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Search Results: 1 - 10 of 325564 matches for " Aramís; Svarch Guerchicoff "
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Relevancia biológica y clínica del inmunofenotipaje celular en la leucemia linfoide aguda del ni?o
Marsán Suárez,Vianed; Cos Padrón,Yanelkys; Sánchez Segura,Miriam; Socarrás Ferrer,Bertha B.; Macías Abraham,Consuelo; del Valle Pérez,Lázaro O.; Nú?ez Quintana,Aramís; González Otero,Alejandro; Svarch Guerchicoff,Eva; Lam Díaz,Rosa M.;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2008,
Abstract: the biological and clinical characteristics of 238 children with acute lymphocytic leukemia (all) were studied for 13 years. the cellular immunophenotyping of samples from the bone marrow was performed by an immunocytochemical ultramicromethod. of the total of studied all, 81.4 % were phenotype b and 18.5 % phenotype t. 48.4 % of the children with b-all were 2-5 years old, whereas 65.9 % with t-all were 6 or over. no statistically significant differences were found when sex and colour of the skin were analyzed in relation to the cellular leukemic phenotype. on diagnosing the disease, 59.3 % of the patients with b-all showed figures of leukocytes in peripheral blood < 20x109/l, whereas in 61.4 % with t-all, the figures were higher than 50x109/l. it was observed a greater incidence of organomegaly, mediastinal adenopathies, hemorrhagic manifestations and initial infiltration of the central nervous system in patients with t-all compared with those suffering b-all. the differences were highly significant. these results proved that the leukemic phenotype in all in children could be considered as a positive or negative prognostic factor of the disease.
Heterogeneidad biológica y clínica de la leucemia linfoide aguda pediátrica de fenotipo T
Marsán Suárez,Vianed; Cos Padrón,Yanelkys; Fuentes de Armas,Lillian Teresa; Socarrás Ferrer,Bertha Beatriz; Sánchez Segura,Miriam; Nú?ez Quintana,Aramís; Svarch Guerchicoff,Eva; Lam Díaz,Rosa; del Valle Pére,Lázaro O; Macías Abraham,Consuelo;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2005,
Abstract: the biological and clinical characteristics of 31 children with phenotype t acute lymphoid leukemia (t-all) were studied in a period of 14 years. the cellular immunophenotyping was performed by the immunocytochemical (umiciq) and alkaline phosphatase anti-alkaline phosphatase (apaap) methods. it was observed a higher incidence (38.5 %) in the age group 2-5. the white male children were the most affected. 61.3 % of the patients showed leukocytes < 20x109/l at the onset of the disease. the mean figure of hemoglobin was 8.8x109/l. 71 % presented lymphadenopathies and splenomegalia. mediastinal mass, hepatomegalia and hemorrhages were found in 32.2 %, 83.9 % and 16.1 %, respectively. 25.8 % had infiltration of the central nervous system. the immunological classification revealed a predominance of the late t-all variety (45.2 %) over the early (29 %) and the cortical (25.8 %). 2 (6,4 %) mi+ t-all were diagnosed. these results proved that the t-all of the child is a clinical and biologically heterogeneous disease
Frecuencia de la leucemia promielocítica en Cuba Frequency of promyelocytic leukemia in Cuba
María Teresa Milanés Roldán,Porfirio Hernández Ramírez,Eva Svarch Guerchicoff,Gisela Martínez Antu?a
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2001,
Abstract: Se realizó el análisis de 1 112 pacientes con LA (leucemias agudas) de todo el país (714 adultos y 398 ni os menores de 15 a os de edad), 637 (554 adultos y 83 ni os) presentaron LMA (leucemia mieloide aguda), y se diagnosticaron 98 casos (72 adultos y 26 ni os) con LPM (leucemia promielocítica aguda). La LPM representó el 8,9 % de todos los casos de LA en adultos y el 6,5 % de todos los casos de LA en ni os. Su frecuencia en relación con el total de casos de LMA sin límite de edad representó el 15,3 %. Sin embargo, cuando esta frecuencia se analizó por grupos de edades se apreció que en los ni os era de 31,3 % mientras que en los adultos fue solo del 13 %. En nuestro estudio se pudo comprobar que de forma similar a lo que ocurre en ciertas regiones geográficas y grupos poblacionales, existe un incremento de la frecuencia de LPM en ni os en relación con los datos de la literatura previa. Nuestros resultados destacan la necesidad de considerar por separado este tipo de leucemia en ni os y adultos, pues su análisis en un solo grupo podría ocultar variaciones en su frecuencia 1 112 patients with AL (acute leukemias) from all over the country (714 adults and 398 children under 15) were analyzed. 