bluuebunny/crossref_metadata_2025 huggingface dataset:
Escola de tempo integral e ludicidade: os pontos de vista de alunos do 1º ano do ensino fundamental
Authors: Azevedo Nair , Betti Mauro
Published: September 2019

Trata-se de um estudo relacionado à ampliação do ensino fundamental de oito para nove anos no Brasil e à implantação da educação integral no município de Presidente Prudente (SP), que recebeu o nome de programa Cidadescola e seguiu as diretrizes e orientações propostas pelo Ministério da Educação, entre as quais, a recomendação de valorizar os jogos e brincadeiras. Nesse cenário, os objetivos deste estudo são: compreender o programa Cidadescola no âmbito das políticas de ampliação do ensino fundamental e da implantação da escola de tempo integral; investigar os pontos de vista das crianças sobre as atividades desenvolvidas nesse contexto, com especial atenção ao tema da “ludicidade”. Trata-se de uma pesquisa etnográfica, que se valeu de observação participante e de várias estratégias para a coleta de dados em campo. Constatou-se que as crianças privilegiam as relações entre os pares e as práticas lúdicas em atividades que propiciam o movimento. Conclui-se também que, no caso da escola estudada, há risco de uma oposição entre o “período regular” e o “contraturno”, como se fossem duas escolas diferentes na mesma unidade escolar, enquanto as diretrizes didático-pedagógicas presentes na literatura especializada e os documentos oficiais, bem como a perspectiva das próprias crianças, apontam para a necessidade de uma escola integral, portanto, inteira.
Políticas públicas de democratização do acesso ao ensino superior e estrutura básica de formação no ensino médio regular
Authors: Carmo Erinaldo , Chagas José Aercio , Figueiredo Filho Dalson , Rocha Enivaldo
Published: September 2019

É resultado de um trabalho de pesquisa sobre os ensinos médio e superior no País. Aborda o processo de formação na educação secundária e a capacitação deste alunado para o exame de seleção do ensino superior. Descreve e analisa, ainda, as políticas públicas voltadas para a democratização do acesso ao ensino universitário, incluindo o ensino público ofertado por meio das esferas federal, estadual e municipal, com atenção aos resultados comparados com os das instituições particulares. Aqui também são observados os novos desafios enfrentados pelo ensino público, compreendendo os principais mecanismos de acesso e considerando a legislação educacional vigente. Por meio de dados oficiais, são estudados os investimentos públicos em educação, as metas educacionais estabelecidas e a diferenciação entre o ensino da rede pública e o da rede privada, avaliado por meio de exames nacionais. Aqui são identificadas algumas ações fundamentais de valorização e melhoria do ensino médio e do ensino superior, como o investimento na capacitação docente, no incentivo ao discente e no fortalecimento do ensino público.
História da pedagogia no Brasil: a contribuição das pesquisas centradas em manuais de ensino
Authors: Kulesza Wojciech
Published: September 2019

Analisa três linhas de pesquisa, desenvolvidas neste século, focadas em manuais de pedagogia em circulação no Brasil. Após descrever seus instrumentos de pesquisa, é discutida a consistência de seus resultados, particularmente no que se refere às classificações dos manuais elaboradas pelas pesquisas. A partir dos tipos de manuais definidos por essas pesquisas, procura-se avaliar as possibilidades futuras desses programas de pesquisa, especialmente no que tange à ampliação de seus recortes temporais e espaciais. Do ponto de vista da história da pedagogia, argumenta-se sobre a maior potencialidade das pesquisas nessa área cuja categorização dos manuais contemple os modelos escolares historicamente adotados pela escola brasileira.
Esboço de uma compreensão da circulação de ideias: orientações para um estudo das modas educacionais
Authors: Cestari Luiz Artur
Published: September 2019

Este texto parte do anúncio da ocorrência de modismos no campo educacional e tem a intenção de estudar como uma ideia circula e se torna mais efetiva nos espaços de disseminação do conhecimento – revistas de divulgação científica, programas dos cursos de formação de educadores, propostas de políticas públicas para a educação ou ainda dissertações e teses publicadas em cursos de pós-graduação. Apresentamos nosso esboço em dois momentos. Inicialmente, tomados pela afirmação de alguns intelectuais no campo educacional, tentaremos expor por que eles anunciam os modismos, mas têm receio de incluir o fenômeno da moda de ideias no domínio de suas percepções. Posteriormente, indo além da constatação desse fenômeno, delimitaremos orientações para estudar a circulação de ideias no campo educacional e, particularmente, dos modismos educacionais, levando em consideração três contextos: emergência, recepção e disseminação.
Ações inclusivas no ensino superior brasileiro
Authors: Da Silva Jefferson
Published: September 2019

Partindo de uma concepção etnográfica das práticas escolares da Universidade Estadual do Centro-Oeste (Unicentro-PR), constatou-se a dinâmica de grupos relativa às interações e fronteiras simbólicas que permeavam as aprendizagens. Esses processos abrangiam o ciclo espaciotemporal do ensino superior: acesso, permanência e sucesso inseridos em uma comunidade de aprendizagem ampla. Nesse sentido, um programa foi desenvolvido para coordenar as ações que pudessem desdobrar as aprendizagens em termos de medidas pedagógicas inclusivas nas interações pedagógicas, funcionais e administrativas, compreendendo o espaço universitário como uma comunidade de aprendizagem ampla, que oferece diversas fontes para otimizar a formação profissional e acadêmica.
Quando o jogo na escola é bem mais que jogo: possibilidades de intervenção pedagógica no jogo de regras Set Game
Authors: De Carvalho Luciana , De Oliveira Francismara
Published: September 2019

