{
  "id": 16797,
  "label": "pulmonary fibrosis-hepatic hyperplasia-bone marrow hypoplasia syndrome",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0016214",
  "properties": {
    "xrefs": [
      "GARD:0020449",
      "MEDGEN:1385709",
      "Orphanet:210136",
      "UMLS:C4510085"
    ],
    "categories": [
      {
        "ref": "MONDO:0005087",
        "name": "respiratory system disorder"
      }
    ],
    "definition": "Pulmonary fibrosis - hepatic hyperplasia - bone marrow hypoplasia, also named Btrimorphic syndromeB (i.e. three (inherited) morbidities, pulmonary, hepatic and cytopenia), is a rare disease reported in 4 cases to date, manifesting with idiopathic pulmonary fibrosis, hepatic nodular regenerative hyperplasia leading to portal hypertension and thrombocytopenia due to bone marrow hypoplasia. The condition was associated with 100% mortality."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 16615,
      "label": "interstitial lung disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6971
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3082",
          "EFO:0004244",
          "ICD10CM:J80-J84",
          "MEDGEN:1788738",
          "MESH:D017563",
          "MedDRA:10022611",
          "NCIT:C164315",
          "Orphanet:182095",
          "SCTID:233703007",
          "UMLS:C5441745"
        ],
        "synonyms": [
          "ILD",
          "interstitial lung disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ],
        "definition": "A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of pulmonary alveoli that extends to the interstitium and beyond leading to diffuse pulmonary fibrosis. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features."
      },
      "child_count": 14,
      "reference_id": "MONDO:0015925"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 16615,
      "label": "interstitial lung disease"
    }
  ]
}