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BACKGROUND AND AIMS: A gonadotropin-releasing hormone (GnRH)-based therapeutic vaccine candidate against hormone-sensitive prostate cancer has demonstrated its safety and signs of efficacy in phase I/II trials. In this study, we characterized the isotype/subclass profiles of the anti-GnRH humoral response generated by the vaccination and analyzed its association with patients' clinical outcomes. METHODS: The immunoglobulin isotypes and IgG subclasses of the antibody responses of 34 patients included in a randomized, open, prospective phase I/II clinical trial were characterized. Every patient included in the study had a diagnosis of locally advanced prostate adenocarcinoma at stages 3 and 4 and received immunization with the vaccine candidate. Additionally, serum testosterone and prostate specific antigen (PSA) concentrations, serving as indicators of tumor response, were determined. The type of anti-GnRH antibody response was correlated to the time elapsed until the first biochemical recurrence in patients and the outcome of the disease. RESULTS: All patients developed strong and prolonged anti-GnRH antibody responses, resulting in a short- to mid-term decrease in serum testosterone and PSA levels. Following immunizations, anti-GnRH antibodies of the IgM/IgG and IgG1/IgG3 subclasses were observed. Following radiotherapy, the humoral response switched to IgG (IgG1/IgG4). Patients who experienced a short-term biochemical relapse were characterized by significantly higher levels of anti-GnRH IgG titers, particularly IgG1 and IgG4 subclasses. These characteristics, along with a high response of specific IgM antibodies at the end of immunizations and the development of anti-GnRH IgA antibody responses following radiotherapy, were observed in patients whose disease progressed, compared to those with controlled disease. CONCLUSION: The nature of the humoral response against anti-GnRH, induced by vaccination may play a key role in activating additional immunological mechanisms. Collectively, these mechanisms could contribute significantly to the regulation of tumor growth.

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INTRODUCCIÓN: Fosfomicina es un antimicrobiano de amplio espectro utilizado para el tratamiento de las infecciones urinarias bajas; tiene actividad sobre bacilos gramnegativos y cocos grampositivos, así también sobre microorganismos multirresistentes, además de ofrecer una alternativa terapéutica de administración vía oral en dosis única, alcanzando una efectividad de 90%. OBJETIVO: Conocer la sensibilidad in vitro de Escherichia coli frente a fosfomicina, en infecciones urinarias provenientes de personas con discapacidad. MATERIAL Y MÉTODO: Estudio observacional, descriptivo, prospectivo, en el que se incluyó un total de 273 muestras de urocultivo, de pacientes de ambos sexos que acudieron a SENADIS, y que en el momento de la consulta presentaban síntomas de infección del tracto urinario, por lo que se les solicitó el análisis de orina simple y cultivo. De las muestras procesadas en el laboratorio de microbiología, que fueron positivas con crecimiento bacteriano significativo, se procedió a la identificación bacteriana y a la realización del antibiograma según las recomendaciones de CLSI. RESULTADOS: De estas 273 muestras, 91 fueron positivas para diferentes uropatógenos, 62/91 (68%) resultaron ser E. coli. De estas cepas de E. coli, 59/62 (95%) mostraron sensibilidad in vitro a fosfomicina. Comentario: Aunque el número de muestra obtenido es pequeño y no extrapolable ampliamente, pretendemos extender el trabajo por un tiempo más para compararlo más adelante. CONCLUSIONES: Se observa que fosfomicina presenta buena actividad in vitro frente a cepas de E. coli aisladas de urocultivo, pudiendo representar una buena alternativa terapéutica a ser utilizada en la población en estudio.

BACKGROUND: Fosfomycin is a broad-spectrum antibiotic used for the treatment of lower urinary tract infections, it is active against gramnegative bacilli and grampositive cocci, as well as against multi-resistant microorganism, in addition to offering a therapeutic alternative for oral administration in a single dose, reaching an effectiveness of 90%. AIM: To study the susceptibility of Escherichia coli to fosfomycin in urinary tract infections, of isolated strains obtained from patients with disabilities. METHODS: It is an observational, descriptive, prospective study in which a total of 273 urine culture samples of patients of both sexes who attended the SENADIS were included, and who at the time of the consultation presented symptoms of urinary tract infection. The urine positive cultures with significant bacterial growth were performed to determine its bacterial identification and the antibiogram according to CLSI recommendations. RESULTS: Of these 273 samples, 91 samples were positive for different uropathogens, with 62/91 (68%) being positive for E. coli. Of these E. coli strains, 59/62 (95%) showed in vitro susceptibility to fosfomycin. Comment: Although the number of samples obtained is small and it cannot be extrapolated, we pretend to extend the work for a while longer to be able to compare it later. CONCLUSION: Fosfomycin has good activity in vitro against E. coli isolated from urine culture in our institution, representing a good alternative to be used in our study population

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Introducción: la esclerosis múltiple (EM), enfermedad crónica del sistema nervioso (SNC), compromete significativamente la cognición. Su prevalencia en Paraguay es 9,2/100.000 habitantes, 72% con recaída remisión (EMRR) e incidencia de 2-3mujeres/hombre, afecta más a personas en edad productiva, con altos costos económicos y afectivos. Objetivos: caracterizar al paciente con EMRR, evaluar sus funciones ejecutivas (FEs) con BaNFE-2; establecer valores de corte ajustados al país. Metodología: con fundamento en teoría de la neurociencia cognitiva, diseño no experimental, cuantitativo, descriptivo, empírico, retrospectivo y transversal; técnica psicométrica y entrevista neuropsicológica en una muestra por conveniencia de 40 pacientes, 82,5% mujeres y edad 25-55 años (37,78±7,89). Resultados: se reportaron datos demográficos y clínicos, se caracterizaron las escalas de BaNFE-2 cuya consistencia interna resultó significativa. Se obtuvo 52,5% de alteración en la escala prefrontal y 42,5% en FEs; relaciones significativas con escolaridad, discapacidad física (DF), cantidad de brotes y deterioro cognitivo (DC); diferencias significativas por sexo, escolaridad, DF y DC. Se calcularon puntuaciones tipificadas por escolaridad, z<-1 establece el corte entre normalidad y alteración. Conclusión: las pruebas de BaNFE-2 perfilan la afectación del tiempo de ejecución, atención, memoria de trabajo y FEs. Este estudio aporta baremos ajustados al país y abre una novedosa línea de investigación aplicando BaNFE-2 en EM.

Introduction: Multiple sclerosis (MS) is a chronic, neurodegenerative, inflammatory disease of the central nervous system (CNS) that significantly compromises cognitive functions. In Paraguay, it occurs with a prevalence of 9.2/100,000 inhabitants, 72% in the clinical form of relapsing remission (RRMS) and an incidence of 2 to 3 women/men, affecting more people of productive age with high economic and emotional costs. Objectives: To characterize the Paraguayan patient with RRMS, to assess the state of their executive functions (EFs) with the BaNFE-2 battery, and to establish cut-off values adjusted to the country. Methods: Research based on the theory of cognitive neuroscience of non-experimental, quantitative and descriptive design for analytical purposes. It is empirical, retrospective and cross-sectional. The psychometric technique and neuropsychological interview were used in a convenience sample of 40 patients aged 25 to 55 (37.78 ± 7.89) and 82.5% women. Results: Demographic and clinical data of the participants were reported. The BaNFE-2 coded and normalized scales were statistically characterized, the internal consistency of which was significant. 52.5% of the alteration was obtained in the anterior prefrontal scale and 42.5% in FEs; there are significant relationships with schooling, physical disability (PD), number of outbreaks and cognitive impairment (CI); there were also significant differences by sex, education, PD and CI. Standardized scores adjusted for schooling were calculated such that z<-1 establishes the cutoff between normality and abnormality. Conclusion: BaNFE-2 battery tests profile the affectation of execution time, attention, working memory, and FEs. This study provides the adjusted scales for the country and opens a new line of research applying the BaNFE-2 battery in people with MS.

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BACKGROUND/AIM: Arylamine N-acetyltransferase 1 and 2 (NAT1 and NAT2) are drug-metabolizing enzymes that play a key role in the development of acute lymphoblastic leukemia (ALL). MATERIALS AND METHODS: This study evaluated NAT1 and NAT2 mRNA and protein expression and their enzymatic activity in peripheral blood mononuclear cells (PBMC) from patients with ALL (n=20) and healthy children (n=19) and explored the mechanisms that regulate these enzymes in ALL such as microRNAs (miR-1290, miR-26b) and SNPs. RESULTS: PBMC from patients with ALL showed a decrease in NAT1 mRNA and protein expression. In addition, NAT1 enzymatic activity was decreased in patients with ALL. There was no influence of SNP 559 C>T or 560 G>A on low NAT1 activity. The lower expression of NAT1 might be related to the loss of acetylated histone H3K14 in the NAT1 gene promoter in patients with ALL and the higher relative expression of miR-1290 in the plasma of patients with relapsed ALL compared with healthy controls. There were significantly fewer CD3+/NAT1+ double-positive cells in patients who relapsed compared with control subjects. Based on a t-distributed stochastic neighbor embedding algorithm, CD19+ cells that reappeared in patients with relapse showed low NAT1 expression. In contrast, for NAT2, there were no significant results. CONCLUSION: The expression and function of NAT1 and miR-1290 levels could be involved in modulating immune cells altered in ALL.

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La fibroelastosis pleuroparenquimatosa es una enfermedad pulmonar inusual con características clínicas, radiológicas y patológicas únicas, que se ha incluido recientemente en el consenso actualizado sobre neumonías intersticiales idiopáticas. Su nombre hace referencia a una combinación de fibrosis que involucra la pleura visceral y cambios fibroelastóticos que predominan en el parénquima pulmonar subpleural. Aunque se han descrito varias asociaciones de enfermedades, no se ha identificado de manera inequívoca ninguna causa única. El diagnóstico se sustenta en criterios clínicos-radiológicos y de hallazgos histopatológicos cuando se dispone de biopsias. Son escasos los reportes sobre la asociación entre esta entidad y la hipertensión pulmonar. No existe tratamiento hasta la fecha, aunque se relatan sucesos en algunos casos de transplante pulmonar. Describimos las características clínicas de un paciente atendido en un hospital del Paraguay.

Pleuroparenchymal fibroelastosis is an unusual lung disease with unique clinical, radiological, and pathological characteristics that has recently been included in the updated consensus on idiopathic interstitial pneumonia. Their name refers to a combination of fibrosis involving the visceral pleura and fibro-elastic changes that predominate in the subpleural pulmonary parenchyma. Although several associations between diseases have been described, no single cause has been unequivocally identified. The diagnosis is based on clinical-radiological criteria and histopathological findings when biopsies are available. There are few reports on the association between this entity and pulmonary hypertension. There is no treatment to date, although some cases of lung transplantation have been successful. We describe the clinical characteristics of a patient treated in a hospital in Paraguay.

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Enterococci exhibit clumping under the selective pressure of antibiotics. The aim of this study was to analyze the effect of supernatants from a plasmid-free clone (C29) of Enterococcus faecalis subjected to 0.25×, 0.5×, and 0.75× of the minimal inhibitory concentration (MIC) of ampicillin on the expression of an aggregation substance (AS) by a donor plasmid clone (1390R). A clumping assay was performed. The relative expression of prgB (gene that encodes AS) was determined and semiquantified in 1390R, and iad1 expression was determined and semiquantified in C29. AS expression was analyzed in the stimulated 1390R cells by confocal microscopy, flow cytometry, and ELISA. Adherence was also measured. Maximal clumping was observed with the pheromone medium 0.25×. Only the 1390R strain stimulated with the C29 supernatant without ampicillin and with 0.25× was able to express prgB. No expression of prgB was observed at 0.5× and 0.75×. The difference in relative expression (RE) of 1390R without ampicillin and with 0.25× was 0.5-fold. AS expression in 1390R showed the greatest increase upon stimulation with 0.25×. When 1390R was stimulated with 0.5× and 0.75×, AS expression was also observed but was significantly lower. Ampicillin stimulated C29 switch-off pheromone expression in recipient cells, which in turn switched off AS expression in donor cells. We observed that although prgB was switched off after 0.5× stimulation in C29, the supernatants induced expression in certain 1390R strains. In conclusion, ampicillin was able to modulate pheromone expression in free plasmid clones which, in turn, modulated AS expression in plasmid donor cells. The fact that PrgB gene expression was switched off after the ampicillin stimulus at 0.5× MIC, whereas AS proteins were present on the surface of the bacteria, suggested that a mechanism of rescue associated with mechanism pheromone sensing may be involved.

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Es imposible separar la enfermedad física de la enfermedad mental, puesto que la fisiopatología de cualquier enfermedad contiene a la esfera psicológica dentro de sus múltiples elementos. En ese sentido, muchas especialidades médicas pueden reclamar su relación con la Psiquiatría, puesto que son innumerables las enfermedades que tienen nexo con signos y síntomas psiquiátricos (por ejemplo, la enfermedad inflamatoria intestinal en Gastroenterología; el asma bronquial en Neumología, entre otras). No obstante, con la Dermatología la relación es mucho más evidente. La Psicodermatología es una especialidad que surge de la conjunción de dos ramas aparentemente divergentes de la Medicina: la Psiquiatría y la Dermatología. En Psicodermatología, algunos pacientes presentan enfermedades principalmente dermatológicas con comorbilidades psicosociales secundarias, mientras que otros tienen trastornos psiquiátricos primarios con sintomatología cutánea significativa. Así también, debido a que varias enfermedades dermatológicas no solo se acompañan frecuentemente de dolor e incomodidad, sino que son inmediatamente visibles para los demás, las personas afectadas por estas condiciones pueden sufrir consecuencias sociales y emocionales. Con base en lo anterior, este artículo de revisión presenta los conceptos, nosología y modelos de abordaje de esta ciencia médica, haciendo hincapié que, debido a la interacción permanente entre la mente y la piel, se hace necesario que el paciente sea tratado como una unidad constituida por varios niveles, incluyendo aspectos cutáneos, emocionales y mentales.

It is impossible to separate the physical illness from the mental illness, since the pathophysiology of any disease contains the psychological sphere within its multiple elements. In this sense, many medical specialties can claim their relationship with Psychiatry, since there are innumerable diseases that have a link with psychiatric signs and symptoms (for example, inflammatory bowel disease in Gastroenterology; bronchial asthma in Pneumology, among others). However, with Dermatology the relationship is much more evident. Psychodermatology is a specialty that arises from the conjunction of two apparently divergent branches of Medicine: Psychiatry and Dermatology. In Psychodermatology, some patients present mainly dermatological diseases with secondary psychosocial comorbidities, while others have primary psychiatric disorders with significant cutaneous symptoms. Also, because various dermatologic diseases are not only frequently accompanied by pain and discomfort, but are immediately visible to others, people affected from these conditions can suffer social and emotional consequences. Based on the above, this review article presents the concepts, nosology, and approach models of this medical science, emphasizing that due to the permanent interaction between the mind and the skin, it is necessary for the patient to be treated as an unit made up of several levels, including cutaneous, emotional and mental aspects.

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PURPOSE: To develop and validate a population pharmacokinetic model of Methotrexate (MTX) in Mexican children with acute lymphoblastic leukemia (ALL) for the design of personalized dosage regimens based on the anthropometric and physiological characteristics of each patient. METHODS: A prospective study was developed in 50 children (1-15 years old) with ALL diagnosis attended at Pediatric Hemato-Oncology Service from Hospital Central "Dr. Ignacio Morones Prieto" and under treatment with high doses of MTX administered in 24-h continuous intravenous infusion. Plasma concentrations of MTX were determined in blood samples collected at 24, 36, 42 or 48 h post-infusion, by means of the CMIA immunoassay. The development of the population pharmacokinetic model was performed using the NONMEM® software evaluating the covariates that influence in clearance (CL), intercompartmental clearance (Q), central (Vc) and peripheral (Vp) volume of distribution of MTX. RESULTS: A two-compartment open model was selected to describe concentration-time data and body surface area (BSA) was the covariate that influences on MTX total CL. The population pharmacokinetic model obtained was: CL (L/h) = 6.5 × BSA0.62, Vc (L) = 0.36 × Weight, Q (L/h) = 0.41 and Vp (L) = 3.2. Internal validation was performed by bootstrap and visual predictive check. Predictive performance of final model was evaluated by external validation in a different group of patients. Initial MTX dosing regimens were established by stochastic simulation with final population pharmacokinetic model. CONCLUSIONS: The establishment of MTX dosing criteria in children with ALL should be adjusted based on the BSA of each patient to optimize oncological therapy and reduce the development of adverse effects. Therapeutic drug monitoring is an essential tool to individualize MTX doses to reduce toxicity and improve patients' outcomes.

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The clinical characteristics of the pediatric population infected with the SARS-CoV-2 virus in general are not as severe as in the adult population, so they can be considered asymptomatic carriers. The pediatric patient with congenital heart disease are considered a high risk group of contagion in the SARS-CoV-2 pandemic, so healthcare personnel who interact with patients must have established guidelines to avoid transmission and spread of the disease. Each country is commanded by the central guidelines established by its health system considering operative definitions and protocols, but in certain places these guidelines do not fulfill international standards, as those proposed by the World Health Organization. In this communication we have done a current literature review and adaptation of the recommendations to face the infectious outbreak due to the SARS-CoV-2 virus in pediatric cardiovascular surgery programs, specifically in the ​​anesthesiology area. We also analyze the type of personal protective equipment that should be used in each area of ​​patient management, changes in the environment of work areas, shift times of health personnel, the protection of personnel performing transesophageal echocardiography, modification of the airway management algorithms, proper placement and withdrawal of personal protective equipment, patients transfer between wards or other services, and adequate disinfection of airway equipment used.

Las características clínicas de la población pediátrica contagiada del virus SARS-CoV-2 en general no son tan severas como en la población adulta, por lo que pueden ser considerados portadores asintomáticos. El paciente pediátrico con cardiopatía congénita pertenece a un grupo de alto riesgo de contagio dentro de la pandemia producida por el SARS-CoV-2, por lo que el personal sanitario que interactúe con los pacientes debe tener lineamientos establecidos para evitar la transmisión y propagación de la enfermedad. Cada país se rige por las guías centrales establecidas por su sistema de salud en cuanto a definiciones operativas y protocolos, pero en algunos lugares estas directrices no cumplen las metas internacionales, como las propuestas por la Organización Mundial de la Salud. En este comunicado hemos realizado una revisión de la literatura actual y adaptación de las recomendaciones para enfrentar el brote infeccioso por el virus SARS-CoV-2 en los programas de cirugía cardiovascular pediátrica, específicamente en el área de anestesiología. También analizamos el tipo de equipo de protección personal que debe ser utilizado en cada área del manejo de pacientes, cambios del ambiente de las áreas de trabajo, rotación de personal, la protección del personal que realiza ecocardiografía transesofágica, modificación de los algoritmos de manejo de la vía aérea, colocación y retiro correctos del equipo de protección personal, traslado de los pacientes entre servicios, y adecuada desinfección del equipo utilizado en el manejo de la vía aérea.

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The incidence of COVID-19 has rapidly evolved into a pandemic. The accelerated spread of the SARS-CoV-2 virus worldwide has started a health system race to contain the disease. Guides have been created for the different medical branches for the proper management of patients suspected or positive for the disease. The procedures carried out in endoscopic procedures clinics must modify their rules and regulations due to the high risk of transmission, through aerosol-generating procedures (PGA) such as upper endoscopy and colonoscopy, and consideration of the possibility of fecal ­ oral transmission in the practice of colonoscopy. Most anesthesiology protocols for COVID-19 focus on the in-hospital setting and omit out-of-theater procedures performed under anesthetic sedation. This document presents the protocol for the evaluation and anesthetic management of the patient for endoscopic procedures in the COVID-19 outbreak, according to current literature. It is designed to be adapted to the work policies of different institutions dedicated to performing endoscopic procedures during the epidemic.

La incidencia de COVID-19 ha evolucionado rápidamente a pandemia. La diseminación acelerada del virus SARS-CoV-2 a nivel mundial ha iniciado una carrera del sistema de salud para la contención de la enfermedad. Se han creado guías de las diferentes ramas médicas para el manejo adecuado de los pacientes sospechosos o positivos para la enfermedad. Los procedimientos llevados a cabo en las clínicas de procedimientos endoscópicos deben modificar sus normas y disposiciones debido al elevado riesgo de transmisión, por medio de los procedimientos generadores de aerosol (PGA) como lo es la endoscopia superior y colonoscopía, y consideración de la posibilidad de transmisión fecal ­ oral en la práctica de la colonoscopía. La mayor parte de los protocolos de anestesiología para COVID-19 se enfocan al entorno intrahospitalario, y omiten los procedimientos fuera de quirófano realizados bajo sedación anestésica. En este documento se expone el protocolo de evaluación y manejo anestésico del paciente para procedimientos endoscópicos en el brote de COVID-19, según la literatura actual. Está diseñado para que se adecue a las políticas de trabajo de diferentes instituciones dedicadas a la realización de procedimientos endoscópicos durante la epidemia.

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Heberprovac is a GnRH based vaccine candidate containing 2.4 mg of the GnRHm1-TT peptide as the main active principle; 245 µg of the very small size proteoliposomes adjuvant (VSSP); and 350 µL of Montanide ISA 51 VG oil adjuvant. The aim of this study was to assess the safety and tolerance of the Heberprovac in advanced prostate cancer patients as well as its capacity to induce anti-GnRH antibodies, the subsequent effects on serum levels of testosterone and PSA and the patient overall survival. The study included eight patients with histologically-proven advanced prostate cancer with indication for hormonal therapy, who received seven intramuscular immunizations with Heberprovac within 18 weeks. Anti-GnRH antibody titers, testosterone and PSA levels, as well as clinical parameters were recorded and evaluated. The vaccine was well tolerated. Significant reductions in serum levels of testosterone and PSA were seen after four immunizations. Castrate levels of testosterone were observed in all patients at the end of the immunization schedule, which remained at the lowest level for at least 20 months. In a 10-year follow-up three out of six patients who completed the entire trial survived. In contrast only one out eight patients survived in the same period in a matched randomly selected group receiving standard anti-hormonal treatment. Heberprovac vaccination showed a good security profile, as well as immunological, biochemical and, most importantly, clinical benefit. The vaccinated group displayed survival advantage compared with the reference group that received standard treatment. These results warrant further clinical trials with Heberprovac involving a larger cohort.

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Las infecciones del tracto urinario (ITU) en niños con vejiga neurogénica, pueden producir alteraciones tanto morfológicas como funcionales, y desencadenan una respuesta inmune que no siempre es evidente. Las ITU son una de las principales causas de morbilidad y mortalidad. Resulta de suma importancia un correcto diagnóstico de las mismas, para un buen tratamiento y así evitar las complicaciones y secuelas que puedan ocasionar. El objeto del trabajo fue analizar la prevalencia y la etiología de las ITU en pacientes con vejiga neurogénica, y su patrón de resistencia. En un estudio descriptivo, retrospectivo se incluyeron 46 muestras de orina para cultivo de niños de 1 a 18 años, de ambos sexos, con vejiga neurogénica, que fueron procesadas en un laboratorio de microbiología. De 46 urocultivos, 19 correspondieron a varones (19/46) 41% y 27 a niñas (27/46) 59%, los niños tenían una edad promedio de 11(2. De estos 21 resultaron positivos (21/46) 46%, aislando en mayor proporción E. coli. La frecuencia de ITU en niños con vejiga neurogénica fue de 21/46, 46%, siendo el porcentaje en este tipo de pacientes mucho más elevado que en niños que no presentan dicha anomalía. Los uropatógenos aislados con mayor frecuencia fueron E coli y K pneumoniae. El uso de sondas y pañales, así como la mala higiene predisponen a estas infecciones. En este estudio se vio buena sensibilidad a la nitrofurantoina y cefixima(AU)

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OBJECTIVE: To compare the costs and clinical consequences of treating mild-to-moderate joint bleeds with recombinant activated factor VII (rFVIIa) versus plasma-derived activated prothrombin complex concentrate (pd-aPCC) in pediatric patients with hemophilia A with inhibitors in Mexico. METHODS: A cost-effectiveness model was developed using TreeAge Pro v14.2.2 software (licensed in the USA) and adapted from a previously published model, with adjustments to reflect local clinical practice. Expert opinion was sought regarding patients' clinical management and resource utilization in Mexico to ensure that the current model was appropriate and relevant. The model compared rFVIIa and pd-aPCC for the treatment of mild-to-moderate joint bleeds in children <14 years old (assumed average weight: 30 kg). The analysis outcome was incremental cost per resolved mild-to-moderate joint bleed. One-way sensitivity analysis and probabilistic sensitivity analysis were used to assess specific assumptions and to address any uncertainty in the model. RESULTS: The cost of treating mild-to-moderate joint bleeds was lower for rFVIIa versus pd-aPCC after 7 days (MX$105,581 vs. MX$132,024), assuming complete bleed resolution. After 48 hours, rFVIIa was associated with an 8% improvement in bleed resolution versus pd-aPCC, resulting in cost savings of MX$16,754. Probabilistic sensitivity analysis indicated that rFVIIa treatment was more cost-effective than pd-aPCC in 67% (at 7 days) and 72% (at 48 hours) of Monte Carlo simulations. CONCLUSION: Accounting for model uncertainty, rFVIIa provided cost savings over pd-aPCC for the Mexican public health care payer in the management of mild-to-moderate joint bleeds in pediatric hemophilia A with inhibitors.

