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Singhal KK, Singh R. Chronic Suppurative Lung Disease in Children: A Case Based Approach. Indian J Pediatr 2023; 90:920-926. [PMID: 37389774 DOI: 10.1007/s12098-023-04665-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/17/2023] [Accepted: 05/04/2023] [Indexed: 07/01/2023]
Abstract
Bronchiectasis is a pathologic state of conducting airways manifested radiographically by evidence of bronchial dilation and clinically by chronic productive cough. Considered an "orphan disease" for long, it remains a major contributor to morbidity and mortality in both developed and underdeveloped countries. With the advances in the medical field accompanied by widespread access to vaccines and antibiotics, improved health services and better access to nutrition, the incidences of bronchiectasis have markedly decreased, particularly in developed countries. This review summarizes the current knowledge pertaining to the clinical definition, etiology, clinical approach and management related to pediatric bronchiectasis.
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Affiliation(s)
- Kamal Kumar Singhal
- Division of Pediatric Pulmonology, Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, Shaheed Bhagat Singh Marg, New Delhi, India.
| | - Robin Singh
- Division of Pediatric Pulmonology, Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, Shaheed Bhagat Singh Marg, New Delhi, India
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Gallucci M, Candela E, Di Palmo E, Miniaci A, Pession A. Non-Cystic Fibrosis Bronchiectasis in Pediatric Age: A Case Series in a Metropolitan Area of Northern Italy. CHILDREN 2022; 9:children9091420. [PMID: 36138729 PMCID: PMC9497485 DOI: 10.3390/children9091420] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 08/08/2022] [Revised: 09/12/2022] [Accepted: 09/17/2022] [Indexed: 11/24/2022]
Abstract
Non-cystic fibrosis bronchiectasis is an emergent disease characterized by endobronchial suppuration, dilated airways with neutrophilic inflammation and chronic wet cough due to recurrent lower airway infections. A regular clinical follow-up and adequate management of exacerbations are essential to reduce symptoms and the worsening of lung injury. We report a retrospective study comprising 15 children and adolescents with NCFB followed in our hospital center of pediatric pulmonology. We retrospectively analyzed the main comorbidities associated with the presence of NCFB, the radiological aspect associated with the different etiologies and the therapeutic approach used. We also emphasized the importance of an effective preventive strategy to reduce and prevent pulmonary exacerbations.
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Affiliation(s)
- Marcella Gallucci
- Pediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy
| | - Egidio Candela
- Specialty School of Pediatrics, Alma Mater Studiorum, University of Bologna, 40126 Bologna, Italy
- Correspondence: or ; Tel.: +39-3473878582
| | - Emanuela Di Palmo
- Pediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy
| | - Angela Miniaci
- Pediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy
| | - Andrea Pession
- Pediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy
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Rojas C, Gálvez-Jirón F, De Solminihac J, Padilla C, Cárcamo I, Villalón N, Kurte M, Pino-Lagos K. Crosstalk between Body Microbiota and the Regulation of Immunity. J Immunol Res 2022; 2022:6274265. [PMID: 35647199 PMCID: PMC9135571 DOI: 10.1155/2022/6274265] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2022] [Revised: 04/19/2022] [Accepted: 04/25/2022] [Indexed: 11/26/2022] Open
Abstract
The microbiome corresponds to the genetic component of microorganisms (archaea, bacteria, phages, viruses, fungi, and protozoa) that coexist with an individual. During the last two decades, research on this topic has become massive demonstrating that in both homeostasis and disease, the microbiome plays an important role, and in some cases, a decisive one. To date, microbiota have been identified at different body locations, such as the eyes, lung, gastrointestinal and genitourinary tracts, and skin, and technological advances have permitted the taxonomic characterization of resident species and their metabolites, in addition to the cellular and molecular components of the host that maintain a crosstalk with local microorganisms. Here, we summarize recent studies regarding microbiota residing in different zones of the body and their relationship with the immune system. We emphasize the immune components underlying pathological conditions and how they interact with local (and distant) microbiota.
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Affiliation(s)
- Carolina Rojas
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
- Facultad de Odontología, Universidad de Chile, Santiago, Chile
| | - Felipe Gálvez-Jirón
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
| | - Javiera De Solminihac
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
| | - Cristina Padilla
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
| | - Ignacio Cárcamo
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
| | - Natalia Villalón
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
| | - Mónica Kurte
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
| | - Karina Pino-Lagos
- Facultad de Medicina, Centro de Investigación e Innovación Biomédica, Universidad de los Andes, Santiago, Chile
- IMPACT, Center of Interventional Medicine for Precision and Advanced Cellular Therapy, Santiago, Chile
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Kapur N, Stroil-Salama E, Morgan L, Yerkovich S, Holmes-Liew CL, King P, Middleton P, Maguire G, Smith D, Thomson R, McCallum G, Owens L, Chang AB. Factors associated with "Frequent Exacerbator" phenotype in children with bronchiectasis: The first report on children from the Australian Bronchiectasis Registry. Respir Med 2021; 188:106627. [PMID: 34592538 DOI: 10.1016/j.rmed.2021.106627] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2021] [Revised: 09/16/2021] [Accepted: 09/23/2021] [Indexed: 10/20/2022]
Abstract
INTRODUCTION In adults with bronchiectasis, multicentre data advanced the field including disease characterisation and derivation of phenotypes such as 'frequent exacerbator (FE)' (≥3 exacerbations/year). However, paediatric cohorts are largely limited to single centres and no scientifically derived phenotypes of paediatric bronchiectasis yet exists. Using paediatric data from the Australian Bronchiectasis Registry (ABR), we aimed to: (a) describe the clinical characteristics and compare Indigenous with non-Indigenous children, and (b) determine if a FE phenotype can be identified and if so, its associated factors. METHODS We retrieved data of children (aged <18-years) with radiologically confirmed bronchiectasis, enrolled between March 2016-March 2020. RESULTS Across five sites, 540 children [288 Indigenous; median age = 8-years (IQR 6-11)] were included. Baseline characteristics revealed past infection/idiopathic was the commonest (70%) underlying aetiology, most had cylindrical bronchiectasis and normal spirometry. Indigenous children (vs. non-Indigenous) had significantly more environmental tobacco smoke exposure (84% vs 32%, p < 0.0001) and lower birth weight (2797 g vs 3260 g, p < 0.0001). FE phenotype present in 162 (30%) children, was associated with being younger (ORadjusted = 0.85, 95%CI 0.81-0.90), more recent diagnosis of bronchiectasis (ORadjusted = 0.67; 95%CI 0.60-0.75), recent hospitalization (ORadj = 4.51; 95%CI 2.45-8.54) and Pseudomonas aeruginosa (PsA) infection (ORadjusted = 2.43; 95%CI 1.01-5.78). The FE phenotype were less likely to be Indigenous (ORadjusted = 0.14; 95%CI 0.03-0.65). CONCLUSION Even within a single country, the characteristics of children with bronchiectasis differ among cohorts. A paediatric FE phenotype exists and is characterised by being younger with a more recent diagnosis, PsA infection and previous hospitalization. Prospective data to consolidate our findings characterising childhood bronchiectasis phenotypes are required.
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Affiliation(s)
- Nitin Kapur
- Department of Respiratory & Sleep Medicine, Queensland Children's Hospital and Faculty of Medicine, University of Queensland, QLD, Australia.
| | | | - Lucy Morgan
- Concord Clinical School, Faculty of Medicine and Health, University of Sydney, NSW, Australia
| | - Stephanie Yerkovich
- Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, NT, Australia; Australian Centre for Health Services Innovation, Queensland University of Technology, Australia
| | - Chien-Li Holmes-Liew
- Department of Thoracic Medicine, Royal Adelaide Hospital, South Australia, Australia
| | - Paul King
- Monash Respiratory and Sleep Medicine, Monash Medical Centre, VIC, Australia
| | - Peter Middleton
- Department of Respiratory & Sleep Medicine, Westmead Hospital, Westmead, NSW, Australia
| | - Graeme Maguire
- Western Clinical School, University of Melbourne, Melbourne, VIC, Australia
| | - Daniel Smith
- Thoracic Medicine, The Prince Charles Hospital, Brisbane, QLD, Australia
| | - Rachel Thomson
- Department of Respiratory Medicine, Greenslopes Private Hospital and Gallipoli Medical Research Institute, University of Queensland, Greenslopes, QLD, Australia
| | - Gabrielle McCallum
- Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, NT, Australia
| | - Louisa Owens
- Department of Respiratory Medicine, Sydney Children's Hospital, NSW, Australia
| | - Anne B Chang
- Department of Respiratory & Sleep Medicine, Queensland Children's Hospital and Faculty of Medicine, University of Queensland, QLD, Australia; Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, NT, Australia; Australian Centre for Health Services Innovation, Queensland University of Technology, Australia
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Eralp EE, Gokdemir Y, Atag E, Ikizoglu NB, Ergenekon P, Yegit CY, Kut A, Ersu R, Karakoc F, Karadag B. Changing clinical characteristics of non-cystic fibrosis bronchiectasis in children. BMC Pulm Med 2020; 20:172. [PMID: 32546272 PMCID: PMC7298950 DOI: 10.1186/s12890-020-01214-7] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2019] [Accepted: 06/11/2020] [Indexed: 12/14/2022] Open
Abstract
Background The prevalence of non-cystic fibrosis (CF) bronchiectasis is increasing in both developed and developing countries in recent years. Although the main features remain similar, etiologies seem to change. Our aim was to evaluate the clinical and laboratory characteristics of our recent non-CF bronchiectasis patients and to compare these with our historical cohort in 2001. Methods One hundred four children with non-CF bronchiectasis followed between 2002 and 2019 were enrolled. Age of diagnosis, underlying etiology and microorganisms in sputum culture were recorded. Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months. Results Mean FEV1 and FVC %predicted at presentation improved compared to historical cohort (76.6 ± 17.1 vs. 63.3 ± 22.1 and 76.6 ± 15.1 vs. 67.3 ± 23.1, respectively; p < 0.001). There was a significant decrease in pulmonary exacerbation rate from 6.05 ± 2.88 at presentation to 3.23 ± 2.08 during follow-up (p < 0.0001). In 80.8% of patients, an underlying etiology was identified. There was an increase in primary ciliary dyskinesia (PCD) (32.7% vs. 6.3%; p = 0.001), decrease in idiopathic cases (19.2% vs. 37.8%; p = 0.03) with no change in postinfectious and immunodeficiencies as underlying etiology. Sputum cultures were positive in 77.9% of patients which was 46.9% in the historical cohort (p = 0.001). Conclusion Baseline pulmonary function tests were better and distribution of underlying etiology had changed with a remarkable increase in diagnosis of PCD in the recent cohort.
