Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare, genetic interstitial lung disease characterized by accumulation of lipoproteins in the pulmonary alveoli leading to restrictive lung disease and respiratory failure. Patients present with dyspnea, tachypnea, cough, failure to thrive, and digital clubbing. Liver disease have been described in some cases including hepatomegaly, steatosis, fibrosis or cirrhosis. Disease data Classification Disease Synonyms Hereditary pulmonary alveolar proteinosis with hepatic involvement PAP, typ wyspy Reunion Płucna proteinoza pęcherzykowa, typ wyspy Reunion Śródmiąższowa choroba płuc i wątroby Interstitial lung and liver disease PAP, Reunion island type Pulmonary alveolar proteinosis, Reunion island type ORPHA code 440427 OMIM code 615486 ICD10 code J84.0 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl