Bloqueo del ganglio estrellado como tratamiento de la tormenta arrítmica: estudio observacional / Stellate ganglion block as a treatment for arrhythmic storm: an observational study

Introducción: La tormenta arrítmica (TA) es una situación de emergencia potencialmente letal, con una elevada tasa de mortalidad. Cuando el tratamiento convencional agudo es inefectivo, el bloqueo del ganglio estrellado puede ayudar a controlar la arritmia, aportando un bloqueo simpático cervicotorácico visceral. El objetivo de este estudio es valorar la efectividad y seguridad de los bloqueos del ganglio estrellado (BGE) para el tratamiento de la TA refractaria. Método: Seguimiento de una cohorte de pacientes con TA refractaria que cumplieron los criterios para la realización de BGE. Dicho bloqueo fue ecoguiado al nivel de C6, utilizando un anestésico y un esteroide, de manera unilateral izquierda en primer lugar, y bilateral de no existir respuesta, realizándose posteriormente ablación mediante radiofrecuencia (RFC) guiada por fluoroscopio en C7 de no existir respuesta favorable, sino recidiva subsiguiente. Resultados: Se incluyeron siete pacientes, con una tasa de mortalidad durante el ingreso de 14,29%. Cuatro pacientes recibieron bloqueos unilaterales del ganglio estrellado, y en tres pacientes se realizaron bloqueos bilaterales. En seis de ellos se aplicó ablación, y uno de ellos tenía implantado un cardioversor-desfibrilador. La TA fue controlada temporalmente, más allá del efecto del anestésico local en todos los pacientes. Tres de ellos recibieron ablación por RFC, y dos simpatectomías torácicas quirúrgicas. El único efecto secundario fue el síndrome de Horner, que se observó en todos los casos tras realizar el bloqueo del ganglio estrellado con anestésico local. Dos pacientes murieron tras recibir el alta, y cuatro siguen en sus casas, tres de ellos sin haber sido ingresados a causa de episodios ventriculares durante más de dos años. Conclusión: El bloqueo ecoguiado del ganglio estrellado es una técnica efectiva y segura para el tratamiento de la TA refractaria, como complemento del tratamiento cardiológico habitual.(AU)

Introduction: Arrhythmic storm is a life-threatening emergency with a high mortality rate. When acute conventional treatment is ineffective, a stellate ganglion block can contribute to the control of the arrhythmia by providing a visceral cervicothoracic sympathetic block. The objective of the study is to assess the effectiveness and safety of stellate ganglion blocks for the treatment of refractory arrhythmic storm. Method: Follow-up of a cohort of patients with refractory arrhythmic storm that met the criteria for performing stellate ganglion blocks. The block was ultrasound-guided at C6-level using local anaesthetic and a steroid, left unilateral first, bilateral if no response, and followed by fluoroscopy-guided radiofrequency ablation at C7 if there was a favourable response but subsequent relapse. Results: Seven patients were included, with a mortality rate during admission of 14.29%. Four patients received unilateral and three bilateral stellate ganglion blocks. Six were ablated and one of them had an implanted cardioverter-defibrillator. Arrhythmic storm was controlled temporarily beyond the effect of the local anaesthetic in all patients. Three underwent radiofrequency ablation and two underwent surgical thoracic sympathectomy. The only side effect was Horner's syndrome, which was observed in all cases after administering a stellate ganglion block with local anaesthetic. Two died after discharge and four are still at home, three of them without further admission due to ventricular events for more than two years. Conclusion: An ultrasound-guided stellate ganglion block is an effective and safe technique in the treatment of refractory arrhythmic storm as a complement to the usual cardiological treatment.(AU)

Stellate ganglion block for the management of electrical storm: An observational study.