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Affiliation(s)
- Ela Erdem Eralp
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey.
| | - Yasemin Gokdemir
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
| | - Emine Atag
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
| | - Nilay Bas Ikizoglu
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
| | - Pinar Ergenekon
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
| | - Cansu Yilmaz Yegit
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
| | - Arif Kut
- Division of Pediatric Pulmonology, Maltepe University, School of Medicine, Istanbul, Turkey
| | - Refika Ersu
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
| | - Fazilet Karakoc
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
| | - Bulent Karadag
- Division of Pediatric Pulmonology, Marmara University, School of Medicine, Istanbul, Turkey
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Bush A, Floto RA. Pathophysiology, causes and genetics of paediatric and adult bronchiectasis. Respirology 2019; 24:1053-1062. [PMID: 30801930 DOI: 10.1111/resp.13509] [Citation(s) in RCA: 26] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2018] [Accepted: 01/16/2019] [Indexed: 02/06/2023]
Abstract
Bronchiectasis has historically been considered to be irreversible dilatation of the airways, but with modern imaging techniques it has been proposed that 'irreversible' be dropped from the definition. The upper limit of normal for the ratio of airway to arterial development increases with age, and a developmental perspective is essential. Bronchiectasis (and persistent bacterial bronchitis, PBB) is a descriptive term and not a diagnosis, and should be the start not the end of the patient's diagnostic journey. PBB, characterized by airway infection and neutrophilic inflammation but without significant airway dilatation may be a precursor of bronchiectasis, and there are many commonalities in the microbiology and the pathology, which are reviewed in this article. A high index of suspicion is essential, and a history of chronic wet or productive cough for more than 4-8 weeks should prompt investigation. There are numerous underlying causes of bronchiectasis, although in many cases no cause is found. Causes include post-infectious, especially after tuberculosis, adenoviral or pertussis infection; aspiration syndromes; defects in host defence, which may solely affect the airways (cystic fibrosis, not considered in this review, and primary ciliary dyskinesia); and primary ciliary dyskinesia or be systemic, such as common variable immunodeficiency; genetic syndromes; and anatomical defects such as intraluminal airway obstruction (e.g. foreign body), intramural obstruction (e.g. complete cartilage rings) and external airway compression (e.g. by tuberculous lymph nodes). Identification of the underlying cause is important, because some of these conditions have specific treatments and others genetic implications for the family.
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Affiliation(s)
- Andrew Bush
- Department of Paediatrics, Imperial College, London, UK.,Department of Paediatric Respirology, National Heart and Lung Institute, London, UK.,Royal Brompton Harefield NHS Foundation Trust, London, UK
| | - R Andres Floto
- Department of Respiratory Biology, University of Cambridge, Cambridge, UK.,Cambridge Centre for Lung Infection, Royal Papworth Hospital, Cambridge, UK
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Chang AB, Redding GJ. Bronchiectasis and Chronic Suppurative Lung Disease. KENDIG'S DISORDERS OF THE RESPIRATORY TRACT IN CHILDREN 2019. [PMCID: PMC7161398 DOI: 10.1016/b978-0-323-44887-1.00026-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
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Gallucci M, di Palmo E, Bertelli L, Camela F, Ricci G, Pession A. A pediatric disease to keep in mind: diagnostic tools and management of bronchiectasis in pediatric age. Ital J Pediatr 2017; 43:117. [PMID: 29284507 PMCID: PMC5747121 DOI: 10.1186/s13052-017-0434-0] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/08/2017] [Accepted: 12/05/2017] [Indexed: 12/21/2022] Open
Abstract
Bronchiectasis in pediatric age is a heterogeneous disease associated with significant morbidity.The most common medical conditions leading to bronchial damage are previous pneumonia and recurrent lower airway infections followed by underlying diseases such as immune-deficiencies, congenital airway defects, recurrent aspirations and mucociliary clearance disorders.The most frequent symptom is chronic wet cough. The introduction of high-resolution computed tomography (HRCT) has improved the time of diagnosis allowing earlier treatment.However, the term "bronchiectasis" in pediatric age should be used with caution, since some lesions highlighted with HRCT may improve or regress. The use of chest magnetic resonance imaging (MRI) as a radiation-free technique for the assessment and follow-up of lung abnormalities in non-Cystic Fibrosis chronic lung disease is promising.Non-Cystic Fibrosis Bronchiectasis management needs a multi-disciplinary team. Antibiotics and airway clearance techniques (ACT) represent the pillars of treatment even though guidelines in children are lacking. The Azithromycin thanks to its antinflammatory and direct antimicrobial effect could be a new strategy to prevent exacerbations.
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Affiliation(s)
- Marcella Gallucci
- Pediatric Unit, Department of Medical and Surgical Sciences, S. Orsola - Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138, Bologna, Italy
| | - Emanuela di Palmo
- Pediatric Unit, Department of Medical and Surgical Sciences, S. Orsola - Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138, Bologna, Italy
| | - Luca Bertelli
- Pediatric Unit, Department of Medical and Surgical Sciences, S. Orsola - Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138, Bologna, Italy
| | - Federica Camela
- Pediatric Unit, Department of Medical and Surgical Sciences, S. Orsola - Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138, Bologna, Italy
| | - Giampaolo Ricci
- Pediatric Unit, Department of Medical and Surgical Sciences, S. Orsola - Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138, Bologna, Italy.
| | - Andrea Pession
- Pediatric Unit, Department of Medical and Surgical Sciences, S. Orsola - Malpighi Hospital, University of Bologna, Via Massarenti 11, 40138, Bologna, Italy
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Moustaki M, Loukou I, Priftis KN, Douros K. Role of vitamin D in cystic fibrosis and non-cystic fibrosis bronchiectasis. World J Clin Pediatr 2017; 6:132-142. [PMID: 28828295 PMCID: PMC5547424 DOI: 10.5409/wjcp.v6.i3.132] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/11/2017] [Revised: 02/27/2017] [Accepted: 06/13/2017] [Indexed: 02/06/2023] Open
Abstract
Bronchiectasis is usually classified as cystic fibrosis (CF) related or CF unrelated (non-CF); the latter is not considered an orphan disease any more, even in developed countries. Irrespective of the underlying etiology, bronchiectasis is the result of interaction between host, pathogens, and environment. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects such as down-regulation of pro-inflammatory cytokines and chemokines. Respiratory epithelial cells constitutively express 1α-hydroxylase leading to the local transformation of the inactive 25(OH)-vitamin D to the active 1,25(OH)2-vitamin D. The latter through its autocrine and paracrine functions up-regulates vitamin D dependent genes with important consequences in the local immunity of lungs. Despite the scarcity of direct evidence on the involvement of vitamin D deficiency states in the development of bronchiectasis in either CF or non-CF patients, it is reasonable to postulate that vitamin D may play some role in the pathogenesis of lung diseases and especially bronchiectasis. The potential contribution of vitamin D deficiency in the process of bronchiectasis is of particular clinical importance, taking into consideration the increasing prevalence of vitamin D deficiency worldwide and the significant morbidity of bronchiectasis. Given the well-established association of vitamin D deficiency with increased inflammation, and the indicative evidence for harmful consequences in lungs, it is intriguing to speculate that the administration of vitamin D supplementation could be a reasonable and cost effective supplementary therapeutic approach for children with non-CF bronchiectasis. Regarding CF patients, maybe in the future as more data become available, we have to re-evaluate our policy on the most appropriate dosage scheme for vitamin D.
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Ishak A, Everard ML. Persistent and Recurrent Bacterial Bronchitis-A Paradigm Shift in Our Understanding of Chronic Respiratory Disease. Front Pediatr 2017; 5:19. [PMID: 28261574 PMCID: PMC5309219 DOI: 10.3389/fped.2017.00019] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2016] [Accepted: 01/24/2017] [Indexed: 02/01/2023] Open
Abstract
The recent recognition that the conducting airways are not "sterile" and that they have their own dynamic microbiome, together with the rapid advances in our understanding of microbial biofilms and their roles in the causation of respiratory diseases (such as chronic bronchitis, sinusitis, and chronic otitis media), permit us to update the "vicious circle" hypothesis of the causation of bronchiectasis. This proposes that chronic inflammation driven by persistent bacterial bronchitis (PBB) causes damage to both the epithelium, resulting in impaired mucociliary clearance, and to the airway wall, which eventually manifests as bronchiectasis. The link between a "chronic bronchitis" and a persistence of bacterial pathogens, such as non-typable Haemophilus influenzae, was first made more than 100 years ago, and its probable role in the causation of bronchiectasis was proposed soon afterward. The recognition that the "usual suspects" are adept at forming biofilms and hence are able to persist and dominate the normal dynamically changing "healthy microbiome" of the conducting airways provides an explanation for the chronic colonization of the bronchi and for the associated chronic neutrophil-dominated inflammation characteristic of a PBB. Understanding the complex interaction between the host and the microbial communities of the conducting airways in health and disease will be a key component in optimizing pulmonary health in the future.
