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Search Results: 1 - 10 of 37527 matches for " Porfirio; Fernández Delgado "
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Síndrome mielodisplásico: I. Biología y clínica
Fernández Delgado,Norma; Hernández Ramírez,Porfirio;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2000,
Abstract: myelodysplastic syndromes (mds) include a group of clonic disorders characterized by progressive cytopenias and dishematopoiesis. etiology of primary mds is unknow, and secondary ones may be due to use of antineoplastic agents, chemicals and in children, are related to constitutional diseases. their general biological features include cytogenetic, molecular, and immunologic alterations accompaning hematopoiesis alterations. for diagnosis it is necessary 10% of cellular involvement in each serie, where dishematopoiesis be the cause. in 80% of bone marrow biopsies, we found signs of dismegacaryopoiesis and disarrangement in normal hematopoietic structure. recently, it was proposed existence of mds variants, including hyperfibrotic type, the early one, and mds with features of myeloproliferative syndrome. diagnosis of mds is by exclusion and in differential diagnosis, it is necessary rule out some processes presenting myelodisplastic alterations, including anemias by vitamin b12, folic acid, or piridoxin deficiency, chronic liver disease, chronic diseases anemia, chemotherapy, aids virus infection, and medullary aplasia, etc
Síndrome mielodisplásico: I. Biología y clínica Myelodysplastic syndrome: I. Biology and Clinic
Norma Fernández Delgado,Porfirio Hernández Ramírez
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2000,
Abstract: Los síndromes mielodisplásicos (SMD) constituyen un grupo de trastornos clonales caracterizados por citopenias progresivas y dishematopoyesis. La etiología de los SMD primarios es desconocida y los secundarios pueden deberse al uso de agentes antineoplásicos, productos químicos y en los ni os, se asocian con enfermedades constitucionales. Sus características biológicas generales incluyen las alteraciones de la hematopoyesis, que pueden ir acompa adas por alteraciones citogenéticas, moleculares e inmunológicas. Para el diagnóstico es necesario que la dishematopoyesis afecte, al menos, el 10 % de las células en cada una de las series. En el 80 % de las biopsias de médula ósea se observan signos de dismegacariopoyesis y desorganización en la arquitectura hematopoyética habitual. Recientemente se ha planteado la existencia de variantes de SMD, entre ellos el hiperfibrótico, el temprano y el SMD con características de síndrome mieloproliferativo. El diagnóstico de SMD es por exclusión, por lo que es necesario en el diagnóstico diferencial destacar algunos procesos que pueden presentar alteraciones mielodisplásicas, entre ellos las anemias por deficiencia de vitamina B12, ácido fólico o piridoxina, las hepatopatías crónicas, la anemia de las enfermedades crónicas, el tratamiento con quimioterápicos, la infección por el virus del SIDA y la aplasia medular, entre otros Myelodysplastic syndromes (MDS) include a group of clonic disorders characterized by progressive cytopenias and dishematopoiesis. Etiology of primary MDS is unknow, and secondary ones may be due to use of antineoplastic agents, chemicals and in children, are related to constitutional diseases. Their general biological features include cytogenetic, molecular, and immunologic alterations accompaning hematopoiesis alterations. For diagnosis it is necessary 10% of cellular involvement in each serie, where dishematopoiesis be the cause. In 80% of bone marrow biopsies, we found signs of dismegacaryopoiesis and disarrangement in normal hematopoietic structure. Recently, it was proposed existence of MDS variants, including hyperfibrotic type, the early one, and MDS with features of myeloproliferative syndrome. Diagnosis of MDS is by exclusion and in differential diagnosis, it is necessary rule out some processes presenting myelodisplastic alterations, including anemias by vitamin B12, folic acid, or piridoxin deficiency, chronic liver disease, chronic diseases anemia, chemotherapy, AIDS virus infection, and medullary aplasia, etc
Matriptasa 2:: nuevo eslabón en el metabolismo del hierro
Forrellat Barrios,Mariela; Fernández Delgado,Norma; Hernández Ramírez,Porfirio;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: the maintenance of iron homeostasis is essential for the normal physiological functioning of the living beings. recently, it was discovered the matriptase 2, a type ii trans-membrane serine protease, also known by english acronyms tmprss6, having an essential function in homeostasis of this mineral. this finding initially derived from the observation that mice deficient of this protein had anemia as consequence of rise of hepcidine levels and the interruption of iron intestinal absorption. the in vitro later analyses showed that 2 matriptase suppress the stimulation way of hepcidine transcription involving to hemojuvelin as a co-receptor. in agreement with the anemia pattern of mutant mice, we founded that pattern from subjects presenting with iron-deficiency anemia and iron-resistant (irida) was created by tmprsst gen mutations involving the elimination of enzymatic proteolytic activity. in present paper are showed the features related to identification and characterization of the 2 matriptase, as well as, its involvement in iron metabolism.
