For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
0
views
8
references
 Top references
cited by
0
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      55
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Resolving persistent air leaks associated with autosomal dominant hyper-IgE syndrome using one-way endobronchial valves: report of cases

      case-report

      Read this article at

      ScienceOpenPublisherPMC
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Background

          Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare genetic syndrome that alters typical post-operative wound healing. AD-HIES patients are prone to develop persistent air leaks (PALs) due to bronchopleural fistulas. This report is unique in that it describes a novel approach to managing PALs in this complex population.

          Case Description

          Two patients with AD-HIES were identified in the setting of a PAL. The first patient was a 31-year-old male with recurrent pneumonia, who developed a large hydropneumothorax following re-presentation with fever and cough. A chest tube was inserted, which required continuous suction in the setting of what developed into a PAL. Subsequently, an endobronchial valve (EBV) was deployed to successfully manage the PAL. The second patient was a 25-year-old male, who developed a post-operative large volume air leak following a complicated surgical resection of a giant pneumatocele. Several attempts of placing multiple EBVs were required to finally address the PAL. In both cases, EBVs were successfully employed to manage and eventually resolve symptoms caused by PAL.

          Conclusions

          Our experience suggests that EBVs are successful in treating PAL in the setting of AD-HIES, which often manifests as highly complex scenarios. Hence, EBVs represent a valuable addition to the therapeutic armamentarium against recalcitrant PAL. EBVs were well-tolerated in patients afflicted by AD-HIES, with no progressive infections noted. Both patients ultimately were able to resolve their PAL following placement of the EBV.

          Related collections

          Most cited references8

          • Record: found
          • Abstract: found
          • Article: not found

          STAT3 mutations in the hyper-IgE syndrome.

          Steven M. Holland, Frank DeLeo, Houda Z Elloumi … (2007)
          The hyper-IgE syndrome (or Job's syndrome) is a rare disorder of immunity and connective tissue characterized by dermatitis, boils, cyst-forming pneumonias, elevated serum IgE levels, retained primary dentition, and bone abnormalities. Inheritance is autosomal dominant; sporadic cases are also found. We collected longitudinal clinical data on patients with the hyper-IgE syndrome and their families and assayed the levels of cytokines secreted by stimulated leukocytes and the gene expression in resting and stimulated cells. These data implicated the signal transducer and activator of transcription 3 gene (STAT3) as a candidate gene, which we then sequenced. We found increased levels of proinflammatory gene transcripts in unstimulated peripheral-blood neutrophils and mononuclear cells from patients with the hyper-IgE syndrome, as compared with levels in control cells. In vitro cultures of mononuclear cells from patients that were stimulated with lipopolysaccharide, with or without interferon-gamma, had higher tumor necrosis factor alpha levels than did identically treated cells from unaffected persons (P=0.003). In contrast, the cells from patients with the hyper-IgE syndrome generated lower levels of monocyte chemoattractant protein 1 in response to the presence of interleukin-6 (P=0.03), suggesting a defect in interleukin-6 signaling through its downstream mediators, one of which is STAT3. We identified missense mutations and single-codon in-frame deletions in STAT3 in 50 familial and sporadic cases of the hyper-IgE syndrome. Eighteen discrete mutations, five of which were hot spots, were predicted to directly affect the DNA-binding and SRC homology 2 (SH2) domains. Mutations in STAT3 underlie sporadic and dominant forms of the hyper-IgE syndrome, an immunodeficiency syndrome involving increased innate immune response, recurrent infections, and complex somatic features. Copyright 2007 Massachusetts Medical Society.
          Article 0 comments Cited 127 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            IL-6/STAT3 promotes regeneration of airway ciliated cells from basal stem cells.

            Larry S Barak, Brigid L M Hogan, T Tadokoro … (2014)
            The pseudostratified airway epithelium of the lung contains a balanced proportion of multiciliated and secretory luminal cells that are maintained and regenerated by a population of basal stem cells. However, little is known about how these processes are modulated in vivo, and about the potential role of cytokine signaling between stem and progenitor cells and their niche. Using a clonal 3D organoid assay, we found that IL-6 stimulated, and Stat3 inhibitors reduced, the generation of ciliated vs. secretory cells from basal cells. Gain-of-function and loss-of-function studies with cultured mouse and human basal cells suggest that IL-6/Stat3 signaling promotes ciliogenesis at multiple levels, including increases in multicilin gene and forkhead box protein J1 expression and inhibition of the Notch pathway. To test the role of IL-6 in vivo genetically, we followed the regeneration of mouse tracheal epithelium after ablation of luminal cells by inhaled SO2. Stat3 is activated in basal cells and their daughters early in the repair process, correlating with an increase in Il-6 expression in platelet-derived growth factor receptor alpha(+) mesenchymal cells in the stroma. Conditional deletion in basal cells of suppressor of cytokine signaling 3, encoding a negative regulator of the Stat3 pathway, results in an increase in multiciliated cells at the expense of secretory and basal cells. By contrast, Il-6 null mice regenerate fewer ciliated cells and an increased number of secretory cells after injury. The results support a model in which IL-6, produced in the reparative niche, functions to enhance the differentiation of basal cells, and thereby acts as a "friend" to promote airway repair rather than a "foe."
            Article 0 comments Cited 113 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Treatment of persistent pulmonary air leaks using endobronchial valves.

