ResumenLa enfermedad de células falciformes (ECF) es un trastorno genético autosómico recesivo. Es la hemoglobinopatía estructural más frecuente en todo el mundo y se produce por alteración en los genes de la cadena de globina. En Chile, no hay datos sobre la prevalencia de la ECF ya que es considerada una condición muy rara. La incidencia de esta enfermedad ha venido aumentando debido a la migración de personas de áreas con mayor prevalencia de ECF. Por esta razón resulta importante conocer y considerar este diagnóstico en un grupo seleccionado de pacientes con anemia, para prevenir y tratar las diferentes complicaciones de la enfermedad. En este artículo se revisan los nuevos aportes en el conocimiento de la fisiopatología, con especial énfasis en aquellas publicaciones de consenso y guías relacionadas al diagnóstico y manejo de esta entidad. AbstractSickle cell disease (SCD) is an autosomal recessive genetic disorder. It is the most frequent structural hemoglobinopathy worldwide, and it is produced by an alteration in the globin chain genes. In Chile, there is no data on the prevalence of SCD since it is considered a very rare condition. The incidence of this disease has been increasing due to migration of people from areas with greater presence of SCD. It is important to know and consider this diagnosis in a selected group of patients with anemia, in order to prevent and treat the different complications of this disease. This article reviews the most recent information that shows new concepts in the knowledge of the physiopathology, and especially publications of guidelines and consensus in relation to the diagnosis and management of this condition. ARTÍCULO DE REVISIÓNEnfermedad de Células Falciformes -P. Zúñiga C. et al

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Children and Death The present article intends to create a more healthy relationship between death and the pediatric patient and, besides, deliver minimal tools to physicians, parents and children, in order to live this experience in a better way. To achieve this purpose, we based our study on bibliographical research and patientspsychologists-physicians experiences from pediatric oncology at our hospital. Piaget defines a clear progression in the child idea of death, ranging from intuition to abstraction. Although language will not be the same in every case, there is no minimum age for a child to deal in its own way with the death of the people who surround him. Not allowing children to suffer for their loved persons will not help them heal the wounds they experiment. An abnormal approach can determine mistaken, painful, and guilty conclusions that we must avoid.

Los autores declaran no tener conflicto de intereses. Este estudio no cuenta con financiamiento.

HSCT is an effective treatment for patients with WAS. The procedure should be done as soon as diagnosis is confirmed and before life threatening infections occur.