RESUMO
Venous thromboembolism (VTE) is a serious complication in hematologic neoplasms, so finding adequate prevention strategies is an urgent requirement. However, prospective studies with large enough cohorts are scarce, limiting the development of evidence-based thromboprophylaxis guidelines. The present position paper is addressed to all hematologists treating patients affected by hematologic neoplasms with the aim to provide clinicians with a useful tool for the prevention of VTE.
Assuntos
Neoplasias Hematológicas , Embolia Pulmonar , Tromboembolia Venosa , Anticoagulantes/uso terapêutico , Consenso , Neoplasias Hematológicas/complicações , Humanos , Estudos Prospectivos , Embolia Pulmonar/tratamento farmacológico , Tromboembolia Venosa/tratamento farmacológico , Tromboembolia Venosa/etiologia , Tromboembolia Venosa/prevenção & controleRESUMO
OBJECTIVE: To identify potential markers at admission predicting the need for critical care in patients with COVID-19 pneumonia. MATERIAL AND METHODS: An approved, observational, retrospective study was conducted between March 15 to April 15, 2020. 150 adult patients aged less than 75 with Charlson comorbidity index ≤6 diagnosed with COVID-19 pneumonia were included. Seventy-five patients were randomly selected from those admitted to the critical care units (critical care group [CG]) and seventy-five hospitalized patients who did not require critical care (non-critical care group [nCG]) represent the control group. One additional cohort of hospitalized patients with COVID-19 were used to validate the score. MEASUREMENTS AND MAIN RESULTS: Multivariable regression showed increasing odds of in-hospital critical care associated with increased C-reactive protein (CRP) (odds ratio 1.052 [1.009-1.101]; Pâ¯=â¯0.0043) and higher Sequential Organ Failure Assessment (SOFA) score (1.968 [1.389-2.590]; Pâ¯<â¯0.0001), both at the time of hospital admission. The AUC-ROC for the combined model was 0.83 (0.76-0.90) (vs AUC-ROC SOFA Pâ¯<â¯0.05). The AUC-ROC for the validation cohort was 0.89 (0.82-0.95) (Pâ¯>â¯0.05 vs AUC-ROC development). CONCLUSION: Patients COVID-19 presenting at admission SOFA scoreâ¯≥â¯2 combined with CRPâ¯≥â¯9.1â¯mg/mL could be at high risk to require critical care.
Assuntos
COVID-19 , Sepse , Adulto , Proteína C-Reativa , Cuidados Críticos , Humanos , Prognóstico , Curva ROC , Estudos Retrospectivos , SARS-CoV-2 , EspanhaRESUMO
OBJECTIVE: To identify potential markers at admission predicting the need for critical care in patients with COVID-19 pneumonia. MATERIAL AND METHODS: An approved, observational, retrospective study was conducted between March 15 to April 15, 2020. 150 adult patients aged less than 75 with Charlson comorbidity index ≤6 diagnosed with COVID-19 pneumonia were included. Seventy-five patients were randomly selected from those admitted to the critical care units (critical care group [CG]) and seventy-five hospitalized patients who did not require critical care (non-critical care group [nCG]) represent the control group. One additional cohort of hospitalized patients with COVID-19 were used to validate the score. MEASUREMENTS AND MAIN RESULTS: Multivariable regression showed increasing odds of in-hospital critical care associated with increased C-reactive protein (CRP) (odds ratio 1.052 [1.009-1.101]; P=.0043) and higher Sequential Organ Failure Assessment (SOFA) score (1.968 [1.389-2.590]; P<.0001), both at the time of hospital admission. The AUC-ROC for the combined model was 0.83 (0.76-0.90) (vs AUC-ROC SOFA P<.05). The AUC-ROC for the validation cohort was 0.89 (0.82-0.95) (P>0.05 vs AUC-ROC development). CONCLUSION: Patients COVID-19 presenting at admission SOFA score ≥2 combined with CRP ≥9,1mg/mL could be at high risk to require critical care.
RESUMO
OBJECTIVE: To identify potential markers at admission predicting the need for critical care in patients with COVID-19 pneumonia. MATERIAL AND METHODS: An approved, observational, retrospective study was conducted between March 15 to April 15, 2020. 150 adult patients aged less than 75 with Charlson comorbidity index ≤ 6 diagnosed with COVID-19 pneumonia were included. Seventy-five patients were randomly selected from those admitted to the critical care units (critical care group [CG]) and seventy-five hospitalized patients who did not require critical care (non-critical care group [nCG]) represent the control group. One additional cohort of hospitalized patients with COVID-19 were used to validate the score. MEASUREMENTS AND MAIN RESULTS: Multivariable regression showed increasing odds of in-hospital critical care associated with increased C-reactive protein (CRP) (odds ratio 1.052 [1.009-1.101]; P = .0043) and higher Sequential Organ Failure Assessment (SOFA) score (1.968 [1.389-2.590]; P < .0001), both at the time of hospital admission. The AUC-ROC for the combined model was 0.83 (0.76-0.90) (vs AUC-ROC SOFA P < .05). The AUC-ROC for the validation cohort was 0.89 (0.82-0.95) (P > 0.05 vs AUC-ROC development). CONCLUSION: Patients COVID-19 presenting at admission SOFA score ≥ 2 combined with CRP ≥ 9,1 mg/mL could be at high risk to require critical care.
Assuntos
Benzoatos/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Hidrazinas/administração & dosagem , Pirazóis/administração & dosagem , Trombocitopenia/tratamento farmacológico , Adulto , Idoso , Aloenxertos , Benzoatos/efeitos adversos , Feminino , Humanos , Hidrazinas/efeitos adversos , Masculino , Pessoa de Meia-Idade , Pirazóis/efeitos adversos , Estudos Retrospectivos , Trombocitopenia/sangue , Trombocitopenia/etiologiaRESUMO
Essentials Diagnosis of sitosterolemia, a rare recessive or syndromic disorder, is usually delayed. Peripheral blood smear is extremely useful for establishing the suspicion of sitosterolemia. High-throughput sequencing technology enables the molecular diagnosis of inherited thrombocytopenias. Accurate characterization of sitosterolemia helps us determine appropriate management. SUMMARY: Background Sitosterolemia (STSL) is a recessive inherited disorder caused by pathogenic variants in the ABCG5 and ABCG8 genes. Increased levels of plasma plant sterols (PSs) usually result in xanthomas and premature coronary atherosclerosis, although hematologic abnormalities may occasionally be present. This clinical picture is unfamiliar to many physicians, and patients may be at high risk of misdiagnosis. Objectives To report two novel ABCG5 variants causing STSL in a Spanish patient, and review the clinical and mutational landscape of STSL. Patient/Methods A 46-year-old female was referred to us with lifelong macrothrombocytopenia. She showed familial hypercholesterolemia-related xanthomas. Molecular analysis was performed with high-throughput sequencing. Plasma PS levels were evaluated with gas-liquid chromatography. The STSL landscape was reviewed with respect to specific online databases and all reports published since 1974. Results A blood smear revealed giant platelets and stomatocytes. Novel compound heterozygous variants were detected in exons 7 (c.914C>G) and 13 (c.1890delT) of ABCG5. The patient showed an increased plasma level of sitosterol. These findings support the diagnosis of STSL. In our review, we identified only 25 unrelated STLS patients who presented with hematologic abnormalities including macrothrombocytopenia. It remains unknown why only some patients develop hematologic abnormalities. Conclusions This is the first Spanish STSL patient to be reported and molecularly characterized. The early diagnosis of STLS is strongly supported by the presence of stomatocytes in blood smears. The definitive diagnosis of STSL by measurement of serum PS levels and molecular analyses prompted the use of ezetimibe therapy.