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Abstract Myotonic dystrophy is a disease affecting the muscle fibers with loss of muscle mass. The principal characteristic of the disease is myotony or slow muscle relaxation following muscle contraction that is further aggravated as a result of stress, pain, cold, or by the administration of succinylcholine. Similar to other muscle pathologies, myotonic dystrophy is considered a multisystem disorder, usually with cardiac and respiratory involvement, a fact to be kept in mind when planning anesthesia. Moreover, there is a potential association with malignant hyperthermia or rhabdomyolysis associated with some muscle diseases. The case herein discussed is an example of the management of anesthesia in this group of patients to avoid the potential triggers of a myotonic crisis.

Resumen La distrofia miotónica es una enfermedad de las fibras musculares que cursa con pérdida de masa muscular y cuya característica principal es la miotonía, que describe la relajación muscular lenta tras una contracción muscular, situación agravada por estrés, dolor, frío, o por la administración de succinilcolina. Como toda enfermedad muscular, es considerada multisistémica, con afectación cardíaca y respiratoria en la mayoría de los casos, lo cual deberá tenerse en cuenta a la hora de elaborar un plan anestésico. Además, se debe considerar la posible relación con el desarrollo de hipertermia maligna o rabdomiólisis asociada a algunas enfermedades musculares. El caso que presentamos es un ejemplo del manejo anestésico de estos pacientes evitando los posibles desencadenantes de una crisis miotónica.

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Neonatal hypotonia is a relatively common cause of consultation in daily pediatric practice. It is part of the clinical presentation of a large group of heterogeneous diseases, many of which have an important and classifiable genetic background. Identification of the specific disorder can help optimize the management and treatment of the patient and inform genetic counseling for the family, and therefore input from clinical geneticists is critical at the earliest stages of medical management. Here we present 30 patients with hypotonia of unknown etiology referred by a neuropediatrician to clinical genetics. Clinical, genetic, and molecular evaluation of each patient was performed. Sixty-nine percent of the patients included in the study had a genetic disease, including eight with Prader-Willi syndrome, three with spinal muscular atrophy, one with Rett syndrome, and one with Sotos syndrome harboring a previously undescribed mutation. Our data demonstrate that a multidisciplinary approach used from the outset that includes molecular analysis can help improve diagnosis and management of hypotonic infants.

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Central hypotonic is one of the most difficult issues in neurology, ruling out neurogenetic syndromic causes is critical, Prader-Willi syndrome (PWS) it is the most frequent genetic syndrome, it is caused by the loss of expression of the paternal allele in a group of imprinted genes within 15q11-q13, and is characterized by severe prenatal and postnatal hypotonia. SNURF-SNRPN gene methylation detects 99% of the cases but fluorescent in situ hybridization (FISH) analysis is necessary to confirm chromosome microdeletions. The advantage of SNRP-quantitative strategy of methylated alleles is that it makes it possible to make the diagnosis and identify deletions and mosaicism in one reaction. In infants clinical diagnosis is difficult. It has been proposed that around 40% of hypotonic patients have PWS but an accurate percentage has not been established. Twenty-four central hypotonic infants were studied by this molecular strategy, showing 41.5% with the disease. This molecular approach also permitted calculation of gene dosage and detection of those cases with microdeletion.

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Prolactin (PRL) is a 23-kDa protein hormone that is synthesized mainly by the anterior pituitary gland. However, PRL can also be synthesized and secreted by extrapituitary tissues, particularly immune cells. A biallelic polymorphism (-1149 G/T) in the prolactin promoter has been shown to be functionally important, as modulation of prolactin expression has been associated with SLE in some populations. We have performed an association study using Mexican patients with SLE. We used qPCR to determine the SNP allele and genotype frequencies. We did not find statistically significant differences in allele and genotype frequencies between patients and healthy controls. However, we found a statistically significant association between the G allele and the presence of anti-dsDNA antibodies in serum (Allele frequency (G): P = 0.005; Genotyping frequency (GG): P = 0.001, OR = 7.8, 95% CI 3.59-27.1). Our data demonstrate that the prolactin promoter polymorphism -1149 G/T does not significantly contribute to SLE disease susceptibility but does predispose carriers to other immunological changes.

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Introducción: la paracoccidioidomicosis (PCM) es una micosis profunda, sistémica y endémica en áreas rurales de Centro y Sudamérica, cuya fuente de infección es la tierra contaminada con el hongo; siendo por ello la población rural agricultora susceptible de adquirirla. Objetivos: describir la frecuencia y características clínico-patológicas de los pacientes con PCM en la Cátedra de Dermatología del Hospital de Clínicas. Facultad de Ciencias Médicas. Universidad Nacional de Asunción (FCMUNA). Analizar el tratamiento recibido y la evolución de los pacientes. Materiales y métodos: El presente trabajo es un estudio descriptivo de tipo serie de casos, de 68 306 consultas en la Cátedra de Dermatología del Hospital de Clínicas. FCM-UNA en el periodo 2005-2011. Resultados: Se encontraron 36 casos de micosis profunda (0.05% de frecuencia) y de ellos 16 pacientes tenían diagnóstico de PCM (0.02% de frecuencia), representando el 44% de las micosis profundas. Los varones (15/16), agricultores (10/16) fueron los más frecuentemente afectados. Conclusión: Las micosis profundas, y particularmente la PCM, constituyen patologías de consulta importante en la Cátedra de Dermatología del Hospital de Clínicas y debería considerarse su inclusión dentro de los programas de salud pública solventados por el estado paraguayo.

Background: Paracoccidioidomycosis (PCM) is a deep mycosis, systemic and endemic in rural areas of Central and South America, which source of infection is the contaminated soil with the fungus, and therefore rural farmers are very susceptible to acquired it. Aims: to describe the frequency and clinic-pathological characteristics of patients with PCM in the Department of Dermatology at Clinicas Hospital. Faculty of Medical Sciences. National University of Asunción (FCM-UNA). To analyze their treatments and outcomes. Methods: descriptive study from 68 306 consultations at the Department of Dermatology, Clinicas Hospital in the period 2005-2011. Results: there were 36 patients with deep mycosis (0.05% frequency), and of these 16 had the diagnosis of PCM (0.02% frequency), accounting for 44% of deep mycoses. Males (15/16) and farmers (10/16) were most frequently affected. Conclusion: deep mycoses and particularly PCM are particular important reasons for consultation in the Department of Dermatology at Clinicas Hospital, FCM-UNA, and its inclusion in public health programs funded by the Paraguayan government should be considered.

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La leishmaniasis es una enfermedad infecciosa, no contagiosa, de evolución crónica, causada por un protozoario del género Leishmania y transmitida al hombre a través de la picadura del flebótomo hembra infectado, vector de la enfermedad. Presentamos el caso de un lactante menor con leishmaniasis cutánea localizada, que tuvo buena respuesta al tratamiento con antimoniato de N-metilglucamina.

Leishmaniasis is a chronic, infectious but not contagious disease caused by a protozoan of the genus Leishmania that is transmitted to humans through the bite of infected female sand fly, which is the vector of the disease. We present the case of an infant with localized cutaneous leishmaniasis who had a good response to the treatment with N-methylglucamine antimoniate.

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Se destacan los hechos más relevantes que han incidido en el desarrollo de la educación médica superior revolucionaria al arribar a su medio siglo de existencia, en lo referente a trayectoria histórica, misión, cambios en el sector salud, éxodo de médicos, depuración de profesores y estudiantes, reforma universitaria, integración de los hospitales docentes, descentralización de la formación de médicos, prohibición del ejercicio privado de la medicina y estomatología, plan de becas universitarias, red de centros formadores, formación en pregrado y posgrado, claustro, carreras, planes y programas de estudios, formación de estudiantes extranjeros, nuevos proyectos formativos, educación médica en el exterior, universalización de la enseñanza en salud, egresados, ubicación laboral, efectos del bloque y el período especial y adversidades confrontadas, pero a pesar de todo los logros alcanzados y los resultados obtenidos son notorios.

The more significant facts affecting the development of the revolutionary higher medical education at its half century of existence, concerning to the historical trajectory, mission, health sector changes, physicians exodus, purge of professors and students, university reform, teaching hospital integration, decentralization of the physicians training, ban from to exert the private medicine and stomatology, university scholarships plan, training centers framework, pregraduate and postgraduate training, staff, careers, study plans and programs, foreign students training, new formative projects, medical education abroad, health teaching universalization, graduates, working location, blockade effects and the special period as well as the confronted adversities, but despite these obstacles the achievements and the results are significant.

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