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Search Results: 1 - 10 of 4969 matches for " Eva Svarch "
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El accidente vascular encefálico en ni?os con drepanocitosis
Svarch,Eva;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: stroke is one of the more severe complications of drepanocytemia. it appears mainly in the first decade of life, with a trend to recurrence, and with cognitive and neurologic permanents. its optimal prevention and treatment are not accurately established, but nowadays, the more used strategies with better results are the red-blood cell transfusions and the hydroxyurea. transcranial doppler ultrasound (tcus) is an usefulness and safe method to predict stroke in many patients.
Fisiopatología de la drepanocitosis
Svarch,Eva;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: hemoglobin s (hb) is the consequence of a point mutation in of b globin chain 6 position resulting in glutamic acid substitution by valine. hb s polymerizes in conditions of oxygen low tension deforming the erythrocyte, the latter with the hb polymerized, has a shorten survival occluding the microcirculation, causing a chronic hemolytic anemia, and painful vasoconstrictive crises noting down to large extent the clinical picture of disease. vascular occlusion is a complex process due mainly to transit time extension of erythrocytes through microcirculation and/or decrease of delayed time of polymerization of hbs. nowadays, occlusion in the microcirculation is considered as an injury way of reperfusion in which oxidative stress of coagulation participates in the process. hemolytic anemia contributes to vascular occlusion because of the reticulocytes, with more adhesive molecules in its surface than the mature ones, to determines its greater adhesion to endothelium, and because of the plasma free hb to consume nitric oxide (no). no acts in different ways, where the most important is the deviation of normal balance between vasodilatation and vasoconstriction. as consequence from these facts we describe a sub-phenotype characterized by pulmonary hypertension, stroke, priapism, and malleolar ulcer. there are genetic factors influencing in clinical picture severity, e.g. 158 c-t mutation in globin gen g, and a-thalassemia. the first one increase the hbf, and the second one decrease the chcm, the number of thick cells, and the hemolysis identity. however, relation between a-thalassemia and severity of disease is not clear.
Programa cubano de atención integral al paciente con drepanocitosis Cuban program of comprehensive medical care to patients with sickle cell disease
Eva Svarch
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2011,
Abstract:
Fisiopatología de la drepanocitosis Physiopathologic features of drepanocytemia
Eva Svarch
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: La hemoglobina (Hb) S es la consecuencia de una mutación puntual en la posición 6 de la cadena de la globina que resulta en la sustitución del ácido glutámico por la valina. La HbS polimeriza en condiciones de baja tensión de oxígeno y deforma al hematíe. El hematíe con Hb polimerizada tiene una sobrevida acortada y ocluye la microcirculación, lo que da lugar a la anemia hemolítica crónica y a las crisis vasooclusivas dolorosas que marcan en gran medida el cuadro clínico de la enfermedad. La oclusión vascular es un proceso complejo debido fundamentalmente a la prolongación del tiempo de tránsito de los hematíes a través de la microcirculación y/o a la disminución del tiempo de demora de polimerización de la HbS. En la actualidad se considera a la oclusión en la microcirculación como una forma de injuria de reperfusión en la que el estrés oxidativo y la inflamación llevan al da o crónico de los órganos. Los leucocitos y factores de la coagulación participan en el proceso. La anemia hemolítica contribuye a la oclusión vascular porque el aumento de reticulocitos, con más moléculas de adhesión en su superficie que las células maduras, determina su mayor adhesión al endotelio y porque la Hb libre en plasma consume óxido nítrico. El óxido nítrico actúa en diferentes formas, una de las más importantes es que desvía el balance normal entre vasodilatación-vasoconstricción hacia la vasoconstricción. Como consecuencia de estos hechos se describe un subfenotipo caracterizado por hipertensión pulmonar, accidente vascular encefálico, priapismo y úlcera maleolar. Existen factores genéticos que influyen en la severidad del cuadro clínico como la mutación 158 C-T en el gen de la globina g y la a talasemia. La primera aumenta la HbF y la segunda disminuye la CHCM, el número de células densas y la intensidad de la hemólisis. Sin embargo, la relación entre a talasemia y la severidad de la enfermedad no es clara. Hemoglobin S (Hb) is the consequence of a point mutation in of b globin chain 6 position resulting in glutamic acid substitution by valine. HB S polymerizes in conditions of oxygen low tension deforming the erythrocyte, the latter with the HB polymerized, has a shorten survival occluding the microcirculation, causing a chronic hemolytic anemia, and painful vasoconstrictive crises noting down to large extent the clinical picture of disease. Vascular occlusion is a complex process due mainly to transit time extension of erythrocytes through microcirculation and/or decrease of delayed time of polymerization of HbS. Nowadays, occlusion in the microcirculation is consi
El accidente vascular encefálico en ni os con drepanocitosis Stroke present in children presenting drepanocytemia
Eva Svarch
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: El accidente vascular encefálico (AVE) es una de las complicaciones más graves de la drepanocitosis. Se presenta, sobre todo, en la primera década de la vida, tiene tendencia a recurrir y puede dejar secuelas neurológicas y cognoscitivas permanentes. Su prevención y tratamiento óptimos no se han establecido con precisión, pero en el momento actual, los que más se utilizan con buenos resultados son las transfusiones de glóbulos rojos y la hidroxiurea. El ultrasonido doppler transcraneal (UDTC) es un método útil y no invasivo para predecir el AVE en un número grande de pacientes. Stroke is one of the more severe complications of drepanocytemia. It appears mainly in the first decade of life, with a trend to recurrence, and with cognitive and neurologic permanents. Its optimal prevention and treatment are not accurately established, but nowadays, the more used strategies with better results are the red-blood cell transfusions and the hydroxyurea. Transcranial Doppler ultrasound (TCUS) is an usefulness and safe method to predict stroke in many patients.
