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1.
J Cyst Fibros ; 2021 Sep 23.
Artigo em Inglês | MEDLINE | ID: mdl-34565705

RESUMO

BACKGROUND: Studies in separate cohorts suggest possible discrepancies between inhaled medicines supplied (median 50-60%) and medicines used (median 30-40%). We performed the first study that directly compares CF medicine supply against use to identify the cost of excess medicines supply. METHODS: This cross-sectional study included participants from 12 UK adult centres with ≥1 year of continuous adherence data from data-logging nebulisers. Medicine supply was measured as medication possession ratio (MPR) for a 1-year period from the first suitable supply date. Medicine use was measured as electronic data capture (EDC) adherence over the same period. The cost of excess medicines was calculated as whole excess box(es) supplied after accounting for the discrepancy between EDC adherence and MPR with 20% contingency. RESULTS: Among 275 participants, 133 (48.4%) were females and mean age was 30 years (95% CI 29-31 years). Median EDC adherence was 57% (IQR 23-86%), median MPR was 74% (IQR 46-96%) and the discrepancy between measures was median 14% (IQR 2-29%). Even with 20% contingency, mean potential cost of excess medicines was £1,124 (95% CI £855-1,394), ranging from £183 (95% CI £29-338) for EDC adherence ≥80% to £2,017 (95% CI £1,507-2,526) for EDC adherence <50%. CONCLUSIONS: This study provides a conservative estimate of excess inhaled medicines supply cost among adults with CF in the UK. The excess supply cost was highest among those with lowest EDC adherence, highlighting the importance of adherence support and supplying medicine according to actual use. MPR provides information about medicine supply but over-estimates actual medicine use.

2.
BMJ Open Respir Res ; 7(1)2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32928787

RESUMO

The SARS-CoV-2 can lead to severe illness with COVID-19. Outcomes of patients requiring mechanical ventilation are poor. Awake proning in COVID-19 improves oxygenation, but on data clinical outcomes is limited. This single-centre retrospective study aimed to assess whether successful awake proning of patients with COVID-19, requiring respiratory support (continuous positive airways pressure (CPAP) or high-flow nasal oxygen (HFNO)) on a respiratory high-dependency unit (HDU), is associated with improved outcomes. HDU care included awake proning by respiratory physiotherapists. Of 565 patients admitted with COVID-19, 71 (12.6%) were managed on the respiratory HDU, with 48 of these (67.6%) requiring respiratory support. Patients managed with CPAP alone 22/48 (45.8%) were significantly less likely to die than patients who required transfer onto HFNO 26/48 (54.2%): CPAP mortality 36.4%; HFNO mortality 69.2%, (p=0.023); however, multivariate analysis demonstrated that increasing age and the inability to awake prone were the only independent predictors of COVID-19 mortality. The mortality of patients with COVID-19 requiring respiratory support is considerable. Data from our cohort managed on HDU show that CPAP and awake proning are possible in a selected population of COVID-19, and may be useful. Further prospective studies are required.


Assuntos
Pressão Positiva Contínua nas Vias Aéreas/métodos , Infecções por Coronavirus/terapia , Oxigenoterapia/métodos , Posicionamento do Paciente/métodos , Pneumonia Viral/terapia , Decúbito Ventral , Idoso , Idoso de 80 Anos ou mais , Betacoronavirus , COVID-19 , Infecções por Coronavirus/mortalidade , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Ventilação não Invasiva/métodos , Razão de Chances , Pandemias , Pneumonia Viral/mortalidade , Estudos Retrospectivos , SARS-CoV-2 , Resultado do Tratamento , Reino Unido , Vigília
3.
Artigo em Inglês | MEDLINE | ID: mdl-32839209

