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	Provedor de dados BJMBR (2) ABCL (1) Autor Cao,Qing-Hua (1) Espinoza-Mora,María del Rosario (1) Espreafico,E.M. (1) Gurgel-Gianetti,J. (1) Izidoro-Toledo,T.C. (1) Li,Jun-Xun (1) Meschede,I.P. (1) Monge-Rodríguez,Silvia Leticia (1) Ouyang,Yin (1) Ouyang,Zhao-Zhong (1) Peng,Ting-Sheng (1) Santos,T.O. (1) Wei,Qiu-Xiang (1) Zhan,Hong (1) Mais... Palavra-chave Hemophagocytic lymphohistiocytosis (3) Case report (1) Cerebellitis (1) Cytotoxic granules (1) Exocitosis (1) Griscelli syndrome (1) Gránulos citotóxicos (1) Immunodeficiency (1) Linfohistiocitosis hemofagocítica (1) Multiple organ failure (1) RAB27A (1) Rhabdomyolysis (1) T-cell lymphoma (1) Mais... Tipo do documento Info:eu-repo/semantics/article (2) Info:eu-repo/semantics/report (1) Ano 2018 (1) 2017 (1) 2008 (1) País Brazil (2) Argentina (1) Idioma Inglês (2) Espanhol (1)		Ordenar por:  Relevância Autor Título Ano Registros recuperados: 3 Primeira ... 1 ... Última Mecanismo de exocitosis de gránulos citotóxicos: claves dadas a partir de la linfohistiocitosis hemofagocítica primaria ABCL Monge-Rodríguez,Silvia Leticia; Espinoza-Mora,María del Rosario. La linfohistiocitosis hemofagocítica (HLH) es un síndrome clínico de hiperinflamación que se caracteriza por ser una respuesta inmune altamente estimulada pero inefectiva. En la HLH primaria se encuentran alterados el proceso de exocitosis de gránulos citotóxicos o los efectores que se encuentran en éstos, también existe afección de la activación de las células citotóxicas. Durante la exocitosis existe disfunción en la fase de transporte y maduración vesicular, en la regulación del proceso de docking y priming o en los complejos v-SNARE y t-SNARE. La conexión entre la célula citotóxica y célula diana se compromete si se afecta la proteína efectora perforina. SAP y XIAP se relacionan con la activación de las células inmunitarias. Aunque actualmente se... Tipo: Info:eu-repo/semantics/article Palavras-chave: Linfohistiocitosis hemofagocítica; Exocitosis; Gránulos citotóxicos; Hemophagocytic lymphohistiocytosis; Exocitosis; Cytotoxic granules. Ano: 2017 URL: http://www.scielo.org.ar/scielo.php?script=sci_arttext&pid=S0325-29572017000100016 T-cell lymphoma with rhabdomyolysis: case report and literature review BJMBR Ouyang,Zhao-Zhong; Peng,Ting-Sheng; Cao,Qing-Hua; Ouyang,Yin; Li,Jun-Xun; Wei,Qiu-Xiang; Zhan,Hong. Rhabdomyolysis refers to the destruction or disintegration of striated muscles. This syndrome is characterized by muscle breakdown and necrosis, resulting in the leakage of intracellular muscle constituents into the circulation and extracellular fluid. We report a rare case of rhabdomyolysis complicating multi-organ failure caused by T-cell lymphoma in a 32-year-old woman. The final diagnosis was rhabdomyolysis caused by peripheral T-cell lymphoma based on bone marrow aspirate and biopsy. Tipo: Info:eu-repo/semantics/report Palavras-chave: Rhabdomyolysis; Multiple organ failure; T-cell lymphoma; Hemophagocytic lymphohistiocytosis; Case report. Ano: 2018 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2018001100501 Griscelli syndrome-type 2 in twin siblings: case report and update on RAB27A human mutations and gene structure BJMBR Meschede,I.P.; Santos,T.O.; Izidoro-Toledo,T.C.; Gurgel-Gianetti,J.; Espreafico,E.M.. Griscelli syndrome (GS) is a rare autosomal recessive disorder caused by mutation in the MYO5A (GS1, Elejalde), RAB27A (GS2) or MLPH (GS3) genes. Typical features of all three subtypes of this disease include pigmentary dilution of the hair and skin and silvery-gray hair. Whereas the GS3 phenotype is restricted to the pigmentation dysfunction, GS1 patients also show primary neurological impairment and GS2 patients have severe immunological deficiencies that lead to recurrent infections and hemophagocytic syndrome. We report here the diagnosis of GS2 in 3-year-old twin siblings, with silvery-gray hair, immunodeficiency, hepatosplenomegaly and secondary severe neurological symptoms that culminated in multiple organ failure and death. Light microscopy... Tipo: Info:eu-repo/semantics/article Palavras-chave: Griscelli syndrome; RAB27A; Hemophagocytic lymphohistiocytosis; Cerebellitis; Immunodeficiency. Ano: 2008 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2008001000002 Registros recuperados: 3 Primeira ... 1 ... Última

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