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BACKGROUND: Disease-specific therapy aims to improve symptoms, stabilise current disease and delay progression in patients with Fabry disease. In Australia, treatment access is subject to eligibility criteria initially established in 2004. Patients and their clinicians question why these criteria have remained unchanged despite significant progress in disease understanding. AIMS: Appraise the clinical quality of the Australian treatment access criteria. METHODS: The Fabry Australia Medical Advisory Committee (N = 6) used the Appraisal of Guidelines for REsearch and Evaluation Global Rating Scale (AGREE II GRS) to assess the clinical quality of the current treatment eligibility criteria. They reviewed the literature, developed 17 clinical statements to help guide reforms of the eligibility criteria and achieved consensus (achievement of ≥75% agreement in the range 5-7 on a 7-point Likert scale) through anonymous voting. The findings were applied to develop proposals for revised classification and treatment initiation criteria. RESULTS: The current treatment eligibility criteria underperformed on the AGREE II GRS. They are pragmatic but out-of-step with contemporary data. Consensus was achieved on all 17 proposed clinical statements. There was strong agreement to differentiate classical male Fabry patients to facilitate timelier access to Fabry-specific treatment. There was also agreement on the value of adopting relevant organ involvement criteria in classical female patients and patients with non-classical disease. CONCLUSIONS: Australian access criteria are misaligned with current clinical evidence. The clinical statements and proposed classification and initiation criteria should prompt discussions to support more equitable access to treatment and better align Australian practice with contemporary evidence and international guidelines.
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Enfermedad de Fabry , Enfermedad de Fabry/terapia , Humanos , Australia , Masculino , Femenino , Guías de Práctica Clínica como Asunto/normas , Selección de Paciente , Determinación de la Elegibilidad/métodos , Terapia de Reemplazo Enzimático , ConsensoRESUMEN
BACKGROUND: Prevalence of Fabry disease amongst Chronic Kidney Disease (CKD) patients on haemodialysis has been shown to be approximately 0.2%. METHODS: We undertook a cross-sectional study employing a cascade screening strategy for Fabry Disease amongst 3000 adult, male and female patients affected by CKD stage 1-5D/T at public, specialty renal practices within participating Queensland Hospital and Health Services from October 2017 to August 2019. A multi-tiered FD screening strategy, utilising a combination of dried blood spot (DBS) enzymatic testing, and if low, then lyso-GB3 testing and DNA sequencing, was used. RESULTS: Mean (SD) age was 64.0 (15.8) years (n = 2992), and 57.9% were male. Eight participants withrew out of the 3000 who consented. Of 2992 screened, 6 (0.20%) received a diagnosis of FD, 2902 (96.99%) did not have FD, and 84 (2.81%) received inconclusive results. Of the patients diagnosed with FD, mean age was 48.5 years; 5 were male (0.29%) and 1 was female (0.08%); 4 were on kidney replacement therapy (2 dialysis and 2 transplant); 3 were new diagnoses. CONCLUSIONS: Estimated overall FD prevalence was 0.20%. Screening of the broader CKD population may be beneficial in identifying cases of FD. TRIAL REGISTRATION: The aCQuiRE Study has been prospectively registered with the Queensland Health Database of Research Activity (DORA, https://dora.health.qld.gov.au ) as pj09946 (Registered 3rd July 2017).
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Enfermedad de Fabry , Insuficiencia Renal Crónica , Adulto , Estudios Transversales , Enfermedad de Fabry/diagnóstico , Enfermedad de Fabry/epidemiología , Enfermedad de Fabry/genética , Femenino , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Diálisis Renal , Insuficiencia Renal Crónica/epidemiología , Insuficiencia Renal Crónica/terapiaRESUMEN
We report a case of a 67-year-old male recipient of a second renal allograft, presenting with a 9-month history of progressive cognitive and physical decline with features of Parkinsonism. He was HIV-negative. Serum and cerebrospinal fluid (CSF) cryptococcal antigen was positive though CSF culture was sterile. He had progressive deterioration despite induction and consolidation antifungal treatment. Postmortem brain examination confirmed a large burden of yeast forms in the substantia nigra with widespread chronic meningitis. The significant delay in presentation and diagnosis owing to the atypical, subacute neurocognitive features serves as a timely reminder of the variety of neurological presentations that may be associated with cryptococcal infection in solid organ transplant recipients.