Embasado no aporte da Epistemologia Genética, teve como objetivo identificar e analisar as implicações pedagógicas do jogo Set Game para alunos avaliados com dificuldades de aprendizagem e que frequentam a Sala de Apoio à Aprendizagem, um programa oficial do governo do Paraná. De natureza qualitativa, na modalidade de estudo de caso descritivo-interpretativo, o estudo ocorreu em uma escola estadual da cidade de Londrina (PR). Dele participaram dez alunos frequentadores do programa no ano de 2012, em 18 encontros na modalidade de oficina. Os resultados indicaram a importância de oportunizar, por meio do jogo, a construção de processos cognitivos por alunos que frequentam programas de apoio à aprendizagem escolar.
Agradecimentos
Authors: Executiva Editoria
Published: September 2019

Os números 239, 240, 241 da Revista Brasileira de Estudos Pedagógicos, correspondente ao volume 95, não teriam sido publicados sem a relevante colaboração dos seguintes pareceristas ad hoc (01/08/2013 à 31/07/2014):
Instruções aos Colaboradores
Authors: Executiva Editoria
Published: September 2019

APRESENTAÇÃO Criada em 1944, a Revista Brasileira de Estudos Pedagógicos (Rbep) é um periódico editado em formato impresso e eletrônico e tem periodicidade quadrimestral. Publica artigos inéditos de natureza técnico-científica, resultantes de estudos e pesquisas que contribuam para o desenvolvimento do conhecimento educacional. Seu público-leitor é formado por professores, pesquisadores e alunos de graduação e pós-graduação, técnicos e gestores da área de educação.
Agradecimentos
Authors: Executiva Editoria
Published: September 2019

Os números 242, 243, 244 da Revista Brasileira de Estudos Pedagógicos, correspondente ao volume 96, não teriam sido publicados sem a relevante colaboração dos seguintes pareceristas ad hoc (01/08/2014 a 31/07/2015).
Índice do Volume 96
Authors: Arisawa Elisangela , Pereira Aline
Published: September 2019

Índice do Volume
Instruções aos Colaboradores
Authors: Executiva Editoria
Published: September 2019

Instruções aos Colaboradores
En los márgenes de la artisticidad
Authors: González Roblero Vladimir
Published: September 2019

«En los márgenes de la artisticidad» es un ejercicio autoetnográfico en el que reflexiono sobre las cualidades del arte y su articulación a mi práctica académica en la docencia, la investigación y la escritura. Considero que en el espacio universitario ha habido la intención de domesticar al arte con criterios de cientificidad. De manera contraria, me pregunto si en el trabajo de investigación suceden operaciones cercanas a la creación artística. Identifico, entonces, un espacio fronterizo entre el arte y la ciencia que orienta mi práctica de la investigación/creación.
Anacronismos visuales
Authors: Cortese Julia
Published: September 2019

El texto busca realizar un aporte a los marcos para pensar las obras de los artistas contemporáneos latinoamericanos. Se las aborda desde sus complejidades específicas, lo que demanda un corrimiento de relatos universales, homogéneos y dogmáticos. En reiteradas ocasiones, estos se hacen visibles en categorías de análisis, conceptos y jerarquías heredadas del proceso de conquista y de colonización del continente. Entendiendo las obras como relatos situados, el análisis se estructura a partir de un caso particular, el Quipu menstrual, de Cecilia Vicuña, para indagar en cómo resignifica esa obra rasgos formales del pasado ancestral precolombino. De este modo, se comprenden esas supervivencias como un aporte a las teorías decoloniales.
Los cuerpos que no de la historia del arte. O cuando aprendemos solo el relato cisheteronormado
Authors: Correbo María Noel
Published: September 2019

La historia de las artes visuales en su relato sobre la representación de los cuerpos dejó por fuera identidades disidentes a la norma heteropatriarcal. Su construcción institucionalizada escindió cuerpos que importan de otros que no, siendo funcional al sistema hegemónico capitalista y colonial también en el ámbito educativo. Operar de forma binaria, en los mecanismos de su escritura y en la inscripción de género de los cuerpos, reprodujo una imagen moralizante con la que subjetivamos socialmente nuestra corporalidad. Los activismos contemporáneos desactivaron la norma incluso entre teoría y práctica, y la ley de Educación Sexual Integral habilitó otras posibilidades de existencia.
El diseño y las personas. Elementos de una teoría del contexto proyectual
Authors: Marincoff Gustavo A.
Published: September 2019

La formación para la actividad proyectual como disciplina científico-tecnológica debe entrenar al futuro profesional para responder adecuadamente frente a las demandas de la Cuarta Revolución Industrial y la sociedad del conocimiento que le sirve de matriz. Dado que el desempeño profesional del diseñador tiene que evolucionar en concordancia con el contexto en el que se despliega, la formación del futuro profesional del diseño debe incluir herramientas conceptuales que le permitan detectar, identificar, evaluar y, en lo posible, anticipar las transformaciones en su contexto de desempeño, con el propósito general de brindarle los medios para una respuesta adaptativa al cambio.
Desplegar teorías. Diálogo con Guillermina Mongan
Authors: Ruvituso Federico , Delle Donne Sofía
Published: September 2019

