Abstract Alpha-thalassemias are among the most common genetic diseases in the world. They are characterized by hypochromic and microcytic anemia and great clinical variability, ranging from a practically asymptomatic phenotype to severe anemia, which can lead to intrauterine or early neonatal death. Deletions affecting the α-globin genes, located on chromosome 16p13.3, are the main causes of α-thalassemia. Multiplex ligation-dependent probe amplification (MLPA) can be used to detect rearrangements that cause α-thalassemia, particularly large deletions involving the whole α cluster and/or deletions in the HS-40 region. Here, MLPA was used to investigate the molecular basis of α-thalassemia in five unrelated patients, three of whom had Hb H disease. In addition to the -α3.7 deletion identified in the patients with Hb H disease, four different α0 deletions removing 15 to 225 kb DNA segments were found: two of them remove both the α genes, one affects only the regulatory element (HS-40) region, and another one extends over the entire α cluster and the HS-40 region. These results illustrate the diversity of α-thalassemia deletions in the Brazilian population and highlight the importance of molecular investigation in cases that present with microcytosis and hypochromia without iron deficiency and normal or reduced Hb A2 levels..

O início do século XXI é testemunha da ascensão ao poder de novos governos de esquerda e centro-esquerda na América do Sul, que apresentam pelo menos duas características em comum: o questionamento das políticas e reformas pró-mercado ocorridas na década anterior e a volta do Estado como ator central da vida econômica. A despeito desse ponto em comum, uma análise mais aprofundada nos permite perceber certa heterogeneidade de natureza programática e organizacional dos novos governos progressistas. Nesse sentido, propomos analisar as respostas dadas por Argentina e Brasil à crise do neoliberalismo, dando especial ênfase às políticas econômicas para o desenvolvimento e suas implicações para a integração regional. Esta análise mais sistemática permite-nos perceber que a Argentina e o Brasil têm feito escolhas distintas no que diz respeito ao tipo de política adotada. O Brasil tem recorrido a práticas mais ortodoxas, como políticas monetárias restritivas para conter as expectativas de inflação, enquanto a Argentina tem preferido medidas heterodoxas, como controle de preços e restrição às exportações, bem como outras políticas de incentivo ao consumo. Em certa medida, a diferença entre as escolhas pode ser atribuída à própria trajetória econômica e política de ambos os países: as reformas pró-mercado, por exemplo, foram mais intensas na Argentina do que no Brasil, o que implicou, no que diz respeito à estrutura produtiva, uma maior desindustrialização e a extinção de algumas instituições desenvolvimentistas.

The early 21st century is witness to the rise to power of new leftist and center-left governments in South America which have at least two common traits: the questioning of pro-market policies and reforms which were carried out over the preceding decade and the return of the State as a central actor in economic life. In spite of this convergence, a deeper analysis would allow us to perceive a certain heterogeneity in the programmatic and organizational character of the new progressive governments. In this regard, we propose an analysis of the responses to the crisis of neo-liberalism that the Brazilian and Argentine governments have provided, placing special emphasis on economic development policies and their implications for regional integration. This more systematic analysis enables us to see that Argentina and Brazil have made different choices regarding what type of policies they have adopted. Brazil has resorted to more orthodox practices such as restrictive monetary policies meant to contain inflation, while Argentina has preferred more heterodox methods such as price controls and export restriction, as well as other policies that encourage consumption. To a certain degree, the differences between the choices made can be attributed to the very economic and political trajectory of each country: pro-market reforms were more intense in Argentina than in Brazil, which meant, regarding productive structure, greater de-industrialization and the extinction of several developmentalist institutions.

Le début du XXIème siècle est témoin de l'ascension au pouvoir de nouveaux gouvernements de gauche et de centre-gauche en Amérique du sud, qui ont au moins deux caractéristiques en commum : la mise en question des politiques et réformes favorables au marché survenues dans les annéesprécédentes et le retour de l'État en acteur central de la vie économique. En dépit de ce point commum, une analyse plus approfondie nous permet de remarquer cette hétérogéneité de nature programmatique et organisationnelle des nouveaux gouvernements progressistes. En ce sens, nous proposons d'analyser les réponses données par l'Argentine et le Brésil à la crise du néolibéralisme, en soulignant les politiques économiques pour le développement et ses retombées concernant l'intégration régionale. Cette analyse plus pointue nous permet de comprendre que l'Argentine et le Brésil ont fait des choix différents en ce qui concerne le type de politique à adopter. Le Brésil a fait appel à des actions plus orthodoxes, comme les politiques monétaires restrictives pour contrôler les expectatives d'inflation, tandis que l'Argentine a préféré les mesures hétérodoxes, comme le contrôle de prix et la restriction aux exportations, ainsi que d'autres politique d'encouragement à la consommation. D'une certaine façon, la différence entre les choix peut être attribuée à la trajectoire économique elle-même et à la politiques des deux pays : les réformes en faveur du marché, par exemple, ont été plus intenses en Argentine qu'au Brésil, ce qui a entraîné, en ce qui concerne la structure productive, une plus forte désindustrialisation et l'extinction de certaines institutions pour le développement.