Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report
<p>Abstract</p> <p>Background</p> <p>Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by accumulation of lipoproteinaceous material within alveoli, occurring in three clinically distinct forms: congenital, acquired and secondary. Among the latter, ly...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2007-03-01
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| Series: | Orphanet Journal of Rare Diseases |
| Online Access: | http://www.OJRD.com/content/2/1/14 |
| _version_ | 1811317818265698304 |
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| author | Baritussio Aldo Bolongaro Antonia Adami Andrea Stella Giulia M Rodi Giuseppe Ceruti Michele Pozzi Ernesto Luisetti Maurizio |
| author_facet | Baritussio Aldo Bolongaro Antonia Adami Andrea Stella Giulia M Rodi Giuseppe Ceruti Michele Pozzi Ernesto Luisetti Maurizio |
| author_sort | Baritussio Aldo |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Background</p> <p>Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by accumulation of lipoproteinaceous material within alveoli, occurring in three clinically distinct forms: congenital, acquired and secondary. Among the latter, lysinuric protein intolerance (LPI) is a rare genetic disorder caused by defective transport of cationic amino acids. Whole Lung Lavage (WLL) is currently the gold standard therapy for severe cases of PAP.</p> <p>Case presentation</p> <p>We describe the case of an Italian boy affected by LPI who, by the age of 10, developed digital clubbing and, by the age of 16, a mild restrictive functional impairment associated with a high-resolution computed tomography (HRCT) pattern consistent with pulmonary alveolar proteinosis. After careful assessment, he underwent WLL.</p> <p>Conclusion</p> <p>Two years after WLL, the patient has no clinical, radiological or functional evidence of pulmonary disease recurrence, thus suggesting that WLL may be helpful in the treatment of PAP secondary to LPI.</p> |
| first_indexed | 2024-04-13T12:14:59Z |
| format | Article |
| id | doaj.art-5f233d3e7a194c1c9a213b51f1f34ca3 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-13T12:14:59Z |
| publishDate | 2007-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-5f233d3e7a194c1c9a213b51f1f34ca32022-12-22T02:47:23ZengBMCOrphanet Journal of Rare Diseases1750-11722007-03-01211410.1186/1750-1172-2-14Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case reportBaritussio AldoBolongaro AntoniaAdami AndreaStella Giulia MRodi GiuseppeCeruti MichelePozzi ErnestoLuisetti Maurizio<p>Abstract</p> <p>Background</p> <p>Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by accumulation of lipoproteinaceous material within alveoli, occurring in three clinically distinct forms: congenital, acquired and secondary. Among the latter, lysinuric protein intolerance (LPI) is a rare genetic disorder caused by defective transport of cationic amino acids. Whole Lung Lavage (WLL) is currently the gold standard therapy for severe cases of PAP.</p> <p>Case presentation</p> <p>We describe the case of an Italian boy affected by LPI who, by the age of 10, developed digital clubbing and, by the age of 16, a mild restrictive functional impairment associated with a high-resolution computed tomography (HRCT) pattern consistent with pulmonary alveolar proteinosis. After careful assessment, he underwent WLL.</p> <p>Conclusion</p> <p>Two years after WLL, the patient has no clinical, radiological or functional evidence of pulmonary disease recurrence, thus suggesting that WLL may be helpful in the treatment of PAP secondary to LPI.</p>http://www.OJRD.com/content/2/1/14 |
| spellingShingle | Baritussio Aldo Bolongaro Antonia Adami Andrea Stella Giulia M Rodi Giuseppe Ceruti Michele Pozzi Ernesto Luisetti Maurizio Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report Orphanet Journal of Rare Diseases |
| title | Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report |
| title_full | Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report |
| title_fullStr | Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report |
| title_full_unstemmed | Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report |
| title_short | Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report |
| title_sort | successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance a case report |
| url | http://www.OJRD.com/content/2/1/14 |
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