En la siguiente entrevista, la historiadora del arte Guillermina Mongan debate sobre el cruce entre la teoría y la práctica a partir de la muestra La teoría como acción (Parque de la memoria, 2018) en la cual se desempeñó como asistente en curaduría e investigación, bajo la curaduría general de Ana Longoni. En este diálogo, Mongan realiza un recorrido por la utilización del espacio expositivo, la relación obradocumentos- archivos y la vinculación de la Historia del Arte con el arte contemporáneo.
Oro digital en el capitalismo tardío. La producción de riqueza basada en datos
Authors: Dalponte Guido
Published: September 2019

Silicon Valley no es solo un área tecnológico industrial del norte de California, también representa un espíritu, una ideología y un modelo productivo para el resto del mundo, maquillado de valores legítimos como el de hacer del mundo un lugar mejor y atravesados por el espíritu individualista del progreso capitalista y la filosofía new age. La silicolonización del mundo aborda la historia, las características socioculturales y algunas de las consecuencias a mediano plazo del proceso de construcción de esta nueva hegemonía mundial.
Plataformas y aplicaciones. Nuevas tendencias para la economía digital
Authors: Vallefín Juan Cruz
Published: September 2019

En la siguiente reseña se señalan los aciertos de Nick Srnicek al postular un capitalismo de plataformas junto con las limitaciones metodológicas que implica centrarse en el accionar del capital en vez de la fuerza de trabajo social. Asimismo, se indican posibles recorridos teóricos a realizarse —tanto a nivel laboral como político—, enfocando el análisis desde el trabajo en vez del capital como principio metodológico.
El arte de materilizar un método. Sus preguntas y certezas
Authors: Rodríguez Maité Soledad
Published: September 2019

El Método Diana Aisenberg es el fruto del trabajo de su autora homónima. Durante más de treinta años, la artista y docente ha formado a sus pares y ha influido positivamente en la producción y carrera de muchos de los que han transitado por su taller. Nos concede afirmaciones sinceras y sentidas en cuanto función del docente, siendo su antirreferente la formación formal en su sentido restrictivo. Su método es un trabajo en proceso que tensiona los límites e invita a repensar la praxis artística.
Sobre el lenguaje y la tarea filosófica
Authors: Iannone Rocío
Published: September 2019

En el libro ¿Qué es la filosofía? (2016), Giorgio Agamben aborda la tarea filosófica con relación al lenguaje utilizando el concepto de la voz, lo decible, la exigencia, lo proemial y lo musaico. Se detiene especialmente en los pensamientos platónico, aristotélico y estoico, para reinterpretarlos. Agamben define la filosofía mediante la tarea filosófica que ocupa un lugar transformador de la realidad y nos interpela desde las diferentes formas de pensar y llevar a cabo el lenguaje.
Cómo pintar la pampa
Authors: Macario María Teresa
Published: September 2019

El artículo resume los objetivos y los avances del proyecto de tesis para la Licenciatura en Historia del Arte de la Facultad de Bellas Artes (FBA) de la Universidad Nacional de La Plata (UNLP), titulado Experiencia y representación pampeana. Aportes para la comprensión del género paisaje a partir del estudio de la obra de Eduardo Sívori. La investigación fue financiada por el programa de becas Estímulo a la Vocación Científica, del Consejo Interuniversitario Nacional. Fue presentado y aprobado en 2018 y su escritura sigue en curso.
El arte en los museos memoriales
Authors: Dubois Pamela Sofía
Published: September 2019

Esta investigación se propone analizar las representaciones de los acontecimientos generados por el terrorismo de Estado de la última Dictadura Militar (1976-1983). Toma como caso de estudio las representaciones en museos memoriales donde las prácticas artísticas activan las narrativas de la memoria social sobre el pasado reciente. Se considera que esas representaciones aportan a los reclamos contemporáneos de verdad, justicia y reparación, insertas en espacios que actúan como lugares de memoria consagrados al recuerdo de las víctimas, y a su transmisión intergeneracional. El caso específico a estudiar es el Museo de la Memoria de la ciudad de Rosario, provincia de Santa Fe.
Calcos medievales en la facultad de bellas artes. Investigación histórica e iconográfica
Authors: Vernieri Julieta Zulema
Published: September 2019

En este artículo presentamos el recorrido realizado en una tesis de grado en la cual se realiza una investigación histórico-artística del tipo iconográfico al que pertenece un conjunto de calcos de la colección del Área Museo, Exposiciones y Conservación de la Facultad de Bellas Artes (FBA) de la Universidad Nacional de La Plata (UNLP). Se trata de tres calcos realizados en yeso por el taller de moulage del Museo de Escultura Comparada de París, cuyos originales son relieves correspondientes al ciclo calendario esculpido en uno de los portales de la catedral Notre-Dame de París.
Platelet functional activity: physiology and laboratory diagnostic methods
Authors: Ponomarenko E. A. , Ignatova A. A. , Fedorova D. V. , Zharkov P. A. , Panteleev M. A.
Published: September 2019

Platelets perform numerous important functions not only in the process of normal functioning of hemostatic system, but also in other physiological processes, such as: vessel wall integrity regulation, wound healing, inflammatory response. Its malfunction can be found in various diseases and conditions (including oncohematological disorders, solid tumors, inflammatory diseases, sepsis, autoimmune disorders), is triggered by injury or medications and can lead to dangerous consequences, such as bleeding and thrombosis. However, platelets functional activity quantity assessment tools are extremely limited, the perception what platelet functional activity is about is also quite unclear. This review considers platelets function, its' abnormalities, possibilities for its' assessment by existing methods as well as promising directions for their development.
A modern view on the complement system
Authors: Shakhidzhanov S. S. , Filippova A. E. , Butilin A. A. , Ataullakhanov F. I.
Published: September 2019

