Term Name:	proprotein convertase 1/3 deficiency
Synonyms:	obesity and endocrinopathy due to impaired processing of prohormones, obesity due to prohormone convertase I deficiency, obesity with impaired prohormone processing, PCI deficiency
Definition:	A syndrome characterized by severe childhood obesity, hypoadrenalism, hypogonadism, reactive hypoglycaemia, and elevated circulating levels of certain prohormones that has_material_basis_in homozygous or compound heterozygous mutation in the PCSK1 gene on chromosome 5q15.
Ontology:	Human Disease [DOID:0111698]   ( DOID:0111698 )