La drepanocitosis en cuba. Estudio en niños

Eva Svarch, Beatriz Marcheco-Teruel, Sergio Machín-García, Andrea Menéndez-Veitía, Ileana Nordet-Carrera, Alberto Arencibia-Núñez, Aramís Núñez-Quintana, Lic Raúl Martínez-Triana, Claudio Scherle-Matamoros, Eliécer San Román-García, Alejandro González-Otero, Ernesto de la Torre-Montejo

Producción científica: Contribución a una revistaArtículorevisión exhaustiva

11 Citas (Scopus)

Resumen

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.

Título traducido de la contribuciónDrepanocytosis in cuba: Study in children
Idioma originalEspañol
Páginas (desde-hasta)51-67
Número de páginas17
PublicaciónRevista Cubana de Hematologia, Inmunologia y Hemoterapia
Volumen27
N.º1
EstadoPublicada - ene. 2011
Publicado de forma externa

Palabras clave

  • Drepanocytosis
  • Hydroxyurea
  • Partial splenectomy
  • Splenic sequestration crisis
  • Thoracic syndrome
  • Vaso-occlusive crisis

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