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      Hospitalizations of children with sickle cell disease in the Brazilian Unified Health System in the state of Minas Gerais, Translated title: Internações de crianças com doença falciforme no Sistema Único de Saúde no Estado de Minas Gerais,

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          Abstract

          Abstract Objective: To identify and characterize hospital admissions and readmissions in the Brazilian Unified Public Health System (Sistema Único de Saúde [SUS]) in children with sickle cell disease diagnosed by the Minas Gerais Newborn Screening Program between 1999 and 2012. Methods: Hospital Admission Authorizations with the D57 (International Classification of Diseases-10) code in the fields of primary or secondary diagnosis were retrieved from the SUS Databank (1999-2012). There were 2991 hospitalizations for 969 children. Results: 73.2% of children had hemoglobin SS/Sβ0-thalassemia and 48% were girls. The mean age was 4.3 ± 3.2 years, the mean number of hospitalizations, 3.1 ± 3.3, and the hospital length of stay, 5 ± 3.9 days. Hospital readmissions occurred for 16.7% of children; 10% of admissions were associated with readmission within 30 days after discharge; 33% of readmissions occurred within seven days post-discharge. There were 41 deaths, 95% of which were in-hospital. Secondary diagnoses were not recorded in 96% of admissions, making it impossible to know the reason for admission. In 62% of cases, hospitalizations occurred in the child's county of residence. The total number of hospitalizations of children under 14 with sickle cell disease relative to the total of pediatric hospitalizations increased from 0.12% in 1999 to 0.37% in 2012. Conclusions: A high demand for hospital care in children with sickle cell disease was evident. The number of hospitalizations increased from 1999 to 2012, suggesting that the disease has become more "visible." Knowledge of the characteristics of these admissions can help in the planning of care for these children in the SUS.

          Translated abstract

          Resumo Objetivo: Identificar e caracterizar as internações e reinternações hospitalares pelo Sistema Único de Saúde (SUS) de crianças com doença falciforme, diagnosticadas pelo Programa de Triagem Neonatal de Minas Gerais entre 1999 e 2012. Métodos: Extraíram-se do banco de dados do SUS as Autorizações de Internação Hospitalar com o código D57 (Classificação Internacional de Doenças10) nos campos de diagnóstico primário ou secundário (1999-2012). Identificaram-se 969 crianças, total de 2.991 internações. Resultados: Das crianças, 73,2% tinham hemoglobina SS/Sβ0- talassemia e 48% eram meninas. A média foi de 4,3 ± 3,2 anos, a do número de internações, 3,1 ± 3,3 e a do tempo de permanência, 5 ± 3,9 dias. As readmissões hospitalares ocorreram em 16,7% das crianças; 10% das internações se associaram à readmissão em até 30 dias pós-alta; 33% das readmissões ocorreram em até 7 dias pós-alta. Ocorreram 41 óbitos, 95% em ambiente hospitalar. O diagnóstico secundário não foi registrado em 96% das internações, impossibilitou conhecer o motivo da internação. Em 62% dos casos, as internações ocorreram no município de residência da criança. O total de internações de crianças até 14 anos com doença falciforme em relação ao total das internações pediátricas passou de 0,12% em 1999 para 0,37% em 2012. Conclusões: Constatou-se elevada demanda por cuidados hospitalares, cujo aumento relativo entre 1999 e 2012 sugere incremento da "visibilidade" da doença falciforme. O conhecimento das características dessas internações pode contribuir para o planejamento do cuidado na rede assistencial do SUS.

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          Definitions of the phenotypic manifestations of sickle cell disease.

          Sickle cell disease (SCD) is a pleiotropic genetic disorder of hemoglobin that has profound multiorgan effects. The low prevalence of SCD ( approximately 100,000/US) has limited progress in clinical, basic, and translational research. Lack of a large, readily accessible population for clinical studies has contributed to the absence of standard definitions and diagnostic criteria for the numerous complications of SCD and inadequate understanding of SCD pathophysiology. In 2005, the Comprehensive Sickle Cell Centers initiated a project to establish consensus definitions of the most frequently occurring complications. A group of clinicians and scientists with extensive expertise in research and treatment of SCD gathered to identify and categorize the most common complications. From this group, a formal writing team was formed that further reviewed the literature, sought specialist input, and produced definitions in a standard format. This article provides an overview of the process and describes 12 body system categories and the most prevalent or severe complications within these categories. A detailed Appendix provides standardized definitions for all complications identified within each system. This report proposes use of these definitions for studies of SCD complications, so future studies can be comparably robust and treatment efficacy measured. Use of these definitions will support greater accuracy in genotype-phenotype studies, thereby achieving a better understanding of SCD pathophysiology. This should nevertheless be viewed as a dynamic rather than final document; phenotype descriptions should be reevaluated and revised periodically to provide the most current standard definitions as etiologic factors are better understood, and new diagnostic options are developed. (c) 2009 Wiley-Liss, Inc.
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            Aspectos epidemiológicos e sociais da doença falciforme