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Affiliation(s)
- Alya Ishak
- Department of Respiratory Medicine, Princess Margaret Hospital, Subiaco, WA, Australia
| | - Mark L. Everard
- Department of Respiratory Medicine, Princess Margaret Hospital, Subiaco, WA, Australia
- University of Western Australia, Crawley, WA, Australia
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11
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McCallum GB, Binks MJ. The Epidemiology of Chronic Suppurative Lung Disease and Bronchiectasis in Children and Adolescents. Front Pediatr 2017; 5:27. [PMID: 28265556 PMCID: PMC5316980 DOI: 10.3389/fped.2017.00027] [Citation(s) in RCA: 54] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2016] [Accepted: 02/01/2017] [Indexed: 02/04/2023] Open
Abstract
In the modern era, the global burden of childhood chronic suppurative lung disease (CSLD) remains poorly captured by the literature. What is clear, however, is that CSLD is essentially a disease of poverty. Disadvantaged children from indigenous and low- and middle-income populations had a substantially higher burden of CSLD, generally infectious in etiology and of a more severe nature, than children in high-income countries. A universal issue was the delay in diagnosis and the inconsistent reporting of clinical features. Importantly, infection-related CSLD is largely preventable. A considerable research and clinical effort is needed to identify modifiable risk factors and socioeconomic determinants of CSLD and provide robust evidence to guide optimal prevention and management strategies. The purpose of this review was to update the international literature on the epidemiology, etiology, and clinical features of pediatric CSLD.
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Affiliation(s)
- Gabrielle B McCallum
- Child Health Division, Menzies School of Health Research, Charles Darwin University , Darwin, NT , Australia
| | - Michael J Binks
- Child Health Division, Menzies School of Health Research, Charles Darwin University , Darwin, NT , Australia
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12
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Abstract
Chronic suppurative lung disease (CSLD), characterized by a bronchiectasis-like syndrome in the absence of bronchial dilatation, is well described in the pediatric literature. In some patients, it may be a precursor of bronchiectasis. In adults, this syndrome has not been well described. We present four adult patients without obvious causative exposures who presented with prolonged cough and purulent sputum. Sputum cultures revealed a variety of Gram negative bacteria, fungi and mycobacteria. High resolution CT scanning did not reveal bronchiectasis. Evaluation revealed underlying causes including immunodeficiency in two, and Mycobacterium avium infection. One patient subsequently developed bronchiectasis. All patients improved with therapy. CSLD occurs in adults and has characteristics that distinguish it from typical chronic bronchitis. These include the lack of causative environmental exposures and infection with unusual pathogens. Evaluation and treatment of these patients similar to bronchiectasis patients may lead to clinical improvement.
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Affiliation(s)
- Mark L Metersky
- Division of Pulmonary and Critical Care Medicine, University of Connecticut School of Medicine, Farmington, CT, USA
| | - Antranik Mangardich
- Department of Internal Medicine, University of Connecticut Health Center, Farmington, CT, USA
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13
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Smarda M, Efstathopoulos E, Mazioti A, Kordolaimi S, Ploussi A, Priftis K, Kelekis N, Alexopoulou E. High-Resolution Computed Tomography Examinations for Chronic Suppurative Lung Disease in Early Childhood: Radiation Exposure and Image Quality Evaluations with Iterative Reconstruction Algorithm Use. Can Assoc Radiol J 2016; 67:218-24. [DOI: 10.1016/j.carj.2015.10.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2015] [Revised: 09/05/2015] [Accepted: 10/28/2015] [Indexed: 10/21/2022] Open
Abstract
Purpose High radiosensitivity of children undergoing repetitive computed tomography examinations necessitates the use of iterative reconstruction algorithms in order to achieve a significant radiation dose reduction. The goal of this study is to compare the iDose iterative reconstruction algorithm with filtered backprojection in terms of radiation exposure and image quality in 33 chest high-resolution computed tomography examinations performed in young children with chronic bronchitis. Methods Fourteen patients were scanned using the filtered backprojection protocol while 19 patients using the iDose protocol and reduced milliampere-seconds, both on a 64-detector row computed tomography scanner. The iDose group images were reconstructed with different iDose levels (2, 4, and 6). Radiation exposure quantities were estimated, while subjective and objective image qualities were evaluated. Unpaired t tests were used for data statistical analysis. Results The iDose application allowed significant effective dose reduction (about 80%). Subjective image quality evaluation showed satisfactory results even with iDose level 2, whereas it approached excellent image with iDose level 6. Subjective image noise was comparable between the 2 groups with the use of iDose level 4, while objective noise was comparable between filtered backprojection and iterative reconstruction level 6 images. Conclusions The iDose algorithm use in pediatric chest high-resolution computed tomography reduces radiation exposure without compromising image quality. Further evaluation with iterative reconstruction algorithms is needed in order to establish high-resolution computed tomography as the gold standard low-dose method for children suffering from chronic lung diseases.
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Affiliation(s)
- Magdalini Smarda
- Radiology Department, University General Hospital ATTIKON, Chaidari, Athens, Greece
| | | | - Argyro Mazioti
- Radiology Department, University General Hospital ATTIKON, Chaidari, Athens, Greece
| | - Sofia Kordolaimi
- Radiology Department, University General Hospital ATTIKON, Chaidari, Athens, Greece
| | - Agapi Ploussi
- Radiology Department, University General Hospital ATTIKON, Chaidari, Athens, Greece
| | - Konstantinos Priftis
- Department of Pediatrics, University General Hospital ATTIKON, Chaidari, Athens, Greece
| | - Nikolaos Kelekis
- Radiology Department, University General Hospital ATTIKON, Chaidari, Athens, Greece
| | - Efthymia Alexopoulou
- Radiology Department, University General Hospital ATTIKON, Chaidari, Athens, Greece
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Patria MF, Longhi B, Lelii M, Tagliabue C, Lavelli M, Galeone C, Principi N, Esposito S. Children with recurrent pneumonia and non-cystic fibrosis bronchiectasis. Ital J Pediatr 2016; 42:13. [PMID: 26861259 PMCID: PMC4748602 DOI: 10.1186/s13052-016-0225-z] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/07/2015] [Accepted: 02/04/2016] [Indexed: 11/13/2022] Open
Abstract
BACKGROUND Recurrent pneumonia (RP) is one of the most frequent causes of pediatric non-cystic fibrosis (CF) bronchiectasis (BE) and a consequent accelerated decline in lung function. The aim of this study was to analyse the clinical records of children with RP in attempt to identify factors that may lead to an early suspicion of non-CF BE. METHODS We recorded the demographic and clinical data, and lung function test results of children without CF attending our outpatient RP clinic between January 2009 to December 2013 who had undergone chest high-resolution computed tomography ≥ 8 weeks after an acute pneumonia episode and ≤ 6 months before enrolment. RESULTS The study involved 42 patients with RP: 21 with and 21 without non-CF BE. The most frequent underlying diseases in both groups were chronic rhinosinusitis with post-nasal drip and recurrent wheezing (81 % and 71.4 % of those with, and 85.7 % and 71.4 % of those without BE). FEV1 and FEF25-75 values were significantly lower in the children with non-CF BE than in those without (77.9 ± 17.8 vs 96.8 ± 12.4, p = 0.004; 69.3 ± 25.6 vs 89.3 ± 21.9, p = 0.048). Bronchodilator responsiveness was observed in seven children with BE (33.3 %) and two without (9.5 %; p = 0.13). CONCLUSIONS Reduced FEV1 and FEF25-75 values seem associated with an increased risk of developing non-CF BE in children with RP. This suggests a need for further studies to confirm the diagnostic usefulness use of spirometry in such cases.
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Affiliation(s)
- Maria Francesca Patria
- Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
| | - Benedetta Longhi
- Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
| | - Mara Lelii
- Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
| | - Claudia Tagliabue
- Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
| | - Marinella Lavelli
- Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
| | - Carlotta Galeone
- Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Via Commenda 9, 20122, Milan, Italy.
| | - Nicola Principi
- Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
| | - Susanna Esposito
- Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Commenda 9, 20122, Milan, Italy.
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15
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Gupta AK, Lodha R, Kabra SK. Non Cystic Fibrosis Bronchiectasis. Indian J Pediatr 2015; 82:938-44. [PMID: 26307756 DOI: 10.1007/s12098-015-1866-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2015] [Accepted: 07/28/2015] [Indexed: 10/23/2022]
Abstract
Bronchiectasis is a pathological abnormality of the airways in which there is permanent dilatation and thickening of the airways. Precise incidence/prevalence in India is not known. Recent data suggests that about 1 % young children admitted in a hospital with pneumonia may develop bronchiectasis. Due to significant burden of pneumonia in young children in developing countries including India, it may be a significant problem that is possibly under recognized. Causes of bronchiectasis depend on the burden of respiratory infections and availability of the investigations for identification of the underlying cause. Post infectious causes are common in countries where infections are more common; however, since these countries are usually resource constrained and therefore, are not able to appropriately diagnose the other causes, leading to more than real overrepresentation of infections as a cause. In countries with less of infectious illnesses and good diagnostic facilities, malformations of airways, immune deficiency disorders and primary ciliary dyskinesia are common causes of bronchiectasis. High resolution CT scan of chest confirms the diagnosis. Treatment is supportive care and consists of maintenance of nutrition, airway clearance and antibiotics for exacerbations. Medical treatment is successful in the majority.
