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1.
Clin Appl Thromb Hemost ; 26: 1076029620955240, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32873056

RESUMO

The management of sickle cell disease (SCD) and its complications in the COVID-19 era is very challenging. The recurrent sickling process in SCD causes tissue hypoxemia and micro-infarcts, resulting in end organ damage. Since the outbreak of SARS-CoV-2 pandemic, little data has been published about SCD concerning clinical presentation with COVID-19 and management. Hydroxyurea has been the cornerstone of management in children and adults with SCD, with evidence of its effect on controlling end organ damage. There are several anti-sickling drugs that have been approved recently that might have an additive value toward the management of SCD and its complications. The role of simple and exchange transfusions is well established and should always be considered in the management of various complications. The value of convalescent plasma has been demonstrated in small case series, but large randomized controlled studies are still awaited. Immunomodulatory agents may play a role in reducing the damaging effects of cytokines storm that contributes to the morbidity and mortality in advanced cases. Prophylactic anticoagulation should be considered in every management protocol because SCD and COVID-19 are thrombogenic conditions. Management proposals of different presentations of patients with SCD and COVID-19 are outlined.


Assuntos
Anemia Falciforme/tratamento farmacológico , Infecções por Coronavirus/epidemiologia , Hidroxiureia/administração & dosagem , Controle de Infecções/métodos , Pandemias/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Síndrome Respiratória Aguda Grave/epidemiologia , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Infecções por Coronavirus/prevenção & controle , Gerenciamento Clínico , Feminino , Seguimentos , Humanos , Masculino , Pandemias/prevenção & controle , Pneumonia Viral/prevenção & controle , Medição de Risco , Síndrome Respiratória Aguda Grave/diagnóstico , Síndrome Respiratória Aguda Grave/terapia , Resultado do Tratamento
2.
Pan Afr Med J ; 36: 81, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32774640

RESUMO

Sickle cell disease is a major concern of public health significance in Africa. Nearly 2/3rd of the global burden of sickle cell disease (SCD) is found to be in sub-Saharan Africa. There is increased mortality risk in sickle cell disease patients in Africa due to associated complications such as acute chest syndrome, asthma, pulmonary emboli and sepsis. Sickle cell disease management is the major contributor of financial burden on the government. Moreover, there is a shortage of medical specialists in Africa. COVID-19 pandemic has further led to devastating impact on economy and health globally. The chances of SCD patient contracting COVID-19 infections are higher as these patients are immunocompromised and may be at a higher risk of mortality. Practicing preventive measures including isolation and social distancing by these patients will prevent mortality rates as well as economic burden on government in the present unprecedented COVID-19 pandemic.


Assuntos
Anemia Falciforme/epidemiologia , Infecções por Coronavirus/epidemiologia , Efeitos Psicossociais da Doença , Pneumonia Viral/epidemiologia , África ao Sul do Saara/epidemiologia , Anemia Falciforme/complicações , Anemia Falciforme/mortalidade , Infecções por Coronavirus/economia , Infecções por Coronavirus/prevenção & controle , Humanos , Hospedeiro Imunocomprometido , Pandemias/economia , Pandemias/prevenção & controle , Pneumonia Viral/economia , Pneumonia Viral/prevenção & controle , Saúde Pública , Isolamento Social
3.
Niger J Clin Pract ; 23(8): 1079-1086, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32788485

