Autosomal recessive hereditary disorder	Chronic lung disease	Connective tissue hereditary disorder	Hereditary disorder by system	Interstitial lung disease	Pulmonary alveolar proteinosis
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Severe early-onset pulmonary alveolar proteinosis due to methionyl-transfer ribonucleic acid synthetase 1 deficiency (disorder)
	Severe early-onset pulmonary alveolar proteinosis due to MARS deficiency
	Severe early-onset pulmonary alveolar proteinosis due to methionyl-transfer ribonucleic acid synthetase 1 deficiency Hereditary pulmonary alveolar proteinosis with hepatic involvement Interstitial lung and liver disease Pulmonary alveolar proteinosis Reunion island type
	A rare genetic interstitial lung disease with characteristics of 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 has been described in some cases including hepatomegaly, steatosis, fibrosis or cirrhosis.

Id	1228876007
Status	Primitive

Clinical course

Finding site

Structure of interstitial tissue of lung

Associated morphology	Protein deposition
Finding site	Pulmonary alveolar structure

ICD-10 complex map reference set

Target	J84.0
Rule	TRUE
Advice	ALWAYS J84.0 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified