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08.09.2018 | Original Article

National registry of hemoglobinopathies in Greece: updated demographics, current trends in affected births, and causes of mortality

verfasst von: Ersi Voskaridou, Antonis Kattamis, Christina Fragodimitri, Alexandra Kourakli, Panagiota Chalkia, Michael Diamantidis, Efthymia Vlachaki, Marouso Drosou, Stilianos Lafioniatis, Konstantinos Maragkos, Fotini Petropoulou, Eftihios Eftihiadis, Marina Economou, Evangelos Klironomos, Freideriki Koutsouka, Konstantina Nestora, Ioanna Tzoumari, Ourania Papageorgiou, Artemis Basileiadi, Ioannis Lafiatis, Efthimia Dimitriadou, Anastasia Kalpaka, Chrysoula Kalkana, Georgios Xanthopoulidis, Ioannis Adamopoulos, Panagiotis Kaiafas, Aikaterini Mpitzioni, Anastasia Goula, Ioannis Kontonis, Chrisoula Alepi, Athanasios Anastasiadis, Margarita Papadopoulou, Polixeni Maili, Dionisia Dionisopoulou, Antigoni Tsirka, Alexandros Makis, Stavroula Kostaridou, Marianna Politou, Ioannis Papassotiriou, on behalf of the Greek Haemoglobinopathies Study Group

Erschienen in: Annals of Hematology | Ausgabe 1/2019

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Abstract

National registries constitute an invaluable source of information and contribute to the improvement of hemoglobinopathy management. Herein, we present the second updated report of the National Registry for Haemoglobinopathies in Greece (NRHG) and critically discuss the time trends in demographics, affected births, and causes of mortality. Thirty-eight Greek hemoglobinopathy units reported data from diagnosis to the last follow-up or death by retrospectively completing an electronic form. Four thousand thirty-two patients were eligible for inclusion; more than half of them had thalassaemia major. Compared to the previous report, a reduction in the total number of all hemoglobinopathies except for hemoglobinopathy “Η” was evident. The total number of affected births was also reduced; most of them were attributable to diagnostic errors and lack of awareness. Importantly, data on iron overload are reported for the first time; although most patients had low or moderate liver iron concentration (LIC) values, a non-negligible proportion of patients had high LIC. The burden due to heart iron overload was less prominent. Cardiac- and liver-related complications are the major causes of morbidity and mortality. From 2000 to 2015, a decrease in heart-related deaths along with an increase in liver-associated fatalities was observed. The Hellenic Prevention Program along with advances in chelation regimens and iron status monitoring have resulted in improved patient outcomes. The NRHG gives insight into the effectiveness of prevention programs, the therapeutic management of hemoglobinopathies and associated outcomes. NRHG may contribute to the formulation of a roadmap for hemoglobinopathies in Europe and promote the implementation of effective public health policies.

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Titel: National registry of hemoglobinopathies in Greece: updated demographics, current trends in affected births, and causes of mortality
verfasst von: Ersi Voskaridou
Antonis Kattamis
Christina Fragodimitri
Alexandra Kourakli
Panagiota Chalkia
Michael Diamantidis
Efthymia Vlachaki
Marouso Drosou
Stilianos Lafioniatis
Konstantinos Maragkos
Fotini Petropoulou
Eftihios Eftihiadis
Marina Economou
Evangelos Klironomos
Freideriki Koutsouka
Konstantina Nestora
Ioanna Tzoumari
Ourania Papageorgiou
Artemis Basileiadi
Ioannis Lafiatis
Efthimia Dimitriadou
Anastasia Kalpaka
Chrysoula Kalkana
Georgios Xanthopoulidis
Ioannis Adamopoulos
Panagiotis Kaiafas
Aikaterini Mpitzioni
Anastasia Goula
Ioannis Kontonis
Chrisoula Alepi
Athanasios Anastasiadis
Margarita Papadopoulou
Polixeni Maili
Dionisia Dionisopoulou
Antigoni Tsirka
Alexandros Makis
Stavroula Kostaridou
Marianna Politou
Ioannis Papassotiriou
on behalf of the Greek Haemoglobinopathies Study Group
Publikationsdatum: 08.09.2018
Verlag: Springer Berlin Heidelberg
Erschienen in: Annals of Hematology / Ausgabe 1/2019
Print ISSN: 0939-5555
Elektronische ISSN: 1432-0584
DOI: https://doi.org/10.1007/s00277-018-3493-4

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