Assuntos
Membro 5 da Subfamília G de Transportadores de Cassetes de Ligação de ATP/genética , Hipercolesterolemia/genética , Enteropatias/genética , Erros Inatos do Metabolismo Lipídico/genética , Lipoproteínas/genética , Mutação , Fitosteróis/efeitos adversos , Trombocitopenia/genética , Xantomatose/genética , Anticolesterolemiantes/uso terapêutico , Análise Mutacional de DNA , Ezetimiba/uso terapêutico , Feminino , Predisposição Genética para Doença , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Hipercolesterolemia/sangue , Hipercolesterolemia/diagnóstico , Hipercolesterolemia/tratamento farmacológico , Enteropatias/sangue , Enteropatias/diagnóstico , Enteropatias/tratamento farmacológico , Erros Inatos do Metabolismo Lipídico/sangue , Erros Inatos do Metabolismo Lipídico/diagnóstico , Erros Inatos do Metabolismo Lipídico/tratamento farmacológico , Pessoa de Meia-Idade , Fenótipo , Fitosteróis/sangue , Fitosteróis/genética , Sitosteroides/sangue , Espanha , Trombocitopenia/sangue , Trombocitopenia/diagnóstico , Xantomatose/sangue , Xantomatose/diagnósticoRESUMO
Very few data exist on the management of adult patients diagnosed with primary immune thrombocytopenia (ITP). The objectives of this study were to describe the diagnostic and treatment patterns for ITP and to compare the findings to recent ITP guidelines. We retrospectively analyzed the medical records of adult ITP patients diagnosed with primary ITP between January 2011 and June 2012 and examined whether management strategies were consistent or not with eight recent guideline-recommended practices. Overall, median age at the diagnosis of the disease (n = 101) was 58 years and median platelet count 12 × 10(9)/L with 75.2 % of patients having symptoms of ITP. The study perceived two major shortcomings in the diagnostic approach: (1) failure to perform peripheral blood film examination in 22.8 % of patients, a test that is mandatory by all guidelines, and (2) ordinary bone marrow assessment in more than half of the patients at diagnosis (50.5 %), a test not routinely recommended by guidelines. Low appropriateness in therapeutic management of patients included (1) unjustified use of intravenous immunoglobulin in the absence of bleeding in 54.8 % of patients and (2) splenectomy not being deferred until 6-12 months from diagnosis (median 161 days). Data also reflect a trend towards the early use of thrombopoietin receptor agonists in the treatment of patients who are refractory to any first-line therapy. We have recognized important areas of inapropriateness in the diagnostic and therapeutic management of adult ITP patients. Compliance with established guidelines should be encouraged in order to improve patient outcomes.
Assuntos
Gerenciamento Clínico , Fidelidade a Diretrizes/normas , Guias de Prática Clínica como Assunto/normas , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
INTRODUCTION: Molecular testing of Inherited bleeding coagulation disorders (IBCDs) not only offers confirmation of diagnosis but also aids in genetic counselling, prenatal diagnosis and in certain cases genotype-phenotype correlations are important for predicting the clinical course of the disease and to allow tailor-made follow-up of individuals. Until recently, genotyping has been mainly performed by Sanger sequencing, a technique known to be time consuming and expensive. Currently, next-generation sequencing (NGS) offers a new potential approach that enables the simultaneous investigation of multiple genes at manageable cost. AIM: The aim of this study was to design and to analyse the applicability of a 23-gene NGS panel in the molecular diagnosis of patients with IBCDs. METHODS: A custom target enrichment library was designed to capture 31 genes known to be associated with IBCDs. Probes were generated for 296 targets to cover 86.3 kb regions (all exons and flanking regions) of these genes. Twenty patients with an IBCDs phenotype were studied using NGS technology. RESULTS: In all patients, our NGS approach detected causative mutations. Twenty-one pathogenic variants were found; while most of them were missense (18), three deletions were also identified. Six novel mutations affecting F8, FGA, F11, F10 and VWF genes, and 15 previously reported variants were detected. NGS and Sanger sequencing were 100% concordant. CONCLUSION: Our results demonstrate that this approach could be an accurate, reproducible and reliable tool in the rapid genetic diagnosis of IBCDs.