Complement is a part of the immune system which provides host cells with protection against pathogen cells and particles. It is activated when a pathogen invasion is detected. The results of numerous investigations have led to growing realization of the important role of this system in maintaining normal organism homeostasis. This review summarizes a modern view on the complement system.
Relevance and management of secondary hypogammaglobulinema in clinical practice
Authors: Balashov D. N.
Published: September 2019

Advance protocols for the treatment most of oncology, hematology and some inherited disorders may lead to development severe secondary hypogammaglobulinaemia. Particularly, it is often caused by therapy with monoclonal antibodies binding B-cells (such as rituximab, belimumab, inotuzumab), therapy with inhibitors of tyrosine-kinase (imatinib, desatinib), as well as applying a variety of immunosuppressive and chemotherapy agents (steroids, azathioprine, cyclophosphamide etc.). It should be note, that chronic lymphoid leukemia and multiple myeloma could be complicated with hypogammaglobulinaemia, not only because of specific therapy, but also as features of the diseases. Hematopoietic stems cells transplantation can also lead to development severe and prolonged hypogammaglobulinaemia. This is associated with intensive immune/myeloablative therapy, as well as with immunologic reconstitution after transplantation. Modern intravenous immunoglobulins (IVIG) have a wide repertoire of pathogen-specific activity with high safety profile and constitute essential part of therapy patients with secondary hypogammaglobulinaemia. The paper presents literature review of IVIG usage in various clinical situations, as well as several clinical examples of personal experience.
Epilemiological characteristics of Diamond–Blackfen anemia in pediatric population of the Russian Federation
Authors: Chernov V. M. , Ovsyannikova G. S. , Yudina M. B. , Rogov A. V. , Sokolova N. E. , Shamin A. V. , Suriyaninova O. V. , Spichak I. I. , Burlutskaya T. I. , Leandrova L. A. , Chizhkova A. N. , Kostyurina T. L. , Yunusova I. M. , Kumikova Zh. Kh. , Burnatseva M. V. , Popova N. A. , Shapochnik A. P. , Bunina E. G. , Ponomareva I. V. , Galiulin R. Kh. , Mazurok L. A. , Ksenzova T. I. , Gushchanskaya I. I. , Fokin V. N. , Strelets V. G. , Volodicheva E. M. , Tsedenisheeva E. Kh. , Novichkova G. A. , Smetanina N. S.
Published: September 2019

Diamond–Blackfen Anemia (DBA) is a rare, clinically and genetically heterogeneous disorder from the group of congenital syndromes of bone marrow failure. The purpose of this work is to identify the main medical-frequency characteristics of DBA (incidence, prevalence, mortality, cartographic analysis) in children in the Russian Federation during the observation period 2011–2016. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The Russian register of DBA patients, which had been developed and maintained since 2012 year by NMITS DGOI, was used to obtained epidemiological data and their analysis. At the end of 2018 year 141 patients from 137 families were included in the register. The average annual incidence rate of children with DBA for the Russian Federation was 0.63 ± 0.034, the average annual prevalence rate – 5.75 ± 0.87 per 100 thousand newborns born alive; mortality rate – 2.12%; the cartographic method of research showed that the largest number of patients was registered in the Central Federal District of the country, which is explained by the high population living in this district.
Peculiarities of hemostasis in infants with congenital heart diseases in the first 12 hours of their life
Authors: Shumakova O. V. , Morozova N. Ya. , Burov A. A. , Podurovskaya Yu. L. , Bockeriya E. L. , Nefedova I. E. , Berishvily D. O. , Degtyarev D. N.
Published: September 2019

The article is focused on the main parameters hemostasis and platelet count of newborns with congenital heart diseases who could require an early cardiac surgery during the first 12 hours of their life. Some known disorders of hemostasis in children with congenital heart diseases are described. A clinical case of the thrombotic complications in the child after cardiac surgery are presented, the tactic of drug therapy is described. The study was approved by the Independent Ethics Committee of the V.I. Kulakov Obstetrics, Gynecology and Perinatology Research Centre.
Immunophenotypic characterization of acute megakaryoblastic leukaemia in children
Authors: Alexenko M. Yu. , Illarionova O. I. , Verzhbitskaya N/ Yu. , Zerkalenkova E. A. , Novikova I. A. , Panferova A. V. , Fechina L. G. , Tsaur G. A. , Olshanskaya Yu. V. , Popov A. M.
Published: September 2019

Acute megakaryoblastic leukemia (AMKL) is a rare subtype of acute myeloid leukemia, in which the bone marrow produce increased numbers of immature abnormal megakaryoblasts. AMKL is rare both in children and adults, but is the most frequent subtype of acute myeloid leukemia (AML) in children with Down syndrome. Morphological diagnosis of this disease could be complicated, thus flow cytometry plays a crucial role in the diagnostics of AMKL. The aim of the present study was to investigate the immunophenotypic characteristics of AMKL in children. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The study group included 103 patients with AMKL. Antigen expression profile was assessed by multicolor flow cytometry. We identified three groups of patients according to different levels of CD45 expression, and in majority of patients (74%) high level of CD45 expression was detected. Significant immunophenotypic differences between these groups were found. In 56% of patients trisomy of 21 chromosome was detected. Among these patients, 86% belonged to group of high CD45 expression. Moreover, children with trisomy 21 represented the majority in the group with high level of CD45 expression (64%). Also, there were found several significant differences between patients with and without trisomy 21 within the group of high CD45 expression. This study demonstrated the wide immunophenotypic heterogeneity of AMKL. In general, the revealed diversity obviously reflects the biological heterogeneity of this AML subtype.
Fisher-Evans Syndrome in Children: an Analysis of Genetic Defects and Therapy Response
Authors: Kuzminova Z. A. , Pashanov E. D. , Pavlova A. V. , Kurnikova M. A. , Mersiyanova I. V. , Plyasunova S. A. , Smetanina N. S.
Published: September 2019