INTRODUCTION: Electrical storm is a life-threatening emergency with a high mortality rate. When acute conventional treatment is ineffective, stellate ganglion block can help control arrhythmia by providing a visceral cervicothoracic sympathetic block. The objective of this study is to assess the effectiveness and safety of stellate ganglion block in the management of refractory arrhythmic storm. METHOD: Follow-up of a cohort of patients with refractory electrical storm that met the criteria for performing stellate ganglion block. The block was ultrasound-guided at C6 using local anaesthetic and a steroid - left unilateral first, bilateral if no response, followed by fluoroscopy-guided radiofrequency ablation at C7 if there was a favourable response but subsequent relapse. RESULTS: Seven patients were included. The in-hospital mortality rate was 14.29%. Four patients received unilateral and 3 bilateral stellate ganglion block. Six were ablated and 1 received an implantable cardioverter-defibrillator. Electrical storm was controlled temporarily beyond the effect of the local anaesthetic in all patients. Three patients underwent radiofrequency ablation and 2 underwent surgical thoracic sympathectomy. The only side effect was Horner's syndrome, which was observed in all cases after administering a stellate ganglion block with local anaesthetic. Two patients died after discharge and 4 are alive at the time of writing, 3 of them have not been re-admitted for ventricular events for more than 2 years. CONCLUSION: Ultrasound-guided stellate ganglion block is an effective and safe complement to standard cardiological treatment of refractory electrical storm.

Sintomatología neuropsiquiátrica en el síndrome post-COVID. Propuesta de manejo y derivación desde atención primaria / Neuropsychiatric symptomatology in post-COVID syndrome. Intervention proposal and referral from primary care

La infección por SARS-CoV-2 es una enfermedad multiorgánica. Tiene un amplio espectro de manifestaciones clínicas, entre ellas neurológicas y psiquiátricas, que se expresan en todos los estadios evolutivos de la enfermedad. En muchas ocasiones presenta sintomatología a largo plazo que se ha denominado síndrome post-COVID. Entre la sintomatología neuropsiquiátrica derivada del mencionado síndrome, nos centramos en este manuscrito, por su prevalencia, en la cefalea, el deterioro cognitivo, las alteraciones del gusto y olfato, la depresión, los trastornos de ansiedad y el insomnio. En este documento se revisa esta sintomatología y se proponen algoritmos de manejo y criterios de derivación de atención primaria (AP) a otros especialistas (AU)

SARS-CoV-2 infection is a multiorgan disease with a wide spectrum of clinical manifestations, including neurological and psychiatric, which are expressed in all stages of the disease and often has long-term symptoms, called post-COVID syndrome. Among the neuropsychiatric symptoms derived from this syndrome, in this article we focus on headache, cognitive impairment, taste and smell alterations, depression, anxiety and sleep disorders. Intervention algorithms for these symptoms in primary care establishing criteria for referral to specialized care are proposed (AU)

[Neuropsychiatric symptomatology in post-COVID syndrome. Intervention proposal and referral from primary care]. / Sintomatología neuropsiquiátrica en el síndrome post-COVID. Propuesta de manejo y derivación desde atención primaria.

SARS-CoV-2 infection is a multiorgan disease with a wide spectrum of clinical manifestations, including neurological and psychiatric, which are expressed in all stages of the disease and often has long-term symptoms, called post-COVID syndrome. Among the neuropsychiatric symptoms derived from this syndrome, in this article we focus on headache, cognitive impairment, taste and smell alterations, depression, anxiety and sleep disorders. Intervention algorithms for these symptoms in primary care establishing criteria for referral to specialized care are proposed.

