For Publishers
DiscoveryMetadataPeer reviewHostingPublishing
For Researchers
JoinPublishReviewCollect
Register
Blog
About
Search
Advanced search
My ScienceOpen
Search
For Publishers
For Researchers
Blog
About
1
views
17
references
 Top references
cited by
7
    
0 reviews
 Review
0
comments
 Comment
0
recommends
+1 Recommend
0 collections
    0
    shares
      198
      similar
       All similar
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Double lung transplantation for end-stage Kartagener syndrome: a case report and literature review

      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

          Kartagener syndrome (KS) is an autosomal recessive disorder characterized by situs inversus, paranasal sinusitis and bronchiectasis. We report the successful use of double lung transplant (DLTx) to treat end-stage KS. A 49-year-old Han woman was admitted to Renmin Hospital (Wuhan University, China) in September 2017 with a ≥15 year history of chronic productive cough that had worsened during the past year. Clinical examination and imaging investigations revealed respiratory failure and situs inversus consistent with KS. The patient was successfully treated with DLTx involving bilateral bronchial anastomoses. DLTx is a feasible treatment option for end-stage KS.

          Related collections

          Most cited references17

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

          Primary Ciliary Dyskinesia.

          Michael John Knowles, Maimoona Zariwala, Margaret Leigh (2016)
          Primary ciliary dyskinesia (PCD) is a recessive genetically heterogeneous disorder of motile cilia with chronic otosinopulmonary disease and organ laterality defects in ∼50% of cases. The prevalence of PCD is difficult to determine. Recent diagnostic advances through measurement of nasal nitric oxide and genetic testing has allowed rigorous diagnoses and determination of a robust clinical phenotype, which includes neonatal respiratory distress, daily nasal congestion, and wet cough starting early in life, along with organ laterality defects. There is early onset of lung disease in PCD with abnormal airflow mechanics and radiographic abnormalities detected in infancy and early childhood.
          Article 0 comments Cited 87 times – based on 0 reviews     Review now
            Bookmark
            • Record: found
            • Abstract: found
            • Article: found
            Is Open Access

            Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review

            Adam Shapiro, Maimoona Zariwala, Thomas Ferkol … (2015)
            Summary Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, rare lung disease resulting in chronic oto‐sino‐pulmonary disease in both children and adults. Many physicians incorrectly diagnose PCD or eliminate PCD from their differential diagnosis due to inexperience with diagnostic testing methods. Thus far, all therapies used for PCD are unproven through large clinical trials. This review article outlines consensus recommendations from PCD physicians in North America who have been engaged in a PCD centered research consortium for the last 10 years. These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long‐term therapeutics in PCD patients. Pediatr Pulmonol. 2016;51:115–132. © 2015 The Authors. Pediatric Pulmonology Published by Wiley Periodicals, Inc.
            Article 0 comments Cited 81 times – based on 0 reviews     Review now
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome.

              Ian Davis, L. Carson, Jessica Leigh … (2009)
              Primary ciliary dyskinesia is a genetically heterogeneous disorder of motile cilia. Most of the disease-causing mutations identified to date involve the heavy (dynein axonemal heavy chain 5) or intermediate(dynein axonemal intermediate chain 1) chain dynein genes in ciliary outer dynein arms, although a few mutations have been noted in other genes. Clinical molecular genetic testing for primary ciliary dyskinesia is available for the most common mutations. The respiratory manifestations of primary ciliary dyskinesia (chronic bronchitis leading to bronchiectasis, chronic rhino-sinusitis, and chronic otitis media)reflect impaired mucociliary clearance owing to defective axonemal structure. Ciliary ultrastructural analysis in most patients (>80%) reveals defective dynein arms, although defects in other axonemal components have also been observed. Approximately 50% of patients with primary ciliary dyskinesia have laterality defects (including situs inversus totalis and, less commonly, heterotaxy, and congenital heart disease),reflecting dysfunction of embryological nodal cilia. Male infertility is common and reflects defects in sperm tail axonemes. Most patients with primary ciliary dyskinesia have a history of neonatal respiratory distress, suggesting that motile cilia play a role in fluid clearance during the transition from a fetal to neonatal lung. Ciliopathies involving sensory cilia, including autosomal dominant or recessive polycystic kidney disease, Bardet-Biedl syndrome, and Alstrom syndrome, may have chronic respiratory symptoms and even bronchiectasis suggesting clinical overlap with primary ciliary dyskinesia.
              Article 0 comments Cited 65 times – based on 0 reviews     Review now
                Bookmark
                All references

                Author and article information

                Journal
                Journal ID (nlm-ta): J Thorac Dis
                Journal ID (iso-abbrev): J Thorac Dis
                Journal ID (publisher-id): JTD
                Title: Journal of Thoracic Disease
                Publisher: AME Publishing Company
                ISSN (Print): 2072-1439
                ISSN (Electronic): 2077-6624
                Publication date (Print and electronic): April 2020
                Publication date (Print): April 2020
                Volume: 12
                Issue: 4
                Pages: 1588-1594
                Affiliations
                [1 ]Department of Thoracic Surgery, Renmin Hospital of Wuhan University , Wuhan 430060, China;
                [2 ]Department of Thoracic Surgery, Nanjing Medical University, Affiliated Wuxi People’s Hospital , Wuxi 214000, China;
                [3 ]Division of Cardiothoracic Surgery, Washington University in St. Louis , Saint Louis, Missouri, USA;
                [4 ]Department of Thoracic Surgery and Anesthesia, Lung Center of the Philippines , Quezon City, Philippines;
                [5 ]Department of Cardiothoracic Surgery, Queen Mary Hospital, High West, Hong Kong, China
                Author notes
                Correspondence to: Huiqing Lin. Department of Thoracic Surgery, Renmin Hospital of Wuhan University, Wuhan 430060, China. Email: huiqing.lin@ 123456whu.edu.cn .
                Article
                Publisher ID: jtd-12-04-1588
                DOI: 10.21037/jtd.2020.02.28
                PMC ID: 7212169
                PubMed ID: 32395296
                SO-VID: aa81b8f6-6225-4643-832b-44033819fcfa
                Copyright © 2020 Journal of Thoracic Disease. 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 : 08 May 2019
                Date accepted : 04 February 2020
                Categories
                Subject: iMDT Corner

                Keywords: kartagener syndrome,lung transplantation,primary ciliary dyskinesia,situs inversus
                Data availability:
                Keywords: kartagener syndrome, lung transplantation, primary ciliary dyskinesia, situs inversus

                Comments

                Comment on this article

                scite_

                Similar content198

                See all similar

                Cited by7

                See all cited by

                Most referenced authors186

                See all reference authors