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1.
Matern Child Health J ; 28(1): 24-30, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38006564

RESUMO

PURPOSE: To assess the potential of workplace support to protect public health equity workers against job burnout and to identify key workplace support components. DESCRIPTION: This mixed-methods, explanatory sequential study analyzed survey and interview data collected between August 2020 and June 2021. Participants included governmental and non-governmental public health employees whose programs largely focus on Maternal and Child Health populations and who reported that their jobs involved working to reduce health inequities ("equity work"). Regression analysis tested the effect of emotional labor on job burnout, and whether workplace support modified that effect. Qualitative analysis of interview transcripts explored possible components of needed workplace support. ASSESSMENT: Emotional labor was positively associated with job burnout (p < .001), and there was a significant negative interaction between emotional labor and workplace support, meaning workplace support appeared to reduce the effect of emotional labor on burnout (p = .036). Qualitative analysis identified four support components: peer-to-peer mentoring connections, workplace accommodations, engaged and empathetic supervision, and mental health resources. CONCLUSION: Workplace support is associated with reduced job burnout for public health equity workers, especially those whose jobs involve high levels of emotional labor. Few public health employers are providing needed emotional supports for their equity workers, but certain supports appear to be helpful in reducing job burnout.


Assuntos
Esgotamento Profissional , Equidade em Saúde , Criança , Humanos , Saúde da Criança , Saúde Pública , Local de Trabalho/psicologia , Esgotamento Profissional/prevenção & controle , Esgotamento Profissional/psicologia , Inquéritos e Questionários , Satisfação no Emprego
2.
Semin Thromb Hemost ; 49(4): 319-329, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36750218

RESUMO

The hemostasis system is composed of procoagulant, anticoagulant, and fibrinolytic proteins that interact with endothelial and blood cells and with each other in a complex system of checks and balances to maintain blood flow while preventing both hemorrhage and thrombosis. Pregnancy is a unique physiological state in which biological alterations predispose both mother and fetus to both bleeding and clotting. The placenta is a vascular interface for maternal and fetal blood exchange which predisposes the mother to hemorrhage. Maternal hemostasis presents a compensatory hypercoagulability including elevated factor VIII, von Willebrand factor, fibrinogen and thrombin generation, decreased thrombin regulation with resistance to activated protein C and decreased free protein S, and decreased fibrinolysis with increased plasminogen activator inhibitors. The placental vascular surface is of fetal trophoblastic origin that derives many characteristics of endothelium but differs in that tissue factor is constitutively expressed. Ontogeny of fetal hemostasis is characteristic. Platelets, von Willebrand factor, factor VIII, and fibrinogen are expressed and mature early in gestation, while vitamin K-dependent and contact factors exhibit delayed development. The fetal hemostatic system has a decreased capacity to generate or regulate thrombin, resulting in a fragile balance with little capacity to compensate under stress conditions, particularly in the infant born prematurely. Dysfunction of the maternal/placental/fetal unit gives rise to gestational disorders including preeclampsia, fetal growth restriction, placental abruption, and premature delivery. Knowledge of normal hemostasis levels and function are critical to evaluate bleeding or clotting syndromes in the pregnant woman and her fetus or newborn infant.


Assuntos
Hemostasia , Feminino , Humanos , Lactente , Recém-Nascido , Gravidez , Fator VIII/metabolismo , Feto , Fibrinogênio/metabolismo , Placenta/metabolismo , Gestantes , Trombina/metabolismo , Fator de von Willebrand/metabolismo
3.
Cell Tissue Res ; 387(3): 479-491, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35275281

RESUMO

Soluble fibrin (SF) in blood consists of monomers lacking both fibrinopeptides A with a minor population in multimeric clusters. It is a substantial component of isolated fibrinogen (fg), which spontaneously self-assembles into protofibrils progressing to fibers at sub-physiologic temperatures, a process enhanced by adsorption to hydrophobic and some metal surfaces. Comparisons of SF-rich (FR) and SF-depleted (FD) fg isolates disclosed distinct molecular imprints of each via an adsorption/desorption procedure using gold surfaced silica microplates. Accelerated plasminogen activator-induced lysis and decreased stiffness (G') of thrombin-induced FR fg clots were revealed by thomboelastography. Erythrocyte sedimentation (ESR) in afibrinogenemic plasma (Hematocrit 25-33%) was accelerated by FR fg nearly threefold that of FD fg. Stained smears disclosed frequent rouleaux formations and fibers linking stacked erythrocytes in contrast to no rouleaux by FD fg. Rouleaux formations were more pronounced at 4 °C than at ambient temperatures and at fiber-membrane contacts displayed irregular, knobby membrane contours. One of several FR fg isolates also displayed incomplete fiber networks in cell-free areas. What is more, pre-mixing FR fg with each of three monoclonal IgG anti-fg antibodies at 1.5 mol/mol fg, that inhibited fibrin polymerization, prevented rouleaux formation save occasional 2-4 erythrocyte aggregates. We conclude that spontaneously generated SF fibers bound to erythrocytes forming intercellular links culminating in rouleaux formation and ensuing ESR acceleration which in clinical settings reflects hypercoagulability. Also, the results can explain the reported fg binding to erythrocytes via ligands such as CD47, stable in vivo RBC aggregates in capillaries, and red areas of pathologic thrombi.


