Conditions / Endocrine

Mahvash Disease

info ยท Endocrine

An endocrine pancreas disease characterized by pancreatic alpha cell hyperplasia, pancreatic neuroendocrine tumors and increased serum glucagon levels that has_material_basis_in homozygous or compound heterozygous inactivating mutation of the GCGR gene on chro

An endocrine pancreas disease characterized by pancreatic alpha cell hyperplasia, pancreatic neuroendocrine tumors and increased serum glucagon levels that has_material_basis_in homozygous or compound heterozygous inactivating mutation of the GCGR gene on chromosome 17q25.3.

Signs and symptoms

  • Increased glucagon level
  • Vertigo
  • Pancreatic alpha-cell hyperplasia
  • Palpitations
  • Recurrent pancreatitis
  • Type II diabetes mellitus
  • Abdominal pain

Also known as: GCGR-related hyperglucagonemia; nesidioblastosis, alpha cell hyperplasia, microglucagonoma, and nonfunctioning islet cell tumor