Τίτλος:
National registry of hemoglobinopathies in Greece: updated demographics,
current trends in affected births, and causes of mortality
Περίληψη:
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 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.
Συγγραφείς:
Voskaridou, Ersi
Kattamis, Antonis
Fragodimitri, Christina and
Kourakli, Alexandra
Chalkia, Panagiota
Diamantidis, Michael and
Vlachaki, Efthymia
Drosou, Marouso
Lafioniatis, Stilianos and
Maragkos, Konstantinos
Petropoulou, Fotini
Eftihiadis, Eftihios
and Economou, Marina
Klironomos, Evangelos
Koutsouka, Freideriki
and Nestora, Konstantina
Tzoumari, Ioanna
Papageorgiou, Ourania
and Basileiadi, Artemis
Lafiatis, Ioannis
Dimitriadou, Efthimia
and Kalpaka, Anastasia
Kalkana, Chrysoula
Xanthopoulidis,
Georgios
Adamopoulos, Ioannis
Kaiafas, Panagiotis
Mpitzioni,
Aikaterini
Goula, Anastasia
Kontonis, Ioannis
Alepi,
Chrisoula
Anastasiadis, Athanasios
Papadopoulou, Margarita and
Maili, Polixeni
Dionisopoulou, Dionisia
Tsirka, Antigoni and
Makis, Alexandros
Kostaridou, Stavroula
Politou, Marianna and
Papassotiriou, Ioannis
Greek Haemoglobinopathies Study
