| Quantity and structure of surfactant proteins vary among patients with alveolar proteinosis. | |
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MedLine Citation:
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PMID: 9476887 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Alveolar proteinosis (AP) is an idiopathic condition characterized by excess alveolar surfactant. Although the surfactant proteins (SP) are known to be aberrant, little is known of their variation between patients or their abundance relative to the lipids. We have examined surfactant composition in lavage fluid from 16 normal subjects and 13 patients with AP, one of whom was lavaged on 11 occasions over approximately 13 mo. In this patient we have examined composition on each occasion and in each sequential lavage aliquot. Composition was constant between right and left lung, but it differed markedly between patients. The cholesterol/disaturated phospholipid ratios (CHOL/DSP) were invariably elevated, on average by approximately 7-fold, whereas the SP-A/DSP and SP-B/DSP ratios were generally elevated, in some cases by as much as approximately 40- and approximately 100-fold, respectively. Although AP lavage generally contained more non-thiol-dependent SP-A aggregates and low Mr isoforms, the two-dimensional immunochemical staining patterns varied between patients and right and left lung. In the patient lavaged on multiple occasions, the SP-A/DSP and SP-B/DSP ratios progressively decreased as the patient's condition resolved. Because the SP-B/SP-A ratio was normal in all cases, we suggest that structural changes to the proteins occurred secondarily and that caution must be used in comparing functional data derived using SP-A obtained from patients with AP. |
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Authors:
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I R Doyle; K G Davidson; H A Barr; T E Nicholas; K Payne; J Pfitzner |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: American journal of respiratory and critical care medicine Volume: 157 ISSN: 1073-449X ISO Abbreviation: Am. J. Respir. Crit. Care Med. Publication Date: 1998 Feb |
Date Detail:
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Created Date: 1998-03-12 Completed Date: 1998-03-12 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: 658-64 Citation Subset: AIM; IM |
Affiliation:
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Department of Human Physiology School of Medicine, Flinders University of South Australia, Australia. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Aged Bronchoalveolar Lavage Fluid / chemistry Female Humans Immunochemistry / methods Male Middle Aged Proteolipids / analysis Pulmonary Alveolar Proteinosis / metabolism* Pulmonary Surfactant-Associated Protein A Pulmonary Surfactant-Associated Proteins Pulmonary Surfactants / analysis, chemistry*, metabolism* |
| Chemical | |
Reg. No./Substance:
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0/Proteolipids; 0/Pulmonary Surfactant-Associated Protein A; 0/Pulmonary Surfactant-Associated Proteins; 0/Pulmonary Surfactants |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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