Appraisal of patient-reported outcome measures in analogous diseases and recommendations for use in phase II and III clinical trials of pyruvate kinase deficiency

Purpose: Pyruvate kinase deficiency (PKD) is a rare disease and understanding of its epidemiology and associated burden remains limited. With no current curative therapy, clinical manifestations can be life threatening, clinically managed by maintaining adequate hemoglobin levels through transfusion and subsequent support, but with frequent complications. Treatment goals are to maintain/improve the patient’s quality of life. With new therapies, reliable, valid, and relevant patient-reported outcome (PRO) tools are required for use in clinical trials. Methods: Systematic literature search identified no current PRO tools for capturing/measuring the impact of PKD and treatments in clinical trials. Therefore, the search strategy was revised to consider conditions analogous to PKD in terms of symptoms and impacts that might serve as parallels to the experience in PKD; this included sickle cell anemia, thalassemia, and hemolytic anemia. Psychometric properties, strengths, and weakness of selected appropriate PRO instruments were compared, and recommendations made for choice of PRO tools. Results: In adult populations, EORTC QLQ C30 and SF-36v2 are recommended, the former being a basic minimum, covering generic HRQoL, and core symptoms such as fatigue. In pediatric populations, PedsQL Generic Core Scale to measure HRQoL and PedsQL MFS scale to measure fatigue are recommended. Conclusions: Some symptoms/life impacts may be unique to PKD and not observable in analogous conditions. A ‘Physico-Psychosocial Model’ derived from the ‘Medical Model’ is proposed to form the basis for a hypothesized conceptual framework to address the development of PKD-specific PRO instruments.Peer reviewedFinal Published versio

Scavenging in changing environments: woody encroachment shapes rural scavenger assemblages in Europe

Rural abandonment and subsequent vegetation regeneration (‘passive rewilding') are expected to increase worldwide, producing cascades of dynamic socioeconomic, landscape and biological changes. Although landscape characteristics strongly influence the structure and functioning of scavengers, little is known about the ecological consequences of passive rewilding due to woody encroachment (i.e.‘landscape closure') on scavenging assemblages. We investigated differences in ‘scavenger assemblage composition' (species richness and abundances) and ‘scavenging efficiency' (scavenging frequency, detection and consumption times and consumption rates) in a mountain agroecosystem (Pyrenees) undergoing passive rewilding. We monitored 178 carcasses in three landscapes: ‘open', ‘shrubland' and ‘forest', and evaluated the effects of landscape type on ‘scavenger assemblage composition' and ‘scavenging efficiency' at the community and species levels, while accounting for the influences of carcass size, type and placement time. We also examined whether the locally most abundant and efficient scavenger (i.e. the griffon vulture Gyps fulvus) affects scavenging patterns. We found that landscape type was the main factor governing scavenging dynamics. Overall and average scavenger richness were similar in open and shrubland landscapes, while forests contained the lowest number of scavengers, mainly comprising mammals. Unlike mammals, avian scavenging frequency decreased as vegetation cover increased, especially for obligate scavengers (i.e. vultures). Scavenger abundances were highest in open landscapes, and carcasses were detected and consumed more rapidly in these landscapes. Carcass size did not influence detection and consumption times, although it did affect average scavenger richness, abundances and consumption rates. Consumption rates were higher in open landscapes and were strongly associated with the presence of griffon vultures. Interestingly, we found that griffon vultures influenced scavenging dynamics via facilitation processes. However, woody encroachment could reduce the scavenging role of this species, while favoring mammalian facultative scavengers. Finally, our findings highlight the pivotal role of griffon vultures, mediated by landscape characteristics, in reducing carcass persistence.POV and AM were funded within the framework of the project RTI2018-099609-B-C22, from the I + D + I National Plan funded by the Ministry of Science, Innovation and Universities. ESG was funded by a Ramón y Cajal fellow (RYC-2019-027216-I)

Concepción de la felicidad en los estudiantes del cuarto año del nivel secundario de una institución educativa de la provincia de Chincha- Ica 2018