637 (554 adults and 83 children) had AML (acute myeloid leukemia) and 98 cases (72 adults and 26 children) were diagnosed APL (acute promyelocytic leukemia). The APL accounted for 8.9 % of all the cases of AL in adults and 6.5 % of all the cases of AL in children. Its frequency in relation to the total of cases of AML with no age limit represented 15.3 %. However, when this frequency was analyzed by age group, it was observed that it was 31.3 % in children, whereas in adults it was just 13 %. In our study, it was possible to prove that similarly to what happens in certain geographic regions and population groups, there is an increase of the frequency of APL in children compared with the data found in the previous literature. Our results stress the need to consider by separate this type of leukemia in children and adults, since its analysis in only one group may occult variations in its frequency
Frecuencia de la leucemia promielocítica en Cuba
Milanés Roldán,María Teresa; Hernández Ramírez,Porfirio; Svarch Guerchicoff,Eva; Martínez Antu?a,Gisela; Ballester Santovenia,José M;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2001,
Abstract: 1 112 patients with al (acute leukemias) from all over the country (714 adults and 398 children under 15) were analyzed. 637 (554 adults and 83 children) had aml (acute myeloid leukemia) and 98 cases (72 adults and 26 children) were diagnosed apl (acute promyelocytic leukemia). the apl accounted for 8.9 % of all the cases of al in adults and 6.5 % of all the cases of al in children. its frequency in relation to the total of cases of aml with no age limit represented 15.3 %. however, when this frequency was analyzed by age group, it was observed that it was 31.3 % in children, whereas in adults it was just 13 %. in our study, it was possible to prove that similarly to what happens in certain geographic regions and population groups, there is an increase of the frequency of apl in children compared with the data found in the previous literature. our results stress the need to consider by separate this type of leukemia in children and adults, since its analysis in only one group may occult variations in its frequency
Tratamiento con dosis moderada de hidroxiurea en la drepanocitosis Treatment with moderate doses of hydroxyurea in drepanocytosis
Sergio Machín García,Eva Svarch,Olga Agramonte Llanes,Aramís Nú?ez Quintana
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2008,
Abstract: En el período comprendido entre junio del 2003 y junio del 2005 se trataron con hidroxiurea 45 pacientes con anemia drepanocítica; 16 ni os y 29 adultos, al menos con una de las manifestaciones siguientes: más de 3 crisis vasooclusivas (CVO) dolorosas al a o en los 3 a os previos al estudio, uno o más episodios de síndrome torácico agudo (STA) al a o en los 2 a os previos al estudio, o accidente vascular encefálico (AVE) en el a o anterior. La hidroxiurea se administró en dosis de 15mg/kg/día. El número de CVO dolorosas, STA, AVE, ingresos y transfusiones disminuyó significativamente (p<0,001). No hubo disminución de los parámetros hematológicos ni aumento de la creatinina o alaninoaminotransferasa. Se observo un incremento importante de los valores de hemoglobina fetal (p<0,008). No se presentaron manifestaciones tóxicas y el cumplimiento fue bueno. En este trabajo se demuestra que no es necesario utilizar la dosis máxima tolerada de hidroxiurea para obtener mejoría del cuadro clínico en la anemia drepanocítica, lo que tiene 2 ventajas: su toxicidad es menor y el tratamiento es menos costoso. Esto haría posible que un número mayor de ni os en los países de escasos recursos puedan beneficiarse con dicho tratamiento. From June 2003 to June 2005, 45 patients with drepanocytic anemia, 16 children and 29 adults, with at least one of the following manifestations were treated with hydroxyurea: more than 3 painful vasoocclusive crises (VOC) at a year and 3 years before the study, one or more episodes of acute thoracic syndrome (ATS) at a year and 2 years previous to the study, or vascular encephalic accident (VEA) in the previous year. Hydroxyurea was administered at doses of 15mg/kg/day. The number of painful VOC, ATS, VEA, admissions and transfusions decreased significantly (p<0.001). There was not either reduction of the hematological parameters or increase of creatinin or alanine aminotransferase. An important rise of fetal hemoglobin values (p<0.008) was reported. There were no toxic manifestations and the fulfilment was good. It was proved in this paper that it is not necessary to take the maximum tolerated dose of hydroxyurea to improve the clinical picture in drepanocytic anemia. Two of its advantages are its lower toxicity and the less expensive treatment. This would make possible that a greater amount of children in those countries with scarce resources may benefit from the treatment.