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Disfunción Cognitiva , Criptococosis , Cryptococcus neoformans , Trasplante de Riñón , Meningitis Criptocócica , Trastornos Parkinsonianos , Anciano , Disfunción Cognitiva/diagnóstico , Disfunción Cognitiva/etiología , Fluconazol , Humanos , Trasplante de Riñón/efectos adversos , Masculino , Meningitis Criptocócica/diagnóstico , Meningitis Criptocócica/tratamiento farmacológico , Trastornos Parkinsonianos/etiologíaRESUMEN
Judicious use of medicines that considers evidence-based practice, together with cost-effectiveness, is a priority for all health care organisations. We describe an initiative to lead a Medication Utilisation Program, incorporating medication quality improvement and research activities. In August 2020 an advanced pharmacist position was implemented to lead the Program. The purpose was to provide oversight and facilitate initiatives promoting medication optimisation to create sustainable change in practice. A strategic plan was developed with key performance indicators. A governance structure was implemented with relevant reporting mechanisms. Strategic planning and collaboration with medical, nursing and allied health professionals has seen the successful implementation of seven codesigned medication-use evaluations and eight quality improvement projects centred around patient safety, quality and value-based care. Several research studies have been designed with subsequent commencement of pharmacists enrolled in university Research Higher Degree programs. Cost containment initiatives have realised potential savings approximating AUD 250,000. Educational programs included protocol design, ethics approvals and report writing. Key success criteria for a Medication Utilisation Program include dedicated pharmacist resources, structured governance and reporting mechanisms. Alignment of study complexity with staff experience and interdisciplinary collaboration are also critical.
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BACKGROUND: Cardiac involvement in Anderson-Fabry disease (AFD) is associated with increased left ventricular (LV) wall thickness. The aim of this study was to evaluate if two-dimensional global and regional strain in patients with AFD can identify early myocardial involvement (when LV wall thickness and function are normal). Additionally, the association of altered strain with adverse cardiovascular events was evaluated. METHODS: In a retrospective cross-sectional study, 43 patients with AFD, before enzyme replacement therapy (mean age, 44 ± 12 years; 58.1% men), were compared with age- and gender-matched healthy control subjects. The mean follow-up duration among patients with AFD for major adverse cardiovascular events (MACE) was 82 months. RESULTS: LV ejection fraction was similar between groups (patients with AFD vs control subjects, 61 ± 8% vs 61 ± 6%; P = .89). However, global longitudinal strain (LS) was impaired in patients with AFD compared with control subjects (-16.5 ± 3.8% vs -20.2 ± 1.7%, P < .001), with greater impairment in patients with AFD with increased LV wall thickness (-15.4 ± 3.9% vs -18.7 ± 2.3%, P < .006). Additionally, LS was most impaired in the basal segments in patients with AFD (-14.8 ± 3.7% vs -20.3 ± 1.1%, P < .001). MACE occurred in 19 of 43 patients (four women, 15 men), and Kaplan-Meier analysis demonstrated that MACE were associated with impaired basal LS. CONCLUSIONS: In patients with AFD, altered basal LS is present even in those with normal LV wall thickness and is associated with MACE. Therefore, basal LS should be considered when screening for cardiac involvement in AFD, particularly in female patients with AFD with normal LV wall thickness.
