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Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years.
MedLine Citation:
PMID:  25130790     Owner:  NLM     Status:  Publisher    
Mounier-Kuhn syndrome is a rare disease with abnormal enlargement of major airways, but epidemiological studies are lacking and currently the most available data about it come from case reports, making it difficult to collate changes in a particular patient to those in previously published cases. The aim of this work is to systematically review cases published in the last 25 years and to use descriptive statistics to summarize the patient demographic and clinical information therein in order to acquire details about patient clinical characteristics. Cases published in world literature between 1987 and 2013 were sought and reviewed according to PRISMA guidelines. Cases were included only if patient's age was at least 18 years and a CT scan with tracheal measurements was available. A 8:1 male predominance was found in 89 identified reports (128 cases). Mean age was 53.9 years, average tracheal diameter - 36.1 mm. No correlation between increasing age and increasing tracheal diameter was found. Bronchiectasis, tracheal diverticulosis and tracheobronchial dyskinesia were common (49.2%, 33.6% and 28.9% respectively). Cough, dyspnoea and recurrent respiratory infections (71.1%, 51.6% and 50.8% respectively) were the most common complaints. The data confirm that syndrome mostly manifests with non-specific respiratory symptoms and is significantly more common in males. Importantly there was no connection between age and airway diameter, a fact which could mean that the enlargement does not progress over time and its severity depends on some other yet undetermined factors.
Eduards Krustins
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-8-3
Journal Detail:
Title:  The clinical respiratory journal     Volume:  -     ISSN:  1752-699X     ISO Abbreviation:  Clin Respir J     Publication Date:  2014 Aug 
Date Detail:
Created Date:  2014-8-18     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101315570     Medline TA:  Clin Respir J     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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This article is protected by copyright. All rights reserved.
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