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.
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.
was calculated. Considering a switch to aztreonam lysine, the reduction of exacerbation was estimated in order to maintain the overall treatment cost. For the base case, ex-factory prices were considered; for sensitivity analysis, discounts ranged from 15 to 50%. Results: Switching to aztreonam lysine would not increase overall treatment costs in 8/12 options; even maintaining current exacerbation episodes could lead to SNS savings. In the remaining options, the required reduction of exacerbations varies depending on the patient's current situation, ranging from 110% (one episode/ year) to 18% (six episodes/year). In the sensitivity analysis, there are 3 options in which switching to aztreonam lysine would not imply higher treatment costs. For the remaining alternatives, the required reduction of exacerbations does not change significantly from the base case: from 116% (one episode/year) to 19% (six episodes/ year). ConClusions: In CF patients with chronic pulmonary infection by PA, hospitalizations costs due to exacerbations have a greater economic impact than drug treatment costs. Using aztreonam lysine, instead of other treatments, would not imply a budget impact in a wide range of patient profiles if exacerbation episodes can be reduced. As the number of current exacerbations increases, a lesser reduction of these events is required by a switch to aztreonam lysine to avoid incremental treatment costs.objeCtives: Meprobamate is a constituent of various combination analgesics in South Africa. Due to its abuse potential, the scheduling status of meprobamate is currently under review with the possibility of introducing stricter controls on its availability. The primary aim was to determine the impact of generic substitution on meprobamate-containing combination analgesic prescribing. Methods: A retrospective, cross-sectional drug utilisation study was conducted on prescription data of a medical insurance scheme administrator in South Africa for 2011. Results: A total of 97 491 analgesics were dispensed to 31 854 patients during the year. Within ATC category N02B, 62.10% of prescriptions were for analgesic combinations, of which 20 326 prescriptions were for meprobamate-containing analgesics at a cost of R282 930. A total of 10 404 patients received meprobamate-containing analgesics. Overall, 20.85% of all analgesics contained meprobamate. Patients who received meprobamate-containing analgesics were slightly older (39.52 years) compared to patients who received analgesics in general (33.61 years). Mandatory generic substitution exists in South Africa, unless otherwise indicated by the prescriber or if the patient refuses. Twenty-two trade names of meprobamate-containing analgesics were dispensed, of which 17 (the originator plus 16 branded generics) contained exactly the same dosages of active ingredients (320 mg paracetamol, 8 mg codeine phosphate, 32 mg caffeine and 150 mg meprobamate). In previous studies, the originator product was the most often prescribed, yet in this study the originator product only consti...
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