Introduction:Tuberous sclerosis complex is a rare genetic disorder leading to the growth of hamartomas in multiple organs, including cardiac rhabdomyomas. Children with symptomatic cardiac rhabdomyoma require frequent admissions to intensive care units, have major complications, namely, arrhythmias, cardiac outflow tract obstruction and heart failure, affecting the quality of life and taking on high healthcare cost. Currently, there is no standard pharmacological treatment for this condition, and the management includes a conservative approach and supportive care. Everolimus has shown positive effects on subependymal giant cell astrocytomas, renal angiomyolipoma and refractory seizures associated with tuberous sclerosis complex. However, evidence supporting efficacy in symptomatic cardiac rhabdomyoma is limited to case reports. The ORACLE trial is the first randomised clinical trial assessing the efficacy of everolimus as a specific therapy for symptomatic cardiac rhabdomyoma.Methods:ORACLE is a phase II, prospective, randomised, placebo-controlled, double-blind, multicentre protocol trial. A total of 40 children with symptomatic cardiac rhabdomyoma secondary to tuberous sclerosis complex will be randomised to receive oral everolimus or placebo for 3 months. The primary outcome is 50% or more reduction in the tumour size related to baseline. As secondary outcomes we include the presence of arrhythmias, pericardial effusion, intracardiac obstruction, adverse events, progression of tumour reduction and effect on heart failure.Conclusions:ORACLE protocol addresses a relevant unmet need in children with tuberous sclerosis complex and cardiac rhabdomyoma. The results of the trial will potentially support the first evidence-based therapy for this condition.

Objective: Estimate the economic burden of type 1 diabetes (T1D) in the Dominican Republic and its impact on treatment adherence and patients' quality of life (QoL). Materials and Methods: Cross-sectional observational study about T1D treatment cost and adherence plus patients QoL. The total monthly cost of treatment regimes was calculated through microcosting analysis and correlated with a national minimum wage (NMW) and average household income. Sociodemographic, clinical, treatment adherence and QoL data were obtained through an online questionnaire. In an exploratory approach, all variables were compared with the categorization of household income. Results: Fixed doses (FD) regime has the lowest cost (46% of the NMW) and continuous subcutaneous insulin infusion (CSII) the highest (540% of the NMW). The lower household income group had less insurance coverage (P= 0.034), purchased fewer diabetes management supplies for cost-related reasons (P= 0.014), performed less glycemic monitoring (P= 0.016), and had more cost-related factors limiting appropriate treatment and follow-up (P= 0.030). Also, the FD treatment modality predominated in this group, while Multiple Daily Injections and constant subcutaneous insulin injection prevailed on the higher income group (P= 0.005). The QoL mean scores were 31.63 (SD 8.02) in the lowerincome group and 32.52 (SD 8.81) in the higher-income one. Conclusions: T1D has a high monthly economic impact, potentially worsening treatment adherence and QoL, especially in the lower socioeconomic status population. Efforts must be made by the healthcare system to enhance the economic support and management of this disease.

Introduction: Critical thinking is an ideal tool for evaluating information as a guiding action. With the conjunct use of the internet and applied pedagogy, PPCR looks to develop its alumni's superior cognitive skills. We aim to characterize alumni and their experiences in the Dominican Republic for over nine years. Method: This descriptive, cross-sectional study used a survey instrument formatted with closed and semi-closed questions, which was sent to each participant's email. The survey had two segments, the first with twelve questions related to the participant's personal information and characteristics related to their background education and profession. The second section evaluated satisfaction during their participation in the PPCR course. Results: Out of a total of 62 graduates from 2012 to 2019, data was collected from 52 participants, of which 92% are medical doctors. The mean age was 36 years, with a predominance of the female gender (56%). The vast majority of participants are Dominican (90%), with a postgraduate program (61.2%). More than half carry out university teaching activities (54%), and a part of them continued to be affiliated in the program as Teaching Assistants, performing pedagogical assistance tasks (38%). The global satisfaction rate was high (92%), with a low dropout rate (2%). Conclusions: Based on our study's characteristics, this course is estimated to be a novel element capable of promoting scientific production and promoting clinical research activities in developing countries. It creates connections and exploits virtuality to improve knowledge through the critical analysis of information. This experience plays a prominent role in the enrichment of many teachers by providing them with methodological tools, developing the excellent training of educators.

