Aspectos electroclínicos de la epilepsia temporal mesial / Neuropsychological risk in temporal lobe epilepsy

Introducción: La epilepsia del lóbulo temporal mesial se considera la más frecuente de las epilepsias focales, con signos y síntomas característicos que ayudan a definir su diagnóstico. Contenidos: Dentro de su historia natural, las crisis pueden iniciar en los primeros años de vida, usualmente como episodios febriles con un periodo de remisión, para reaparecer en la adolescencia o en el adulto joven. La presentación electroencefalográfica tiene un patrón característico, con aparición de puntas y ondas agudas interictales en la región temporal anterior, por lo general unilaterales, y con actividad ictal generalmente theta en la misma localización. La causa más frecuente es la esclerosis del hipocampo. El tratamiento con medicamentos anticrisis puede controlar la epilepsia, aunque algunos casos pueden evolucionar a la farmacorresistencia, en la cual la cirugía de epilepsia está indicada, y tiene buenos resultados. Conclusiones: Esta revisión se centra en la descripción de las características electroclínicas de la epilepsia temporal mesial, para hacer un diagnóstico temprano e iniciar un tratamiento adecuado, a efectos de lograr un mejor pronóstico y una mejor calidad de vida para los pacientes con epilepsia y sus familiares.

Introduction: Mesial temporal lobe epilepsy is considered the most common of the focal epilepsies, with characteristic signs and symptoms that help define its diagnosis. Contents: In the natural history of the disease, seizures can begin in the first years of life, usually as febrile seizures with a period of remission, to reappear in adolescence or in the young adult. The electroencephalographic presentation has a characteristic pattern with the appearance of interictal sharp waves and spikes in the anterior temporal region, usually unilateral, and with generally theta ictal activity in the same location. The most common cause is hippocampal sclerosis. Treatment with antiseizure medication can control epilepsy. However, in some cases evolution of drug resistance can occur, leading to epilepsy surgery as the most appropriate treatment, based on its good results. Conclusions: This review focuses on the description of the electroclinical characteristics of temporal mesial epilepsy, in order to make an early diagnosis and adequate treatment, thus providing a better prognosis and quality of life for patients with epilepsy and their families.

The natural history of autosomal dominant polycystic kidney disease. A strategy for grouping families and mutations.

Autosomal dominant polycystic kidney disease (ADPKD) is a main cause of end-stage renal disease. Today, knowledge of its genetic basis has made it possible to develop strategies that prevent the transmission of the disease. OBJECTIVES: The objective of the study was to analyze the natural history of ADPKD in the province of Córdoba and to design a database that allows grouping families with different mutations. PATIENTS AND METHODS: All patients (n = 678) diagnosed with ADPKD followed by the Córdoba nephrology service are included. Various clinical variables (age and sex), genetic variables (mutation in PKD1, PKD2) and the need for renal replacement therapy (RRT) were retrospectively analyzed. RESULTS: The prevalence was 61 cases per 100,000 inhabitants. Median renal survival was significantly worse in PKD1 (57.5 years) than in PKD2 (70 years) (log-rank p = 0.000). We have genetically identified 43.8% of the population, detecting PKD1 mutations in 61.2% and PKD2 mutations in 37.4% of cases, respectively. The most frequent mutation, in PKD2 (c.2159del), appeared in 68 patients belonging to 10 different families. The one with the worst renal prognosis was a truncating mutation in PKD1 (c.9893 G > A). These patients required RRT at a median age of 38.7 years. CONCLUSIONS: Renal survival of ADPKD in the province of Córdoba is similar to that described in the literature. We detected PKD2 mutations in 37.4% of cases. This strategy allows us to know the genetic basis of a large proportion of our population while saving resources. This is essential to be able to offer primary prevention of ADPKD through preimplantation genetic diagnosis.

Síndrome de Angelman en el adulto / Angelman syndrome in adulthood

Introducción: El síndrome de Angelman (SA) está ampliamente descrito en la infancia, pero existen escasos estudios en edad adulta y la mayoría recoge un pequeño número de pacientes o condiciones específicas, como epilepsia o sueño. Objetivo: El objetivo de este estudio es describir el SA en la edad adulta en nuestro centro, sus necesidades especiales, y el soporte médico y social para mejorar la atención y ofrecer una mejor transición del servicio de pediatría a las unidades de adultos. Pacientes y métodos: Se recogen pacientes con SA genéticamente confirmado, y describimos datos demográficos, médicos y sociales mediante la revisión de historias clínicas, entrevistas telefónicas con el cuidador principal y tres escalas estandarizadas de sueño, dependencia y calidad de vida. Resultados: Se incluye a 30 pacientes con una mediana de edad de 22,7 años: 22 son deleciones, 27 presentan antecedente de epilepsia y 13 están en tratamiento con, al menos, dos fármacos antiepilépticos. Las comorbilidades más frecuentes después de la epilepsia fueron los síntomas psiquiátricos, la escoliosis, el sobrepeso, el estreñimiento y problemas oftalmológicos. El 40% precisó ingresos hospitalarios en la edad adulta, cinco están institucionalizados y 24 reciben terapias no médicas. El médico a cargo es el neurólogo en la mayoría, seguido del neuropediatra. Conclusiones: Es necesario realizar estudios de historia natural más allá de la infancia. Ésta es la primera revisión española de adultos con SA que recoge un amplio espectro de condiciones sociales y médicas de estos pacientes.(AU)

Introduction: Angelman syndrome (AS) is widely described in childhood, but few studies have been conducted in adulthood and most of them report a small number of patients or specific conditions, such as epilepsy or sleep. Aim: The aim of this study is to describe AS in adulthood in our centre, the special needs it requires, and the medical and social support to improve care and to provide a better transition from the paediatric service to units for adults. Patients and methods: We collected patients with genetically confirmed AS, and described demographic, medical and social data by reviewing medical records, telephone interviews with the primary caregiver and three standardised sleep, dependency and quality of life scales. Results: Thirty patients with a median age of 22.7 years were included: 22 were deletions, 27 had a history of epilepsy and 13 were on treatment involving at least two antiepileptic drugs. The most frequent comorbidities after epilepsy were psychiatric symptoms, scoliosis, overweight, constipation and ophthalmological problems. Forty per cent required hospital admissions in adulthood, five were institutionalised and 24 received non-medical therapies. The doctor in charge was the neurologist in most cases, followed by the neuropaediatrician. Conclusions: Studies that examine the natural history beyond childhood are warranted. This is the first Spanish review of adults with AS that covers a broad spectrum of social and medical conditions of these patients.(AU)

Un caso de leucismo en el Juil de Jamapa, Rhamdia laticauda (Siluriformes, Heptapteridae) en el noreste del estado de Oaxaca, México

Se reporta la primera observación de un espécimen leucístico en el Juil de Jamapa, Rhamdia laticauda en el noreste del estado de Oaxaca. En un estudio ictiológico de la Reserva de la Biosfera Tehuacán-Cuicatlán en el río Xiquila de la cuenca alta del río Papaloapan, el pez se capturó con pesca eléctrica y se fotografió. El bagre vivo mostró una reducción de pigmentos en la piel, manchas blancas y ojos negros normales. Esto contribuye al conocimiento de la variabilidad intraespecífica y la historia natural de R. laticauda. Se discuten las causas potenciales de esta anomalía de color.

Here, we report the first observation of a leucistic specimen in the Rock catfish, Rhamdia laticauda in the Northeast of Oaxaca, Mexico. We caught the specimen by electric fishing and then photographed during an ichthyological survey carried out in the Tehuacán-Cuicatlán Biosphere Reserve in the Xiquila River of the upper Papaloapan River basin. The live catfish showed a lack of pigment in the skin, white spots, and normal eye color. This information contributes to the knowledge about intraspecific variability and natural history of R. laticauda. We discuss the potential causes of this color anomaly.

Incidencia e historia natural de la enfermedad inflamatoria intestinal en Castilla y León: estudio prospectivo, multicéntrico y poblacional / Incidence, and natural history of inflammatory bowel disease in Castilla y León: Prospective and multicenter epidemiological study

Introducción: La incidencia de la enfermedad inflamatoria intestinal (EII) está aumentando en todo el mundo. Objetivos: Evaluar la incidencia de EII en la comunidad autónoma de Castilla y León y describir las características clínicas de los pacientes al diagnóstico, el tipo de tratamiento recibido y la evolución clínica durante el primer año. Material y métodos: Estudio prospectivo, multicéntrico y poblacional en el que se incluyeron pacientes adultos diagnosticados de EII (enfermedad de Crohn [EC], colitis ulcerosa [CU] o colitis indeterminada [CI]) durante el año 2017 procedentes de 8 centros de Castilla y León. Se incluyeron variables epidemiológicas, clínicas y terapéuticas. Se calculó la incidencia global y por enfermedades. Resultados: Doscientos noventa pacientes fueron diagnosticados de EII (54,5% de CU, 45.2% de EC y 0,3% de CI), con una mediana de seguimiento de 9 meses (rango 8-11). La tasa de incidencia fue de 16,6 casos/100.000 habitantes-año (9/105 casos de CU y 7,5/105 casos de EC), con una proporción CU/EC de 1,2:1. Los pacientes con EC recibieron significativamente más corticoides sistémicos (47% vs. 30%; p=0,002), más tratamiento inmunomodulador (81% vs. 19%; p=0,000), más tratamiento biológico (29% vs. 8%; p=0,000) y mayor necesidad de cirugía (11% vs. 2%; p=0,000). Conclusiones: La incidencia de pacientes con CU en nuestro medio se incrementa, mientras que la de EC se mantiene estable, con una historia natural de la enfermedad peor (uso de corticoides, inmunosupresores, biológicos y cirugía) para los pacientes con EC comparado con los pacientes con CU en el primer año de seguimiento.(AU)

Introduction: The incidence of inflammatory bowel disease (IBD) is increasing worldwide. Objectives: To evaluate the incidence of IBD in Castilla y León describing clinical characteristics of the patients at diagnosis, the type of treatment received and their clinical course during the first year. Materials and methods: Prospective, multicenter and population-based incidence cohort study. Patients aged >18 years diagnosed during 2017 with IBD (Crohn's disease [CD], ulcerative colitis [UC] and indeterminate colitis [IC]) were included from 8 hospitals in Castilla y León. Epidemiological, clinical, and therapeutic variables were registered. The global incidence and disease incidence were calculated.Results290 patients were diagnosed with IBD (54.5% UC, 45.2% CD, and 0.3% IC), with a median follow-up of 9 months (range 8−11). The incidence rate of IBD in Castilla y Leon in 2017 was 16.6 cases per 10,000 inhabitants-year (9/105 UC cases and 7.5/105 CD cases), with a UC/CD ratio of 1.2:1. Use of systemic corticosteroids (47% vs 30%; P=.002), immunomodulatory therapy (81% vs 19%; P=.000), biological therapy (29% vs 8%; P=.000), and surgery (11% vs 2%; p=.000) were significatively higher among patients with CD comparing with those with UC. Conclusions: The incidence of patients with UC in our population increases while the incidence of patients with CD remains stable. Patients with CD present a worse natural history of the disease (use of corticosteroids, immunomodulatory therapy, biological therapy and surgery) compared to patients with UC in the first year of follow-up.(AU)

Historia natural de la poliquistosis renal autosómica dominante en Córdoba: utilidad de una base de datos para agrupar familias y mutaciones / The natural history of autosomal dominant polycystic kidney disease. A strategy for grouping families and mutations