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Enfermedad de Fabry , Disfunción Ventricular Izquierda , Adulto , Estudios Transversales , Enfermedad de Fabry/complicaciones , Enfermedad de Fabry/diagnóstico , Femenino , Humanos , Masculino , Miocardio , Estudios Retrospectivos , Función Ventricular IzquierdaRESUMEN
BACKGROUND: Fabry disease (FD) is a rare, lysosomal storage disorder caused by the absence or deficiency of the enzyme alpha-galactosidase A (α-Gal A) that leads to the abnormal accumulation of the lipid globotriaosylceramide (GB3) in a variety of cell types and tissues throughout the body. FD has an x-linked inheritance pattern. Previously thought to be only carriers, females can also experience FD symptomatology. Symptoms vary in type and severity from patient to patient and tend to increase in severity with age. FD symptoms are non-specific and may be shared with those of other diseases. Misdiagnoses and diagnostic delays are common, often resulting in progressive, irreversible tissue damage. The estimated prevalence of FD in the general population is 1:40,000 to 1:117,000 individuals. However, it is estimated that the prevalence of FD in the dialysis population is 0.12 to 0.7%. Little is known about the prevalence of FD in the broader Chronic Kidney Disease (CKD) population. METHODS: This is an epidemiological study of the prevalence of FD in CKD patents identified from the public renal speciality practices in Queensland, Australia. A cascade approach to screening is being employed with dried blood spot testing for blood levels of alpha-galactosidase A (Alpha-Gal), with follow-up testing for patients with abnormal results by plasma levels of globotriaosylsphingosine (Lyso-GB3) for females and non-definitive cases in males. A diagnosis of FD is confirmed through genetic testing of the GLA gene in cases suspected of having FD based upon Alpha-Gal and Lyso-GB3 testing. DISCUSSION: Expected outcomes of this study include more information about the prevalence of FD at all stages of CKD, including for both males and females. The study may also provide information about common characteristics of FD to assist with diagnosis and optimal management/treatment. Screening is also available for family members of diagnosed patients, with potential for early diagnosis of FD and intervention for those individuals. TRIAL REGISTRATION: Queensland Health Database of Research Activity (DORA, https://dora.health.qld.gov.au) pj09946 (Registered 3rd July 2017).
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Enfermedad de Fabry/epidemiología , Insuficiencia Renal Crónica/etiología , Adulto , Protocolos Clínicos , Diagnóstico Tardío , Enfermedad de Fabry/complicaciones , Enfermedad de Fabry/diagnóstico , Enfermedad de Fabry/genética , Femenino , Glucolípidos/sangre , Humanos , Masculino , Prevalencia , Estudios Prospectivos , Queensland/epidemiología , Diálisis Renal , Insuficiencia Renal Crónica/genética , Esfingolípidos/sangre , alfa-Galactosidasa/sangreRESUMEN
OBJECTIVES: This study sought to measure the impact on all-cause death or readmission of adding center-based exercise training (ET) to disease management programs for patients with a recent acute heart failure (HF) hospitalization. BACKGROUND: ET is recommended for patients with HF, but evidence is based mainly on ET as a single intervention in stable outpatients. METHODS: A randomized, controlled trial with blinded outcome assessor, enrolling adult participants with HF discharged from 5 hospitals in Queensland, Australia. All participants received HF-disease management program plus supported home exercise program; intervention participants were offered 24 weeks of supervised center-based ET. Primary outcome was all-cause 12-month death or readmission. Pre-planned subgroups included age (<70 years vs. older), sex, left ventricular ejection fraction (≤40% vs. >40%), and exercise adherence. RESULTS: Between May 2008 and July 2013, 278 participants (140 intervention, 138 control) were enrolled: 98 (35.3%) age ≥70 years, 71 (25.5%) females, and 62 (23.3%) with a left ventricular ejection fraction of >40%. There were no adverse events associated with ET. There was no difference in primary outcome between groups (84 of 140 [60.0%] intervention vs. 90 of 138 [65.2%] control; p = 0.37), but a trend toward greater benefit in participants age <70 years (OR: 0.56 [95% CI: 0.30 to 1.02] vs. OR: 1.56 [95% CI: 0.67 to 3.64]; p for interaction = 0.05). Participants who exercised to guidelines (72 of 101 control and 92 of 117 intervention at 3 months) had a significantly lower rate of death and readmission (91 of 164 [55.5%] vs. 41 of 54 [75.9%]; p = 0.008). CONCLUSIONS: Supervised center-based ET was a safe, feasible addition to disease management programs with supported home exercise in patients recently hospitalized with acute HF, but did not reduce combined end-point of death or readmission. (A supervised exercise programme following hospitalisation for heart failure: does it add to disease management?; ACTRN12608000263392).
