DREPANOCITOSIS PDF

Se estudiaron pacientes adultos con drepanocitosis, seguidos entre enero de y diciembre de ; del sexo femenino y del masculino. Introducción: la drepanocitosis es una enfermedad hematológica hereditaria y crónica. Se caracteriza por anemia crónica y diferentes trastornos asociados al. CARTA AL EDITOR. Program for comprehensive sickle cell disease care in Cuba . Programa de atención a la drepanocitosis en Cuba. Prof. Eva Svarch1, Dr.

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Ayuda con formatos de archivos: Hematology, 5pp.

Etiology Sickle cell anemia is determined by combinations of two abnormal alleles of the beta globin gene among which at least one carries the beta 6 glu-val mutation Hb S. Rev Cubana Hematol Inmunol Hemoter. Diagnostic methods Diagnosis is based on analysis of hemoglobin using isoelectric drepanocitois, by HPLC, solubility test Itano test and molecular analysis. Partial splenectomy in children with sickle cell disease.

Implications for pharmacological intervention. Genetic counseling Transmission is autosomal recessive. No fume y trate de evitar el humo de segunda mano.

La sobrevida en los enfermos en este estudio es mayor en la AD, similar en la HSC a la encontrada en algunos trabajos 3,20 superior a lo descrito en otros. This study reproduces the data described in the literaturefrom countries with a high prevalence of the disease.

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Se estudiaron pacientes adultos con drepanocitosis, seguidos entre enero de y diciembre de ; del drpeanocitosis femenino y del masculino. Hematology, 41pp.

Investigaciones para mejorar su salud. Eleven patients received hydroxyureafor recurrent vaso-occlusive crises with favorableresults; one patient underwent splenectomy and anotherreceived an allogenic bone marrow transplant from anHLA-identical brother with excellent results.

Morbiletalidad en pacientes adultos con drepanocitosis

Objective To present the casuistics of a pediatric hospital: An orphan drug based on hydroxycarbamide hydroxyurea has obtained European marketing authorization for the severe forms of the disease. Se evaluaron 23 episodios en 8 de 12 pacientes controlados por drepanocitosis. Sin embargo, las personas con el rasgo falciforme son portadores de un gen defectuoso de la hemoglobina S, de manera que drepanociotsis pasarlo a sus hijos. The presence of fetal hemoglobin means that the disease doesn’t manifest until after 3 months.

La hemoglobina falciforme no es como la hemoglobina normal. Temas de salud relacionados Sickle Cell Disease.

Drepanocitosis: experiencia de un centro | Anales de Pediatría

Puede haber desprendimiento de la retina. Predictors of fetal hemoglobin response in children with sickle cell anemia receiving hydroxyurea therapy.

Decrease of very late activation antigen-4 and CD36 on reticulocytes in sickle ell patients treated with hydroxyurea. Es posible que algunas personas deban ser admitidas en un hospital para recibir un tratamiento intenso. In 87 patients a neurocognitive study with the Wecshler intelligence scale was performed.

Additional information Further information on this disease Classification s 9 Drepnocitosis s 1 Disability Clinical signs and symptoms Publications in Drepanoocitosis Other website s A two-year pilot trial of hydroxyurea in very young children with sickle-cell anemia.

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Many diverse complications can occur. In 5 pregnancies, molecular prenatal diagnosis was performed and foetuses were found with sicklecelldisease SCD personal communication. The comprehensive care includes: Conclusions Acute thoracic syndrome is frequent in sickle cell disease and is more severe in children older than 3 years.

Hubo 4 muertes maternas y 10 muertes perinatales. Ortega Molina a A.

Síntomas y tratamientos

Pediatr Clin North Am, 47pp. Esto es especialmente cierto en las primeras semanas del tratamiento. Methods We performed a retrospective review of all the episodes of acute thoracic syndrome diagnosed at our center in patients younger than 18 years of age with sickle cell anemia. Esto causa anemia severa. Get Access Get Access. Elsevier About ScienceDirect Remote access Shopping cart Contact and support Terms and conditions Privacy policy We use cookies to help provide and enhance our service and tailor content and ads.

Cervera Bravo a M. En raras ocasiones, puede empeorar la anemia. Physiologic decline in fetal hemoglobin parameters in infants with sickle cell disease: