Document Detail

Short-term supplementation therapy does not affect elastin degradation in severe alpha(1)-antitrypsin deficiency. The American-Italian AATD Study Group.
MedLine Citation:
PMID:  11112116     Owner:  NLM     Status:  MEDLINE    
We evaluated the ability of intravenous supplementation therapy with alpha(1)-antitrypsin (AAT) to reduce the rate of urinary excretion of desmosine (DES), a specific marker of elastin degradation, in eight men and four women with emphysema due to severe, congenital deficiency of AAT (range 17-69 mg/dl). Nine were former cigarette smokers, two were current smokers, and one reported never smoking; their mean age was 54 (SD 12) yr and their mean FEV(1) was 41 (18%) of predicted. Urinary DES was measured by isotope dilution and HPLC. Prior to the start of AAT supplementation, mean DES excretion was 13.0 (5.0) microg/g creatinine, 73% higher than in healthy nonsmokers. During 8 wk of supplementation therapy, mean urinary DES excretion was 13.0 (5.9) microg/g creatinine, unchanged from the baseline period (p = 0.85 by repeated measures ANOVA). We conclude that baseline levels of elastin degradation in emphysematous patients with severe AAT deficiency were abnormally high and that 8 wk of AAT supplementation therapy did not appreciably reduce the rate of elastin degradation. These findings raise the possibilities that protective levels of AAT in the lungs are insufficient or that elastin degradation in the lungs of these subjects is not dependent upon neutrophil elastase at this time.
D J Gottlieb; M Luisetti; P J Stone; L Allegra; J M Cantey-Kiser; C Grassi; G L Snider
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.    
Journal Detail:
Title:  American journal of respiratory and critical care medicine     Volume:  162     ISSN:  1073-449X     ISO Abbreviation:  Am. J. Respir. Crit. Care Med.     Publication Date:  2000 Dec 
Date Detail:
Created Date:  2001-01-24     Completed Date:  2001-01-26     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9421642     Medline TA:  Am J Respir Crit Care Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  2069-72     Citation Subset:  AIM; IM    
Department of Medicine, Boston University School of Medicine, MA 02118-2394, USA.
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MeSH Terms
Acute Disease
Analysis of Variance
Cotinine / urine
Desmosine / urine
Elastin / metabolism*
Infusions, Intravenous
Lung / metabolism
Pulmonary Emphysema / drug therapy,  etiology,  metabolism
Time Factors
alpha 1-Antitrypsin / administration & dosage*,  analysis
alpha 1-Antitrypsin Deficiency / complications,  congenital,  drug therapy*,  metabolism
Reg. No./Substance:
0/alpha 1-Antitrypsin; 11003-57-9/Desmosine; 486-56-6/Cotinine; 9007-58-3/Elastin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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