Surfactant protein deficiency in familial interstitial lung disease
Identifieur interne : 002307 ( Main/Exploration ); précédent : 002306; suivant : 002308Surfactant protein deficiency in familial interstitial lung disease
Auteurs : Raouf S. Amin [États-Unis] ; Susan E. Wert [États-Unis] ; Robert P. Baughman [États-Unis] ; Joseph F. Tomashefski Jr [États-Unis] ; Lawrence M. Nogee [États-Unis] ; Alan S. Brody [États-Unis] ; William M. Hull [États-Unis] ; Jeffrey A. Whitsett [États-Unis]Source :
- The Journal of Pediatrics [ 0022-3476 ] ; 2001.
English descriptors
- KwdEn :
- Teeft :
- Adult patients, Adult subjects, Alveolar, Alveolar epithelial cell surface, Alveolar epithelial cells, Alveolar epithelium, Alveolar lumen, Alveolar proteinosis, Alveolar septa, Alveolar space, Alveolar type, Amin, Apparent absence, Arrowhead, Balf, Balf samples, Biopsy, Bronchoalveolar lavage, Brosis, Cholesterol granulomas, Coding sequences, Control samples, Control subjects, Direct sequencing, Epithelial, Familial form, Family members, Flexible bronchoscopy, Foamy macrophages, Hereditary form, Heteroduplex analysis, Histologic abnormalities, Histopathologic, Human albumin, Human surfactant, Hyperplastic alveolar type, Idiopathic, Immunohistochemical analysis, Immunostaining, Initial evaluation, Interstitial, Interstitial lung disease, Interstitial pneumonia, Iron stain, Lavage, Luminal material, Luminal surface, Lung biopsy, Lung biopsy specimen, Lung biopsy specimens, Lung disease, Lung diseases, Lung tissues, Macrophage, Mechanical ventilation, Mutation, Pediatric patients, Pediatrics, Pediatrics july, Pediatrics volume, Periodic stain, Plasma cells, Pneumocytes, Polymerase chain reaction products, Proteinaceous, Proteinaceous material, Pulmonary alveolar proteinosis, Pulmonary malignancy, Pulmonary medicine, Pulmonary surfactant protein, Radiographic studies, Respir crit care, Respiratory distress syndrome, Sequence analysis, Surfactant, Surfactant protein, Systemic steroids, Total protein, Tumor necrosis, Variable degrees, Wash buffer, Western blot, Western blot analysis, Whitsett.
Abstract
Abstract: Objective: To determine the contribution of surfactant protein abnormalities to the development of chronic lung injury in a familial form of interstitial lung disease. Study design: An 11-year-old girl, her sister, and their mother who were diagnosed with chronic interstitial lung disease underwent laboratory investigation of surfactant protein expression in bronchoalveolar lavage fluid and lung biopsy specimens. Nineteen patients with idiopathic pulmonary fibrosis and 9 patients who were investigated for pulmonary malignancy but who did not have interstitial lung disease served as control subjects. Results: The 3 family members were found to have absent surfactant protein C (SP-C) and decreased levels of SP-A and SP-B in bronchoalveolar lavage fluid (BALF). Immunostaining for pulmonary surfactant proteins in lung biopsy specimens obtained from both children demonstrated a marked decrease of pro-SP-C in the alveolar epithelial cells but strong staining for pro-SP-B, SP-B, SP-A, and SP-D. No deviations from published surfactant protein B or C coding sequences were identified by DNA sequence analysis. All control subjects had a detectable level of SP-C in the BALF. Conclusion: The apparent absence of SP-C and a decrease in the levels of SP-A and SP-B are associated with familial interstitial lung disease. (J Pediatr 2001;139:85-92)
Url:
DOI: 10.1067/mpd.2001.114545
Affiliations:
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Nogee</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>Children’s Hospital Medical Center, Department of Pediatrics, Cincinnati, Ohio; University of Cincinnati College of Medicine, Department of Pulmonary Medicine, Cincinnati, Ohio; MetroHealth Medical Center, Department of Pathology, Cleveland, Ohio; and Johns Hopkins School of Medicine, Baltimore</wicri:cityArea></affiliation></author><author><name sortKey="Brody, Alan S" sort="Brody, Alan S" uniqKey="Brody A" first="Alan S." last="Brody">Alan S. 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Whitsett</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><placeName><region type="state">Maryland</region></placeName><wicri:cityArea>Children’s Hospital Medical Center, Department of Pediatrics, Cincinnati, Ohio; University of Cincinnati College of Medicine, Department of Pulmonary Medicine, Cincinnati, Ohio; MetroHealth Medical Center, Department of Pathology, Cleveland, Ohio; and Johns Hopkins School of Medicine, Baltimore</wicri:cityArea></affiliation></author></analytic><monogr></monogr><series><title level="j">The Journal of Pediatrics</title><title level="j" type="abbrev">YMPD</title><idno type="ISSN">0022-3476</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="2001">2001</date><biblScope unit="volume">139</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="85">85</biblScope><biblScope unit="page" to="92">92</biblScope></imprint><idno type="ISSN">0022-3476</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0022-3476</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>BALF</term><term>ELISA</term><term>GER</term><term>HRCT</term><term>ILD</term><term>RDS</term><term>SP</term></keywords><keywords