Nuevos conocimientos sobre el metabolismo del hierro
Forrellat Barrios,Mariela; Fernández Delgado,Norma; Hernández Ramírez,Porfirio;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2005,
Abstract: iron is a very important mineral for the organism and its regulation requires a complex molecular network. only 3 proteins that took part in iron metabolism were known a few years ago, but in the last decade, new proteins that participate in iron homeostasis and that are involved in its transportation, absorption, recycling and balance in the organism have been discovered in a sequential way, starting from the study of some genetical diseases, such as hereditary hemochromatosis. the identification and isolation of these proteins lead inevitably to the modification of the classical models of regulation of the homeostasis of this powerful mineral. a review of the esential elements known up to now of each of these new proteins and the interaction among them was made in this paper
Matriptasa 2:: nuevo eslabón en el metabolismo del hierro Matriptase 2:: a new link in iron metabolism
Mariela Forrellat Barrios,Norma Fernández Delgado,Porfirio Hernández Ramírez
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: El mantenimiento de la homeostasia del hierro es esencial para el funcionamiento fisiológico normal de los seres vivos. Recientemente se descubrió que la matriptasa 2, una serin proteasa transmembrana tipo II, también conocida por las siglas en inglés TMPRSS6, tiene una función esencial en la homeostasis de este mineral. Este hallazgo derivó inicialmente de la observación de que ratones con deficiencia de esta proteína presentaban anemia como consecuencia de la elevación de los niveles de hepcidina y la interrupción de la absorción intestinal de hierro. Los análisis posteriores in vitro demostraron que la matriptasa 2 suprime la vía de estimulación de la transcripción de la hepcidina que implica a la hemojuvelina como co-receptor. En concordancia con el patrón de anemia de los ratones mutantes, se encontró que el patrón de individuos con anemia por deficiencia de hierro resistente al hierro (en inglés IRIDA), era ocasionado por mutaciones del gen Tmprss6 que implican anulación de la actividad proteolítica de la enzima. En este trabajo se presentan aspectos relacionados con la identificación y caracterización de la matriptasa 2, así como su participación en el metabolismo del hierro. The maintenance of iron homeostasis is essential for the normal physiological functioning of the living beings. Recently, it was discovered the Matriptase 2, a type II trans-membrane serine protease, also known by English acronyms TMPRSS6, having an essential function in homeostasis of this mineral. This finding initially derived from the observation that mice deficient of this protein had anemia as consequence of rise of hepcidine levels and the interruption of iron intestinal absorption. The in vitro later analyses showed that 2 Matriptase suppress the stimulation way of hepcidine transcription involving to hemojuvelin as a co-receptor. In agreement with the anemia pattern of mutant mice, we founded that pattern from subjects presenting with iron-deficiency anemia and iron-resistant (IRIDA) was created by Tmprsst gen mutations involving the elimination of enzymatic proteolytic activity. In present paper are showed the features related to identification and characterization of the 2 Matriptase, as well as, its involvement in iron metabolism.
