Binding by calmodulin is coupled to transient unfolding of the third FF domain of Prp40A.

Human pre-mRNA processing protein 40 homolog A (hPrp40A) is a splicing factor that interacts with the Huntington's disease protein huntingtin (Htt). Evidence has accumulated that both Htt and hPrp40A are modulated by the intracellular Ca2+ sensor calmodulin (CaM). Here we report characterization of the interaction of human CM with the third FF domain (FF3 ) of hPrp40A using calorimetric, fluorescence and structural approaches. Homology modeling, differential scanning calorimetry and small angle X-ray scattering (SAXS) data show FF3 forms a folded globular domain. CaM was found to bind FF3 in a Ca2+ -dependent manner with a 1:1 stoichiometry and a dissociation constant (Kd ) of 25 ± 3 µM at 25°C. NMR studies showed that both domains of CaM are engaged in binding and SAXS analysis of the FF3 -CaM complex revealed CaM occupies an extended configuration. Analysis of the FF3 sequence showed that the anchors for CaM binding must be buried in its hydrophobic core, suggesting that binding to CaM requires unfolding of FF3 . Trp anchors were proposed based on sequence analysis and confirmed by intrinsic Trp fluorescence of FF3 upon binding of CaM and substantial reductions in affinity for Trp-Ala FF3 mutants. The consensus model of the complex showed that binding to CaM binding occurs to an extended, non-globular state of the FF3 , consistent with coupling to transient unfolding of the domain. The implications of these results are discussed in the context of the complex interplay of Ca2+ signaling and Ca2+ sensor proteins in modulating Prp40A-Htt function.

Neumonías complicadas. ¿Un problema aún?

Introducción. La neumonía adquirida en la comunidad complicada (CCAP) se caracteriza por una enfermedad grave, hospitalización y un curso prolongado. Las complicaciones de la neumonía se produ­cen cuando la infección no se limita al parén­quima pulmonar, sino que se extiende a áreas vecinas, o cuando el desarrollo de la infección es más complejo que el habitual por diferentes motivos. Estas complicaciones pulmonares suponen solo el 1% al 3% de las mismas, pero este porcentaje aumenta a casi un 40% en caso de precisar ingreso hospi­talario, por lo que se considera una patología fundamentalmente hospitalaria. Objetivo. El propósito de este estudio fue analizar las complicaciones de la neumonía bacteriana adquirida en la comunidad y describir sus características epidemiológicas, clínicas y etiológicas en el periodo de estudio.Material y método. Este es un estudio retrospectivo y descriptivo de las neumonías bacterianas complicadas y sus características en el Hospital del Niño Dr. José Renán Esquivel. Se realizó la búsqueda de los expedientes clínicos de los pacientes con diagnóstico de egreso de neumonía bacteriana adquirida en la comunidad complicada con derrame pleural, neumotórax, pioneumotórax, neumonía necrotizante y absceso pulmonar en el periodo del 1 de enero de 2015 al 31 de diciembre de 2020.Todos los expedientes médicos recuperados se analizaron para obtener información de las complicaciones de la neumonía bacteriana adquirida en la comunidad con relación a datos epidemiológicos, frecuencia, características, tratamiento y evolución clínica. Resultados. De acuerdo con los datos obtenidos de los boletines estadísticos del Hospital Del Niño Dr. José Renán Esquivel para el periodo comprendido entre el 1 de enero de 2015 al 31 de diciembre de 2020 se encontraron 2519 neumonías bacterianas, correspondiendo al 30% de las neumonías. De las neumonías bacterianas, 277 casos de neumonía complicada cumplieron los criterios de inclusión representando un 11% de las neumonías bacterianas. La complicación más frecuente, en todos los grupos de edad, fue el derrame pleural paraneumónico con una frecuencia de 170 casos (61%). La edad promedio de los pacientes con neumonías complicadas fue de 51.7 meses (rango de 27 días a 164 meses) y el grupo de 1 ­ 4 años fue el más afectado presentando 118 (43%) casos de complicaciones. La duración promedio de hospitalización fue de 14.8 días con un rango de 9- 38 días. Los pacientes recibieron antibióticos endovenosos de amplio espectro siendo el tratamiento empírico inicial más utilizado ceftriaxona más clindamicina. La duración promedio del tratamiento con antibióticos fue de 12.7 días con un rango de 10 a 25 días y varió de acuerdo con el tipo de complicación. Se reportaron 68 cultivos positivos, 39 en muestras de líquido pleural y 29 en hemocultivos. Encontramos que el rendimiento de los cultivos bacterianos fue mayor en los cultivos de líquido pleural en comparación con los hemocultivos, 28% vs 10% respectivamente. Los patógenos más frecuentemente aislados fueron el Staphylococcus aureus en 34 (50 %) pacientes, el Streptococcus pneumoniae en 21 (31%) pacientes y el Haemophilus influenzae en 6 (9%) pacientes en los 68 cultivos positivos. Conclusiones. El grupo de 1 ­ 4 años es el más afectado presentando 118 (43%) casos de complicaciones. El derrame pleural es la complicación más frecuente de la neumonía bacteriana adquirida en la comunidad. Se reportaron 68 cultivos positivos y el rendimiento fue mayor en los cultivos de líquido pleural en comparación con los hemocultivos. Los patógenos más frecuentemente aislados fueron el Staphylococcus aureus y el Streptococcus pneumoniae. La mayoría de los pacientes se recuperaron a pesar de la importante morbilidad. (provisto por Infomedic International)

Introduction. Complicated community-acquired pneumonia (CCAP) is characterized by severe illness, hospitalization, and a long course. The complications of pneumonia occur when the infection is not limited to the lung parenchyma, but spreads to neighboring areas, or when the development of the infection is more complex than usual for different reasons. These pulmonary complications account for only 1% to 3% of them, but this percentage increases to almost 40% if hospital admission is required, which is why it is considered a fundamentally hospital pathology.   Objective. The purpose of this study was to analyze the complications of community-acquired bacterial pneumonia and describe its epidemiological, clinical, and etiological characteristics in the study period. Material and method. This is a retrospective and descriptive study of complicated bacterial pneumonias and their characteristics at the Hospital del Niño Dr. José Renán Esquivel. A search was made of the clinical records of patients with a diagnosis of discharge from community-acquired bacterial pneumonia complicated by pleural effusion, pneumothorax, pioneumothorax, necrotizing pneumonia and lung abscess in the period from January 1, 2015 to December 31, 2020. All medical records retrieved were analyzed to obtain information on the complications of community-acquired bacterial pneumonia in relation to epidemiological data, frequency, characteristics, treatment, and clinical evolution. Results. According to the data obtained from the statistical bulletins of the Hospital Del Niño Dr. José Renán Esquivel for the period from January 1, 2015 to December 31, 2020, 2,519 bacterial pneumonias were found, corresponding to 30% of pneumonia. Of the bacterial pneumonias, 277 cases of complicated pneumonia met the inclusion criteria, representing 11% of the bacterial pneumonia. The most frequent complication, in all age groups, was parapneumonic pleural effusion with a frequency of 170 cases (61%). The average age of patients with complicated pneumonia was 51.7 months (range from 27 days to 164 months) and the 1-4-year-old group was the most affected, presenting 118 (43%) cases of complications. The average length of hospitalization was 14.8 days with a range of 9-38 days. Patients received broad-spectrum intravenous antibiotics, the most widely used initial empirical treatment being ceftriaxone plus clindamycin. The average duration of antibiotic treatment was 12.7 days with a range of 10 to 25 days and varied according to the type of complication. 68 positive cultures were reported, 39 in pleural fluid samples and 29 in blood cultures. We found that the yield of bacterial cultures was higher in pleural fluid cultures compared to blood cultures, 28% vs 10% respectively. The most frequently isolated pathogens were Staphylococcus aureus in 34 (50%) patients, Streptococcus pneumoniae in 21 (31%) patients, and Haemophilus influenzae in 6 (9%) patients in the 68 positive cultures. Conclusions. The 1­4-year-old group is the most affected, presenting 118 (43%) cases of complications. Pleural effusion is the most common complication of community-acquired bacterial pneumonia. 68 positive cultures were reported positive, and the yield was higher in pleural fluid cultures compared to blood cultures. The most frequently isolated pathogens were Staphylococcus aureus and Streptococcus pneumoniae. Most of the patients recovered despite significant morbidity. (provided by Infomedic International)

Fibrosis assessment in patients with nonalcoholic fatty liver disease: Adherence to proposed algorithms and barriers to complying with them.

INTRODUCTION AND AIMS: Fibrosis staging in patients with nonalcoholic fatty liver disease (NAFLD) is carried out through the application of stepwise algorithms but there is little real-world data on their use. Our aim was to calculate the number of patients with NAFLD and indeterminate or high risk for fibrosis, assessed through noninvasive scores, that consequently underwent further staging evaluation. MATERIALS AND METHODS: A cross-sectional multicenter cohort study was conducted on patients with NAFLD evaluated by hepatologists within the time frame of June 1 and July 31, 2018. The FIB-4 and NAFLD fibrosis scores were calculated in all the patients, and if at least one of the scores suggested indeterminate or high risk for fibrosis, we believed the patient should have undergone additional fibrosis staging assessment. RESULTS: The study included 238 patients. The median time interval from NAFLD diagnosis and inclusion in the analysis was 12.2 months (IQR 3.0-36.5). A total of 128 (54%) patients had at least one noninvasive score that suggested indeterminate or high risk for fibrosis but studies to confirm the fibrosis grade (elastography, biopsy, etc.) were performed on only 72 (56%). The main barriers encountered by the physicians for applying the staging algorithms were related to health insurance coverage and imaging study costs. CONCLUSIONS: A high percentage of patients with NAFLD were at indeterminate or high risk for fibrosis, according to noninvasive scores, but additional studies were carried out on only half of them, showing low adherence to current recommendations.

Spontaneous and deliberate creative cognition during and after psilocybin exposure.