Aspectos clínicos, bioquímicos, moleculares y tratamiento de 2 pacientes con enfermedad de Gaucher
Lavaut Sánchez,Kalia; Nú?ez Quintana,Aramís; Nordet Carreras,Ileana; González Otero,Alejandro; Svarch,Eva; Machín García,Sergio; Giraldo,Pilar; Sá Miranda,Clara;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2010,
Abstract: gaucher's disease is a hereditary entity related to sphingolipids metabolism with an autosomal recessive hereditary pattern determined by a failure of the acid b-glucosidase enzyme. in present paper authors present the case of two pediatric patients (1 female and 1 male) both presenting with anemia and hepatosplenomegaly by ultrasound (us). bone marrow aspirate showed infiltration by storage cells, low levels of enzymatic activity of b-glucocerebroside and a molecular diagnosis of potential known mutations confirmed the disease in both patients, who are under treatment with substitutive enzymatic therapy (imiglucerase) with a favorable course in clinical and humoral features.
Morbiletalidad en pacientes adultos con drepanocitosis
Machín García,Sergio; Guerra Alfonso,Tamara; Svarch,Eva; Espinosa Martínez,Edgardo; Mesa Cuervo,José R; Dorticós Balea,Elvira; Plasencia,Alelí; González Otero,Alejandro; Nú?ez Quintana,Aramís; Wade Mateo,Maura;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2004,
Abstract: a study was performed on 397 adult patients with sickle cell anemia, who had been followed up from january 1973 to december 1997. two hundred were females and 197 males; 305 of these patients presented with sickle cell anemia, 63 with sc hemoglobinopathy and 29 with sb thalassemia. the mean follow-up period was 14,3 years. the yearly admission and blood transfusion mean was lower in sc hemoglobinopathy(p<0,001). painful vasoocclusive crisis was more frequent in sb thalassemia whereas infections often occurred in sickle cell anemia (p<0,001). one hundred four pregnancies were developed from 87 females. there were 4 maternal deaths and 10 perinatal deaths. differences were found in hemoglobin and reticulocyte values among hemoglobinopathies (p<0,001) but no difference was seen between sexes or among age groups (18-29 y, 30-40 y and over 40 y). fetal hemoglobin values did not show significant difference between sexes, although they were higher in sickle cell anemia. global survival estimates were 53 years in sickle cell anemia, 59 years in sc hemoglobinopathy and 48 in sb thalassemia (p< 0,05). the most frequent cases of death were hepatic complications, encephalic vascular attacks and infections
Función esplénica en la anemia drepanocítica
Nordet Carrera,Ileana; Nú?ez Quintana,Aramís; Menéndez Veitía,Andrea; Machín García,Sergio; García Peralta,Tania; Torres la Roche,Leonel; Batista Cuéllar,Juan; Wade Mateo,Maura; González Otero,Alejandro; Svarch,Eva;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2004,
Abstract: spleen scan with technetium-99m methyldiphosphonate and technetium-99m sulfur colloid was performed on 30 children suffering from sickle cell anemia on basal conditions included in three groups: 0-5 years-old children in the first, 6-10 years-olds in the second and 11-15 years-olds in the third. the annual average rate of infections for each patient was determined and the hemoglobin and reticulocyte figures at the time of study were taken into account. most of children up to 5 years showed normal results in the scintigraphic studies, but abnormal results increased in the second group and were pathological in the third one. in the latter, 7 children had functional asplenia and organic asplenia was shown in all of them. hemoglobin and reticulocyte figures did not show differences among the groups. annual average rates of infections were 0.28 for the first group; 0,32 for the second and 0,59 for the third (p 0.05). scintigraphy was a useful procedure to study splenic function in children with sickle cell anemia and it was demonstrated that the rate of infections was higher in the group exhibiting more scintigraphic alterations