              John Travaline, Robert McKenna, Tiziano De Giacomo … (2009)
              Prolonged pulmonary air leaks are a significant source of frustration for patients and physicians. When conventional therapy fails, an alternative to prolonged chest tube drainage or surgery is needed. Bronchoscopic blockage of a bronchus can be performed with the hope of accelerating closure of the air leak by reducing the flow of air through the leak. To our knowledge, this article presents the largest series of patients with prolonged air leaks treated with an endobronchial valve. With Internal Review Board approval, endobronchial valves were compassionately placed using flexible bronchoscopy in patients with prolonged air leaks at 17 international sites. Between December 2002 and January 2007, 40 patients (15 women; mean age +/- SD, 60 +/- 14 years) were treated with one to nine endobronchial valves per patient. The air leaks had recurrent spontaneous pneumothorax (n = 21), postoperative (n = 7), iatrogenic (n = 6), first-time spontaneous pneumothorax (n = 4), bronchoscopic lung volume reduction (n = 1), and trauma (n = 1) etiologies. Nineteen patients (47.5%) had a complete resolution of the air leak, 18 (45%) had a reduction, 2 had no change, and 1 had no reported outcome. The mean time from valve insertion to chest tube removal was 21 days (median, 7.5 days; interquartile range [IQR], 3 to 29 days) and from valve procedure to hospital discharge was 19 +/- 28 days (median, 11 days; IQR, 4 to 27 days). Use of endobronchial valves is an effective, nonsurgical, minimally invasive intervention for patients with prolonged pulmonary air leaks.
              Article 0 comments Cited 35 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Journal
                Journal ID (nlm-ta): AME Case Rep
                Journal ID (iso-abbrev): AME Case Rep
                Journal ID (publisher-id): ACR
                Title: AME Case Reports
                Publisher: AME Publishing Company
                ISSN (Electronic): 2523-1995
                Publication date (Electronic): 20 March 2024
                Publication date Collection: 2024
                Volume: 8
                Electronic Location Identifier: 43
                Affiliations
                [1 ]deptDepartment of Surgery , Walter Reed National Military Medical Center , Bethesda, MD, USA;
                [2 ]deptLaboratory of Clinical Immunology & Microbiology , National Institute of Allergy & Infectious Diseases (NIAID) , Bethesda, MD, USA;
                [3 ]deptClinical Center , National Institute of Allergy & Infectious Diseases (NIAID) National Institutes of Health (NIH) , Bethesda, MD, USA;
                [4 ]deptPulmonary Branch, National Heart, Lung and Blood Institute , National Institutes of Health (NIH) , Bethesda, MD, USA;
                [5 ]deptThoracic Surgery Branch, National Cancer Institute , National Institutes of Health (NIH) , Bethesda, MD, USA
                Author notes

                Contributions: (I) Conception and design: AF Freeman, CD Hoang; (II) Administrative support: NN Sartain; (III) Provision of study materials or patients: AF Freeman, KN Olivier; (IV) Collection and assembly of data: All authors; (V) Data analysis and interpretation: KN Olivier, AF Freeman, CD Hoang; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.

                Correspondence to: Alexandra F. Freeman, MD. Laboratory of Clinical Immunology & Microbiology, National Institute of Allergy & Infectious Diseases (NIAID), NIH Building 10 Room 12C103, 9000 Rockville Pike, Bethesda, MD 20892, USA. Email: freemaal@ 123456mail.nih.gov .
                Author information
                https://orcid.org/0000-0003-4016-1434
                Article
                Publisher ID: acr-08-23-35
                DOI: 10.21037/acr-23-35
                PMC ID: 11070981
                PubMed ID: 38711888
                SO-VID: a3fc8383-58c3-4058-a5a5-4cfd7de6caa1
                Copyright © 2024 AME Case Reports. All rights reserved.
                License:

                Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0.

                History
                Date received : 07 March 2023
                Date accepted : 27 October 2023
                Funding
                Funded by: the National Institutes of Health (NIH) Intramural Research Program
                Award ID: No. ZIA BC 011657
                Categories
                Subject: Case Report

                Keywords: endobronchial valves (ebvs),persistent air leak (pal),case report
                Data availability:
                Keywords: endobronchial valves (ebvs), persistent air leak (pal), case report

                Comments

                Comment on this article