Effects of a dual-task activity on gait parameters of people with and without intellectual disabilities.

BACKGROUND: The main objective of this study was to evaluate gait parameters in people with intellectual disability (ID) and without intellectual disability (WID) in two different walking conditions [single task vs. dual task (DT)]. A secondary aim was to evaluate the dual-task cost (DTC) that the DT causes in each group. METHODS: A total of 119 participants joined in this study: 56 ID (30 men) and 63 WID (30 men). The OptoGait system was used to assess gait. In addition, Witty photocells were added to assess gait under the DT condition. RESULTS: Single support time was lower for participants with ID (P < 0.01), while double support time was higher (P < 0.05). All coefficients of variation for gait parameters were higher in participants with ID. Additionally, changes in gait were observed in both groups during the DT condition compared with the single-task condition. These changes were larger for participants with ID in step length, double support time and gait speed (P < 0.001), resulting in a higher DTC in these variables in the ID group (P < 0.01). CONCLUSIONS: Both groups reduced gait performance in the DT condition. However, greater gait variability occurred in the ID group. In addition, DTC was higher for the ID group in all variables analysed. Therefore, people with ID show worse gait performance during a DT than people WID.

Validation and comparison of Breast Graded Prognostic Assessment scores in patients with breast cancer and brain metastases

Validation and comparison of Breast Graded Prognostic Assessment scores in patients with breast cancer and brain metastases (AU)

Validation and comparison of Breast Graded Prognostic Assessment scores in patients with breast cancer and brain metastases.

PURPOSE: Brain metastases (BM) occur in 15-35% of patients with metastatic breast cancer, conferring poor prognosis and impairing quality of life. Clinical scores have been developed to classify patients according to their prognosis. We aimed to check the utility of the Breast Graded Prognostic Assessment (B-GPA) and its modified version (mB-GPA) and compare them in routine clinical practice. METHODS: This is an ambispective study including all patients with breast cancer BM treated in a single cancer comprehensive center. We analyzed the overall survival (OS) from BM diagnosis until death. The Kaplan-Meier method and Cox proportional hazard regression model were used in the analyses. ROC curves were performed to compare both scores. RESULTS: We included 169 patients; median age was 50 years. HER2-positive and triple negative patients were 33.7% and 20.7%, respectively. At the last follow-up, 90% of the patients had died. Median OS was 12 months (95% confidence interval 8.0-16.0 months). OS was worse in patients with > 3 BM and in patients with triple negative subtype. CONCLUSIONS: In our series, we confirm that B-GPA and mB-GPA scores correlated with prognosis. ROC curves showed that B-GPA and mB-GPA have similar prognostic capabilities, slightly in favor of mB-GPA.

Evaluación del proceso de atención urológica de pacientes pediátricos con mielomeningocele / Evaluation of the urological care process of pediatric patients with myelomeningocele

Introducción: El mielomeningocele (MMC) es una de las malformaciones congénitas más severas compatible con la vida. El 90% de los pacientes presenta vejiga neurogénica que debe ser evaluada y tratada precozmente. Objetivos: Describir la evaluación y tratamiento nefrourológico recibido por pacientes con MMC hasta el momento de la primera consulta en el Hospital Garrahan (periodo pre-ingreso). Describir la evaluación realizada y el tratamiento urológico implementado a partir del ingreso al hospital Garrahan (periodo post-ingreso). Evaluar la prevalencia de Enfermedad Renal Crónica (ERC). Población y Métodos: Se realizó un estudio con diseño clínico analítico, retrospectivo, longitudinal sobre pacientes con MMC de 1 mes a 18 años derivados al Hospital Garrahan para atención ambulatoria en los años 2011 y 2012. Resultados: Se incluyeron115 pacientes. Al momento de la derivación al hospital ("pre-ingreso") 7% de los pacientes habían logrado completar evaluación nefrourológica, (ecografía vesicorenal, urodinamia, Cistouretrografía, Centellograma renal y Creatininemia). Tratamiento: 33% vaciaban vejiga por CIL o vesicostomía y 21% recibían Oxibutinina. A partir del ingreso al seguimiento en el Garrahan 83% lograron completar la evaluación, y en función del resultado de la misma se indicó CIL en 87% y Oxibutinina en el 66% de los pacientes. La prevalencia de ERC al ingreso fue de 43%; la mayoría en estadio I. Conclusiones: La mayoría de los pacientes con MMC fueron derivados al hospital de tercer nivel con evaluaciones urológicas incompletas y sin el tratamiento adecuado de la vejiga neurogénica. El inicio del seguimiento interdisciplinario en un hospital de alta complejidad facilitó la realización de las evaluaciones necesarias y la implementación del tratamiento adecuado (AU)