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Affiliation(s)
- Anand K Gupta
- Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Rakesh Lodha
- Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Sushil K Kabra
- Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India.
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16
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Andrade CF, Melo IA, Holand ARR, Silva ÉF, Fischer GB, Felicetii JC. Surgical treatment of non-cystic fibrosis bronchiectasis in Brazilian children. Pediatr Surg Int 2014; 30:63-9. [PMID: 24105331 DOI: 10.1007/s00383-013-3420-7] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 09/25/2013] [Indexed: 01/31/2023]
Abstract
PURPOSE To determine the clinical characteristics of patients submitted to surgical treatment for non-cystic fibrosis (CF) bronchiectasis, the indications for surgery, and the results obtained at a referral facility for pediatric thoracic surgery. METHODS Between January 1998 and December 2009, we retrospectively reviewed the medical charts of 109 pediatric patients with non-CF bronchiectasis who underwent surgical treatment. These findings were subsequently analyzed by focusing on postoperative complications and long-term results. RESULTS Of the 109 patients undergoing pulmonary resection, the mean age was 7.6 years (ranging from 1 to 15.5 y-o) with male predominance (59 %). The most common procedure was segmentectomy (43 %) followed by left lower lobectomy (38 %). Minor postoperative complications occurred in 36 % of the patients; the most common was transient atelectasis (26 %), followed by air leak (6 %), and postoperative pain (4 %). There was one death within the 30-day postoperative period, but it was unrelated to the procedure. Eighty-three children were followed after discharge, with a mean follow-up period of 667 days. Sixty-five (76 %) patients showed improvement of clinical symptoms after surgery. CONCLUSIONS Lung resection for the treatment of non-CF bronchiectasis in children is a safe procedure, with no life-treating morbidity and low mortality. This procedure also leads to significant improvements in symptoms and quality of life.
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Affiliation(s)
- Cristiano Feijó Andrade
- Postgraduate Program in Pulmonary Sciences, Federal University of Rio Grande do Sul (UFRGS), Porto Alegre, Brazil,
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17
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Truong T. The overlap of bronchiectasis and immunodeficiency with asthma. Immunol Allergy Clin North Am 2012; 33:61-78. [PMID: 23337065 DOI: 10.1016/j.iac.2012.10.007] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Bronchiectasis should be considered as a differential diagnosis for, as well as a comorbidity in, patients with asthma, especially severe or long-standing asthma. Chronic airway inflammation is thought to be the primary cause, as with chronic or recurrent pulmonary infection and autoimmune conditions that involve the airways. Consequently, immunodeficiencies with associated increased susceptibility to respiratory tract infections or chronic inflammatory airways also increase the risk of developing bronchiectasis. Chronic bronchiectasis is associated with impaired mucociliary clearance and increased bronchial secretions, leading to airway obstruction and airflow limitation, which can lead to exacerbation of underlying asthma or increased asthma symptoms.
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Affiliation(s)
- Tho Truong
- Allergy and Clinical Immunology, National Jewish Health, Denver, CO, USA.
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18
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19
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Abstract
Non-cystic fibrosis (CF) bronchiectasis is said to be a declining problem in developed countries, although its prevalence in indigenous communities in Australia and New Zealand is among the highest reported in the world. Early childhood pneumonia and underlying conditions such as immunodeficiency and primary ciliary dyskinesia need to be considered in the aetiology. A high-resolution computerised tomography scan is the key investigation in children with a chronic wet cough in whom bronchiectasis is suspected. Regardless of the cause, the treatment of bronchiectasis is centred upon facilitating the clearance of airway secretions and the treatment of pulmonary exacerbations. This review aims to provide general paediatricians with an update on the presenting features, investigation and management of non-cystic fibrosis bronchiectasis.
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Affiliation(s)
- Haya Al Subie
- Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, New South Wales, Australia
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20
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Cobanoglu U, Yalcinkaya I, Er M, Isik AF, Sayir F, Mergan D. Surgery for bronchiectasis: The effect of morphological types to prognosis. Ann Thorac Med 2011; 6:25-32. [PMID: 21264168 PMCID: PMC3023867 DOI: 10.4103/1817-1737.74273] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/16/2010] [Accepted: 10/26/2010] [Indexed: 11/04/2022] Open
Abstract
BACKGROUND Although the incidence has declined over the past years in societies with high socioeconomic status, bronchiectasis is still an important health problem in our country. AIM To review and present our cases undergoing surgery for bronchiectasis in the past 12 years and their early and late term postoperative outcomes and our experience in bronchiectasis surgery and the effect of morphological type on the prognosis. METHODS The medical records of 62 cases undergoing surgical resection for bronchiectasis in the Clinics of Thoracic and Pediatric Surgery were evaluated retrospectively. The disease was on the left in 33 cases, on the right in 26 and bilateral in three cases. The most common surgical procedure was lobectomy. Forty one patients underwent pneumonectomy, lobectomy and complete resection including bilobectomy. Twenty-one (33.87%) cases underwent incomplete resection, of whom 11 (17.74%) underwent segmentectomy and 10 (16.13%) underwent lobectomy + segmentectomy. RESULTS It was found that the rate of being asymptomatic was significantly higher in patients undergoing complete resection compared to those undergoing incomplete resection. Spirometric respiratory function tests were performed to assess the relationship between morphological type and the severity of disease. All parameters of respiratory function were worse in the saccular type and FEV(1)/FVC showed a worse obstructive deterioration in the saccular type compared to the tubular type. CONCLUSION The success rate of the procedure increases with complete resection of the involved region. The morphological type is more important than the number and extension of the involved segments in showing the disease severity.
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Affiliation(s)
- Ufuk Cobanoglu
- Department of Thoracic Surgery, University of Yuzuncu Yil, Van, Turkey
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21
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Kapur N, Karadag B. Differences and similarities in non-cystic fibrosis bronchiectasis between developing and affluent countries. Paediatr Respir Rev 2011; 12:91-6. [PMID: 21458736 DOI: 10.1016/j.prrv.2010.10.010] [Citation(s) in RCA: 34] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
Non-CF bronchiectasis remains a major cause of morbidity not only in developing countries but in some indigenous groups of affluent countries. Although there is a decline in the prevalence and incidence in developed countries, recent studies in indigenous populations report higher prevalence. Due to the lack of such data, epidemiological studies are required to find the incidence and prevalence in developing countries. Although the main characteristics of bronchiectasis are similar in developing and affluent countries, underlying aetiology, nutritional status, frequency of exacerbations and severity of the disease are different. Delay of diagnosis is surprisingly similar in the affluent and developing countries possibly due to different reasons. Long-term studies are needed for evidence based management of the disease. Successful management and prevention of bronchiectasis require a multidisciplinary approach, while the lack of resources is still a major problem in the developing countries.
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Affiliation(s)
- Nitin Kapur
- Department of Respiratory Medicine, 3rd Floor, Woolworths Building, Royal Children's Hospital, Herston, QLD 4029, Australia.
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22
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Abstract
Current diagnostic labelling of childhood bronchiectasis by radiology has substantial limitations. These include the requirement for two high resolution computerised tomography [HRCT] scans (with associated adversity of radiation) if criteria is adhered to, adoption of radiological criteria for children from adult data, relatively high occurrence of false negative, and to a smaller extent false positive, in conventional HRCT scans when compared to multi-detector CT scans, determination of irreversible airway dilatation, and absence of normative data on broncho-arterial ratio in children. A paradigm presenting a spectrum related to airway bacteria, with associated degradation and inflammation products causing airway damage if untreated, entails protracted bacterial bronchitis (at the mild end) to irreversible airway dilatation with cystic formation as determined by HRCT (at the severe end of the spectrum). Increasing evidence suggests that progression of airway damage can be limited by intensive treatment, even in those predestined to have bronchiectasis (eg immune deficiency). Treatment is aimed at achieving a cure in those at the milder end of the spectrum to limiting further deterioration in those with severe 'irreversible' radiological bronchiectasis.
