A national registry of haemoglobinopathies in Greece: Deducted demographics, trends in mortality and affected births
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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.
KeywordsRegistry Thalassaemia Sickle cell disease Greece
The authors would like to thank the patients, their families and the medical and nursing staff of the following centers: General Hospital of Athens "Laiko", Children’s University Hospital of Athens "Agia Sofia", University General Hospital of Thessaloniki AHEPA, University Hospital of Thessaloniki Ippokrateion, University Hospital of Patras, University Hospital Hippocration of Athens, University Hospital of Larissa, General Hospital "Achilopouleio" of Volos, "DRAKOPOULEIO" Blood Centre of Athens, University Hospital of Ioannina, Venizeleio Hospital of Heraklion, General Hospital of Corfu, General Hospital of Karditsa, General Hospital of Corinthos, "Karamandaneio" Children's Hospital, Children’s Hospital of Penteli, General Hospital of Rhodes, Children's Hospital "Pan. & Aglaia Kyriakou" of Athens, General Hospital "Agios Panteleimon" of Nikea, General Hospital of Chalkida, General Hospital "Agios Pavlos" of Thessaloniki, General Hospital of Chania, General Hospital of Kalamata, General Hospital Vostanion of Mytilini, General Hospital "G. Gennimatas" Athens, General Hospital of Xanthi, General Hospital of Trikala, General Hospital of Arta, General Hospital of Serres, Social Security Institute of Pireaus, General Hospital of Sparte, University Hospital of Alexandroupoli, University Hospital of Heraklio "PEPAGNI", General Hospital of Katerini, General Hospital of Kavala, General Hospital of Kozani "Mamatseio", Hospital "Chatzikostas" of Ioannina, General Hospital of Amaliada, General Hospital of Rethymno, General Hospital of Kos, General Hospital of Athens “Aretaieio”, General Hospital of Tripoli, Children’s Hospital of Athens "Agia Sofia". Furthermore, the authors want to especially thank Mrs. Maria Tsalkani for her effort to collect this large amount of data in a limited period of time.
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