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1.
Pan Afr Med J ; 38: 100, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33889266

RESUMO

Introduction: sickle cell disease (SCD) is a chronic illness. Individuals affected by this disease are at risk for lifelong complications including episodes of acute pain, chronic pain and multi-organ injury that leads to reduced quality of life and a shortened life span. There is a wealth of data on acute care utilization for SCD in the United States. However, data from the Caribbean region is limited. The objective of this study is to explore Emergency Department (ED) utilization for SCD in St. Vincent and the Grenadines by describing: i) the characteristics of SCD related ED encounters; ii) the urgency of these encounters as defined by resource utilization; iii) the disposition for these ED encounters. Methods: the study was a cross-sectional study utilizing data from the ED log books at the Milton Cato Memorial Hospital (MCMH) during non-consecutive time periods between January 1st, 2012 - December 31st, 2016. Results: there were 666 SCD-related ED encounters during the study period. Thirty-four percent of encounters resulted in hospitalization and 66% of encounters met criteria for an urgent visit. The most commonly reported diagnosis was vaso-occlusive crisis and accounted for 84% of all encounters. The most frequently documented age group was the 18-30 age category at 43%. Conclusion: although SCD comprised less than 2% of all ED visits, the majority of these visits could be classified as urgent visits based on resource utilization. This study adds to the emerging data on the burden of this disease in this St. Vincent and the Grenadines.


Assuntos
Anemia Falciforme/terapia , Serviço Hospitalar de Emergência/estatística & dados numéricos , Hospitalização/estatística & dados numéricos , Adolescente , Adulto , Idoso , Anemia Falciforme/complicações , Anemia Falciforme/fisiopatologia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Qualidade de Vida , São Vicente e Granadinas , Adulto Jovem
2.
Br J Haematol ; 193(1): 43-51, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33538335
5.
Am J Hematol ; 96(5): 538-544, 2021 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-33534136

RESUMO

Neurologic complications are common in patients with sickle cell anemia (SCA), but conventional tools such as MRI and transcranial Doppler ultrasonography (TCD) do not fully assess cerebrovascular pathology. Cerebral tissue oximetry measures mixed oxygen saturation in the frontal lobes (SCT O2 ) and provides early prognostic information about tissue at risk of ischemic injury. Untreated patients with SCA have significantly lower SCT O2 than healthy controls that declines with age. Hydroxyurea is effective in preventing many SCA-related complications, but the degree to which it preserves normal neurophysiology is unclear. We analyzed participants enrolled in the Therapeutic Response Evaluation and Adherence Trial (TREAT, NCT02286154), which enrolled participants initiating hydroxyurea using individualized dosing (new cohort) and those previously taking hydroxyurea (old cohort) and was designed to monitor the long-term benefits of hydroxyurea. Cerebral oximetry was performed at baseline and annually. For the new cohort (median starting age = 12 months, n = 55), mean baseline SCT O2 was normal before starting hydroxyurea (mean 65%, 95% CI 58-72%) and significantly increased after 2 years (mean 72%, 95% CI 65-79%, p < .001). The SCT O2 for patients receiving long-term hydroxyurea (median age = 9.6 years) was normal at study entry (mean 66%, 95% CI 58-74%) and remained stable across 2 years. Both cohorts had significantly higher SCT O2 than published data from predominantly untreated SCA patients. Cerebral oximetry is a non-invasive method to assess cerebrovascular pathology that complements conventional imaging. Our results indicate that hydroxyurea suggests protection against neurophysiologic changes seen in untreated SCA.


Assuntos
Anemia Falciforme/tratamento farmacológico , Antidrepanocíticos/uso terapêutico , Circulação Cerebrovascular/efeitos dos fármacos , Hidroxiureia/uso terapêutico , Oximetria/métodos , Adolescente , Anemia Falciforme/sangue , Anemia Falciforme/fisiopatologia , Antidrepanocíticos/administração & dosagem , Antidrepanocíticos/farmacocinética , Antidrepanocíticos/farmacologia , Criança , Pré-Escolar , Relação Dose-Resposta a Droga , Intervenção Médica Precoce , Feminino , Humanos , Hidroxiureia/administração & dosagem , Hidroxiureia/farmacocinética , Hidroxiureia/farmacologia , Lactente , Masculino , Oximetria/instrumentação , Oxigênio/sangue , Oxiemoglobinas/análise , Medicina de Precisão , Estudos Prospectivos , Adulto Jovem
7.
Arch Pediatr ; 28(2): 136-140, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33446428