Creativity is an essential cognitive ability linked to all areas of our everyday functioning. Thus, finding a way to enhance it is of broad interest. A large number of anecdotal reports suggest that the consumption of psychedelic drugs can enhance creative thinking; however, scientific evidence is lacking. Following a double-blind, placebo-controlled, parallel-group design, we demonstrated that psilocybin (0.17 mg/kg) induced a time- and construct-related differentiation of effects on creative thinking. Acutely, psilocybin increased ratings of (spontaneous) creative insights, while decreasing (deliberate) task-based creativity. Seven days after psilocybin, number of novel ideas increased. Furthermore, we utilized an ultrahigh field multimodal brain imaging approach, and found that acute and persisting effects were predicted by within- and between-network connectivity of the default mode network. Findings add some support to historical claims that psychedelics can influence aspects of the creative process, potentially indicating them as a tool to investigate creativity and subsequent underlying neural mechanisms. Trial NL6007; psilocybin as a tool for enhanced cognitive flexibility; https://www.trialregister.nl/trial/6007 .

Fibrosis assessment in patients with nonalcoholic fatty liver disease: Adherence to proposed algorithms and barriers to complying with them. / Evaluación de fibrosis en pacientes con enfermedad por hígado graso no alcohólico: adherencia a los algoritmos propuestos y barreras para cumplir con ellos.

INTRODUCTION AND AIMS: Fibrosis staging in patients with nonalcoholic fatty liver disease (NAFLD) is carried out through the application of stepwise algorithms but there is little real-world data on their use. Our aim was to calculate the number of patients with NAFLD and indeterminate or high risk for fibrosis, assessed through noninvasive scores, that consequently underwent further staging evaluation. MATERIALS AND METHODS: A cross-sectional multicenter cohort study was conducted on patients with NAFLD evaluated by hepatologists within the time frame of June 1 and July 31, 2018. The FIB-4 and NAFLD fibrosis scores were calculated in all the patients, and if at least one of the scores suggested indeterminate or high risk for fibrosis, we believed the patient should have undergone additional fibrosis staging assessment. RESULTS: The study included 238 patients. The median time interval from NAFLD diagnosis and inclusion in the analysis was 12.2months (IQR 3.0-36.5). A total of 128 (54%) patients had at least one noninvasive score that suggested indeterminate or high risk for fibrosis but studies to confirm the fibrosis grade (elastography, biopsy, etc.) were performed on only 72 (56%). The main barriers encountered by the physicians for applying the staging algorithms were related to health insurance coverage and imaging study costs. CONCLUSIONS: A high percentage of patients with NAFLD were at indeterminate or high risk for fibrosis, according to noninvasive scores, but additional studies were carried out on only half of them, showing low adherence to current recommendations.

Migración de fragmento de bezoar gástrico gigante como causa de obstrucción intestinal distal / Giant gastric bezoar fragment migration as a cause of distal intestinal obstruction

Introducción. Comunicar un caso clínico poco habitual y revisar el manejo. Material y Métodos. Presentación de un caso clínicoResultados. La obstrucción intestinal es una urgencia frecuente. La impactación de un bezoar es una etiología poco habitual.Cuando el bezoar intestinal asocia un bezoar gástrico se generan dudas sobre la etiología del bezoar intestinal, fragmentación y migración versus formación primaria en intestino delgado, y sobre la actitud a tomar con el bezoar gástrico, que generalmente es asintomático. Conclusiones. La enterotomía y extracción del bezoar intestinal es el tratamiento de elección. Debe asociar la extracción de los bezoares en otras localizaciones. (AU)

Introduction. Report an unusual clinical case and review the management. Material and methods. Presentation of a clinical caseResults. Bowel obstruction is a common emergency. Bezoar impaction is an unusual etiology. When the intestinal bezoar associates a gastric bezoar, doubts are raised about the etiology of the intestinal bezoar, fragmentation and migration versus primary formation in the small intestine, and about the attitude to take with the gastric bezoar, which is generally asymptomatic. Conclusions. Enterotomy and removal of the intestinal bezoar is the treatment of choice. It must associate the extraction of the bezoars in other locations. (AU)

Access to direct-acting antivirals for the treatment of hepatitis C in a country with limited resources. / Evaluación del acceso a antivirales para el tratamiento de la hepatitis C en un país con recursos limitados.

AIMS: To estimate the number of patients that have access to treatment of hepatitis C with direct-acting antivirals in Argentina and evaluate the factors associated with the lack of access. MATERIALS AND METHODS: A cross-sectional cohort study was conducted that included all the consecutive prescriptions of direct-acting antivirals issued at health centers that participated in the ECHOTM telemedicine project directed by the Hospital Italiano de Buenos Aires, within the time frame of January 2016 and February 2017. RESULTS: A total of 143 treatment prescriptions were included and overall access was 70% (95% CI 62-77%). The only independent factor associated with a lack of treatment access was coverage by a public healthcare system (OR 4.98 [95% CI 2.05- 12.09]). CONCLUSION: Patients with hepatitis C that were covered by a public healthcare system had a 4 times higher chance of not having access to treatment with direct-acting antivirals than patients covered by other healthcare systems (private insurance or the social welfare system).

