{
  "id": 3655,
  "label": "pulmonary alveolar proteinosis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0001437",
  "properties": {
    "xrefs": [
      "DOID:12120",
      "ICD9:516.0",
      "MEDGEN:1763046",
      "MESH:D011649",
      "NANDO:1200746",
      "NCIT:C85037",
      "SCTID:10501004",
      "UMLS:C5400698",
      "icd11.foundation:1869739196"
    ],
    "synonyms": [
      "PAP",
      "pulmonary alveolar proteinosis"
    ],
    "categories": [
      {
        "ref": "MONDO:0005087",
        "name": "respiratory system disorder"
      }
    ],
    "definition": "A rare lung disorder characterized by the filling of the pulmonary alveoli with proteinaceous material which stains positive with periodic acid-Schiff stain. It may be idiopathic or secondary due to hematologic malignancies or the inhalation of mineral dusts. Signs and symptoms include dyspnea, cough and low grade fever."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 3,
  "parents": [
    {
      "id": 6971,
      "label": "lung disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        2809
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:850",
          "EFO:0003818",
          "ICD9:518.89",
          "MEDGEN:7399",
          "MESH:D008171",
          "NCIT:C3198",
          "SCTID:19829001",
          "UMLS:C0024115"
        ],
        "synonyms": [
          "disease of lung",
          "disease or disorder of lung",
          "disorder of lung",
          "lung disease",
          "lung disease or disorder",
          "lung disorder",
          "lung disorders",
          "pulmonary disease",
          "pulmonary diseases",
          "pulmonary disorder",
          "pulmonary disorders"
        ],
        "categories": [
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ],
        "definition": "A disease involving the lung."
      },
      "child_count": 33,
      "reference_id": "MONDO:0005275"
    }
  ],
  "children": [
    {
      "id": 13626,
      "label": "autoimmune pulmonary alveolar proteinosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3655,
        8586
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0007499",
          "MEDGEN:410079",
          "MESH:C567049",
          "NANDO:1200747",
          "NANDO:1200748",
          "NORD:1633",
          "OMIM:610910",
          "Orphanet:747",
          "SCTID:707443007",
          "UMLS:C1970472",
          "icd11.foundation:676409940"
        ],
        "synonyms": [
          "APAP",
          "Pulmonary Alveolar Proteinosis",
          "autoimmune PAP",
          "iPAP",
          "idiopathic PAP",
          "idiopathic pulmonary alveolar proteinosis",
          "PAP",
          "PAP acquired",
          "Pap, acquired",
          "acquired pulmonary alveolar proteinosis",
          "pulmonary alveolar lipoproteinosis acquired",
          "pulmonary alveolar lipoproteinosis, acquired",
          "pulmonary alveolar proteinosis acquired",
          "pulmonary alveolar proteinosis autoimmune",
          "pulmonary alveolar proteinosis, acquired",
          "pulmonary alveolar proteinosis, autoimmune"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ],
        "definition": "Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of a lipoproteinaceous substance in the distal air spaces which positively stains with periodic acid-Schiff (PAS)."
      },
      "child_count": 0,
      "reference_id": "MONDO:0012579"
    },
    {
      "id": 13627,
      "label": "hereditary pulmonary alveolar proteinosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3655,
        5714
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0004582",
          "MEDGEN:777976",
          "MESH:C535832",
          "NANDO:1200746",
          "NANDO:1200750",
          "NANDO:2200200",
          "OMIMPS:265120",
          "Orphanet:264675",
          "SCTID:707442002",
          "UMLS:C3711368"
        ],
        "synonyms": [
          "congenital PAP",
          "congenital pulmonary alveolar proteinosis",
          "hereditary pulmonary alveolar proteinosis",
          "inborn error of pulmonary surfactant metabolism",
          "inborn error of surfactant metabolism",
          "pulmonary alveolar proteinosis, congenital",
          "sufactant metabolism dysfunction, pulmonary"
        ],
        "categories": [
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ],
        "definition": "Congenital pulmonary alveolar proteinosis is a very rare primary interstitial lung disease due to pulmonary surfactant accumulation within the alveolar macrophages and alveoli, characterized by a variable clinical course ranging from an asymptomatic clinical presentation and spontaneous remission, to symptoms such as dyspnea and cough, or to severe respiratory failure."
      },
      "child_count": 16,
      "reference_id": "MONDO:0012580"
    },
    {
      "id": 18521,
      "label": "secondary pulmonary alveolar proteinosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3655
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021744",
          "MEDGEN:845105",
          "NANDO:1200749",
          "Orphanet:420259",
          "SCTID:707510005",
          "UMLS:C3873302",
          "icd11.foundation:1480338606"
        ],
        "synonyms": [
          "SPAP",
          "secondary PAP"
        ],
        "categories": [
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ],
        "definition": "A form of pulmonary alveolar proteinosis that arises in association with hematological disorders, medications, certain infections, acute silicosis, and immunodeficiency."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018483"
    }
  ],
  "roots": [
    {
      "id": 6971,
      "label": "lung disorder"
    }
  ]
}