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      Survival and mortality among users and non-users of hydroxyurea with sickle cell disease

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          Abstract

          OBJECTIVE:

          to estimate survival, mortality and cause of death among users or not of hydroxyurea with sickle cell disease.

          METHOD:

          cohort study with retrospective data collection, from 1980 to 2010 of patients receiving inpatient treatment in two Brazilian public hospitals. The survival probability was determined using the Kaplan-Meier estimator, survival calculations (SPSS version 10.0), comparison between survival curves, using the log rank method. The level of significance was p=0.05.

          RESULTS:

          of 63 patients, 87% had sickle cell anemia, with 39 using hydroxyurea, with a mean time of use of the drug of 20.0±10.0 years and a mean dose of 17.37±5.4 to 20.94±7.2 mg/kg/day, raising the fetal hemoglobin. In the comparison between those using hydroxyurea and those not, the survival curve was greater among the users (p=0.014). A total of 10 deaths occurred, with a mean age of 28.1 years old, and with Acute Respiratory Failure as the main cause.

          CONCLUSION:

          the survival curve is greater among the users of hydroxyurea. The results indicate the importance of the nurse incorporating therapeutic advances of hydroxyurea in her care actions.

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          Most cited references53

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          The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS).

          The aim of this prospective study was to evaluate the long-term efficacy and safety of hydroxyurea (HU) in patients with sickle cell disease (SCD). Thirty-four patients with sickle cell anemia (hemoglobin S [HbS]/HbS), 131 with HbS/beta(0)-thal, and 165 with HbS/beta(+)-thal participated in this trial. HU was administered to 131 patients, whereas 199 patients were conventionally treated. The median follow-up period was 8 years for HU patients and 5 years for non-HU patients. HU produced a dramatic reduction in the frequency of severe painful crises, transfusion requirements, hospital admissions, and incidence of acute chest syndrome. The probability of 10-year survival was 86% and 65% for HU and non-HU patients, respectively (P = .001), although HU patients had more severe forms of SCD. The 10-year probability of survival for HbS/HbS, HbS/beta (0)-thal, and HbS/IVSI-110 patients was 100%, 87%, and 82%, respectively, for HU patients and 10%, 54%, and 66%, for non-HU patients. The multivariate analysis showed that fetal hemoglobin values at baseline and percentage change of lactate dehydrogenase between baseline and 6 months were independently predicted for survival in the HU group. These results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients.
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            Hydroxyurea for the treatment of sickle cell anemia.

            Manu Platt (2008)
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              Hydroxyurea: effects on hemoglobin F production in patients with sickle cell anemia.

              Patients with sickle cell anemia were treated with daily doses of hydroxyurea, to assess pharmacokinetics, toxicity, and increase in fetal hemoglobin (Hb) production in response to the drug. Plasma hydroxyurea clearances were not a useful guide to maximum tolerated doses of the drug. The mean daily single oral dose that could be maintained for at least 16 weeks was 21 mg/kg (range, 10 to 35 mg/kg). Among 32 patients, last HbF levels were 1.9% to 26.3% (mean, 14.9%) with increases in HbF over initial values of 1.4% to 20.2% (mean, 11.2%). The most significant predictors of last HbF were last plasma hydroxyurea level, initial white blood count and initial HbF concentration. Last HbF was not related to beta globin haplotype or alpha globin gene number. No serious toxicity was encountered. Clinically significant bone marrow depression was avoided, and chromosome abnormalities after 2 years of treatment were no greater than those observed before treatment. The period of observation has been too short to evaluate the risk of carcinogenesis. Patient's red cells developed striking macrocytosis. Median red cell Hb concentrations did not change. Hb concentrations increased, on average 1.2 g/dL, but serum erythropoietin levels increased. Patients' body weights increased, and some returned to work or school, but no conclusions regarding therapeutic efficacy could be drawn from this uncontrolled open-label study.
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                Author and article information

                Journal
                Rev Lat Am Enfermagem
                Rev Lat Am Enfermagem
                Revista Latino-Americana de Enfermagem
                Escola de Enfermagem de Ribeirão Preto / Universidade de São Paulo
                0104-1169
                1518-8345
                Jan-Feb 2015
                Jan-Feb 2015
                : 23
                : 1
                : 67-73
                Affiliations
                [1 ]PhD, Adjunct Professor, Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brazil
                [2 ]PhD, Associate Professor, Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brazil
                [3 ]PhD, Adjunct Professor, Universidade Federal do Rio Grande do Norte, Natal, RN, Brazil
                [4 ]MSc, RN, Universidade Federal de Mato Grosso do Sul, Três Lagoas, MS, Brazil
                [5 ]Doctoral student, Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brazil. Physician, Universidade Federal de Mato Grosso do Sul, Campo Grande, MS, Brazil
                Author notes
                Corresponding Author: Olinda Maria Rodrigues de Araujo Universidade Federal de Mato Grosso do Sul. Unidade XII - Curso de Enfermagem Avenida Senador Felinto Müller, s/n. Caixa Postal nº 549 Cidade Universitária CEP: 79070-900, Campo Grande, MS, Brasil E-mail: olinda_araujo@ 123456yahoo.com.br
                Article
                10.1590/0104-1169.3385.2526
                4376033
                25806633
                519e955e-4e25-49c9-9e66-b8656f98d357

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 11 July 2013
                : 24 November 2014
                Page count
                Figures: 6, Tables: 6, Equations: 0, References: 25, Pages: 7
                Categories
                Original Articles

                survivorship (public health),mortality,hemoglobin, sickle,hydroxyurea,nursing

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