<unk>  is an autosomal recessive, hereditary pancreatic neuroendocrine tumor syndrome. The genetic defect that causes<unk>  is biallelic inactivating mutations of the glucagon receptor gene (GCGR).<unk>  was discovered by American physician Run Yu and his colleagues in 2008.<unk>  is very rare. There have been over 10 cases of<unk>  described by the end of 2018.<unk>  occurs in both females and males.<unk>  is also called “glucagon cell hyperplasia and neoplasia” or “glucagon cell adenomatosis” by some authors but<unk>  is a distinct disease entity while the two alternative terms are mostly histological descriptions. Some patients with “glucagon cell hyperplasia and neoplasia” do not have glucagon receptor mutations.
Mahvash disease
Dejerine–Sottas disease
Keshan disease
Yushō disease
Barakat syndrome
Satoyoshi syndrome
Sugarman syndrome
Nicolau–Balus syndrome