This comprehensive State of the Art review summarizes the current published knowledge base regarding the pathophysiology and microbiology of pulmonary disease in cystic fibrosis (CF). The molecular basis of CF lung disease including the impact of defective cystic fibrosis transmembrane regulator (CFTR) protein function on airway physiology, mucociliary clearance, and establishment of Pseudomonas aeruginosa infection is described. An extensive review of the microbiology of CF lung disease with particular reference to infection with P. aeruginosa is provided. Other pathogens commonly associated with CF lung disease including Staphylococcal aureus, Burkholderia cepacia, Stenotrophomonas maltophilia, Achromobacter xylosoxidans and atypical mycobacteria are also described. Clinical presentation and assessment of CF lung disease including diagnostic microbiology and other measures of pulmonary health are reviewed. Current recommendations for management of CF lung disease are provided. An extensive review of antipseudomonal therapies in the settings of treatment for early P. aeruginosa infection, maintenance for patients with chronic P. aeruginosa infection, and treatment of exacerbation in pulmonary symptoms, as well as antibiotic therapies for other CF respiratory pathogens, are included. In addition, the article discusses infection control policies, therapies to optimize airway clearance and reduce inflammation, and potential future therapies.

Pathophysiology and management of pulmonary infections in cystic fibrosis.

The diagnosis, investigation and particularly management of bronchiectasis has been largely empirical and the subject of relatively few controlled clinical trials. There are no clear guidelines, although an Australian position statement has been published concerning bronchiectasis in children. The purposes of these guidelines were therefore threefold: (1) to identify relevant studies in non-cystic fibrosis (CF) bronchiectasis; (2) to provide guidelines on management based on published studies where possible or a consensus view; and (3) to identify gaps in our knowledge and identify areas for future study.

https://thorax.bmj.com/content/thoraxjnl/65/Suppl_1/i1.full.pdf

British Thoracic Society guideline for non-CF bronchiectasis.

Sleep-disordered breathing, particularly the obstructive sleep apnea syndrome, is common during childhood. Complications include neurocognitive deficits, growth failure, and pulmonary hypertension. Nevertheless, sleep-disordered breathing is often unrecognized in children. New syndromes, such as the upper airway resistance syndrome, have recently been described. Polysomnography is invaluable for the evaluation of sleep-disordered breathing. Advances in diagnosis and management can alleviate much of the morbidity previously associated with sleep-related respiratory disorders.

Sleep-disordered breathing in children.

https://www.cysticfibrosisjournal.com/article/S1569-1993(02)00032-2/pdf

Nutrition in patients with cystic fibrosis: a European Consensus

### Steering group

Julia Bott (Chair), support to Section 6, Consultant Physiotherapist, Surrey PCT NW Locality

Sheric Ellum, support to Section 5, Consultant Physiotherapist, Guy's & St Thomas' NHS Trust, London

Dr Rachel Garrod, support to Section 1, Reader, School of Physiotherapy, Faculty of Health and Social Care Sciences, Kingston University and St George's, University of London.

Dr Jennifer Pryor, support to Sections 3 and 4, Senior Research Fellow in Physiotherapy, Royal Brompton & Harefield NHS Trust

Dr Lesley Rimington, support to Section 2, Lecturer School of Health and Rehabilitation, Keele University

### Section 1—COPD

Sharon Baines, Clinical Specialist Physiotherapist, Chronic Lung Disease Service, NHS Central Lancashire

Amanda Dryer, Physiotherapy Clinical Lead in Respiratory Care, Central Manchester and Manchester Children's University Hospital

Robert Goddard, Superintendent Physiotherapist, County Durham and Darlington NHS Foundation Trust

Catherine Thompson, Senior Lecturer, York St John University

Dr John White (Chair), Respiratory Physician, York Hospitals NHS Trust

### Section 2—Asthma and disordered breathing

Caroline Falconer (Co-Chair), Senior Physiotherapist, Papworth Hospital NHS Foundation Trust

Lianne Jongepier, Respiratory Specialist Physiotherapist, Service Lead COPD Team, Primary Care Centre, Colchester

Melanie Lincoln (Co-Chair), Team Leader Physiotherapist, Papworth Hospital NHS Foundation Trust

