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1.
Sleep Med X ; 7: 100115, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-39022329

RESUMO

Current UK guidance on OSA management recommends only selective use of sleep studies - when there is diagnostic uncertainty, in children with comorbidities or to evaluate perioperative risk in those with suspected severe OSA. Routine use of sleep studies to confirm a diagnosis of obstructive sleep apnoea (OSA) in children before adenotonsillectomy is not currently recommended. We report the findings of a novel paediatric sleep service based on routine use of multi-channel sleep studies (MCSS) before adenotonsillectomy and present the results of a service evaluation assessing the impact of our practise on treatment outcomes and cost. We conducted a retrospective study of 264 children with sleep disordered breathing seen in our centre between July 2018-June 2019, using medical records and a sleep study database to determine treatment outcomes and costs. Using responses from a questionnaire completed by otolaryngologists for a separate prospective study, we compare our costs with estimates of those associated with a standard UK model of care i.e. with selective use of sleep studies. We estimate that our routine use of MCSS reduced the number of adenotonsillectomies by 44 % but at higher monetary costs than those estimated for the standard model of care. We note however, that reconfiguring our service to arrange a sleep study before the initial appointment, rather than after, would result in the service being cost neutral compared with the standard model. We also estimate that use of home multi-channel studies in our service would bring a significant cost saving (∼£50,000 - £80,000 per annum) compared to standard care.

2.
Sleep Med X ; 7: 100111, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38800098

RESUMO

There are significant variations in practice regarding the use of sleep studies in children with symptoms of sleep disordered breathing (SDB) prior to adenotonsillectomy. Current UK guidance recommends the selective use of sleep studies to confirm a diagnosis of obstructive sleep apnoea (OSA) when there is diagnostic uncertainty, in children with comorbidities, or to assess perioperative risk when severe OSA is suspected. We have developed a novel paediatric sleep service over the past decade based on the routine use of multi-channel sleep studies (MCSS) before adenotonsillectomy. We present the results of a prospective evaluation assessing the impact of our service on treatment outcomes. We conducted a prospective service evaluation of 49 children with SDB seen between July 2021 and August 2022. We used medical records and a sleep study database to determine treatment outcomes. Otolaryngologists completed a questionnaire before each multi-channel sleep study to help evaluate the impact of sleep study findings on surgical decision making. Questionnaire responses before MCSS showed that clinicians thought 66 % of children were 'likely', 'very likely' or 'definitely' would require surgery but only 54 % of children underwent surgery following their sleep study. We estimate that the use of MCSS was associated with a 21 % reduction in children undergoing surgery in this small sample. We conclude that our use of MCSS facilitates conservative management, allowing a significant reduction in the number of children with SDB undergoing surgery, but further validation of MCSS against polysomnography is required.

3.
J Cyst Fibros ; 23(2): 187-202, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38233247

RESUMO

This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey.


Assuntos
Fibrose Cística , Fibrose Cística/terapia , Humanos , Europa (Continente) , Sociedades Médicas
4.
Cochrane Database Syst Rev ; 9: CD015236, 2023 09 27.
Artigo em Inglês | MEDLINE | ID: mdl-37753791

RESUMO

BACKGROUND: Cystic fibrosis (CF) is a multisystem disease; the importance of growth and nutritional status is well established given their implications for lung function and overall survivability. Furthermore, it has been established that intestinal microbial imbalance and inflammation are present in people with CF. Oral prebiotics are commercially available substrates that are selectively utilised by host intestinal micro-organisms and may improve both intestinal and overall health. OBJECTIVES: To evaluate the benefits and harms of prebiotics for improving health outcomes in children and adults with CF. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis Trials Register compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews. Date of last search: 19 October 2022. We also searched PubMed and online trials registries. Date of last search: 13 January 2023. SELECTION CRITERIA: Randomised controlled trials (RCTs) and quasi-RCTs assessing the efficacy of prebiotics in children and adults with CF. We planned to only include the first treatment period from cross-over RCTs, regardless of washout period. DATA COLLECTION AND ANALYSIS: We did not identify any relevant trials. MAIN RESULTS: We did not identify any relevant trials for inclusion in this review. AUTHORS' CONCLUSIONS: This review did not find any evidence for the use of prebiotics in people with CF. Until such evidence is available, it is reasonable for clinicians to follow any local guidelines and to discuss the use of dietary prebiotics with their patients. Large and robust RCTs assessing the dietary prebiotics of inulin or galacto-oligosaccharides or fructo-oligosaccharides, or any combination of these, are needed. Such studies should be of at least 12 months in duration and assess outcomes such as growth and nutrition, gastrointestinal symptoms, pulmonary exacerbations, lung function, inflammatory biomarkers, hospitalisations, intestinal microbial profiling, and faecal short-chain fatty acids. Trials should include both children and adults and aim to be adequately powered to allow for subgroup analysis by age.