RESUMO

BACKGROUND: Cystic Fibrosis (CF) is a life-limiting illness. Audit of the care of patients dying of CF has not been published to date. METHODS: Newcastle and Oxford teams adapted the National Audit of Care at the End of Life and agreed additional questions that were particularly pertinent for patients dying as a consequence of their CF. Data were extracted and analysed for 15 patients. RESULTS: On recognition that the patient was dying, the CF teams were less good at reviewing the need for physiological observations (50% vs national 70%) but better at reviewing the need for capillary blood glucose monitoring, oxygen support and intravenous antibiotics compared with the national average for all patients.On recognition that the patient was dying, the CF teams were better at assessing pain (87% vs national 80%) and breathlessness (93% vs national 73%), but less good at assessing nausea and vomiting (47% vs national 74%).There was documented evidence that 100% of families and 64% of patients were aware that the patient was at risk of dying. CONCLUSION: Comparing care of this sample of patients dying with CF against the national data is a useful first step in understanding that many aspects of care are of high quality. This audit identifies the need to offer earlier conversations to patients as their voices may be missing from the conversation. Undertaking a national audit would provide a more reliable and a fuller picture.

4.
Int J Mycobacteriol ; 9(1): 48-52, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32474488

RESUMO

Background: Treatment of Mycobacterium abscessus pulmonary disease (PD) is challenging with frequent side effects and uncertain rates of success. Methods: We performed a retrospective review of all patients at our center with at least one respiratory sample positive for M. abscessus between 2014 and 2019. Electronic health records were reviewed to determine factors associated with M. abscessus infection and clinical outcomes. Results: Thirty-seven patients were identified including 24 with cystic fibrosis (CF), 10 with bronchiectasis, two with chronic obstructive PD (COPD), and one with asthma. American Thoracic Society/Infectious Diseases Society of America criteria for nontuberculous mycobacteria PD were met in 21/37 (56.8%) of cases. Evidence of Aspergillus lung disease was noted in 18 (75.0%) CF patients compared with 3 (23.1%) non-CF patients (P = 0.005). Induction therapy for M. abscessus was given to 22/37 (59.5%) patients (18/24 [75%] with CF and 4/13 [30.8%] without CF). Median duration of induction therapy was 6 weeks (range 3-12). Maintenance antibiotic therapy was prescribed to 17/22 (77.3%) of treated patients. Culture conversion was seen in 15/24 (62.5%) of CF patients compared with 3/13 (23.1%) in the non-CF group (P = 0.034). Culture conversion occurred in 10/22 (45.5%) of treated patients compared with 8/15 (53.3%) untreated patients. Three patients (8.1%) died during follow-up: one with CF and two with COPD. Conclusions: Culture conversion following isolation of M. abscessus from respiratory samples not only is more common in CF than in patients without CF but also frequently occurs spontaneously in both groups. Targeted treatment for M. abscessus did not clearly impact rates of culture conversion.


Assuntos
Antibacterianos/uso terapêutico , Pulmão/microbiologia , Infecções por Mycobacterium não Tuberculosas/microbiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/farmacologia , Coinfecção/tratamento farmacológico , Coinfecção/microbiologia , Fibrose Cística/complicações , Fibrose Cística/microbiologia , Registros Eletrônicos de Saúde , Feminino , Humanos , Masculino , Testes de Sensibilidade Microbiana , Pessoa de Meia-Idade , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Infecções por Mycobacterium não Tuberculosas/mortalidade , Mycobacterium abscessus/efeitos dos fármacos , Mycobacterium abscessus/patogenicidade , Estudos Retrospectivos , Escarro/microbiologia , Resultado do Tratamento , Adulto Jovem
7.
Paediatr Respir Rev ; 20 Suppl: 27-9, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27374622

RESUMO

Achromobacter spp. are emerging pathogens in the lungs of patients with cystic fibrosis. We report the case of an adult patient with cystic fibrosis and chronic A. xylosoxidans infection who experienced rapid, progressive clinical deterioration. Metagenomic analysis of the sputum revealed that the airway microbiota was almost entirely dominated by A. xylosoxidans. We review the impact of this organism on lung function and the airway microbiome in cystic fibrosis, and discuss the potential for cross-infection between patients.


