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1.
Ann Hematol ; 99(11): 2497-2505, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32869184

RESUMO

To add to the limited existing evidence on clinical outcomes and healthcare use in sickle cell disease (SCD) among beneficiaries of the US Medicaid program, we conducted a cohort study using nationwide Medicaid claims data (2000-2013). Patients were included based on HbSS SCD diagnosis and followed until Medicaid disenrollment, death, bone marrow transplant, or end of data availability to assess vasoocclusive crises (VOC), emergency room (ER) visits, hospitalizations, outpatient visits, and blood transfusions. Annualized event rates (with 95% confidence intervals [CI]) were reported. The impact of VOCs on the risk of mortality was analyzed using a multivariable Cox model with VOC modeled as time-varying and updated annually. In a total of 44,033 SCD patients included with a mean (SD) age of 15.7 (13.6) years, the VOC rate (95% CI) was 3.71 (3.70-3.72) per person-year, with highest rate among patients 19-35 years who had ≥ 5 VOCs at baseline (13.20 [13.15-13.26]). Event rates (95% CI) per person per year for other outcomes were 2.97 (2.97-2.98) ER visits, 2.39 (2.38-2.40) hospitalizations, 5.80 (5.79-5.81) outpatient visits, and 0.91 (0.90-0.91) blood transfusions. A higher VOC burden in the preceding year was associated with an increased risk of mortality, with a hazard ratio (95% CI) of 1.26 (1.14-1.40) for 2-4 VOC vs. < 2 and 1.57 (1.41-1.74) for ≥ 5 VOC vs < 2. In conclusion, we documented a substantial burden of SCD in US Medicaid enrollees, especially during early adulthood and noted that ongoing burden of VOC is associated with mortality in these patients.


Assuntos
Anemia Falciforme/mortalidade , Anemia Falciforme/terapia , Medicaid , Aceitação pelo Paciente de Cuidados de Saúde , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Mortalidade , Fatores de Risco , Estados Unidos/epidemiologia
3.
Soins Pediatr Pueric ; 41(314): 35-41, 2020.
Artigo em Francês | MEDLINE | ID: mdl-32771206

RESUMO

The effect of a standardized musical intervention for adolescents with sickle cell disease was studied. Two groups were evaluated using the visual analog scale of pain and the anxiety-state inventory before and after a standardized musical intervention or breathing intervention. A significant decrease in scores was observed, most notably for the group benefiting from the standardized musical intervention. This intervention could be integrated into the overall management of adolescents with sickle cell disease.


Assuntos
Anemia Falciforme/terapia , Ansiedade/prevenção & controle , Musicoterapia , Manejo da Dor/métodos , Dor/prevenção & controle , Adolescente , Anemia Falciforme/complicações , Anemia Falciforme/psicologia , Ansiedade/etiologia , Humanos , Dor/etiologia , Resultado do Tratamento
4.
PLoS One ; 15(8): e0236998, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32790687

RESUMO

There are over 12,000 people with sickle cell disease (SCD) in the UK, and 4-12% of patients who develop Sickle Cell Nephropathy (SCN) progress to End Stage Renal Disease (ESRD). Renal transplantation offers the best outcomes for these patients with but their access to transplantation is often limited. Regular automated exchange blood transfusions (EBT) reduce the complications of SCD and may improve outcomes. However, concerns over alloimmunisation limit its widespread implementation. In this retrospective multicenter study, data were collected on 34 SCD patients who received a kidney transplant across 6 London Hospitals between 1997 and 2017. 20/34 patients were on an EBT program, pre or post renal transplantation. Overall patient and graft survival were inferior to contemporaneous UK data in the ESRD population as a whole, a finding which is well-recognised. However, patient survival (CI 95%, p = 0.0032), graft survival and graft function were superior at all time-points in those who received EBT versus those who did not. 4/20 patients (20%) on EBT developed de novo donor specific antibodies (DSAs). 3/14 patients (21%) not on EBT developed de novo DSAs. The incidence of rejection in those on EBT was 5/18 (28%), as compared with 7/13 (54%) not on EBT. In conclusion, our data, while limited by an inevitably small sample size and differences in the date of transplantation, do suggest that long-term automated EBT post renal transplant is effective and safe, with improvement in graft and patient outcomes and no increase in antibody formation or graft rejection.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/cirurgia , Transfusão Total , Falência Renal Crônica/etiologia , Falência Renal Crônica/cirurgia , Transplante de Rim , Adulto , Anemia Falciforme/terapia , Terapia Combinada , Feminino , Sobrevivência de Enxerto , Humanos , Estimativa de Kaplan-Meier , Falência Renal Crônica/terapia , Londres , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
5.
Ann Hematol ; 99(11): 2483-2495, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32852615

RESUMO

Sickle cell disease (SCD), a genetic disorder affecting up to 100,000 patients in the USA, impacts multiple organ systems. The emergency department (ED) is frequently utilized by patients with SCD who have severe pain from vaso-occlusive crises. The goal of this systematic literature review is to identify predictors for ED use among patients with SCD in the USA, as high ED reliance is not ideal because of the potential for discontinuity of care as well as higher costs. PubMed and Embase were searched for articles containing the keywords "sickle cell disease" AND ("emergency" OR "acute care") AND ("utilization" OR "health care") published between 2000 and 26 September 2019. A total of 26 publications were identified meeting the following inclusion criteria: report of ED or acute care clinic use; report of health care utilization for SCD; and report of ED visits independent of hospital admission, ED revisits, inpatient care visits, and SCD care unit visits. Articles unavailable in English or those focused on populations outside the USA were excluded. Of the 26 articles included, 4 were prospective and the remainder were retrospective. Qualitative analysis of the articles revealed a higher rate of ED utilization among adults than children, patients with public insurance than private insurance, and patients with more comorbidities, complications, or pain. Age and pain levels were both commonly cited as predictors of ED utilization. Additional prospective and interventional studies are needed to further define predictors of ED utilization and to uncover treatments that decrease ED visits.


Assuntos
Anemia Falciforme , Serviços Médicos de Emergência , Serviço Hospitalar de Emergência , Fatores Etários , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Fatores de Risco
6.
Public Health Rep ; 135(4): 442-451, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32639897

RESUMO

OBJECTIVES: Coordinated measurement strategies are needed to inform collaborative approaches to improve access to and quality of care for persons with sickle cell disease (SCD). The objective of our study was to develop a multilevel measurement strategy to assess improvements in access to and quality of care for persons with SCD in 4 US regions. METHODS: From 2014 through 2017, regional grantees in the Sickle Cell Disease Treatment Demonstration Program collected administrative and patient-level electronic health record (EHR) data to assess quality improvement initiatives. Four grantees-covering 29 US states and territories and an SCD population of 56 720-used a collective impact model to organize their work. The grantees collected administrative data from state Medicaid and Medicaid managed care organizations (MCOs) at multiple points during 2014-2017 to assess improvements at the population level, and local patient-level data were abstracted from site-level EHRs at regular intervals to track improvements over time. RESULTS: Administrative data were an important source of understanding population-level improvements but were delayed, whereas patient-level data were more sensitive to small-scale quality improvements. CONCLUSIONS: We established a shared measurement approach in partnership with Medicaid and Medicaid MCO stakeholders that can be leveraged to effectively support quality improvement initiatives for persons with SCD in the United States.


Assuntos
Anemia Falciforme/terapia , Assistência à Saúde/estatística & dados numéricos , Assistência à Saúde/normas , Guias de Prática Clínica como Assunto , Melhoria de Qualidade/estatística & dados numéricos , Melhoria de Qualidade/normas , Humanos , Estados Unidos/epidemiologia
7.
Ann Hematol ; 99(9): 2047-2055, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32691114

RESUMO

Manual erythroexchange (MEEX) was proven to be effective and safe in the management of sickle cell disease (SCD). The goal is to quickly reduce the percentage of hemoglobin S (HbS%). A national survey of the Italian Society for Thalassemia and Hemoglobinopathies (SITE) observed a great variability among MEEX protocols none of which were found to be predictive of the values of HbS% and hemoglobin (Hb) after the exchange. Two equations to estimate the HbS% and Hb values to be obtained after MEEX were developed based on the results of the MEEX procedures in place in the centers participating in the present study. A standard protocol was subsequently defined to evaluate the volumes to exchange to obtain the target values of HbS% and Hb. The protocol was tested in 261 MEEX performed in SCD patients followed in the 5 participating centers that belong to the Italian Hemoglobinopathy Comprehensive Care Network, with the support of the SITE. The results showed a correlation between the estimated and measured values of HbS% and Hb (Rp 0.95 and 0.65 respectively, p < 0.001). A negligible bias was found for the prediction of HbS% and a bias of 1 g/dl for Hb. From consecutive MEEX, a rate of increase of HbS% between two exchanges of around 0.4% per day (p < 0.001) was measured. This protocol was shown to be effective and safe, as all patients reached the target value of HbS%. All the MEEX procedures were carried out with single venous access. No adverse events or reactions such as hypotension or electrolyte imbalance were reported nor were any complaints concerning the procedures received from patients.


Assuntos
Anemia Falciforme/sangue , Anemia Falciforme/terapia , Determinação do Volume Sanguíneo/normas , Volume Sanguíneo/fisiologia , Transfusão de Eritrócitos/normas , Hemoglobina Falciforme/metabolismo , Adulto , Anemia Falciforme/epidemiologia , Determinação do Volume Sanguíneo/métodos , Transfusão de Eritrócitos/métodos , Feminino , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Adulto Jovem
8.
PLoS One ; 15(7): e0235192, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32663844

RESUMO

INTRODUCTION: As marijuana use becomes more common, it is essential clinicians understand the relationship between marijuana use and health behaviors. METHODS: Using a retrospective cohort of adolescents and adults with sickle cell disease (SCD) stratified into a young (<25 years) and older cohort (> = 25 years), we conducted multiple linear regression examining relationship of marijuana use (independent variable) on each dependent variable (SCD self-management score and pain management). RESULTS: Among young cohort, 16.9% used marijuana compared to 21.8% of older cohort. The younger cohort reporting marijuana use had lower mean self-care scores (ß = -2.74;p = 0.009) and were more likely to have admissions to the hospital for pain (ß = 0.87;p = 0.047) compared to non-users. In contrast, the older cohort reporting marijuana use had more days treating pain at home (ß = 0.44;p = 0.035). CONCLUSIONS: Only a minority of patients with SCD reported lifetime marijuana use. Among those reporting marijuana use, there were different associations with self-care and health-related behaviors by age. The older cohort who endorsed marijuana use reported more days of treating pain at home, although this did not translate into increased acute care visits for pain crisis. Among youth, endorsing marijuana use was associated with worse SCD self-care.


Assuntos
Anemia Falciforme/terapia , Comportamentos Relacionados com a Saúde , Uso da Maconha/epidemiologia , Manejo da Dor/estatística & dados numéricos , Autocuidado/estatística & dados numéricos , Adulto , Fatores Etários , Anemia Falciforme/complicações , Feminino , Humanos , Masculino , Uso da Maconha/psicologia , Dor/etiologia , Manejo da Dor/métodos , Manejo da Dor/psicologia , Estudos Retrospectivos , Autocuidado/psicologia , Estados Unidos , Adulto Jovem
10.
PLoS One ; 15(7): e0236360, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32706825

RESUMO

In 2011 Yale New Haven Hospital, in response to high utilization of acute care services and widespread patient and health care personnel dissatisfaction, set out to improve its care of adults living with sickle cell disease. Re-organization components included recruitment of additional personnel; re-locating inpatients to a single nursing unit; reducing the number of involved providers; personalized care plans for pain management; setting limits upon access to parenteral opioids; and an emphasis upon clinic visits focused upon home management of pain as well as specialty and primary care. Outcomes included dramatic reductions in inpatient days (79%), emergency department visits (63%), and hospitalizations (53%); an increase in outpatient visits (31%); and a decrease in costs (49%). Providers and nurses viewed the re-organization and outcomes positively. Most patients reported improvements in pain control and life style; many patients thought the re-organization process was unfair. Their primary complaint was a lack of shared decision-making. We attribute the contrast in these perspectives to the inherent difficulties of managing recurrent acute and chronic pain with opioids, especially within the context of the imbalance in wellness, power, and privilege between persons living with sickle cell disease, predominantly persons of color and poor socio-economic status, and health care organizations and their personnel.