Assuntos
Transtornos Herdados da Coagulação Sanguínea/genética , Testes Genéticos/métodos , Adolescente , Adulto , Transtornos Herdados da Coagulação Sanguínea/patologia , Criança , Pré-Escolar , DNA/química , DNA/isolamento & purificação , DNA/metabolismo , Feminino , Mutação da Fase de Leitura , Deleção de Genes , Estudos de Associação Genética , Genótipo , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Mutação de Sentido Incorreto , Análise de Sequência de DNA , Adulto JovemRESUMO
BACKGROUND: Prosthetic material (mesh) is commonly used to repair inguinal hernias. Its implantation close to the common femoral vein (CFV) can induce slow flow and favor the appearance of venous thromboembolism (VTE) events. AIM: To investigate the speed of flow, diameter and area of the CFV after inguinal hernioplasty. METHODS: Two hundred and fifty patients receiving open hernioplasty with a non-resorbable mesh for the repair of a unilateral, primary, simple inguinal hernia were prospectively investigated. Patients were stratified, by consensus, into a low or a moderate risk of VTE group. The moderate-risk group (n = 163) received low molecular weight heparin. On day 10 post-operation a blinded Echo-Doppler was carried out, and repeated 7 days later in patients with a venous flow of <15 cm/s. The speed of flow (cm/s), diameter (cm), and area (cm(2)) of the ipsilateral and contralateral CFV of the groin operated upon were measured. RESULTS: No event symptomatic of VTE was documented. One case of asymptomatic deep vein thrombosis (1/163, 0.6%) was found in the moderate-risk group. In 29 patients (2 and 27 in the low- and moderate-risk groups, respectively; p < 0.001) a maximum blood flow velocity of <15 cm/s was found in the ipsilateral CFV; these flows were close to normal in the second measurement. Taking the entire sample into account, the maximum venous blood flow found in the ipsilateral CFV of the operated groin was less than that measured in the contralateral CFV (20.88 vs. 24.01 cm/s; p < 0.001); this difference was significant in both VTE risk groups. The diameter and area of the CFV were both greater in the ipsilateral than the contralateral CFV (p < 0.01); this finding proved to be significant only in hernias of the left groin (p < 0.001). CONCLUSIONS: In the immediate postoperative period, inguinal hernioplasty with mesh induces a temporarily slow venous flow in the ipsilateral CFV. However, this does not lead to an increase in the incidence of VTE.
Assuntos
Veia Femoral/cirurgia , Hérnia Inguinal/cirurgia , Herniorrafia/efeitos adversos , Adulto , Idoso , Anticoagulantes/uso terapêutico , Velocidade do Fluxo Sanguíneo , Feminino , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Telas Cirúrgicas/efeitos adversos , Insuficiência Venosa/etiologia , Insuficiência Venosa/prevenção & controle , Tromboembolia Venosa/etiologia , Tromboembolia Venosa/prevenção & controle , Adulto JovemRESUMO
AIM: Telomerase is a ribonucleoprotein that maintains telomere length. Telomeres and telomerase are involved in cellular ageing and have been connected to some ageing related diseases, like cardiovascular disease. Telomerase dysfunction could be the main underlying mechanism in this connection but this point is still unclear. The aim of this article is to investigate the possible influence of cellular ageing, measured by two telomerase polymorphisms, TERC-63G>A (rs2293607) and TERT-1327C>T (rs2735940), on the whole spectrum of acute coronary artery disease (CAD). METHODS: We studied 150 middle aged men admitted for an acute coronary syndrome (ACS). Cardiovascular risk factors prevalence was collected at admission. Severity variables analyzed were Killip class and number of vessels affected. Telomerase polymorphisms were studied by real time PCR in DNA samples extracted from peripheral blood leukocytes. Clinical follow-up had been developed for more than 600 days and a prognostic combined event was defined. RESULTS: C allele of TERT polymorphism was more prevalent among hypertensive patients (OR: 3.19; 95% CI: 1.37-7.42; P=0.006). None of polymorphisms showed any prognostic value or relation to CAD severity. CONCLUSION: Telomerase dysfunction could be involved in hypertension prevalence. This finding could support new screening strategies in high risk population. The two telomerase polymorphisms analyzed did not show any prognostic value or connection to CAD severity. However, further studies are required to determine the molecular mechanisms responsible for cellular ageing in ACS.