Assuntos
Fibrina , Trombofilia , Aceleração , Sedimentação Sanguínea , Eritrócitos , Humanos
4.
JAMA ; 327(2): 129-137, 2022 01 11.
Artigo em Inglês | MEDLINE | ID: mdl-35015038

RESUMO

Importance: Among patients younger than 21 years of age, the optimal duration of anticoagulant therapy for venous thromboembolism is unknown. Objective: To test the hypothesis that a 6-week duration of anticoagulant therapy for provoked venous thromboembolism is noninferior to a conventional 3-month therapy duration in patients younger than 21 years of age. Design, Setting, and Participants: Randomized clinical trial involving 417 patients younger than 21 years of age with acute, provoked venous thromboembolism enrolled at 42 centers in 5 countries from 2008-2021. The main exclusions were severe anticoagulant deficiencies or prior venous thromboembolism. Patients without persistent antiphospholipid antibodies and whose thrombi were resolved or not completely occlusive upon repeat imaging at 6 weeks after diagnosis underwent randomization. The final visit for the primary end points occurred in January 2021. Interventions: Total duration for anticoagulant therapy of 6 weeks (n = 207) vs 3 months (n = 210) for provoked venous thromboembolism. Main Outcomes and Measures: The primary efficacy and safety end points were centrally adjudicated symptomatic recurrent venous thromboembolism and clinically relevant bleeding events within 1 year blinded to treatment group. The primary analysis was noninferiority in the per-protocol population. The noninferiority boundary incorporated a bivariate trade-off that included an absolute increase of 0% in symptomatic recurrent venous thromboembolism with an absolute risk reduction of 4% in clinically relevant bleeding events (1 of 3 points on the bivariate noninferiority boundary curve). Results: Among 417 randomized patients, 297 (median age, 8.3 [range, 0.04-20.9] years; 49% female) met criteria for the primary per-protocol population analysis. The Kaplan-Meier estimate for the 1-year cumulative incidence of the primary efficacy outcome was 0.66% (95% CI, 0%-1.95%) in the 6-week anticoagulant therapy group and 0.70% (95% CI, 0%-2.07%) in the 3-month anticoagulant therapy group, and for the primary safety outcome, the incidence was 0.65% (95% CI, 0%-1.91%) and 0.70% (95% CI, 0%-2.06%). Based on absolute risk differences in recurrent venous thromboembolism and clinically relevant bleeding events between groups, noninferiority was demonstrated. Adverse events occurred in 26% of patients in the 6-week anticoagulant therapy group and in 32% of patients in the 3-month anticoagulant therapy group; the most common adverse event was fever (1.9% and 3.4%, respectively). Conclusions and Relevance: Among patients younger than 21 years of age with provoked venous thromboembolism, anticoagulant therapy for 6 weeks compared with 3 months met noninferiority criteria based on the trade-off between recurrent venous thromboembolism risk and bleeding risk. Trial Registration: ClinicalTrials.gov Identifier: NCT00687882.


Assuntos
Anticoagulantes/administração & dosagem , Hemorragia/induzido quimicamente , Tromboembolia Venosa/tratamento farmacológico , Adolescente , Fatores Etários , Anticoagulantes/efeitos adversos , Criança , Pré-Escolar , Esquema de Medicação , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Recidiva , Terapêutica , Fatores de Tempo , Tromboembolia Venosa/etiologia , Adulto Jovem
5.
Blood ; 143(7): 569-570, 2024 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-38358847
6.
Haemophilia ; 27 Suppl 1: 5-7, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33522655

RESUMO

Current treatments in the field of haemophilia are changing the phenotype of many patients with severe haemophilia to that of mild haemophilia. Despite this improvement, those with mild-to-moderate haemophilia A and haemophilia B continue to experience unmet needs. Whereas some patients with mild-to-moderate haemophilia experience similar complications to those of patients with severe haemophilia, they possess several unique attributes. These include a challenging diagnosis and variability in bleeding symptoms and treatment needs. In addition, haemophilia is an under-recognized condition in women even though many women with mild-to-moderate haemophilia experience the same symptoms and complications as men with haemophilia. These women also have their own unique challenges with this disease. This supplement highlights many of the unmet needs in men and women with mild-to-moderate haemophilia. The conclusions of each of these papers reinforce the need for additional research and resources for this patient population.