La felicidad es concebida de distintas formas: Felicidad sobrenatural, natural y también Materialista, hedonista, relativista. Los medios de comunicación, la familia, las Instituciones Educativas les falta un compromiso serio, con propuestas claras centradas en la persona, con respecto a la búsqueda de la verdadera y auténtica felicidad. Es necesario recalcar que los jóvenes están en una etapa de encontrar el sentido a su vida. Ellos tienen ideales, quieren cooperar con la transformación de la sociedad. Si los jóvenes tienen claro lo que significa la felicidad podrían descubrir de dónde vienen, quiénes son y hacia dónde van, preguntas fundamentales. En este sentido, la naturaleza de la investigación responde a la pregunta: ¿Cuáles son las concepciones de la felicidad que más predominan en los estudiantes del cuarto año de secundaria de la Institución Educativa “Melchorita Saravia” en el año 2018? Se planteó como objetivo: Identificar las concepciones de la felicidad que más predominan en los estudiantes antes mencionados. La investigación fue de tipo positivista, de diseño descriptivo simple, conformado por una muestra de 99 estudiantes del cuarto año de secundaria. Se concluyó que existen concepciones erróneas de la felicidad sobre todo en la postura pragmática del interés con un 45% de acuerdo asumiendo una vida comodona y sin riesgos. Sobre todo tenemos posturas de concepciones verdaderas acerca de la felicidad en la dimensión natural un 61% tienen objetivos claros en su vida y en la postura sobrenatural un 64 % manifiesta que la felicidad está en Jesucristo

Current Practices in the Processing, Diagnosis, and Reporting of Endometrial Carcinoma: Results of a Web-based Survey by the International Society of Gynecological Pathologists (ISGyP)

There have been significant advances in our understanding of the biology and classification of endometrial carcinoma, over the last few years, and the new prediction models proposed for prognostication. To accurately diagnose and stage tumors and apply these prediction models, it is necessary that there be standardized processing of specimens, and a common understanding and usage of the diagnostic terminology of endometrial carcinoma. The International Society of Gynecological Pathologists embarked on an ambitious project to achieve this goal in 2015. An early step in the process was to collect baseline information on existing practices with regard to the processing, diagnosis, and reporting of endometrial carcinomas among the members of the society. This was carried out using a web-based survey comprising 112 questions. The results are presented herein and reveal areas of uniformity but also areas of substantial variation among pathologists. The results of the survey assisted in developing the subsequent recommendations that follow as separate articles in this issue of the journal with regard to processing, diagnosis, and reporting of endometrial carcinomas

The International Society of Gynecological Pathologists (ISGyP) Endometrial Carcinoma Project

Endometrial carcinoma is the commonest gynecological malignancy in developed countries, and the various aspects of the pathology report are critical for patient management. There are many areas of controversy with regard to the handling of resection specimens and the pathologic reporting of endometrial carcinomas. These controversies include those related to sampling, diagnosis, reporting of parameters important for staging, and the undertaking of ancillary studies. These controversies stimulated the International Society of Gynecological Pathologists (ISGyP) endometrial carcinoma project. The project was devised at the ISGyP Board of Directors meeting in March 2015 under the Presidency of Richard Zaino. An organizing committee was selected from the members of the Board of Directors and the education committee of the ISGyP. The organizing committee (comprising the 5 authors of this editorial), as a first step, devised a comprehensive survey, which was emailed to all members of the ISGyP; the survey covered all aspects of endometrial cancer reporting, including specimen dissection and sampling, diagnosis, staging, prognostic factors, and ancillary studies

El Convento del Desierto de Calanda (Teruel, Aragón). Informe histórico-artístico.

El Convento del Desierto de Calanda (Teruel) es un convento barroco de grandes dimensiones perteneciente a la Orden de los Carmelitas Descalzos. Ubicado en un entorno privilegiado de agrestes y bellos paisajes, está formado por iglesia, claustro y dependencias conventuales. Testigo directo de la historia bajoaragonesa, ha sufrido varios incendios y graves destrucciones en contiendas bélicas, además de expolio y vandalismo. Estos hechos, sumados a la desidia y el abandono, han hecho que se encuentre en un estado de ruina y de grave riesgo pese a tratarse de un Bien Catalogado y contar con protección legal, por lo que es necesario poner de manifiesto la necesidad de actuar para su salvaguarda con un plan de conservación y protección

Distributed System for Cognitive Stimulation Over Interactive TV.

