Obesity due to prohormone convertase I deficiency
Obesity due to prohormone convertase I deficiency
Definition
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 — per MONDO