Assuntos
Hemofilia A , Hemofilia B , Feminino , Hemofilia A/complicações , Hemofilia B/complicações , Hemorragia/etiologia , Humanos , Masculino , Fenótipo
7.
Haemophilia ; 27(1): 149-155, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32856388

RESUMO

INTRODUCTION: Persons with haemophilia (PwH) have abnormally low bone density and increased risk of fractures. We previously demonstrated decreased skeletal health in factor VIII (FVIII)-deficient mice. Thus, we hypothesized factor deficiency is an independent risk factor for decreased skeletal health. AIM: We seek to identify differences in bone-related cytokine expression among PwH and healthy controls. METHODS: We evaluated plasma samples from 79 participants with severe FVIII deficiency and 51 age-matched healthy controls. Plasma samples were assessed for RANKL and OPG, cytokines that regulate bone metabolism, and CTX-1, a biomarker for bone resorption, as well as 10 bone-related cytokines. RESULTS: CTX-1 is higher among samples from FVIII-deficient participants compared to controls (P < .01) but not among participants with recent factor use (within 24 hours of sample collection) (P = .21). Among PwH greater than 16 years of age (PwH ≥ 16), OPG is increased with recent factor use (P < .01) but not without (P = .34). Lower levels of TNF-α (P < .01), interleukin (IL)-12 (P < .01) and IL-10 (P < .001) were found among samples from PwH. Controlling for subject age, IL-12 and IL-10 levels are lower in PwH ≥ 16 (P < .01, P < .001) but not PwH under 16 (PwH < 16) (P > .05). Levels of TNF-α were lower among PwH < 16 only (P < .05). These differences are not observed in participants with recent factor use. CONCLUSIONS: In PwH, markers of bone metabolism and circulating cytokine levels are abnormal. Recent factor use reverses many of these differences suggesting FVIII replacement ameliorates this pathology. This study suggests bone disease present in PwH is intrinsic to FVIII deficiency.


Assuntos
Doenças Ósseas , Hemofilia A , Adolescente , Biomarcadores , Osso e Ossos , Citocinas , Hemofilia A/complicações , Humanos
8.
Haemophilia ; 27 Suppl 3: 87-95, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33398908

RESUMO

Options for management of haemophilia are increasing rapidly with completely novel therapeutic approaches that cannot be compared using traditional factor assays. In addition, as prophylaxis regimens have improved, bleeding rates have decreased, and consequently, it is difficult to show an impact of novel therapies on rates of spontaneous bleeding. There is currently an urgent need for a panel of outcome measures to compare therapies that are dissimilar in many essential ways. Conventional objective outcome measures including joint physical examination and joint imaging continue to hold a central importance. Factor assays are essential for evaluation of products derived from native factor genes, but are not applicable to some extended half-life factors or non-factor bypassing agents. Global assays including thrombin generation and chromogenic assays of factor X activation are under investigation for their usefulness in haemophilia assessment. Bleeding rate is a conventional subjective patient-reported outcome that, while decreasing in frequency, is indispensable as an outcome given that the primary manifestation of haemophilia is bleeding. Other patient-reported outcomes such as pain intensity and interference, health-related quality of life and activities and participation are increasingly important to distinguish superior outcomes in comparative trials. This review of outcome measures for haemophilia presents examples of existing outcome measures with an emphasis on their strengths and limitations.


Assuntos
Hemofilia A , Fator VIII , Meia-Vida , Hemorragia/etiologia , Humanos , Avaliação de Resultados em Cuidados de Saúde , Qualidade de Vida
9.
Acta Haematol ; 144(6): 672-677, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33915533

RESUMO

INTRODUCTION: In response to the increasing complexity of care for patients with bleeding disorders, we established new clinical teams for our hemophilia treatment center (HTC). AIMS: We undertook a quality improvement project to improve the coordination and communication with our patients by establishing primary assignments of clinical staff to individual patients (primary teams). METHODS: A quality improvement project group was formed that established the goals and assignment of primary teams. Patients were surveyed for their knowledge of their primary teams as well as their ability to schedule and contact their primary providers. As a measure of the effects on clinical staff, a balancing survey was also conducted among providers impacted by the clinical assignment of teams. RESULTS: Our results demonstrate improvements across both coordination and communication as reported by patients. Additionally, the assignment of primary teams was met with high satisfaction and improvement in coordination and communication as reported by the clinical staff members of the HTC. CONCLUSIONS: Initiation of a quality improvement project and the creation of a primary team system were feasible at a large HTC and resulted in improvements in both patient-reported and staff-reported outcomes of coordination and communication of care.


