{
  "id": 19398,
  "label": "immunotactoid or fibrillary glomerulopathy",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0019605",
  "properties": {
    "xrefs": [
      "GARD:0012741",
      "MEDGEN:1842578",
      "Orphanet:91137",
      "UMLS:C5680195"
    ],
    "synonyms": [
      "Immunotactoid or fibrillary glomerulonephritis",
      "fibrillary glomerulonephritis and immunotactoid glomerulopathy"
    ],
    "categories": [
      {
        "ref": "MONDO:0002118",
        "name": "urinary system disorder"
      }
    ],
    "definition": "A group of very rare glomerular diseases, composed of immunotactoid glomerulopathy (ITG) and non-amyloid fibrillary glomerulopathy (non-amyloid FGP), that are characterized by mesangial deposition of monoclonal microtubular or polyclonal fibrillar deposits. Both present clinically with nephrotic range proteinuria, hematuria and renal insufficiency leading to renal failure in many cases. ITG is more likely to manifest with underlying lymphoproliferative disease, hypocomplementemia, dysproteinemia, monoclonal gammopathy or occult cryoglobulinemia. Non-amyloid FGP is 10 times more frequent than ITG."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 2,
  "parents": [
    {
      "id": 19482,
      "label": "glomerular disorder",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6948
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:1002049",
          "GTR:AN0966176",
          "ICD10CM:N00-N08",
          "MEDGEN:451033",
          "NCIT:C120887",
          "Orphanet:93548",
          "SCTID:197679002",
          "UMLS:C0268731"
        ],
        "synonyms": [
          "disease of renal glomerulus",
          "disease or disorder of renal glomerulus",
          "disorder of renal glomerulus",
          "glomerulopathy",
          "renal glomerulus disease",
          "renal glomerulus disease or disorder",
          "glomerulopathies"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "A disease involving the renal glomerulus."
      },
      "child_count": 6,
      "reference_id": "MONDO:0019722"
    }
  ],
  "children": [
    {
      "id": 19700,
      "label": "non-amyloid fibrillary glomerulopathy",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        19398
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0012740",
          "MEDGEN:907201",
          "NORD:2024",
          "Orphanet:97566",
          "SCTID:718192000",
          "UMLS:C4273674"
        ],
        "synonyms": [
          "Congo red-negative amyloidosis-like glomerulopathy",
          "Fibrillary Glomerulonephritis ",
          "non-amyloid fibrillary glomerulonephritis",
          "fibrillary glomerulonephritis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "Non-amyloid fibrillary glomerulopathy (non-amyloid FGP) is a rare cause of glomerulonephritis (GN) characterized by glomerular accumulation of non-amyloid fibrils in the mesangium and the glomerular (and rarely tubular) basement membrane, that mainly presents with renal insufficiency, micro-hematuria and nephrotic range proteinuria. Non-amyloid FGP and immunotactoid glomerulopathy (ITG) are often grouped together as pathogenetically related diseases."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019990"
    },
    {
      "id": 19701,
      "label": "immunotactoid glomerulopathy",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        4542,
        19398
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0012048",
          "ICD9:583.9",
          "MEDGEN:1684714",
          "NCIT:C158968",
          "NCIT:C96182",
          "Orphanet:97567",
          "SCTID:73305009",
          "UMLS:C5202806"
        ],
        "synonyms": [
          "FGN",
          "Immunotactoid glomerulonephritis",
          "fibrillary glomerulonephritis"
        ],
        "categories": [
          {
            "ref": "MONDO:0002118",
            "name": "urinary system disorder"
          }
        ],
        "definition": "Immunotactoid glomerulopathy (ITG) is a very rare condition characterized by glomerular accumulation of microtubules in the mesangium and the glomerular basement membrane, that mainly presents with proteinuria, micro-hematuria, nephrotic syndrome, renal insufficiency and hematologic malignancy. ITG and non-amyloid fibrillary glomerulopathy (non-amyloid FGP) are often grouped together as pathogenetically related diseases."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019991"
    }
  ],
  "roots": [
    {
      "id": 19482,
      "label": "glomerular disorder"
    }
  ]
}