Em espanhol: Enfermedad de células falciformes: conductas básicas para el tratamiento .. a amostra 3 (Hb SS,com anemia falciforme; a amostra 4 (Hb S. PDF | Guía de Práctica Clínica | ResearchGate, the professional network Lesión crónica cerebral en la anemia falciforme y su relación con la. de muitos passos, envolvendo células sanguíneas, proteínas plasmá- hemoglobina (Hb) S nas hemácias: anemia falciforme (Hb SS),. Hb SC, S- talassemias.
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pressas anomalamente, interação entre hemácia falciforme, células endoteliais, tes, como anemia, dor e insuficiência de múltiplos órgãos são abordadas. Introducción. La enfermedad de células falciformes (ECF), anemia de origen genético, es un problema de salud emergente en España. La Sociedad Española. DOI: /S(03) Full text access. Anemia de células falciformes: una enfermedad emergente en España. Visits. Download PDF.
In two studies, children with SCD, who were also at an increased risk of having a first or second stroke, were randomly selected to receive hydroxyurea and phlebotomy collection of blood or blood transfusion and chelation administration of agents to remove excess iron from the body.
There was moderate quality evidence from these six studies that those receiving hydroxyurea experienced significant reductions in the frequency of pain crises, increases in fetal haemoglobin and decreases in neutrophil white blood cell counts compared to the comparator treatment.
There was no difference between people receiving hydroxyurea or other treatments in terms of quality of life, deaths during the studies and side effects including serious and life-threatening side effects ; however, there is less information about these outcomes in the studies, so the quality of this evidence is low.
Two further studies were included in the review. In one study , 22 children with SCD, who were also at an increased risk of having a stroke, were randomly selected to receive hydroxyurea or no treatment observation only and in one study 44 adults and children were randomly selected to receive treatments with or without adding hydroxyurea.
Both studies showed an increase in fetal haemoglobin for people receiving hydroxyurea compared to the comparator treatment and there were no deaths during the studies.
There was no difference between people receiving hydroxyurea or other treatments in terms of pain crises and side effects including serious or life-threatening side effects and these studies did not measure quality of life. Conclusions The evidence shows that hydroxyurea is likely to be effective in the short term at decreasing the frequency of painful episodes and raising fetal haemoglobin levels in the blood in people with SCD.
Hydroxyurea is also likely to be effective in preventing first strokes for those at an increased risk of stroke and does not seem to be associated with an increase in any side effects including serious and life-threatening side effects. There is currently not much evidence on whether hydroxyurea is beneficial over a long period of time, what the best dose to take is, or whether treatment causes any long-term or serious side effects.
More studies are needed to answer these questions. However, there is still insufficient evidence on the long-term benefits of hydroxyurea, particularly in preventing chronic complications of SCD, recommending a standard dose or dose escalation to maximum tolerated dose.
There is also insufficient evidence about the long-term risks of hydroxyurea, including its effects on fertility and reproduction. Evidence is also limited on the effects of hydroxyurea on individuals with HbSC genotype.
Future studies should be designed to address such uncertainties. Read the full abstract Background: Sickle cell disease SCD is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea hydroxycarbamide , an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin.
Objectives: To assess the effects of hydroxyurea therapy in people with SCD all genotypes , of any age, regardless of setting. Search strategy: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Register, comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.
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