Assuntos
Achromobacter denitrificans/patogenicidade , Antibacterianos/uso terapêutico , Infecção Hospitalar/tratamento farmacológico , Infecção Hospitalar/microbiologia , Fibrose Cística/microbiologia , Infecções por Bactérias Gram-Negativas/tratamento farmacológico , Infecções por Bactérias Gram-Negativas/microbiologia , Adulto , Fibrose Cística/cirurgia , Humanos , Transplante de Pulmão , Masculino , Escarro/microbiologia
8.
J Clin Microbiol ; 53(7): 2022-9, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25878338

RESUMO

Respiratory infection in cystic fibrosis (CF) is polymicrobial, but standard sputum microbiology does not account for the lung microbiome or detect changes in microbial diversity associated with disease. As a clinically applicable CF microbiome surveillance scheme, total sputum nucleic acids isolated by a standard high-throughput robotic method for accredited viral diagnosis were profiled for bacterial diversity using ribosomal intergenic spacer analysis (RISA) PCR. Conventional culture and RISA were performed on 200 paired sputum samples from 93 CF adults; pyrosequencing of the 16S rRNA gene was applied to 59 patients to systematically determine bacterial diversity. Compared to the microbiology data, RISA profiles clustered into two groups: the emerging nonfermenting Gram-negative organisms (eNFGN) and Pseudomonas groups. Patients who were culture positive for Burkholderia, Achromobacter, Stenotrophomonas, and Ralstonia clustered within the eNFGN group. Pseudomonas group RISA profiles were associated with Pseudomonas aeruginosa culture-positive patients. Sequence analysis confirmed the abundance of eNFGN genera and Pseudomonas within these respective groups. Low bacterial diversity was associated with severe lung disease (P < 0.001) and the presence of Burkholderia (P < 0.001). An absence of Streptococcus (P < 0.05) occurred in individuals with lung function in the lowest quartile. In summary, nucleic acids isolated from CF sputum can serve as a single template for both molecular virology and bacteriology, with a RISA PCR rapidly detecting the presence of dominant eNFGN pathogens or P. aeruginosa missed by culture (11% of cases). We provide guidance for how this straightforward CF microbiota profiling scheme may be adopted by clinical laboratories.


Assuntos
Bactérias/isolamento & purificação , Biodiversidade , Fibrose Cística/complicações , Técnicas de Diagnóstico Molecular/métodos , Pneumonia Bacteriana/diagnóstico , Escarro/microbiologia , Adulto , Automação Laboratorial/métodos , Bactérias/classificação , Bactérias/genética , Feminino , Humanos , Masculino , Pneumonia Bacteriana/microbiologia , Pneumonia Viral/diagnóstico , Pneumonia Viral/microbiologia , Manejo de Espécimes/métodos , Escarro/virologia , Vírus/classificação , Vírus/genética , Vírus/isolamento & purificação , Adulto Jovem
9.
Thorax ; 69(3): 247-53, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24127019

RESUMO

BACKGROUND: Viral respiratory infection (VRI) is a common cause of pulmonary exacerbations in children with cystic fibrosis (CF). The importance of VRI in adult CF populations is unclear. OBJECTIVE: To determine the incidence and clinical impact of VRI among adults with CF. METHODS: One hundred adults with CF were followed up prospectively for 12 months. Sputum, nose swabs and throat swabs were collected every 2 months and at onset of pulmonary exacerbation. PCR assays for adenovirus, influenza A&B, human metapneumovirus, parainfluenza 1-3, respiratory syncytial virus and human rhinovirus were performed on each sample. Symptom scores, spirometry and inflammatory markers were measured at each visit. RESULTS: One or more respiratory viruses were detected in 191/626 (30.5%) visits. Human rhinovirus accounted for 72.5% of viruses. Overall incidence of VRI was 1.66 (95% CI 1.39 to 1.92) cases/patient-year. VRI was associated with increased risk of pulmonary exacerbation (OR=2.19; 95% CI 1.56 to 3.08; p<0.001) and prescription of antibiotics (OR=2.26; 95% CI 1.63 to 3.13; p<0.001). Virus-positive visits were associated with higher respiratory symptom scores and greater C-reactive protein levels. Virus-positive exacerbations had a lower acute fall in FEV1 than virus-negative exacerbations (12.7% vs 15.6%; p=0.040). The incidence of exacerbations, but not VRI, was associated with greater lung function decline over 12 months (-1.79% per pulmonary exacerbation/year; 95% CI -3.4 to -0.23; p=0.025). CONCLUSION: VRI is common in adults with CF and is associated with substantial morbidity. Respiratory viruses are a potential therapeutic target in CF lung disease.


