Obesity due to prohormone convertase-1 deficiency

Preferred Label : Obesity due to prohormone convertase-1 deficiency;

ICD-11 definition : Obesity due to prohormone convertase-I deficiency is the rarest form of monogenic obesity and is characterised by severe childhood obesity, hypoadrenalism, reactive hypoglycaemia, and elevated circulating levels of certain prohormones.;

ICD-11 synonym : PCI deficiency;

Details

Origin ID

331161581;

Automatic exact mappings (from CISMeF team)
- proprotein convertase 1/3 deficiency [DO Concept]
Currated CISMeF NLP mapping
- Obesity due to prohormone convertase I deficiency [ORDO Disease]
- Obesity due to prohormone convertase I deficiency (disorder) [SNOMED CT concept]
- Proprotein convertase 1/3 deficiency [OMIM Phenotype]
- proprotein convertase 1 3 deficiency [MeSH Supplementary Concept]
- proprotein convertase 1 3 deficiency [MeSH concept]

Keyword's position in hierarchy(ies) :

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