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Manejo de la Enfermedad , Terapia por Ejercicio/métodos , Tolerancia al Ejercicio/fisiología , Insuficiencia Cardíaca/rehabilitación , Hospitalización , Pacientes Internos , Volumen Sistólico/fisiología , Anciano , Causas de Muerte/tendencias , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Calidad de Vida , Queensland/epidemiología , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Resultado del Tratamiento , Función Ventricular IzquierdaRESUMEN
BACKGROUND: To improve up-titration of medications to target dose in heart failure patients by improving communication from hospital to primary care. METHODS: This quality improvement project was undertaken within three heart failure disease management (HFDM) services in Queensland, Australia. A structured medication plan was collaboratively designed and implemented in an iterative manner, using methods including awareness raising and education, audit and feedback, integration into existing work practice, and incentive payments. Evaluation was undertaken using sequential audits, and included process measures (use of the titration plan, assignment of responsibility) and outcome measures (proportion of patients achieving target dose) in HFDM service patients with reduced left ventricular ejection fraction. RESULTS: Comparison of the three patient cohorts (pre-intervention cohort A n=96, intervention cohort B n=95, intervention cohort C n=89) showed increase use of the titration plan, a shift to greater primary care responsibility for titration, and an increase in the proportion of patients achieving target doses of angiotensin converting enzyme inhibitors/angiotensin receptor blockers (ACEI/ARB) (A 37% vs B 48% vs C 55%, p=0.051) and beta-blockers (A 38% vs B 33% vs C 51%, p=0.045). Combining all three cohorts, patients not on target doses when discharged from hospital were more likely to achieve target doses of ACEI/ARB (p<0.0001) and beta blockers (p<0.0001) within six months if they received a medication titration plan. CONCLUSIONS: A medication titration plan was successfully implemented in three HFDM services and improved transitional communication and achievement of target doses of evidence-based therapies within six months of hospital discharge.
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Antagonistas de Receptores de Angiotensina/administración & dosificación , Inhibidores de la Enzima Convertidora de Angiotensina/administración & dosificación , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/epidemiología , Administración del Tratamiento Farmacológico/normas , Alta del Paciente/normas , Anciano , Anciano de 80 o más Años , Australia/epidemiología , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Heart failure (HF) medications improve clinical outcomes, with optimal doses defined in clinical trials. Patient, provider and system barriers may limit achievement of optimal doses in real life settings, although disease management programs (HF-DMPs) can facilitate up-titration. METHODS AND RESULTS: Secondary analysis of a prospective cohort of 216 participants recently hospitalized with systolic HF, attending 5 HF-DMPs in Queensland, Australia. Medication history at baseline (6weeks after discharge) and 6months provided data to describe prescription rates, dosage and optimal titration of HF medications, and associations with patient and system factors were explored. At baseline, 94% were on an angiotensin converting enzyme inhibitor/angiotensin II receptor blocker (ACEI/ARB), 94% on a beta-blocker (BB) and 42% on a mineralocorticoid receptor antagonist (MRA). The proportion of participants on optimal doses of ACEI/ARB increased from 38% (baseline) to 52% (6months, p=0.001) and on optimal BB dose from 23% to 49% (p<0.001). Significant barriers to ACEI/ARB up-titration were body mass index (BMI)<25, female gender, polypharmacy, previously diagnosed HF, and tertiary hospital. Significant barriers for BB up-titration were BMI<25, previously diagnosed HF and non-cardiologist care. CONCLUSIONS: Effective up-titration in HF DMPs is influenced by patient, disease and service factors. Better understanding of barriers to effective up-titration in women, normal weight, and established HF patients may help provide targeted strategies for improving outcomes in these groups.