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Affiliation(s)
- A.B. Chang
- Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, NT; Queensland Children's Respiratory Centre and Queensland Children's Medical Research Institute, Royal Children's Hospital, Brisbane, Australia
| | - C.A. Byrnes
- Paediatric Department, Faculty of Health & Medical Sciences, University of Auckland, Private Bag 92019, Auckland, New Zealand
| | - M.L. Everard
- Paediatric Respiratory Unit and Sheffield Children's Hospital, Western Bank, Sheffield, UK
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Thomson M, Myer L, Zar HJ. The Impact of Pneumonia on Development of Chronic Respiratory Illness in Childhood. PEDIATRIC ALLERGY IMMUNOLOGY AND PULMONOLOGY 2010. [DOI: 10.1089/ped.2010.0056] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Affiliation(s)
- Mairi Thomson
- Division of Biostatistics and Epidemiology, School of Public Health and Health Sciences, University of Massachusetts, Amherst, Massachusetts
| | - Landon Myer
- Center for Infectious Diseases Epidemiology and Research, School of Public Health and Family Medicine, University of Cape Town, Cape Town, South Africa
- International Center for AIDS Care and Treatment Programs and Department of Epidemiology, Mailman School of Public Health, Columbia University, New York, New York
| | - Heather J. Zar
- Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, University of Cape Town, Cape Town, South Africa
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Khemiri M, Khaldi F, Hamzaoui A, Chaouachi B, Hamzaoui M, Becher SB, Bellagha I, Barsaoui S. [Cystic pulmonary malformations: clinical and radiological polymorphism. A report on 30 cases]. REVUE DE PNEUMOLOGIE CLINIQUE 2009; 65:333-340. [PMID: 19995653 DOI: 10.1016/j.pneumo.2009.08.002] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/05/2008] [Revised: 06/03/2009] [Accepted: 08/23/2009] [Indexed: 05/28/2023]
Abstract
OBJECTIVES This report describes different clinical pictures of cystic pulmonary malformation (CPM) and problems in diagnosis. PATIENTS AND METHODS Cases of CPM between 01 January 1994 and 31 December 2004 diagnosed in our institution were reviewed. RESULTS Thirty-three cases of CPM were diagnosed in 30 children. They consisted of 17 boys and 13 girls ranging from 20 days to 16 years of age at the time of the diagnosis. The CPM included: 17 cases of congenital lobar emphysema (CLE), seven bronchogenic cysts (BC), five cystic adenomatoid malformations (CAM) and four pulmonary sequestrations (PS). Three patients presented two associated lung malformations. The mean ages at the time of diagnosis varied from 2 to 88 months. The symptoms consisted of respiratory distress (n=14, 46.6%); recurrent attacks of respiratory embarrassment (n=6, 20%); pulmonary infection (n=8, 26.6%) associated with haemoptysis in two cases; haemothorax (n=1) and a chance discovery (n=1). Radiological investigations led to the diagnosis in all cases of CLE and CAM although it contributed less to the diagnosis of BC and PS. Twenty-nine patients required chirurgical treatment involving lobectomy (n=22), pneumonectomy (n=2) and cystectomy (n=8). The histopathological examinations confirmed the diagnosis in all cases and rectified the preoperative diagnosis in four cases. Except for one patient with CLE, who died a few days after a lobectomy due to acute nosocomial pneumonia, the postoperative period was uneventful in 26 children with a mean of follow-up of 24 months (4 months to 7 years). Three patients developed transient and episodic attacks of dyspnoea. CONCLUSION CPM may be responsible for many clinical and radiological pictures that present difficulties in their diagnosis. Polymorphism is related to the type of malformation, its topography and the evolutive complications.
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Affiliation(s)
- M Khemiri
- Service médecine infantile A, hôpital d'Enfants Bab Saadoun-Jabbary, CP 1007 Tunis, Tunisie.
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25
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Kapur N, Masters IB, Chang AB. Exacerbations in noncystic fibrosis bronchiectasis: Clinical features and investigations. Respir Med 2009; 103:1681-7. [PMID: 19501498 DOI: 10.1016/j.rmed.2009.05.007] [Citation(s) in RCA: 50] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/12/2009] [Revised: 04/25/2009] [Accepted: 05/06/2009] [Indexed: 10/20/2022]
Abstract
UNLABELLED Children with bronchiectasis have recurrent acute pulmonary exacerbations and many of these exacerbations require hospital admission when oral therapies fail. However there is no standardized definition and little published data is available about the features of an exacerbation. Our aim was to determine the clinical and investigational features of exacerbations in bronchiectasis, the proportion that fail to resolve on oral antibiotics and the factors associated with it. METHODS A retrospective cohort study of 115 respiratory exacerbations from 30 children with noncystic fibrosis bronchiectasis diagnosed on HRCT chest. Clinical features, investigations and treatment related to the exacerbations were extracted and analysed. RESULTS Increase in frequency of cough (88%) and a change in its character (67%) were the most common symptoms associated with an exacerbation. Fever (28%), increase in sputum volume (42%) and purulence (35%) were also common features. Chest pain, dyspnea, hemoptysis and tachypnea were rare. 56% had a worsening in their chest auscultatory findings during an exacerbation. Spirometry was not significantly different between stable and exacerbation state. 35% of exacerbations failed to respond to oral antibiotic therapy and required hospital admission. Prophylactic antibiotic therapy was the only significant predictor of failure of oral therapy with adjusted odds ratio of 6.77 (95% CI 2.06-19.90; p=0.003). CONCLUSIONS Important clinical features of non-CF exacerbation in bronchiectasis were changes in cough frequency or character, and worsening chest signs; which resolved on therapies. However there is a high failure rate of oral antibiotic therapy and use of prophylactic antibiotic therapy increases this risk.
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Affiliation(s)
- Nitin Kapur
- Queensland Children's Respiratory Centre, Royal Children's Hospital, Australia.
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26
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Chang AB, Redding GJ, Everard ML. Chronic wet cough: Protracted bronchitis, chronic suppurative lung disease and bronchiectasis. Pediatr Pulmonol 2008; 43:519-31. [PMID: 18435475 DOI: 10.1002/ppul.20821] [Citation(s) in RCA: 167] [Impact Index Per Article: 9.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
The role of persistent and recurrent bacterial infection of the conducting airways (endobronchial infection) in the causation of chronic respiratory symptoms, particularly chronic wet cough, has received very little attention over recent decades other than in the context of cystic fibrosis (CF). This is probably related (at least in part) to the (a) reduction in non-CF bronchiectasis in affluent countries and, (b) intense focus on asthma. In addition failure to characterize endobronchial infections has led to under-recognition and lack of research. The following article describes our current perspective of inter-related endobronchial infections causing chronic wet cough; persistent bacterial bronchitis (PBB), chronic suppurative lung disease (CSLD) and bronchiectasis. In all three conditions, impaired muco-ciliary clearance seems to be the common risk factor that provides organisms the opportunity to colonize the lower airway. Respiratory infections in early childhood would appear to be the most common initiating event but other conditions (e.g., tracheobronchomalacia, neuromuscular disease) increases the risk of bacterial colonization. Clinically these conditions overlap and the eventual diagnosis is evident only with further investigations and long term follow up. However whether these conditions are different conditions or reflect severity as part of a spectrum is yet to be determined. Also misdiagnosis of asthma is common and the diagnostic process is further complicated by the fact that the co-existence of asthma is not uncommon. The principles of managing PBB, CSLD and bronchiectasis are the same. Further work is required to improve recognition, diagnosis and management of these causes of chronic wet cough in children.
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Affiliation(s)
- A B Chang
- Child Health Division, Menzies School of Health Research, Charles Darwin University, Darwin, Australia.
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27
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Toiviainen-Salo S, Kajosaari M, Piilonen A, Mäkitie O. Patients with cartilage-hair hypoplasia have an increased risk for bronchiectasis. J Pediatr 2008; 152:422-8. [PMID: 18280853 DOI: 10.1016/j.jpeds.2007.11.040] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/04/2007] [Revised: 09/25/2007] [Accepted: 11/28/2007] [Indexed: 10/22/2022]
Abstract
OBJECTIVE To assess the prevalence, predictors, and course of bronchiectasis in patients with cartilage-hair hypoplasia. STUDY DESIGN Patients who had undergone high-resolution computed tomography of the lungs or bronchography were included in the study. Hospital records were coorelated for clinical features, respiratory symptoms, and laboratory variables. Imaging studies were correlated for changes suggestive of bronchiectasis and findings correlated with clinical and immunological measurements. RESULTS The study included 15 patients (5 male; median height Z-score, -7.7) aged from 2 to 39 years (median, 10 years). Cell-mediated immunity was impaired in 79% of the subjects, and humoral immunity was impaired in 71% of the subjects. 8 patients (52%) had bronchiectasis, diagnosed by means of high-resolution computed tomography (n = 6) or bronchography (n = 2). The findings ranged from localized mild dilatation of the airways to severe bronchiectasis with saccular airway dilatation. Bronchiectasis progressed during follow-up in 2 patients. Patients with bronchiectasis tended to have more severe growth failure and more often had defective humoral immunity than the general cartilage-hair hypoplasia population. CONCLUSION Patients with cartilage-hair hypoplasia are at risk of the development of bronchiectasis.
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Notarangelo LD, Plebani A, Mazzolari E, Soresina A, Bondioni MP. Genetic causes of bronchiectasis: primary immune deficiencies and the lung. Respiration 2007; 74:264-75. [PMID: 17534129 DOI: 10.1159/000101784] [Citation(s) in RCA: 35] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
Primary immune deficiencies (PID) comprise a heterogeneous group of genetically determined disorders that affect development and/or function of innate or adaptive immunity. Consequently, patients with PID suffer from recurrent and/or severe infections that frequently involve the lung. While the nature of the immune defect often dictates the type of pathogens that may cause lung infection, there is substantial overlap of radiological findings, so that appropriate laboratory tests are mandatory to define the nature of the immune defect and to prompt appropriate treatment. At the same time, the recent identification of a large number of PID-causing genes now allows early, even presymptomatic diagnosis, thus representing an essential tool for prevention of lung damage. This review article describes the most common forms of PID, their cellular and molecular bases, and the associated lung abnormalities, and reports on available treatment.