Assuntos
Anemia Falciforme/terapia , Hospitais Universitários , Atenção Primária à Saúde/organização & administração , Adulto , Assistência Ambulatorial/estatística & dados numéricos , Analgésicos Opioides/uso terapêutico , Custos e Análise de Custo/estatística & dados numéricos , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Pacientes Internados/estatística & dados numéricos , Masculino , Enfermeiras e Enfermeiros/estatística & dados numéricos , Manejo da Dor/estatística & dados numéricos , Avaliação de Resultados da Assistência ao Paciente , Médicos/estatística & dados numéricos , Fatores Socioeconômicos
18.
Ann Hematol ; 99(6): 1217-1223, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32367178

RESUMO

While fluid replacement therapy is a primary treatment modality used in vaso-occlusive crises for sickle cell disease, data is limited on its safety, efficacy, and variability. We performed a retrospective analysis on 157 unique patient encounters from 49 sickle cell patients hospitalized with a vaso-occlusive episode at our institution from 2013 to 2017. The median length of hospital stay was 4 days (IQR 2-7). The mean total amount of intravenous fluid administered during the hospitalization was 7.4 L (Std 9.6). The mean total amount of fluid intake including intravenous fluids, blood transfusions, and oral fluids was 14.2 L (Std 18.2). Multivariate analyses revealed significant associations between the development of any adverse event (including a new oxygen requirement, acute chest syndrome, aspiration event, other hospital-acquired infection, acute kidney injury, and intensive care unit transfer) and the following variables: intravenous fluid administered in the first 24 h (p = 0.001, OR 1.899, 95% CI 1.319-2.733), total amount of intravenous fluid administered (p = 0.005, OR 1.081, 95% CI 1.023-1.141), and total amount of fluid intake including oral fluids, blood transfusions, and intravenous fluids (p = 0.009, OR 1.046, 95% CI 1.011-1.081). Other factors found to be significantly associated with any adverse event were dialysis dependence prior to admission (p < 0.001, OR 12.984, 95% CI 3.660-46.056) and admission to an inpatient service versus an emergency room or observation unit (p = 0.008, OR 3.201, 95% CI 1.346-7.612). While fluid administration may theoretically slow the sickling process, this data suggests that fluid administration during a vaso-occlusive episode, and especially total volume given in the first 24 h, may also lead to adverse events.


Assuntos
Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Hidratação/tendências , Manejo da Dor/tendências , Dor/epidemiologia , Administração Intravenosa , Adulto , Anemia Falciforme/diagnóstico , Feminino , Hidratação/métodos , Hospitalização/tendências , Humanos , Masculino , Dor/diagnóstico , Manejo da Dor/métodos , Estudos Retrospectivos , Resultado do Tratamento
20.
Lancet Haematol ; 7(6): e469-e478, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32470438