Descripción y evaluación de sistema de estimulación cognitiva a través de la TDT orientada a personas con enfermedad de Parkinson, con supervisión por parte de sus terapeutas de forma remota. Abstract: This paper details the full design, implementation, and validation of an e-health service in order to improve the community health care services for patients with cognitive disorders. Specifically, the new service allows Parkinson’s disease patients benefit from the possibility of doing cognitive stimulation therapy (CST) at home by using a familiar device such as a TV set. Its use instead of a PC could be a major advantage for some patients whose lack of familiarity with the use of a PC means that they can do therapy only in the presence of a therapist. For these patients this solution could bring about a great improvement in their autonomy. At the same time, this service provides therapists with the ability to conduct follow-up of therapy sessions via the web,benefiting from greater and easier control of the therapy exercises performed by patients and allowing them to customize new exercises in accordance with the particular needs of each patient. As a result, this kind of CST is considered to be a complement of other therapies oriented to the Parkinson patients. Furthermore, with small changes, the system could be useful for patients with a different cognitive disease such as Alzheimer’s or mild cognitive impairment

Bryoflora checklist of the Palencia provinee (Spain)

FUERTES, E., ACÚN, M., MUNíN, E., OLívA, R. & OLIvÁN, 0. 1998. Catálogo de la brioliora de la provincia de Palencia. Bat. complutensis 22: 113-132. Se presenta el catálogo de 269 briófitos (47 hepáticas y 222 musgos) de la provincia de Palencia; 85 táxones son nuevas aportaciones. El 17 % son cosmopolitas y subeosmopolitas; el 56 % pertenece al elemento circumboreal, el 9 % son atlánticas, subatiánticas y oceánicas y el 18 % son mediterráneas, submediterráneas y mediterráneo-atlánticas. Destacamos la presencia de Lophozia bantriensis, Cephaloziella hampeana, Gymnomitrium obtusum, Scapania paludicola, Sphagnum russowii , Andreaea alpestris, A. rothii subsp. rothii, Polytrichum strictum. Rhizo,nnium pseudopunctatum (B. & 5.) Kop. se excluye de esta provincia.FUERTES, E., ACÓN, M., MUNÍN, E., OLívÁ, R. & OLIvÁN, 0. 1998. Bryotlora check-list of the Palencia province (Spain). BoL Co¡nplu¡ensis 22: 113-132. A Iist of 269 bryophytes (47 hepatics and 222 mosses) from Palencia province is given, 85 taxa are new recorós br Ibis provinee. 17 % are cosmpolitan -anó subeosmopolitan; 56 % of them belong to the circumboreal element, 9 % are atlanties, subatlanties and oceanics and 18 % are mediterraneans, submediterraneans and mediterranean-atánties. We remark <he presence of species such as: Lophozia bantriensis, C?ephaloziella hampeana, Gymnomitrium obtusum, Scapania paludicola, Sphagnum russowii, Andreaca alpesiris, A, rothii subsp. rothii, Polytrichum strictum. Rhizomn¿urn 1,seudo,uunctatuni (B. & 5.) Kop. is excluded from this province

El certamen literario de microrrelato en la formación docente. Un análisis de identidades, temas y personajes

Partiendo de la conceptualización que realiza Van Dijk de la competencia literaria, en la que se concibe no solo como la capacidad de interpretar el hecho literario sino también de producir textos; el Departamento de Didáctica de la Lengua y la Literatura de la Universidad de Cádiz, lleva convocando desde el año 2007 el Certamen Universitario “CAMPUS-MICRORRELATO” con la intención de fomentar la creatividad literaria entre la comunidad universitaria y, en especial, entre los futuros docentes que se forman en la facultad de Ciencias de la Educación. En nuestro estudio se ha realizado un análisis documental de los diversos textos que quedaron finalistas y que aparecen recogidos en las publicaciones que la organización realiza de cada convocatoria del certamen. De esta forma, indagamos en la identidad de género de los participantes, los temas, la caracterización de los personajes, las inquietudes… que los futuros docentes desarrollaron a lo largo de las diferentes ediciones en las que pasaron de ser meros lectores de obras literarias a escritores de una microficción que compartirán en el aula