RESUMO

Background: Poor growth and nutritional status are common features of sickle cell anemia (SCA) in children. The rising trend of obesity in children in developing countries has been reported despite a huge burden of undernutrition in these settings. In SCA, overweight/obesity is being increasingly reported. Aims: To evaluate the nutritional status and its determinants in children with SCA and to compare the same with hemoglobin AA (HbAA) controls of similar age, gender, and socioeconomic status. Methods: The study was a cross-sectional analytical study involving 175 subjects and controls aged 1-18 years who met the inclusion criteria. Weight and height were measured and body mass index (BMI) was calculated. Z scores were computed for the anthropometric measurements using the World Health Organization (WHO) standard reference. Hemoglobin concentration was determined using HemoCue Hb201+ Analyzer. Results: Subjects had significantly lower Z- scores for weight, height, and BMI compared with controls. Stunting, wasting, and overweight/obesity were observed in 10.9%, 24.6%, and 5.1% of subjects compared with 2.3%, 5.7%, and 9.7% respectively in controls. Wasting, stunting and overweight/obesity in SCA were significantly associated with age while overweight/obesity was significantly associated with upper social class (P = 0.001). Conclusions: Poor growth and nutritional status are still prevalent while overweight and obesity are emerging comorbidities among children with SCA in our environment. Regular nutritional assessment of children with SCA should be encouraged while those at risk of under/over-nutrition should receive adequate nutritional rehabilitation to prevent possible complications.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/epidemiologia , Desnutrição/epidemiologia , Estado Nutricional , Obesidade Pediátrica/epidemiologia , Adolescente , Índice de Massa Corporal , Peso Corporal , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos Transversais , Feminino , Transtornos do Crescimento/complicações , Humanos , Lactente , Masculino , Nigéria/epidemiologia , Avaliação Nutricional , Sobrepeso/complicações , Sobrepeso/epidemiologia , Obesidade Pediátrica/complicações , Prevalência , Classe Social , Fatores Socioeconômicos
4.
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.
Niger Postgrad Med J ; 27(3): 190-195, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32687118

RESUMO

Background: Haemoglobin (Hb) disorders are among the most common blood genetic disorders worldwide, and they constitute an important cause of morbidity and mortality, especially in Nigeria. Despite the clinical significance of early diagnosis, newborn screening for these conditions is not routinely done in Nigeria. Objective: This study was undertaken to document the pattern of Hb phenotypes of newborn babies at the National Hospital Abuja and highlight the relevance of neonatal screening for early diagnosis of abnormal Hb phenotypes in Nigeria. Subjects and Methods: A prospective study of eligible newborn babies delivered in the hospital at the study site was undertaken following parental informed consent. Venous blood was collected from the babies into an ethylenediaminetetraacetic acid sample bottles. The samples were analysed using high-performance liquid chromatography (HPLC) techniques, and the Hb phenotypes obtained were documented. Data were analysed using the Statistical Package for Social Sciences (SPSS) version 20 (IBM-SPSS, Armonk, NY, USA). Results: Three hundred and eleven newborns (male = 173, female = 138) aged 0-28 days were recruited. Two hundred and thirty-six (75.9%) babies had Hb AA (FA) phenotype, 63 (20.3%) Hb AS (FAS), 6 (1.9%) Hb SS (FS), 4 (1.3%) Hb AC (FAC) and 2 (0.6%) had abnormal HbA variants. The overall prevalence of abnormal Hb phenotype was 24.1%. The results showed a significant association of sex (P = 0.003) and ethnicity (P = 0.047) with Hb phenotype. Conclusion: There is a wide spectrum of abnormal Hb phenotypes in Nigeria, and these phenotypes can easily be detected at birth using HPLC. We, therefore, recommend routine neonatal screening for sickle cell disease by HPLC in Nigeria.


Assuntos
Anemia Falciforme/sangue , Cromatografia Líquida de Alta Pressão/métodos , Hemoglobinas Anormais/análise , Hemoglobinas/análise , Recém-Nascido/sangue , Traço Falciforme/sangue , Adolescente , Adulto , Grupo com Ancestrais do Continente Africano , Anemia Falciforme/epidemiologia , Anemia Falciforme/genética , Criança , Pré-Escolar , Hemoglobina Falciforme , Hemoglobinas/classificação , Hemoglobinas Anormais/genética , Humanos , Lactente , Nigéria/epidemiologia , Fenótipo , Prevalência , Estudos Prospectivos , Adulto Jovem
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.
Emerg Infect Dis ; 26(10): 2473-2476, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32639228

RESUMO

Sickle cell disease (SCD) disproportionately affects Black or African American persons in the United States and can cause multisystem organ damage and reduced lifespan. Among 178 persons with SCD in the United States who were reported to an SCD-coronavirus disease case registry, 122 (69%) were hospitalized and 13 (7%) died.


