{
  "id": 18311,
  "label": "primary essential cutis verticis gyrata",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0018167",
  "properties": {
    "xrefs": [
      "GARD:0021537",
      "MEDGEN:1632625",
      "Orphanet:357220",
      "SCTID:765135003",
      "UMLS:C4707327",
      "icd11.foundation:895947895"
    ],
    "categories": [
      {
        "ref": "MONDO:0002051",
        "name": "integumentary system disorder"
      }
    ],
    "definition": "Primary essential cutis verticis gyrata is a rare, progressive dermis disorder characterized by thickening of the scalp resulting in redundancy of the skin which gives rise to folds and grooves that give the scalp a cerebriform appearance. Folds cannot be corrected by pressure or traction and typically are symmetric and extend anteroposteriorly from vertex to occiput and/or transversely in occipital region. Additional features may include mild subungual hyperkeratosis and distal onycholysis of the nail plates of the great toes. It is not associated with neurological and ophthalmological changes, nor with secondary causes."
  },
  "isLeaf": true,
  "isRoot": false,
  "child_count": 0,
  "parents": [
    {
      "id": 18945,
      "label": "primary cutis verticis gyrata",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        20387
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0001643",
          "ICD9:757.39",
          "MEDGEN:78095",
          "Orphanet:671",
          "SCTID:51603000",
          "UMLS:C0263417",
          "icd11.foundation:71926572"
        ],
        "synonyms": [
          "cutis verticis gyrata"
        ],
        "categories": [
          {
            "ref": "MONDO:0002051",
            "name": "integumentary system disorder"
          }
        ],
        "definition": "Cutis verticis gyrata (CVG) is a progressive cutaneous disorder predominantly affecting males and characterized by hypertrophy and thickening of the skin of the scalp forming convoluted furrows with deep, tender, and cerebriform cutaneous folds. Hair is usually normal in the furrows and sparse on the folds. CG can be isolated (essential CVG) or associated with other abnormalities such as intellectual deficit, epilepsy, cataract, blindness, and deafness (non essential CVG)."
      },
      "child_count": 3,
      "reference_id": "MONDO:0019033"
    }
  ],
  "children": [],
  "roots": [
    {
      "id": 18945,
      "label": "primary cutis verticis gyrata"
    }
  ]
}