Assuntos
Transtornos da Coagulação Sanguínea/psicologia , Melhoria de Qualidade , Adolescente , Adulto , Transtornos da Coagulação Sanguínea/diagnóstico , Humanos , Assistência Centrada no Paciente , Melhoria de Qualidade/organização & administração , Inquéritos e Questionários , Adulto Jovem
10.
J Pediatr ; 225: 198-206.e2, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32553867

RESUMO

OBJECTIVE: To evaluate the impact of early disseminated intravascular coagulation (DIC) on illness severity in children using a database of emergency department ED encounters for children with suspected sepsis, in view of similar associations in adults. STUDY DESIGN: Laboratory and clinical data were extracted from a registry of emergency department encounters of children with suspected sepsis between April 1, 2012, and June 26, 2017. International Society of Thrombosis and Hemostasis DIC scores were calculated from laboratory values obtained within 24 hours of emergency department admission. Univariate logistic regression, multivariable logistic regression, and Cox regression were used to assess the influence of DIC scores on vasopressor use (primary outcome), mortality, ventilator requirement, pediatric intensive care unit admission, and hospital duration (secondary outcomes). The optimal DIC score cutoff for outcome prediction was determined. RESULTS: Of 1653 eligible patients, 284 had DIC scores within 24 hours, including 92 who required vasopressors and 23 who died within 1 year. An initial DIC score of ≥3 was the most sensitive and specific DIC score for predicting adverse outcomes. Those with a DIC score of ≥3 vs <3 had increased odds of vasopressor use in both univariate (OR, 4.48; 95% CI, 2.63-7.62; P < .001) and multivariable (OR, 3.78; 95% CI, 1.82-7.85; P < .001) analyses. Additionally, those with a DIC score of ≥3 vs <3 had increased 1-year mortality with a hazard ratio of 3.55 (95% CI, 1.46-8.64; P = .005). CONCLUSIONS: A DIC score of ≥3 was an independent predictor for both vasopressor use and mortality in this pediatric cohort, distinct from the adult overt DIC score cutoff of ≥5.


Assuntos
Coagulação Intravascular Disseminada/diagnóstico , Coagulação Intravascular Disseminada/mortalidade , Sepse/diagnóstico , Sepse/mortalidade , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Coagulação Intravascular Disseminada/tratamento farmacológico , Coagulação Intravascular Disseminada/etiologia , Feminino , Humanos , Unidades de Terapia Intensiva Pediátrica , Masculino , Modelos de Riscos Proporcionais , Curva ROC , Sistema de Registros , Respiração Artificial/estatística & dados numéricos , Sepse/complicações , Vasoconstritores/uso terapêutico
11.
Haemophilia ; 26(6): 1072-1080, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33058441

RESUMO

INTRODUCTION: The Hemophilia Joint Health Score (HJHS) was developed to detect early changes in joint health in children and adolescents with haemophilia. The HJHS is considered by some to be too time consuming for clinical use and this may limit broad adoption. AIM: This study was a first step to develop a shorter and/or more convenient version of the HJHS for the measurement of joint function in children and young adults with haemophilia, by combining real-life data and expert opinion. METHODS: A cross-sectional multicenter secondary analysis on pooled data of published studies using the HJHS (0-124, optimum score 0) in persons with haemophilia A/B aged 4-30 was performed. Least informative items, scoring options and/or joints were identified. An expert group of 19 international multidisciplinary experts evaluated the results and voted on suggestions for adaptations in a structured meeting (consensus set at ≥ 80%). RESULTS: Original data on 499 persons with haemophilia from 7 studies were evaluated. Median age was 15.0 years [range 4.0-29.9], 83.2% had severe haemophilia and 61.5% received prophylaxis. Median (IQR) HJHS total was 6.0 (1.0-17.0). The items 'duration swelling' and 'crepitus' were identified as clinically less informative and appointed as candidates for reduction. CONCLUSION: Analysis of 499 children and young adults with haemophilia showed that the HJHS is able to discriminate between children and adults and different treatment regimens. Reduction of the items 'duration swelling' and 'crepitus' resulted in the HJHSshort , which had the same discriminative ability. Additional steps are needed to achieve a substantially shorter HJHS assessment.