Tratamiento con dosis moderada de hidroxiurea en la drepanocitosis
Machín García,Sergio; Svarch,Eva; Agramonte Llanes,Olga; Nú?ez Quintana,Aramís; Menéndez Veitía,Andrea; Hernández Padrón,Carlos; Mesa Cuervo,José; Nordet Carrera,Ileana; Boch,Lisbet;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2008,
Abstract: from june 2003 to june 2005, 45 patients with drepanocytic anemia, 16 children and 29 adults, with at least one of the following manifestations were treated with hydroxyurea: more than 3 painful vasoocclusive crises (voc) at a year and 3 years before the study, one or more episodes of acute thoracic syndrome (ats) at a year and 2 years previous to the study, or vascular encephalic accident (vea) in the previous year. hydroxyurea was administered at doses of 15mg/kg/day. the number of painful voc, ats, vea, admissions and transfusions decreased significantly (p<0.001). there was not either reduction of the hematological parameters or increase of creatinin or alanine aminotransferase. an important rise of fetal hemoglobin values (p<0.008) was reported. there were no toxic manifestations and the fulfilment was good. it was proved in this paper that it is not necessary to take the maximum tolerated dose of hydroxyurea to improve the clinical picture in drepanocytic anemia. two of its advantages are its lower toxicity and the less expensive treatment. this would make possible that a greater amount of children in those countries with scarce resources may benefit from the treatment.
La drepanocitosis en Cuba. Estudio en ni?os: Study in children
Svarch,Eva; Marcheco-Teruel,Beatriz; Machín-García,Sergio; Menéndez-Veitía,Andrea; Nordet-Carrera,Ileana; Arencibia-Nú?ez,Alberto; Nú?ez-Quintana,Aramís; Martínez-Triana,Raúl; Scherle-Matamoros,Claudio; San Román-García,Eliécer; González-Otero,Alejandro; de la Torre-Montejo,Ernesto;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2011,
Abstract: drepanocytosis is the hemolytic anemia more frequent genetically determined in the world. in cuba, the frequency of carrier status is of 3,08% in general population. the pathophysiology of vascular occlusion is very complex; includes the polymerization of the hb s, the alterations of red-blood cells, the adhesion molecules, the inflammatory cytokines, the coagulation factors and the lesions of the vascular endothelium. the more frequent clinical manifestations are: painful vaso-occlusive crises, the acute thoracic syndrome, the splenic sequestration crisis, the aplastic crisis, the aseptic necrosis of femur head and malleolar ulcer. the clinical picture is very variable: from children dying early in life up to patients achieve the sixth decade of life. in the institute of hematology and immunology there is an integral care program including: systematic follow-up from early in life in a specialized consultation, permanent administration of folic acid and of prophylactic oral penicillin during the first 5 years of age; as well as the child education and of parents. from 1986 it is carried out the partial splenectomy in crises of splenic sequestration with excellent results. between 2004-2008 in all the country deceased only 16 patients and in 397 adults the survival rate was of 53 years in the drepanocythemia and of 58 in the sc hemoglobinopathy. as result of this program, in past years the survival has increased, the quality of life of patient improved and the costs spent in treatment of complications has decrease.
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