Management of interruptions to fractionated radiotherapy treatments: Four and a half years of experience.

BACKGROUND AND PURPOSE: The study objective was to report our four-and-a half years experience (March 1 2011-August 15 2015) of a program to manage interruptions in fractionated radiotherapy treatments. METHODS: A program was developed, based on a specific database, to manage treatment interruptions. Benefits of the program were analyzed in reference to previously published data. Analysis was also performed of two measures to reduce OTT prolongation and improve treatment outcomes: working on public holidays and conducting treatment unit maintenance on Saturdays. RESULTS: The study included 2352 patients. Patients with head and neck cancer obtained the greatest benefit from the program, with a mean increase in TCP of 3.5% and a benefit of at least 4% in 45.6% of them. In prostate cancer patients, the likelihood of biochemical failure was reduced by a mean of 2.0% and was reduced by at least 4% in 19.7% of them. Application of the two proposed measures would have improved the TCP by a mean of 5.4% in head and neck cancer patients. The impact of the compensations program and proposed measures is lesser in the remaining cancer types studied. CONCLUSIONS: Implementation of a compensation program has a significant impact on patients with head and neck or prostate cancer but OTT prolongation remains excessive in many treatments. The introduction of fractions on public holidays would assist in the meeting of recommendations for these patients.

Diurnal variations of airborne pollen concentration and the effect of ambient temperature in three sites of Mexico City.

Pollen is an important cause of allergic respiratory ailments in the Mexico City Metropolitan Area (MCMA). However, very little is known if ambient air temperature correlates with the early blooming of plants observed in other urban areas around the world. A research study was conducted during the dry season of 2012-2013 at three representative sites of the MCMA with different urban characteristics with the aim to understand the relationships between the profusion and diversity of pollen against temperature and other meteorological variables and degree of urbanization. Pollen samples were collected using a Hirst-type trap sampler in the sites: Merced (highly urbanized), Iztapalapa (medium-high urbanized) and Coyoacan (moderately urbanized). Urbanization levels were determined using a composite index based on population density, proportion of surface covered by construction and asphalt, and urban heat island intensity. A set of representative pollen sampling tapes were assayed under a light microscope at magnification of ×1,000 and converted to grains per cubic meter. The most representative pollen types found in the three sites were, regardless of urbanization levels were: Fraxinus, Cupressaceae/Taxodiaceae, Casuarina, Alnus, Myrtaceae, and Pinus. Total pollen concentration was greatest in the moderately urbanized area, although earlier blooming took place at the highly urbanized zone. Total pollen concentration in the medium-high urbanized site has the lowest because the green areas in this zone of MCMA are few. In a diurnal basis, the most abundant pollen types peaked near midday or in the afternoon evening at the three sites. A Spearman test showed a positive correlation among bihourly pollen concentrations, temperature and relative humidity in all sites, but wind speed just correlated in Iztapalapa and Coyoacan. The results obtained suggest that Urban Heat Island Intensity can disturb flowering periods and pollen concentrations, largely in the highly urbanized areas. A principal components analysis established that the concentrations of each pollen type differed across the urbanization gradients. Additionally, it was found that a large number of allergenic pollens are produced by ornamental trees, some only recently introduced by urban planners.

[Orbital emphysema after Valsalva maneuver. Report of one case]. / Enfisema orbitario espontáneo tras la maniobra de Valsalva.

Orbital emphysema is a rare complication of orbital and facial trauma, involving the sinuses and occasionally the nasal cavity. Most cases occur as a result of trauma but spontaneous orbital emphysema has been reported. Clinical manifestations are often delayed until the upper airway pressure increases as when blowing or coughing. Along with the fracture, the rupture of the underlying mucosa is essential for emphysema to manifest. We report a 29-years-old male who consulted in the emergency room due to eyelid swelling after a Valsalva maneuver, a couple of hours after suffering a facial injury secondary to a fall. In the presence of eyelid emphysema, the patient underwent craniofacial imaging studies, confirming an ethmoidal lamina papyracea fracture.

Enfisema orbitario espontáneo tras la maniobra de Valsalva / Orbital emphysema after Valsalva maneuver. Report of one case

Orbital emphysema is a rare complication of orbital and facial trauma, involving the sinuses and occasionally the nasal cavity. Most cases occur as a result of trauma but spontaneous orbital emphysema has been reported. Clinical manifestations are often delayed until the upper airway pressure increases as when blowing or coughing. Along with the fracture, the rupture of the underlying mucosa is essential for emphysema to manifest. We report a 29-years-old male who consulted in the emergency room due to eyelid swelling after a Valsalva maneuver, a couple of hours after suffering a facial injury secondary to a fall. In the presence of eyelid emphysema, the patient underwent craniofacial imaging studies, confirming an ethmoidal lamina papyracea fracture.

Superior vena cava syndrome due to a leiomyosarcoma of the anterior mediastinum: A case report and literature overview.