Assuntos
Hemofilia A/complicações , Articulações/fisiopatologia , Adolescente , Criança , Estudos Transversais , Feminino , Humanos , Masculino
12.
N Engl J Med ; 374(21): 2054-64, 2016 May 26.
Artigo em Inglês | MEDLINE | ID: mdl-27223147

RESUMO

BACKGROUND: The development of neutralizing anti-factor VIII alloantibodies (inhibitors) in patients with severe hemophilia A may depend on the concentrate used for replacement therapy. METHODS: We conducted a randomized trial to assess the incidence of factor VIII inhibitors among patients treated with plasma-derived factor VIII containing von Willebrand factor or recombinant factor VIII. Patients who met the eligibility criteria (male sex, age <6 years, severe hemophilia A, and no previous treatment with any factor VIII concentrate or only minimal treatment with blood components) were included from 42 sites. RESULTS: Of 303 patients screened, 264 underwent randomization and 251 were analyzed. Inhibitors developed in 76 patients, 50 of whom had high-titer inhibitors (≥5 Bethesda units). Inhibitors developed in 29 of the 125 patients treated with plasma-derived factor VIII (20 patients had high-titer inhibitors) and in 47 of the 126 patients treated with recombinant factor VIII (30 patients had high-titer inhibitors). The cumulative incidence of all inhibitors was 26.8% (95% confidence interval [CI], 18.4 to 35.2) with plasma-derived factor VIII and 44.5% (95% CI, 34.7 to 54.3) with recombinant factor VIII; the cumulative incidence of high-titer inhibitors was 18.6% (95% CI, 11.2 to 26.0) and 28.4% (95% CI, 19.6 to 37.2), respectively. In Cox regression models for the primary end point of all inhibitors, recombinant factor VIII was associated with an 87% higher incidence than plasma-derived factor VIII (hazard ratio, 1.87; 95% CI, 1.17 to 2.96). This association did not change in multivariable analysis. For high-titer inhibitors, the hazard ratio was 1.69 (95% CI, 0.96 to 2.98). When the analysis was restricted to recombinant factor VIII products other than second-generation full-length recombinant factor VIII, effect estimates remained similar for all inhibitors (hazard ratio, 1.98; 95% CI, 0.99 to 3.97) and high-titer inhibitors (hazard ratio, 2.59; 95% CI, 1.11 to 6.00). CONCLUSIONS: Patients treated with plasma-derived factor VIII containing von Willebrand factor had a lower incidence of inhibitors than those treated with recombinant factor VIII. (Funded by the Angelo Bianchi Bonomi Foundation and others; ClinicalTrials.gov number, NCT01064284; EudraCT number, 2009-011186-88.).


Assuntos
Anticorpos Neutralizantes/sangue , Fator VIII/imunologia , Hemofilia A/tratamento farmacológico , Isoanticorpos/análise , Fator de von Willebrand/uso terapêutico , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Fator VIII/antagonistas & inibidores , Fator VIII/uso terapêutico , Hemofilia A/complicações , Hemofilia A/imunologia , Hemorragia/etiologia , Humanos , Incidência , Lactente , Injeções Subcutâneas/efeitos adversos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Adulto Jovem
13.
Blood ; 129(17): 2368-2374, 2017 04 27.
Artigo em Inglês | MEDLINE | ID: mdl-28183693

RESUMO

This analysis of the US Hemophilia Treatment Center Network and the Centers for Disease Control and Prevention surveillance registry assessed trends in prophylaxis use and its impact on key indicators of arthropathy across the life-span among participants with severe hemophilia A. Data on demographics, clinical characteristics, and outcomes were collected prospectively between 1999 and 2010 at annual clinical visits to 134 hemophilia treatment centers. Trends in treatment and outcomes were evaluated using cross-sectional and longitudinal analyses. Data analyzed included 26 614 visits for 6196 males; mean age at first registry visit was 17.7 years; and median was 14 (range, 2 to 69). During this time, prophylaxis use increased from 31% to 59% overall, and by 2010, 75% of children and youths <20 years were on prophylaxis. On cross-sectional analysis, bleeding rates decreased dramatically for the entire population (P < .001) in parallel with increased prophylaxis usage, possibly because frequent bleeders adopted prophylaxis. Joint bleeding decreased proportionately with prophylaxis (22%) and nonprophylaxis (23%), and target joints decreased more with prophylaxis (80% vs 61%). Joint, total, and target joint bleeding on prophylaxis were 33%, 41%, and 27%, respectively, compared with nonprophylaxis. On longitudinal analysis of individuals over time, prophylaxis predicted decreased bleeding at any age (P < .001), but only prophylaxis initiation prior to age 4 years and nonobesity predicted preservation of joint motion (P < .001 for each). Using a national registry, care providers in a specialized health care network for a rare disorder were able to detect and track trends in outcomes over time.