The Fisher–Evans syndrome (FES), also known as Evans syndrome (ES) is an immune hemopathy characterized by bicytopenia or pancytopenia in the absence of other diseases. FES may be either primary/idiopathic, or secondary, occurring against the background of other diseases. In children, FES is frequently related to a primary immunodeficiency disorder (PID). This article presents a retrospective data analysis of a cohort of pediatric patients (n = 14) with FES, the aim of which is to demonstrate the necessity of conducting a molecular genetic investigation for PID diagnosis. The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. The patients from this cohort were presented at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology during the period 2012–2019. All of these patients underwent Next Generation Sequencing (NGS). The median age of disease onset was 5.5 years (from 5 months to 16 years). The male-to-female ratio was 1.8:1. Thanks to the results of the genetic investigation conducted on 14 patients with FES, it was possible to identify 10 children (71.4%) who had secondary FES. The median age of disease onset in patients with secondary FES was 4 years, the minimum age was 5 months, the maximum age was 12 years. The male-to-female ratio was 7:3. Most frequently, the disease onset was associated with ITP, immune thrombocytopenia (35.7% of patients) or simultaneously with ITP and AIHA, autoimmune hemolytic anemia (35.7% of patients); less frequently, the disease onset was associated with AIHA, with a subsequent occurrence of ITP (21.4% of patients). A pancytopenia was identified in 1 patient with FES associated with a PID. Among our patients with a disease onset associated with AIHA, there was not a single case with a PID. We noted an extremely low effectiveness of glucocorticoids and IVIgs for the treatment of FES: in practically 100% of cases, the patients in the analysed group required second and subsequent lines of therapy. Among other therapy types, effective for both primary and secondary FES, we should note rituximab (62% of patients stay in remission for up to 1 year) and MMF (80% of patients stay in remission for up to 1 year). On the basis of the conducted study we can conclude that male patients with FES under 18 years of age whose disease onset was associated with ITP need to be thoroughly tested for PID, including genetic testing, since patients in this group have the greatest PID incidence. Treating FES with glucocorticoids and IVIgs is nearly always not very effective and second and subsequent lines of therapy are required. The drugs of choice which are most effective for patients with FES are rituximab and MMF.
Combined treatment of diffuse sclerosing osteomyelitis of mandible in children
Authors: Kugushev A. Yu. , Lopatin A. V. , Yasonov S. A. , Sinitsyn D. S.
Published: September 2019

Diffuse sclerosing osteomyelitis of the jaw (DSO) is a rare and poorly understood disease. Nowadays treatment protocols (steroid or painkillers, corticotomy) do not capable of to get good and stabile results or are associated with the development of potentially serious side effects. The treatment algorithm developed by us, including the course treatment with bisphosphonates, was well tolerated and capable of to get clinical and radiological remission in the case of previous decortication and antibacterial therapy was done. For these reasons, this treatment protocol is not without interest and need of further investigates. The study was approved by the Local Ethics Committee of Russian Children's Clinical Hospital.
ANKRD26-related thrombocytopenia: case report and literature review of inherited thrombocytopenias with predisposition to malignancies
Authors: Erdomaeva Ya. A. , Fedorova D. V. , Zharkov P. A. , Kurnikova M. A. , Mann S. G. , Raykina E. V.
Published: September 2019

ANKRD26-related thrombocytopenia (previously known as thrombocytopenia-2) is a rare form of inherited platelet disorders. Patients with ANKRD26-related thrombocytopenia usually do not suffer from severe bleeding but have predisposition to acute myeloid leukemia and other malignancies. Patients with ANKRD26-related thrombocytopenia and their relatives need genetic consultation and long term follow-up in view of risk of malignant blood disorders. The clinical case of ANKRD26-related thrombocytopenia in two siblings is presented in this paper. Review of literary data on pathogenesis, treatment and follow-up of patients with ANKRD26-related thrombocytopenia is performed. Common questions of diagnosis and management in patients with congenital thrombocytopenias with predisposition to malignant blood disorders are also reviewed. Parents gave their permission for using personal data for clinical research and publications.
Family case hemolytic anemia due to abnormal unstable hemoglobin with low oxygen affinity (Hb Cheverly)
Authors: Krivosheina E. L. , Koval N. Yu. , Egorova E. N. , Gorshkova M. A. , Karamyan N. A. , Litvin E. A. , Mann S. G.
Published: September 2019

Hereditary hemolytic anemia caused by unstable hemoglobin is a rare pathology, characterized by variability of clinical manifestations. This disease is characterized by the hemolytic crises, which are frequently associated with infections or taking drugs that cause hemolysis. Age of debut depends on which of the globin chains mutation occurs. Among these diseases, hemolytic anemia associated with the presence of a structurally abnormal unstable hemoglobin with low oxygen affinity in the erythrocytes is a special group. With this type of abnormal hemoglobin, pulse oximetry demonstrates falsely low oxygen saturation of the blood, with increased oxygen delivery to the tissues. It is important to identify unstable hemoglobin in order to avoid the misinterpretation of low oxygen saturation during the pulse oximetry in emergency cases, surgeries or intensive therapy. The article presents an analysis of the family with hereditary hemolytic anemia due to unstable hemoglobin Cheverly. The main clinical and diagnostic markers of the disease are described in detail. Parents gave their permission for using personal data for clinical research and publications.
Vitamin B12-deficiency anemia in infants: clinical presentation, current diagnostic and treatment
Authors: Lunyakova M. A. , Demikhov V. G. , Dronova S. N. , Kalinina Yu. Yu. , Zhurina O. N.
Published: September 2019

