Intravenous antibiotics for pulmonary exacerbations in people with cystic fibrosis.

Background: Cystic fibrosis is a multisystem disease characterised by the production of thick secretions causing recurrent pulmonary infection, often with unusual bacteria. Intravenous (IV) 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. This is an update of a previously published review.

Biomarkers in Ataxia-Telangiectasia: a Systematic Review.

Ataxia-Telangiectasia (A-T) is a very rare multisystem disease of DNA repair, associated with progressive disabling neurological symptoms, respiratory failure, immunodeficiency and cancer predisposition, leading to premature death. There are no curative treatments available for A-T but clinical trials have begun. A major limiting factor in effectively evaluating therapies for A-T is the lack of suitable outcome measures and biomarkers.

The Use of Diuretics in Infants with Established or Evolving Bronchopulmonary Dysplasia and Its Impact on the Duration of Home Oxygen Therapy.

Objective: Bronchopulmonary dysplasia (BPD), defined according to the level of respiratory support and supplemental oxygen administered at 36 weeks postmenstrual age, has multi-factorial causes. Diuretics have been used to prevent or treat established BPD and are the most frequently prescribed medication for the management of severe BPD. There is significant variation in the use of diuretics, and there is limited evidence showing improvement in medium to long term outcomes.

Infection prevention and control in cystic fibrosis: An update of a systematic review of interventions.

Preventing transmissible infection is a priority in cystic fibrosis (CF) care. This is an update of a systematic review of the evidence for infection prevention and control interventions in CF. Our full protocol can be found on PROSPERO (CRD42018109999).

Retrospective review of treatment outcomes and costs in children with sleep disordered breathing assessed with multi-channel studies.

Current UK guidance on OSA management recommends only 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.

Prospective evaluation of the impact of multi-channel studies on treatment outcomes in children with sleep disordered breathing.

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.

Prebiotics for people with cystic fibrosis.

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.

Bronchopulmonary dysplasia prediction models: a systematic review and meta-analysis with validation.

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.

The Clinical Research Bias Index (CRBI): A novel journal ranking method applied to child health respiratory studies.

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.

Association Between Treatments and Short-Term Biochemical Improvements and Clinical Outcomes in Post-Severe Acute Respiratory Syndrome Coronavirus-2 Inflammatory Syndrome.

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.

Assessing the impact of posture on diaphragm morphology and function using an open upright MRI system-A pilot study.

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.

Postprandial changes in gastrointestinal function and transit in cystic fibrosis assessed by Magnetic Resonance Imaging.

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.

Observational Study of Pulse Transit Time in Children With Sleep Disordered Breathing.

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. 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.

β-agonists do not work in children under 2 years of age: myth or maxim?

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Key paediatric messages from the 2018 European Respiratory Society International Congress.

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.

Gaps in the evidence for treatment decisions in cystic fibrosis: a systematic review.

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.

Do guidelines for treating chest disease in children use Cochrane Reviews effectively? A systematic review.

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.

Growth and nutrition in children with ataxia telangiectasia.

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.

The pharmacokinetics and toxicity of morning vs. evening tobramycin dosing for pulmonary exacerbations of cystic fibrosis: A randomised comparison.

Background: Circadian variation in renal toxicity of aminoglycosides has been demonstrated in animal and human studies. People with CF are frequently prescribed aminoglycosides. Altered pharmacokinetics of aminoglycosides are predictive of toxicity.

Intravenous antibiotics for pulmonary exacerbations in people with cystic fibrosis.

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.

Rate of improvement of CF life expectancy exceeds that of general population--observational death registration study.

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).