La poliquistosis renal autosómica dominante (PQRAD) es una de las principales causas de insuficiencia renal terminal. Hoy día el conocimiento de sus bases genéticas está permitiendo desarrollar estrategias que eviten la transmisión de la enfermedad. Objetivos: El objetivo del estudio fue analizar la historia natural de la PQRAD en la provincia de Córdoba y diseñar una base de datos que permita agrupar a las familias y a las diferentes mutaciones. Pacientes y métodos: Se incluyen todos los pacientes (n=678) diagnosticados de PQRAD seguidos en el servicio de Nefrología de Córdoba. Se analizaron de manera retrospectiva diversas variables clínicas (edad y sexo), necesidad de terapia renal sustitutiva (TRS) y genéticas. Resultados: La prevalencia fue de 61 casos por 100.000 habitantes. La mediana de supervivencia renal fue significativamente peor en PKD1 (57,5 años) que en PKD2 (70 años) (Log-rank p=0,000). Tenemos identificada genéticamente al 43,8% de la población, detectando mutaciones en PKD1 en el 61,2% y en PKD2 en el 37,4% de los casos. La mutación más frecuente fue detectada en PKD2 (c.2159del) en 68 pacientes pertenecientes a 10 familias diferentes. La de peor pronóstico renal fue una mutación truncante detectada en PKD1 (c.9893G>A). Conclusiones: La supervivencia renal de la PQRAD en la provincia de Córdoba es similar a la descrita en la literatura. Con nuestra metodología y estudiando genéticamente al 43,8% de la población, detectamos mutaciones en PKD2 en una mayor proporción de pacientes, en el 37,4% de los casos. Esta estrategia permite conocer las bases genéticas de gran parte de nuestra población con ahorro de recursos. Esto es fundamental para poder ofrecer prevención primaria de la PQRAD mediante diagnóstico genético preimplantacional. (AU)

Autosomal dominant polycystic kidney disease (ADPKD) is one of the main causes of end-stage renal disease. Today, the knowledge of its genetic base has made it possible to develop strategies that prevent the transmission of the disease. Objectives: The objective of the study was to analyze the natural history of ADPKD in the Province of Córdoba and to design a database that allows families and different mutations to be grouped. Patients and methods: All patients (n=678) diagnosed with ADPKD followed up in the Cordoba nephrology service are included. Various clinical variables (age and sex), genetic variables (mutation in PKD1, PKD2) and the need for renal replacement therapy (RRT) were retrospectively analyzed. Results: The prevalence was 61 cases per 100,000 inhabitants. Median renal survival was significantly worse in PKD1 (57.5 years) than in PKD2 (70 years) (Log-rank p=0.000). We have genetically identified 43.8% of the population, detecting mutations in PKD1 in 61.2% and in PKD2 in 37.4% of cases. The most frequent mutation was detected in PKD2 (c.2159del) in 68 patients belonging to 10 different families. The one with the worst renal prognosis was detected in PKD1 (c.9893G>A). These patients required RRT at a median age of 38.7 years. Conclusions: The renal survival of ADPKD in the Province of Córdoba is similar to that described in the literature. Mutations in PKD2 were detected in 37.4%. Our strategy allows us to know the genetic bases of our population with a great saving of resources. This is essential to be able to offer primary prevention of ADPKD through preimplantation genetic diagnosis. (AU)

Incidence, and natural history of inflammatory bowel disease in Castilla y León: Prospective and multicenter epidemiological study. / Incidencia e historia natural de la enfermedad inflamatoria intestinal en Castilla y León: estudio prospectivo, multicéntrico y poblacional.

INTRODUCTION: The incidence of inflammatory bowel disease (IBD) is increasing worldwide. OBJECTIVES: To evaluate the incidence of IBD in Castilla y León describing clinical characteristics of the patients at diagnosis, the type of treatment received and their clinical course during the first year. MATERIALS AND METHODS: Prospective, multicenter and population-based incidence cohort study. Patients aged >18 years diagnosed during 2017 with IBD (Crohn's disease [CD], ulcerative colitis [UC] and indeterminate colitis [IC]) were included from 8 hospitals in Castilla y León. Epidemiological, clinical, and therapeutic variables were registered. The global incidence and disease incidence were calculated. RESULTS: 290 patients were diagnosed with IBD (54.5% UC, 45.2% CD, and 0.3% IC), with a median follow-up of 9 months (range 8-11). The incidence rate of IBD in Castilla y Leon in 2017 was 16.6 cases per 10,000 inhabitants-year (9/105 UC cases and 7.5/105 CD cases), with a UC/CD ratio of 1.2:1. Use of systemic corticosteroids (47% vs 30%; P=.002), immunomodulatory therapy (81% vs 19%; P=.000), biological therapy (29% vs 8%; P=.000), and surgery (11% vs 2%; p=.000) were significatively higher among patients with CD comparing with those with UC. CONCLUSIONS: The incidence of patients with UC in our population increases while the incidence of patients with CD remains stable. Patients with CD present a worse natural history of the disease (use of corticosteroids, immunomodulatory therapy, biological therapy and surgery) compared to patients with UC in the first year of follow-up.