Introducción: el síndrome coronario agudo (SCA) es una serie de signos y síntomas que se refieren a isquemia miocárdica repentina. Estas condiciones clínicas son Síndrome Coronario agudo sin Elevación del Segmento ST (SCASEST) y Síndrome Coronario Agudo con Elevación del Segmento ST (SCACEST). A nivel mundial hay hallazgos controversiales con respecto a la diferencia de presentación de SCA en hombres y mujeres. Objetivo: determinar las diferencias en presentación del síndrome coronario agudo según el género en República Dominicana. Métodos: en este estudio observacional retrospectivo unicéntrico, 3,548 pacientes con SCA llevados al laboratorio de cateterismo fueron observados durante el período de agosto de 2016 a septiembre de 2019. Resultados: la edad promedio de presentación fue mayor en las mujeres, con una media de 63 años (DE ± 12.2). El SCACEST fue más frecuente en hombres (69.35 %) y la Angina Inestable en mujeres (50.14 %, p <0.0001). Angiográficamente, la enfermedad coronaria multivascular fue más común en hombres y la angiografía coronaria normal en mujeres (41.30 % y 35.37 %, p <0.0001). Conclusión: dentro de la población dominicana, la mujer tiende a ser mayor al momento de presentación de SCA, con menor tendencia a someterse a intervención coronaria terapéutica.

Introducción: la diabetes tipo 1 es una enfermedad crónica de alto impacto económico con gran capacidad de ser controlada, la misma no tiene ninguna descripción local previa. Su principal causa de mortalidad es los eventos cardiovasculares y el manejo adecuado la disminuye considerablemente. Objetivo: determinar el riesgo cardiovascular en pacientes adultos con diabetes tipo 1 en la ciudad de Santiago de los Caballeros, República Dominicana. Método: se realizó un estudio descriptivo transversal multicéntrico con 39 pacientes en el período de junio a noviembre de 2019. La calculadora “Steno T1 Risk Engine” se utilizó para estimar el riesgo cardiovascular. Resultados: se obtuvo una relación significativa entre la albuminuria (p = 0.0127), presión arterial sistólica (p = 0.0002), tiempo de diagnóstico (p = 0.0037) y nivel de riesgo cardiovascular. La hemoglobina glucosilada (p = 0,7884) y la actividad física (p = 0.706) no mostraron una relación significativa con el riesgo cardiovascular. Conclusión: el nivel de riesgo cardiovascular promedio es bajo, con probabilidades <10 % de un evento cardiovascular agudo dentro de los 10 años. Esta herramienta permite incluir una evaluación cardiovascular rutinaria con datos que perfilen el tratamiento orientado a disminuir complicaciones vasculares, mortalidad y aumentar adherencia al tratamiento.

Introducción: la malnutrición infantil representa uno de los problemas de salud pública más importantes de la República Dominicana (RD) y el mundo. A pesar de esto, actualmente, no existen estudios en la RD que describa el estado nutricional en los niños de la escuela primaria. Material y métodos: este estudio transversal describió las principales variables antropométricas en niños de 1ero a 6to de primaria en Santiago, RD, además de determinar la relación existente entre la antropometría y variables sociodemográficas. Resultados: de los 2,271 estudiantes estudiados, la media del peso fue 33,2 ± 11,4 kg, la talla fue 1,36 ± 0,13 m. La media del percentil fue 65,33 %. El 3.92 % (n=89) estuvo en bajo peso, el 17.57 % (n=399) estuvo en sobrepeso y el 22.94 % (n=521) estuvo en obesidad. Conclusión: el sobrepeso y la obesidad infantil fueron los trastornos más comunes en zonas rurales y urbanas, y tanto en centros privados como públicos.

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Objective. The prognostic value of N-terminal procerebral natriuretic peptide (NT-proBNP) in patients with heart failure (HF) is well established. In contrast, its role as an early predictor of mortality in patients hospitalized for heart failure with preserved ejection fraction (HF-EF) and heart failure with reduced ejection fraction (HF-EF) is less well documented. Therefore, the objective of this study is to evaluate the usefulness and prognostic value of plasma NT-proBNP in these patients. Method. This retrospective observational study included 620 patients admitted for acute heart failure, classified into 3 groups according to their left ventricular ejection fraction (LVEF): HF-EF (LVEF ≥ 50%), HF-mEF (heart failure with ejection fraction mean) (LVEF 35-49%) and HF-rEF (LVEF <40%), whose plasma levels of NT-proBNP and clinical data were determined at hospital admission. Univariate and multivariate logistic regression was used to perform prognostic values of NT-proBNP levels for 3.4 years of all-cause mortality in each group. Results: The mean plasma levels of NT-proBNP in patients with HF-cEF (35%) and borderline HF-cEF (43%) was 1001-5000 pg / ml; patients with HF-rEF were similarly distributed between the groups 1001-5000pg / ml (30%), 5001-15000pg / ml (31%) and> 15001pg / ml (30.6%). The mortality rate increased significantly in patients with NT-proBNP concentrations > 15001 pg / ml (40%) and decreased with NT-proBNP levels <250 pg / ml (4%), compared to the other NT-proBNP groups. The mortality rate increased proportionally to elevated baseline NT-proBNP, regardless of LVEF. Conclusion. In patients hospitalized for an acute decompensated event with HF-cEF (LVEF ≥50%) and HF-mEF (LVEF 35-49%), plasma levels of NT-proBNP are a useful tool to predict early mortality, as for HF -FEr (LVEF <40%).