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Antagonistas Adrenérgicos beta/uso terapéutico , Antagonistas de Receptores de Angiotensina/uso terapéutico , Antagonistas de Receptores de Mineralocorticoides/uso terapéutico , Adulto , Anciano , Anciano de 80 o más Años , Cálculo de Dosificación de Drogas , Prescripciones de Medicamentos/estadística & datos numéricos , Femenino , Adhesión a Directriz , Insuficiencia Cardíaca/tratamiento farmacológico , Hospitalización , Humanos , Masculino , Persona de Mediana Edad , Guías de Práctica Clínica como Asunto , Estudios Prospectivos , QueenslandRESUMEN
OBJECTIVE: Cardiac magnetic resonance (CMR) has the potential to provide early detection of cardiac involvement in Fabry disease. We aimed to gain further insight into this by assessing a cohort of Fabry patients using CMR. METHODS/RESULTS: Fifty genotype-positive Fabry subjects (age 45±2â years; 50% male) referred for CMR and 39 matched controls (age 40±2â years; 59% male) were recruited. Patients had a mean Mainz severity score index of 15±2 (range 0-46), reflecting an overall mild degree of disease severity. Compared with controls, Fabry subjects had a 34% greater left ventricular mass (LVM) index (82±5 vs 61±2â g/m(2), p=0.001) and had a significantly greater papillary muscle contribution to total LVM (13±1 vs 6±0.5%, p<0.001), even in the absence of left ventricular hypertrophy (LVH). Late gadolinium enhancement (LGE) was present in 15 Fabry subjects (9/21 males and 6/23 females). The most common site for LGE was the basal inferolateral wall (93%, 14/15). There was a positive association between LVM index and LGE. Despite this, there were two males and three females with no LVH that displayed LGE. Of Fabry subjects who were not on enzyme replacement therapy at enrolment (n=28), six were reclassified as having cardiac involvement (four LVH-negative/LGE-positive, one LVH-positive/LGE-positive and one LVH-positive/LGE-negative). CONCLUSIONS: CMR was able to detect cardiac involvement in 48% of this Fabry cohort, despite the overall mild disease phenotype of the cohort. Of those not on ERT, 21% were reclassified as having cardiac involvement allowing improved risk stratification and targeting of therapy.
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Cardiomiopatías/diagnóstico por imagen , Enfermedad de Fabry/diagnóstico por imagen , Imagen por Resonancia Cinemagnética , Adulto , Cardiomiopatías/tratamiento farmacológico , Cardiomiopatías/genética , Diagnóstico Precoz , Terapia de Reemplazo Enzimático , Enfermedad de Fabry/tratamiento farmacológico , Enfermedad de Fabry/genética , Femenino , Predisposición Genética a la Enfermedad , Humanos , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/genética , Masculino , Persona de Mediana Edad , Nueva Gales del Sur , Fenotipo , Valor Predictivo de las Pruebas , Queensland , Medición de Riesgo , Factores de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
OBJECTIVE: National guidelines for management of intermediate risk patients with suspected acute coronary syndrome, in whom AMI has been excluded, advocate provocative testing to final risk stratify these patients into low risk (negative testing) or high risk (positive testing suggestive of unstable angina). Adults less than 40 years have a low pretest probability of acute coronary syndrome. The utility of exercise stress testing in young adults with chest pain suspected of acute coronary syndrome who have National Heart Foundation intermediate risk features was evaluated. METHODS: A retrospective analysis of exercise stress testing performed on patients less than 40 years was evaluated. Patients were enrolled on a chest pain pathway and had negative serial ECGs and cardiac biomarkers before exercise stress testing to rule-out acute coronary syndrome. Chart review was completed on patients with positive stress tests. RESULTS: The 3987 patients with suspected intermediate risk acute coronary syndrome underwent exercise stress testing. One thousand and twenty-seven (25.8%) were aged less than 40 years (age 33.3 ± 4.8 years). Four of these 1027 patients had a positive exercise stress test (0.4% incidence of positive exercise stress testing). Of those, three patients had subsequent non-invasive functional testing that yielded a negative result. One patient declined further investigations. Assuming this was a true positive exercise stress test, the incidence of true positive exercise stress testing would have been 0.097% (95% confidence interval: 0.079-0.115%) (one of 1027 patients). CONCLUSIONS: Routine exercise stress testing has limited value in the risk stratification of adults less than 40 years with suspected intermediate risk of acute coronary syndrome.
