Sickle cell disease: looking back but towards the future

Cançado, Rodolfo Delfini

doi:10.5581/1516-8484.20120041

Cited by 14 publications

(12 citation statements)

References 16 publications

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“…Sickle cell disease (SCD) is a multisystem disease associated with episodes of acute illness and progressive organ damage, and it represents a major public health problem because of its associated morbidity and mortality [ 1 ]. The prevalence of sickle hemoglobin (Hb S) in Basra is 6.48%, with a gene frequency of 0.0324% [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq

Salman¹,

Hassan

2015

Anemia

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Objectives. Despite improvements in the management of sickle cell disease (SCD), many patients still experience disease-related complications requiring hospitalizations. The objectives of this study were to identify causes of hospitalization among these patients and factors associated with the length of hospital stay (LOS) and readmission. Methods. Data from 160 patients (<14 years old) with SCD who were admitted to the Basra Maternity and Children's Hospital from the first of January 2012 through July 2012 were analyzed. Results. The main causes of hospitalization were acute painful crises (73.84%), infections (9.28%), acute chest syndrome (8.02%), and acute splenic sequestration crisis (6.32%). The mean LOS was 4.34 ± 2.85 days. The LOS for patients on hydroxyurea (3.41 ± 2.64 days) was shorter than that for patients who were not (4.59 ± 2.86 days), P < 0.05. The readmission rate (23.1%) was significantly higher among patients with frequent hospitalizations in the previous year (OR 9.352, 95% CI 2.011–43.49), asthma symptoms (OR 4.225, 95% CI 1.125–15.862), and opioid use (OR 6.588, 95% CI 1.104–30.336). Patients on hydroxyurea were less likely to be readmitted (OR 0.082, 95% CI 0.10–0.663). Conclusions. There is a relatively high readmission rate among patients with SCD in Basra. The use of hydroxyurea significantly decreases the LOS and readmission rate.

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Section: Introductionmentioning

confidence: 99%

Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq

Salman¹,

Hassan

2015

Anemia

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“…(21) The advent of neonatal screening, antibiotic prophylaxis, better vaccines, safer blood transfusion, iron chelation, and hydroxyurea therapy have improved the survival of patients with SCD. (17,(23)(24)(25)(26)(27) The life expectancy of children has increased, and SCD changed from a childhood disease to become a chronic disease. (14,16,24,26) However, for adult patients, mortality remains high.…”

Section: Discussionmentioning

confidence: 99%

“…Up to the fifth year of life, the period of the highest rates of serious complications and death, "prophylactic treatment is basically the essence of therapy". (7,24) However, regular treatment, adherence to treatment, family support, and lifestyle are important for reducing morbidity and mortality. (9) In the state of MS, human and technological resources are concentrated in the municipalities of Campo Grande and Dourados, revealing a low resolution for the treatment of hemoglobinopathies in health microregions.…”

Section: Discussionmentioning

confidence: 99%

Prevalência da doença falciforme em adultos com diagnóstico tardio

et al. 2019

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Resumo Objetivo: Estimar a prevalência da doença falciforme em adultos com diagnóstico tardio, em tratamento nos ambulatórios de hematologia na rede de saúde do Estado do Mato Grosso do Sul de 2013 a 2017; descrever as características sociodemográficas; verificar associações entre os genótipos em relação a idade atual, os genótipos e a idade ao diagnóstico. Métodos: Estudo transversal, retrospectivo, com dados coletados em dois hospitais de ensino. As variáveis investigadas foram: ano do atendimento, genótipo, sexo, data de nascimento, idade ao diagnóstico, naturalidade e procedência. A prevalência foi estimada por ponto (%) e intervalo de confiança de 95%. Resultados: A prevalência foi 3,9%, com 103 adultos com doença falciforme, sendo 60 do sexo feminino e 43 do masculino. Predominou o genótipo HbSS, seguido pelo HbSC. A mediana de idade foi de 35 para os HbSS e 31 para os HbSC. A mediana de idade ao diagnóstico foi cinco anos para os HbSS e 21 para HbSC. Não houve associação entre idade (anos) dos pacientes e genótipo (teste Qui-quadrado p=0,601) e nem entre genótipo e faixa etária (teste Qui-quadrado p= 0,318). Conclusão: O genótipo mais frequente foi o HbSS, seguido pelo HbSC. O diagnóstico dos pacientes com SC foi mais tardio do que naqueles com genótipo SS. As variáveis sociodemográficas e o diagnóstico tardio alertam para a necessidade de fortalecimento de ações na rede de saúde, que interferem sensivelmente na morbimortalidade de adultos com Doença Falciforme.

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“…Sickle cell disease (SCD) is an inherited haemoglobinopathy, which is a severe monogenic condition that affects millions of people worldwide, and characterized by formation of abnormal hemoglobin called Hb S, repeated cycles of sickling and unsickling, multiple episodes of vascular obstruction, and hemolytic anemia (1) . During sickling process, interaction between the rigid sickle cells and the vascular wall leads to, intravascular thrombosis and subsequent end-organ ischemia (1,2) . Stroke caused by vaso-occlusion is a major complication of SCD.…”

Section: Introductionmentioning

confidence: 99%

Silent Brain Changes in Children with Sickle Cell Disease

Hashim¹,

Mokhtar²,

Allam³

2023

The Egyptian Journal of Hospital Medicine

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Background: Cerebrovascular steno-occlusive disease constitutes one of the most dangerous complications of sickle cell disease (SCD). It could result in overt or silent cerebral infarctions (SCI). Objective: The aim of this study was to demonstrate silent parenchymal and vascular brain changes that are incidentally observed in neurologically free SCD children using screening MRI and MRA. Patients and methods: This prospective cross-sectional study was conducted on 78 children diagnosed with SCD who were neurologically free. Magnetic resonance imaging (MRI) and Magnetic Resonance Angiography (MRA) were done to all the participant children. Results: Thirty (38.4%) of our children demonstrated pathologic parenchymal features on their MRI. There were 29 (37.17%) patients had pathologic changes in MRA; 6 of them had isolated vascular abnormality, whereas the remaining patients had concurrent parenchymal abnormalities. There was significant high positive correlation between low hemoglobin and presence of silent cerebral infarction (SCI). Moya-moya vasculopathy showed significant positive correlation with arterial occlusion and old SCI. HB SS phenotype was significantly correlated with moya-moya vasculopathy, arterial occlusion and old SCI. Conclusions: Silent cerebral and cerebrovascular changes in SCD children are frequent abnormalities requiring utilization of MRI and MRA to find out their exact prevalence and their risk factors.

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Sickle cell disease: looking back but towards the future

Cited by 14 publications

References 16 publications

Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq

Hospitalization Events among Children and Adolescents with Sickle Cell Disease in Basra, Iraq

Prevalência da doença falciforme em adultos com diagnóstico tardio

Silent Brain Changes in Children with Sickle Cell Disease

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