| Short-term supplementation therapy does not affect elastin degradation in severe alpha(1)-antitrypsin deficiency. The American-Italian AATD Study Group. | |
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MedLine Citation:
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PMID: 11112116 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S. |
Journal Detail:
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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:
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Created Date: 2001-01-24 Completed Date: 2001-01-26 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 9421642 Medline TA: Am J Respir Crit Care Med Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 2069-72 Citation Subset: AIM; IM |
Affiliation:
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Department of Medicine, Boston University School of Medicine, MA 02118-2394, USA. dgottleib@lung.bumc.bu.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acute Disease Adult Analysis of Variance Cotinine / urine Desmosine / urine Elastin / metabolism* Female Humans Infusions, Intravenous Lung / metabolism Male Pulmonary Emphysema / drug therapy, etiology, metabolism Time Factors alpha 1-Antitrypsin / administration & dosage*, analysis alpha 1-Antitrypsin Deficiency / complications, congenital, drug therapy*, metabolism |
| Chemical | |
Reg. No./Substance:
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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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