{
    "synonyms": [
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "BCKDH deficiency"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease, type 2"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease; MSUD"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "dihydrolipoamide dehydrogenase deficiency"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease, thiamine-responsive"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease, classic"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease, intermittent"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease, type 1B"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease, type 1A"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "branched chain ketoaciduria"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "branched-chain ketoaciduria"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "MSUD"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "Ketoacidaemia"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "branched-chain 2-ketoacid dehydrogenase deficiency"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "BCKD deficiency"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "branched-chain Alpha-Keto acid dehydrogenase deficiency"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "Keto acid decarboxylase deficiency"
        }, 
        {
            "url": "https://monarchinitiative.org/disease/MONDO:0009563", 
            "description": "", 
            "resource": "MONDO", 
            "id": "MONDO:0009563", 
            "name": "maple syrup urine disease, Intermediate"
        }, 
        {
            "url": "http://disease-ontology.org/term/DOID:9269", 
            "description": "", 
            "resource": "Disease Ontology", 
            "id": "DOID:9269", 
            "name": "Ketoacidaemia"
        }, 
        {
            "url": "http://disease-ontology.org/term/DOID:9269", 
            "description": "", 
            "resource": "Disease Ontology", 
            "id": "DOID:9269", 
            "name": "branched chain ketoaciduria"
        }, 
        {
            "url": "https://www.omim.org/entry/248600", 
            "description": "", 
            "resource": "MIM", 
            "id": "MIM:248600", 
            "name": "Maple syrup urine disease 2"
        }
    ], 
    "recommended_name": {
        "url": "http://disease-ontology.org/term/DOID:9269", 
        "description": "An organic acidemia that is caused by a deficiency of decarboxylase leading to high concentrations of valine, leucine, isoleucine, and alloisoleucine in the blood, urine, and cerebrospinal fluid and characterized by an odor of maple syrup to the urine, vomiting, hypertonicity, severe mental retardation, seizures, and eventually death unless the condition is treated with dietary measures.", 
        "resource": "Disease Ontology", 
        "id": "DOID:9269", 
        "name": "maple syrup urine disease"
    }, 
    "evidence": [
        {
            "url": "https://www.omim.org/entry/248600", 
            "id": "248600", 
            "database": "MIM"
        }
    ]
}