Introduction: Myelomeningocele (MMC) is one of the most severe congenital malformations compatible with life. Of all the patients, 90% presents with a neurogenic bladder requiring early evaluation and treatment. Objectives: To describe the uronephrological evaluation and treatment received by patients with MMC up to the first consultation at Garrahan Hospital (pre-follow-up period). To describe the urological evaluation and treatment implemented from referral to Garrahan Hospital (follow-up period). To evaluate the prevalence of chronic kidney disease (CKD). Population and Methods: A retrospective, longitudinal study with a clinical, analytical design was conducted in patients with MMC between 1 months and 18 years of age referred to Garrahan Hospital for outpatient care in 2011 and 2012. Results: 115 patients were included. At the time of referral to the hospital ("pre-follow-up") 7% of the patients had undergone complete uronephrological evaluation (kidney-bladder ultrasonography, urodynamic studies, cystourethrography, renal scintigraphy, and creatininemia levels). Treatment: 33% emptied their bladder by CIC or vesicostomy and 21% received oxybutynin. From follow-up initiation at Garrahan Hospital, 83% underwent complete evaluation, and based on the results CIC was indicated in 87% and oxybutynin in 66% of the patients. On admission, prevalence of CKD was 43%; with stage I in the majority of the patients. Conclusions: The majority of the patients with MMC were referred to a third-level hospital with incomplete urological studies and without adequate treatment of the neurogenic bladder. Initiation of interdisciplinary follow-up at a tertiary hospital allowed for the necessary studies and implementation of adequate treatment (AU)

Bypass cerebro vascular para manejo de aneurisma cerebral gigante: reporte de primer caso realizado en Bolivia / Cerebrovascular bypass for management of giant cerebral aneurysm: report of the first case carried out in Bolivia

Se presenta un caso del primer Bypass cerebro vascular realizado en Bolivia por un aneurisma gigante de arteria carotida interna derecha, en segmento cavernoso tratado mediante bypass de alto flujo de arteria carótida externa a arteria cerebral media derecha, con injerto de arteria radial y posterior oclusión y exclusión del aneurisma. Se desciben los pasos de la cirugia y se destacan las dificultades de la tecnica y los beneficios de la cirugia. SE discuten los elementos a considerar en la toma de decisiones para la indicación y realización del bypass en el tratamiento de los aneurismas gigantes de la ACI.

Pupil inversion Mach-Zehnder interferometry for diffraction-limited optical astronomical imaging.

We present theoretical and laboratory experimental results on a robust interferometric device based on pupil inversion, or 180° rotational shearing interferometry. The image of an astronomical object degraded by the atmosphere turbulence can be restored (ideally up to the diffraction limit) by a numerical post-processing of the interferogram. Unlike previous Michelson configurations that return half of the light to the sky, the Mach-Zehnder interferometer has no fundamental losses when both outputs are used. The interferogram is formed by two overlapped images of the telescope pupil, but one of them is spatially inverted, and out of phase by π/2 only in its half. This optical operation is achieved in a robust way by inserting a refractive optical image inverter and a binary phase plate in one of the arms of the interferometer. In this way, the system has no polarization dependence or moving parts since the plate allows the object to be retrieved numerically from just one interferogram (single exposition) or a few independent interferograms. For that, several algorithms are proposed. Likewise, we include a laboratory proof-of-concept in which a diffraction-limited image is obtained in spite of presence of aberrations and photon noise.

Lung recruitment maneuvers: opening the door to a hidden enemy. / Maniobras de reclutamiento pulmonar: abriendo la puerta a un enemigo oculto.

Recruitment manoeuvres (RM) are common practice in anaesthesiology; however, they can have adverse effects. We present an unforeseen complication in a patient undergoing surgical resection of a bronchial tumour who presented cardiac arrest due to pulseless electrical activity immediately after RMs. A transoesophageal echocardiogram performed after return of spontaneous circulation showed a patent foramen ovale (PFO), left ventricular dysfunction with segmental changes, and air in the left ventricle, leading to suspicion of paradoxical air embolism. The contractility changes normalised spontaneously, and postoperative evolution was uneventful. RMs cause changes in intracavitary pressures that can lead to opening of a PFO (present in up to 30% of the population) and reversal of the physiological left-right shunt. Transoesophageal echocardiography facilitated immediate diagnosis and follow-up.