Las láminas zootómicas del profesor Paul Pfurtscheller de Viena: un material científico renovador en las aulas españolas en el primer tercio de siglo XX / [The zootomic slides of Professor Paul Pfurtscheller from Vienna: innovative scientific material in Spanish classrooms in the first third of the 20th century]

Paul Pfurtscheller fue uno de los creadores de material didáctico más universales y re-conocidos dentro de la enseñanza de la Historia Natural, sus láminas de pared de zoología tuvieron presencia a nivel internacional en escuelas normales, institutos de segunda enseñanza e incluso en gabinetes y laboratorios universitarios. Doctor por la Universidad de Viena con una tesis de Botá-nica desarrolló una carrera prominente como profesor de secundaria de la asignatura de Historia Natural en el Gymnasium (Instituto) Franz Josef de Viena, desarrollando un gabinete museo para su asignatura y creando 39 láminas zootómicas como apoyo a su docencia personal. La edición de sus láminas a través de prestigiosa casa editorial vienesa A. Pichlers Witwe & Sohn y posteriormente por la holandesa Martinus Nijhoff permitió la difusión de su trabajo a través de Europa y América. Tras una aproximación biográfica del personaje basada en los documentos encontrados en la Biblioteca Nacional de Austria y en los de la Universidad de Viena, se ha indagado en su papel como docente y en la génesis de su colección, en su éxito pedagógico y el papel que tuvieron en las aulas espa-ñolas de principios de siglo XX. Dentro de las fuentes a considerar destacan las propias láminas, los catálogos comerciales, las leyendas explicativas, los manuales de clase, memorias anuales de los centros estudiados y las imágenes de las aulas, no como meras ilustraciones, sino como objeto de estudio para analizar el espacio escolar y el papel didáctico de las láminas. (AU)

O Gabinete de Curiosidades da Universidade de Coimbra: uma proposta museológica e científica / The Cabinet of Curiosities at the University of Coimbra: a museological and scientific initiative

Resumo Os gabinetes de curiosidades constituem a origem do museu moderno. Na utopia da Wunderkammer existe a aspiração à convivência entre arte e ciência. Como docente do novo Curso de Philosofia Natural da Universidade de Coimbra (1772), Portugal contratou o naturalista italiano Domingos Vandelli, que transportou consigo uma coleção, com objetos do seu gabinete em Pádua, à qual anexou uma outra coleção, recolhida em Portugal (1764-1768), repositório ainda não tributário do paradigma naturalista de Lineu. É com fundamento na descrição dessas duas coleções que defendemos a relevante proposta - para a ciência e para a museologia - do Gabinete de Curiosidades do Museu da Ciência da Universidade de Coimbra, inaugurado em maio de 2022.

Abstract Cabinets of curiosities are the origin of modern museums. The utopia these Wunderkammer contain also includes aspirations for art and science to coexist. The Italian naturalist Domingos Vandelli was contracted as an instructor for the new course in natural philosophy at the University of Coimbra (1772), and brought objects from his own cabinet in Padua; he combined these with another collection mounted in Portugal (1764-1768), which did not yet conform to Linneus's naturalistic paradigm. Based on descriptions of these two collections, we defend the important initiative underway at the Cabinet of Curiosities at the University of Coimbra's Museum of Science, which was inaugurated in May 2022.

Mimetismo entre serpentes e invertebrados: convergência ou coincidência? Uma revisão de literatura

Mimicry is a topic widely studied since the last century, firstly mentioned by the naturalists Henry Walter Bates and Alfred Russel Wallace, the studies were mainly about insects. Currently, mimicry research comprises both vertebrate and invertebrate. But there are few that mention a possible mimicry complex between snakes and invertebrates. This work aims to realize a bibliography review about possible mimicry complexes that comprises both snakes and invertebrates, comment about these researches and correlate with distribution data. The surveys were made using digital magazines and libraries, as well as databases for geographic distribution references. Nine publications were found that suggest possible evidence for a mimicry complex between species of invertebrates with snakes. However, there are not, at the present moment, works that indicate datation or origin of the groups here presented so that we can infer who is the mimic and who is the model. Therefore, the conclusions were that more research is necessary, mainly focused to datation analysis.

O Mimetismo é um tema amplamente estudado desde o século passado, iniciado pelos pesquisadores naturalistas Henry Walter Bates e Alfred Russel Wallace, onde os estudos foram principalmente sobre insetos. Atualmente os estudos de mimetismo englobam tanto vertebrados quanto invertebrados, mas são poucos trabalhos que mencionam um possível complexo mimético entre serpentes e invertebrados. Este trabalho objetiva realizar um levantamento bibliográfico acerca de possíveis complexos miméticos que englobam serpentes e invertebrados, discorrer sobre essas pesquisas e relacioná-los com dados de distribuição geográfica. O levantamento foi feito através de pesquisas em bibliotecas e revistas digitais, bem como bancos de dados de espécies para referências de distribuição geográfica. Foram achados no total nove publicações que apontam possíveis evidências para um possível complexo mimético entre espécies de invertebrados com serpentes. No entanto, não existem, até o momento, trabalhos que indiquem a datação ou origem dos grupos aqui apresentados para que possamos inferir quem seria o mímico e quem seria o modelo. Sendo assim, as conclusões do trabalho foram de que são necessárias mais pesquisas, sobretudo voltadas para as análises de datação.

Hígado graso (parte 1): aspectos generales, epidemiología, fisiopatología e historia natural / Non-alcoholic fatty liver disease part 1: general aspects, epidemiology. pathophysiology and natural history