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Síndrome Coronario Agudo/diagnóstico , Prueba de Esfuerzo/normas , Adulto , Dolor en el Pecho/diagnóstico , Servicio de Urgencia en Hospital , Prueba de Esfuerzo/métodos , Reacciones Falso Positivas , Femenino , Humanos , Masculino , Queensland , Estudios Retrospectivos , Medición de Riesgo , Adulto JovenRESUMEN
OBJECTIVE: Chest pain is one of the most common complaints in patients presenting to an emergency department. Delays in management due to a lack of readily available objective tests to risk stratify patients with possible acute coronary syndromes can lead to an unnecessarily lengthy admission placing pressure on hospital beds or inappropriate discharge. The need for a co-ordinated system of clinical management based on enhanced communication between departments, timely and appropriate triage, clinical investigation, diagnosis, and treatment was identified. METHODS: An evidence-based Chest Pain Management Service and clinical pathway were developed and implemented, including the introduction of after-hours exercise stress testing. RESULTS: Between November 2005 and March 2013, 5662 patients were managed according to a Chest Pain Management pathway resulting in a reduction of 5181 admission nights by more timely identification of patients at low risk who could then be discharged. In addition, 1360 days were avoided in high-risk patients who received earlier diagnosis and treatment. CONCLUSIONS: The creation of a Chest Pain Management pathway and the extended exercise stress testing service resulted in earlier discharge for low-risk patients; and timely treatment for patients with positive and equivocal exercise stress test results. This service demonstrated a significant saving in overnight admissions.
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Dolor en el Pecho/diagnóstico , Implementación de Plan de Salud/organización & administración , Hospitalización/estadística & datos numéricos , Tiempo de Internación/estadística & datos numéricos , Atención al Paciente/métodos , Medición de Riesgo/métodos , Manejo de la Enfermedad , Prueba de Esfuerzo , Femenino , Humanos , Masculino , Manejo del DolorRESUMEN
PURPOSE: Beliefs about medicines impact on adherence, but eliciting core beliefs about medicines in individual patients is difficult. One method that has the potential to elicit individual core beliefs is the "repertory grid technique." This study utilized the repertory grid technique to elicit individuals' beliefs about their heart failure treatment and to investigate whether generated constructs were different between adherent and nonadherent patients. METHODS: Ninety-two patients with heart failure were interviewed using a structured questionnaire that applied the repertory grid technique. Patients were asked to compare and contrast their medicines and self-care activities for their heart failure. This lead to the generation of individual constructs (perceptions towards medicines), and from these, beliefs were elicited about their heart failure treatment, resulting in the generation of a repertory grid. Adherence was measured using the Medication Adherence Report Scale (MARS). Patients with a MARS score ≥ 23 were categorized as "adherent" and those with a score ≤ 22 as "nonadherent." The generated grids were analyzed descriptively and constructs from all grids themed and the frequency of these constructs compared between adherent and nonadherent patients. RESULTS: Individual grids provided insight into the different beliefs that patients held about their heart failure treatment. The themed constructs "related to water," "affect the heart," "related to weight," and "benefit to the heart" occurred more frequently in adherent patients compared with nonadherent patients. CONCLUSION: The repertory grid technique elicited beliefs of individual participants about the treatment of their heart failure. Constructs from self-reported adherent patients were more likely to reflect that their medicines and self-care activities were related to water and weight, and affect and benefit to the heart. Providing clinicians with better insight into individuals' beliefs about their treatment may facilitate the development of tailored interventions to improve adherence.
