BRONCHIECTASIS and PRIMARY CILIARY DYSKINESIA
The September 2011 issue of Paediatric Respiratory Reviews is devoted to non-cystic paediatric bronchiectasis (volume 12, issue 2).

BTS GUIDELINES (2010)
www.brit-thoracic.org.uk/Portals/0/Clinical%20Information/Bronchiectasis/non-CF-Bronchiectasis-guideline.pdf


Angrill J, Agusti C, de Celis R (2002) Bacterial colonization in patients with bronchiectasis. Thorax, 57, 15-19.
thorax.bmjjournals.com/cgi/content/full/57/1/15?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=Angrill+J&andorexactfulltext=and&searchid=1121537321503_1039&stored_search=&FIRSTINDEX=0&sortspec=relevance&volume=57&firstpage=15&resourcetype=1&journalcode=thoraxjnl
• periodic sputum culture needed to identify bugs, which colonise distal airways of 60% of patients

Barker AF (2000) Tobramycin solution for inhalation reduces sputum pseudomonas aeruginosa density in bronchiectasis. Am J Respir Crit Care Med, 62, 481-485
ajrccm.atsjournals.org/cgi/content/full/162/2/481
• inhaled tobramycin reduces sputum P. aeruginosa density

Barker AF (2002) Bronchiectasis. New Eng J Med, 346, 1383-93
• comprehensive information

Benhamou D, Muir JF, Radpaud C (1997) Long-term efficiency of home nasal mask ventilation in patients with diffuse bronchiectasis and severe chronic respiratory failure. Chest, 112, 1259-66
www.chestjournal.org/cgi/reprint/112/5/1259?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=Benhamou+D&andorexactfulltext=and&searchid=1121540953305_3840&stored_search=&FIRSTINDEX=0&sortspec=relevance&resourcetype=1&journalcode=chest
• NIV has shown reduced hospitalizations and improved functional status in diffuse disease

Bradley J, Moran F (2006) Pulmonary rehabilitation improves exercise tolerance in patients with bronchiectasis. Austr J Physiother, 52, 65.
• commentary on article describing improved exercise tolerance in people with bronchiectasis

Bush A, Cole P, Hariri M (1998) Primary ciliary dyskinesia: diagnosis and standards of care. Eur Resp J, 12, 982-8
erj.ersjournals.com/cgi/content/abstract/12/4/982?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=Bush+A&andorexactfulltext=and&searchid=1121541384825_558&stored_search=&FIRSTINDEX=0&sortspec=relevance&volume=12&firstpage=982&resourcetype=1&journalcode=erj

Cowan MJ, Gladwin MT, Shelhamer JH (2001) Disorders of ciliary motility. Am J Med Sci, 321, 3-10

Daviskas E (1999) Inhalation of dry powder mannitol improves clearance of mucus in patients with bronchiectasis. Am J Respir Crit Care Med, 159, 1843-8
ajrccm.atsjournals.org/cgi/content/full/159/6/1843?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=Daviskas+E+&searchid=1121542701290_2369&stored_search=&FIRSTINDEX=0&volume=159&firstpage=1843&journalcode=ajrccm
• dry powder mannitol decreases sputum volume

Dentice R (2009) Airway clearance physiotherapy improves quality of life in people with bronchiectasis. Austr J Physiotherapy, 55, 4, 285
proquest.umi.com/pqdweb?index=4&did=1936835881&SrchMode=3&sid=1&Fmt=6&VInst=PROD&VType=PQD&RQT=309&VName=PQD&TS=1269187944&clientId=64672&aid=2
• Acapella b.d. improves quality of life and exercise capacity

Dhillon SS (2000) Lady Windermere syndrome: middle lobe bronchiectasis and mycobacterium avium complex infection due to voluntary cough suppression. Clin Infectious Dis, 30, 3, 572-575
• cough suppression should not be used for social reasons

Eaton T, Young P, Zeng I, et al (2007) A randomized evaluation of the acute efficacy, acceptability and tolerability of flutter and active cycle of breathing with and without postural drainage in non-cystic fibrosis bronchiectasis. Chron Respir Dis, 4, 23–30
crd.sagepub.com/content/4/1/23.abstract?ijkey=18cc79bd2061f7685b648226fab353b3264eedfe&keytype2=tf_ipsecsha
• ACB has shown superiority to the Flutter or postural drainage