RESUMO

OBJECTIVES: Besides infectious pneumonia and death risks, the COVID-19 pandemic has prompted negative psychological impacts on communities, especially on people with chronic diseases. We aimed to evaluate COVID-19 and sickle cell disease (SCD)-related experiences, and the clinical course during the outbreak, to measure anxiety levels of adolescent and young adult patients with homozygous SCD, to analyze the correlations between their COVID-19 experiences and anxiety levels and painful episodes. METHODS: In this cross-sectional study, 47 patients aged between 14 and 24 years responded to a descriptive instrument and the State-Trait Anxiety Inventory. Clinical features requiring hospitalization for the same period (between March 10 and May 10) of two sequential years were compared. RESULTS: Sixty-six percent of the patients had at least one negative COVID-19 experience of dizziness, sleep disturbance, tonic immobility, appetite loss or nausea/abdominal distress. The number of negative COVID-19 experiences was correlated with the state anxiety score, the trait anxiety score, and the number of painful episodes (ρ=0.552, P<0.001; ρ=0.529, P<0.001; ρ=0.448, P=0.002, respectively). Both median state anxiety and trait anxiety scores were below the cut-off scores indicating significant clinical symptoms. The number of hospitalizations requiring vaso-occlusive crisis management and blood/exchange transfusion were similar for the same period of two sequential years, 2019 and 2020. CONCLUSION: These descriptive and correlation findings are the first reported on COVID-19-related anxiety in SCD patients. To develop screening and support strategies for mental health needs in pandemic times, further SCD studies should be conducted.


Assuntos
Anemia Falciforme/psicologia , Ansiedade/etiologia , /psicologia , Adolescente , Anemia Falciforme/fisiopatologia , Ansiedade/diagnóstico , Ansiedade/fisiopatologia , Estudos Transversais , Progressão da Doença , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Escalas de Graduação Psiquiátrica , Turquia , Adulto Jovem
8.
BMJ Case Rep ; 14(1)2021 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-33509861

RESUMO

We describe the case of a 21-year-old man with a background of sickle cell disease (SCD) who was on acute presentation in a sickle cell crisis required immediate intensive care admission with red blood cell exchange and ventilatory support. He had right frontal lobe infarcts and extensive bilateral deep white matter lesions most likely secondary to fat embolism. Inpatient investigations demonstrated a patent foramen ovale, explaining the route of spread of the fat embolus. He then had a transcatheter closure of the atrial defect. The patient needed prolonged inpatient rehabilitation. He was discharged from hospital in a wheelchair secondary to severe lower limb neurology and bilateral knee heterotopic ossification. He lives with the possibility of early onset dementia and cognitive decline, requiring constant care. The case highlights the multiple manifestations of SCD and their diverse and debilitating consequences.


Assuntos
Anemia Falciforme/fisiopatologia , Infarto Encefálico/fisiopatologia , Disfunção Cognitiva/fisiopatologia , Embolia Gordurosa/fisiopatologia , Leucoencefalopatias/fisiopatologia , Neuralgia/fisiopatologia , Polineuropatias/fisiopatologia , Quadriplegia/fisiopatologia , Anemia Falciforme/complicações , Anemia Falciforme/terapia , Infarto Encefálico/diagnóstico por imagem , Infarto Encefálico/etiologia , Disfunção Cognitiva/etiologia , Contratura/etiologia , Contratura/fisiopatologia , Ecocardiografia , Embolia Gordurosa/etiologia , Transfusão de Eritrócitos , Forame Oval Patente/complicações , Lobo Frontal/diagnóstico por imagem , Humanos , Unidades de Terapia Intensiva , Articulação do Joelho/diagnóstico por imagem , Leucoencefalopatias/diagnóstico por imagem , Leucoencefalopatias/etiologia , Imagem por Ressonância Magnética , Masculino , Neuralgia/etiologia , Ossificação Heterotópica/diagnóstico por imagem , Ossificação Heterotópica/etiologia , Ossificação Heterotópica/fisiopatologia , Plasma , Transfusão de Plaquetas , Polineuropatias/etiologia , Quadriplegia/etiologia , Adulto Jovem
9.
Adv Rheumatol ; 61: 11, 2021. tab
Artigo em Inglês | LILACS | ID: biblio-1152745