            A doença falciforme (DF) é uma doença genética frequente. Predomina entre negros e pardos e, no Brasil, a cada ano, nascem 3.500 crianças com DF e 200.000 com traço falciforme. As hemácias com hemoglobina S assumem, em condições de hipóxia, forma semelhante à foice, podendo levar à oclusão dos capilares, provocando lesões teciduais agudas e crônicas de órgãos, quase sempre acompanhadas de dor. Através de visitas domiciliares objetivou-se ampliar o conhecimento dos aspectos epidemiológicos e de enfrentamento da doença. Foram estudados 47 pacientes falcêmicos da cidade de Uberaba (MG), com idade igual ou superior a 18 anos, sendo 78,7% negros, 17% pardos e 4,3% brancos, preponderando o gênero feminino (59,6%). Quanto ao tipo de hemoglobinopatia, 63,8% eram SS, 17% SC e 10,6% β-talassemia. A maioria (74,5%) residia em bairros periféricos; 42,5% tinham o segundo grau completo; 38,2% eram aposentados ou não tinham profissão definida. A renda per capita de 48,9% era de até um salário mínimo e 19,1% não percebiam qualquer rendimento. Quanto aos sentimentos relacionados à doença, 38,3% referiram aceitação, 21,3% revolta, 17% tristeza; 46,8% relatavam, após o diagnóstico, mudança para pior em relação ao trabalho e 31,9% em relação ao lazer. Verificou-se neste estudo que a DF reflete negativamente nas atividades laborais e na qualidade de vida do paciente. Contudo, as dificuldades no enfrentamento da doença, habitação precária, desemprego e/ou trabalho mal remunerado poderiam ser minimizadas com acompanhamento social e psicoterápico adequados, que deveriam ser disponibilizados a todo doente falciforme.
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              Comparisons of high versus low emergency department utilizers in sickle cell disease.

              Patients with sickle cell disease often receive a substantial amount of their health care in the emergency department (ED) and some come to the ED frequently, seeking treatment for pain. As a result, patients with sickle cell disease are often stigmatized as opioid-seeking ED overutilizers. We describe the proportion of sickle cell disease patients who are high utilizers of the ED and compare them with other sickle cell disease patients on demographics, pain characteristics, health data, psychosocial characteristics, and quality of life. Two hundred thirty-two patients completed baseline data and at least 30 days of daily diary data. Baseline data included demographics, health data, and quality of life (Medical Outcome Study 36 Item Short Form). Daily diary data included ED utilization for sickle cell pain and descriptors of pain and distress. Eighty-two (35.5%) patients were found to be high ED utilizers. Clinically important and statistically significant differences were found between high ED utilizers and all other sickle cell disease patients: lower hematocrit level, more transfusions, more pain days, more pain crises, higher mean pain and distress, and worse quality of life on Medical Outcome Study 36 Item Short Form physical function summary scales. After controlling for severity and frequency of pain, high ED utilizers did not use opioids more frequently than other sickle cell disease patients. A substantial minority of sickle cell disease patients are high ED utilizers. However, high ED utilizers with sickle cell disease are more severely ill as measured by laboratory variables, have more pain, more distress, and have a lower quality of life.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Journal
                jped
                Jornal de Pediatria
                J. Pediatr. (Rio J.)
                Sociedade Brasileira de Pediatria (Porto Alegre, RS, Brazil )
                0021-7557
                1678-4782
                June 2017
                : 93
                : 3
                : 287-293
                Affiliations
                [2] Belo Horizonte MG orgnameUniversidade Federal Minas Gerais orgdiv1Departamento de Pediatria Brazil
                [1] Belo Horizonte MG orgnameUniversidade Federal Minas Gerais orgdiv1Faculdade de Medicina orgdiv2Núcleo de Ações e Pesquisa em Apoio Diagnóstico Brazil
                Article
                S0021-75572017000300287
                10.1016/j.jped.2016.07.005
                27932225
                a1ee45b1-64e6-401f-9f7c-60757ac237c5

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 04 April 2016
                : 27 July 2016
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 23, Pages: 7
                Product

                SciELO Brazil


                Sistema Único de Saúde,Doença falciforme,Serviço Hospitalar de Admissão de Pacientes,Epidemiologia,Triagem neonatal,Brasil,Sickle cell disease,Hospital Admitting Department,Epidemiology,Health system,Newborn screening,Brazil

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