Go to the content. | Move to the navigation | Go to the site search | Go to the menu | Contacts | Accessibility

| Create Account

Ceruti, Michele and Rodi, Giuseppe and Stella, Giulia M and Adami, Andrea and Bolongaro, Andrea and Baritussio, Aldo and Pozzi, Ernesto and Luisetti, Maurizio (2007) Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report. [Online journal papers]

Full text disponibile come:

Documento PDF

Per gentile concessione di: http://www.OJRD.com/content/2/1/14

Abstract (english)


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.

Case presentation

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.


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.

Statistiche Download - Aggiungi a RefWorks
EPrint type:Online journal papers
Anno di Pubblicazione:2007
Key Words:Pulmonary alveolar proteinosis, cationic amino acids
Settori scientifico-disciplinari MIUR:Area 06 - Scienze mediche > MED/09 Medicina interna
Struttura di riferimento:Dipartimenti > pre 2012 - Dipartimento di Scienze Mediche e Chirurgiche
Codice ID:1217
Depositato il:09 Dec 2008
Simple Metadata
Full Metadata
EndNote Format

Download statistics

Solo per lo Staff dell Archivio: Modifica questo record