INTRODUCTION: Leiomyosarcomas are an infrequent cause of malignant superior vena cava syndrome (VCS). PRESENTATION OF CASE: A 51-year old male patient was admitted for a three-day history of dyspnoea, dysphagia and erythema of the head and neck. Computed tomography and magnetic resonance imaging showed a lesion arising on the anterior mediastinum, which was in close proximity with a thrombus in the superior vena cava. Surgical excision was performed, including open resection of the primary tumour and an atrio-innominate vein bypass with 8-mm polytetrafluoroethylene (PTFE). Histology confirmed a leiomyosarcoma and postoperative radiotherapy sessions were performed. Due to evidence of enlargement of the thrombus, a second intervention was undertaken. In this procedure, a remainder of the primary tumour was resected and the superior vena cava reconstructed with an autologous pericardium patch. The patient recovered satisfactorily and was discharged on the seventh postoperative day, with no evidence for relapse after 10 months of follow-up. DISCUSSION: Leiomyosarcomas comprise less than 2% of the tumours of the mediastinum and are a rare cause of paraneoplastic VCS. Male patients in their sixties are most commonly affected. Relapses seem to be common, and thus a careful follow-up is often recommended. CONCLUSION: In spite of the limited data on the management of thoracic leiomyosarcomas, surgery is currently considered the mainstay of treatment.

Características epidemiológicas, consecuencias físicas y emocionales a corto y largo plazo en niños de 4 a 15 años con sospechas de abuso sexual. Hospital del Niño. Panamá 2009 al 2011

El abuso sexual se refiere a las conductas sexuales coercitivas , impuestas a una persona menor de edad, por otra persona, que puede ser físicamente superior, con más experiencia y recursos, que utiliza incorrectamente su poder o autoridad. Es un hecho significativo que cada día emerge con más fuerza e involucra la vulneración de los derechos humanos. Por esta razón ante el creciente interés en el tema a nivel mundial, ante las devastadoras consecuencias sobre el niño, su entorno, salud física, emocional y posterior desempeño escolar y social, hemos decidido abordarlo. Objetivo: Identificar las características sociodemográficas, consecuencias físicas en niños de 4 a 15 años con sospecha de abuso sexual, atendidos en el Hospital del Niño entre los años 2009 al 2011 y determinar si existe relación a corto y largo plazo entre la agresión y alteraciones psicoemocionales. Metodología: Se realizó un estudio en dos fases: La primera fase un estudio descriptivo retrospectivo simple y la segunda fase, analítica de seguimiento, en un periodo de 18 a 24 meses posterior a sufrir agresión sexual, de los casos referidos a los Juzgados de Niñez y Adolescencia del Distrito de Panamá, mediante la aplicación de La Escala Children's Depression Inventory y la Escala de Ansiedad de Spence. La muestra fue de 92 casos. Se analizaron los datos con Excel 2010. Resultados: El mayor número de casos de abuso sexual se encontró en la adolescencia temprana 36 %, con una media de edad de 9.6 años, encontrando un factor de asociación de ocurrencia en el ámbito extra familiar con 21 %; en donde el 79 % el agresor era conocido por las víctimas. Sólo un 45 % de los casos referidos se considero abuso según juzgados. Sólo un 26 % cumplió la ruta crítica de forma integral. Conclusiones: Encontramos la mayor afectación en el grupo de adolescencia intermedia. La agresión se realizó por un conocido de la víctima y el examen físico no fue concluyente para el dictamen de abuso. En los casos a quienes se aplicaron las herramientas de evaluación psicoemocionales se evidenció predominio de alteraciones de tipo emocionales.

The sexual abuse refers to coercive sexual behaviors imposed to a minor by another person who can be physically superior, with more experience, resources, using in a wrong way his authority. It is significant fact that every day more strongly emerges and involves the violation of human rights. For this reason at the global increase of the topic with devastating consequences for the child, their environment, physical and emotional health and subsequent school and social performance, we decide to address this issue. Objective: Identify the sociodemographic characteristics and physical consequences in children from 4 to15 years with suspected sexual abuse, attended in Hospital del Niño between years 2009-2011 and determine if there exists association in a short or long term between the aggression and psycho-emotional disorders. Methods: The study was conducted in two phases: the first one was a single retrospective study and the second phase was a follow-up analysis in a period from 18 to 24 months after the sexual attack, in those cases referred to the courts for Childhood and Adolescence Panama District, through the application of Children's Depression Inventory Scale and the Spence Anxiety Scale. The sample was 92 cases. Data were analyzed with excel 2010. Results: The most cases of child sexual abuse were in the middle adolescence (36%), with a mean age of 9.6 years. We found association of occurrence in the extra-familiar environment with 21%, where the 79% of the aggressors were known people from the victim. 45% of the cases were considered sexual abuse by the Tribunals. Just 26% complete the entire process (Critical Route). The psycho-emotional disturbances were seen in a short term with 16% in adolescents. Conclusions: the most affected group was the middle adolescence. The attack was done by a known person of the victim and the physical examination was not conclusive to determine sexual abuse. In the cases evaluated with the anxiety and depression scales the most resulted in emotional disturbances.