Assuntos
Fator VIII/uso terapêutico , Hemartrose/prevenção & controle , Hemofilia A/tratamento farmacológico , Hemorragia/prevenção & controle , Prevenção Primária/estatística & dados numéricos , Sistema de Registros , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Pré-Escolar , Estudos Transversais , Hemartrose/diagnóstico , Hemartrose/fisiopatologia , Hemofilia A/diagnóstico , Hemofilia A/fisiopatologia , Hemorragia/diagnóstico , Hemorragia/fisiopatologia , Humanos , Articulações/irrigação sanguínea , Articulações/efeitos dos fármacos , Articulações/fisiopatologia , Masculino , Pessoa de Meia-Idade , Visita a Consultório Médico/estatística & dados numéricos , Estudos Prospectivos , Amplitude de Movimento Articular/efeitos dos fármacos , Estados Unidos
14.
Haemophilia ; 25(5): 867-875, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31115111

RESUMO

INTRODUCTION: The Joint Outcome Study (JOS) demonstrated that previously untreated children with severe haemophilia A treated with prophylactic factor VIII (FVIII) concentrate had superior joint outcomes at age 6 years compared to those children treated episodically for bleeding. However, variation in joint outcome within each treatment arm was not well explained. AIM: In this study, we sought to better understand variation in joint outcomes at age 6 years in participants of the JOS. METHODS: We evaluated the influence of FVIII half-life, treatment adherence, constitutional coagulant and anticoagulant proteins, and global assays on joint outcomes (number of joint bleeds, total number of bleeds, total MRI score and joint physical exam score). Logistic regression was used to evaluate the association of variables with joint failure status on MRI, defined as presence of subchondral cyst, surface erosion or joint-space narrowing. Each parameter was also correlated with each joint outcome using Spearman correlations. RESULTS: Prophylaxis treatment arm and FVIII trough were each found to reduce risk of joint failure on univariate logistic regression analysis. When controlling for treatment arm, FVIII trough was no longer significant, likely because of the high level of covariation between these variables. We found no consistent correlation between any laboratory assay performed and any joint outcome parameter measured. CONCLUSION: In the JOS, the effect of prescribed prophylactic FVIII infusions on joint outcome overshadowed the contribution of treatment adherence, FVIII half-life, global assays of coagulation and constitutional coagulation proteins. (ClinicalTrials.gov number, NCT00207597).


Assuntos
Fator VIII/uso terapêutico , Hemofilia A/complicações , Hemofilia A/tratamento farmacológico , Artropatias/etiologia , Fator VIII/farmacologia , Feminino , Hemofilia A/patologia , Hemostasia , Humanos , Masculino
15.
BMC Cardiovasc Disord ; 19(1): 99, 2019 04 29.
Artigo em Inglês | MEDLINE | ID: mdl-31035921

RESUMO

BACKGROUND: The cornerstone of effective management in heart failure (HF) is the ability to self-care. Aims include i) To determine factors influencing self-care in HF patients with cognitive impairment (CI) and ii) to determine the influence of cognitive domains on self-care in patients with HF and CI. METHODS: MEDLINE, CINAHL, EMBASE, EBSCOHost, PsychINFO, ProQuest Research Library, Health Technology Assessment Database, The Cochrane Library, Web of Science and Scopus databases were systematically searched. Original research describing the relationship between cognition and HF self-care in community-dwelling older persons with dementia/CI in English, published in a peer-reviewed journal from 1stJanuary(2000)-22ndMarch(2016) was identified. Study and population characteristics, data sources, self-care processes, methods of cognitive assessment, cognitive domains affected, study outcomes, impact of impairment, and other risk factors of self-care impairment were abstracted by two reviewers. RESULTS: Of 10,688 studies identified, 14 met the inclusion criteria. Patients with HF and CI ranged from 14 to 73%. Where reported, self-care maintenance adequacy ranged from 50 to 61%; self-care management adequacy ranged from 14 to 36% and self-care confidence adequacy ranged from 0 to 44% on the Self-care of Heart Failure Index (SCHFI). All but one study predicted poor self-care ability according to poor outcome on cognitive testing. Additionally, specific cognitive domain deficits impaired self-care. Subjects with lower cognitive scores were less likely to seek assistance while subjects with depression had poor self-care abilities. CONCLUSIONS: Clinicians must consider the type and severity of impairments in cognitive domains to tailor management. Awareness of depression, self-confidence and support access may modulate self-care ability.