Vitamin B12 (cobalamin) deficiency in infants exclusively breastfed is usually the result of its deficiency in the mothers (vegetarians or in the presence of unrecognized pernicious anemia or malabsorption syndrome). Cobalamin is crucial for the normal development of the nervous system, the production of erythrocytes and the synthesis of DNA. The classic manifestation of its deficiency is megaloblastic anemia and, in advanced cases, pancytopenia. However, infants often present neurological symptoms (fatigue, failure to thrive, regress of developmental skills, lethargy, motor disorders), as a consequence of inadequate myelination of the brain. Cobalamin treatment is effective in the most children, however, the neurological deficit varying degrees may persist. The long-term prognosis depends on overall duration of vitamin B12 deficiency and severity of symptoms. The article presents our own interesting clinical observations. Parents gave their permission for using personal data for clinical research and publications.
Syndromе associated with multiple venous malformations (Bean syndrome)
Authors: Hachatryan L. A. , Kletskaya I. S. , Orekhova E. V.
Published: September 2019

Blue rubber bleb nevus syndrome (BRBNS) also called Bean's syndrome is a rare disorder characterized by mucocutaneous angiomatose multiple cutaneous venous malformations. Pathogenesis of the BRBNS is caused by the somatic mutations in angiopoietin receptor gene TEK. These mutations cause ligand-independent activation of the tyrosine kinase receptor and the PI3K / AKT pathway and involve the mTOR in the pathological process in a specific way. There are no universally effective methods for treating systemic forms of vascular malformations currently. However, recent numerous reports have shown the advantages of rapamycin, an mTOR inhibitor, as a well-tolerated and effective therapy in patients with vascular abnormalities, in particular with diffuse angiomatose. This article presents a clinical case of a 5-years old patient with a diagnosis of iron deficiency anemia, who has been treated for a long time and received iron supplements therapy without achieving any effect. The diagnosis of Bean's syndrome was established after 5 years from the first clinical manifestation and based on clinical, laboratory and instrumental methods. The cause of iron deficiency anemia was established. It was repeated chronic bleedings from multiple sites of the gastrointestinal mucosa vessels. Prescribed rapamycin therapy allowed to contain gastrointestinal bleeding, to reduce the size of vascular lesions and to prevent the formation of new ones. Parents gave their consent to use information about the child in the article.
Invasive aspergillosis in children in debut of acute myeloid leukemia
Authors: Dinikina Y. V. , Shadrivova O. V. , Belogurova M. B. , Dokhina N. N. , Khostelidi S. N. , Ignatyeva S. M. , Bogomolova T. S. , Klimko N. N.
Published: September 2019

Invasive aspergillosis (IA) is a life-threatening complication in pediatric patients with hematological malignancies. The highest incidence rates of IA were found in pediatric patients with acute myeloid leukemia (AML). We presented a case of successful treatment of IA in a patient with debut of AML with Down syndrome, analysis of IA cases in children with AML according data of the register and a literature review. 46 pediatric patients with IA were included, among them 16 (34.7%) with AML. In 3 (18.75%) pts AML was diagnosed in presentation of AML. Risk factors of IA were prolonged neutropenia (100%) and lymphocytopenia (81%). The most common clinical signs were: fever > 38.5 ºС (93.7%), cough (75%) and respiratory failure (43.7%). The lung involvement was diagnosed in 93.7% of pts, more than 2 sites of IA – in 12.5%, endophthalmitis – in 6.25%. Typical imaging finding was «halo sign» – 37.5%. According received results «probable IA» was diagnosed in 87.5% of pts and «proven» in 12.5%. Antifungal treatment received 100% of patients, with voriconazole only – 50%, combined antifungal therapy – 33.3%. Overall 12-week survival was 87.5%. Parents patients agreed to use personal data in research and publications.
Clericusio syndrome (poikiloderm with neutropenia)
Authors: Deordieva E. A. , Shvets О. A. , Serova E. S. , Pavlova A. V. , Raykina Е. V. , Plyasunova S. A. , Mandzhieva A. I. , Pshonkin A. V. , Shcherbina A. Yu.
Published: September 2019

Clericusio syndrome is a rare genetic disease belonging to the group of congenital neutropenias, first described by S. Clericuzio et al. in 1991, in 14 patients from the Navajo tribe. It is characterized by poikiloderma and neutropenia, with recurrent infections and a high risk of developing myelodysplastic syndrome. Approaches to therapy are not optimized due to the rarity of this pathology. The article provides an overview of the data on the disease available to date, and discusses the case of Clericusio syndrome in a 5-year-old boy, from an early age observed in Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology. Parents gave their permission for using personal data for clinical research and publications.
The Moderating Effect of Organizational Characteristics on The Relationship Between Information Technology Integration and Performance: Empirical Evidence from Selected Public Hospitals in Kenya
Authors: Malongo Kenneth Iloka , Muathe Stephen , Waithaka Stephen Titus
Published: September 2019