Regulación de la hepcidina y homeostasis del hierro: avances y perspectivas Regulation of hepcidin and iron homeostasis: progress and prospects
Mariela Forrellat-Barrios,Norma Fernández-Delgado,Porfirio Hernández-Ramírez
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2012,
Abstract: El estudio de los desórdenes genéticos del metabolismo del hierro, la identificación de sus transportadores y el descubrimiento de la hepcidina, hormona reguladora de la homeostasia del hierro, han contribuido grandemente a aumentar los conocimientos sobre este metabolismo y han cambiado sustancialmente la visión sobre las enfermedades relacionadas con alteraciones del metabolismo férrico. En la última década, no solo se han esclarecido elementos de la patogénesis de estas enfermedades, sino que ya se vislumbran aplicaciones terapéuticas de estos avances. Así, ya se habla de una nueva era basada en el tratamiento de los desórdenes de la homeostasia del hierro a través de la modulación de la hepcidina. The study of genetic disorders of iron metabolism, identification of transporters and the discovery of hepcidin- a hormone regulating iron homeostasis- have contributed greatly to increase awareness of this metabolism. Substantially, the vision on diseases related to disorders of iron metabolism has been changed. In the last decade, elements of the pathogenesis of these diseases have not only been clarified, but therapeutic applications of these advances are looming. Thus, there are expectations of a new era based on the treatment of iron homeostasis disorders through hepcidin modulation.
Nuevos conocimientos sobre el metabolismo del hierro New knowledge of iron metabolism
Mariela Forrellat Barrios,Norma Fernández Delgado,Porfirio Hernández Ramírez
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2005,
Abstract: El hierro es un mineral de elevada importancia para el organismo y su regulación requiere de una red molecular compleja. Hasta hace unos a os solo se conocían 3 proteínas que intervenían en el metabolismo del hierro, pero en la última década, se han descubierto de forma secuencial, y a partir del estudio de algunas enfermedades genéticas como la hemocromatosis hereditaria, nuevas proteínas que participan en la homeostasia del hierro y que están implicadas en su transporte, absorción, reciclaje y balance en el organismo. La identificación y aislamiento de estas proteínas lleva inevitablemente a la modificación de los modelos clásicos de regulación de la homeostasia de este importante mineral. En este trabajo se realizó una revisión de los elementos esenciales conocidos hasta la actualidad de cada una de estas nuevas proteínas y la interacción entre ellas Iron is a very important mineral for the organism and its regulation requires a complex molecular network. Only 3 proteins that took part in iron metabolism were known a few years ago, but in the last decade, new proteins that participate in iron homeostasis and that are involved in its transportation, absorption, recycling and balance in the organism have been discovered in a sequential way, starting from the study of some genetical diseases, such as hereditary hemochromatosis. The identification and isolation of these proteins lead inevitably to the modification of the classical models of regulation of the homeostasis of this powerful mineral. A review of the esential elements known up to now of each of these new proteins and the interaction among them was made in this paper
Espectro funcional de las plaquetas: de la hemostasia a la medicina regenerativa Platelet Functional spectrum: from Hemostasis to Regenerative Medicine
Norma Fernández-Delgado,Porfirio Hernández-Ramírez,Mariela Forrellat-Barrios
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2012,
Abstract: La aplicación terapéutica de los factores solubles que intervienen en los procesos biológicos es uno de los pilares en que se sustenta la medicina regenerativa. En los últimos a os, múltiples investigaciones han mostrado la participación de los factores de crecimiento en la regeneración de diferentes tejidos. Las plaquetas pueden considerarse como una bomba o coctel de biomoléculas, por su contenido en factores de crecimiento que son liberados por los gránulos plaquetarios después de su activación. Desde hace más de 2 decenios, varios investigadores han publicado los beneficios del uso de los factores de crecimiento derivados de las plaquetas en la reparación y regeneración de diferentes tejidos, pero en los a os recientes su uso se ha extendido con el empleo de diferentes métodos para su obtención y la utilización, tanto de plaquetas antólogas, como alogénicas. El procedimiento para su obtención es fácil, rápido y económico y los resultados referidos en la mayoría de las publicaciones son alentadores, sin que se hayan comunicado reacciones adversas. One of the fundamental basis of regenerative medicine is the therapeutic application of soluble factors involved in biological processes. In recent years, many investigations have shown the involvement of growth factors in the regeneration of different tissues. Platelets can be considered as biological pump or cocktail, due to their content in growth factors which are released by the platelet granules upon activation. For more than two decades, several researchers have reported the benefits of the use of growth factors derived from platelets in the repair and regeneration of different tissues, but in recent years its use has been extended with the employment of different methods for their collection in both, autologous and allogeneic platelets. The procedure for obtaining platelet factor is easy, quick and inexpensive and the results reported in most publications are encouraging, without any reported adverse reactions.