RESUMO

Abstract Background: Sickle cell disease (SCD) is an autosomal recessive genetic disease in which a mutation occurs in the β-globin chain gene, resulting in abnormal hemoglobin levels. In an environment with reduced oxygen concentration, red blood cells change their conformation, resulting in chronic hemolysis and consequent anemia and vaso-occlusive crises with injuries to several organs, with a significant impairment of the osteoarticular system. This study aimed to verify the chronic osteoarticular alterations and their association with clinical and laboratory characteristics of patients with SCD with a more severe phenotype (SS and Sβ0), on a steady-state fasis. Methods: Fifty-five patients were referred to a medical consultation with a specialized assessment of the locomotor system, followed by laboratory tests and radiographic examinations. Results: In total, 74.5% patients had hemoglobinopathy SS; 67.3% were female; and 78.2% were non-whites. The mean patient age was 30.5 years. Most patients (61.8%) reported up to three crises per year, with a predominance of high-intensity pain (65.5%). Radiographic alterations were present in 80% patients. A total of 140 lesions were identified, most which were located in the spine, femur, and shoulders. Most lesions were osteonecrosis and osteoarthritis and were statistically associated with the non-use of hydroxyurea. Conclusions: There was a high prevalence of chronic osteoarticular alterations, which was statistically associated only with the non-regular use of hydroxyurea.(AU)


Assuntos
Humanos , Osteoartrite/etiologia , Osteonecrose/etiologia , Doenças Ósseas Metabólicas/etiologia , Hidroxiureia/administração & dosagem , Anemia Falciforme/fisiopatologia , Prognóstico , Estudos Transversais/instrumentação , Fatores de Risco , Hidroxiureia/efeitos adversos
11.
Rev. Soc. Esp. Dolor ; 27(4): 257-268, jul.-ago. 2020. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-196842

RESUMO

Los pacientes con enfermedad de células falciformes (ECF), también denominada drepanocítica, sufren un dolor intenso que suele comenzar durante la infancia y aumenta su gravedad a lo largo de la vida, lo que lleva a la hospitalización y a una mala calidad de vida a lo largo de los años. Una característica única de la ECF son las crisis vaso-oclusivas (CVO), caracterizadas por episodios recurrentes e impredecibles de dolor agudo. La obstrucción microvascular durante una CVO provoca una disminución del suministro de oxígeno a la periferia y una lesión por isquemia y posterior reperfusión, inflamación, estrés oxidativo y disfunción endotelial, todo lo cual puede perpetuar un microambiente nocivo que provoca dolor. Por otro lado, además de los dolores agudos episódicos, los pacientes con ECF también padecen dolor crónico, definido como dolor casi diario durante un periodo de 6 meses, asociado a trastornos psicosociales. Pueden deberse a lesiones crónicas como úlceras cutáneas, necrosis avascular ósea o infartos en diversos órganos. Asimismo, la sensibilización central parece estar directamente involucrada en la cronicidad del dolor y existe un componente de dolor neuropático claramente infradiagnosticado e infratratado. El tratamiento actual del dolor moderado a intenso en la ECF se basa principalmente en la administración de los opioides, vía oral, de liberación rápida ambulatoria o en forma de analgesia controlada por el paciente vía intravenosa intrahospitalaria. Sin embargo, el uso de opioides a largo plazo está asociado con múltiples efectos secundarios. Esta revisión presenta los últimos avances en la comprensión de la fisiopatología del dolor en la ECF y se describen los mecanismos subyacentes que pueden ayudar a desarrollar nuevas estrategias terapéuticas y/o preventivas para mejorar el dolor en la ECF