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Affiliation(s)
- Luigi D Notarangelo
- Division of Immunology, Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
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30
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Choudhury SR, Chadha R, Mishra A, Kumar V, Singh V, Dubey NK. Lung resections in children for congenital and acquired lesions. Pediatr Surg Int 2007; 23:851-9. [PMID: 17671788 DOI: 10.1007/s00383-007-1940-8] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/05/2007] [Indexed: 11/25/2022]
Abstract
We reviewed a single-center experience of pediatric lung resections for various congenital and acquired benign lung conditions. Thirty-five children underwent lung resections between 1998 and 2006, their age ranging from 8 days to 12 years (mean 3 years), with a male:female ratio of 4:1. Twelve patients were neonates. Antenatal diagnosis was available in only one patient. The presenting symptoms were respiratory distress and respiratory tract infections. Imaging with chest X-ray with/without a CT scan picked up the lesion in all cases. Preoperative ventilation was required for five patients. One patient had pneumothorax at presentation; however, ten patients had inadvertent intercostal tube insertion before surgical referral. The surgical procedures performed included lobectomy (28), segmentectomy (3), and pneumonectomy in 4 cases. Twenty-one patients underwent emergency surgery. Six patients required postoperative ventilation. The histopathological diagnosis was congenital lobar emphysema (CLE) (9), congenital cystic adenomatoid malformation (CCAM) (9), bronchiectasis (9), sequestration (3), atelectasis (1), lung abscess (1), unilobar tuberculosis (1), hydatid cyst (1), and foreign body with collapse (1). There was considerable discrepancy between the preoperative diagnosis based on imaging and the postoperative histopathological diagnosis. Postoperative complications included atelectasis (2), pneumothorax (2) and fluid collection (4 cases). Three patients died, one from compromised cardiac function, one from overwhelming sepsis and one from respiratory failure due to severe bilateral CCAM; the rest of the patients made a satisfactory recovery. At short-term follow-up all patients were doing well. Pulmonary resections are necessary for various congenital and acquired lung lesions in children and can be done safely in a pediatric hospital setup. Proper preoperative diagnosis can avoid inadvertent intercostal tube insertion in patients with congenital cystic lung lesions. The histopathological diagnosis often differs from the radiological diagnosis. Emergency lobectomies for acute respiratory distress, even in neonates, result in a satisfactory outcome.
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Affiliation(s)
- Subhasis Roy Choudhury
- Department of Pediatric Surgery, Kalawati Saran Children's Hospital and Lady Hardinge Medical College, New Delhi 110001, India.
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Abstract
Cystic fibrosis (CF) typically follows a more severe clinical course than non-CF bronchiectasis. Despite this recognized difference, the underpinnings of respiratory biology support a common pathogeneses of the anatomic deformations of bronchiectasis. This article reviews the observed manifestations among the related diseases of bronchiectasis and CF and discusses some of their similarities and differences. As more details of the mechanisms of bronchiectasis are unveiled, more parallels among the seemingly disparate causes of CF and non-CF bronchiectasis are recognized. With these insights, more opportunities to halt the vicious circle have become apparent.
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Affiliation(s)
- Brian M Morrissey
- Division of Pulmonary/Critical Care Medicine, Department of Internal Medicine, School of Medicine, University of California-Davis, 4150 V Street, Suite 3400, Sacramento, CA 95817, USA.
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Skorpinski EW, Kung SJ, Yousef E, McGeady SJ. Diagnosis of common variable immunodeficiency in a patient with primary ciliary dyskinesia. Pediatrics 2007; 119:e1203-5. [PMID: 17438081 DOI: 10.1542/peds.2006-2396] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
Abstract
In this case report we describe the first account in the literature of a patient with primary ciliary dyskinesia and common variable immunodeficiency. A 17-year-old boy with previously diagnosed Kartagener syndrome and stable lung disease developed a deteriorating clinical course that prompted the search for a secondary diagnosis. Although both of these rare conditions can result in similar lung pathology, they require different management strategies, which illustrates the need to consider associated diagnoses in complicated clinical situations.
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Abstract
Bronchiectasis has been defined as the abnormal and permanent dilation of bronchi. It has a variety of causes and has traditionally been viewed as a condition that is irreversible, often progressive and associated with significant morbidity and mortality. In the past, patients had relatively advanced disease by the time the diagnosis was established. By using high-resolution computed tomography (HRCT) scanning of the chest, the potential now exists for the much earlier detection and treatment of children with lesser degrees of bronchial dilation and bronchial wall thickening than was previously possible. In some, the HRCT changes have been seen to improve or completely resolve. This calls into question exactly what now should be termed bronchiectasis and how the parents of children with such HRCT findings should be counselled about the likely prognosis and the necessary or desirable treatment options.
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Affiliation(s)
- Andrew Fall
- James Cook University Hospital, Marton Road, Middlesbrough, UK.
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Abstract
Nowadays, bronchiectasis tends to be considered a rare disease. This is really the case in developed countries, where good standards of living have been prevalent for many decades. But it might not be the case in the developing world, where better sanitary conditions are still needed. This article reviews non-cystic fibrosis bronchiectasis, emphasising differences between developed and developing countries. Diagnostic methods and therapeutic issues are discussed as is the Latin American experience of postviral bronchiectasis.
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Affiliation(s)
- Paulo José Cauduro Marostica
- Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Bua Ramiro Barcelos 2350, 90035-003 Porto Alegre RS, Brazil.
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Santamaria F, Montella S, Camera L, Palumbo C, Greco L, Boner AL. Lung structure abnormalities, but normal lung function in pediatric bronchiectasis. Chest 2006; 130:480-6. [PMID: 16899848 DOI: 10.1378/chest.130.2.480] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
Abstract
BACKGROUND Bronchiectasis is not considered to be uncommon in children anymore. The relationship between pulmonary function and severity of bronchiectasis is still controversial. STUDY OBJECTIVES To assess the extent and severity of bronchiectasis through high-resolution CT (HRCT) scan score, and to correlate it with clinical, microbiological, and functional data. PATIENTS AND METHODS Forty-three white children with HRCT-diagnosed bronchiectasis were studied. Bronchiectasis extent, bronchial wall thickening severity, and bronchial wall dilatation severity were evaluated using the Reiff score. Clinical, microbiological, and spirometry results were related to total HRCT scan score and to subscores as well. RESULTS The percentages of affected lobes were as follows: right lower lobe, 65%; middle lobe, 56%; left lower lobe, 51%; right upper lobe, 37%; lingula, 30%; and left upper lobe, 30% (chi(2) = 18.4; p = 0.002). The mean (+/- SEM) HRCT score was 20 +/- 2.6. Total score or subscores of bronchiectasis extent, bronchial wall thickening severity, and bronchial wall dilatation severity were not significantly related to FEV(1) and FVC. Seventy-four percent of patients had asthma. The age at the onset of cough correlated with age at the time of the HRCT scan (p = 0.004) and with the presence of asthma (p = 0.01). Positive findings of deep throat or sputum cultures were found more frequently in atopic patients (p = 0.02) and asthmatic (p < 0.01) patients, and in children who were < 2 years of age at the onset of cough (p < 0.01). CONCLUSIONS Normal lung function may coexist with HRCT scan abnormalities and does not exclude damage to the bronchial structure. Pulmonary function is not an accurate method for assessing the severity of lung disease in children with bronchiectasis.
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Abstract
Suppurative cough can be defined as a cough where purulent sputum is produced. Chronic suppurative cough may be associated with the destruction of the bronchial wall (bronchiectasis). As mild forms of the disease are not associated with respiratory limitation or failure to thrive, such children may not present for investigation and therefore the true incidence of suppurative cough is difficult to gauge. Chronic suppurative cough remains an important health problem in developing countries and some indigenous populations of developed countries. The purpose of this review is to present the appropriate investigations and evaluate the evidence for current management strategies in children with suppurative cough. To accomplish this, a brief discussion on the aetiology of suppurative cough in childhood is presented. The most commonly identifiable cause of suppurative cough is cystic fibrosis. A detailed discussion on cystic fibrosis is beyond the scope of this review. Other causes of chronic suppurative cough in pre-school children may be classified according to congenital malformations of the airway, immunodeficiency, ciliary dysfunction and, unusually, acquired causes. Microbiology of sputum culture or bronchoalveolar lavage, assessment of immune function, the role of exhaled nitric oxide and ciliary studies, and medical imaging are discussed in detail. One can conclude that the evidence for management strategies for children with suppurative cough is, at best, level 3 evidence, i.e. non-randomised, controlled or cohort studies.
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Affiliation(s)
- Hiran Selvadurai
- Department of Respiratory Medicine, The Childrens Hospital at Westmead, Locked Bag 4001, Westmead, Sydney 2145, Australia.
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Doğru D, Nik-Ain A, Kiper N, Göçmen A, Ozçelik U, Yalçin E, Aslan AT. Bronchiectasis: the consequence of late diagnosis in chronic respiratory symptoms. J Trop Pediatr 2005; 51:362-5. [PMID: 15890722 DOI: 10.1093/tropej/fmi036] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/14/2022]
Abstract
Bronchiectasis is still common among some developing countries like Turkey. The aim of this study was to document the number of children with non-cystic fibrosis (CF) bronchiectasis, to evaluate the risk factors and to emphasize early diagnosis and treatment. All children, except those diagnosed with CF, with bronchiectasis established by chest radiogram, bronchography and/or computed tomography or biopsy material, were retrospectively reviewed. They were tested for serum total eosinophil count, nasal smear, serum levels of immunoglobulins A, G, M, E, and serum alpha-1 antitrypsin level. Pulmonary function tests, rigid bronchoscopy, nasal biopsy, lung scintigraphy, and echocardiogram were also performed. There were 204 patients whose most common presenting symptoms were cough, sputum expectoration, and dyspnea. Bronchiectasis was present mostly in the left lower lobe. The cause could not be determined in 49 per cent of patients. Among the identified causes, infection was present in most patients, followed by asthma, primary ciliary dyskinesia, congenital immune deficiency, and foreign body aspiration. It is possible to prevent bronchiectasis in children with vaccinations and improved nutrition in developing countries. Early diagnosis and treatment will increase the quality of life and survival of patients with bronchiectasis, which has irreversible and progressive complications if untreated.
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Affiliation(s)
- Deniz Doğru
- Department of Pediatrics, Pulmonary Medicine Unit, Hacettepe University, Ankara, Turkey.
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38
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Spencer DA. From hemp seed and porcupine quill to HRCT: advances in the diagnosis and epidemiology of bronchiectasis. Arch Dis Child 2005; 90:712-4. [PMID: 15970614 PMCID: PMC1720502 DOI: 10.1136/adc.2004.054031] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
Over the last decade there has been a significant improvement in our ability to recognise non-cystic fibrosis (CF) bronchiectasis in children. The precise incidence is uncertain, and it varies greatly depending on the populations studied and the methods used to make the diagnosis. It is unlikely that many of the underlying causes of non-CF bronchiectasis will be eradicated in the near future, and so it must be expected that with ever improving technology this diagnosis will be made with increasing frequency. This emphasises the need to improve our understanding of the aetiology, pathophysiology, epidemiology, and management options for children with this group of conditions.
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Affiliation(s)
- D A Spencer
- Regional Cardiothoracic Centre, Freeman Hospital, Newcastle upon Tyne NE7 7DN, UK.
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Karadag B, Karakoc F, Ersu R, Kut A, Bakac S, Dagli E. Non-Cystic-Fibrosis Bronchiectasis in Children: A Persisting Problem in Developing Countries. Respiration 2005; 72:233-8. [PMID: 15942290 DOI: 10.1159/000085362] [Citation(s) in RCA: 104] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2004] [Accepted: 08/25/2004] [Indexed: 11/19/2022] Open
Abstract
BACKGROUND Non-cystic-fibrosis (non-CF) bronchiectasis in childhood is still one of the most common causes of childhood morbidity in developing countries. The management of these patients remains problematic, and there are few studies of long-term outcome. OBJECTIVE The aim of this retrospective study was to define the general characteristics, underlying causative factors and long-term follow-up results of non-CF bronchiectasis patients. METHODS One hundred and eleven consecutive children, diagnosed with non-CF bronchiectasis were included in the study. General characteristics and underlying causes were recorded from the medical records. Clinical outcomes were evaluated in terms of lung function tests, annual exacerbation rates and patient/parent perception of health status. RESULTS Mean age of the patients was 7.4 +/- 3.7 years at presentation, and patients had been followed 4.7 +/- 2.7 years on average. In 62.2% of the patients, an underlying etiology was identified, whereas postinfectious bronchiectasis was the most common (29.7%). In spite of intensive medical treatment, 23.4% of the patients required surgery. The annual lower respiratory infection rate has decreased from a mean of 6.6 +/- 4.0 to 2.9 +/- 2.9 during follow-up (p < 0.0001). Lung function tests were also found to be improved (mean FEV1% 63.3 +/- 21.0 vs. 73.9 +/- 27.9; p = 0.01; mean FVC% 68.1 +/- 22.2 vs. 74.0 +/- 24.8; p = 0.04). There was clinical improvement in both the surgical (73%) and medical (70.1%) groups (p > 0.05). CONCLUSION In conclusion, bronchiectasis remains a disease of concern to pediatricians, particularly in developing countries. Infections are still important causes of bronchiectasis, and clinical improvement can be achieved by appropriate treatment. Although medical treatment is the mainstay of management, surgery should be considered in selected patients.
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Affiliation(s)
- B Karadag
- Division of Pediatric Pulmonology, Marmara University, Istanbul, Turkey.
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40
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Abstract
Search for an etiology of bronchiectasis consists in identifying constitutional or acquired defense mechanisms of the respiratory mucosa. The question is timely because causes change. In developing countries, presumed sequelae of infection account for about 30% of the cases despite vaccination campaigns, control of endemic tuberculosis, and widespread use of antibiotics. Genetic diseases account for 20% of the causes when identified by high-performance prospective diagnostic tests (CFTR mutation). Computed tomography enables the identification of frequent associations between bronchiectasis and rheumatoid disease or ulcerative colitis. Recent diseases such as HIV infection or GVHD can also lead to bronchiectasis. Nevertheless, the cause remains unknown in 30-50% of patients. After a detailed analysis of the clinical presentation and diagnostic criteria specific for each etiology, we propose a two-phase diagnostic procedure. The first step, used for all patients (careful history taking, physical examination, imaging, bronchofibroscopy, limited blood tests) enables detecting localized bronchial obstacles and obvious etiologies (situs inversus of primary ciliary dyskinesia, known systemic disease, HIV...). If the first step is negative, the second phase is oriented by the clinical context. Sequelae of infection (tuberculosis...) in older subjects or migrants, a genetic cause in younger subjects, particularly if there is a familial history and/or infertility, a systemic disease or allergic bronchopulmonary aspergillosis if there is an extra-respiratory context. This etiological search should help improve patient management and provide a better prognosis and prevention of bronchiectasis.
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Affiliation(s)
- H Lioté
- Service de Pneumologie, Hôpital Tenon, 4 rue de la Chine, 75020 Paris.
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41
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Valery PC, Torzillo PJ, Mulholland K, Boyce NC, Purdie DM, Chang AB. Hospital-based case-control study of bronchiectasis in indigenous children in Central Australia. Pediatr Infect Dis J 2004; 23:902-8. [PMID: 15602188 DOI: 10.1097/01.inf.0000142508.33623.2f] [Citation(s) in RCA: 78] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
BACKGROUND Childhood pneumonia has been reported to be associated with the development of bronchiectasis but there are no case-control studies that have examined this. This study examined the relationship between hospital admission for episode(s) of pneumonia and the risk of radiologically proven bronchiectasis. METHODS A medical record-based case-control study of bronchiectasis in Indigenous children was conducted in Central Australia. Controls (183), matched to cases (61) by gender, age and year of diagnosis, were Indigenous children hospitalized with other conditions. RESULTS There was a strong association between a history of hospitalized pneumonia and bronchiectasis [odds ratio (OR), 15.2; 95% confidence interval (95% CI) 4.4-52.7]. This was particularly evident in recurrent hospitalized pneumonia (P for trend < 0.01), severe pneumonia episodes with longer hospital stay (P for trend < 0.01), presence of atelectasis (OR 11.9; 95% CI 3.1-45.9) and requirement for oxygen (P for trend < 0.01). The overall number of pneumonia episodes, rather than its site, was associated with bronchiectasis. Although the total number of pneumonia episodes in the first year of life did not increase the risk of bronchiectasis, more severe episodes early in life did. Malnutrition, premature birth and being small for gestational age were more common findings among cases. Breast-feeding appeared to be a protective factor (OR 0.2; 95% CI 0.1-0.7). CONCLUSIONS Although we cannot fully answer the question of why bronchiectasis is much more common in Indigenous children, we have provided strong evidence of an association between bronchiectasis and severe and recurrent pneumonia episodes in infancy and childhood.
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Affiliation(s)
- Patricia C Valery
- Queensland Institute of Medical Research, Population Studies and Human Genetics, Brisbane, Australia
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42
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Abstract
Bronchiectasis in women may act more virulently. Identified gender and sex differences range from increased exposure risks to altered inflammatory responses. Common among the most well-documented examples is a differential immune response. There is sufficient evidence to suggest that chronic airway infection, most notably non-CF bronchiectasis, is a more common and more virulent disease in women. This is particularly evident in CF-and non-HIV-related environmental mycobacterial respiratory tract infections. Whether this represents an inflammatory-immune process, or environmental, anatomic, or other genetic difference remains to be detailed fully.
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Affiliation(s)
- Brian M Morrissey
- Department of Internal Medicine, Division of Pulmonary/Critical Care Medicine, University of California-Davis School of Medicine, 4150 V Street, Suite 3400, Sacramento, CA 95817, USA.
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Eastham KM, Fall AJ, Mitchell L, Spencer DA. The need to redefine non-cystic fibrosis bronchiectasis in childhood. Thorax 2004; 59:324-7. [PMID: 15047953 PMCID: PMC1763810 DOI: 10.1136/thx.2003.011577] [Citation(s) in RCA: 138] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
BACKGROUND Non-cystic fibrosis (CF) bronchiectasis has previously been reported to be rare and progressive in children living in western societies. METHOD A clinical and radiological review was undertaken of 93 children with non-CF bronchiectasis defined by high resolution computed tomographic (HRCT) scanning presenting to a tertiary paediatric respiratory centre since 1996. RESULTS Cases constituted 9.6% of all new referrals. Male to female ratio was 2:1. Median age at symptom onset was 1.1 years (range 0-16) and of HRCT diagnosis was 7.2 years (1.6-18.8). The most common referral diagnosis of asthma was refuted in 39 of 45 cases. Associations were previous pneumonic illness (30%), immunocompromise (21%), obliterative bronchiolitis (9%), congenital lung abnormality (5%), chronic aspiration (3%), eosinophilic oesophagitis (2%), familial syndrome (2%), primary ciliary dyskinesia (1%), and right middle lobe syndrome (1%). 8% had two associated diagnoses and 18% were idiopathic. There was agreement between the chest radiograph and HRCT scan for diagnosis and lobe affected in only five cases (5%). A repeat HRCT scan in 18 cases at a minimum interval of 18 months showed total resolution of the changes in six, improvement in one, progression in five, and was unchanged in six. CONCLUSIONS Radiologically defined non-CF bronchiectasis in children is not uncommon. Diagnostic delay is a problem. The most common association is a previous pneumonia. Chest radiography is of little diagnostic value, but resolution is possible on HRCT scanning. Bronchiectasis is currently defined as a condition which is both permanent and progressive. This term is not necessarily appropriate for all paediatric patients for whom we suggest an alternative nomenclature.