Assuntos
Proteína C-Reativa/metabolismo , Fibrose Cística/diagnóstico , Fibrose Cística/virologia , Infecções por Vírus Respiratório Sincicial/complicações , Vírus Sinciciais Respiratórios/isolamento & purificação , Adulto , Biomarcadores/sangue , Fibrose Cística/sangue , Fibrose Cística/epidemiologia , Progressão da Doença , Feminino , Seguimentos , Volume Expiratório Forçado , Humanos , Incidência , Masculino , Valor Preditivo dos Testes , Estudos Prospectivos , Infecções por Vírus Respiratório Sincicial/diagnóstico , Infecções por Vírus Respiratório Sincicial/epidemiologia , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Espirometria , Reino Unido/epidemiologia
10.
J Clin Microbiol ; 51(11): 3893-6, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-23966488

RESUMO

Rhinovirus is a common cause of exacerbations of cystic fibrosis (CF) and is usually considered a self-limiting infection. We report a case of chronic infection with rhinovirus A type 33 in a 43-year-old male with CF which has persisted for over 2 years.


Assuntos
Fibrose Cística/complicações , Infecções por Picornaviridae/diagnóstico , Infecções por Picornaviridae/patologia , Rhinovirus/isolamento & purificação , Adulto , Doença Crônica , Análise por Conglomerados , Genótipo , Humanos , Masculino , Mucosa Nasal/virologia , Faringe/virologia , Filogenia , Reação em Cadeia da Polimerase , Rhinovirus/classificação , Rhinovirus/genética , Análise de Sequência de DNA , Homologia de Sequência , Escarro/virologia
12.
Thorax ; 67(10): 931-2, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22678138

RESUMO

The authors present the case of an older patient with cystic fibrosis (CF) and recurrent haemoptysis complicated by acute pulmonary embolism. The patient was treated successfully with a combination of anticoagulation and bronchial artery embolisation. The management of CF-related haemoptysis, the impact of an ageing CF population and the incidence of thromboembolic disease in CF are discussed.


Assuntos
Fibrose Cística/complicações , Hemoptise/etiologia , Hemoptise/terapia , Embolia Pulmonar/etiologia , Embolia Pulmonar/terapia , Idoso , Biomarcadores/sangue , Comorbidade , Fibrose Cística/genética , Eletrocardiografia , Embolização Terapêutica , Hidratação , Humanos , Masculino , Recidiva , Tomografia Computadorizada por Raios X
13.
J Cyst Fibros ; 11(3): 187-92, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-22177738

RESUMO

BACKGROUND: Non-invasive ventilation (NIV) is accepted as a bridge to lung transplantation in cystic fibrosis (CF) but there is little evidence to support its use outside this setting. METHODS: We reviewed the records of all patients with CF who received domiciliary NIV at our centre between 1991 and 2010. RESULTS: Of 47 patients studied, 36% underwent lung transplantation, 28% died without transplantation and 30% remain alive on NIV. Median duration of NIV was 16 months (range 2-90). Mean FEV(1) fell by 212 ml over the year before NIV but increased by 18 ml in the following year (p<0.01). Individual response to NIV was associated with lower baseline and more rapid decline in FEV(1). From 1991 to 2000, 70% underwent lung transplantation; from 2001 to 2010 only 27% were transplanted. CONCLUSIONS: NIV may slow or reverse the decline in lung function in advanced CF. NIV is increasingly used beyond a bridge to transplantation at our centre.


Assuntos
Fibrose Cística/terapia , Respiração com Pressão Positiva/métodos , Cuidados Pré-Operatórios/métodos , Insuficiência Respiratória/terapia , Adolescente , Adulto , Fibrose Cística/complicações , Fibrose Cística/mortalidade , Feminino , Seguimentos , Volume Expiratório Forçado , Humanos , Transplante de Pulmão , Masculino , Pessoa de Meia-Idade , Prognóstico , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/fisiopatologia , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Reino Unido/epidemiologia , Adulto Jovem
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