Patients with sickle cell disease (SCD) suffer from severe pain that often begins in childhood and increases in severity over the course of a lifetime, leading to hospitalization and poor quality of life over the years. A unique feature of SCD is vase-occlusive crises (VOC) characterized by recurrent and unpredictable episodes of acute pain. Microvascular occlusion during a VOC results in decreased oxygen supply to the periphery and injury from ischemia and subsequent reperfusion, inflammation, oxidative stress and endothelial dysfunction, all of which can perpetuate a harmful pain-causing microenvironment. On the other hand, in addition to episodic acute pain, SCD patients also report chronic pain, defined as almost daily pain over a 6-month period associated to either sicologic or social morbidities. They may be due to chronic lesions such as skin ulcers, avascular bone necrosis or infarctions in various organs. In addition, central sensitization appears to be directly involved in the chronicity of pain and there is a clearly under-diagnosed and under-treated component of neuropathic pain. Current treatment of moderate to severe pain in SCD is based primarily on opioids; either as an oral quick release outpatient or in the form of patient-controlled intravenous analgesia in the hospital. However, long-term opioid use is associated with multiple side effects. This review presents the latest advances in the understanding of the pathology of pain in SCD and describes objectives based on mechanisms that may help to develop new therapeutic and/or preventive strategies to improve pain in SCD


Assuntos
Humanos , Anemia Falciforme/fisiopatologia , Dor Aguda/fisiopatologia , Dor Crônica/fisiopatologia , Analgésicos Opioides/uso terapêutico , Dor Aguda/tratamento farmacológico , Dor Crônica/tratamento farmacológico , Analgesia/métodos , Manejo da Dor/métodos
12.
PLoS Comput Biol ; 16(7): e1008069, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32716940

RESUMO

Nitric oxide (NO) is a gaseous signaling molecule that plays an important role in neurovascular coupling. NO produced by neurons diffuses into the smooth muscle surrounding cerebral arterioles, driving vasodilation. However, the rate of NO degradation in hemoglobin is orders of magnitude higher than in brain tissue, though how this might impact NO signaling dynamics is not completely understood. We used simulations to investigate how the spatial and temporal patterns of NO generation and degradation impacted dilation of a penetrating arteriole in cortex. We found that the spatial location of NO production and the size of the vessel both played an important role in determining its responsiveness to NO. The much higher rate of NO degradation and scavenging of NO in the blood relative to the tissue drove emergent vascular dynamics. Large vasodilation events could be followed by post-stimulus constrictions driven by the increased degradation of NO by the blood, and vasomotion-like 0.1-0.3 Hz oscillations could also be generated. We found that these dynamics could be enhanced by elevation of free hemoglobin in the plasma, which occurs in diseases such as malaria and sickle cell anemia, or following blood transfusions. Finally, we show that changes in blood flow during hypoxia or hyperoxia could be explained by altered NO degradation in the parenchyma. Our simulations suggest that many common vascular dynamics may be emergent phenomena generated by NO degradation by the blood or parenchyma.


Assuntos
Encéfalo/fisiologia , Circulação Cerebrovascular , Óxido Nítrico/metabolismo , Anemia Falciforme/fisiopatologia , Arteríolas , Transfusão de Sangue , Sistema Livre de Células , Simulação por Computador , Difusão , Células Endoteliais/metabolismo , Eritrócitos/metabolismo , Hemodinâmica , Humanos , Processamento de Imagem Assistida por Computador , Imageamento Tridimensional , Malária/fisiopatologia , Mitocôndrias/metabolismo , Músculo Liso/metabolismo , Oscilometria , Distribuição de Poisson , Transdução de Sinais , Vasodilatação
13.
Pediatr Pulmonol ; 55(8): 2064-2073, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32484996

RESUMO

RATIONALE: Pulmonary complications are the leading cause of morbidity and mortality in sickle cell disease (SCD) patients. Research in SCD has predominantly been conducted on African-Americans, and the disease burden of SCD in other races and ethnicities, including Hispanic patients, is not well characterized. OBJECTIVE: To compare pulmonary disease burden between Hispanic and non-Hispanic ethnic groups among children with SCD. METHODS: In a retrospective chart review on 566 SCD patients followed at the Children's Hospital at Montefiore, NY, we compared the pulmonary disease burden and disease management in Hispanic patients to their non-Hispanic counterparts. We also compared the contribution of demographic and clinical variables to acute chest syndrome (ACS), vaso-occlusive crisis (VOC), and hospitalizations for SCD related complications between the two ethnic groups. RESULTS: Hispanic patients had a greater proportion of ACS, and had lower forced expiratory volume (FEV1), forced vital capacity, and vital capacity, compared to non-Hispanics. Hispanic patients were more likely to be evaluated in pulmonary clinic and to be on inhaled corticosteroids, short-acting ß agonizts, and leukotriene receptor antagonists. In addition, Hispanic children were more likely to be on hydroxyurea, and receive exchange transfusions. However, the association of asthma with the proportion of ACS did not differ between Hispanics and non-Hispanics. CONCLUSION: Hispanic children with SCD had differences in their pulmonary function profile and received more pulmonary evaluations than non-Hispanic children.


