Obesity due to prohormone convertase I deficiency

MONDO:0010961

Prohormone convertase-I deficiency is the rarest form of monogenic obesity. The disorder is characterized by severe childhood obesity, hypoadrenalism, reactive hypoglycaemia, and elevated circulating levels of certain prohormones.

Also known as: PCI deficiency, PCSK1 Deficiency, obesity and endocrinopathy due to impaired processing of prohormones, proprotein convertase 1/3 deficiency

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