How the alternating degeneracy in rotational Raman spectra of CO2 and C2H2 reveals the vibrational temperature.

The contribution of higher vibrational levels to the rotational spectrum of linear polyatomic molecules with a center of symmetry (CO2 and C2H2) is assessed. An apparent nuclear degeneracy is analytically formulated by vibrational averaging and compared to numerical averaging over vibrational levels. It enables inferring the vibrational temperature of the bending and asymmetric stretching modes from the ratio of even to odd peaks in the rotational Raman spectrum. The contribution from higher vibrational levels is already observable at room temperature as g˜e/o=0.96/0.04 for CO2 and g˜e/o=1.16/2.84 for C2H2. The use of the apparent degeneracy to account for higher vibrational levels is demonstrated on spectra measured for a CO2 microwave plasma in the temperature range of 300-3500 K, and shown to be valid up to 1500 K.

Orchid seed removal by ants in Neotropical ant-gardens.

Most plants that inhabit ant-gardens (AGs) are cultivated by the ants. Some orchids occur in AGs; however, it is not known whether their seeds are dispersed by AG ants because most orchid seeds are tiny and dispersed by wind. We performed in situ seed removal experiments, in which we simultaneously provided Azteca gnava ants with seeds of three AG orchid species and three other AG epiphyte species (Bromeliaceae, Cactaceae and Gesneriaceae), as well as the non-AG orchid Catasetum integerrimum. The seeds most removed were those of the bromeliad Aechmea tillandsioides and the gesneriad Codonanthe uleana, while seeds of AG orchids Coryanthes picturata, Epidendrum flexuosum and Epidendrum pachyrachis were less removed. The non-AG orchid was not removed. Removal values were positively correlated with the frequency of the AG epiphytes in the AGs, and seeds of AG orchids were larger than those of non-AG orchids, which should favour myrmecochory. Our data show that Azt. gnava ants discriminate and preferentially remove seeds of the AG epiphytes. We report for the first time the removal of AG orchid seeds by AG ants in Neotropical AGs.

Intratumoural evolutionary landscape of high-risk prostate cancer: the PROGENY study of genomic and immune parameters.

BACKGROUND: Intratumoural heterogeneity (ITH) is well recognised in prostate cancer (PC), but its role in high-risk disease is uncertain. A prospective, single-arm, translational study using targeted multiregion prostate biopsies was carried out to study genomic and T-cell ITH in clinically high-risk PC aiming to identify drivers and potential therapeutic strategies. PATIENTS AND METHODS: Forty-nine men with elevated prostate-specific antigen and multiparametric-magnetic resonance imaging detected PC underwent image-guided multiregion transperineal biopsy. Seventy-nine tumour regions from 25 patients with PC underwent sequencing, analysis of mutations, copy number and neoepitopes combined with tumour infiltrating T-cell subset quantification. RESULTS: We demonstrated extensive somatic nucleotide variation and somatic copy number alteration heterogeneity in high-risk PC. Overall, the mutational burden was low (0.93/Megabase), but two patients had hypermutation, with loss of mismatch repair (MMR) proteins, MSH2 and MSH6. Somatic copy number alteration burden was higher in patients with metastatic hormone-naive PC (mHNPC) than in those with high-risk localised PC (hrlPC), independent of Gleason grade. Mutations were rarely ubiquitous and mutational frequencies were similar for mHNPC and hrlPC patients. Enrichment of focal 3q26.2 and 3q21.3, regions containing putative metastasis drivers, was seen in mHNPC patients. We found evidence of parallel evolution with three separate clones containing activating mutations of ß-catenin in a single patient. We demonstrated extensive intratumoural and intertumoural T-cell heterogeneity and high inflammatory infiltrate in the MMR-deficient (MMRD) patients and the patient with parallel evolution of ß-catenin. Analysis of all patients with activating Wnt/ß-catenin mutations demonstrated a low CD8+/FOXP3+ ratio, a potential surrogate marker of immune evasion. CONCLUSIONS: The PROGENY (PROstate cancer GENomic heterogeneitY) study provides a diagnostic platform suitable for studying tumour ITH. Genetic aberrations in clinically high-risk PC are associated with altered patterns of immune infiltrate in tumours. Activating mutations of Wnt/ß-catenin signalling pathway or MMRD could be considered as potential biomarkers for immunomodulation therapies. CLINICAL TRIALS.GOV IDENTIFIER: NCT02022371.