Las histiocitosis
Svarch,Eva; Arteaga,Rafael; Pavón Morán,Valia; González Otero,Alejandro;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2001,
Abstract: the term histiocytosis identifies a group of disorders that have in common the proliferation of dentritic cells (dc) and macrophages and is frequently diagnosed in children. among the fundamental variants of histiocytosis related with dc, we find langerhans cell histiocytosis (lch). langerhans cell histiocytosis has very variable clinical behavior that ranges from a lesion involving only one site or system to a multisystem disease. treatment depends on the spread of the process. an only lesion tends to spontaneously disappear. also diagnostic biopsy with or without steroid injection may lead to healing. those patients with multisystem disease may benefit from an steroid and cytostatic-based treatment or even from progenitor hematopoietic cell transplantation. sinus histiocytosis with massive lymphadenopathies or rosai dorfman disease is a benign and usually self-limited disease which is caused by the macrophage proliferation; it generally affects children and young adults. hemophagocytic lymphohistiocytosis is also caused by macrophage proliferation and is a rare disease with a high mortality rate. it can be familiar hemophagocytic lymphohistiocytosis (recessive autosomal) or secondary to viral infections, being the latter form the most frequent in infants. at present, mainly in the familiar variant, the progenitor hematopoietic allogenic transplant may serve as the only curative option
Limitación cognitiva en ni?os con anemia drepanocítica sin historia de afectación neurológica
Martínez Triana,Raúl; Svarch,Eva; Menéndez Veitía,Andrea;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: in past years cognitive difficulties are reported in children with drepanocytemia (sca) without evident neurologic affection, and we emphasized the need of a specialized care. a total of 29 patients were studied without any element evidencing affection of central nervous system (cns) in registered in their medical records. we applied the wechsler intelligence scale compared to health children. we get information from their teachers on performance in subjects like mathematics and spanish, interest and discipline during the classes. we note a decrease in intelligence quotient (ic) from the total scale (p= 0,014), and from rendition scale (p= 0,008), and also in similarity scales (p= 0,048), to figures array (p= 0,017), and block design (p= 0,001). professors considered that mathematics and spanish performance (40 % and 36 %, respectively) lower than in the other pupils. neurocognitive deficit is present in children with sca without a prior history of cns affection, thus, this area must to be periodically assessed as part of its integral care.