Assuntos
Anemia Falciforme/epidemiologia , Infecções por Coronavirus/epidemiologia , Hospitalização/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Adolescente , Adulto , Idoso , Infecções Assintomáticas/epidemiologia , Betacoronavirus , Criança , Pré-Escolar , Comorbidade , Infecções por Coronavirus/mortalidade , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Pandemias , Gravidade do Paciente , Pneumonia Viral/mortalidade , Sistema de Registros , Estados Unidos/epidemiologia , Adulto Jovem
11.
Ann Hematol ; 99(6): 1225-1230, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32363415

RESUMO

Sickle cell anemia (SCA) is the most severe form of sickle cell disease caused by homozygosity of the ßS-gene (S/S or ßSßS) and has worldwide distribution. Six polymorphic sites in the ß-globin gene cluster were analyzed from a sample of 56 chromosomes of patients with SCA from the state of Maranhão, northeastern Brazil. PCR-RFLP showed that the CAR haplotype was predominant with a frequency of 64.28%, followed by the BEN haplotype (28.57%). Atypical haplotypes were identified at a frequency of 7.15%. Genotypes CAR/CAR, BEN/BEN, and CAR/BEN were present in 46.43%, 10.71%, and 35.71% of patients, respectively. ß-Globin haplotype determination is important not only for the monitoring and prognosis of patients with SCA, but it also serves to inform anthropological studies that contribute to elucidating any peculiarities associated with African influences that contributed to the ethnological, economic, cultural, and social formation of Brazil. The high frequency of the CAR/CAR and CAR/BEN haplotypes in this study, which are associated with low levels of fetal hemoglobin, may ultimately reflect a severe clinical course and poor prognosis in patients with SCA in Maranhão.


Assuntos
Anemia Falciforme/epidemiologia , Anemia Falciforme/genética , Haplótipos/genética , Adolescente , Adulto , Anemia Falciforme/diagnóstico , Brasil/epidemiologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Adulto Jovem
12.
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
13.
Ann Hematol ; 99(7): 1453-1463, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32447424

RESUMO

Fetal hemoglobin (HbF) ameliorates clinical severity of sickle cell anemia (SCA). The major loci regulating HbF levels are HBB cluster, BCL11A, and HMIP-2 (HBS1L-MYB). However, the impact of noncoding single-nucleotide polymorphisms (SNPs) in these loci on clinical outcomes and their functional role on regulating HbF levels should be better elucidated. Therefore, we performed comprehensive association analyses of 14 noncoding SNPs in five loci with HbF levels and with clinical outcomes in a cohort of 250 children with SCA from Southeastern Brazil, and further performed functional annotation of these SNPs. We found SNPs independently associated with HbF levels: rs4671393 in BCL11A (ß-coefficient = 0.28), rs9399137 in HMIP-2A (ß-coefficient = 0.16), and rs4895441 in HMIP-2B (ß-coefficient = 0.15). Patients carrying minor (HbF-boosting) alleles for rs1427407, rs93979137, rs4895441, rs9402686, and rs9494145 showed reduced count of reticulocytes (p < 0.01), while those carrying the T allele of rs9494145 showed lower white blood cell count (p = 0.002). Carriers of the minor allele for rs9402686 showed higher peripheral saturation of oxygen (p = 0.002). Patients carrying minor alleles in BCL11A showed lower risk of transfusion incidence rate ratio (IRR ≥ 1.3; p < 0.0001). This effect was independent of HbF effect (p = 0.005). Carriers of minor alleles for rs9399137 and rs9402686 showed lower risk of acute chest syndrome (IRR > 1.3; p ≤ 0.01). Carriers of the reference allele for rs4671393 showed lower risk of infections (IRR = 1.16; p = 0.01). In conclusion, patients carrying HbF-boosting alleles of BCL11A and HMIP-2 were associated with milder clinical phenotypes. Higher HbF concentration may underlie this effect.


Assuntos
Anemia Falciforme/diagnóstico , Anemia Falciforme/genética , Hemoglobina Fetal/metabolismo , Proteínas de Ligação ao GTP/genética , Genes myb , Polimorfismo de Nucleotídeo Único , Proteínas Repressoras/genética , Alelos , Anemia Falciforme/sangue , Anemia Falciforme/epidemiologia , Brasil/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Hemoglobina Fetal/genética , Predisposição Genética para Doença , Estudo de Associação Genômica Ampla , Humanos , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos
14.
Ann Hematol ; 99(7): 1465-1474, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32451712

