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Ann Hematol ; 91(9): 1451-8, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-22526366

RESUMEN

Haemoglobinopathies are the most common hereditary disorders in Greece. Although there is a successful national prevention program, established 35 years ago, there is lack of an official registry and collection of epidemiological data for haemoglobinopathies. This paper reports the results of the first National Registry for Haemoglobinopathies in Greece (NRHG), recently organized by the Greek Society of Haematology. NRHG records all patients affected by thalassaemia major (TM), thalassaemia intermedia (TI), "H" Haemoglobinopathy (HH) and sickle cell disease (SCD). Moreover, data about the annual rate of new affected births along with deaths, between 2000 and 2010, are reported. A total of 4,506 patients are registered all over the country while the number of affected newborns was significantly decreased during the last 3 years. Main causes for still having affected births are: (1) lack of medical care due to financial reasons or low educational level; (2) unawareness of time limitations for prenatal diagnosis (PD); due either to obstetricians' malpractice or to delayed demand of medical care of couples at risk; and (3) religious, social or bioethical reasons. Cardiac and liver disorders consist main causes for deaths while life expectancy of patients lengthened after 2005 (p < 0.01). The NRHG of patients affected by haemoglobinopathies in Greece provides useful data about the haemoglobinopathies in the Greek population and confirms the efficacy of the National Thalassaemia Prevention Program on impressively decreasing the incidence of TM and sickle cell syndromes.


Asunto(s)
Hemoglobinopatías/epidemiología , Sistema de Registros , Aborto Eugénico/psicología , Aborto Eugénico/estadística & datos numéricos , Anemia de Células Falciformes/economía , Anemia de Células Falciformes/epidemiología , Anemia de Células Falciformes/prevención & control , Causas de Muerte , Emigrantes e Inmigrantes/estadística & datos numéricos , Fertilización In Vitro , Asesoramiento Genético , Pruebas Genéticas , Grecia , Hemoglobinopatías/economía , Hemoglobinopatías/mortalidad , Hemoglobinopatías/prevención & control , Humanos , Incidencia , Recién Nacido , Educación del Paciente como Asunto , Diagnóstico Prenatal , Factores Socioeconómicos , Talasemia/economía , Talasemia/epidemiología , Talasemia/prevención & control
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