Assuntos
Fibrose Cística , Adulto , Criança , Humanos , Fezes , Hospitalização , Inflamação , Estado Nutricional , Prebióticos
5.
Pediatr Res ; 94(1): 43-54, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36624282

RESUMO

Prediction models could identify infants at the greatest risk of bronchopulmonary dysplasia (BPD) and allow targeted preventative strategies. We performed a systematic review and meta-analysis with external validation of identified models. Studies using predictors available before day 14 of life to predict BPD in very preterm infants were included. Two reviewers assessed 7628 studies for eligibility. Meta-analysis of externally validated models was followed by validation using 62,864 very preterm infants in England and Wales. A total of 64 studies using 53 prediction models were included totalling 274,407 infants (range 32-156,587/study). In all, 35 (55%) studies predated 2010; 39 (61%) were single-centre studies. A total of 97% of studies had a high risk of bias, especially in the analysis domain. Following meta-analysis of 22 BPD and 11 BPD/death composite externally validated models, Laughon's day one model was the most promising in predicting BPD and death (C-statistic 0.76 (95% CI 0.70-0.81) and good calibration). Six models were externally validated in our cohort with C-statistics between 0.70 and 0.90 but with poor calibration. Few BPD prediction models were developed with contemporary populations, underwent external validation, or had calibration and impact analyses. Contemporary, validated, and dynamic prediction models are needed for targeted preventative strategies. IMPACT: This review aims to provide a comprehensive assessment of all BPD prediction models developed to address the uncertainty of which model is sufficiently valid and generalisable for use in clinical practice and research. Published BPD prediction models are mostly outdated, single centre and lack external validation. Laughon's 2011 model is the most promising but more robust models, using contemporary data with external validation are needed to support better treatments.


Assuntos
Displasia Broncopulmonar , Doenças do Prematuro , Lactente , Recém-Nascido , Humanos , Recém-Nascido Prematuro , Displasia Broncopulmonar/diagnóstico , Recém-Nascido de muito Baixo Peso , Inglaterra
6.
Health Sci Rep ; 5(5): e739, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35949680

RESUMO

Background and Aims: Journal impact factor has historically been taken as a proxy for quality. However, this is open to significant manipulation and bias. There is currently not widely adopted, robust journal and paper ranking metric which is focused solely on risk of bias. Methods: Risk of bias data was extracted from all Cochrane database systematic reviews in Child Health, Lungs, and Airways for the years 2017-2019. A novel paper quality score, the Clinical Research Bias Index (CRBI), was applied. Individual paper data were pooled for each journal. A comparison was made to journal impact factors, individual paper citations, reads, and altmetric scores. Results: 927 papers were analyzed for risk of bias. 119 (12·8%) scored a CRBI of 100%, with a mean score of 70%. A journal's overall CRBI risk of bias score was poorly correlated with impact factor (r 0.25). Citations (r 0.02), and reads (r 0.01) of individual papers showed very little association with the paper's risk of bias. Likewise, reads were not correlated with citations (r 0.03). H-index and Altmetric scores were similarly poorly correlated with CRBI. Conclusion: The novel research quality tool CRBI demonstrates the poor correlation between journal impact factor, citations, and risk of bias. Journal and paper ranking metrics should ensure that they are fit for purpose, and enable the dissemination of high-quality research for the benefit of patients. We propose the CRBI as a potential solution which is resistant to manipulation and will reward the creation and publication of bias-free research.

