[Consensus document for the diagnosis and treatment of pyruvate kinase deficiency]. / Documento de consenso para el diagnóstico y tratamiento del déficit de piruvato quinasa.

Pyruvate kinase (PK) deficiency is the second most frequent enzymopathy and the most common cause of chronic hereditary non-spherocytic haemolytic anaemia. Its global prevalence is underestimated due to low clinical suspicion of mild cases, associated with difficulties in the performance and interpretation of PK enzymatic activity assays. With the advent of next generation sequencing techniques, a better diagnostic approach is achieved. Treatment remains based on red blood cell transfusions and splenectomy, with special attention to iron overload, not only in transfusion-dependent patients. Nowadays, allogeneic hematopoietic stem cell transplantation is the only curative treatment, recommended only in selected cases of severely affected patients with an HLA-identical donor. Novel pharmacological and gene therapies are in clinical trials, with promising results. In this article, the Spanish Erythropathology Group reviews the current situation of PK deficiency, paying special attention to the usefulness of different diagnostic techniques and to actual and emerging treatments.

Autoimmune haemolytic anaemias: A retrospective study of 93 patients. / Anemias hemolíticas autoinmunes: estudio retrospectivo de 93 pacientes.

INTRODUCTION: Autoimmune haemolytic anaemia (AIHA) is an infrequent and heterogeneous disease in its pathophysiology and clinical behaviour, therefore it is generally managed empirically. PATIENTS AND METHODS: We conducted an observational, retrospective and multicentre study of 93 patients diagnosed with AHAI in 9 Spanish hospitals between 1987 and 2017, with a median follow-up of 28 months. RESULTS: Median age of 67 years; 85% AHAI for hot antibodies and 64% primary AHAI. The lowest haemoglobin values at diagnosis related to patients under 45 years of age and serological type IgG+C. Of the patients, 92% received first line treatment, 54% second line, and 27% third line. The warm AHAI were treated in first line with steroids, with overall responses of 83% and complete of 58%. Rituximab in monotherapy or in association with steroids was administered to 34 patients with overall responses close to 100% (complete responses 40-60%), relegating splenectomy to the third line. The immunosuppressive treatment was administered in patients with autoimmune diseases or in corticoid-dependent patients. DISCUSSION: We found high rates of response to steroids, with very prolonged treatments that cause side effects and corticoid dependence in a third of patients. The combination of steroids with rituximab in the first line, could be indicated in patients with low levels of haemoglobin and serological type IgG+C. The high relapse rates make necessary the development of randomised studies with new drugs or the combination with existing ones, which allow longer response times and with fewer side effects.

[Paroxysmal nocturnal hemoglobinuria therapy with eculizumab: Spanish experience]. / Tratamiento de la hemoglobinuria paroxística nocturna con eculizumab: experiencia en España.

BACKGROUND AND OBJECTIVES: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disease characterized by complement-mediated hemolysis, bone marrow failure and thrombosis. Eculizumab is a humanized monoclonal antibody that blocks the cytolytic component of the complement system by binding to complement C5. MATERIAL AND METHODS: We report the results of eculizumab treatment in 25 PNH patients from different centers in Spain. Statistical analysis was perfomed with a SPSS v15.0 software. RESULTS: Fifty-eight per cent of the patients achieved transfusional independence after a median of 14 months. Transfusion requirements were reduced in 60% of the remaining cases. Fatigue resolved in 96% of the patients and smooth muscle dystony-related symptoms in all cases. A single case of treatment-related infection was observed. CONCLUSIONS: Eculizumab controls effectively hemolysis and greatly improves clinical symptoms. The drug is safe and well tolerated, without significant adverse effects except meningococcal infection. Patients with suboptimal response to treatment must be assessed for bone marrow insufficiency and extravascular haemolysis.

Nuevas configuraciones de lo escolar: pensar la autoridad pedagógica emancipatoria en la educación de jóvenes y adultos / New school configurations: thinking pedagogical authority in youth and adult education

En el marco del proyecto de investigación "configuraciones de la autoridad en educación"¹, este artículo se propone caracterizar y analizar experiencias educativas que colaboran en la configuración de nuevas formas de autoridad pedagógica en escenarios escolares destinados a jóvenes y adultos en situaciones de vulnerabilidad educativa. El análisis se organiza en torno a dos ejes que consisten en la problematización de la relación pedagógica tradicional y en el desdibujamiento de la frontera entre saber escolar y no escolar. En función de ello, nos preguntamos cuáles son aquellos rasgos, gestos y iguras que surgen en las escenas educativas y que habilitan nuevos procesos de subjetivación acercándonos a imaginar una educación emancipadora.

Whitin de framework of the research "configurations of the educational authority"², this article proposes characterize and analyze educational experiences which contribute to new forms of pedagogical authority into schools for youths and adults in a vulnerability situation. This project is organized around two central themes based on the critical analysis of the tradicional pedagogical relationship and the undefined line between school knowledge and non school knowledge. Based on this, we look for those characteristics, gestures and igures that emerge in educational scenes and enable new processes of subjectivation in order to imagine an emancipatory education.

Factores asociados al consumo de tabaco en una población de adultos / Risk factors associated with tobacco consumption in an adult population

Esta investigación se planteó identificar factores asociados a la adicción al tabaco. El abordaje se basó en una articulación entre el paradigma transteorético de Prochaska y la perspectiva comunitaria de la salud pública. El universo lo constituyeron los integrantes de escuelas estatales para adultos del Partido de Avellaneda, Provincia de Buenos Aires. El total de la muestra fue de 888 sujetos. Se trabajó por censo. Se evaluaron los factores que se asociaban al consumo de tabaco en la población diana y de las variables que definían la fase en que se encontraban los sujetos según el modelo Transteorético de Prochaska. En nuestra población a diferencia de lo encontrado por Prochaska, existe una proporción mayor de sujetos en etapa de precontemplación (51,8 por ciento). Además, dentro del síndrome de abstinencia, predomina el síntoma de ansiedad. Por último, son las situaciones de afecto negativo las que provocan mayor tentación de fumar.