<unk>  is a form of reactive pancreatic alpha cell hyperplasia. The loss of normal glucagon signaling (particularly in the liver) due to inactive glucagon receptor results in enzymatic changes in the liver so that the liver releases abnormally large quantities of amino acids into the circulation. High concentration of amino acids in the circulation (hyperaminoacidemia) stimulates the pancreatic alpha cells (the cells that normally produce glucagon) to proliferate and to produce and release much more glucagon. The enormous proliferative activity of the pancreatic alpha cells predisposes them to tumor formation. All patients with<unk>  have hyperaminoacidemia, hyperglucagonemia (high concentration of glucagon in the circulation), and pancreatic alpha cell hyperplasia, and develop pancreatic neuroendocrine tumors in adulthood.
Mahvash disease
Dejerine–Sottas disease
Keshan disease
Yushō disease
Barakat syndrome
Satoyoshi syndrome
Sugarman syndrome
Nicolau–Balus syndrome