7.
Pediatr Crit Care Med ; 22(5): e285-e293, 2021 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-33767074

RESUMO

OBJECTIVES: To 1) analyze the short-term biochemical improvements and clinical outcomes following treatment of children with post-severe acute respiratory syndrome coronavirus-2 inflammatory syndrome (multisystem inflammatory syndrome in children/pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2) admitted to U.K. PICUs and 2) collate current treatment guidance from U.K. PICUs. DESIGN: Multicenter observational study. SETTING: Twenty-one U.K. PICUs. PATIENTS: Children (< 18 yr) admitted to U.K. PICUs between April 1, 2020, and May 10, 2020, fulfilling the U.K. case definition of pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Routinely collected, deidentified data were analyzed. Propensity score and linear mixed effects models were used to analyze the effect of steroids, IV immunoglobulin, and biologic agents on changes in C-reactive protein, platelet counts, and lymphocyte counts over the course of PICU stay. Treatment recommendations from U.K. clinical guidelines were analyzed. Over the 6-week study period, 59 of 78 children (76%) received IV immunoglobulin, 57 of 78 (73%) steroids, and 18 of 78 (24%) a biologic agent. We found no evidence of a difference in response in clinical markers of inflammation between patients with multisystem inflammatory syndrome in children/pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 who were treated with IV immunoglobulin, steroids, or biologics, compared with those who were not. By the end of the study period, most patients had received immunomodulation. The 12 patients who did not receive any immunomodulators had similar decrease in inflammatory markers as those treated. Of the 14 guidelines analyzed, the use of IV immunoglobulin, steroids, and biologics was universally recommended. CONCLUSIONS: We were unable to identify any short-term benefit from any of the treatments, or treatment combinations, administered. Despite a lack of evidence, treatment guidelines for multisystem inflammatory syndrome in children/pediatric inflammatory multisystem syndrome temporally associated with severe acute respiratory syndrome coronavirus-2 have become very similar in advising step-wise treatments. Retaining clinical equipoise regarding treatment will allow clinicians to enroll children in robust clinical trials to determine the optimal treatment for this novel important condition.


Assuntos
COVID-19 , Criança , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica
8.
J Cyst Fibros ; 20(4): 591-597, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-32561324

RESUMO

BACKGROUND: Cystic fibrosis (CF) is a multi-system genetic disorder affecting >72,000 people worldwide. Most CF patients experience gastrointestinal symptoms and can develop complications. However, the mechanisms of CF gut disease are not well understood. We evaluated gut function and transit in CF using magnetic resonance imaging (MRI). We hypothesised oro-caecal transit time (OCTT) is longer in CF; with lower small bowel water content (SBWC). METHODS: Twelve CF patients aged 12-40 years and 12 age and sex-matched controls underwent serial MRIs over 1 day with standardised meals. The primary endpoint was OCTT, assessed by the appearance of a food bolus in the caecum. Other measures included corrected SBWC and corrected colonic volume (both area under the curve, AUC), gastric half-emptying time and gastrointestinal symptoms. RESULTS: OCTT was longer in CF (CF 330 mins [270, >360] vs. controls 210 mins [173, 315], p = 0.04), with no difference in gastric half-emptying times. Corrected SBWC was higher in CF (CF 62 L.min/m2 [36, 80] vs. controls 34 L.min/m2 [28, 41], p = 0.021); minimal postprandial decrease between T240 and T300 (CF 13 mL/m2 [-13, 57] vs. controls 102 mL/m2 [67, 108], p = 0.002) suggests impaired ileal emptying. Corrected colonic volumes were higher in CF (CF 186 L.min/m2 [167, 206] vs. controls 123 L.min/m2 [89, 146], p = 0.012). There were no differences in gastrointestinal symptoms. CONCLUSIONS: MRI provides novel insights into CF pathophysiology. Sub-clinical ileal obstruction may be more prevalent than previously thought. Gastrointestinal MRI shows promise as an investigational tool in CF.