Aplasia medular: Actualización
Machín García,Sergio; Svarch,Eva; Dorticós Balea,Elvira;
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 1999,
Abstract: bone marrow aplasia according to its etiology may be congenital or acquired. the latter is the most frequent. haemopoietic failure seems to be caused by several factors. the causes of acquired medullary aplasia and its physiopathological mechanisms are reviewed. emphasis is made on th immune mechanisms, which play an important role in its physiopathology. the diagnostic criteria as well as the elements of an unfavorable diagnosis and the disease that must be taken into consideration to make the differential diagnosis are analyzed in this paper. the most effective treatments at present are the immunosuppressors and bone marrow transplantation. each has advantages and disadvantages and requires specific indications
Tratamiento de la leucemia mieloide aguda del ni o en Cuba Treatment of children acute myeloid leukemia in Cuba
Andrea Menéndez Veitía,Alejandro González Otero,Eva Svarch,Edelis Rosell Monzón
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2013,
Abstract: Introducción: la leucemia mieloide aguda representa alrededor del 20 % de las leucemias agudas de la ni ez con una respuesta al tratamiento y supervivencia menores que la leucemia linfoide aguda. Objetivo: describir los resultados obtenidos con el tratamiento de la leucemia mieloide aguda del ni o en algunos centros de Cuba en el período 2000-2008. Métodos: se trataron 46 pacientes con leucemia mieloide aguda, se excluyeron los casos con leucemia promielocítica, diagnosticados a partir del a o 2000 en las provincias occidentales, Sancti Spíritus y Villa Clara. Se aplicaron dos esquemas de tratamiento tipo BFM en dos períodos: en el primero (2000 -2003) se incluyeron 27 enfermos y en el segundo (2004-2008), 19. La diferencia fundamental entre los dos períodos consistió en la consolidación que en la segunda etapa fue de ciclos más intensos y cortos. Resultados: predominó el sexo masculino (n = 32) y la mediana de edad fue de 9 a os. La remisión inicial fue del 71 % en la primera etapa y 89 % en la segunda. La supervivencia libre de eventos (SLE) en todos los pacientes fue del 40 % a los 5 a os y la supervivencia global (SV) fue del 44 % en igual período. En la SLE en las dos etapas se encontraron diferencias significativas siendo mayor en la segunda. En los a os comprendidos entre el 2000 y el 2003 la SV a los 5 a os fue del 31 %, mientras que entre 2004 y 2008 fue del 63 %. No se empleó el trasplante de células progenitoras hematopoyéticas de forma sistemática. Conclusiones: estos resultados muestran un nivel comparable a los alcanzados a internacionalmente, lo que representa un importante logro del Sistema Nacional de Salud de Cuba. Introduction. acute myeloid leukemia represents about 20 % of all leukemias in childhood with results and survival smaller than in acute lymphoid leukemia. Objectives: to describe the results obtained with the treatment of acute myeloid leukemia in children in some Cuban centers in from 2000 to 2008. Methods: forty-six patients from Western provinces of Cuba including Sancti Spiritus and Villa Clara were treated. The majority of the patients were males. The median age was 9 years old. Two periods of treatment were applied, the first from 2000 to 2003 with 27 patients; and the second from 2004 to 2008 with 19 cases. The difference between the two periods was that in the second one the consolidation had short and intensive cycles. Results: the initial remission was 71 % in the first period and 89 % in the second. Free survival was 40 % in 5 years and the overall survival (OS) was 44 %, but in the first period the OS was 30,8 %
Limitación cognitiva en ni os con anemia drepanocítica sin historia de afectación neurológica Cognitive limitation present in children with drepanocytemia without a neurologic affection history
Raúl Martínez Triana,Eva Svarch,Andrea Menéndez Veitía
Revista Cubana de Hematolog?-a, Inmunolog?-a y Hemoterapia , 2009,
Abstract: En los últimos a os se comunican dificultades cognitivas en ni os con anemia drepanocítica (AD) sin afectación neurológica evidente y se insiste en la necesidad de atención especializada. Se estudiaron 29 pacientes sin ningún elemento recogido en sus historias clínicas que evidenciara afectación del SNC. Se aplicó la escala de inteligencia de Wechsler y se comparó con ni os sanos. Se obtuvo información de sus maestros sobre el rendimiento en las asignaturas de Matemática y Espa ol, interés mostrado en clases y disciplina. Se observó disminución en los cocientes de inteligencia (CI) de la escala total (p= 0,014) y de la escala ejecutiva (p= 0,008) y también en las subescalas semejanzas (p= 0,048), ordenar figuras (p= 0,017) y dise o de bloques (p= 0,001). Los maestros consideraron el rendimiento en Matemática (40 %) y Espa ol (36 %) menor que en los demás alumnos. El déficit neurocognitivo esta presente en ni os con AD sin historia previa de afectación del SNC, por lo que esta área debe evaluarse periódicamente como parte de su atención integral. In past years cognitive difficulties are reported in children with drepanocytemia (SCA) without evident neurologic affection, and we emphasized the need of a specialized care. A total of 29 patients were studied without any element evidencing affection of central nervous system (CNS) in registered in their medical records. We applied the Wechsler intelligence scale compared to health children. We get information from their teachers on performance in subjects like Mathematics and Spanish, interest and discipline during the classes. We note a decrease in intelligence quotient (IC) from the total scale (p= 0,014), and from rendition scale (p= 0,008), and also in similarity scales (p= 0,048), to figures array (p= 0,017), and block design (p= 0,001). Professors considered that Mathematics and Spanish performance (40 % and 36 %, respectively) lower than in the other pupils. Neurocognitive deficit is present in children with SCA without a prior history of CNS affection, thus, this area must to be periodically assessed as part of its integral care.
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