RESUMO

Sickle cell disease (SCD) describes a set of chronic inherited anemias characterized by hemolysis, episodes of vaso-occlusion, and high infectious risk, with high morbidity and mortality. Newborn screening (NBS) for SCD allows family health education and early start of infectious prophylaxis. In the Community of Madrid, a pilot universal NBS study found that the SCA birth prevalence was 1/5851 in newborns, higher than expected, confirming the need to include early detection in the NBS program. The aim of the present prospective single-center study is to analyze the results of newborn SCD screening in Madrid in terms of epidemiological data and its inclusion in a comprehensive care program during the last 15 years, between 1st of May 2003 and 1st of May 2018. During the study period, 1,048,222 dried bloodspots were analyzed. One hundred ninety-seven patients were diagnosed with possible SCD (HPLC phenotype of FS, FSA, FSC, FSE, FSDPunjab, FSOArab), with 187 patients finally confirmed (birth prevalence 1/5552 newborns, 0.18 per 1000 live births), and 1 out of 213 infants carried Hb S. All of them were seen by a specialist clinician; median age at the first visit consultation was 35 days and median age at the beginning of penicillin treatment was 66 days. The Madrid SCD NBS program achieved high rates of sensitivity and specificity and good quality of care assistance. Establishing a good relationship with the family, a strong education program, and a multidisciplinary team that includes social workers and a psychologist are needed to ensure the success of early intervention.


Assuntos
Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Triagem Neonatal , Europa (Continente)/epidemiologia , Feminino , História do Século XXI , Humanos , Recém-Nascido , Masculino , Triagem Neonatal/história , Triagem Neonatal/tendências , Prevalência , Estudos Prospectivos , Espanha/epidemiologia
15.
Ann Hematol ; 99(9): 2057-2064, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32458066

RESUMO

Sleep disturbance is common among children with sickle cell disease (SCD) and is related to neurocognitive difficulties. However, research on sleep disturbances and related variables among adults with SCD is extremely limited. The present study examined the relationship between sleep, executive functioning, and emotional functioning among 62 adults (29 females; M age = 32 years, SD = 7.79) with SCD preparing to undergo a stem cell transplant. Participants were administered a neurocognitive evaluation that included objective and subjective measures of executive functioning, and they completed PROMIS self-report measures of anxiety, depression, and pain intensity. Results showed that about 17% of participants endorsed clinically significant sleep disruptions, while 16.1% and 8% endorsed clinically significant symptoms of anxiety and depression, respectively. Sleep disturbance in these adults was not significantly correlated with objective or subjective measures of executive functioning. Moreover, anxiety, but not depression, was a significant mediator between self-reported sleep difficulties and both objective and subjective measures of executive functioning while controlling for pain intensity. Future research on sleep interventions will be essential for ameliorating the effects of sleep disturbance on executive functioning and anxiety among adults with SCD.


Assuntos
Anemia Falciforme/psicologia , Emoções/fisiologia , Função Executiva/fisiologia , Transtornos do Sono-Vigília/psicologia , Adulto , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Ansiedade/diagnóstico , Ansiedade/epidemiologia , Ansiedade/psicologia , Estudos Transversais , Depressão/diagnóstico , Depressão/epidemiologia , Depressão/psicologia , Feminino , Transplante de Células-Tronco Hematopoéticas/psicologia , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos do Sono-Vigília/diagnóstico , Transtornos do Sono-Vigília/epidemiologia , Adulto Jovem
16.
Ann Hematol ; 99(9): 2073-2079, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32377816

RESUMO

Vasculopathy is a hallmark of sickle cell disease ultimately resulting in chronic end organ damage. Leg ulcer is one of its sequelae, occurring in ~ 5-10% of adult sickle cell patients. The majority of leg ulcer publications to date have emanated from single center cohort studies. As such, there are limited studies on the geographic distribution of leg ulcers and associated risk factors worldwide. The Consortium for the Advancement of Sickle Cell Research (CASiRe) was formed to improve the understanding of the different phenotypes of sickle cell disease patients living in different geographic locations around the world (USA, UK, Italy, Ghana). This cross-sectional cohort sub-study of 659 sickle cell patients aimed to determine the geographic distribution and risk factors associated with leg ulcers. The prevalence of leg ulcers was 10.3% and was associated with older age, SS genotype, male gender, and Ghanaian origin. In fact, the highest prevalence (18.6%) was observed in Ghana. Albuminuria, proteinuria, increased markers of hemolysis (lower hemoglobin, higher total bilirubin), lower oxygen saturation, and lower body mass index were also associated with leg ulceration. Overall, our study identified a predominance of leg ulcers within male hemoglobin SS patients living in sub-Saharan Africa with renal dysfunction and increased hemolysis.


