{
  "id": 18187,
  "label": "complement 3 glomerulopathy",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018013",
  "properties": {
    "xrefs": [
      "GARD:0017507",
      "MEDGEN:1672497",
      "Orphanet:329918",
      "UMLS:C4087273"
    ],
    "synonyms": [
      "C3 glomerulopathy",
      "C3G",
      "non-Ig-mediated MPGN",
      "non-Ig-mediated membranoproliferative glomerulonephritis",
      "non-immunoglobulin-mediated MPGN",
      "non-immunoglobulin-mediated membranoproliferative glomerulonephritis"
    ],
    "categories": [
      {
        "ref": "MONDO:0002118",
        "name": "urinary system disorder"
      }
    ],
    "definition": "A rare primary membranoproliferative glomerulonephritis characterized by complement dysregulation occurring in the fluid phase and in the glomerular microenvironment, which results in prominent complement C3 deposition in kidney biopsy samples."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 3,
  "parents": [
    {
      "id": 18832,
      "label": "primary membranoproliferative glomerulonephritis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        4542
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0011982",
          "ICD9:583.2",
          "MedDRA:10018370",
          "NANDO:1200725",
          "NANDO:2200123",
          "Orphanet:54370"
        ],
        "synonyms": [
          "MPGN",
          "Mesangiocapillary glomerulonephritis",
          "membranoproliferative glomerulonephritis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "A rare glomerular disease characterized by a pattern of glomerular injury on kidney biopsy with characteristic light microscopic changes: mesangial hypercellularity, endocapillary proliferation, and thickening of the glomerular basement membrane (GBM). On the basis of immunofluorescence (IF) the disorder is divided into C3 glomerulopathy (C3G) or immunoglobulin-mediated membranoproliferative glomerulonephritis. Through electron microscopy C3G is further divided into Dense deposit disease, with highly electrondense deposits in the glomerular basement membrane, and C3 glomerulonephritis, with mesangial, intramembranous, subendothelial and subepithelial deposits. Secondary causes (autoimmune, infectious, malignancies) are excluded."
      },
      "child_count": 3,
      "reference_id": "MONDO:0018904"
    }
  ],
  "children": [
    {
      "id": 13404,
      "label": "complement factor H deficiency",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7021,
        18187
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0018551",
          "ICD9:279.8",
          "MEDGEN:96024",
          "MESH:C562875",
          "NANDO:2200791",
          "OMIM:609814",
          "SCTID:234622003",
          "UMLS:C0398777"
        ],
        "synonyms": [
          "complement factor H deficiency",
          "CFHD",
          "Cfh deficiency",
          "factor H deficiency"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ]
      },
      "child_count": 2,
      "reference_id": "MONDO:0012350"
    },
    {
      "id": 14904,
      "label": "C3 glomerulonephritis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        7021,
        18187
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0016487",
          "MEDGEN:884569",
          "NCIT:C123043",
          "OMIM:614809",
          "Orphanet:329931",
          "UMLS:C4055342"
        ],
        "synonyms": [
          "complement-mediated membranoproliferative glomerulonephritis",
          "nephropathy due to CFHR5 deficiency",
          "CFHR5 deficiency"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "Glomerulonephritis characterized by C3 accumulation with little or absent deposition of immunoglobulin, in the absence of ultrastructural electron-dense transformation seen in dense deposit disease."
      },
      "child_count": 0,
      "reference_id": "MONDO:0013892"
    },
    {
      "id": 19494,
      "label": "dense deposit disease",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18187
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0008555",
          "MEDGEN:124345",
          "NANDO:1200739",
          "NCIT:C123039",
          "Orphanet:93571",
          "SCTID:722760002",
          "UMLS:C0268743"
        ],
        "synonyms": [
          "Mesangiocapillary glomerulonephritis type 2",
          "membranoproliferative glomerulonephritis type 2",
          "MPGN 2",
          "glomerulonephritis membranoproliferative type 2",
          "membranoproliferative glomerulonephritis type II"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "A histological subtype of C3 glomerulopathy characterized by C3 deposition in renal tissue in the absence or near-absence of immunoglobulin deposits, in a patient with the classic clinical features of glomerulonephritis and electron microscopic findings of highly electron-dense intra-membranous, osmiophilic deposits."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019736"
    }
  ],
  "roots": [
    {
      "id": 18832,
      "label": "primary membranoproliferative glomerulonephritis"
    }
  ]
}