scheme="Teeft" xml:lang="en"><term>Adult patients</term><term>Adult subjects</term><term>Alveolar</term><term>Alveolar epithelial cell surface</term><term>Alveolar epithelial cells</term><term>Alveolar epithelium</term><term>Alveolar lumen</term><term>Alveolar proteinosis</term><term>Alveolar septa</term><term>Alveolar space</term><term>Alveolar type</term><term>Amin</term><term>Apparent absence</term><term>Arrowhead</term><term>Balf</term><term>Balf samples</term><term>Biopsy</term><term>Bronchoalveolar lavage</term><term>Brosis</term><term>Cholesterol granulomas</term><term>Coding sequences</term><term>Control samples</term><term>Control subjects</term><term>Direct sequencing</term><term>Epithelial</term><term>Familial form</term><term>Family members</term><term>Flexible bronchoscopy</term><term>Foamy macrophages</term><term>Hereditary form</term><term>Heteroduplex analysis</term><term>Histologic abnormalities</term><term>Histopathologic</term><term>Human albumin</term><term>Human surfactant</term><term>Hyperplastic alveolar type</term><term>Idiopathic</term><term>Immunohistochemical analysis</term><term>Immunostaining</term><term>Initial evaluation</term><term>Interstitial</term><term>Interstitial lung disease</term><term>Interstitial pneumonia</term><term>Iron stain</term><term>Lavage</term><term>Luminal material</term><term>Luminal surface</term><term>Lung biopsy</term><term>Lung biopsy specimen</term><term>Lung biopsy specimens</term><term>Lung disease</term><term>Lung diseases</term><term>Lung tissues</term><term>Macrophage</term><term>Mechanical ventilation</term><term>Mutation</term><term>Pediatric patients</term><term>Pediatrics</term><term>Pediatrics july</term><term>Pediatrics volume</term><term>Periodic stain</term><term>Plasma cells</term><term>Pneumocytes</term><term>Polymerase chain reaction products</term><term>Proteinaceous</term><term>Proteinaceous material</term><term>Pulmonary alveolar proteinosis</term><term>Pulmonary malignancy</term><term>Pulmonary medicine</term><term>Pulmonary surfactant protein</term><term>Radiographic studies</term><term>Respir crit care</term><term>Respiratory distress syndrome</term><term>Sequence analysis</term><term>Surfactant</term><term>Surfactant protein</term><term>Systemic steroids</term><term>Total protein</term><term>Tumor necrosis</term><term>Variable degrees</term><term>Wash buffer</term><term>Western blot</term><term>Western blot analysis</term><term>Whitsett</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: Objective: To determine the contribution of surfactant protein abnormalities to the development of chronic lung injury in a familial form of interstitial lung disease. Study design: An 11-year-old girl, her sister, and their mother who were diagnosed with chronic interstitial lung disease underwent laboratory investigation of surfactant protein expression in bronchoalveolar lavage fluid and lung biopsy specimens. Nineteen patients with idiopathic pulmonary fibrosis and 9 patients who were investigated for pulmonary malignancy but who did not have interstitial lung disease served as control subjects. Results: The 3 family members were found to have absent surfactant protein C (SP-C) and decreased levels of SP-A and SP-B in bronchoalveolar lavage fluid (BALF). Immunostaining for pulmonary surfactant proteins in lung biopsy specimens obtained from both children demonstrated a marked decrease of pro-SP-C in the alveolar epithelial cells but strong staining for pro-SP-B, SP-B, SP-A, and SP-D. No deviations from published surfactant protein B or C coding sequences were identified by DNA sequence analysis. All control subjects had a detectable level of SP-C in the BALF. Conclusion: The apparent absence of SP-C and a decrease in the levels of SP-A and SP-B are associated with familial interstitial lung disease. (J Pediatr 2001;139:85-92)</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Maryland</li></region></list><tree><country name="États-Unis"><region name="Maryland"><name sortKey="Amin, Raouf S" sort="Amin, Raouf S" uniqKey="Amin R" first="Raouf S." last="Amin">Raouf S. Amin</name></region><name sortKey="Baughman, Robert P" sort="Baughman, Robert P" uniqKey="Baughman R" first="Robert P." last="Baughman">Robert P. Baughman</name><name sortKey="Brody, Alan S" sort="Brody, Alan S" uniqKey="Brody A" first="Alan S." last="Brody">Alan S. Brody</name><name sortKey="Hull, William M" sort="Hull, William M" uniqKey="Hull W" first="William M." last="Hull">William M. Hull</name><name sortKey="Nogee, Lawrence M" sort="Nogee, Lawrence M" uniqKey="Nogee L" first="Lawrence M." last="Nogee">Lawrence M. Nogee</name><name sortKey="Tomashefski Jr, Joseph F" sort="Tomashefski Jr, Joseph F" uniqKey="Tomashefski Jr J" first="Joseph F." last="Tomashefski Jr">Joseph F. Tomashefski Jr</name><name sortKey="Wert, Susan E" sort="Wert, Susan E" uniqKey="Wert S" first="Susan E." last="Wert">Susan E. Wert</name><name sortKey="Whitsett, Jeffrey A" sort="Whitsett, Jeffrey A" uniqKey="Whitsett J" first="Jeffrey A." last="Whitsett">Jeffrey A. Whitsett</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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