Seguridad del paciente: percepción de estudiantes de una universidad pública / Patient safety: Perceptions of students in a public university / Segurança do paciente: percepção de estudantes de uma universidade pública

RESUMEN Introducción: La seguridad del paciente es una prioridad mundial para los sistemas de salud. Las instituciones educativas formadoras de recursos humanos deben incluir la temática en su currículo. Objetivo: Identificar la percepción sobre la seguridad del paciente en los estudiantes de licenciatura en enfermería. Metodología: Estudio descriptivo y transversal. Población total de 516 alumnos de licenciatura en enfermería de la Universidad Autónoma de Tamaulipas, México. Se utilizó el cuestionario APQS-III y se diseñó una cédula con variables sociodemográficas. Resultados: La mayoría de los participantes son mujeres, edad promedio: 20 años. La mitad de los alumnos no tiene cursos que avalen sus conocimientos sobre seguridad del paciente. En la dimensión entrenamiento recibido, 63.5 % concuerda que la formación de pregrado los capacita para entender las causas de un evento adverso por enfermería, los habilita para prevenir errores y desarrollar una adecuada comprensión en materia de seguridad. 50.3 % está totalmente de acuerdo en la dimensión confianza en el reporte del error y es semejante la proporción en la dimensión horas de trabajo como causas de error. Discusión: La identificación positiva de la percepción que se tiene acerca de la seguridad del paciente por parte de los estudiantes, favorece desde el pregrado la creación de una cultura de seguridad que fomenta la confianza y el interés de estos para desarrollar un enfoque integrado en el tema estudiado. Conclusiones: Los estudiantes en su mayoría refieren actitudes positivas confiados en que su entrenamiento sobre seguridad los prepara para entender y prevenir las causas de errores en seguridad del paciente.

ABSTRACT Introduction: Patient safety is a priority of health systems worldwide and therefore, the education institutions responsible for the formation of human resources for health must address this goal in detail. Objective: To identify the perceptions of baccalaureate nursing students regarding patient safety. Methodology: This is a descriptive and transversal study on 516 baccalaureates in nursing students from the Universidad Autónoma de Tamaulipas, Mexico. The APQS-III and a specific social-demographic data questionnaire were used. Results: The majority of the participants were female. The average age was 20 years old. Nearly half of the students do not have enough courses to strengthen their knowledge on patient safety. Regarding the training received, 63.5 % of the participants agree that the baccalaureate level formation prepares them to understand the causes of adverse events in nursing, to prevent errors, and to have an adequate understanding of security. 50.3 % of the participants approved the dimension's confidence approach regarding error reporting. Many participants considered that the excessive long hours working dimension is a cause of errors. Discussion: The perception of patient safety as being very important favors and further promotes a culture of security. Conclusions: The majority of the students trust their training regarding patient safety and know how to prevent possible causes of errors.

RESUMO Introdução: A segurança do paciente é uma prioridade mundial para os sistemas de saúde. As instituições educacionais formadoras de recursos humanos devem incluir a temática em seu currículo. Objetivo: Identificar a percepção sobre a segurança do paciente nos estudantes de graduação em enfermagem. Metodologia: Estudo descritivo e transversal. População total de 516 alunos de graduação em enfermagem da Universidad Autónoma de Tamaulipas, México. Foi utilizado o questionário APQS-III e realizou-se um questionário com variáveis sociodemográficas. Resultados: A maioria dos participantes são mulheres, idade média: 20 anos. Metade dos estudantes não tem cursos que avaliem seus conhecimentos sobre segurança do paciente. Na dimensão do treinamento recebido, 63,5 % concordam que a formação de graduação os treina para entender as causas de um evento de enfermagem adverso, lhes permite prevenir erros e desenvolver uma compreensão adequada da segurança. 50,3 % concordam fortemente sobre a dimensão de confiança no relatório dos erros e a proporção e da mesma forma sobre a dimensão das horas de trabalho como causa dos erros. Discussão: A identificação positiva da percepção que se tem sobre a segurança do paciente por parte dos alunos, favorece desde a graduação a criação de uma cultura de segurança que fomente a confiança e o interesse destes em desenvolver uma abordagem integrada na o assunto estudado. Conclusões: A maioria dos alunos relata atitudes positivas, confiantes de que seu treinamento em segurança os prepara para compreender e prevenir as causas dos erros na segurança do paciente.

Heterozygous CAPN3 missense variants causing autosomal-dominant calpainopathy in seven unrelated families.