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BACKGROUND: residents of aged care are old, frail and frequently require hospital management of intercurrent illness, but hospital outcomes are poor. OBJECTIVE: to identify the impact of an interdisciplinary care model on medical inpatients admitted from residential aged care (RAC). DESIGN: pre-planned subgroup analysis of controlled trial. SETTING: general medical units of a teaching hospital in Brisbane, Australia. SUBJECTS: consecutive patients aged over 65 admitted from RAC (n = 189) or the community (n = 815). METHODS: all admitted general medical patients were allocated by existing cyclical roster to control (usual care) or intervention units (interdisciplinary care consisting of improved allied health staffing, consistent teams, daily team meetings and early discharge planning). Patient characteristics and outcomes of care were compared between RAC and community subgroups. In the RAC subgroup, outcomes were compared between the control and intervention groups. RESULTS: patients admitted from RAC had much higher in-hospital mortality (13 versus 6%) and 6-month mortality (35 versus 17%) than those from community. RAC residents receiving the intervention had a significant reduction in in-hospital mortality (4 versus 22% P < 0.001) sustained at 6 months (28 versus 44% P = 0.02). CONCLUSIONS: poor hospital outcomes for RAC residents may reflect prevailing models of inpatient care.
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Hogares para Ancianos , Hospitalización/estadística & datos numéricos , Casas de Salud , Grupo de Atención al Paciente , Características de la Residencia/estadística & datos numéricos , Anciano , Anciano de 80 o más Años , Australia , Femenino , Hogares para Ancianos/estadística & datos numéricos , Mortalidad Hospitalaria , Humanos , Masculino , Casas de Salud/estadística & datos numéricos , Evaluación de Resultado en la Atención de Salud , Grupo de Atención al Paciente/normas , Estudios ProspectivosRESUMEN
BACKGROUND: In Australia, enzyme replacement therapy (ERT) for Fabry Disease (FD), both Agalsidase alfa (Replagal, Shire HGT) and beta (Fabrazyme, Genzyme), is funded and monitored through a specific government program. Agalsidase beta supply has been rationed by Genzyme since 2009 due to manufacturing issues. Consequently, the Australian Fabry Disease Advisory Committee has treated patients on Agalsidase beta at 50% of their usual dose from mid-2009, with a further reduction to 30% for some patients from late 2009. AIM: To determine the clinical effect of Agalsidase beta dose reduction in the Australian FD patient cohort. METHODS: A questionnaire assessing FD symptoms was administered to 40 patients on long-term ERT. Clinical data from The Fabry Registry for patients receiving Agalsidase alfa or beta, for at least 2 years prior to the time of enforced Agalsidase beta dose reduction, were reviewed. Disease burden and quality of life (QOL) were graded using the Disease Severity Scoring System, Mainz Severity Score Index, Brief Pain Inventory and Short Form 36 Health Survey at 2 years before dose reduction, at the time of dose reduction and at the most recent clinical review following dose reduction. RESULTS: Disease severity and QOL scores did not change between the ERT groups. Males on Agalsidase beta reported lower energy levels after dose reduction, while no change was reported by females on either product or by males on a stable dose of Agalsidase alfa. CONCLUSION: This study suggests that energy levels in male patients worsen after dose reduction of Agalsidase beta.
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AIMS: The Exercise Joins Education: Combined Therapy to Improve Outcomes in Newly-discharged Heart Failure (EJECTION-HF) study will evaluate the impact of a supervised exercise training programme (ETP) on clinical outcomes in recently hospitalized heart failure patients attending a disease management programme (DMP). Methods This multisite, pragmatic randomized controlled trial enrols patients discharged from participating hospitals with clinical evidence of heart failure who are willing and able to participate in a DMP and considered clinically safe to exercise. Enrolment includes participants with impaired and preserved left ventricular systolic function. Baseline assessment and programme commencement occur within 6 weeks of hospital discharge. The control group DMP includes individualized education and follow-up from a multidisciplinary heart failure team; a weekly education programme for 12 weeks; self-management advice; and medical follow-up. Home exercise is recommended for all participants. In addition, intervention participants are offered 36 supervised, structured gym-based 1 h exercise sessions over 24 weeks. Sessions are tailored to exercise capacity and include aerobic, resistance, and balance exercises. Enrolment target is 350 participants. Primary outcome is 12-month mortality and readmissions. Secondary outcomes include blinded evaluation of depressive symptoms, sleep quality, cognition, and functional status (activities of daily living, 6 min walk distance, grip strength) at 3 and 6 months. A cost-utility analysis will be conducted. CONCLUSION: This study will enrol a representative group of hospitalized heart failure patients and measure a range of patient and health service outcomes to inform the design of post-hospital DMPs for heart failure. Enrolment will be completed in 2013. ACTRN12608000263392.
