{
  "id": 23764,
  "label": "mast cell activation syndrome",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0100004",
  "properties": {
    "xrefs": [
      "GARD:0012981",
      "MEDGEN:1698540",
      "MESH:D000090267",
      "UMLS:C5200989"
    ],
    "synonyms": [
      "MACS",
      "disorder of mast cell activation",
      "mast cell activation disease"
    ],
    "categories": [
      {
        "ref": "MONDO:0005046",
        "name": "immune system disorder"
      },
      {
        "ref": "MONDO:0005570",
        "name": "hematologic disorder"
      }
    ],
    "definition": "A clinically defined disease states with a largely unknown morphological background. Acute mast cell activation (MCA) is commonly seen in allergic reactions and often leads to the clinical signs and symptoms of anaphylaxis. Severe or even life‐threatening MCA may occur when the burden of mast cells is high and/or these cells are in an hyperactivated state. Mastocytosis may be associated with mast cell activation syndrome (MCAS)."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 3,
  "parents": [
    {
      "id": 6569,
      "label": "leukocyte disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6778,
        7217
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9500",
          "ICD9:288",
          "ICD9:288.9",
          "MEDGEN:7325",
          "MESH:D007960",
          "SCTID:54097007",
          "UMLS:C0023510"
        ],
        "synonyms": [
          "disease of leukocyte",
          "disease or disorder of leukocyte",
          "disorder of leukocyte",
          "disorder, leukocyte",
          "disorders, leukocyte",
          "leukocyte disease",
          "leukocyte disease or disorder",
          "leukocyte disorder",
          "white blood cell disorder"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "A disease involving leukocytes."
      },
      "child_count": 46,
      "reference_id": "MONDO:0004805"
    }
  ],
  "children": [
    {
      "id": 22759,
      "label": "monoclonal mast cell activation syndrome",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        4370,
        23764
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0012980",
          "ICD10CM:D89.41",
          "MEDGEN:1672509",
          "NCIT:C181652",
          "Orphanet:529468",
          "UMLS:C4267893"
        ],
        "synonyms": [
          "primary MCAS",
          "primary mast cell activation syndrome"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Mast cell activation syndrome where KIT-mutated and clonal mast cells are detected."
      },
      "child_count": 0,
      "reference_id": "MONDO:0033954"
    },
    {
      "id": 23765,
      "label": "secondary mast cell activation syndrome",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        23764
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0026014",
          "ICD10CM:D89.43",
          "MEDGEN:1843472",
          "UMLS:C4267895"
        ],
        "synonyms": [
          "secondary MACS"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Mast cell activation syndrome where an underlying inflammatory disease is present, often in the form of an IgE-dependent allergy, but there are no KIT-mutated mast cells."
      },
      "child_count": 0,
      "reference_id": "MONDO:0100006"
    },
    {
      "id": 23804,
      "label": "idiopathic mast cell activation syndrome",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        23764,
        24405
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0026026",
          "ICD10CM:D89.42",
          "MEDGEN:1786072",
          "UMLS:C4267894"
        ],
        "synonyms": [
          "idiopathic MACS"
        ],
        "categories": [
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005570",
            "name": "hematologic disorder"
          }
        ],
        "definition": "Mast cell activation syndrome where neither an allergy or other underlying disease is association, nor KIT-mutated mast cells are detectable."
      },
      "child_count": 0,
      "reference_id": "MONDO:0100051"
    }
  ],
  "roots": [
    {
      "id": 6569,
      "label": "leukocyte disorder"
    }
  ]
}