RESUMEN
The thalassaemias are a group of genetic disorders of haemoglobin which are endemic in the tropics but are now found worldwide due to migration. Basic standard of care therapy includes regular transfusions to maintain a haemoglobin level of around 10 g/dL, together with iron chelation therapy to prevent iron overload. Novel therapies, bone marrow transplantation, and gene therapy are treatment options that are unavailable in many countries with stressed economies. This Wider Perspectives article presents the strategies for management of an adolescent refugee patient with beta thalassaemia, as it would be performed by expert haematologists in six countries: Italy, Lebanon, Oman, the Sudan, Thailand and the United States. The experienced clinicians in each country have adapted their practice according to the resources available, which vary greatly. Even in the current modern era, providing adequate transfusions and chelation is problematic in many countries. On the other hand, ensuring adherence to therapy, particularly during adolescence, is a similar challenge seen in all countries. The concluding section highlights the disparities in available therapies and puts the role of novel therapies into a societal context.
Asunto(s)
Sobrecarga de Hierro , Talasemia , Talasemia beta , Adolescente , Humanos , Talasemia/epidemiología , Talasemia/terapia , Talasemia beta/epidemiología , Talasemia beta/terapia , Terapia por Quelación , Sobrecarga de Hierro/terapia , Sobrecarga de Hierro/tratamiento farmacológico , Transfusión SanguíneaRESUMEN
BACKGROUND: Enhanced external counterpulsation (EECP) has been reported to reduce blood pressure (BP) using clinic BP readings. The aim of this study was to assess the effect of a course of EECP on BP using ambulatory BP (ABP) measurements. HYPOTHESIS: EECP has a lasting BP lowering effect. METHODS: Patients referred for EECP due to refractory angina pectoris were consecutively included in the study. The ABPs were measured for 24 hours using a Spacelabs Ultralite 90217 device at 5 time points during the study period: 2 months before the EECP course, just before the EECP course, just after the EECP course, and at 3 and 12 months after EECP. Antihypertensive medication was held constant during the study period. Changes in BP were tested by repeated measures analysis. RESULTS: Fifty patients were included in the study. The mean age was 63 years, and 72% were male. The Canadian Cardiovascular Society Angina Grading Scale class improved from a mean of 2.6 to 1.5. The mean daytime ABPs were 114/69, 114/70, 115/71, 114/70, and 116/71 mm Hg and the mean nighttime ABPs were 107/63, 108/63, 106/62, 108/63, and 107/62 mm Hg at 2 months before the EECP course, just before the EECP course, just after the EECP course, and at 3 and 12 months after EECP, respectively (all P > 0.10). Further, when controlling for quartiles of baseline ABP level, no significant change in ABP was found. CONCLUSIONS: Enhanced external counterpulsation treatment has no lasting effect on ABP.