Edwards EA, Narang I, Li A, et al (2004) HRCT lung abnormalities are not a surrogate for exercise limitation in bronchiectasis. Eur Respir J, 24, 538 - 44
erj.ersjournals.com/cgi/content/abstract/24/4/538
• high resolution computed tomography is not related to exercise capacity

Feng W, Deng WW (1998) Short-term efficacy of RC-Cornet in bronchiectasis. Chest, 114, 320
• the Cornet decreases sputum viscosity

Goeminne P (2010) Non-cystic fibrosis bronchiectasis: diagnosis and management in 21st century. Postgrad Med J, 86, 493-501
pmj.bmj.com/content/86/1018/493.full
• patients need regular screening for infection
• inhaled mannitol may reduce the tenacity of mucus

Jones A, Rowe BH (2000) Bronchopulmonary hygiene physical therapy in bronchiectasis and COPD. Heart Lung, 29, 125-9
www.heartandlung.org/article/PIIS0147956300900086/abstract
• systematic review but research on chest clearance for bronchiectasis is limited

Kellet F, Robert NM (2011) Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis. Respir Med, 105, 12, 1831-5

King PT (2009) The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis, 4, 411-9
www.ncbi.nlm.nih.gov/pubmed/20037680?dopt=Abstract

King PT, Daviskas E (2010) Pathogenesis and diagnosis of bronchiectasis. Breathe, 6, 342-51
www.ers-education.org/pages/default.aspx?id=2041
• short and sweet, with pretty pictures.

Lavery K, O'Neill B, Elborn JS, Reilly J, Bradley JM (2007) Self-management in bronchiectasis: the patients' perspective. Eur Respir J, 29, 541-7
erj.ersjournals.com/cgi/content/full/29/3/541
• reported benefits of physiotherapy include reduced shortness of breath, increased exercise capacity and better quality of life

Lee AL (2010) The effects of pulmonary rehabilitation in patients with non-cystic fibrosis bronchiectasis. BMC Pulm Med, 10, 5, doi:10.1186/1471-2466-10-5
www.biomedcentral.com/1471-2466/10/5

Martinez-Garcia MA, Perpina-Tordera M, Roman-Sanchez P (2005) Quality of life determinants in patients with clinically stable bronchiectasis. Chest, 128, 739–45
chestjournal.chestpubs.org/content/128/2/739.full
• sputum and breathlessness are the main symptoms affecting quality of life

Maziθres J (2003) Limited operation for severe multisegmental bilateral bronchiectasis.
Ann Thorac Surg; 75, 382-387
• limited surgery may be indicated in non-localized bilateral bronchiectasis with cystic and functionless areas

Meeks M (2000) Primary ciliary dyskinesia. Ped Pulmonol, 29, 307-16
www3.interscience.wiley.com/cgi-bin/abstract/71001853/ABSTRACT

Murray MP, Pentland JL, Hill AT (2009) A randomised crossover trial of chest physiotherapy in non-cystic fibrosis bronchiectasis. Eur Respir J, 34,1086-109
• 3 months of twice daily oscillatory positive expiratory pressure increases sputum clearance, exercise capacity and SGRQ score

Murray MP, Pentland JL, Turnbull K (2009) Sputum colour: a useful clinical tool in non-cystic fibrosis bronchiectasis. Eur Respir J, 34, 361-4
erj.ersjournals.com/cgi/content/abstract/34/2/361
• patient’s interpretation of sputum colour is reliable

Newall C, Stockley RA, Hill SL (2005) Exercise training and inspiratory muscle training in patients with bronchiectasis. Thorax, 60, 943–8
thorax.bmj.com/cgi/content/full/60/11/943
• pulmonary rehabilitation improves exercise tolerance, and inspiratory muscle training may extend the training effect

O’Callaghan C (2007) Diagnosing primary ciliary dyskinesia. Thorax, 62, 656-7

O'Donnell AE (2008) Bronchiectasis. Chest, 134, 4, 815-23
chestjournal.chestpubs.org/content/134/4/815.full
• pathology, diagnosis and treatment

