{
  "id": 17855,
  "label": "chronic intestinal pseudoobstruction",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0017574",
  "properties": {
    "xrefs": [
      "GARD:0012744",
      "MEDGEN:536759",
      "NANDO:1200458",
      "NANDO:2200946",
      "NORD:970",
      "Orphanet:2978",
      "SCTID:235828008",
      "UMLS:C0238062"
    ],
    "synonyms": [
      "CIPO",
      "Chronic Intestinal Pseudo-Obstruction",
      "chronic intestinal pseudo-obstruction",
      "cipo",
      "intestinal pseudo-obstruction, chronic"
    ],
    "categories": [
      {
        "ref": "MONDO:0004335",
        "name": "digestive system disorder"
      }
    ],
    "definition": "Chronic intestinal pseudo-obstruction (CIPO) is a rare gastrointestinal motility disorder characterized by recurring episodes resembling mechanical obstruction in the absence of organic, systemic, or metabolic disorders, and without any physical obstruction being detected by X-ray or during surgery. CIPO develops predominantly in children and may be present at birth."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 5,
  "parents": [
    {
      "id": 4829,
      "label": "intestinal pseudo-obstruction",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6365
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:3878",
          "GARD:0006789",
          "ICD9:569.89",
          "MEDGEN:5864",
          "MESH:D007418",
          "NCIT:C34733",
          "SCTID:235825006",
          "UMLS:C0021847"
        ],
        "synonyms": [
          "Chronic intestinal pseudo-obstruction.",
          "intestinal pseudo-obstruction",
          "intestine pseudoobstruction",
          "pseudo-obstruction of intestine",
          "hollow visceral myopathy",
          "intestinal pseudoobstruction"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ],
        "definition": "Intestinal pseudo-obstruction is a digestive disorder in whichthe intestinal walls are unable to contract normally (called hypomotility); the conditionresembles a true obstruction, but no actual blockage exists. Signs and symptoms may include abdominal pain; vomiting; diarrhea; constipation; malabsorption of nutrients leading to weight loss and/or failure to thrive ; and other symptoms. It may be classified as neuropathic (from lack of nerve function)or myopathic (from lack of muscle function), depending on the source of the abnormality. The condition is sometimes inherited (in an X-linked recessive or autosomal dominant manner)and may be caused by mutations in the FLNA gene; it may also be acquired after certain illnesses. The goal of treatment is to provide relief from symptoms andensure that nutritional support is adequate."
      },
      "child_count": 2,
      "reference_id": "MONDO:0002803"
    },
    {
      "id": 20415,
      "label": "intestinal motility disease",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6756
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "MEDGEN:586448",
          "UMLS:C0400865"
        ],
        "synonyms": [
          "disorder of intestinal motility"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ],
        "definition": "A disease that has its basis in the disruption of intestinal motility."