Assuntos
Fibrose Cística/fisiopatologia , Trato Gastrointestinal/diagnóstico por imagem , Trato Gastrointestinal/fisiopatologia , Trânsito Gastrointestinal , Imageamento por Ressonância Magnética , Período Pós-Prandial , Adolescente , Adulto , Criança , Feminino , Humanos , Masculino , Estudos Prospectivos , Adulto Jovem
10.
Eur J Radiol ; 130: 109196, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32739780

RESUMO

PURPOSE: The diaphragm is the most important muscle of respiration. Disorders of the diaphragm can have a deleterious impact on respiratory function. We aimed to evaluate the use of an open-configuration upright low-field MRI system to assess diaphragm morphology and function in patients with bilateral diaphragm weakness (BDW) and chronic obstructive pulmonary disease (COPD) with hyperinflation. METHOD: The study was approved by the National Research Ethics Committee, and written consent was obtained. We recruited 20 healthy adult volunteers, six subjects with BDW, and five subjects with COPD with hyperinflation. We measured their vital capacity in the upright and supine position, after which they were scanned on the 0.5 T MRI system during 10-s breath-holds at end-expiration and end-inspiration in both positions. We developed and applied image analysis methods to measure the volume under the dome, maximum excursion of hemidiaphragms, and anterior-posterior and left-right extension of the diaphragm. RESULTS: All participants were able to complete the scanning protocol. The patients found scanning in the upright position more comfortable than the supine position. All differences in the supine inspiratory-expiratory parameters, excluding left-right extension, were significantly smaller in the BDW and COPD groups compared with healthy volunteers. No significant correlation was found between the postural change in diaphragm morphology and vital capacity in either group. CONCLUSION: Our combined upright-supine MR imaging approach facilitates the assessment of the impact of posture on diaphragm morphology and function in patients with BDW and those with COPD with hyperinflation.


Assuntos
Diafragma/diagnóstico por imagem , Diafragma/fisiopatologia , Imageamento por Ressonância Magnética/instrumentação , Postura/fisiologia , Adulto , Expiração/fisiologia , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Doença Pulmonar Obstrutiva Crônica/diagnóstico por imagem , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Respiração , Volume de Ventilação Pulmonar/fisiologia , Capacidade Vital/fisiologia
11.
Front Neurol ; 11: 316, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32457689

RESUMO

Background: Pulse transit time (PTT) is a non-invasive measure of arousals and respiratory effort for which we aim to identify threshold values that detect sleep disordered breathing (SDB) in children. We also compare the sensitivity and specificity of oximetry with the findings of a multi-channel study. Methods: We performed a cross-sectional observational study of 521 children with SDB admitted for multi-channel sleep studies (pulse oximetry, ECG, video, sound, movement, PTT) in a secondary care centre. PTT data was available in 368 children. Studies were categorised as normal; primary snoring; upper airway resistance syndrome (UARS); obstructive sleep apnoea (OSA), and "abnormal other." Receiver operator characteristic curves were constructed for different PTT (Respiratory swing; Arousal index) thresholds using a random sample of 50% of children studied (training set); calculated thresholds of interest were validated against the other 50% (test set). Study findings were compared with oximetry categories (normal, inconclusive, abnormal) using data (mean and minimum oxygen saturations; oxygen desaturations > 4%) obtained during the study. Results: Respiratory swing of 17.92 ms identified SDB (OSA/UARS) with sensitivity: 0.80 (C.I. 0.62-0.90) and specificity 0.79 (C.I. 0.49-0.87). PTT arousal index of 16.06/ hour identified SDB (OSA/UARS) with sensitivity: 0.85 (95% C.I. 0.67-0.92) and specificity 0.37 (95% C.I. 0.17-0.48). Oximetry identified SDB (OSA) with sensitivity: 0.38 (C.I. 0.31-0.46) and specificity 0.98 (C.I. 0.97-1.00). Conclusions: PTT is more sensitive but less specific than oximetry at detecting SDB in children. The additional use of video and sound enabled detection of SDB in twice as many children as oximetry alone.

13.
Breathe (Sheff) ; 15(4): 273-276, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31803260

RESUMO

Wheezy infants do not respond to bronchodilators despite evidence of functioning ß-adrenoceptors. This is because the predominant aetiology, bronchiolitis, is characterised by small airway oedema and increased mucus, for which ß2-agonists are ineffective. http://bit.ly/2Ws9ffh.

14.
ERJ Open Res ; 5(2)2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31044141

RESUMO

In this article, the Group Chairs and early career members of the European Respiratory Society (ERS) Paediatric Assembly highlight some of the most interesting findings in the field of paediatrics which were presented at the 2018 international ERS Congress.