Christine Mikelsons, Consultant Respiratory Physiotherapist, Royal Free Hospital

Dr Mike Thomas, General Practitioner, Asthma UK Senior Research Fellow, University of Aberdeen

Jo Williams, Senior Pulmonary Rehabilitation Specialist Glenfield Hospital, University Hospitals of Leicester NHS Trust

### Section 3—Cystic fibrosis

On behalf of the Association of Chartered Physiotherapists in Cystic Fibrosis

Penny Agent, Service Lead Physiotherapist, Royal Brompton & Harefield NHS Trust

Gillian Davie, Senior I Physiotherapist, Cystic Fibrosis Team, Aberdeen Royal Infirmary

Mary Dodd, Consultant Physiotherapist in Cystic Fibrosis, University Hospital of South Manchester NHS Foundation Trust

Dr Sarah Elkin, Respiratory Physician, St Mary's Hospital, London

Tracey Hughes (Chair), Senior I Physiotherapist, Leeds Regional Adult Cystic Fibrosis Unit, Leeds Teaching Hospitals NHS Trust

Margaret MacLeod, Senior I Physiotherapist, Cystic Fibrosis Team, Aberdeen Royal Infirmary

Nicola …

/pdf/guidelines-for-the-physiotherapy-management-of-the-adult-9g87sbxufz.pdf

Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient

Nocturnal Nasal IPPV Stabilizes Patients with Cystic Fibrosis and Hypercapnic Respiratory Failure

Benefits of Nocturnal Nasal CPAP in Patients With Cystic Fibrosis

Background and Purpose. The purpose of this investgation was to measure the effects of a 10- to 14-day comprehensive, intensive hospital treatment program on peak exercise capacity, endurance capacity, respiratory function, weight change, and maximum inspiratory and expiratory mouth pressures in patients with cystic fibrosis with a pulmonary exacerbation. Subjects. Fourteen young adults with cystic fibrosis admitted to a hospital for an exacerbation of their pulmonary disease were studied. Methods. Subjects performed pulmonary function tests, inspiratory and expiratory mouth pressure tests, and stationary bicycle exercise tests at admission and discharge. Comprehensive therapy provided during the hospital admission consisted of intravenous antibiotics, physical therapy, high-calorie diet, and daily medical review. Results. The patients showed improvements in forced expiratory volume in 1 second (46%–55% of predicted values) and forced vital capacity (62%–68% of predicted values). Maximum inspiratory and expiratory mouth pressures also improved (118%–131% and 78%–92% of predicted values, respectively). There was a mean weight gain of 2 kg. Maximum work capacity on a bicycle ergometer improved from a mean of 45% to 52% of predicted values. The most impressive result was the marked increase in exercise endurance time from a mean of 9.5 minutes on admission to 16.6 minutes at discharge. Conclusion and Discussion. This study indicates that young adults with cystic fibrosis and an exacerbation of their pulmonary disease obtain measurable benefits from a comprehensive, intensive treatment program, particularly improvement in their capacity for endurance exercise.

The Effect of a Comprehensive, Intensive Inpatient Treatment Program on Lung Function and Exercise Capacity in Patients With Cystic Fibrosis

sleep (nCPAP89± 6% vs control 83± 6%,p<0.05). Transcutaneous CO2measurements were notsignificantly different between thecontrol andthenCPAP studies. TheRDI was alsosignificantly reduced with nCPAPespecially during REM sleep (9 ± 7events per hour vscontrol 25± 11events perhour, p<0.05). Nasal CPAPcaused no change intotal sleep timeorsleep efficiency yetsignificantly reduced theRDIandimproved baseline SaO2during bothNREM andREM sleep. (Chest 1994; 106:1717-24)

Sally Parker

Papers

Nocturnal Nasal IPPV Stabilizes Patients with Cystic Fibrosis and Hypercapnic Respiratory Failure

Benefits of Nocturnal Nasal CPAP in Patients With Cystic Fibrosis

The Effect of a Comprehensive, Intensive Inpatient Treatment Program on Lung Function and Exercise Capacity in Patients With Cystic Fibrosis

Benefits ofNocturnal NasalCPAPin Patients WithCystic Fibrosis

WS04.06 Neoplastic disease and treatment in cystic fibrosis (CF): a comprehensive single centre case series