AIMS: Recessive variants in CAPN3 gene are the cause of the commonest form of autosomal recessive limb girdle muscle dystrophy. However, two distinct in-frame deletions in CAPN3 (NM_000070.3:c.643_663del21 and c.598_621del15) and more recently, Gly445Arg and Arg572Pro substitutions have been linked to autosomal dominant (AD) forms of calpainopathy. We report 21 affected individuals from seven unrelated families presenting with an autosomal dominant form of muscular dystrophy associated with five different heterozygous missense variants in CAPN. METHODS: We have used massively parallel gene sequencing (MPS) to determine the genetic basis of a dominant form of limb girdle muscular dystrophy in affected individuals from seven unrelated families. RESULTS: The c.700G> A, [p.(Gly234Arg)], c.1327T> C [p.(Ser443Pro], c.1333G> A [p.(Gly445Arg)], c.1661A> C [p.(Tyr554Ser)] and c.1706T> C [p.(Phe569Ser)] CAPN3 variants were identified. Affected individuals presented in young adulthood with progressive proximal and axial weakness, waddling walking and scapular winging or with isolated hyperCKaemia. Muscle imaging showed fatty replacement of paraspinal muscles, variable degrees of involvement of the gluteal muscles, and the posterior compartment of the thigh and minor changes at the mid-leg level. Muscle biopsies revealed mild myopathic changes. Western blot analysis revealed a clear reduction in calpain 3 in skeletal muscle relative to controls. Protein modelling of these variants on the predicted structure of calpain 3 revealed that all variants are located in proximity to the calmodulin-binding site and are predicted to interfere with proteolytic activation. CONCLUSIONS: We expand the genotypic spectrum of CAPN3-associated muscular dystrophy due to autosomal dominant missense variants.

Altered myogenesis and premature senescence underlie human TRIM32-related myopathy.

TRIM32 is a E3 ubiquitin -ligase containing RING, B-box, coiled-coil and six C-terminal NHL domains. Mutations involving NHL and coiled-coil domains result in a pure myopathy (LGMD2H/STM) while the only described mutation in the B-box domain is associated with a multisystemic disorder without myopathy (Bardet-Biedl syndrome type11), suggesting that these domains are involved in distinct processes. Knock-out (T32KO) and knock-in mice carrying the c.1465G > A (p.D489N) involving the NHL domain (T32KI) show alterations in muscle regrowth after atrophy and satellite cells senescence. Here, we present phenotypical description and functional characterization of mutations in the RING, coiled-coil and NHL domains of TRIM32 causing a muscle dystrophy. Reduced levels of TRIM32 protein was observed in all patient muscle studied, regardless of the type of mutation (missense, single amino acid deletion, and frameshift) or the mutated domain. The affected patients presented with variable phenotypes but predominantly proximal weakness. Two patients had symptoms of both muscular dystrophy and Bardet-Biedl syndrome. The muscle magnetic resonance imaging (MRI) pattern is highly variable among patients and families. Primary myoblast culture from these patients demonstrated common findings consistent with reduced proliferation and differentiation, diminished satellite cell pool, accelerated senescence of muscle, and signs of autophagy activation.

Validación del instrumento: Competencias del personal de enfermería en Salud Pública, en el contexto mexicano / Validation of instrument: Competencies among nursing personnel of public health in the Mexican context / Validação do instrumento: Competências do pessoal de enfermagem em Saúde Pública, no contexto mexicano

Introducción Cuando se utilicen cuestionarios desarrollados en otros países e idiomas en estudios científicos, además de traducirlos, es necesario su adaptación cultural y validación, el objetivo de esta investigación es contar con un instrumento válido y confiable para evaluar las competencias del personal de enfermería en salud pública, para aplicarlo en el contexto mexicano. Metodología El estudio fue de tipo descriptivo-transversal, multietápico, la población se integró en 3 grupos para las 3 diferentes etapas, en la primera, por un grupo de 10 expertos en investigación de la Facultad de Enfermería de Tampico, de la Universidad Autónoma de Tamaulipas, México y 17 profesionales que laboran en el primer nivel de atención; en la segunda, 30 profesionales de enfermería y en la tercera 177 profesionales que trabajan en el campo de la salud pública, todos ellos de tres jurisdicciones del Sur de Tamaulipas, México, el muestreo fue por conveniencia. Resultados En cuanto a los tres procesos que se realizaron para la validación del instrumento, se mostraron resultados adecuados y satisfactorios para su aplicación, ya que en la prueba de V-Aiken se obtuvo un resultado cercano a 1, en el proceso semántico sólo se realizó modificación a una palabra; a través de la prueba piloto se obtuvo un resultado de 0.97 en el Alfa de Cronbach. Conclusiones El instrumento se considera válido para iniciar con la evaluación de las mencionadas competencias, para contar con un diagnóstico inicial de las áreas de oportunidad para personal de enfermería.

Introduction When using questionnaires developed in other countries and languages for scientific studies, besides the translation, it is necessary their cultural adaptation and validation. The objective of this work was to assure the use of an instrument which is valid and reliable while assessing the competencies among nursing personnel of public health in the Mexican context. Methodology This is a descriptive-transversal and multi-step study. The population was represented by 3 groups for 3 different stages. The first group was integrated by 10 experts in research from the Nursing Faculty of the University of Tamaulipas, Tampico, and 17 professionals working in the first level of attention. The second group was integrated by 30 professionals of nursing. The third group was integrated by 177 professionals working in the field of public health. All professionals were from three jurisdictions in the south of Tamaulipas, Mexico. The sampling process was by convenience. Results The three validation processes showed satisfactory results for the adaptation of the instrument. The V-Aiken test score was close to 1. Regarding the semantics, only one word was modified. The pilot test had a final Cronbach Alpha of 0.97. Conclusions The instrument was considered valid for the assessment of the herein referenced competencies.

Introdução Quando se utilizem questionários desenvolvidos em outros países e idiomas em estudos científicos, além de traduzi-los, é necessário sua adaptação cultural e validação, o objetivo desta pesquisa é contar com um instrumento válido e confiável para avaliar as competências do pessoal de enfermagem em saúde pública, para aplicá-lo no contexto mexicano. Metodologia O estudo foi de tipo descritivo-transversal, multietápico, a população integrou-se em 3 grupos para as 3 diferentes etapas, na primeira, por um grupo de 10 experientes na pesquisa da Faculdade de Enfermagem de Tampico, da Universidad Autónoma de Tamaulipas, México e 17 profissionais que laboram no primeiro nível de atenção; na segunda, 30 profissionais de enfermagem e na terceira 177 profissionais que trabalham no campo da saúde pública, todos eles de três jurisdições do Sul de Tamaulipas, México, a amostra foi por conveniência. Resultados Em referência aos três processos que se realizaram para a validação do instrumento, mostraram-se resultados adequados e satisfatórios para sua aplicação, já que na prova de V-Aiken obteve-se um resultado próximo a 1, no processo semântico só se realizou modificação a una palavra; a través da prova piloto obteve-se um resultado de 0.97 na alfa de cronbach. Conclusões O instrumento considera-se válido para iniciar com a avaliação das mencionadas competências, para contar com um diagnóstico inicial das áreas de oportunidade para pessoal de enfermagem.

A novel MYH7 founder mutation causing Laing distal myopathy in Southern Spain.

MYH7 gene mutations are associated with wide clinical and genetic heterogeneity. We report a novel founder mutation in MYH7 in Southern Spain (Andalucía). We studied two index patients and 24 family members from two apparently independent families by physical examination, serum creatine-kinase, muscle MRI, sequencing studies and genetic linkage analysis. Sixteen individuals were heterozygous for a (p.R1560P) variant in the MYH7 gene. Haplotype was consistent with a common ancestor for the two families. The patients displayed the classic Laing distal myopathy phenotype, with hanging first toe as the initial presentation, even in mildly affected patients who declared themselves asymptomatic, although neck flexor weakness was revealed as an early sign in some cases. MRI showed that the sartorius was the first muscle involved, even in two out of three asymptomatic carriers. Our findings support the novel variant p.R1560P in MYH7 as a founder mutation in Andalucía. The early involvement of the sartorius muscle in MRI may be useful as an indicator of affection status.

