Document Detail

Alpha-1 antitrypsin deficiency and computed tomography findings.
MedLine Citation:
PMID:  16012317     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To investigate the severity of bronchiectasis and associated emphysema and the correlation with phenotype in patients with Alpha-1 antitrypsin deficiency. METHODS: The scoring system of Ooi and his colleagues for bronchiectasis was modified to include the degree of dilatation of bronchi in affected segments and degree of emphysema. This was applied to 26 high-resolution computed tomography thorax scans of the study population. All criteria were scored on a scale of 0-3. RESULTS: Nine patients (35%) were female and 17 (65%) were male. The median age was 56 years (range: 17-76 years). Twenty-one patients had a ZZ phenotype, 3 patients had an MZ phenotype, and 2 patients had an SZ phenotype. The median forced expiratory volume in 1 second/forced vital capacity ratio was 43% (range: 24%-87%). A total of 156 lobes were assessed, and 38 (24%) had evidence of bronchiectasis. The overall median total score in affected patients for the extent of bronchiectasis was 2, and all had a ZZ phenotype. Fourteen patients (54%) had a degree of dilatation score of 1 or more, all had a ZZ phenotype, and 4 (15%) had no evidence of emphysema. Bronchiectasis was seen most commonly affecting the upper lobes. CONCLUSION: The ZZ phenotype was associated with bronchiectasis most commonly affecting the upper lobes, with moderate emphysema throughout all lobes. Numbers of patients having the SZ and MZ phenotypes are too small to derive accurate conclusions, but none had evidence of bronchiectasis.
Michelle A McMahon; Michael J O'Mahony; Shane J O'Neill; Noel G McElvaney; P Mark Logan
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of computer assisted tomography     Volume:  29     ISSN:  0363-8715     ISO Abbreviation:  J Comput Assist Tomogr     Publication Date:    2005 Jul-Aug
Date Detail:
Created Date:  2005-07-13     Completed Date:  2005-08-16     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7703942     Medline TA:  J Comput Assist Tomogr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  549-53     Citation Subset:  IM    
Department of Radiology, Beaumont Hospital, Dublin 9, Ireland.
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MeSH Terms
Bronchiectasis / etiology*,  pathology,  radiography
Emphysema / etiology*,  pathology,  radiography
Middle Aged
Retrospective Studies
Severity of Illness Index
Tomography, X-Ray Computed
alpha 1-Antitrypsin Deficiency / radiography*

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