15.
Thorax ; 74(3): 229-236, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30301819

RESUMO

INTRODUCTION: Cystic fibrosis (CF) is a multisystem disorder. Treatment is complex and evidence for treatment decisions may be absent. Characterising gaps in the research evidence will highlight treatment uncertainties and help prioritise research questions. We systematically identified the evidence gaps for treatment decisions in CF. METHODS: We searched for systematic reviews and guidelines on treatment interventions in CF. Two researchers identified eligible reviews with arbitration from a third. Using a structured framework, we extracted and characterised evidence gaps. RESULTS: There were 73 reviews and 21 guidelines that met our inclusion criteria. From these, we identified 148 evidence gaps across a range of treatment areas. We found 111 evidence gaps through systematic reviews and a further 37 from guidelines. The reason for an evidence gap could only be reliably characterised for systematic reviews. In most cases, there was more than one explanation-most commonly few or no trials (97/111 evidence gaps). Other important factors leading to evidence gaps were small sample size (49/111), inadequate duration of follow-up (38/111) or intervention (37/111) and factors relating to outcomes (35/111). Evidence gaps from both systematic reviews and guidelines fell into the following categories: Respiratory (91); Gastrointestinal (20); PhysiotherapyandExercise (16); Musculoskeletal (6); Endocrine (4); Basic defect of CF (8); Psychosocial (2); Ears, Nose and Throat (1). CONCLUSIONS: We have compiled an up-to-date list of treatment uncertainties in CF and the reasons for these uncertainties. These can be used as a resource to aid researchers and funders when planning future trials. PROSPERO REGISTRATION NUMBER: Pre-results; CRD42015030111.


Assuntos
Tomada de Decisão Clínica , Fibrose Cística/terapia , Fibrose Cística/complicações , Fibrose Cística/diagnóstico , Humanos
16.
Thorax ; 2017 Apr 26.
Artigo em Inglês | MEDLINE | ID: mdl-28446662

RESUMO

Cochrane Reviews summarise best evidence and should inform guidelines. We assessed the use of Cochrane Reviews in the UK guidelines for paediatric respiratory disease. We found 21 guidelines which made 1025 recommendations, of which 96 could be informed by a Cochrane Review. In 38/96 recommendations (40%), some or all of the relevant Cochrane Reviews were not cited. We linked recommendations to 140 Cochrane Reviews. In 37/140 (26%) cases, the guideline recommendation did not fully agree with the Cochrane Review. Guideline developers may fail to use Cochrane Reviews or may make recommendations which are not in line with best evidence.

17.
Arch Dis Child ; 101(12): 1137-1141, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27573920

RESUMO

BACKGROUND: Ataxia telangiectasia (A-T) is a rare multisystem disease with high early mortality from lung disease and cancer. Nutritional failure adversely impacts outcomes in many respiratory diseases. Several factors influence nutrition in children with A-T. We hypothesised that children with A-T have progressive growth failure and that early gastrostomy tube feeding (percutaneous endoscopic gastrostomy, PEG) is a favourable management option with good nutritional outcomes. METHODS: Data were collected prospectively on weight, height and body mass index (BMI) at the national paediatric A-T clinic. Adequacy and safety of oral intake was assessed. Nutritional advice was given at each multidisciplinary review. RESULTS: 101 children (51 girls) had 222 measurements (32 once, 32 twice, 24 thrice) between 2009 and 2016. Median (IQR) age was 9.3 (6.4 to 13.1) years. Mean (SD) weight, height and BMI Z-scores were respectively -1 (1.6), -1.2 (1.2) and -0.4 (1.4). 35/101 children had weight Z-scores below -2 on at least one occasion. Weight, height and BMI Z-scores declined over time. Decline was most obvious after 8 years of age. 14/101 (14%) children had a PEG, with longitudinal data available for 12. In a nested case control study, there was a trend for improvement in weight in those with a PEG (p=0.10). CONCLUSIONS: Patients with A-T decline in growth over time. There is an urgent need for new strategies, including an understanding of why growth falters. We suggest early proactive consideration of PEG from age 8 years onwards to prevent progressive growth failure.