Assuntos
Anemia Falciforme/etnologia , Hispano-Americanos/estatística & dados numéricos , Pneumopatias/etnologia , Adolescente , Corticosteroides/uso terapêutico , Agonistas Adrenérgicos beta/uso terapêutico , Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Pneumopatias/tratamento farmacológico , Pneumopatias/fisiopatologia , Masculino , Testes de Função Respiratória , Estudos Retrospectivos
14.
Acta Cir Bras ; 35(3): e202000301, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32401830

RESUMO

PURPOSE: To analyze the serum levels of nitric oxide and correlate them with the levels of thiobarbituric acid reactive substances (TBARS) in liver, brain and spinal cord of animals using L-NAME and treated with hydroxyurea. METHODS: Eighteen male albino Wistar rats were divided into three groups. NG-nitro-L-arginine methyl ester (L-NAME) was intraperitoneally administered to induce oxidative stress. TBARS and plasma nitric oxide levels were analyzed in all groups. Histopathology of the liver and vascular tissue was performed. RESULTS: Statistically significant differences were seen in liver, brain and spinal cord TBARS levels. CONCLUSIONS: Following the use of L-NAME, hepatic tissue increased the number of Kupffer cells as oxidative stress and inflammatory response increased. The use of L-NAME caused an increase in lipid peroxidation products and, consequently, in oxidative stress in animals. Hydroxyurea doses of 35 mg / kg / day reduced TBARS values in liver, brain and spinal cord.


Assuntos
Anemia Falciforme/tratamento farmacológico , Encéfalo/metabolismo , Hidroxiureia/uso terapêutico , Fígado/metabolismo , Estresse Oxidativo/fisiologia , Medula Espinal/metabolismo , Anemia Falciforme/metabolismo , Anemia Falciforme/fisiopatologia , Animais , Modelos Animais de Doenças , Masculino , NG-Nitroarginina Metil Éster , Ratos , Ratos Wistar
15.
Pediatr Pulmonol ; 55(8): 2055-2063, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32462802

RESUMO

BACKGROUND: Sickle cell disease (SCD) is relatively common in Bahrain, and airway inflammation in patients with SCD is usually multifactorial. This study aimed to evaluate lung function and induced sputum levels of interleukin-6 (IL-6) in Bahraini children and adolescents with SCD and assess their relationship with the recurrence of acute chest syndrome (ACS). METHODS: A total of 139 children and adolescents with SCD and 123 healthy children (control group) were included in the present study. Patients were further stratified according to age and history of ACS. The patient and control groups underwent pulmonary function tests (PFTs), including spirometry and assessments of lung volume, diffusion of carbon monoxide (DLCO), and induced sputum IL-6 levels. RESULTS: Forced expiratory volume in 1 second (FEV1 ), force vital capacity (FVC), FEV1 /FVC, total lung capacity, DLCO, and DLCOc (ie, hemoglobin-corrected DLCO) were significantly lower, while residual volume and sputum IL-6 levels were significantly higher in the patient group than in the control group. PFT parameters were more compromised in the patient subgroup with a history of ACS and older than 12 years compared with the subgroup without a history of ACS and the subgroup under 12 years of age. PFTs revealed significant negative correlations with age, number of ACS events, and sputum IL-6 levels. CONCLUSION: Pulmonary function was observed to worsen with disease progression, and it worsened with older age and repeated occurrence of ACS. Induced sputum IL-6 levels reflected the degree of lung inflammation in affected patients and were associated with more impairment in various PFT parameters.