Polymicrobial pneumococcal bacteraemia: a case-control study.

Polymicrobial bacteraemia involving Streptococcus pneumoniae and other bacteria (e.g. Escherichia coli, Staphylococcus aureus, Pseudomonas aeruginosa, Haemophilus influenza, viridans streptococci, Salmonella spp.) occurred in 3.4% of our pneumococcal bacteraemia cases. Compared with 308 controls (monomicrobial bacteraemia), the 77 polymicrobial cases included more males (83 vs 62%, p = 0.001), had serious underlying diseases (100 vs 80%, p < 0.001), abdominal infection (18 vs 5%, p < 0.001), nosocomial infection (33 vs 8%, p < 0.001), shock (40 vs 13%, p < 0.001), and higher mortality (52 vs 18%, p < 0.001). Clinicians must be aware that some patients with pneumococcal bacteraemia may have other bacteria in their blood, which would confer higher mortality and may lead to inappropriate or incomplete antibiotic therapy.

Acoso laboral entre personal de enfermería / Mobbing among nursing staff / Assédio laboral entre trabalhadores de enfermagem

Objetivo Analizar los trabajos, sobre el acoso entre profesionales de enfermería: prevalencia, factores de riesgo y consecuencias. Método: Revisión sistemática de las publicaciones científicas sobre el acoso entre el personal de enfermería, en la última década. Se consultaron diferentes bases de datos nacionales (Dialnet, ISOC Ciencias Sociales y Humanidades, IME Biomedicina) e internacionales (CINHAL, Medline, PsycINFO, PubMed Central), así como directorios de revistas con acceso abierto (DOAJ). En todos los casos, se limitó la búsqueda al intervalo 2005-2014. Los criterios establecidos fueron: 1) estudios que analicen el acoso laboral entre el personal de enfermería; 2) cualquier diseño metodológico; 3) que aporten datos empíricos sobre el estado de la cuestión; 4) con acceso al texto completo; y 5) escritos en inglés, español o portugués. De un total de 96 publicaciones, fueron seleccionadas 18. Resultados: Los resultados muestran una prevalencia de acoso, entre el personal de enfermería, en torno al 17-20%. Se identifican factores de riesgo como: edad inferior a 30 años, corta trayectoria profesional, y trabajar en determinadas unidades o turnos. Las consecuencias para la víctima fueron: problemas psicológicos, bajo rendimiento y deterioro de relaciones sociales. Conclusiones: Destacar la escasa producción científica sobre el tema y la necesidad de desarrollar instrumentos, que permitan una evaluación ajustada de las conductas de intimidación y acoso, entre profesionales de la enfermería.

Objective To analyze studies on mobbing among nursing professionals, risk factors, and consequences. Method: This is a systematic review of scientific works on mobbing among nursing staff published during the last decade (2005-2014). National (Dialnet, ISOC Ciencias Sociales y Humanidades, IME Biomedicina) and international (CINHAL, Medline, PsycINFO, PubMed Central) databases, as well as directories of open access journals were consulted. Established criteria were: 1) studies analyzing mobbing among nursing staff; 2) any methodological design; 3) offering empirical data on the topic; 4) access to the full text; 5) written in English, Spanish, or Portuguese. From a total of 96 studies identified, 18 were finally selected. Results: The findings show a prevalence of mobbing among nursing staff of around 17-20%. Risk factors such as age younger than 30 years old, brief professional trajectory, and being working in specific units or shifts were identified. Among the consequences, psychological problems, low productivity, and social relations deteriorations were identified. Conclusions: It is important to highlight the scanty scientific production on the topic, and also the necessity to develop instruments which allow an adequate assessment of harassment conducts among nursing professionals.