Assuntos
Síndrome Coronariana Aguda/genética , Doença da Artéria Coronariana/genética , Hipertensão/genética , Telomerase/genética , Síndrome Coronariana Aguda/fisiopatologia , Idoso , Alelos , Senescência Celular/genética , Estudos de Coortes , Doença da Artéria Coronariana/fisiopatologia , Seguimentos , Humanos , Hipertensão/epidemiologia , Masculino , Pessoa de Meia-Idade , Polimorfismo Genético , Prevalência , Prognóstico , Estudos Prospectivos , Reação em Cadeia da Polimerase em Tempo Real , Fatores de Risco , Índice de Gravidade de Doença , Telômero/metabolismoRESUMO
INTRODUCTION: The Ankle-Brachial Index (ABI) makes it possible to identify patients with peripheral artery disease (PAD). Intermittent claudication (IC) is the first major symptom of PAD, although many patients with an ABI ≤ 0.9 do not exhibit IC, and the range of ABI among those who do have IC is very variable. This study evaluates the correlation between ABI and the perception (symptomatology) of claudicant patients. MATERIAL AND METHODS: An observational, cross-sectional and multicentre, study of 920 patients with IC. Clinical history, ABI, Walking Impairment Questionnaire (WIQ) and European Quality of Life Questionnaire (EQ-5D) were recorded. Associations were analysed using Spearman's correlation coefficient. RESULTS: The mean ABI of the series was 0.63 (SD = 0.19). The mean WIQ-distance was 34.07 (SD = 26.77), values being smaller for lower ABI values (r = 0.343, p < 0.001). The mean EQ-5D score of the series was 0.58 (SD = 0.21), also showing lower values as the ABI decreased (r = 0.278, p < 0.001). The correlations of WIQ and EQ-5D with ABI were statistically significant in both cases, but always less than 0.400 (between 0.278 and 0.343). CONCLUSIONS: The correlations of ABI with the questionnaires of walking capacity and quality of life are weak. For this reason, although in clinical practice the ABI of CI patients is commonly measured, decisions should not be taken during the development of IC exclusively on the basis of the ABI.
Assuntos
Índice Tornozelo-Braço/estatística & dados numéricos , Claudicação Intermitente/diagnóstico , Doença Arterial Periférica/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Índice Tornozelo-Braço/mortalidade , Estudos Transversais , Feminino , Humanos , Claudicação Intermitente/mortalidade , Masculino , Pessoa de Meia-Idade , Doença Arterial Periférica/mortalidade , Qualidade de Vida , Espanha , Inquéritos e QuestionáriosRESUMO
Transplantation-associated thrombotic microangiopathy (TA-TMA) is a feared complication of allogeneic hematopoietic SCT (HSCT) owing to its high mortality rate. The use of calcineurin inhibitors or sirolimus (SIR) for GVHD prophylaxis has been suggested as a potential risk factor. However, the impact of tacrolimus (TAC) and SIR combinations on the increased risk of TA-TMA is currently not well defined. We retrospectively analyzed the incidence of TA-TMA in 102 allogeneic HSCT recipients who consecutively received TAC plus SIR (TAC/SIR) (n=68) or plus MTX (TAC/MTX)±ATG (n=34) for GVHD prophylaxis. No significant differences were observed in the incidence of TA-TMA between patients receiving TAC/SIR vs TAC/MTX±ATG (7.4% vs 8.8%, P=0.8). Only grade III-IV acute GVHD, previous HSCT and serum levels of TAC >25 ng/mL were associated with a greater risk of TA-TMA. Patients developing TA-TMA have significantly poorer survival (P<0.001); however, TA-TMA ceased to be an independent prognostic factor when it was included in a multivariate model. In conclusion, the combination of TAC/SIR does not appear to pose a higher risk of TA-TMA. By contrast, we identified three different risk groups for developing TA-TMA.
