Background. Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab. Methods. We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA resolution, defined by a normalization of platelet count (>150 × 109/L) and haemoglobin, disappearance of all the markers of microangiopathic haemolytic anaemia (MAHA), and improvement of renal function, with a ≥25% reduction of serum creatinine from the onset of eculizumab administration. Results. Twenty-nine patients with secondary aHUS (15 drug-induced, 8 associated with systemic diseases, 2 with postpartum, 2 with cancer-related, 1 associated with acute humoral rejection and 1 with intestinal lymphangiectasia) were included in this study. The reason to initiate eculizumab treatment was worsening of renal function and persistence of TMA despite treatment of the TMA cause and plasmapheresis. All patients showed severe MAHA and renal function impairment (14 requiring dialysis) prior to eculizumab treatment and 11 presented severe extrarenal manifestations. A rapid resolution of the TMA was observed in 20 patients (68%), 15 of them showing a ≥50% serum creatinine reduction at the last follow-up. Comprehensive genetic and molecular studies in 22 patients identified complement pathogenic variants in only 2 patients. With these two exceptions, eculizumab was discontinued, after a median of 8 weeks of treatment, without the occurrence of aHUS relapses. Conclusion. Short treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition.
Los beneficios de la Educación Física en la niñez y adolescencia han sido abordados por múltiples investigadores. Sin embargo, en la infancia, etapa de 0 a 6 años, existen pocos trabajos que abordasen el estudio de los efectos producidos por programas de Educación Física estructurados. Este trabajo tiene como principal objetivo realizar una revisión sistemática sobre los principales trabajos de investigación que abordaron los efectos de programas de Educación Física en el desarrollo motor, cognitivo, social, afectivo-emocional y salud de niños de 0 a 6 años. Se ha seguido el protocolo PRISMA utilizándose un total de 5 bases de datos internacionales y nacionales (Web of Science, Scopus, Sport Discus, Psycinfo y Dialnet). Se obtuvieron un total de 1370 artículos potenciales, de los cuales 30 cumplieron los criterios de inclusión y exclusión. Todos ellos son estudios empíricos realizados en diferentes países. Los resultados confirman un mayor número de evidencias científicas centradas en el área del desarrollo cognitivo, seguida de la esfera motriz. Se han encontrado también diversos estudios que abordan mejoras en la salud de los niños, la esfera social, afectiva y emocional. Se concluye la necesidad de implementar más programas de Educación Física estructurados en la etapa de 0 a 6 años, a fin de favorecer el desarrollo de diferentes áreas de los niños y mejorar su salud. En ningún estudio se encontraron efectos negativos para su desarrollo. Por ello, se invita a las administraciones educativas a incorporar un mayor número de horas de Educación Física en la Educación Infantil.
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