Resumen El hígado graso no alcohólico (NAFLD) se define por la presencia de grasa o esteatosis en los hepatocitos y abarca un espectro que va desde la esteatosis simple, pasa por la esteatohepatitis no alcohólica (NASH) con inflamación y fibrosis, y finaliza en la cirrosis. Se considera una prevalencia mundial global cercana al 25% en la población general y se diagnóstica entre los 40 y 50 años, con variaciones respecto al sexo predominante y con diferencias étnicas (la población hispana es la más afectada). El hígado graso está asociado al síndrome metabólico (SM), y la obesidad se considera el principal factor de riesgo con su presencia y con su progresión. El hígado graso es un trastorno complejo y muy heterogéneo en su fisiopatología, que resulta de la interacción de múltiples elementos: factores genéticos, epigenéticos, ambientales, culturales, entre otros. Todo ello en conjunto lleva a incremento paulatino de grasa hepática, resistencia a la insulina y alteraciones hormonales y de la microbiota intestinal, lo que genera un daño hepatocelular a través de la formación de radicales libres de oxígeno y activación de la fibrogénesis hepática. La historia natural del hígado graso es dinámica: los pacientes con esteatosis simple tienen bajo riesgo de progresión a cirrosis, mientras que en los pacientes con NASH este riesgo se aumenta; sin embargo, el proceso puede ser reversible y algunas personas tendrán una mejoría espontánea. La fibrosis parece ser el determinante de la mortalidad global y de los desenlaces asociados a la enfermedad hepática; se considera que en todos los pacientes la fibrosis empeora una etapa cada 14 años y en NASH empeora en una etapa cada 7 años. Estudios previos concluyen que aproximadamente 20% de los casos de esteatosis simple progresan a NASH y que, de ellos, aproximadamente el 20% progresan a cirrosis, con presencia de hepatocarcinoma (HCC) en el 5% a 10% de ellos.

Abstract Fatty liver or NAFLD is defined by the presence of fat or steatosis in hepatocytes and covers a spectrum that goes from simple steatosis, through steatohepatitis (NASH), with inflammation and fibrosis and ending in cirrhosis. It is considered a global world prevalence close to 25% in the general population and is diagnosed between 40 and 50 years, with variations regarding the predominant sex and with ethnic differences, affecting more the Hispanic population. Fatty liver is associated with metabolic syndrome (MS), and obesity is considered the main risk factor for its presence and progression. Fatty liver is a complex and very heterogeneous disorder in its pathophysiology, resulting from the interaction of multiple elements, genetic, epigenetic, environmental, cultural factors, etc. All this together leads to an accumulation of hepatic fat, insulin resistance, hormonal and intestinal microbiota alterations, generating hepatocellular damage through the formation of free oxygen radicals and activation of hepatic fibrogenesis. The natural history of fatty liver is dynamic, patients with simple steatosis have a low risk of progression to cirrhosis, in patients with NASH this risk is increased, however, the process may be reversible, and some people will have spontaneous improvement. Fibrosis seems to be the determinant of overall mortality and outcomes associated with liver disease, it is considered that in all patients fibrosis worsens one stage every 14 years, in NASH it worsens one stage every seven years. Previous studies conclude that approximately 20% of cases of simple steatosis progress to NASH and that approximately 20% of them progress to cirrhosis, with the presence of hepatocellular carcinoma (HCC) in 5 to 10% of them.

Antigüedades prehispánicas peruanas en la creación de una "prehistoria" chilena: el caso de la colección Sáenz / Peruvian pre-Hispanic antiquities in the creation of a Chilean "prehistory: " the case of the Sáenz collection

Resumen En 1895, Nicolás Sáenz propuso al gobierno chileno la venta de una colección de objetos "incaicos" traídos desde Lima; adquisición aprobada en 1897. Pese a las dificultades ocasionadas por la Guerra del Pacífico, el Museo Nacional (Chile) continuó adquiriendo antigüedades peruanas, siguiendo una tradición de estudios comparados de cultura material. Dentro del marco del evolucionismo social, estas antigüedades servían como medida de civilización, con la cual contrastar la cultura material de los pueblos prehispánicos de Chile. Este artículo analiza la adquisición de la colección Sáenz, como un punto cúlmine de un proceso de tránsito hacia a una arqueología enfocada en las nuevas adquisiciones territoriales post guerra del Pacífico.

Abstract In 1895, Nicolás Sáenz proposed to the Chilean government to purchase a collection of "Inca" objects brought from Lima. This acquisition was approved in 1897. Despite the difficulties caused by the War of the Pacific, the Museo Nacional de Historia Natural (Chile) continued to acquire Peruvian antiquities, following a tradition of comparative studies of material culture. Within the framework of social evolutionism, these antiquities were a measure of civilization, a reference from which to evaluate the material culture of the pre-Hispanic peoples of Chile. This article analyzes the acquisition of the Sáenz collection as the culmination of a process of transition towards an archeology that focused on the new post-war territorial acquisitions in the Pacific.

Tasas de colectomía en colitis ulcerosa 15 años después del diagnóstico. Datos de la cohorte Navarra 2001-2003 / Colectomy rate in ulcerative colitis 15 years after diagnosis: Results from the 2001-2003 Navarra cohort

Introducción: Conocer la historia natural de la colitis ulcerosa (CU) es esencial para entender la evolución de la enfermedad, evaluar el impacto de las distintas estrategias terapéuticas e identificar factores de mal pronóstico. Uno de los aspectos más relevantes, en este sentido, es la necesidad de cirugía.Objetivos: Analizar la tasa de incidencia de colectomía (TIC) desde el diagnóstico hasta el fin de seguimiento (31 de diciembre del 2017) e identificar factores predictivos de colectomía.Material y métodos: Estudio retrospectivo que incluye los pacientes con diagnóstico definitivo (DD) de CU o colitis inclasificable (CI) en la cohorte Navarra 2001 a 2003.Resultados: Incluimos 174 pacientes con DD de CU (E2 42,8% - E3 26,6%) y cinco de CI: 44,1% mujeres, mediana edad 39,2 años (rango siete a 88), mediana de seguimiento 15,7 años. Se intervienen ocho pacientes (TIC tres colectomías/103pac/a): tres al debut (< 1 mes), dos en los primeros dos años, dos a los cinco años y uno a los 12 años de evolución. Todos habían recibido esteroides, cinco inmunomoduladores y dos biológicos. En siete (87%) la cirugía fue urgente y la indicación, megacolon en tres (37,5%), brote grave en tres (37,5%) y fallo a tratamiento médico en dos (25%). En cinco casos (62,5%) se realizó un reservorio ileoanal y en tres una ileostomía definitiva. En el análisis univariante, los pacientes con pérdida > 5 kg e ingreso al debut presentaron una menor supervivencia libre de colectomía.Conclusiones: En nuestra serie, las tasas de colectomía son más bajas que las comunicadas habitualmente, mayoritariamente se realizan en los primeros cinco años de evolución y se indican con carácter urgente

