{
  "id": 18642,
  "label": "sclerosing cholangitis",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018646",
  "properties": {
    "xrefs": [
      "DOID:14268",
      "EFO:0004268",
      "GARD:0021868",
      "HP:0030991",
      "MEDGEN:3036",
      "NANDO:1200440",
      "NANDO:2100265",
      "NCIT:C4828",
      "Orphanet:447771",
      "SCTID:235917005",
      "UMLS:C0008313"
    ],
    "synonyms": [
      "Primary sclerosing cholangitis",
      "primary sclerosing cholangitis (PSC)",
      "sclerosing cholangitis",
      "sclerosing cholangitis (disease)"
    ],
    "categories": [
      {
        "ref": "MONDO:0004335",
        "name": "digestive system disorder"
      },
      {
        "ref": "MONDO:0005151",
        "name": "endocrine system disorder"
      }
    ],
    "definition": "A chronic, autoimmune inflammatory liver disorder characterized by narrowing and scarring of the lumen of the bile ducts. It is often seen in patients with ulcerative colitis. Signs and symptoms include jaundice, fatigue, and malabsorption. It may lead to cirrhosis and liver failure."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 5,
  "parents": [
    {
      "id": 6556,
      "label": "cholangitis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        7855,
        20399
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:9446",
          "ICD10CM:K83.0",
          "ICD9:576.1",
          "MEDGEN:40258",
          "MESH:D002761",
          "NCIT:C26718",
          "SCTID:82403002",
          "UMLS:C0008311",
          "icd11.foundation:1712178777"
        ],
        "synonyms": [
          "biliary tract infection",
          "biliary tree inflammation",
          "cholangitis",
          "inflammation of biliary tree"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "An acute or chronic inflammatory process affecting the biliary tract."
      },
      "child_count": 18,
      "reference_id": "MONDO:0004789"
    }
  ],
  "children": [
    {
      "id": 12953,
      "label": "neonatal ichthyosis-sclerosing cholangitis syndrome",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        4370,
        16624,
        18642
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0010583",
          "MEDGEN:334382",
          "MESH:C564365",
          "OMIM:607626",
          "Orphanet:59303",
          "SCTID:724278007",
          "UMLS:C1843355"
        ],
        "synonyms": [
          "IHSC",
          "NISCH syndrome",
          "ichthyosis-hypotrichosis-sclerosing cholangitis syndrome",
          "neonatal ichthyosis-sclerosing cholangitis syndrome",
          "ILVASC",
          "Ilvasc",
          "Nisch syndrome",
          "ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis",
          "ichthyosis-sclerosing cholangitis syndrome"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          },
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Neonatal ichthyosis-sclerosing cholangitis (NISCH syndrome) is a very rare complex ichthyosis syndrome characterized by scalp hypotrichosis, scarring alopecia, ichthyosis and sclerosing cholangitis."
      },
      "child_count": 0,
      "reference_id": "MONDO:0011874"
    },
    {
      "id": 14465,
      "label": "primary sclerosing cholangitis",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        18642
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0060643",
          "GARD:0001280",
          "ICD10CM:K83.01",
          "MEDGEN:107565",
          "MedDRA:10036732",
          "NANDO:1200440",
          "NANDO:2100265",
          "NORD:1613",
          "OMIM:613806",
          "Orphanet:171",
          "SCTID:197441003",
          "UMLS:C0566602",
          "icd11.foundation:857962451"
        ],
        "synonyms": [
          "PSC",
          "cholangitis, primary sclerosing"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure."
      },
      "child_count": 1,
      "reference_id": "MONDO:0013433"
    },
    {
      "id": 18641,
      "label": "IgG4-related sclerosing cholangitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        2997,
        3004,
        17611,
        18642
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021867",
          "MEDGEN:927778",
          "NANDO:1200928",
          "Orphanet:447764",
          "SCTID:722870008",
          "UMLS:C4302109",
          "icd11.foundation:1676971795"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005046",
            "name": "immune system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018645"
    },
    {
      "id": 18643,
      "label": "secondary sclerosing cholangitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        18642
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021869",
          "MEDGEN:586530",
          "Orphanet:447774",
          "SCTID:197442005",
          "UMLS:C0400978"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0018647"
    },
    {
      "id": 18760,
      "label": "isolated neonatal sclerosing cholangitis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        5714,
        18642
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0021983",
          "MEDGEN:1393230",
          "OMIM:617394",
          "Orphanet:480556",
          "UMLS:C4479344"
        ],
        "synonyms": [
          "NSC",
          "sclerosing cholangitis, neonatal"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          },
          {
            "ref": "MONDO:0005151",
            "name": "endocrine system disorder"
          }
        ],
        "definition": "Isolated neonatal sclerosing cholangitis is a rare, genetic, biliary tract disease characterized by severe neonatal-onset cholangiopathy with patent bile ducts and absence of ichthyosiform skin lesions. Patients present with jaundice, acholic stools, hepatosplenomegaly and high serum gamma-glutamyltransferase activity. Liver histology shows portal fibrosis, ductular proliferation, hepatocellular metallothionein deposits, and intralobular bile-pigment accumulations. Some patients may also have renal disease."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018816"
    }
  ],
  "roots": [
    {
      "id": 6556,
      "label": "cholangitis"
    }
  ]
}