Non-cystic fibrosis bronchiectasis in children: Clinical profile, etiology and outcome

Kumar, Amit; Lodha, Rakesh; Kumar, Prawin; Kabra, Sushil Kumar

doi:10.1007/s13312-015-0563-8

Cited by 39 publications

(28 citation statements)

References 7 publications

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“…In contrast to China however, work from Thailand (n = 50) indicates that post-infection related bronchiectasis and specifically post-tuberculosis associated disease was commonest. Similarly, a high prevalence of post-infectious bronchiectasis was reported in Indian children (n = 80) followed by primary ciliary dyskinesia and ABPA [61,62]. A high prevalence of bronchiectasis is reported in South Korea (n = 1409) and in one particular study of respiratory patients, 9% were deemed to have bronchiectasis with higher prevalence in females [63].…”

Section: Bronchiectasis In the Asia-pacific Regionmentioning

confidence: 85%

Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis

et al. 2018

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Bronchiectasis is a disease associated with chronic progressive and irreversible dilatation of the bronchi and is characterised by chronic infection and associated inflammation. The prevalence of bronchiectasis is age-related and there is some geographical variation in incidence, prevalence and clinical features. Most bronchiectasis is reported to be idiopathic however post-infectious aetiologies dominate across Asia especially secondary to tuberculosis. Most focus to date has been on the study of airway bacteria, both as colonisers and causes of exacerbations. Modern molecular technologies including next generation sequencing (NGS) have become invaluable tools to identify microorganisms directly from sputum and which are difficult to culture using traditional agar based methods. These have provided important insight into our understanding of emerging pathogens in the airways of people with bronchiectasis and the geographical differences that occur. The contribution of the lung microbiome, its ethnic variation, and subsequent roles in disease progression and response to therapy across geographic regions warrant further investigation. This review summarises the known geographical differences in the aetiology, epidemiology and microbiology of bronchiectasis. Further, we highlight the opportunities offered by emerging molecular technologies such as -omics to further dissect out important ethnic differences in the prognosis and management of bronchiectasis.

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Section: Bronchiectasis In the Asia-pacific Regionmentioning

confidence: 85%

Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis

et al. 2018

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“…The underlying cause of bronchiectasis was PCD in 1–17% of several pediatric case series (33–35). Although the development of bronchiectasis increases with aging (28), it has been reported even in toddlers with PCD (36).…”

Section: Disease Manifestationsmentioning

confidence: 99%

Primary Ciliary Dyskinesia: An Update on Clinical Aspects, Genetics, Diagnosis, and Future Treatment Strategies

2017

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Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities. Most patients report a daily year-round wet cough or nose congestion starting in the first year of life. Daily wet cough, associated with recurrent infections exacerbations, results in the development of chronic suppurative lung disease, with localized-to-diffuse bronchiectasis. No diagnostic test is perfect for confirming PCD. Diagnosis can be challenging and relies on a combination of clinical data, nasal nitric oxide levels plus cilia ultrastructure and function analysis. Adjunctive tests include genetic analysis and repeated tests in ciliary culture specimens. There are currently 33 known genes associated with PCD and correlations between genotype and ultrastructural defects have been increasingly demonstrated. Comprehensive genetic testing may hopefully screen young infants before symptoms occur, thus improving survival. Recent surprising advances in PCD genetic designed a novel approach called “gene editing” to restore gene function and normalize ciliary motility, opening up new avenues for treating PCD. Currently, there are no data from randomized clinical trials to support any specific treatment, thus, management strategies are usually extrapolated from cystic fibrosis. The goal of treatment is to prevent exacerbations, slowing the progression of lung disease. The therapeutic mainstay includes airway clearance maneuvers mainly with nebulized hypertonic saline and chest physiotherapy, and prompt and aggressive administration of antibiotics. Standardized care at specialized centers using a multidisciplinary approach that imposes surveillance of lung function and of airway biofilm composition likely improves patients’ outcome. Pediatricians, neonatologists, pulmonologists, and ENT surgeons should maintain high awareness of PCD and refer patients to the specialized center before sustained irreversible lung damage develops. The recent creation of a network of PCD clinical centers, focusing on improving diagnosis and treatment, will hopefully help to improve care and knowledge of PCD patients.

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“…Surgical intervention is uncommon in children. However, those with severe, poorly controlled, localized disease, or recurrent hemoptysis may require surgical resection of a bronchiectatic lobe …”

Section: Treatments For Bronchiectasis (References In the Text)mentioning

confidence: 99%

Pediatric bronchiectasis: No longer an orphan disease

Goyal

Grimwood

Marchant

et al. 2016

Pediatric Pulmonology

137

140

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Bronchiectasis is described classically as a chronic pulmonary disorder characterized by a persistent productive cough and irreversible dilatation of one or more bronchi. However, in children unable to expectorate, cough may instead be wet and intermittent and bronchial dilatation reversible in the early stages. Although still considered an orphan disease, it is being recognized increasingly as causing significant morbidity and mortality in children and adults in both affluent and developing countries. While bronchiectasis has multiple etiologies, the final common pathway involves a complex interplay between the host, respiratory pathogens and environmental factors. These interactions lead to a vicious cycle of repeated infections, airway inflammation and tissue remodelling resulting in impaired airway clearance, destruction of structural elements within the bronchial wall causing them to become dilated and small airway obstruction. In this review, the current knowledge of the epidemiology, pathobiology, clinical features, and management of bronchiectasis in children are summarized. Recent evidence has emerged to improve our understanding of this heterogeneous disease including the role of viruses, and how antibiotics, novel drugs, antiviral agents, and vaccines might be used. Importantly, the management is no longer dependent upon extrapolating from the cystic fibrosis experience. Nevertheless, substantial information gaps remain in determining the underlying disease mechanisms that initiate and sustain the pathophysiological pathways leading to bronchiectasis. National and international collaborations, standardizing definitions of clinical and research end points, and exploring novel primary prevention strategies are needed if further progress is to be made in understanding, treating and even preventing this often life-limiting disease.

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Non-cystic fibrosis bronchiectasis in children: Clinical profile, etiology and outcome

Abstract: Common causes of non-cystic fibrosis bronchiectasis are post infectious and primary ciliary dyskinesia. There is a need to create awareness about early diagnosis of bronchiectasis as it is often delayed.

Cited by 39 publications

References 7 publications

Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis

Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis

Primary Ciliary Dyskinesia: An Update on Clinical Aspects, Genetics, Diagnosis, and Future Treatment Strategies

Pediatric bronchiectasis: No longer an orphan disease

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