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Affiliation(s)
- K M Eastham
- Sir James Spence Institute of Child Health, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK
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Redding G, Singleton R, Lewis T, Martinez P, Butler J, Stamey D, Bulkow L, Peters H, Gove J, Morray B, Jones C. Early radiographic and clinical features associated with bronchiectasis in children. Pediatr Pulmonol 2004; 37:297-304. [PMID: 15022125 DOI: 10.1002/ppul.10427] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Bronchiectasis among children living in developing regions is associated with respiratory infections during early childhood, but specific risk factors that precede childhood bronchiectasis are not fully characterized. We hypothesized that severe respiratory syncytial viral (RSV) infection in infancy would increase the risk of bronchiectasis among Alaska Native children in rural Alaska. This was a follow-up cohort study of a 1993-1996 case-control study of RSV-hospitalized case patients and their controls. For each 5-8-year-old former case-patient and control subject, we reviewed medical records, interviewed parents, performed physical examinations and spirometry, collected sera, and analyzed all historical chest radiographs. Ten (11%) RSV cases and 10 (9%) controls had radiographic evidence of bronchiectasis. The mean age at radiographic diagnosis of bronchiectasis was 3.3 years (range, 1.2-6.1 years). Children were more likely to develop bronchiectasis if their chest radiographs, when they were < 2 years of age, showed lung parenchymal densities (RR = 3.9, P < 0.013), persistent parenchymal densities > 6 months' duration (RR = 3.0, P = 0.02), or infiltrates on multiple episodes (test for trend, P = 0.003). Radiographic features of hyperinflation and atelectasis among children < 2 years old were not associated with eventual bronchiectasis. A single severe infection with RSV alone did not predispose Alaska Native infants to bronchiectasis. Childhood bronchiectasis was associated with lung and hence airway injury, manifested on radiographs by parenchymal densities or "pneumonia" rather than by hyperinflation or atelectasis.
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Affiliation(s)
- Gregory Redding
- Pediatric Pulmonary Division, University of Washington School of Medicine, Seattle, Washington 98105, USA.
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Oddy WH, Kendall GE, Blair E, de Klerk NH, Silburn S, Zubrick S. Breastfeeding and cognitive development in children. ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY 2004; 554:365-9. [PMID: 15384602 DOI: 10.1007/978-1-4757-4242-8_42] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Affiliation(s)
- W H Oddy
- Centre for Child Health Research, University of Western Australia, TVW Telethon Institute for Child Health Research, West Perth, Western Australia, 6872 Australia.
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Chang AB, Masel JP, Boyce NC, Wheaton G, Torzillo PJ. Non-CF bronchiectasis: clinical and HRCT evaluation. Pediatr Pulmonol 2003; 35:477-83. [PMID: 12746947 DOI: 10.1002/ppul.10289] [Citation(s) in RCA: 134] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
Childhood bronchiectasis not related to underlying disease is still common in some populations in affluent countries. The aims of the study were to: 1) describe demographics, 2) evaluate the effectiveness of routine investigations, and 3) determine the relationship between spirometry and radiology scoring systems, in children with chronic suppurative lung disease (CSLD) living in Central Australia. Data of children living in Central Australia aged 70%) and early hospitalisation for pneumonia were common (median age, 0.5 years). Previous admissions for pneumonia were almost universally present and significantly more common than bronchiolitis (95% CI for proportional difference, 0.4-0.51). Although the majority did not have a treatable underlying cause, investigations had significant impact on management in 12.3% of children. None of the chest HRCT scores related to any spirometry data. In conclusion, CSLD is unacceptably common in indigenous children of this region, commences early in life, and is associated with significant comorbidities. Spirometry data do not reflect the severity of lung disease in HRCT scans. While improvement in the living standards is of utmost importance, the medical management that includes thorough investigations of these children should not be neglected.
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Affiliation(s)
- A B Chang
- Flinders University NT Clinical School, Alice Springs, Northern Territory, Australia
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47
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Abstract
Bronchiectasis is a common complication of primary antibody deficiency but the incidence of antibody deficiency as an underlying cause of bronchiectasis is largely undefined. In this study the humoral immune status of a cohort of bronchiectatic patients was investigated to detect the frequency of significant antibody deficiency and to determine the extent of immunological investigation which is appropriate for routine assessment of bronchiectasis patients. Fifty-six out-patients (with a mean age of 59.6 years) had serum immunoglobulins, IgG subclasses and specific antibodies to capsular polysaccharides of Haemophilus influenzae and Streptococcus pneumoniae measured. Where specific antibody -levels were low, where possible, appropriate immunization with pneumococcal or conjugated Haemophilus polysaccharide vaccines was offered and the responses quantified. Three of 56 patients had low total serum IgG levels. Thirteen of 56 had deficiencies of either a single IgG subclass or combinations of two or more subclasses, with IgG4 being most frequently implicated (9/56). Twenty-nine of 56 had low basal specific polysaccharide antibody levels. Test immunization, where performed, produced satisfactory responses in all cases except one, where a specific defect of responsiveness to pneumococcal polysaccharide was identified. This study indicates that antibody deficiency is an uncommon aetiological/underlying factor in the causation of bronchiectasis beyond the fourth decade and that detailed investigation of humoral immune status as a routine in bronchiectasis patients, at least at this age, is not generally justified.
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Affiliation(s)
- A Stead
- Aberdeen College, Gallowgate Centre, Aberdeen, UK
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48
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Chang AB, Boyce NC, Masters IB, Torzillo PJ, Masel JP. Bronchoscopic findings in children with non-cystic fibrosis chronic suppurative lung disease. Thorax 2002; 57:935-8. [PMID: 12403874 PMCID: PMC1746234 DOI: 10.1136/thorax.57.11.935] [Citation(s) in RCA: 57] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022]
Abstract
BACKGROUND Published data on the frequency and types of flexible bronchoscopic airway appearances in children with non-cystic fibrosis bronchiectasis and chronic suppurative lung disease are unavailable. The aims of this study were to describe airway appearances and frequency of airway abnormalities and to relate these airway abnormalities to chest high resolution computed tomography (cHRCT) findings in a cohort of children with non-cystic fibrosis chronic suppurative lung disease (CSLD). METHODS Indigenous children with non-cystic fibrosis CSLD (>4 months moist and/or productive cough) were prospectively identified and collected over a 2.5 year period at two paediatric centres. Their medical charts and bronchoscopic notes were retrospectively reviewed. RESULTS In all but one child the aetiology of the bronchiectasis was presumed to be following a respiratory infection. Thirty three of the 65 children with CSLD underwent bronchoscopy and five major types of airway findings were identified (mucosal abnormality/inflammation only, bronchomalacia, obliterative-like lesion, malacia/obliterative-like combination, and no macroscopic abnormality). The obliterative-like lesion, previously undescribed, was present in 16.7% of bronchiectatic lobes. Structural airway lesions (bronchomalacia and/or obliterative-like lesion) were present in 39.7% of children. These lesions, when present, corresponded to the site of abnormality on the cHRCT scan. CONCLUSIONS Structural airway abnormality is commonly found in children with post-infectious bronchiectasis and a new bronchoscopic finding has been described. Airway abnormalities, when present, related to the same lobe abnormality on the cHRCT scan. How these airway abnormalities relate to aetiology, management strategy, and prognosis is unknown.
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Affiliation(s)
- A B Chang
- Department of Respiratory Medicine, Royal Children's Hospital, Herston, Queensland, Australia.
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Chang AB, Grimwood K, Mulholland EK, Torzillo PJ. Bronchiectasis in indigenous children in remote Australian communities. Med J Aust 2002; 177:200-4. [PMID: 12175325 DOI: 10.5694/j.1326-5377.2002.tb04733.x] [Citation(s) in RCA: 151] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2002] [Accepted: 05/30/2002] [Indexed: 11/17/2022]
Abstract
The rates of bronchiectasis for Indigenous children from remote Australian communities are unacceptably high, with one study showing 14.7/1000 Aboriginal children. Children with bronchiectasis need to be identified early for optimisation of medical treatment. Under-reporting of cough is common. Bronchiectasis should be suspected in children with recurrent bronchitis or pneumonia, and when, despite appropriate therapy, pulmonary infiltrates or atelectasis persist 12 weeks beyond the index illness. During acute infective episodes, oral antibiotics and chest physiotherapy to clear the airways should produce prompt resolution; otherwise, hospitalisation is necessary. Management follows the cystic fibrosis model of regular review, encouragement of physical activity, optimising nutrition, maintenance of immunisation and avoidance of environmental toxicants, including passive smoke exposure. Successful management and prevention of bronchiectasis will require improvements in housing, nutrition, and education, as well as access to comprehensive healthcare services, with coordination between primary and hospital-based healthcare providers.
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Affiliation(s)
- Anne B Chang
- Northern Territory Clinical School, Flinders University, Alice Springs, NT.
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50
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Abstract
More than a decade ago, bronchiectasis unrelated to cystic fibrosis was termed an "orphan disease", because it had become an uncommon clinical entity among children in the developed world. Bronchiectasis is more common among children in lower socioeconomic classes and in developing countries, presumably due to more frequent and recurrent respiratory infections, environmental airway irritants, poor immunization rates, and malnutrition. Reports from the Southern Pacific and from Alaska Native children reveal persistently high rates of childhood bronchiectasis. Better epidemiologic data throughout the world are needed to reassess the importance of this condition. The pathophysiology includes airway inflammation, mucus production, and regional airway obstruction, yet the reasons why some children develop bronchiectasis while other do not is unclear. The coexistence of asthma with bronchiectasis is associated with more severe disease, yet the impact of asthma therapy in children with both disorders has not been studied. Similarly, the pattern of antibiotic use for children with bronchiectasis varies by region with little data to justify one particular approach. It may be that public health measures aimed at improving living conditions for children and prevention of respiratory infections with antiviral vaccines will have more impact on childhood bronchiectasis than medical treatments in the future.
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Affiliation(s)
- Charles W Callahan
- Department of Pediatrics, Tripler Army Medical Center, Honolulu, Hawaii 96859-5000, USA.
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