in disk diffusion tests with Tenacibaculum maritimum strains and to calculate the MICs of five drugs by the Etest method. Preliminary growth tests performed with 32 strains of this pathogen on each medium revealed that all strains failed to grow on DMHA, while the remaining media supported good growth of all isolates. In the susceptibility tests, which were carried out with the other three media, all strains were resistant to oxolinic acid and were highly susceptible to amoxicillin and trimethoprim-sulfamethoxazole, showing a good correspondence with the Etest values, which ranged from 0.064 to 0.75 and 0.006 to 1.5 mug/ml, respectively. Enrofloxacin and oxytetracycline produced significantly smaller inhibition zones and MICs on MA than on the other media assayed. However, fast, clear, and well-defined zones of inhibition were displayed for all strains at 24 h of incubation only on FMM by both the disk diffusion assay and Etest. In addition, FMM prepared with commercial sea salts instead of seawater was also suitable for bacterial isolation as well as for susceptibility testing. On the basis of these results, the use of FMM to determine the in vitro susceptibility of T. maritimum and its inclusion in a future revision of the NCCLS M42 report are recommended.

Infecciones relacionadas con catéteres venosos: estudio de la incidencia en una unidad de cuidados intensivos pediátricos / Infections related to venous catheters: incidence study in a Pediatric intensive care unit

Se diseñó un trabajo prospectivo para analizar la contaminación de catéteres venosos en una Unidad de Cuidados Intensivos Pediátricos, según técnica semicuantitativa de rolamiento sobre placa de Agar, y las infecciones producidas por catéteres. Se estudiaron 100 catéteres colocados en niños cuya edades oscilaban entre minutos de vida a 12 años, y pesos entre 610 gramos y 68 kilos. Se utilizaron 6 tipos de catéteres. El tiempo que permaneció colocado osciló entre 1 y 24 días, con una media de 7,6 días y un total de días catéter de 766. Resultados: El extremo proximal del catéter se contaminó 12 veces y el distal 14. Los gérmenes predominantes fueron Gram+ (ECN y EA). Hubo un episodio de sepsis por catéter, lo que significa 1,3 episodios de sepsis por 1000 días de catéter. Se contaminaron significativamente los catéteres colocados en menores de 1000 gramos, los que estuvieron colocados menos de 3 días, asociados al territorio cava inferior y los colocados en safena. Conclusiones: La técnica aséptica quirúrgica es fundamental para la colocación y el cuidado de los catéteres. Los que se colocan en territorio infradiafragmático y los que se colocan en menores de 1000 gramos tienen alta chance de contaminación

Estudio leucocitario en animales de laboratorio infectados con virus de encefalitis equina venezolana / Leucocitary study in laboratory animals infected with venezuelan equine encephalitis virus

Se estudió el número de glóbulos blancos y fórmula leucocitaria en animales de laboratorio tales como ratones, ratas y conejos antes y después de producirles una infección experimental aguda con el virus de la Encefalitis equina Venezolana. Se observó una leucopenia que varió de acuerdo a la sensibilidad de la especie animal. Las fórmulas leucocitarias presentaron variaciones, acompañadas de células atípicas las cuales persistieron hasta el final de la infección. Se observó también una vacuolización celular que alcanzó al 6 por ciento y que no estuvo presente nunca en los controles. Los conejos presentaron una eosinofilia que abarcó hasta un 36 por ciento en su fórmula leucocitaria poco antes de morir. Algunas ratas sobrevivieron y al cabo de 14 días presentaron títulos de anticuerpos inhibidores de la hemaglutinación entre 1/80-1/160, lo cual indica su resistencia a la infección viral

Epidemiología de la hipoxia en las unidades de cuidado neonatal / Epidemiology of hypoxia in neonatal intensive care units

En el presente estudio se realizaron 97 registros de saturación de hemoglobina a 61 neonatos críticamente enfermos, internados en cinco Unidades de Cuidado Intensivo Neonatal en el Uruguay. El total de horas registradas fue de 204, siendo el tiempo mínimo de registro de 2 horas por cada niño, período en el que se realiza un ciclo completo de cuidados. Se consideró evento hipóxico cuando la saturación de la hemoglobina registrada en el oxímetro de pulso era de 82 por ciento o menor. Setenta y seis registros (78.9 por ciento ) presentaron eventos hipóxicos. La mediana de eventos por registros fue de 5 y la de duración de 29 segundos. El mayor porcentaje se presentó durante la realización de maniobras. Los cuidados respiratorios y las punciones vasculares fueron los más afectados. Cuando se consideraron 24 horas de registro el tiempo que el neonato estuvo con una saturación menor a 82 por ciento fue de 90 minutos. Esta cifra es tres veces mayor que la mencionada en la literatura como tiempo o evento "no deseable". Se concluye que esta metodología ayuda a evaluar en forma objetiva el nivel del cuidado intensivo neonatal de una unidad, incluso del personal técnico