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Гіперглюкагонемія, повязана з GCGR

ORPHA:438274· ICD-10 E16.3· GCGR-related hyperglucagonemia

Визначення(English summary)

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.

Поширеність
<1 / 1 000 000
Успадкування
Autosomal recessive
Вік початку
Adult