Desmoplastic astrocytoma: new insights into its clinical profile, diagnosis, and treatment.

BACKGROUND: Desmoplastic astrocytoma (DA) is a rare intracranial tumor which usually affects pediatric patients. The aim of this study is to describe the clinical features and management of DA based on a joint analysis of the cases reported in the scientific literature. MATERIAL AND METHODS: A thorough review was carried out, gathering those pathologically proven DAs reported since the first description of this entity. Two new own cases were included in order to illustrate this review. Epidemiological, clinical, radiological, therapeutic, and follow-up data were analyzed with the software SPSS version 20. RESULTS: A total of 52 DAs were recorded. Most cases occurred in the first 2 years of life, although older patients were also reported. Patients mainly presented symptoms and signs of elevated intracranial pressure. According to their radiological features, we were able to classify DAs in four main groups, with distinct differential diagnosis and prognosis. After treatment, 14.2 % of patients presented persistent neurological impairment and the mortality rate was close to 10 %. CONCLUSION: DAs can be diagnosed at any age from birth to adolescence. These neoplasms can show up a wider range of radiological morphologies than previously thought. Surgery represents the treatment of choice for DAs, although chemotherapy can also be useful in the setting of recurrence or progression of the disease. Those DAs lacking classic radiological features, especially type 4 tumors, were linked with a poorer clinical outcome.

p63/MT1-MMP axis is required for in situ to invasive transition in basal-like breast cancer.

The transition of ductal carcinoma in situ (DCIS) to invasive breast carcinoma requires tumor cells to cross the basement membrane (BM). However, mechanisms underlying BM transmigration are poorly understood. Here, we report that expression of membrane-type 1 (MT1)-matrix metalloproteinase (MMP), a key component of the BM invasion program, increases during breast cancer progression at the in situ to invasive breast carcinoma transition. In the intraductal xenograft model, MT1-MMP is required for BM transmigration of MCF10DCIS.com breast adenocarcinoma cells and is overexpressed in cell clusters overlying focal BM disruptions and at the invasive tumor front. Mirrored upregulation of p63 and MT1-MMP is observed at the edge of MCF10DCIS.com xenograft tumors and p63 is required for induction of MT1-MMP-dependent invasive program in response to microenvironmental signals. Immunohistochemistry and analysis of public database reveal that p63 and MT1-MMP are upregulated in human basal-like breast tumors suggesting that p63/MT1-MMP axis contributes to progression of basal-like breast cancers with elevated p63 and MT1-MMP levels.

Developmental study of vitamin C distribution in children's brainstems by immunohistochemistry.

Vitamin C (Vit C) is an important antioxidant, exerts powerful neuroprotective brain effects and plays a role in neuronal development and maturation. Vit C is present in brain tissue at higher concentrations than in other organs, but its detailed distribution in brain is unknown. Immunohistochemical detection of this vitamin has been performed by using a highly specific antibody against Vit C. The aim of the present work was to analyze the distribution of Vit C in children's brainstems during postnatal development, comparing two groups of ages: younger and older than one year of life. In general, the same areas showing neurons with Vit C in young cases are also immunostained at older ages. The distribution of neurons containing Vit C was broader in the brainstems of older children, suggesting that brainstem neurons maintain or even increase their ability to retain Vit C along the life span. Immunohistochemical labeling revealed only cell bodies containing this vitamin, and no immunoreactive fibers were observed. The distribution pattern of Vit C in children's brainstems suggests a possible role of Vit C in brain homeostatic regulation. In addition, the constant presence of Vit C in neurons of locus coeruleus supports the important role of Vit C in noradrenaline synthesis, which seemed to be maintained along postnatal development.

The role of 18FDG, 18FDOPA PET/CT and 99mTc bone scintigraphy imaging in Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocitosis, characterized by multisystemic xanthogranulomatous infiltration by foamy histiocytes that stain positively for CD68 marker but not express CD1a and S100 proteins. Etiology and pathogenesis are still unknown and only about 500 cases are related in the literature. Multisystemic involvement leads to a wide variety of clinical manifestations that results in a poor prognosis although recent advances in treatment. We present the clinical, nuclear medicine findings and therapeutic aspects of a serie of 6 patients with histopathological diagnosis of ECD, who have undergone both bone scintigraphy (BS) and 18F-fluorodeoxyglucose (18FDG)-PET/CT scans in our institution. A complementary 18F-fluorodopa (18FDOPA)-PET/CT was performed in one case. Three different presentations of the disease were observed in our casuistic: most indolent form was a cutaneous confined disease, presented in only one patient. Multifocal involvement with central nervous system (CNS) preservation was observed in two patients. Most aggressive form consisted in a systemic involvement with CNS infiltration, presented in three patients. In our experience neurological involvement, among one case with isolate pituitary infiltration, was associated with mortality in all cases. 18FDG-PET/CT and BS were particularly useful in despite systemic involvement; locate the site for biopsy and the treatment response evaluation. By our knowledge, 18FDOPA-PET/CT not seems useful in the initial staging of ECD. A baseline 18FDG-PET/CT and BS may help in monitoring the disease and could be considered when patients were incidentally diagnosed and periodically 18FDG-PET/CT must be performed in the follow up to evaluate treatment response.

Distribution of peptidergic populations in the human dentate gyrus (somatostatin [SOM-28, SOM-12] and neuropeptide Y [NPY]) during postnatal development.

The postnatal development of the human hippocampal formation establishes the time and place at which we start autobiographical memories. However, data concerning the maturation of the neurochemical phenotypes characteristic of interneurons in the human hippocampus are scarce. We have studied the perinatal and postnatal changes of the dentate gyrus (DG) interneuron populations at three rostrocaudal levels. Immunohistochemically identified neurons and fibers for somatostatin (SOM-12 and SOM-28) and neuropeptide Y (NPY) and the co-localization of SOM-28 and NPY were analyzed. In total, 13 cases were investigated from late pregnancy (1 case), perinatal period (6 cases), first year (1 case), early infancy (3 cases), and late infancy (2 cases). Overall, the pattern of distribution of these peptides in the DG was similar to that of the adult. The distribution of cells was charted, and the cell density (number of positive cells/mm(2)) was calculated. The highest density corresponded to the polymorphic cell layer and was higher at pre- and perinatal periods. At increasing ages, neuron density modifications revealed a decrease from 5 postnatal months onward. In contrast, by late infancy, two immunoreactive bands for SOM-28 and NPY in the molecular layer were much better defined. Double-immunohistochemistry showed that NPY-positive neurons co-localized with SOM-28, whereas some fibers contained only one or other of the neuropeptides. Thus, this peptidergic population, presumably inhibitory, probably has a role in DG maturation and its subsequent functional activity in memory processing.

Developmental study of the distribution of hypoxia-induced factor-1 alpha and microtubule-associated protein 2 in children's brainstem: comparison between controls and cases with signs of perinatal hypoxia.