Infecciones de piel y oídos ¿Histiocitosis? Revisión de casos en el Hospital del Niño años 2002-2012

La histiocitosis de células de Langerhans (HCL) es un desorden proliferativo, raro, caracterizado por la infiltración y acumulación patológica de células de Langerhans en diversos órganos. Durante años han existido diversas clasificaciones basadas inicialmente en sus características clínicas y actualmente en el tipo de célula predominante en el infiltrado del tejido afectado. Aunque se ha demostrado que representa una respuesta proliferativa clonal, no es claro aún si la respuesta celular representa un fenómeno reactivo o neoplásico.Su diagnóstico es clínico-patológico, basado en los hallazgos clínicos y criterios inmunohistoquímicos. Objetivo: Conocer las características de los casos de Histiocitosis diagnosticados en el Hospital del Niño en los últimos 11 años y resaltar los hallazgos clínicos para sospechar su diagnóstico. Material y Métodos: Revisión de historias clínicas con diagnóstico de Histiocitosis en el Hospital del Niño, años 2002-2012. Resultados: Se obtuvo un total de 36 casos con diagnóstico de HCL en los últimos 11 años. Con un predominio del sexo femenino (67%). De los 36 casos se revisaron un total de 26, los cuales en su mayoría corresponden a la variedad granuloma eosinófilo, con mayor afectación de huesos de cráneo. Como manifestación inicial más común se encontró la hepatomegalia, con predominio de inicio de síntomas en menores de 1 año. Conclusiones: A pesar de ser un diagnóstico poco frecuente, debemos tenerlo en cuenta durante nuestra evaluación clínica como diagnóstico diferencial

Langerhans cell histiocytosis is rare proliferative disorder, characterized by the infiltration and accumulation of pathological Langerhan's cell in different organs. For many years there existed different classifications initially based on the clinical manifestations and actually are based on the predominant cellular type infiltrate affecting a specific tissue. Although there is demonstrated that represents a clonal proliferative response, there is no evidence if the cellular response is due to a reactive or a neoplasic phenomenon. The diagnostic is clinical-pathological, based on the clinical and immunohistochemical findings. Objective: To know the characteristics of Histiocytosis cases reported in Hospital del Niño, in the last 11 years and highlight the clinical findings to suspect it. Material and Methods: review of clinical records reported as Histiocytosis in Hospital del Niño, between years 2002-2012. Results: 36 cases were reported as Langerhans cell histiocytosis in the last 11 years. Although the literature reported a major incidence on males, we found female predominance (67%). The most of the cases were reported as eosinophilic granuloma, mostly affecting skull bones. The main initial manifestation reported was hepatomegaly, most of them initiating before the first year of life. Conclusions: Although it's an infrequent diagnostic, we should consider it in our clinical evaluations as a differential diagnostic.

Prevalencia de asma en menores de 6 años en el distrito de Panamá. 2012

Objetivos. Determinar la prevalencia del asma en la población menor de seis años del distrito de Panamá. Determinar la prevalencia específica del asma en el grupo de edad de 0-23 meses y de 2-5 años. Conocer la prevalencia del asma activo en los grupos de estudio y determinar la frecuencia de factores de riesgo que puedan estar relacionados con la enfermedad. Obtener la frecuencia de rinitis alérgica y dermatitis atópica relacionadas con el asma bronquial y asma activo en el grupo de estudio. Material y método. Estudio de prevalencia, analítico observacional, de tipo transversal de base poblacional que se realizó en los Centros de Orientación Infantil del distrito de Panamá mediante la aplicación de una encuesta. Participantes. Padres de los niños menores de 6 años que asisten a los Centros de Orientación Infantil del distrito de Panamá y que firmaron el consentimiento informado. Análisis estadístico. Los datos fueron ingresados al programa SPSS versión 16.0 y se midió la frecuencia, porcentaje, media, desviación estándar y odds radio, se tabularon y representaron en gráficas. Resultados. Contestaron la encuesta y firmaron el consentimiento informado 620 padres de los niños menores de seis años que asisten a los Centros de Orientación Infantil del distrito de Panamá. Se eliminaron 23 encuestas con datos incompletos y se analizaron los datos de 597 encuestas. La edad promedio de los niños fue 45.58 ±14.56 meses. Se estableció el diagnóstico de asma en 162 niños obteniéndose una prevalencia de 27.1%. Cien de los 162 niños asmáticos habían tenido síntomas en los 12 meses previos lo que representó una prevalencia de asma activo de 61.7%, 30% de ellos tenían rinitis alérgica, 23% dermatitis atópica y 10% tenían asma activo, rinitis alérgica y dermatitis atópica. Conclusiones. Las prevalencias de asma y de asma activo en los niños menores de seis años que acuden a los centros de orientación estudiados del distrito de Panamá son altas.