Impacto del tabaquismo como factor de riesgo aterosclerótico en edades tempranas
Fernández-Britto,José E; Wong,Roberto; Contreras,Daniel; Delgado,Juana; Campos,Rosa; Norder,Porfirio;
Revista Cubana de Investigaciones Biom??dicas , 1999,
Abstract: the impact of smoking as an atherogenic risk factor in dead patients aged 5-34 that underwent necropsy was studied. this multinational investigation was carried out by the world health organization and the international federation of cardiology societies from 1986 to 1996. the center for general coordination of this research in malm?, sent 966 left halves of thoracica-orta, 947 left-halves of the abdominal aorta and 959 right coronary arteries.the arteries came from ll countries located in 5 regions of the world health organization: america, africa, europe, southeastern and southwestern asia. these arteries were processed by using the methodology proposed by the world health organization since 1957 for the application of the atherometric system (as), a set of methods and procedures considered as ideal for the pathomorphologic and morphometric characterization of the atherosclerotic lesion. the population of autopsies was divided into 2 groups: smokers and non smokers. data were processed by statistical descriptive, comparative and multivariate analyses. among the most important conclusions are the following: the distribution of adipose strias and fibrous plaques in the 3 studied arteries, thoracic aorta, abdominal aorta and right coronary artery was greater in smokers than in non smokers. the transformation of adipose strias into fibrous plaques began earlier and with much more intensity among smokers. the anova statistical analysis between smokers and non smokers had a great signification in the 3 arteries. the result of the anova statistical analysis showed the importance of the adipose strias in the thoracic aorta and of these strias and the fibrous plaques in the right coronary artery. it was clearly proved in this research that smoking is a strong risk factor for atherosclerosis and, therefore, for its organic consequences, the cardiovascular diseases at early ages.
Implante percutáneo de células mononucleares de sangre periférica movilizadas con factor estimulante de colonias granulocíticas, en la osteoartrosis de rodilla. Primer caso comunicado en Cuba Percutaneous implantation of peripheral blood mononuclear cells mobilized with granulocyte colony stimulating factor in osteoarthritis of the knee. First case reported in Cuba
Aymara María Baganet Cobas,Porfirio Hernández Ramírez,Norma Fernández Delgado,Mariela Forrellat Barrios
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2010,
Abstract: La enfermedad articular degenerativa, también conocida como osteoartrosis, afecta al 10 % de los adultos mayores de 60 a os de edad. Se caracteriza principalmente por dolor de la articulación afectada, crepitación, rigidez matinal y limitación progresiva de los movimientos de esa articulación. Todo esto conduce a un desgaste parcial o completo del cartílago articular. El tratamiento de la osteoartrosis de la rodilla constituye un gran desafío. Los avances recientes en el uso de la medicina regenerativa sugieren que las células madre adultas pudieran representar una alternativa promisoria en el tratamiento de esta enfermedad. En una paciente femenina de 61 a os de edad con osteoartrosis de la rodilla, se realizó el implante percutáneo de células mononucleares autólogas movilizadas a la sangre periférica mediante el factor estimulante de colonias granulocíticas, y se logró una rápida mejoría clínica y radiológica. Este resultado sugiere que el proceder empleado es un método factible, simple, seguro y menos costoso, para el tratamiento de las lesiones degenerativas articulares. The degenerative joint disease, also known as osteoarthrosis affects to 10% of elderlies aged 60. It is mainly characterized by pain in the involved joint, crepitation, morning stiff and a progressive limitation of movement of that joint leading to a partial or total wear of articular cartilage. The treatment of the knee osteoarthrosis is a great challenge. The recent advances in use of regenerative medicine suggest that adult stem cells could represent a promisor alternative in the treatment of this entity. In a female patient aged 61 presenting with knee osteoarthrosis authors placed a percutaneous implant of autologous mononuclear cells mobilized to peripheral blood by granulocyte colony-stimulating factor achieving a fast clinical and radiological improvement. This result suggests that the procedure used is a feasible, simple, safe and less expensive method for treatment of articular degenerative lesions.
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