Assuntos
Anemia Falciforme/fisiopatologia , Adolescente , Anemia Falciforme/imunologia , Criança , Feminino , Humanos , Interleucina-6/imunologia , Masculino , Recidiva , Testes de Função Respiratória , Escarro/imunologia
16.
Trop Med Int Health ; 25(7): 897-904, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32329120

RESUMO

OBJECTIVES: HIV and sickle cell disease (SCD) are significant causes of morbidity and mortality in sub-Saharan Africa. Given their separate roles in immune dysregulation, our objective was to characterise the impact that SCD has on the presentation and progression of paediatric HIV. METHODS: The study was a retrospective cohort study (study period 2004-2018). Cases of HIV + and SCD-afflicted patients (HIV+/SCD+) were obtained via electronic chart review from a paediatric HIV clinic in Kampala, Uganda and matched 1:3 with HIV + controls without SCD (HIV+/SCD-). RESULTS: Thirty-five HIV+/SCD + subjects and 95 HIV+/SCD- controls were analysed (39% female (51/130), age 3.6 years (SD3.9)). At baseline, WHO clinical stage (64% total cohort Stage III/IV) and nutritional status (9.4% severe acute malnutrition) were similar for both groups, whereas HIV+/SCD + had higher though non-significant baseline CD4 count (1036 (SD713) vs 849 (SD638) cells/microlitre, P = 0.20, two-tailed t-test). There were 19 deaths, 6 (17%) HIV+/SCD + and 13 (14%) HIV+/SCD-, with unadjusted/adjusted models showing no significant difference. Nutritional progression and clinical stage progression showed no significant differences between groups. Kaplan-Meier analysis showed a slower rate of treatment failures in the HIV+/SCD + cohort (P = 0.11, log-rank survival test). Trajectory analysis showed that in the time period analysed, the HIV+/SCD + cohort showed a more rapid rise and higher total CD4 count (P = 0.012, regression analysis). CONCLUSION: The study suggests that SCD does not adversely affect the progression of HIV in patients on ART. Further, HIV+/SCD + achieved higher CD4 counts and fewer HIV treatment failures, suggesting physiological effects due to SCD might mitigate HIV progression.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/mortalidade , Infecções por HIV/mortalidade , Infecções por HIV/fisiopatologia , Adolescente , Anemia Falciforme/fisiopatologia , Fármacos Anti-HIV/uso terapêutico , Contagem de Linfócito CD4 , Criança , Pré-Escolar , Progressão da Doença , Feminino , Infecções por HIV/tratamento farmacológico , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Uganda/epidemiologia
17.
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
19.
Am J Hematol ; 95(7): 766-774, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32243618

RESUMO

In the US, mortality in sickle cell disease (SCD) increases after age 18-20 years. Biomarkers of mortality risk can identify patients who need intensive follow-up and early or novel interventions. We prospectively enrolled 510 SCD patients aged 3-20 years into an observational study in 2006-2010 and followed 497 patients for a median of 88 months (range 1-105). We hypothesized that elevated pulmonary artery systolic pressure as reflected in tricuspid regurgitation velocity (TRV) would be associated with mortality. Estimated survival to 18 years was 99% and to 25 years, 94%. Causes of death were known in seven of 10 patients: stroke in four (hemorrhagic two, infarctive one, unspecified one), multiorgan failure one, parvovirus B19 infection one, sudden death one. Baseline TRV ≥2.7 m/second (>2 SD above the mean in age-matched and gender-matched non-SCD controls) was observed in 20.0% of patients who died vs 4.6% of those who survived (P = .012 by the log rank test for equality of survival). The baseline variable most strongly associated with an elevated TRV was a high hemolytic rate. Additional biomarkers associated with mortality were ferritin ≥2000 µg/L (observed in 60% of patients who died vs 7.8% of survivors, P < .001), forced expiratory volume in 1 minute to forced vital capacity ratio (FEV1/FVC) <0.80 (71.4% of patients who died vs 18.8% of survivors, P < .001), and neutrophil count ≥10x109 /L (30.0% of patients who died vs 7.9% of survivors, P = .018). In SCD children, adolescents and young adults, steady-state elevations of TRV, ferritin and neutrophils and a low FEV1/FVC ratio may be biomarkers associated with increased risk of death.


Assuntos
Anemia Falciforme , Insuficiência da Valva Tricúspide , Adolescente , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/mortalidade , Anemia Falciforme/fisiopatologia , Biomarcadores/sangue , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Ferritinas/sangue , Seguimentos , Humanos , Contagem de Leucócitos , Masculino , Neutrófilos , Estudos Prospectivos , Taxa de Sobrevida , Insuficiência da Valva Tricúspide/sangue , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/mortalidade , Insuficiência da Valva Tricúspide/fisiopatologia , Estados Unidos/epidemiologia , Adulto Jovem
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