Anales de pediatría : publicación oficial de la Asociación Española de Pediatría (A.E.P.)
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The prevalence of hemoglobinopathies in Spain is increasing as a result of immigration. Thalassemia major presents with chronic hemolytic anemia that requires regular red blood cell transfusions within the first year of life. Patients with sickle cell disease suffer from chronic anemia, vasculopathy and progressive damage in almost any organ. There is decreased life expectancy in both conditions. Allogeneic hematopoietic stem cell transplantation represents the only potentially curative option. ⋯ We conclude that for thalassemia major and sickle cell disease, allogenic hematopoietic stem cell transplantation from HLA-geno-identical siblings offers a high probability of complication-free survival. Despite good results, morbidity and mortality associated with transplantation from unrelated donors is a risk that might be considered, in contrast to a non-curative medical treatment that offers a long term survival. For thalassemia major groups it could be an option, but not for sickle cell disease, which is still in the investigational phase.
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The objective of this investigation is to determine whether bispectral index (BIS®) monitoring during intravenous anaesthesia with spontaneous breathing for upper gastrointestinal endoscopy (UGE) in a pediatric population is useful for: a) decreasing the amount of drug, b) decreasing the time for awakening, and c) improving patient safety. ⋯ BIS monitoring for diagnostic UGE in spontaneous breathing in a pediatric population is feasible, but does not appear to decrease awakening time or the amount of propofol needed. Furthermore, there was no statistically significant decrease in the number of adverse effects.