With the continuing digital revolution steered by the Internet, organizations are moving towards information technology integration to improve their performance. Regrettably, these developments have in no way been all-inclusive. The health gap between public institutions in first, second and third world nations has broadened. Public organizations in second and third world nations are characterized by poor performance. This study sought to establish the moderating effect of organizational characteristics on the relationship between information technology and performance of public hospitals in Kenya.  The study used Technology Organization Environment (TOE) theory. The study was guided by explanatory and cross-sectional research design. The target population was 98 public hospitals in Kenya. Multi-stage sampling technique was used to select a sample size of 294 respondents. Primary data was collected using semi-structured questionnaires. For data analysis, descriptive statistics and multiple regression analyses were used. The study results established that organizational characteristics moderated the relationship between information technology integration and performance of public hospitals in Kenya. Therefore, study concluded that organizational characteristics play a major role in an organization’s adoption and utilization of information technology integration. The study recommends technologies should be customized to fit the type of organizational characteristics for better performance.
Metabolite Profiling of 96% Ethanol Extract from Marsilea crenata Presl. Leaves Using UPLC-QToF-MS/MS and Anti-Neuroinflammatory Predicition Activity with Molecular Docking
Authors: Ma’arif Burhan , Denis  , Suryadinata Arief , Artabah  , Laswati Hening , Agil Mangestuti
Published: September 2019

Phytoestrogen is a group of compounds that can replace the estrogen function in the body. One of its roles was as anti-neuroinflammatory by inhibiting the microglia M1 polarity activation. Marsilea crenata Presl. is a plant that suspected to contain phytoestrogens. The aim of this research was to determine the metabolite profile of 96% ethanol extract of M. crenata using UPLC-QToF-MS/MS, and prediction the anti-neuroinflammatory activity of compounds with molecular docking. The 100 ppm of 96% ethanol extract in DCM and methanol were injected 5 µl each into the UPLC-QToF-MS/MS, and then analyzed by Masslynx 4.1 software to determine the compounds. The result of metabolite profiling shows a total 59 compounds in both DCM and methanol. Molecular docking was done with Autodock 4.2.6. After being analyzed, there are 3 compounds that are predicted to have activities similar to 17β-estradiol, they are prochlorperazine, 12-Aminododecanoic acid, and 1-methyl-2-[(4-methylpiperazin-1-yl)methyl]benzimidaol-5-amine hydrochloride. The results showed that the three compounds were predicted to be phytoestrogen compounds from M. crenata leaves, which have potential as anti-neuroinflammatory.
ANALYSIS ANALYSIS OF CAFFEINE IN TABLET DOSAGE FORM WITH SPECTROPHOTOMETRIC AND IODOMETRIC BACK TITRATION METHODS
Authors: Edy Agustian Yazid
Published: September 2019

Caffeine (1, 3, 5-trimethylxanthine) is an active ingredient that is often added to analgesics drugs to teat headaches or reduce pain. This study aims to quantitatively analyze caffeine levels in tablet dosage forms by spectrophotometric methods and iodometric back titration. In the spectrophotometric method the measurement of caffeine levels was carried out using a maximum wavelength of 272 nm.The results of caffeine levels were found for bodrex drugs (50.87 ± 0.195 mg/tab), extra panadol (64.92 ± 0.579 mg/tab), saridon (51.38 ± 0.273 mg/tab) and paramex (46.78 ± 0.072 mg/tab). The purity percentage of caffeine was found in the range between 93.57-102.76%. While the iodometric back titration method was obtained for bodrex (48.04 ± 0.889 mg/tab), extra panadol (64.45 ± 0.697 mg/tab), saridon (43.38 ± 0.756 mg/tab) and paramex (42.36 ± 0.889 mg/tab) with a purity range of 84.73-99.15%. The average caffeine content of the two method extant suitability close to the true value as stated on the label. The results of the spectrophotometric method are more accurate, while the titrimetry method is still good to use because it is cheaper and only requires simple apparatus and common chemicals.
Iohexol-Induced Acute Generalized Exanthematous Pustulosis (AGEP): Case Report
Authors: Lerman Irina , Sipprell William H. , Scott Glynis A. , Richardson Christopher T.
Published: September 2019

Acute generalized exanthematous pustulosis (AGEP) is a rare adverse drug reaction characterized by numerous non-follicular sterile pustules overlying edematous, erythematous plaques. The majority of AGEP cases are associated with antimicrobial medications, although other agents and etiologies have also been implicated. Here, we report a patient with recurrent angioimmunoblastic T-cell lymphoma (AITL) who presented with a pruritic and widespread pustular eruption one week following computed tomography (CT) with iohexol contrast administration. Notably, she had a documented prior mild reaction to contrast medium and was appropriately pre-medicated with diphenhydramine and prednisone before imaging. Biopsy revealed intra-epidermal pustules, epidermal spongiosis, and dermal infiltrate of lymphocytes, neutrophils, and some eosinophils – histological findings consistent with AGEP. Systemic and topical corticosteroids plus topical mupirocin resulted in complete resolution of symptoms. While cutaneous reactions to iodinated contrast are common and often self-limited, severe manifestations such as AGEP must be considered particularly in patients with a history of prior contrast allergy.
Sterile Neutrophilic Folliculitis with Vasculopathy in a Young Male Patient with Infective Endocarditis
Authors: Barrick Carl , Chea Emily , Gupta Naeha , McClain Richard , Purcell Steven
Published: September 2019

Sterile neutrophilic folliculitis with vasculopathy is a rare entity histopathologically characterized by neutrophilic or suppurative and granulomatous folliculitis accompanied by a folliculocentric vasculopathy.1 It is described as a cutaneous manifestation of a systemic illness or infectious trigger, and its clinical features vary independently from the underlying medical illness.1 Prompt identification may uncover an underlying systemic disease. Due to the rarity of this clinical entity, there is a paucity of evidence regarding its etiology, diagnosis, and treatment recommendations. We present a case of sterile neutrophilic folliculitis with vasculopathy in a 34-year-old male hospitalized for infective endocarditis.
SkinSpecs: A Mobile Solution that Addresses an Unmet Need for Tracking Chronic Skin Diseases in the Office and at Home
Authors: Afanasiev Olga K , Tabata Mika , Narla Akhila , Scott Gregory , Ko Justin M
Published: September 2019