Assuntos
Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Internacionalidade , Úlcera da Perna/diagnóstico , Úlcera da Perna/epidemiologia , Adolescente , Adulto , Idoso , Anemia Falciforme/sangue , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Gana/epidemiologia , Humanos , Lactente , Itália/epidemiologia , Úlcera da Perna/sangue , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Reino Unido/epidemiologia , Estados Unidos/epidemiologia , Adulto Jovem
19.
Einstein (Sao Paulo) ; 18: eAO5070, 2020.
Artigo em Inglês, Português | MEDLINE | ID: mdl-32321079

RESUMO

OBJECTIVE: To evaluate epidemiological aspects of priapism in patients with sickle cell disease, and these aspects impact on adult sexual function. METHODS: This was a cross-sectional study including individuals with sickle cell disease who were evaluated at a reference center for sickle cell. Participants completed a structured questionnaire about their sociodemographic characteristics and priapism events. Sexual function was assessed using validated two instruments, the Erection Hardness Score and one about the sex life satisfaction. RESULTS: Sixty-four individuals with median aged of 12 (7 to 28) years were interviewed. The prevalence of priapism was 35.9% (23/64). The earliest priapism episode occurred at 2 years of age and the latest at 42 years. The statistical projection was that 71.1% of individuals of the study would have at least one episode of priapism throughout life. Patients with episodes of priapism (10/23) had significantly worse erectile function Erection Hardness Score of 2 [1-3]; p=0.01 and were less satisfied with sexual life 3 [3-5]; p=0.02. CONCLUSION: Priapism is usually present in childhood, and severe episodes are associated with cavernous damage, impairment in the quality of the erection, and lower sexual satisfaction.


Assuntos
Anemia Falciforme/epidemiologia , Anemia Falciforme/fisiopatologia , Disfunção Erétil/epidemiologia , Disfunção Erétil/fisiopatologia , Priapismo/epidemiologia , Priapismo/fisiopatologia , Adolescente , Adulto , Fatores Etários , Brasil/epidemiologia , Criança , Estudos Transversais , Intervalo Livre de Doença , Humanos , Masculino , Ereção Peniana/fisiologia , Prevalência , Priapismo/etiologia , Qualidade de Vida , Estudos Retrospectivos , Estatísticas não Paramétricas , Inquéritos e Questionários , Adulto Jovem
20.
South Med J ; 113(4): 150-155, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32239226

RESUMO

OBJECTIVES: Despite studies demonstrating the negative impact of food insecurity on health in children, limited research has been done to assess the prevalence and sequelae of food insecurity in sickle cell disease (SCD). We tested the hypothesis that food insecurity is common in children with SCD and is associated with increased SCD morbidity. METHODS: Between May and November 2017, we conducted a single-center cross-sectional study using the previously validated, self-administered, US 18-item household food security survey module and the 9-item youth (12-17 years old) food security survey module during regular outpatient clinic visits. We also included the incidence of vaso-occlusive pain or acute chest syndrome requiring hospitalizations in the year before the questionnaire. RESULTS: A total of 75 caregivers and 24 children completed the surveys. The median age of the children was 10.4 years (interquartile range 5.5-15.3), 46.7% were boys. The rate of household food insecurity was 21.3% (16 of 75). Among the 24 children who completed the youth survey, 45.8% were classified as food insecure. Discordance occurred between caregivers' and children's assessment of food insecurity. A total of 81.8% (9 of 11) children reported being food insecure, whereas their caregivers reported to be food secure. The incidence for pain and acute chest syndrome in the year pre-enrollment was not different between food-secure and food-insecure children (59.3 and 43.8/100 patient-years, P = 0.54; 8.5 and 12.5/100 patient-years, P = 0.49, respectively). CONCLUSIONS: In a tertiary care medical center in Tennessee, one in five households with children with SCD were assessed as food insecure, with a substantial discordance between caregiver and child assessment of food insecurity.


Assuntos
Anemia Falciforme/dietoterapia , Crianças com Deficiência/estatística & dados numéricos , Características da Família , Abastecimento de Alimentos/normas , Adolescente , Anemia Falciforme/epidemiologia , Criança , Estudos Transversais , Feminino , Abastecimento de Alimentos/estatística & dados numéricos , Humanos , Masculino , Pacientes Ambulatoriais/estatística & dados numéricos , Tennessee/epidemiologia
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