Objetivo Analisar os trabalhos sobre o assédio entre profissionais de enfermagem: prevalência, fatores de risco e consequências. Método: Revisão sistemática das publicações científicas sobre o assédio entre os trabalhadores de enfermagem na última década. Consultaram-se diferentes bases de dados nacionais (Dialnet ISOC Ciencias Sociales y Humanidades, IME Biomedicina) e internacionais (CINHAL, Medline, PsycINFO, PubMed Central), assim como diretórios de revistas com livre acesso (DOAJ). Em todos os casos, limitou-se à busca do intervalo de 2005-2014. Os critérios estabelecidos foram: 1) estudos que analisem o assédio laboral entre os trabalhadores de enfermagem, 2) qualquer desenho metodológico, 3) que aportem dados empíricos sobre o estado da questão, 4) com acesso ao texto completo, e, 5) escritos em inglês, espanhol e português. De um total de 96 publicações, foram escolhidas 18. Resultados: Os resultados mostram uma prevalência de assédio, entre os trabalhadores de enfermagem, em volta a 17-20%. Identificam-se fatores de risco como: idade inferior a 30 anos, curta trajetória professional, e trabalhar em determinadas unidades ou turnos. As consequências para a vítima foram: problemas psicológicos, baixo rendimento e deterioro de relações sociais. Conclusões: Destacar a escassa produção científica de enfermagem sobre o tema, e a necessidade de desenvolver instrumentos, que permitam uma avaliação ajustada das condutas de intimidação e assédio, entre profissionais da enfermagem.

Hypervirulent Klebsiella pneumoniae clones causing bacteraemia in adults in a teaching hospital in Barcelona, Spain (2007-2013).

Virulent hypermucoviscous Klebsiella pneumoniae strains associated with the magA and rmpA genes have mainly emerged in Asia. We analysed the frequency and the clinical and molecular epidemiology of K. pneumoniae bacteraemia isolates obtained over a 7-year period (2007-2013). Fifty-three of 878 K. pneumoniae invasive isolates (5.4%) showed a hypermucoviscous phenotype (by the string test). Of these, 16 (30.2%) were magA(+)/rmpA(+), 12 (22.6%) were magA(-)/rmpA(+), and the remaining 25 (47.2%) were magA(-)/rmpA(-). After multilocus sequence typing and wzi sequencing, all magA(+)/rmpA(+) isolates were serotype K1 and sequence type (ST)23. Of the 12 magA(-)/rmpA(+) isolates, nine were K2 (ST380, ST86, ST65, ST25 and ST493), and three magA(-)/rmpA(+) isolates had the new wzi allele 122, an unknown serotype, and the new ST1013. The remaining isolates, which were magA(-)/rmpA(-), showed different serotypes and STs. Patients with magA(+)/rmpA(+) or magA(-)/rmpA(+)K. pneumoniae bacteraemia more frequently had pyogenic liver abscesses (PLAs) and pneumonia than patients with magA(-)/rmpA(-)K. pneumoniae bacteraemia (respectively: 21.4% vs. 8%, p 0.26; and 17.9% vs. 0%, p 0.05). In fact, magA(-)/rmpA(-) isolates were similar to the those termed 'classic' K. pneumoniae isolates causing bacteraemia, the urinary and biliary tracts being the main foci of infection. In conclusion, hypervirulent clones (CC23K1, CC86K2, CC65K2, and CC380K2) were infrequent among K. pneumoniae isolates causing bacteraemia in our geographical area. A hypermucoviscous phenotype as determined with the string test is not enough to recognize these clones; additional molecular studies are needed. Patients with magA(+) and/or rmpA(+)K. pneumoniae bacteraemia more frequently had PLAs and pneumonia than patients without hypermucoviscosity genes.

Effects of nanocrystalline hydroxyapatites on macrophage polarization.

Silicon substituted and nanocrystalline hydroxyapatites have attracted the attention of many researchers due to their up-regulation in osteoblast cell metabolism and enhanced bioreactivity, respectively. On the other hand, the biomaterial success or failure depends ultimately on the immune response triggered after its implantation. Macrophages are the main components of the innate immune system with an important role in healing and tissue remodelling due to their remarkable functional plasticity, existing in a whole spectrum of functional populations with varying phenotypic features. The effects of nanocrystalline hydroxyapatite (nano-HA) and nanocrystalline silicon substituted hydroxyapatite (nano-SiHA) on the macrophage populations defined as pro-inflammatory (M1) and reparative (M2) phenotypes have been evaluated in the present study using RAW 264.7 cells and mouse peritoneal macrophages as in vitro models. M1 and M2 macrophage phenotypes were characterized by flow cytometry and confocal microscopy by the expression of CD80 and CD163, known as M1 and M2 markers, respectively. The polarization of primary macrophages towards the M1 or M2 phenotype was induced with the pro-inflammatory stimulus LPS or the anti-inflammatory stimulus IL-10, respectively, evaluating the biomaterial effects under these conditions. Our results show that both nano-HA and nano-SiHA favour the macrophage polarization towards an M2 reparative phenotype, decreasing M1 population and ensuring an appropriate response in the implantation site of these biomaterials designed for bone repair and bone tissue engineering.