Perinatal asphyxia and hypoxia are common causes of morbidity in neonates. Prenatal birth associated with hypoxemia often results in several disorders because of the lack of oxygen in the brain. Survival rates from perinatal hypoxia have improved, but appropriate treatments for recovery are still limited, with great impact on patients, their families, society in general and health systems. The aim of this work is to contribute to a better understanding of the cellular mechanisms underlying the brainstem responses to hypoxia. For this purpose, distributions of two proteins, hypoxia-inducible factor-1 alpha (HIF-1α) and microtubule-associated protein 2 (MAP-2) were analyzed in brainstems of 11 children, four of them showing neuropathological evidence of brain hypoxia. They were included in control or hypoxic groups, and then in several subgroups according to their age. Immunohistochemical labeling for these proteins revealed only cell bodies containing HIF-1α, and both cell bodies and fibers positive for MAP-2 in the children's brainstems. The distribution of HIF-1α was more restricted than that of MAP-2, and it can be suggested that the expression of HIF-1α increased with age. The distribution pattern of MAP-2 in the medulla oblongata could be more due to age-related changes than to a response to hypoxic damage, whereas in the pons several regions, such as the nucleus ambiguus or the solitary nucleus, showed different immunolabeling patterns in controls and hypoxic cases. The distribution patterns of these two proteins suggest that some brainstem regions, such as the reticular formation or the central gray, could be less affected by conditions of hypoxia.

Analysis of Rho GTPase expression in T-ALL identifies RhoU as a target for Notch involved in T-ALL cell migration.

NOTCH1 is frequently mutated in T-cell acute lymphoblastic leukaemia (T-ALL), and can stimulate T-ALL cell survival and proliferation. Here we explore the hypothesis that Notch1 also alters T-ALL cell migration. Rho GTPases are well known to regulate cell adhesion and migration. We have analysed the expression levels of Rho GTPases in primary T-ALL samples compared with normal T cells by quantitative PCR. We found that 5 of the 20 human Rho genes are highly and consistently upregulated in T-ALL, and 3 further Rho genes are expressed in T-ALL but not detectable in normal T cells. Of these, RHOU expression is highly correlated with the expression of the Notch1 target DELTEX-1. Inhibition of Notch1 signalling with a γ-secretase inhibitor (GSI) or Notch1 RNA interference reduced RhoU expression in T-ALL cells, whereas constitutively active Notch1 increased RhoU expression. In addition, Notch1 or RhoU depletion, or GSI treatment, inhibits T-ALL cell adhesion, migration and chemotaxis. These results indicate that NOTCH1 mutation stimulates T-ALL cell migration through RhoU upregulation that could contribute to the leukaemia cell dissemination.

Manejo anestésico del síndrome de Brugada / No disponible

El síndrome de Brugada (SB) fue descrito en 1992 por los hermanos P. y J. Brugada en pacientes fallecidos por paro cardiaco sin defecto cardíaco estructural alguno pero con patrón electrocardiográfico (EKG) de bloqueo incompleto de la rama derecha del haz de His (BIRDHH) y elevación del segmento ST en las derivaciones precordiales V1,2 y 3 (1) .Tras este síndrome reside un defecto genético autosómico dominante con penetrancia incompleta que afecta al funcionamiento de los canales de sodio (2). Se diagnostica por un EKG típico casual o tras un episodio de paro cardiaco resucitado, generalmente episodio de taquiarritmia ventricular polimorfa sostenida que desencadena una fibrilación ventricular. Debido a su potencial letalidad, entendemos pues que ante el hallazgo de un BIRDHH en la consulta de preanestesia tengamos el SB presente para completar una anamnesis más dirigida. Si confirmamos su existencia el manejo anestésico debe ser cuidadoso para evitar complicaciones (AU)

Brugada’s syndrome (BS) was first described at 1992 by P. & J. Brugada’s brothers in patients who died by cardiac arrest without any defect in cardiac structure. They only showed an incompleted Right Bruch Block (RBB) and ST segment raised at V1,2 & 3 precordials (1). Behind this syndrome it hides a genetic disorder affecting the normal function of sodium channels (2). Its diagnosis is based either on the typical EKG changes or after resuscitated cardiacarrest generally polimorphus ventricular tachycardia who unleash ventricular fibrillation. Due to its lethalness we understand that under the finding of RBB in the preoperative evaluation a more conscientious anamnesis should be done. With the certainty of SB a careful anaesthetic management is obligatory (AU)