Introduction: Knowing the natural history of ulcerative colitis (UC) is essential to understand the course of the disease, assess the impact of different treatment strategies and identify poor prognostic factors. One of the most significant matters in this regard is the need for surgery.Objectives: To analyse the Colectomy Incidence Rate (CIR) from diagnosis to end of follow-up (31/12/2017) and identify predictive factors for colectomy.Material and methods: A retrospective study enrolling patients with a definitive diagnosis (DD) of UC or Unclassified Colitis (UnC) in the 2001-03 Navarra cohort.Results: We enrolled 174 patients with a DD of UC (E2 42.8%; E3 26.6%) and 5 patients with a DD of UnC: 44.1% women, median age 39.2 years (range 7-88) and median follow-up 15.7 years. A total of 8 patients underwent surgery (CIR 3 colectomies/103 patient-years: 3 at initial diagnosis (<1 month), 2 in the first 2 years, 2 at 5 years from diagnosis and 1 at 12 years from diagnosis. All had previously received steroids; 5 had received immunomodulators and 2 had received biologics. In 7 patients (87%), surgery was performed on an emergency basis. The indication was megacolon in 3 (37.5%), severe flare-up in 3 (37.5%) and medical treatment failure in 2 (25%). In 5 cases (62.5%), an ileoanal pouch was made, and in 3 cases, a definitive ileostomy was performed. In the univariate analysis, patients with loss of more than 5 kg at diagnosis and admission at diagnosis had a lower rate of colectomy-free survival.Conclusions: In our series, colectomy rates are lower than usually reported. Most colectomies were performed in the first 5 years following diagnosis and had an emergency indication

Colectomy rate in ulcerative colitis 15 years after diagnosis: Results from the 2001-2003 Navarra cohort. / Tasas de colectomía en colitis ulcerosa 15 años después del diagnóstico. Datos de la cohorte Navarra 2001-2003.

INTRODUCTION: Knowing the natural history of ulcerative colitis (UC) is essential to understand the course of the disease, assess the impact of different treatment strategies and identify poor prognostic factors. One of the most significant matters in this regard is the need for surgery. OBJECTIVES: To analyse the Colectomy Incidence Rate (CIR) from diagnosis to end of follow-up (31/12/2017) and identify predictive factors for colectomy. MATERIAL AND METHODS: A retrospective study enrolling patients with a definitive diagnosis (DD) of UC or Unclassified Colitis (UnC) in the 2001-03 Navarra cohort. RESULTS: We enrolled 174 patients with a DD of UC (E2 42.8%; E3 26.6%) and 5 patients with a DD of UnC: 44.1% women, median age 39.2 years (range 7-88) and median follow-up 15.7 years. A total of 8 patients underwent surgery (CIR 3 colectomies/103 patient-years: 3 at initial diagnosis (<1 month), 2 in the first 2 years, 2 at 5 years from diagnosis and 1 at 12 years from diagnosis. All had previously received steroids; 5 had received immunomodulators and 2 had received biologics. In 7 patients (87%), surgery was performed on an emergency basis. The indication was megacolon in 3 (37.5%), severe flare-up in 3 (37.5%) and medical treatment failure in 2 (25%). In 5 cases (62.5%), an ileoanal pouch was made, and in 3 cases, a definitive ileostomy was performed. In the univariate analysis, patients with loss of more than 5 kg at diagnosis and admission at diagnosis had a lower rate of colectomy-free survival. CONCLUSIONS: In our series, colectomy rates are lower than usually reported. Most colectomies were performed in the first 5 years following diagnosis and had an emergency indication.

Breeding biology of Hooded Gnateater Conopophaga roberti Hellmayr, 1905 (Aves: Conopophagidae) / Biologia reprodutiva de Conopophaga roberti Hellmayr, 1905 (Aves: Conopophagidae)

Abstract The Hooded Gnateater Conopophaga roberti Hellmayr, 1905 is an insectivorous understory passeriform with discrete behavior, whose reproductive attributes are poorly-known. In the present study, we describe the reproductive biology of the species and the growth pattern of the nestlings, based on observations conducted in remnants of the Cerrado savanna in eastern Maranhão state, in Brazil. The nests were identified during active searches conducted between June, 2018 and March, 2021. A total of 22 nests were found over the course of three breeding seasons. The cup-shaped nests were supported by small branches and were constructed at a mean height of 40.6 ± 16.1 cm (N = 21) above the ground. The nests were 23.1 ± 3.9 cm in length and 14.1 ± 1.6 cm in width (N = 21). The eggs were beige in color, with irregular brown mottling only at the rounded end of the egg, which had a mean length of 21.3 ± 0.8 mm, width of 17.2 ± 0.8 mm, and mass of 3.1 ± 0.1 g (N = 23). Hatchlings are completely naked and weigh 3.1 ± 0.2 g (N = 7), and when they abandon the nest, they have yet to reach full adult size, with the total length being 65.4% of that of the adult, the wing, 65.4%, the head, 73.9%, the culmen, 74.2%, the body mass, 73.3%, and the tarsus, 89.0% that of the adult. The growth curves are sigmoidal and all the coefficients of determination are at least 0.96, with the body length having the highest value (R2 = 0.98). During the breeding season, the adult pair emitted alarm calls constantly when observers were in the vicinity of the nest. On a number of occasions, members of the breeding pair were observed moving away from the nest as it was approached by observers, while engaging in broken-wing display. The nest architecture, the color of the eggs, and the behaviors presented by this gnateater were similar to those described for other Conopophaga. With this work we contributed to improve the knowledge on the breeding behavior of this poorly know group of understory insectivorous birds.

Resumo O chupa-dente-de-capuz Conopophaga roberti Hellmayr, 1905 é um passeriforme insetívoro de subbosque, de comportamento discreto e cujos atributos reprodutivos são pouco conhecidos. Aqui descrevemos a biologia reprodutiva da espécie e o padrão de desenvolvimento dos ninhegos, com base em observações realizadas em remanescentes de Cerrado no leste do Maranhão, Brasil. Os ninhos foram localizados por busca ativa entre junho de 2018 e março de 2021. Um total de 22 ninhos foram encontrados ao longo de três estações reprodutivas. Os ninhos, em forma de taça, foram sustentados por pequenos galhos e construídos a uma altura média de 40,6 ± 16,1 cm (N = 21) acima do solo. Mediram 23,1 ± 3,9 cm de comprimento por 14,1 ± 1,6 cm de largura. Os ovos são branco-amarelados e mediram 21,3 ± 0,8 mm por 17,2 ± 0,8 mm, com massa de 3,1 ± 0,1 g (N = 23). Ao eclodir, os ninhegos estão completamente nus e pesaram 3,1 ± 0,2 g (N = 7). Abandonam o ninho antes de atingirem o tamanho dos adultos, sendo o comprimento total equivalente a 65,4% do adulto, a asa 65,4%, a cabeça 73,9%, o cúlmen 74,2%, a massa corporal 73,3% e o tarso 89%. As curvas de crescimento apresentaram padrão sigmóide e todos os coeficientes de determinação foram maior ou igual a 0,96, sendo o comprimento do corpo o valor mais alto (R2 = 0.98). Durante o período reprodutivo, o casal adulto emitia alertas constantemente quando os observadores se aproximavam dos ninhos. Em diversas ocasiões foi observado o comportamento de defesa de "asa quebrada" pelo casal reprodutor. A arquitetura do ninho, a cor dos ovos e os comportamentos apresentados por Conopophaga roberti se assemelham aos descritos para outras espécies do gênero. Com este trabalho, melhoramos o conhecimento sobre a biologia reprodutiva deste grupo de aves insetívoras de sub-bosque pouco conhecido.

Desenvolvimento de vacinas profiláticas e terapêuticas para HPV

The correlation between human papillomavirus and induction of cervical cancer has been established since 1974, but the induction of transient idiopathic infertility, attributed to HPV, has also recently been addressed. Therefore, there is a clear need for a more effective combat against the virus, which has periodically been correlated with new diseases that indiscriminately affect men, women, and children of all social classes. Recent technological advances have contributed notably to the improvement of experimental work tools, a fact that provides scientists with new horizons to be explored in the development of new products, free from serious side effects, due to genetic manipulation in the construction of expression vectors directed to therapeutic targets of interest. Thus, this book aims to tell readers a little bit of the natural history about HPV and the advances in prophylaxis and minimally invasive therapy, aiming to eradicate the virus and treat infected patients, who have developed HPV-induced cancer. Periodic exams and vaccination are still the best options to prevent HPV infection and the development of cancers associated with the virus, as well as the possibility of transient infertility occurrence attributed to the HPV.

Desde 1974 foi estabelecida a correlação do papilomavírus humano com a indução de câncer cervical, mas recentemente tem sido abordada também a indução de infertilidade idiopática transiente, atribuída ao HPV. Portanto, fica evidente a necessidade de combate mais efetivo ao vírus, que periodicamente vem sendo correlacionado com novas doenças que afetam indiscriminadamente homens, mulheres e crianças de todas as classes sociais. Os avanços tecnológicos recentes têm contribuído de forma notável para o aprimoramento das ferramentas de trabalho experimental, fato que proporciona aos cientistas novos horizontes a serem explorados no desenvolvimento de novos produtos, isentos de efeitos colaterais graves, devido à manipulação genética na construção de vetores de expressão gênica direcionados a alvos terapêuticos de interesse. Assim, este livro pretende relatar aos leitores um pouco da história natural sobre HPV e os avanços em termos de profilaxia e de terapia minimamente invasiva, visando erradicar o vírus e tratar os pacientes infectados, que desenvolveram câncer induzido por HPV. Os exames periódicos e a vacinação ainda são as melhores opções para se evitar a infecção por HPV e o desenvolvimento de cânceres associados ao vírus, assim como a possibilidade de ocorrência da infertilidade transiente atribuída ao HPV.

Erasing the extinct: the hunt for Caribbean monk seals and museum collection practices / O desaparecimento do que está extinto: a caça às focas-monge-do-caribe e as práticas de acervos museológicos

Abstract The Caribbean monk seal (Monachus tropicalis), the only seal species native to Central America, was declared extinct in 2008, with the last confirmed sighting in 1952. This species historically had a broad range throughout the gulf of Mexico. This article discusses the history of Western science on the monk seal, from its first recorded sighting by a Western colonizer in 1492 to scientific collection in the 1800s and 1900s, as a history of the erasure of this species. Museum practices of collecting and displaying Caribbean monk seals have directly contributed to this erasure, and ways of writing a new history by giving the Caribbean monk seal the capacity to refuse erasure are suggested.

Resumo A foca-monge-do-caribe (Monachus tropicalis), a única espécie de focas nativa da América Central, foi declarada extinta em 2008, tendo o último registro de avistamento confirmado em 1952. Historicamente, essa espécie ocupou vastas áreas do golfo do México. Este artigo discute a história da ciência ocidental sobre a foca-monge, desde o primeiro registro de seu avistamento por um colonizador ocidental, em 1492, até a coleção científica nos anos 1800 e 1900, como uma história de desaparecimento da espécie. As práticas museológicas de coleta e exposição de focas-monge-do-caribe contribuíram diretamente para tal desaparecimento, e aqui são sugeridas maneiras de escrever uma nova história concedendo à foca-monge-do-caribe a possibilidade de recusa ao desaparecimento.

