{
  "id": 18832,
  "label": "primary membranoproliferative glomerulonephritis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018904",
  "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."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 3,
  "parents": [
    {
      "id": 4542,
      "label": "glomerulonephritis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        3410,
        19482
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:2921",
          "GARD:0006516",
          "HP:0000099",
          "ICD9:583.9",
          "MEDGEN:6616",
          "MESH:D005921",
          "NCIT:C26784",
          "SCTID:36171008",
          "UMLS:C0017658"
        ],
        "synonyms": [
          "glomerular nephritis",
          "glomerulonephritis",
          "glomerulonephritis (disease)",
          "nephritis of renal glomerulus",
          "renal glomerulus nephritis",
          "bright's disease"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "A renal disorder characterized by damage in the glomeruli. It may be acute or chronic, focal or diffuse, and it may lead to renal failure. Causes include autoimmune disorders, infections, diabetes, and malignancies."
      },
      "child_count": 40,
      "reference_id": "MONDO:0002462"
    }
  ],
  "children": [
    {
      "id": 11746,
      "label": "membranoproliferative glomerulonephritis, X-linked",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18832
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0024739",
          "MEDGEN:336706",
          "MESH:C564423",
          "OMIM:305800",
          "UMLS:C1844501"
        ],
        "synonyms": [
          "membranoproliferative glomerulonephritis, X-linked",
          "Mesangiocapillary glomerulonephritis, X-linked"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0010596"
    },
    {
      "id": 15015,
      "label": "immunoglobulin-mediated membranoproliferative glomerulonephritis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        4449,
        7021,
        18832
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080388",
          "GARD:0017506",
          "MEDGEN:767244",
          "NANDO:1200726",
          "NCIT:C123055",
          "OMIM:615008",
          "Orphanet:329903",
          "UMLS:C3554330"
        ],
        "synonyms": [
          "Ig-mediated MPGN",
          "Ig-mediated membranoproliferative glomerulonephritis",
          "NPHS7",
          "immune complex mediated membranoproliferative glomerulonephritis",
          "immunoglobulin-mediated MPGN",
          "immunoglobulin-mediated membranoproliferative glomerulonephritis",
          "membranoproliferative glomerulonephritis type I",
          "mesangiocapillary glomerulonephritis type 1",
          "nephrotic syndrome, type 7",
          "nephrotic syndrome, type 7, with membranoproliferative glomerulonephritis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          },
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          }
        ],
        "definition": "Glomerulonephritis characterized by mesangial proliferation, endocapillary proliferation, and glomerular capillary wall remodeling with immune complex deposits from classical complement pathway activation."
      },
      "child_count": 0,
      "reference_id": "MONDO:0014005"
    },
    {
      "id": 18187,
      "label": "complement 3 glomerulopathy",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        18832
      ],
      "type_id": 0,
      "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."
      },
      "child_count": 3,
      "reference_id": "MONDO:0018013"
    }
  ],
  "roots": [
    {
      "id": 4542,
      "label": "glomerulonephritis"
    }
  ]
}