Assuntos
Doença Enxerto-Hospedeiro/prevenção & controle , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Metotrexato/administração & dosagem , Sirolimo/administração & dosagem , Tacrolimo/administração & dosagem , Microangiopatias Trombóticas/etiologia , Adulto , Idoso , Quimioterapia Combinada , Feminino , Doença Enxerto-Hospedeiro/tratamento farmacológico , Doença Enxerto-Hospedeiro/epidemiologia , Transplante de Células-Tronco Hematopoéticas/estatística & dados numéricos , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/sangue , Incidência , Masculino , Metotrexato/sangue , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Sirolimo/sangue , Tacrolimo/sangue , Microangiopatias Trombóticas/epidemiologia , Transplante Homólogo , Adulto JovemRESUMO
OBJECTIVES: Chronic venous disease (CVD) is a frequent disorder with a high socioeconomic impact. Little is known about the possible differences between healed ulcer (C5 group) and active ulcer (C6 group) in terms of disease severity and quality of life (QoL). Our aim was to determine the possible differences in severity disease and QoL between the C5-C6 and C1 (control) group. METHODS: Data from a national, multicentre, observational and cross-sectional study (n = 1598) were used to compare three groups of CVD: C1 (n = 243), C5 (n = 136) and C6 (n = 70). CVD severity was assessed with the Venous Clinical Severity Score (VCSS) and QoL with the Short Form 12 Health Survey (SF-12) and Chronic Lower Limb Venous Insufficiency Questionnaire (CIVIQ-20). RESULTS: Patients with active ulcers had a higher mean total VCSS than patients with healed ulcers (P < 0.05). Both SF-12 and CIVIQ-20 QoL questionnaires indicated a poorer QoL in patients with ulcers than in those with C1 (P < 0.05). Compared with the C5 group, patients with active ulcers (C6) had lower QoL scores, but the differences were not statistically significant. CONCLUSIONS: Patients with venous leg ulcers (C5-C6) are associated with high severity and poor QoL. However, the healing of a leg ulcer did not contribute to improvement of QoL.
Assuntos
Úlcera Varicosa/diagnóstico , Doenças Vasculares/diagnóstico , Adulto , Idoso , Doença Crônica , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Qualidade de Vida , Classe Social , Inquéritos e Questionários , Úlcera Varicosa/complicações , Doenças Vasculares/complicações , Insuficiência Venosa/diagnósticoRESUMO
AIM: Chronic venous disease (CVD) is a well-defined and known disorder which impact on related-health quality of life (QoL). However, individuals factors which determinate quality of life on CVD are not well defined. The purposes of this study were to describe the QoL in patients with CVD and examine socio-demographical and clinical factors which influence QoL METHODS: One thousand five hundred sixty patients with CVD were evaluated. We calculated for each patient two disease-specific severity scores: The "C" grade (clinical) of the CEAP classification and the Venous Clinical Severity Score (VCSS). Additionally, two QoL questionnaires were recorded: Short-Form Health Survey (SF-12) and Chronic Lower Limb Venous Insufficiency Questionnaire (CIVIQ-20). Two multivariate logistic regression analyses (SF-12 and CIVIQ-20 model) were conducted to determine whether demographic and clinical variables were independently associated with QoL. RESULTS: Both QoL questionnaires indicated that the QoL decreased significantly (P<0.05) as the C grade of CEAP increased. Each increase of 0.10 in VCSS score represented 2% worsening in QoL as measured by CIVIQ-20. Three factors were strongly associated with poor QoL on SF-12: increasing age, prior superficial venous thrombosis (SVT) or prior deep venous thrombosis. In the specific disease CIVIQ-20 questionnaire three factors (higher age, prior SVT and higher weight) were strongly associated with decrease QoL. CONCLUSION: Increasing disease severity by VCSS is associated with reductions in QoL. Subgroup analysis indicates that there are several significant individual determinants of worsening QoL.
Assuntos
Qualidade de Vida , Varizes/psicologia , Insuficiência Venosa/psicologia , Adulto , Idoso , Doença Crônica , Efeitos Psicossociais da Doença , Estudos Transversais , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prognóstico , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Espanha/epidemiologia , Inquéritos e Questionários , Varizes/diagnóstico , Varizes/epidemiologia , Insuficiência Venosa/diagnóstico , Insuficiência Venosa/epidemiologiaRESUMO
AIM: The present study aimed to demonstrate how the quality of life (QoL) perceived by patients with chronic venous disease (CVD) is correlated with the severity of their disease objectively assessed by primary care physician. MATERIAL AND METHODS: A total of 1560 patients with CVD were evaluated using four measurement instruments: CEAP clinical classification, Venous Clinical Severity Score (VCSS), SF-12 Health Survey and Chronic Lower Limb Venous Insufficiency Questionnaire (CIVIQ-20). Statistical correlations between these tools were analysed using Spearman's coefficient. RESULTS: Patients were distributed in C0, 58 (3.7%); C1, 243 (15.6%); C2, 328 (21.0%); C3, 357 (22.9%); C4, 368 (23.6%); C5, 136 (8.7%); and C6, 70 (4.5%). The VCSS score for the whole cohort was 0.89 ± 0.53. The correlation between CEAP and VCSS was moderately strong (r = 0.69). The overall QoL scores measured by SF and CIVIQ were 56.84 ± 19.63 and 65.11 ± 14.35, respectively. The correlation between the two QoL questionnaires was very strong (r = 0.81). The correlations of the SF and CIVIQ with the VCSS were moderately strong (r = -0.47 and -0.48). The correlations between QoL questionnaires and CEAP were moderate and lower than those with VSCC. CONCLUSIONS: While there is correlation between VCSS, CEAP, modified CIVIQ and venous ultrasound findings, subgroup analysis indicates that this correlation is driven by different components of VCSS compared with the other venous assessment tools. Patients' opinions about their disease are correlated with those assessed by primary care physicians.