Assuntos
Disfunção Cognitiva/psicologia , Demência/psicologia , Comportamentos Relacionados com a Saúde , Conhecimentos, Atitudes e Prática em Saúde , Insuficiência Cardíaca/terapia , Autocuidado/psicologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Disfunção Cognitiva/diagnóstico , Demência/diagnóstico , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/psicologia , Humanos , Masculino , Saúde Mental , Pessoa de Meia-Idade , Prognóstico , Fatores de Risco
16.
Blood ; 127(20): 2481-8, 2016 05 19.
Artigo em Inglês | MEDLINE | ID: mdl-26862110

RESUMO

von Willebrand disease (VWD) is the most common inherited bleeding disorder, and type 1 VWD is the most common VWD variant. Despite its frequency, diagnosis of type 1 VWD remains the subject of debate. In order to study the spectrum of type 1 VWD in the United States, the Zimmerman Program enrolled 482 subjects with a previous diagnosis of type 1 VWD without stringent laboratory diagnostic criteria. von Willebrand factor (VWF) laboratory testing and full-length VWF gene sequencing was performed for all index cases and healthy control subjects in a central laboratory. Bleeding phenotype was characterized using the International Society on Thrombosis and Haemostasis bleeding assessment tool. At study entry, 64% of subjects had VWF antigen (VWF:Ag) or VWF ristocetin cofactor activity below the lower limit of normal, whereas 36% had normal VWF levels. VWF sequence variations were most frequent in subjects with VWF:Ag <30 IU/dL (82%), whereas subjects with type 1 VWD and VWF:Ag ≥30 IU/dL had an intermediate frequency of variants (44%). Subjects whose VWF testing was normal at study entry had a similar rate of sequence variations as the healthy controls (14%). All subjects with severe type 1 VWD and VWF:Ag ≤5 IU/dL had an abnormal bleeding score (BS), but otherwise BS did not correlate with VWF:Ag. Subjects with a historical diagnosis of type 1 VWD had similar rates of abnormal BS compared with subjects with low VWF levels at study entry. Type 1 VWD in the United States is highly variable, and bleeding symptoms are frequent in this population.


Assuntos
Doença de von Willebrand Tipo 1/sangue , Adolescente , Testes de Coagulação Sanguínea , Hibridização Genômica Comparativa , Feminino , Variação Genética , Hemorragia/etiologia , Humanos , Masculino , Fenótipo , Análise de Sequência de DNA , Inquéritos e Questionários , Estados Unidos/epidemiologia , Adulto Jovem , Doença de von Willebrand Tipo 1/diagnóstico , Doença de von Willebrand Tipo 1/epidemiologia , Fator de von Willebrand/análise , Fator de von Willebrand/genética
17.
Diabetes Metab Res Rev ; 34(6): e3013, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29707902

RESUMO

Self-management is integral to effective chronic disease management. Cognitive impairments (CogImp) associated with dementia have not previously been reviewed in diabetes mellitus (DM) self-care. The aims of this study are to know (1) whether CogImp associated with dementia impact self-care and (2) whether specific CogImp affects key DM self-care processes. A systematic literature search with a narrative review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This review examined studies published from January, 2000 to February, 2016 describing the relationship between cognition and DM self-care domains in community dwelling older adults with dementia/CogImp. Eight studies met inclusion criteria. Decrements in all self-care domains were associated with CogImp. Problem solving was related to reduced disease knowledge (OR 0.87, 95% CI = 0.49-1.55), resulting in poorer glycemic control. Decision-making impairments manifested as difficulties in adjusting insulin doses, leading to more hospital admissions. People without CogImp were better able to find/utilize resources by adhering to recommended management (OR 1.03, 95% CI = 1.02-1.05). A lack of interaction with health care providers was demonstrated through reduced receipt of important routine investigation including eye examinations (ARR = 0.85, 95% CI = 0.85-0.86), HbA1c testing (ARR = 0.96, 95% CI = 0.96-0.97), and LDL-C testing (ARR = 0.91, 95% CI = 0.901-0.914). People without CogImp had better clinic attendance (OR 2.17, 95% CI = 1.30-3.70). Action taking deficits were apparent through less self-testing of blood sugar levels (20.2% vs 24.4%, P = 0.1) resulting in poorer glycemic control, self-care, and more frequent micro/macrovascular complications. Persons with diabetes and CogImp, particularly in domains of learning, memory and executive function, were significantly impaired in all self-care tasks.


