GCGR-related hyperglucagonemia

Orpha code: 438274OMIM code: 619290

Definition

A rare tumor of pancreas caused by mutations in the <i>GCGR</i> 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.

Disease data
Classification

Disease

Synonyms
Mahvash disease
Choroba Mahvasha
ORPHA code
438274
OMIM code
619290
ICD10 code
E16.3
ICD11 code
-

No additional description.

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