A national registry of haemoglobinopathies in Greece: deducted demographics, trends in mortality and affected births.

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.

An angiotensin-converting enzyme inhibitor improves left ventricular systolic and diastolic function in transfusion-dependent patients with beta-thalassemia major.

Background: Cardiac complications are the major cause of death in patients with beta-thalassemia major. The purpose of this study was to assess the impact of long-term treatment with the angiotensin-converting enzyme inhibitor enalapril on left ventricular (LV) performance, with an emphasis on diastolic LV function because diastolic dysfunction has been found to be an early event in an asymptomatic thalassemic population with only mild impairment of LV systolic function.

Methods: We used echocardiography to study the impact of treatment with oral enalapril on the evolution of standard M-mode and Doppler indices, along with a recently introduced Doppler index of combined systolic and diastolic LV performance.

Extramedullary paraspinal hematopoiesis in thalassemia: CT and MRI evaluation.

We present a comparative CT and MRI study of the paraspinal extramedullary hematopoiesis in 32 thalassemic patients. The patients were classified into four groups according to the MRI and CT imaging findings. Active recent extramedullary paraspinal hematopoietic masses show soft tissue behavior in both CT and MRI.