Assuntos
Disfunção Cognitiva/terapia , Demência/terapia , Diabetes Mellitus/psicologia , Diabetes Mellitus/terapia , Autocuidado/métodos , Disfunção Cognitiva/complicações , Demência/complicações , Complicações do Diabetes/psicologia , Complicações do Diabetes/terapia , Humanos , Autocuidado/psicologia , Autocuidado/normas
18.
Int J Geriatr Psychiatry ; 33(8): 1082-1089, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29804299

RESUMO

OBJECTIVES: To examine deaths of Australian nursing home (NH) residents following an unexplained absence. METHODS: Population based cross-sectional study was conducted using coronial data from the National Coronial Information System. Participants are residents of accredited NHs if death followed an unexplained absence and was reported to the Coroner between July 1, 2000 and June 30, 2013. Individual, organisational, environmental, and unexplained absence event factors were extracted from coronial records. Data were analysed using descriptive statistics. RESULTS: Of 21 672 NH deaths, 24 (0.1%) followed an unexplained absence. This comprised 17 unintentional external (injury-related) causes and 7 natural cause deaths. Drowning was the most frequent external cause of death (59%, n = 10). Deaths occurred more frequently in males (83.3%, n = 20), and in the age group 85-94 years (37.5%, n = 9). The majority of NH residents, for whom data were available (n = 15), had a diagnosis of dementia (86.7%, n = 13). Most residents were found in waterways (41.7%, n = 10). Median distance travelled was 0.5 km (IQR: 0.25-2.4 km), with almost 70% of residents found within 1.0 km of their NH. Most residents left the NH by foot (88.2%, n = 15). Half of the residents were found within 6 hours of time last seen (median: 6 hours, 40 minutes; IQR: 6.0-11.45 hours). CONCLUSION: Unexplained absences in elderly NH residents are a relatively common event. This study provides valuable information for aged care providers, governments, and search and rescue teams, and should contribute to debates about balancing issues of safety with independence.


Assuntos
Casas de Saúde/estatística & dados numéricos , Comportamento Errante/estatística & dados numéricos , Idoso , Idoso de 80 Anos ou mais , Austrália/epidemiologia , Causas de Morte , Estudos Transversais , Demência/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mortalidade , Estudos Retrospectivos
19.
Br J Haematol ; 179(2): 298-307, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28699675

RESUMO

The discussion of prophylactic therapy in haemophilia is largely focused on joint outcomes. The impact of prophylactic therapy on intracranial haemorrhage (ICH) is less known. This study aimed to analyse ICH in children with haemophilia, with a focus on different prophylaxis regimens and sequelae of ICH. We conducted a multicentre retrospective and prospective study that included 33 haemophilia centres from 20 countries. Inclusion criteria were children and adolescents born between 1993 and 2014, with severe haemophilia A or B without inhibitors. Participants were categorized by prophylaxis regimen: full, partial or none, based on dose and dose frequency of regular infusions. The cohort study included 1515 children: 29 cases of ICH over 8038 patient years were reported. The incidence of ICH in the prophylaxis group, 0·00033 cases of ICH/patient year, was significantly lower compared to the no prophylaxis group, 0·017 cases of ICH/patient year (RR 50·06; P < 0·001) and the partial prophylaxis group, 0·0050 cases of ICH/patient year (RR 14·92; P = 0·007). In the on-demand-group, 8% (2/24) children with ICH died and 33% had long-term sequelae, including intellectual and behavioural problems, paresis and epilepsy. Children on regular, frequent prophylaxis have a low risk of ICH compared to those using non-frequent or no prophylaxis.


Assuntos
Hemofilia A , Hemofilia B , Hemorragias Intracranianas , Índice de Gravidade de Doença , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Hemofilia A/complicações , Hemofilia A/mortalidade , Hemofilia A/terapia , Hemofilia B/complicações , Hemofilia B/mortalidade , Hemofilia B/terapia , Humanos , Lactente , Hemorragias Intracranianas/etiologia , Hemorragias Intracranianas/mortalidade , Hemorragias Intracranianas/prevenção & controle , Masculino , Estudos Prospectivos , Estudos Retrospectivos
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