RESUMO

BACKGROUND: Transfusion-dependent haemoglobinopathies require lifelong iron chelation therapy with one of the three iron chelators (deferiprone, deferasirox, or deferoxamine). Deferasirox and deferiprone are the only two oral chelators used in adult patients with transfusion-dependent haemoglobinopathies. To our knowledge, there are no randomised clinical trials comparing deferiprone, a less expensive iron chelator, with deferasirox in paediatric patients. We aimed to show the non-inferiority of deferiprone versus deferasirox. METHODS: DEEP-2 was a phase 3, multicentre, randomised trial in paediatric patients (aged 1 month to 18 years) with transfusion-dependent haemoglobinopathies. The study was done in 21 research hospitals and universities in Italy, Egypt, Greece, Albania, Cyprus, Tunisia, and the UK. Participants were receiving at least 150 mL/kg per year of red blood cells for the past 2 years at the time of enrolment, and were receiving deferoxamine (<100 mg/kg per day) or deferasirox (<40 mg/kg per day; deferasirox is not registered for use in children aged <2 years so only deferoxamine was being used in these patients). Any previous chelation treatment was permitted with a 7-day washout period. Patients were randomly assigned 1:1 to receive orally administered daily deferiprone (75-100 mg/kg per day) or daily deferasirox (20-40 mg/kg per day) administered as dispersible tablets, both with dose adjustment for 12 months, stratified by age (<10 years and ≥10 years) and balanced by country. The primary efficacy endpoint was based on predefined success criteria for changes in serum ferritin concentration (all patients) and cardiac MRI T2-star (T2*; patients aged >10 years) to show non-inferiority of deferiprone versus deferasirox in the per-protocol population, defined as all randomly assigned patients who received the study drugs and had available data for both variables at baseline and after 1 year of treatment, without major protocol violations. Non-inferiority was based on the two-sided 95% CI of the difference in the proportion of patients with treatment success between the two groups and was shown if the lower limit of the two-sided 95% CI was greater than -12·5%. Safety was assessed in all patients who received at least one dose of study drug. This study is registered with EudraCT, 2012-000353-31, and ClinicalTrials.gov, NCT01825512. FINDINGS: 435 patients were enrolled between March 17, 2014, and June 16, 2016, 393 of whom were randomly assigned to a treatment group (194 to the deferiprone group; 199 to the deferasirox group). 352 (90%) of 390 patients had ß-thalassaemia major, 27 (7%) had sickle cell disease, five (1%) had thalassodrepanocytosis, and six (2%) had other haemoglobinopathies. Median follow-up was 379 days (IQR 294-392) for deferiprone and 381 days (350-392) for deferasirox. Non-inferiority of deferiprone versus deferasirox was established (treatment success in 69 [55·2%] of 125 patients assigned deferiprone with primary composite efficacy endpoint data available at baseline and 1 year vs 80 [54·8%] of 146 assigned deferasirox, difference 0·4%; 95% CI -11·9 to 12·6). No significant difference between the groups was shown in the occurrence of serious and drug-related adverse events. Three (2%) cases of reversible agranulocytosis occurred in the 193 patients in the safety analysis in the deferiprone group and two (1%) cases of reversible renal and urinary disorders (one case of each) occurred in the 197 patients in the deferasirox group. Compliance was similar between treatment groups: 183 (95%) of 193 patients in the deferiprone group versus 192 (97%) of 197 patients in the deferisirox group. INTERPRETATION: In paediatric patients with transfusion-dependent haemoglobinopathies, deferiprone was effective and safe in inducing control of iron overload during 12 months of treatment. Considering the need for availability of more chelation treatments in paediatric populations, deferiprone offers a valuable treatment option for this age group. FUNDING: EU Seventh Framework Programme.


Assuntos
Deferasirox/uso terapêutico , Deferiprona/uso terapêutico , Transfusão de Eritrócitos/métodos , Hemoglobinopatias/tratamento farmacológico , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/tratamento farmacológico , Administração Oral , Adolescente , Agranulocitose/induzido quimicamente , Agranulocitose/epidemiologia , Albânia/epidemiologia , Anemia Falciforme/terapia , Técnicas de Imagem Cardíaca/métodos , Criança , Pré-Escolar , Chipre/epidemiologia , Deferasirox/administração & dosagem , Deferasirox/economia , Deferiprona/administração & dosagem , Deferiprona/economia , Egito/epidemiologia , Transfusão de Eritrócitos/estatística & dados numéricos , Feminino , Ferritinas/sangue , Ferritinas/efeitos dos fármacos , Grécia/epidemiologia , Hemoglobinopatias/terapia , Humanos , Lactente , Quelantes de Ferro/administração & dosagem , Quelantes de Ferro/economia , Sobrecarga de Ferro/sangue , Itália/epidemiologia , Imagem por Ressonância Magnética , Masculino , Cooperação do Paciente , Resultado do Tratamento , Tunísia/epidemiologia , Reino Unido/epidemiologia , Doenças Urológicas/induzido quimicamente , Doenças Urológicas/epidemiologia , Talassemia beta/terapia
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