Premium
Rapidly progressing case of Mounier‐Kuhn Syndrome in early childhood
Author(s) -
Hubbard Melissa,
Masters I. Brent,
Chang Anne B.
Publication year - 2003
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.10347
Subject(s) - medicine , pneumonia , pediatrics , etiology , respiratory failure , incidence (geometry) , respiratory disease , bronchoscopy , tracheobronchomalacia , high resolution computed tomography , surgery , computed tomography , lung , physics , optics
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. Pediatr Pulmonol. 2003; 36:353–356. © 2003 Wiley‐Liss, Inc.