O’Donnell AE (2011) Bronchiectasis in patients with COPD: a distinct COPD phenotype?
Chest, 140, 5 1107-1108

O'Leary CJ, Wilson CB, Hansell DM (2002) Relationship between psychological well-being and lung health status in patients with bronchiectasis. Respir Med, 96, 686–92
www.ncbi.nlm.nih.gov/pubmed/12243314?dopt=Abstract
• 34% of patients had elevated scores for anxiety and/or depression, with anxiety being the most common

O’Neill B, Bradley JM, McArdle (2002) The current physiotherapy management of patients with bronchiectasis. Int J Clin Pract, 56, 1, 34-5
www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11831830&dopt=Abstract

Ong HK, Lee AL, Hill CJ, Holland AE, Denehy L (2011) Effects of pulmonary rehabilitation in bronchiectasis. Chr Respir Dis, 8, 1, 21-31
• retrospective study supporting pulmonary rehabilitation for bronchiectasis patients

Patel AS, Watkin G, Willig B et al (2011) Improvement in health status following cough-suppression physiotherapy for patients with chronic cough. Chron Respir Dis, 8, 253-8
crd.sagepub.com/content/8/4/253.abstract?etoc
• control of unnecessary coughing by education, breathing retraining and vocal hygiene

Patterson JE, Bradley JM, Hewitt O (2005) Airway clearance in bronchiectasis:
a randomized crossover trial of active cycle of breathing techniques versus Acapella. Respiration, 72, 239–242. DOI: 10.1159/000085363
• ACBT and the Acapella are equally effective, but patients often prefer the Acapella.

Phillips GE (1998) Airway response of children with primary ciliary dyskinesia in exercise and β2 agonist challenge. Eur Resp J, 11, 1389-91
• exercise is more beneficial than drugs in aiding bronchodilation before physiotherapy

Rea H, McAuley S, Donnell O et al (2004) Home based humidification therapy for patients with COPD and bronchiectasis. Eur Respir J, 24: Suppl. 48, 31s
www.ersnet.org/ers/lr/browse/media.aspx?id_dossier=14546&id_fiche=117613
• COPD & bronchiectasis pts find home humidification eases their cough and sputum production

Rea H, McAuley S, Jayaram L (2010) The clinical utility of long-term humidification therapy in chronic airway disease. Respir Med, 104, 525-33
• patients on long-term humidification had fewer exacerbations, higher QoL scores and improved lung function than those without.

Silverman D (2003) Current management of bronchiectasis Heart Lung, 32, 59-64
• review of pathogenesis, diagnosis and treatment – limited information on physiotherapy and it doesn’t mention breathing techniques!

Smith VM, Shoemark A, Nisbet M (2010) When to think of bronchiectasis and the investigations to perform. Clinical Pulm Med, 17, 1, 7-13
• comprehensive report on causes, diagnosis and management.

Steinfort CL, Smith MJ, Harrison NK (1987) Reactive arthritis in bronchiectasis. Am J Resp Dis, 135, A42
• RA associated with bronchiectasis shows improvement with antibiotic treatment for the bronchiectasis

Syed N (2009) Active Cycles of Breathing Technique (ACBT) versus conventional chest physical therapy on airway clearance in bronchiectasis – a crossover trial. Advances in Physiotherapy, 11, 4, 193-8
• both techniques equally effective, but patients tend to prefer ACBT

Tomkinson JL, Bruton A (2009) The 6-minute walk test for patients with bronchiectasis. ACPRC Journal, 41, 16-21
• people with bronchiectasis show same reduction in exercise tolerance as COPD

Wilson CB, Jones PW, O'Leary CJ, Cole PJ, Wilson R (1997) Validation of the St. George's Respiratory Questionnaire in bronchiectasis. Am J Respir Crit Care Med, 156, 2, 1, 536-41
ajrccm.atsjournals.org/cgi/content/abstract/156/2/536
• the SGRQ is a valid measure of health for people with bronchiectasis






ACBT = active cycle of breathing
ACPRC = Association of Chartered Physiotherapists in Respiratory Care
BTS = British Thoracic Society
NIV = non-invasive ventilation
SGRQ = St George's Respiratory Questionnaire (scores symptoms, activity, social & psychological function)
SOB = shortness of breath