Assuntos
Pacientes/psicologia , Médicos de Atenção Primária/psicologia , Qualidade de Vida , Inquéritos e Questionários , Insuficiência Venosa/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Índice de Gravidade de Doença , Espanha , Ultrassonografia , Insuficiência Venosa/diagnóstico por imagem , Insuficiência Venosa/psicologia , Adulto JovemRESUMO
The severity of neutropenia in myelodysplastic syndrome (MDS) has not been completely studied. We analyzed the prognostic significance of severe neutropenia (neutrophils count <0.5×10(9)/L) at diagnosis in 1109 patients with de novo MDS and low/intermediate-1 IPSS included in the Spanish MDS Registry. Severe neutropenia was present at diagnosis in 48 of 1109 (4%). Patients with severe neutropenia were most strongly represented within the groups of refractory cytopenia with multilineage dysplasia (40%) and refractory anemia with excess of blast type 1 (29%). Severe neutropenia had negative effects on the low/intermediate-1 risk group. A significant difference in overall survival was observed between patients with severe neutropenia (28 months) and patients with a neutrophil count higher than 0.5×10(9)/L (66 months) (p<0.0001). Also, severe neutropenia predicted a significantly reduced on leukemia-free survival (p<0.0001). In the multivariate analysis, severe neutropenia retained its independent prognostic influence on overall survival [HR: 2.19, 95% CI (1.41-3.10), p<0.0001] and leukemia free survival [HR: 3.51, 95% CI (1.97-6.26), p<0.0001]. The degree of neutropenia should be considered as additional prognostic factor in low/intermediate-1 IPSS MDS.
Assuntos
Anemia Refratária/complicações , Síndromes Mielodisplásicas/complicações , Neutropenia/diagnóstico , Neutropenia/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia Refratária/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/mortalidade , Estadiamento de Neoplasias , Neutropenia/mortalidade , Prognóstico , Fatores de Risco , Taxa de Sobrevida , Adulto JovemRESUMO
BACKGROUND: Current guidelines support the use of erythropoiesis-stimulating agents for the treatment of anemia associated with low-risk myelodysplastic syndromes (MDS). DESIGN AND METHODS: Single-arm, open-label, multi-center, phase 2 trial that evaluated the efficacy and safety of darbepoetin alfa (DA) in patients with low or intermediate-risk MDS, hemoglobin <100 g/L, erythropoietin (EPO) levels <500 IU/L and transfusion requirements <2 units/month over the preceding 2 months. Erythroid response (major [MaR] or minor [MiR]) and fatigue (Functional Assessment of Cancer Therapy-Fatigue [FACT-F]) were evaluated at 8, 16 and 24 weeks. DA was initiated at 300 µg weekly. For patients who did not achieve MaR by 8 weeks, filgrastim 300 µg weekly was added. CLINICAL TRIAL REGISTRATION: clinicaltrials.gov identifier: NCT01039350. RESULTS: Forty-four patients (72.7% transfusion independent) were included. Median age was 76.0 years (range 41.3-92.4), 54.5% were male, and 90.9% presented ECOG Status (0-1). Eighteen patients received filgrastim. An erythroid response was achieved by 31 of 44 patients (70.5%) at week 8 (47.7% MaR, 22.7% MiR), 31 of 44 patients (70.5%) at week 16 (61.4% MaR, 9.1% MiR), and 32 of 44 patients (72.7%) at week 24 (61.3% MaR, 11.4% MiR). Mean (95% CI) change in FACT-F at week 24 was 3.61 (0.72 to 6.51). Baseline EPO levels <100 IU/L were a predictive factor of response. DA was well tolerated. Four mild (two iron deficiencies, flu syndrome and headache) and one fatal (thromboembolic event) adverse events were considered related to darbepoetin alfa. CONCLUSIONS: A fixed dose of 300 µg of darbepoetin alfa weekly (with or without filgrastim) seems to be an effective and safe treatment for anemic patients with low or intermediate-risk MDS, low transfusion burden and EPO levels <500 IU/L. Results may not be extrapolable to unselected MDS patients.
