GCGR-related hyperglucagonemia

MONDO:0018582

A rare tumor of pancreas caused by mutations in the GCGR gene characterized by pancreatic alpha cell hyperplasia, pancreatic neuroendocrine tumors and markedly increased serum glucagon levels in the absence of a glucagonoma syndrome. Clinical manifestations may include abdominal pain, pancreatitis, fatigue, diarrhea, and diabetes mellitus.

Also known as: MVAH, Mahvash disease, alpha-cell hyperplasia with glucagonemia, nesidioblastosis alpha cell hyperplasia microglucagonoma and nonfunctioning islet cell tumor, nesidioblastosis alpha cell hyperplasia microglucagonoma and nonfunctioning islet cell tumour, nesidioblastosis, alpha cell hyperplasia, microglucagonoma, and nonfunctioning islet cell tumor, nesidioblastosis, alpha cell hyperplasia, microglucagonoma, and nonfunctioning islet cell tumour

1459 clinical trials for this condition and its sub-types, 0 tagged with GCGR-related hyperglucagonemia itself.

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