A flora da antiga capitania de Porto Seguro na viagem de Wied-Neuwied, 1815-1817: prática científica, inventário naturalista e colaboração indígena / The flora of the former Captaincy of Porto Seguro in the journey of Wied-Neuwied, 1815-1817: scientific practice, naturalistic inventory, and indigenous collaboration

Resumo O artigo revisita o relato da Viagem ao Brasil (1815-1817) de Maximiliano de Wied na perspectiva de valorizar as contribuições dos registros da expedição do príncipe naturalista para a história natural, especialmente no levantamento da flora do atual extremo sul da Bahia. A abordagem perpassa a análise das características gerais da produção científica no campo da história natural e dos relatos de viagens do século XIX, bem como do perfil biobibliográfico do viajante. O objetivo central é demonstrar como se deu a relação do naturalista com as populações indígenas na produção do seu inventário florístico, destacando a importância dos saberes e fazeres dos índios para o trabalho do naturalista europeu.

Abstract This article revisits Maximilian zu Wied's Viagem ao Brasil (1815-1817) in terms of the value of this naturalist prince's expedition records for natural history, especially the survey of flora in what is currently south Bahia. The general characteristics of scientific production in the field of natural history and nineteenth-century travel reports are analyzed, as well as the bibliographic profile of the traveler. The main objective is to demonstrate how the naturalist's relationship with indigenous peoples emerged in producing the flora inventory, highlighting the importance of indigenous knowledge and practices to the European naturalist's work.

Ampliación de la distribución geográfica y estado de conservación de Ctenoblepharys adspersa (Tschudi, 1845)

Ampliamos la distribución geográfica de Ctenoblepharys adspersa (Liolaemidae), una especie de saurio endémico y amenazado del desierto peruano. Nuestro nuevo registro extiende la distribucion de esta especie en 60 km (línea recta) de la localidad más oriental previamente conocida, la Reserva Nacional de Paracas. Registramos dos tipos de hábitat nuevos para C. adspersa al interior de las estribaciones andinas e identificamos las plantas nativas asociadas a sus hábitats. Además, revisamos el estado de conservación de esta especie y los desafíos que implican su conservación, resaltando que la mayoría de las poblaciones son vulnerables a los impactos en su hábitat producto del desarrollo de infraestructura urbana y/o agrícola.

We extend the geographical distribution of Ctenoblepharys adspersa (Liolaemidae) an endemic and threatened lizard species from the Peruvian desert. Our new record extends the known species distribution ca. 60 km (straight line) east-southeastern from the eastemost record at Paracas National Reserve. We recorded two new type of habitat for C. adspersa that reach to the Pacific foothills and identified the native plants associated to its habitats. Moreover, we review the conservation status and the challenges that facing it, highlighted that most of its populations are vulnerable to the impacts on their habitat caused by the development of urban and agricultural infrastructure.

Amphibians in a transition region between the Cerrado and the Atlantic Forest, west-central region of the state of São Paulo, Brazil / Anfíbios em uma região de transição entre o Cerrado e a Mata Atlântica, Região Centro-Oeste do estado de São Paulo, Brasil

Studies of the diversity and natural history of amphibians are extremely important and contribute with the knowledge about the patterns of geographical distribution and biology of the species, outlining the factors that influence this distribution, and contributing to the development of conservation projects of them. This study aimed to identify the richness, abundance, spatial and temporal distribution of amphibians in the Bauru region, state of São Paulo, Brazil, and compare the species composition with others regions in the São Paulo state located in the Cerrado and Atlantic Forest. Eight locations were sampled between the years 2009 and 2012. Thirty-seven amphibian species were recorded, which represents 15.61% of the species in the state of São Paulo. In comparison to other regions of the state, Bauru resemble with the north-western region of the state and with the Assis Ecological Station in species composition. The community of amphibians in the studied region is regulated to morphoclimatic conditions, environmental heterogeneity and the conservation of natural resources, which directly reflect on the composition, abundance and species' spatial and temporal distribution of the amphibians in this region. The richness of amphibians of the Bauru region is significant for the state of São Paulo and contributes with the maintenance of environments with conditions for the occurrence of Proceratophrys moratoi, which is considered in threat of extinction. This study contributed with the filling gap on the amphibian knowledge of the state of São Paulo.

Estudos sobre diversidade e história natural de anfíbios são de extrema importância para o conhecimento sobre os padrões de distribuição geográfica e biologia das espécies delineando os fatores que influenciam nessa distribuição, o que contribui para a elaboração de projetos de conservação mais realistas e efetivos. Este estudo visou identificar a riqueza, abundância, distribuição espacial e temporal das espécies de anfíbios da região de Bauru, estado de São Paulo, Brasil, e comparar a composição de espécies com outras regiões de Cerrado e Mata Atlântica do estado de São Paulo. Oito localidades foram amostradas entre os anos de 2009 e 2012. Foram registradas 37 espécies de anfíbios, as quais representam 15,61% das espécies do estado de São Paulo. Em comparação com outras regiões do estado, Bauru se assemelha à Região Noroeste do estado e à Estação Ecológica de Assis na composição de espécies. A comunidade de anfíbios da região estudada é regulada pelas condições morfoclimáticas, heterogeneidade ambiental e estado de conservação dos recursos naturais, que refletem diretamente na composição, na abundância e nos padrões de distribuição espacial e temporal das espécies de anfíbios presentes na região. A riqueza de anfíbios da região de Bauru é significativa para o estado de São Paulo e contribui com a manutenção de ambientes com condições para a ocorrência de Proceratophrys moratoi, considerada em risco de extinção. Este estudo contribui com o preenchimento de lacunas no conhecimento de anfíbios do estado de São Paulo.