      },
      "child_count": 15,
      "reference_id": "MONDO:0021189"
    }
  ],
  "children": [
    {
      "id": 11410,
      "label": "intestinal pseudoobstruction, neuronal, chronic idiopathic, X-linked",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        5714,
        15106,
        17855,
        24405
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080681",
          "GARD:0003017",
          "MEDGEN:412536",
          "MESH:C535532",
          "OMIM:300048",
          "UMLS:C2746068"
        ],
        "synonyms": [
          "congenital short bowel syndrome, X-linked recessive",
          "intestinal pseudoobstruction, neuronal, X-linked recessive",
          "intestinal pseudoobstruction, neuronal, chronic idiopathic, X-linked",
          "CIIP",
          "CIIP X-linked",
          "CIIPX",
          "Ciip, X-linked",
          "Ipox",
          "congenital idiopathic intestinal pseudoobstruction",
          "congenital short bowel syndrome, X-linked",
          "intestinal pseudoobstruction neuronal chronic idiopathic X-linked",
          "intestinal pseudoobstruction, neuronal, chronic idiopathic, with central nervous system involvement"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ],
        "definition": "Intestinal pseudo-obstruction is a condition characterized by impairment of the muscle contractions that move food through the digestive tract. The condition may arise from abnormalities of the gastrointestinal muscles themselves (myogenic) or from problems with the nerves that control the muscle contractions (neurogenic). When intestinal pseudo-obstruction occurs by itself, it is called primary or idiopathic (unknown cause) intestinal pseudo-obstruction. The disorder can also develop as a complication of another medical condition; in these cases, it is called secondary intestinal pseudo-obstruction. Individuals with this condition have symptoms that resemble those of an intestinal blockage (obstruction) but without any obstruction. It may be acute or chronic and is characterized by the presence of dilation of the bowel on imaging. The causes may be unknown or due to alterations (mutations) in the FLNA gene, other genes or are secondary to other conditions. It may be inherited in some cases. Intestinal pseudoobstruction neuronal chronic idiopathic X-linked is caused by alterations (mutations) in the FLNA gene which is located in the X chromosome. There is no specific treatment but several medications and procedures may be used to treat the symptoms."
      },
      "child_count": 0,
      "reference_id": "MONDO:0010232"
    },
    {
      "id": 12147,
      "label": "neuronal intestinal dysplasia, type B",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3157,
        17855
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080680",
          "GARD:0024768",
          "MEDGEN:318658",
          "OMIM:601223",
          "UMLS:C1832589"
        ],
        "synonyms": [
          "NID B",
          "neuronal intestinal dysplasia, type B"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0011021"
    },
    {
      "id": 13373,
      "label": "visceral neuropathy, familial, 3, autosomal dominant",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17855
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080682",
          "GARD:0024859",
          "MEDGEN:351272",
          "OMIM:609629",
          "UMLS:C1864996"
        ],
        "synonyms": [
          "visceral neuropathy, familial, autosomal dominant",
          "enteric neuropathy, familial",
          "pseudoobstruction, chronic intestinal, neuropathic",
          "pseudoobstruction, idiopathic intestinal"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0012317"
    },
    {
      "id": 16104,
      "label": "myopathic intestinal pseudoobstruction",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        17855
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0019853",
          "MEDGEN:1843444",
          "Orphanet:104077",
          "UMLS:C5681739",
          "icd11.foundation:1007742014"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ]
      },
      "child_count": 0,
      "reference_id": "MONDO:0015191"
    },
    {
      "id": 29394,
      "label": "visceral neuropathy, familial, 1, autosomal recessive",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3157,
        17855,
        21281
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:0080679",
          "GARD:0003928",
          "MEDGEN:340946",
          "MESH:C537394",
          "OMIM:243180",
          "Orphanet:99811",
          "UMLS:C1855733"
        ],
        "synonyms": [
          "Argyrophil myenteric plexus deficiency of",
          "Argyrophil myenteric plexus, deficiency of",
          "NID A",
          "intestinal pseudoobstruction due to neuronal disease",
          "neuronal intestinal dysplasia, type a",
          "pseudoobstruction chronic idiopathic intestinal neuronal type",
          "pseudoobstruction, chronic idiopathic intestinal, neuronal type",
          "visceral neuropathy familial",
          "visceral neuropathy, familial, autosomal recessive"
        ],
        "categories": [
          {
            "ref": "MONDO:0004335",
            "name": "digestive system disorder"
          }
        ],
        "definition": "A form of chronic intestinal pseudoobstruction caused by a developmental failure of the enteric neurons to differentiate or migrate properly and manifests as a bowel obstruction."
      },
      "child_count": 0,
      "reference_id": "MONDO:8000011"
    }
  ],
  "roots": [
    {
      "id": 4829,
      "label": "intestinal pseudo-obstruction"
    },
    {
      "id": 20415,
      "label": "intestinal motility disease"
    }
  ]
}