Objectives. To determine the prevalence of asthma in the population under six years of the district of Panama. To determine the specific prevalence of asthma in the age group 0-23 months and 2-5 years. To determine the prevalence of active asthma in the study groups and determine the frequency of risk factors that may be related to the disease. Get the frequency of allergic rhinitis and atopic dermatitis associated with bronchial asthma and active asthma in the study group. Material and methods. Prevalence study, an observational, cross-sectional population-based was conducted in the Child Guidance Centers in the district of Panama through the application of a survey. Subjects. Parents of children under 6 years attending the Child Guidance Centers in the district of Panama and signed informed consent. Statistical analysis. Data were entered into SPSS version 16.0 and measured the frequency, percentage, mean, standard deviation and odds ratio were tabulated and represented in graphs. Results. Responses were received and signed informed consent 620 parents of children under six years attending the Child Guidance Centers in the district of Panama. 23 surveys were eliminated with incomplete data and analyzed data from 597 surveys. The average age of the children was 45.58 ±14.56 months. The diagnosis of asthma in 162 children with a prevalence of 27.1%. One hundred of the 162 children had asthma symptoms in the previous 12 months which represented a prevalence of active asthma 61.7%, 30% of them had allergic rhinitis, atopic dermatitis 23% and 10% had active asthma, allergic rhinitis and atopic dermatitis. Conclusions. The prevalence of asthma and active asthma are high in children under six years of age who attend the counseling centers surveyed in the district of Panama.

Estenosis hipertrofia del píloro: Mitos y verdades

Introducción: La estenosis hipertrofia de piloro es una patología frecuente en edades tempranas. Afecta recién nacidos y lactantes pequeños . Algunos factores de riesgo son sexo masculino, grupo sanguíneos O positivo, uso de macrólidos en embarazo. El diagnóstico se realiza con una adecuada historia clínica y exploración física. Se describen síntomas como vómitos persistentes , pérdida de peso, deshidratación. El diagnóstico se confirma mediante ultrasonografía. El tratamiento en su gran mayoría es quirúrgico, siendo el pronóstico satisfactorio. Objetivo: Describir las principales características epidemiológicas , clínicas , laboratorio, diagnóstico y tratamiento y posibles complicaciones de estos pacientes. Materiales y método: Estudio retrospectivo que incluyó todos los pacientes egresados con diagnóstico de estenosis hipertrófica de píloro, desde el periodo 2005 al 30 de junio 2010 atendidos en el Hospital del Niño. Se recogieron datos mediante formulario de informe prediseñado. Resultados: Se incluyeron 53 pacientes con una media de edad de 5.2 semanas. Se encontraron en su mayoría pacientes de sexo masculino. Las principales manifestaciones clínicas fueron vómitos, disminución de peso, deshidratación. El examen diagnóstico de elección fue el ultrasonido. Todos los pacientes fueron manejado de forma quirúrgica . No se reportaron complicaciones en los pacientes. Discusión: Es una de las principales causas de cirugía en niños pequeños. El avance en el conocimiento de las manifestaciones clínicas llevan a un diagnóstico eficaz y temprano. La historia clínica y la exploración física son la herramienta principal en el diagnóstico ayudado con herramientas tecnológicas como la ultrasonografía por su alta sensibilidad, especificidad y bajo costo. El tratamiento quirúrgico es curativo con complicaciones infrecuentes y buen pronóstico a largo plazo.

Introduction: Hypertrophic pyloric stenosis is a common disease at early ages. It affects newborns and infants. Some risk factors are male sex, blood group O positive, macrolide use in pregnancy. The diagnosis is made with appropriate history and physical examination. We describe symptoms such as vomiting, weight loss dehydration. The diagnosis is confirmed by ultrasonography. Treatment is mostly surgical prognosis remains unsatisfactory. Objetive: To describe the main epidemiological , clinical , laboratory, diagnostic, treatment and possible complications in these patients. Materials and methods: Retrospective study included all patients discharged with the diagnosis of hypertrophic pyloric stenosis, from the period 2005 to June 30, 2010 treated at the Hospital del Niño. Data were collected using pre-designed report form. Results: 53 patients with a mean age of 5.2 weeks. Found mostly male patients. The main clinical manifestations were vomiting, weight loss, dehydration. The diagnostic test of choice was the ultrasound. All patients were managed surgically. There were no complications in patients. Discussion: It is a major cause of surgery in young children. The progress of knowledge in understanding the clinical findings lead to effective early diagnosis. The clinical history and physical examination is the main tool n the diagnosis helped with technological tools such as ultrasound for high sensitivity, specificity and low cost. Surgical treatment is curative with rare complications and good long-term prognosis.

Choque séptico por Pseudomonas aeruginosa en paciente con varicela. Presentación de caso clínico

Paciente masculino de 3 meses de edad que presenta clínica sugestiva de varicela, inicialmente tratada de forma ambulatoria con antihistamínicos y antibioticoterapia. Ante empeoramiento clínico y sospecha de cuadro séptico, se decide ingreso hospitalario. A pesar del manejo adecuado fallece, aislándose en hemocultivo y cultivo de heridas. Pseudomonas aeruginosa.

A case of a male patient, 3 months old with, who presented clinical manifestations suggestive of chickenpox. Initially treated on an outpatient basis with antihistamines and antibiotics. Face with worsening clinical suspicion of sepsis, hospital admission is decided. Despite appropriate management dies and Pseudomonas aeruginosa isolated in blood cultures and wound secretion culture.

Ten-year results of a phase II study with a single fraction of high-dose-rate brachytherapy (FAST-boost) after whole breast irradiation in invasive breast carcinoma

PURPOSE: We present the results of a prospective ten-year follow-up study to prove the effectiveness of a single fraction of 192-Ir high-dose-rate (HDR) brachytherapy (BT) as a boost. PATIENTS AND METHODS: Between 1999 and 2000, 84 consecutive patients with invasive breast carcinoma, with over 4 mm free margins after conservative surgery, were treated. All cases were stages T1-2, except for one case, a stage T3, 81% pN0, 19% pN1-2. Chemotherapy was used in 47% and hormonal therapy in 87%. Whole breast external beam radiotherapy (46 Gy) was followed 1-2 weeks later by an implant with metallic needles. A 7 Gy single dose of HDR BT to the 90% isodose line was delivered on an outpatient basis. Dosimetry was performed theoretically. This technique is called FAST-boost because the whole treatment is delivered in about two hours. RESULTS: With a median follow-up of 120 months, three patients relapsed in the margin of the implant and two in a different quadrant (5/84, 6%). Actuarial local control at five and ten years was 98.5% and 95.6%. Overall survival was 92.7% and 90.2%, and disease-free survival 90.2% and 79.9%. Cosmetic results were good or excellent in 92.5%. CONCLUSIONS: A single-fraction HDR boost with rigid needles (FAST-boost) is a good, quick, simple technique when surgical margins are free (AU)

Mal de Pott: a propósito de un caso

La tuberculosis continúa siendo un problema de salud pública a pesar de los múltiples esfuerzos realizados para su control. En Panamá, aunque con menor incidencia que en el adulto, se siguen presentando casos de tuberculosis infantil. Los datos son inespecíficos y existe baja probabilidad de recuperar el bacilo , por lo que el diagnóstico resulta difícil y debe basarse en una alta sospecha diagnóstica y nexo epidemiológico.

Tuberculosis remains a public health problem despite many efforts to control it. In Panama, although to a lesser extend than in adults, there continue to be cases of childhood tuberculosis. Clinical data are nonspecific and there is low probability of recovering the bacillus, so diagnosis is difficult and based on high diagnostic suspicion and epidemiological link.

Síndrome hemofagocítico asociado a infección aguda por Citomegalovirus: Presentación de caso clínico

Se presenta caso de paciente femenina de etnia china que ingresó al Hospital del Niño con historia de 8 días de evolución con cuadro caracterizado inicialmente pro fiebre mayor de 39ºC sin predominio horario y posteriormente presentó ictericia escleral y de tegumentos asociado a la presencia de acopia, colaría y aumento de perímetro abdominal. Fue tratada inicialmente de forma ambulatoria con tratamiento sintomático sin mejoría por lo cual fue referida al Hospital del Niño. Durante su hospitalización continúo con fiebre hasta 40ºC, desarrollo exantema maculo papular en cara y tronco, asociado a la presencia de adenopatías cervicales y hepatOesplenomegalia. En los laboratorios realizados durante su hospitalización se encontró alteración de las pruebas de función hepática, leucopenia, anemia y trombocitopenia. Las pruebas serológicas pro dengue, hepatitis, Epstein Barr virus , VIH y toxoplasmosis resultaron negativas, la serología para Citomegalovirus (CMV) positiva. En las pruebas químicas se encontró elevación de triglicéridos y enzimas hepáticas, niveles serios de ferrita elevadas, IgA, M y G disminuidas. Se le realizó aspirado de médula ósea que demostró la presencia de fagocitosis. Se administraron antipiréticos, hidratación y coba lamina por la anemia macrocítica, cursando la paciente con buena evolución clínica. descenso de la fiebre y normalización de bromearía hemática.

We describe a case of chinese female patient that was admitted to Hospital del Niño with 8 days of fever (over 39ºC) without time predominance and subsequently icteric sclera, acholia, coluric urine and increase of abdominal volume. Treated initially as an outpatient with symptomatic without improvement by which was referred to our institution. During hospitalization she continued with fever up to 40ºC, developing maculo papular rash in face and trunk, associated with cervical adenopathy and hepatosplenomegaly. Alteration of liver function, leukopenia, anemia and thrombocytopenia were found during laboratories. Serology for dengue, viral hepatitis, Epstein-Barr virus, HIV and toxoplasmosis were negative, serology for cytomegalovirus (CMV) was positive. Serum analysis revealed hypertransaminasemia, hypertriglyceridemia,and ferritin high levels, immunoglobulin A, M and G were low. Bone marrow aspiration showed the presence of phagocytosis. She received symptomatic treatment with antipyretics, hydration and cobalamin because of macrocytic anemia. She had good clinical evolution, fever decrease and standardization of hematologic markers.