A Review of Non-Cystic Fibrosis Pediatric Bronchiectasis

Boren, Eric J.; Teuber, Suzanne S.; Gershwin, M. Eric

doi:10.1007/s12016-007-8036-z

Cited by 21 publications

(29 citation statements)

References 63 publications

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“…Bacterial pathogens were identified in the airways of all immunodeficient children with bronchiectasis and the most common isolates were Streptococcus pneumoniae, followed by Haemophilus influenzae and Pseudomonas aeruginosa, with the first microorganism associated with the most severe bronchiectasis. Similar results from sputum cultures from patients with non-cystic fibrosis bronchiectasis were reported in other investigations [18].…”

Section: Discussionsupporting

confidence: 90%

Clinical immunology Bronchiectasis in children with primary immune deficiency diseases

Szczawińska-Popłonyk¹,

Jończyk‐Potoczna²,

Kycler³

et al. 2012

cejoi

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Background: Primary immune deficiencies are a common cause of bronchiectasis in children.Aim of the study: The aim of this study was to evaluate the incidence and characteristic radiological findings of bronchiectasis in children with primary immune deficiencies. Material and methods: A retrospective review of medical records and pulmonary imaging (chest radiographs and high resolution computed tomography scans) of forty-one patients with primary immune deficiency diseases, aged between 6 months and 18 years was undertaken. Results: Based on high resolution computer tomography (HRCT) imaging, the diagnosis of bronchiectasis was established in 5 patients (12.2%), with Bruton's agammaglobulinemia (XLA), common variable immunodeficiency (CVID) and the hyperimmunoglobulin E syndrome (HIES). In 3 children (7.2%), two with IgG subclass deficiencies (IgGsD) and in one with HIES, pre-bronchiectasis was demonstrated. The HRCT features of bronchiectasis were predominatingly detected in the basilar segments of the lower lobes, followed by the right middle lobe and the lingula. The median age of 5 children with bronchiectasis visualized in HRCT imaging was 13 years. Pyogenic bacteria were recovered in the respiratory tract of all the children with bronchiectasis, with Streptococcus pneumoniae most frequently identified.Conclusions: Bronchiectasis is a frequent pulmonary complication and should be actively looked for in children with primary immune deficiencies and lung infections.

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Section: Discussionsupporting

confidence: 90%

Clinical immunology Bronchiectasis in children with primary immune deficiency diseases

Szczawińska-Popłonyk¹,

Jończyk‐Potoczna²,

Kycler³

et al. 2012

cejoi

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“…Here, we found that acidity in CF ASL is causal for increased ENaC cleavage/ activity and CF ASL volume depletion, suggesting that many of the defects in CF lung innate defense have a similar etiology, namely, reduced ASL pH. Gastric reflux is common in subjects with CF and has also been linked with lung disease in patients who do not have CF (30,31). Thus, in addition to the reduced pH caused by CFTR's absence, exposure of very low pH due to gastric reflux may further exacerbate this issue in patients with CF and contribute to a failure of mucus clearance in patients who do not have CF and are prone to gastric reflux.…”

Section: Discussionmentioning

confidence: 82%

Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways

Garland¹,

Walton²,

Coakley³

et al. 2013

Proc. Natl. Acad. Sci. U.S.A.

168

264

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The ability to maintain proper airway surface liquid (ASL) volume homeostasis is vital for mucus hydration and clearance, which are essential aspects of the mammalian lung's innate defense system. In cystic fibrosis (CF), one of the most common life-threatening genetic disorders, ASL dehydration leads to mucus accumulation and chronic infection. In normal airways, the secreted protein short palate lung and nasal epithelial clone 1 (SPLUNC1) effectively inhibits epithelial Na + channel (ENaC)-dependent Na + absorption and preserves ASL volume. In CF airways, it has been hypothesized that increased ENaC-dependent Na + absorption contributes to ASL depletion, and hence increased disease. However, this theory is controversial, and the mechanism for abnormal ENaC regulation in CF airways has remained elusive. Here, we show that SPLUNC1 is a pH-sensitive regulator of ENaC and is unable to inhibit ENaC in the acidic CF airway environment. Alkalinization of CF airway cultures prevented CF ASL hyperabsorption, and this effect was abolished when SPLUNC1 was stably knocked down. Accordingly, we resolved the crystal structure of SPLUNC1 to 2.8 Å. Notably, this structure revealed two pH-sensitive salt bridges that, when removed, rendered SPLUNC1 pH-insensitive and able to regulate ASL volume in acidic ASL. Thus, we conclude that ENaC hyperactivity is secondary to reduced CF ASL pH. Together, these data provide molecular insights into the mucosal dehydration associated with a range of pulmonary diseases, including CF, and suggest that future therapy be directed toward alkalinizing the pH of CF airways.bacterial permeability-increasing protein | ion channels | COPD innate defense

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“…Bronchiectasis is defined as an irreversible dilatation of the bronchi and is the end result of chronic inflammation and infection of the bronchi 19 (Fig. 9).…”

Section: Bronchiectasismentioning

confidence: 99%