Introduction: Currently there are no portable solutions to robustly document and longitudinally monitor dynamically changing chronic skin conditions. This study set out to engineer and test a mobile-based 3D imaging solution for chronic skin diseases to enhance clinical workflow and patient care.Methods: SkinSpecs uses smartphone-captured videos of patients’ skin disease and renders 3D true-to-life models that were evaluated by Stanford Health Care dermatologists.Results: We utilized video input to accurately reconstruct interactive 3D models of 16 different skin conditions from 31 patients. Assessment of SkinSpecs 3D reconstruction is faster (p<0.05) compared to descriptive exam, standard photographs or original videos. Dermatologists maintained highest accuracy, confidence and satisfaction with 3D reconstruction.  Dermatologist preferred SkinSpecs for documentation over other capture modalities. SkinSpecs was favorably used by dermatologists, with high satisfaction with resolution, breadth of visual information, time and ability to pick up incidental findings.Conclusion: We identified a proof-of-concept solution to objectively and robustly capture skin disease, with an office and home workflow that is acceptable to providers and patients. This fast, scalable method is deployable on smartphones and could be utilized to augment clinical decision making in the clinic and to empower patients at home.
Dermoscopy of Inflammatory Linear Verrucous Epidermal Nevus: Brown and Red Glomerular Structures Over a White Background as an Identifying Feature
Authors: Dickman Jeffrey , Noparstak Michael , Nathoo Rajiv
Published: September 2019

Inflammatory linear verrucous epidermal nevus (ILVEN) is an uncommon variant of keratinocytic epidermal nevus that typically presents as linear erythematous and verrucous papules which often coalesce into plaques. ILVEN is characteristically intensely pruritic and is most commonly found unilaterally on an extremity, the trunk, or buttock in a Blaschkoid pattern. Lesions may be present as early as birth most commonly developing in early childhood before the age of five years old and persisting for months to years. Females are more often affected. We present the case of an adult patient who presented to our clinic for evaluation of her long-standing, treatment-resistant ILVEN. Dermoscopy was used to aid in the diagnosis and exhibited alternating red and brown glomerular-type structures over a white background. We propose that these findings may be added to other previously documented dermoscopic characteristics of ILVEN, as they correspond to known histopathological features of the dermatosis and may aid in non-invasive diagnosis.
Indian Surgeons’ Perspective Regarding Breast-Conserving Surgery: A Cohort Study
Authors: Bothra Sapana , Sabaretnam Mayilvaganan , Chand Gyan , Mishra Anjali , Agarwal Gaurav , Agarwal Amit
Published: September 2019

Objective:

Breast-conserving surgery (BCS) has constantly evolved and recently involves many new facets in the form of SLNB, breast oncoplastic surgery, and brachytherapy. Breast surgeons are required to constantly unlearn and learn to keep abreast with the new guidelines. We aimed to study the Indian surgeons’ perspective about BCS.

Participants:

We mailed a questionnaire containing 20 questions regarding various aspects of BCS to 1200 surgeons. of these, 112 surgeons (40 endocrine surgeons, 40 surgical oncologists, and 32 general surgeons) responded.

Results:

We found that surgeons in the 31 to 40-year age group, surgeons with superspecialty training, surgeons working in private setup, and high-volume surgeons (>100 cases/year) were performing more BCS.

Conclusion:

Indian surgeons should have more robust training in BCS to increase the rates of BCS in India.
Real World Outcomes of Check Point Inhibitors Immunotherapy in Renal and Urothelial Cancers in a Teratiary Care Cancer Center in India
Authors: Talwar Vineet , Bothra Sneha Jatan , Goel Varun , Dash Prasanta Kumar , Jajodia Ankush , Domadia Kshitiz , Choudhary Krishna , Babu Koyyala Venkata Pradeep
Published: September 2019

AIMS:

The real-world data regarding the response rates, tolerability, and toxicities of immunotherapy is very limited. The aim of this study is to analyze these characteristics in patients who have received immunotherapy for metastatic renal cell carcinoma (RCC) or urothelial cancer (UC).

Methods:

Retrospective review of patients over a year from 2017–2018 diagnosed with metastatic RCC and UC in our institute who received checkpoint inhibitors was done. PFS and OS were calculated.

Results:

A total of 16 patients, 11 with metastatic RCC and 5 patients with Metastatic UC were included in this study. All patients were male and Median age was 57.5 years. Median Number of cycles administered was 6. 50% of patients had a partial response to treatment, 16.6% of patients had stable disease and 33.3% of patients had progressive disease. There were no complete responses to therapy. Median Follow up was 9 months. The median PFS of the whole cohort was 6 months, while in RCC was 6 months and in UC was 1 month. Median OS of the whole cohort is 7 months, while the median OS for RCC and UC were 7 months and 3 months respectively. Fatigue was the most common adverse effect noted and Anaemia was the most common hematological side effect seen with immunotherapy in this study.

Conclusion:

This is real-world data of the use of the immune checkpoint inhibitors in the resource-limited setting. The benefit of Immune checkpoint inhibitors may in advanced renal cell cancers and Urothelial cancers may be different from that seen in the Western population.