Assuntos
Anemia/tratamento farmacológico , Eritropoetina/análogos & derivados , Hematínicos/administração & dosagem , Síndromes Mielodisplásicas/tratamento farmacológico , Adulto , Idoso , Anemia/sangue , Anemia/mortalidade , Darbepoetina alfa , Eritropoetina/administração & dosagem , Eritropoetina/efeitos adversos , Eritropoetina/sangue , Feminino , Hematínicos/efeitos adversos , Hemoglobinas/análise , Hemoglobinas/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/sangue , Síndromes Mielodisplásicas/mortalidade , Fatores de RiscoRESUMO
We have prospectively evaluated the efficacy of an individualized pre-operative blood saving protocol in elective total hip arthroplasty (THA) or total knee arthroplasty (TKA). The primary aim was to obtain a pre-operative haemoglobin (Hb) level of > or =14 g dL(-1). A reduction in requirements for allogeneic transfusion was considered the second aim. Several strategies are available for increasing pre-operative Hb levels and reducing red blood cell (RBC) transfusions following THA or TKA, but the success of these programmes depends on selecting the most appropriate treatment for each patient. Three hundred and five patients with an indication of elective THA or TKA were individually assigned to the following strategies according to Hb and ferritin levels and medical conditions: (a) no pre-operative intervention, (b) oral iron therapy, (c) intravenous (i.v.) iron therapy, (d) recombinant human erythropoietin alpha with i.v. iron and (e) pre-operative autologous donation (PAD) plus oral iron. Eighty-two percent of the patients reached a pre-operative Hb level of > or =14 g dL(-1) compared with 62% of patients with Hb levels of > or =14 g dL(-1) at the baseline visit. Treatment with PAD showed a significant reduction in the pre-operative Hb levels. The rate of RBC transfusion was 18.8% compared with 31.5% of matched historic group (P < 0.001). In conclusion, all patients scheduled to undergo THA or TKA should be candidates for an individualized pre-operative blood salvage programme.
Assuntos
Artroplastia de Quadril/métodos , Artroplastia do Joelho/métodos , Transfusão de Sangue , Ferritinas/sangue , Hemoglobinas/análise , Cuidados Pré-Operatórios/métodos , Idoso , Transfusão de Sangue Autóloga , Eritropoetina/administração & dosagem , Eritropoetina/uso terapêutico , Feminino , Humanos , Ferro/administração & dosagem , Ferro/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Proteínas RecombinantesRESUMO
BACKGROUND AND OBJECTIVES: The greatest risk in transfusion medicine is actually human error, resulting in the use of the incorrect blood component. The aim of our study was to identify and evaluate the risk factors involved in the collection and labelling of pretransfusion blood samples. MATERIAL AND METHODS: We prospectively evaluated 6446 samples submitted to the blood bank for pretransfusion testing. Inappropriate samples were classified as 'mislabelled' or 'miscollected'. After 4 months of study, an educational approach was taken. RESULTS: The frequency of inappropriately labelled samples was 6.45%. Such samples were associated with the use of addressograph labels (vs. hand-written labels) [23.4% vs. 1.4%, P < 0.0001], collection by clinical staff (vs. blood bank staff) [8.8% vs. 2.1%, P = 0.001] and emergency situations (vs. routine sampling) [10.1% vs. 6.1%, P = 0.005]. Following educational intervention, the percentage of inappropriately labelled samples decreased from 7.3% (pre-educational) to 5.8% (post-educational), P = 0.005. CONCLUSION: Ongoing monitoring and analysis of labelling and collection should be mandatory in order to improve the safety of transfusion.