Assuntos
Ataxia Telangiectasia/complicações , Transtornos do Crescimento/etiologia , Estatura/fisiologia , Índice de Massa Corporal , Peso Corporal/fisiologia , Estudos de Casos e Controles , Criança , Nutrição Enteral/estatística & dados numéricos , Feminino , Transtornos do Crescimento/dietoterapia , Humanos , Masculino , Estado Nutricional/fisiologia , Estudos Prospectivos , Resultado do Tratamento
19.
Cochrane Database Syst Rev ; (7): CD009730, 2015 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-26226131

RESUMO

BACKGROUND: Cystic fibrosis is a multi-system disease characterised by the production of thick secretions causing recurrent pulmonary infection, often with unusual bacteria. Intravenous antibiotics are commonly used in the treatment of acute deteriorations in symptoms (pulmonary exacerbations); however, recently the assumption that exacerbations are due to increases in bacterial burden has been questioned. OBJECTIVES: To establish if intravenous antibiotics for the treatment of pulmonary exacerbations in people with cystic fibrosis improve short- and long-term clinical outcomes. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews and ongoing trials registers.Date of last search of Cochrane trials register: 27 July 2015. SELECTION CRITERIA: Randomised controlled trials and the first treatment cycle of cross-over studies comparing intravenous antibiotics (given alone or in an antibiotic combination) with placebo, inhaled or oral antibiotics for people with cystic fibrosis experiencing a pulmonary exacerbation. DATA COLLECTION AND ANALYSIS: The authors assessed studies for eligibility and risk of bias and extracted data. MAIN RESULTS: We included 40 studies involving 1717 participants. The quality of the included studies was largely poor and, with a few exceptions, these comprised of mainly small, inadequately reported studies.When comparing treatment with a single antibiotic to a combined antibiotic regimen, those participants receiving a combination of antibiotics experienced a greater improvement in lung function when considered as a whole group across a number of different measurements of lung function, but with very low quality evidence. When limited to the four placebo-controlled studies (n = 214), no difference was observed, again with very low quality evidence. With regard to the review's remaining primary outcomes, there was no effect upon time to next exacerbation and no studies in any comparison reported on quality of life. There were no effects on the secondary outcomes weight or adverse effects. When comparing specific antibiotic combinations there were no significant differences between groups on any measure. In the comparisons between intravenous and nebulised antibiotic or oral antibiotic (low quality evidence), there were no significant differences between groups on any measure. No studies in any comparison reported on quality of life. AUTHORS' CONCLUSIONS: The quality of evidence comparing intravenous antibiotics with placebo is poor. No specific antibiotic combination can be considered to be superior to any other, and neither is there evidence showing that the intravenous route is superior to the inhaled or oral routes. There remains a need to understand host-bacteria interactions and in particular to understand why many people fail to fully respond to treatment.


Assuntos
Antibacterianos/administração & dosagem , Fibrose Cística/tratamento farmacológico , Adolescente , Adulto , Antibacterianos/efeitos adversos , Criança , Fibrose Cística/fisiopatologia , Progressão da Doença , Humanos , Injeções Intravenosas , Capacidade Pulmonar Total/fisiologia
20.
J Cyst Fibros ; 13(4): 410-5, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24418187

RESUMO

BACKGROUND: It is unclear why cystic fibrosis (CF) survival has improved. We wished to quantify increases in CF median age of death in the context of general population survival improvement. METHOD: Death registration data analysis (US, England & Wales (E&W)-1972-2009). RESULTS: CF median age of death is higher in US than E&W and greater for males, opposite to that of death from all causes. CF median age of death has increased by 0.543 life years per year (E&W, US combined (95% confidence interval 0.506, 0.582)). The difference in median age at death between those dying from all causes and CF decreased in both territories. CF median age of death for males is greater than for females in both territories. This gap has not narrowed. CONCLUSION: The median age of death of people with CF is improving more rapidly than that of the general population in US and E&W.


Assuntos
Fibrose Cística/mortalidade , Expectativa de Vida , Vigilância da População/métodos , Sistema de Registros , Adolescente , Adulto , Distribuição por Idade , Fatores Etários , Causas de Morte/tendências , Criança , Pré-Escolar , Inglaterra/epidemiologia , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologia , País de Gales/epidemiologia , Adulto Jovem
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