Document Detail


Rapidly progressing case of Mounier-Kuhn Syndrome in early childhood.
MedLine Citation:
PMID:  12950051     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Reports of Mounier-Kuhn Syndrome in childhood are extremely rare, as it usually presents in the third or fourth decades. In the only other report of a case diagnosed in early childhood, the child was well at the time of publication. We report on a 15-month-old boy who presented with his first respiratory illness at age 3 months. His disease course was more severe than previously reported. Diagnosis was characteristically delayed until acute respiratory failure complicated a respiratory tract infection at 13 months, prompting high-resolution computerized tomography (HRCT) of the chest. He gradually deteriorated, eventually requiring ventilatory support; death occurred at age 15 months. This report illustrates the clinical heterogeneity of the syndrome. We review the theories about etiology and the recognized clinical findings in adults. Incidence may be higher than previously estimated, and tracheobronchomegaly (TBM) should be considered as a cause of respiratory failure and recurrent pneumonia in children where other investigations, including chest X-ray (CXR), are normal. A chest HRCT (cHRCT) scan and bronchoscopy are usually diagnostic.
Authors:
Melissa Hubbard; I Brent Masters; Anne B Chang
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric pulmonology     Volume:  36     ISSN:  8755-6863     ISO Abbreviation:  Pediatr. Pulmonol.     Publication Date:  2003 Oct 
Date Detail:
Created Date:  2003-09-01     Completed Date:  2004-02-05     Revised Date:  2006-03-28    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  353-6     Citation Subset:  IM    
Copyright Information:
Copyright 2003 Wiley-Liss, Inc.
Affiliation:
Academic Department of Paediatrics, City General Hospital, Stoke on Trent, UK.
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MeSH Terms
Descriptor/Qualifier:
Bronchi
Bronchography
Bronchoscopy
Disease Progression
Fatal Outcome
Humans
Infant
Male
Tomography, X-Ray Computed
Tracheobronchomegaly / diagnosis*,  etiology,  radiography,  therapy

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