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Actividades Cotidianas , Manejo de la Enfermedad , Terapia por Ejercicio/métodos , Ejercicio Físico/fisiología , Insuficiencia Cardíaca/rehabilitación , Pacientes Internos , Calidad de Vida , Análisis Costo-Beneficio , Método Doble Ciego , Terapia por Ejercicio/economía , Femenino , Estudios de Seguimiento , Insuficiencia Cardíaca/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Older medical patients often experience a decline in function associated with hospitalization. Some of this decline is already established at hospital admission, whereas some occurs during hospitalization. Objectives of this study were to separately describe pre-hospital and in-hospital functional changes in older Australian medical patients and to identify risk factors associated with these functional changes. METHODS: Secondary analysis of data from a prospective controlled trial conducted in general medical units of an Australian tertiary teaching hospital. Participants were 615 consecutive patients aged 65 years or older admitted under a general medical unit for more than 2 days, discharged alive, and not fully dependent at pre-admission baseline. Activities of daily living measured 2 weeks before admission, at admission, and at discharge were used to calculate rates of pre-hospital and in-hospital decline and of in-hospital recovery to pre-admission function. Potential predictors including age, sex, diagnosis, illness severity, pre-admission function, pre-admission supports, and documented "geriatric syndromes" (dementia, falls, malnutrition) were investigated for each functional change outcome using multiple logistic regression models. RESULTS: Sixty-four percent of participants had pre-hospital functional decline; only 42% of these had recovered to pre-admission function by hospital discharge. Only 7% had in-hospital decline. The different functional change variables had distinct patterns of predictors. CONCLUSIONS: Most decline occurred prior to hospitalization and was associated with common indicators of poor outcomes in hospitalized elders. In-hospital decline was uncommon, suggesting that in-hospital recovery may be a more appropriate intervention target.
Asunto(s)
Actividades Cotidianas , Hospitalización , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Tiempo de Internación , Masculino , Análisis Multivariante , Factores de Riesgo , Factores de TiempoRESUMEN
BACKGROUND: Congestive heart failure (CHF) is an increasingly common condition associated with significant hospital resource utilization. Initiating better disease management at the time of initial hospital admission has the potential to reduce readmissions. OBJECTIVE: To evaluate the impact of a multifaceted quality improvement program on 12-month hospital utilization in patients admitted to hospital with CHF. DESIGN: Prospective longitudinal study comparing baseline and intervention cohorts. PARTICIPANTS: All consecutive patients with CHF discharged alive from 3 metropolitan hospitals during the baseline (October 1, 2000 to April 17, 2001) and intervention (February 15, 2002 to August 31, 2002) study periods. Active prospective case-finding identified 220 baseline and 235 intervention participants; full data was available on 197 baseline and 219 intervention participants. INTERVENTIONS: Education and performance feedback for hospital and primary care practitioners; clinical decision support tools; individualized, guideline-based treatment plans; patient education and self-management support; and improved hospital-community integration. MEASUREMENTS: Twelve-month all-cause hospital readmission, 12-month mortality, readmission-free survival, heart failure-specific readmission, and total hospital days over 12 months. RESULTS: Intervention patients had a higher rate of all-cause readmission (odds ratio [OR] = 1.65; 95% confidence interval [CI] = 1.10-2.46) but a trend to reduction in mortality (OR = 0.68; 95% CI = 0.44-1.07). There was no difference in frequency of hospitalizations per year, number of hospital days, or the composite outcome of death or readmission. CONCLUSIONS: The intervention improved care processes and may have reduced mortality, but at the cost of higher readmission rates. Better understanding of intervention components, intensity, and targeting may optimize the effectiveness of disease management programs.