{
  "id": 18037,
  "label": "primary systemic amyloidosis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0017816",
  "properties": {
    "xrefs": [
      "GARD:0017431",
      "MEDGEN:129028",
      "NANDO:1200209",
      "NCIT:C8299",
      "Orphanet:314701",
      "SCTID:89449005",
      "UMLS:C0281479"
    ],
    "synonyms": [
      "systemic AL amyloidosis",
      "systemic Immunoglobulin Light chain amyloidosis",
      "systemic amyloidosis"
    ],
    "categories": [
      {
        "ref": "MONDO:0002081",
        "name": "musculoskeletal system disorder"
      },
      {
        "ref": "MONDO:0004995",
        "name": "cardiovascular disorder"
      },
      {
        "ref": "MONDO:0005071",
        "name": "nervous system disorder"
      }
    ],
    "definition": "Primary systemic amyloidosis (PSA) is a form of AL amyloidosis caused by the aggregation and deposition of insoluble amyloid fibrils derived from misfolded monoclonal immunoglobulin light chains usually produced by a plasma cell tumor and characterized by multiple organ involvement."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 19265,
      "label": "AL amyloidosis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6799,
        7996,
        16875,
        16883,
        18960
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080933",
          "GARD:0005797",
          "MEDGEN:75674",
          "MESH:C531616",
          "MedDRA:10036673",
          "NANDO:1200211",
          "NCIT:C158963",
          "Orphanet:85443",
          "UMLS:C0268381",
          "icd11.foundation:1061366491",
          "icd11.foundation:113043090"
        ],
        "synonyms": [
          "Light-chain amyloidosis",
          "primary amyloidosis",
          "Light chain amyloidosis",
          "amyloidosis AL",
          "amyloidosis primary systemic",
          "primary AL amyloidosis",
          "primary systemic AL amyloidosis",
          "primary systemic amyloidosis",
          "systemic AL amyloidsis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002081",
            "name": "musculoskeletal system disorder"
          },
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          },
          {
            "ref": "MONDO:0005071",
            "name": "nervous system disorder"
          }
        ],
        "definition": "AL Amyloidosis is a plasma cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ."
      },
      "child_count": 10,
      "reference_id": "MONDO:0019438"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 19265,
      "label": "AL amyloidosis"
    }
  ]
}