Posterior tibial slope accuracy with patient-specific cutting guides during total knee arthroplasty: A preliminary study of 50 cases.

BACKGROUND: Patient-specific cutting guides were recently introduced to facilitate total knee arthroplasty (TKA). Their accuracy in achieving optimal implant alignment remains controversial. The objective of this study was to evaluate postoperative radiographic outcomes of 50 TKA procedures with special attention to posterior tibial slope (PTS), which is difficult to control intraoperatively. We hypothesized that patient-specific cutting guides failed to consistently produce the planned PTS. MATERIAL AND METHODS: The Signature™ patient-specific cutting guides (Biomet) developed from magnetic resonance imaging data were used in a prospective case-series of 50 TKAs. The target PTS was 2°. Standardised digitised radiographs were obtained postoperatively and evaluated by an independent reader. Reproducibility of the radiographic measurements was assessed on 20 cases. The posterior cortical line of the proximal tibia was chosen as the reference for PTS measurement. Inaccuracy was defined as an at least 2° difference in either direction compared to the target. RESULTS: The implant PTS was within 2° of the target in 72% of knees. In the remaining 28%, PTS was either excessive (n=10; maximum, 9°) or reversed (n=4; maximum, -6°). The postoperative hip-knee-ankle angle was 0° ± 3° in 88% of knees, and the greatest deviation was 9° of varus. CONCLUSION: These findings support our hypothesis that patient-specific instrumentation decreases PTS accuracy. They are consistent with recently published data. In contrast, patient-specific instrumentation provided accurate alignment in the coronal plane.

Enfermedad de depósito de ésteres de colesterol. Actualización del diagnóstico y el tratamiento / Cholesteryl ester storage disease. Updates in the diagnosis and treatment

La deficiencia parcial de la enzima lipasa ácida lisosomal (LAL) es una patología hereditaria, autosómica recesiva, del metabolismo de los lípidos que causa la enfermedad por depósitos de ésteres de colesterol (CESD, del inglés cholesteryl ester storage disease), caracterizada por la acumulación de ésteres de colesterol y triglicéridos en el hígado. El diagnóstico se realiza determinando la actividad de la LAL y/o las mutaciones del gen LAL (LIPA). La enfermedad se caracteriza por la aparición de una esteatosis microvesicular que conduce a la insuficiencia hepática, la aterosclerosis acelerada y una muerte prematura. Los pacientes presentan una elevación de colesterol total, c-LDL, triglicéridos y transaminasas con c-HDL bajo. Hasta ahora el tratamiento se realizaba con estatinas y, en última instancia, se efectuaba un trasplante de hígado. Se presenta el caso clínico de un paciente de 10 años de edad, diagnosticado de CESD mediante la determinación enzimática de la LAL en fibroblastos y el estudio de las mutaciones del gen LIPA. Además, se realiza una revisión actualizada de la literatura con el objetivo de presentar las nuevas técnicas diagnósticas y terapéuticas, como la determinación de la actividad de la LAL en muestra de sangre seca y el tratamiento de reposición enzimática con LAL recombinante humana (AU)

The lysosomal acid lipase deficiency (LAL) is a genetic disorder of the lipid metabolism of autosomal recessive inheritance that causes cholesteryl ester storage disease (CESD) resulting of triglyceride and cholesteryl ester accumulation, specifically in the liver. The diagnosis is carried out by liver biopsy and determining of LAL activity or LAL gene mutations (LIPA). The disease is defined by microvesicularsteatosis that provokes liver failure, accelerated atherosclerosis and premature death. The majority of patients has high levels of total serum cholesterol, LDL-cholesterol, triglycerides, and transaminases with low HDL-cholesterol and, until now, the treatment was statin support, and, as a last resort, the liver transplant. We have a clinical case of a 10 years old patient diagnosed of CESD by the determination of LAL activity in fibroblasts and study gene mutations LIPA. Was performed an updated review of the literature with the goal to present the new diagnostic and therapeutic techniques, including the determination of LAL activity in a dried blood sample and enzyme replacement therapy with recombinant human lysosomal acid lipase (AU)

Los métodos alternativos en el estudio de la seguridad de cosméticos / Alternative methods in the study of the safety of cosmetics

El Reglamento 1223/2009 establece las normas que deben cumplir todos los productos cosméticos comercializados en Europa, con objeto de velar por el funcionamiento del mercado interior y lograr un elevado nivel de protección de la salud humana garantizando el uso de métodos alternativos que no impliquen la utilización de animales. El Laboratorio Europeo de Referencia para las Alternativas a la Experimentación con Animales (EURL-EURL- ECVAM) es el laboratorio de referencia en Europa encargado de validar los métodos alternativos. Posteriormente pueden ser homologados por la Organización de Cooperación y Desarrollo Económico (OCDE). Por otro lado, el Comité Científico de Seguridad de los Consumidores (SCCS) asesora a la Comisión sobre todos los temas relacionados con la seguridad de los cosméticos. En esta revisión se detalla una relación de métodos alternativos necesarios para evaluar la seguridad de los ingredientes cosméticos así como los métodos usados y sus limitaciones (AU)

Regulation 1223/2009 apply to all cosmetic products marketed in Europe in order to ensure the internal market and achieve a high level of protection of human health by ensuring the use of alternative methods not involving the use of animals. The European Reference Laboratory for Alternatives to Animal Testing (EURL- EURL-ECVAM) is the European reference laboratory responsible for validating alternative methods. They can also be approved by the Organization for Economic Cooperation and Development (OECD). In addition, the Scientific Committee on Consumer Safety (SCCS) advises the EU Commission on all issues related to cosmetic safety. In this review, alternative methods needed to assess the safety of cosmetic ingredients and the methods used and their limitations are outlined (AU)

Piomiositis con sacroileítis asociada / Pyomyositis with associated sacroiliitis

La piomiositis es la infección por bacterias del músculo esquelético, infrecuente pero con una incidencia en aumento, por lo que se debe tener en cuenta a la hora de realizar el diagnóstico diferencial de la impotencia funcional febril en los miembros inferiores en la edad pediátrica. Se presenta el caso de una niña diagnosticada de piomiositis con sacroileítis asociada tratada en nuestro hospital, y se ha realizado una revisión bibliográfica a propósito del tema (AU)

Pyomyositis is the bacterian infection of skeletal muscles, rare in our area, but with an increasing incidence. That is why we should bear in mind it in the differential diagnosis of functional disability in lower limbs with fever in children. We present the case of a child diagnosed of pyomyositis with associated sacroiliitis treated in our hospital and realize a scientific literature review of pyomyositis (AU)

In vitro evaluation of graphene oxide nanosheets on immune function.

HYPOTHESIS: Graphene oxide (GO) has attracted the scientific community attention due to its novel properties and wide range of potential applications including hyperthermia cancer therapy. However, little is known about the GO effects on the immune function which involves both innate and adaptive defence mechanisms through the activation of different cell populations and secretion of several cytokines. The effect of different GO nanosheets designed for hyperthermia cancer therapy on macrophage and lymphocyte function should be determined before using GO for this application. EXPERIMENTS: The effects of GO nanosheets with 1 (1-GOs) and 6 arms (6-GOs) of polyethylene glycol on RAW-264.7 macrophages and primary splenocytes (as approximation to the in vivo situation) were evaluated through the proinflammatory cytokine secretion and the modulation of cell proliferation in the presence of specific stimuli for either T-lymphocytes (concanavalin A, anti-CD3 antibody) or B-lymphocytes/macrophages (lipopolysaccharide). FINDINGS: 6-GOs significantly increased the secretion of TNF-α by RAW-264.7 macrophages without alteration of IL-6 and IL-1ß levels. The treatment of primary splenocytes with 1-GOs and 6-GOs in the presence of concanavalin A, anti-CD3 antibody and lipopolysaccharide, produced significant dose-dependent decreases of cell proliferation and IL-6 levels, revealing weak inflammatory properties of GOs which are favourable for hyperthermia cancer therapy.