Pancreatic alpha-cell hyperplasia

MedGen UID:: 1800812
•Concept ID:: C5558401
•: Finding

HPO:	HP:4000061

Definition

A diffuse and specific increase in the number of alpha-cells. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPancreatic alpha-cell hyperplasia

Abnormality of the abdominal organs
- Abnormality of the pancreas
  - Abnormality of pancreas physiology
    - Abnormality of endocrine pancreas physiology
      - Abnormality of the pancreatic islet cells
        Pancreatic islet-cell hyperplasia
        Pancreatic alpha-cell hyperplasia

Conditions with this feature

GCGR-related hyperglucagonemia

MedGen UID:: 1677024
•Concept ID:: C4763635
•: Disease or Syndrome

Mahvash disease (MVAH) is an autosomal recessive disorder caused by inactivating mutations in the glucagon receptor, leading to alpha-cell hyperplasia of the pancreas, hyperglucagonemia without glucagonoma syndrome, and occasional hypoglycemia. The disease may lead to glucagonomas and/or primitive neuroectodermal tumors (PNETs).

See: Condition Record

GCGR-related hyperglucagonemia

Recent clinical studies

Diagnosis

Liver Transplantation in a Woman with Mahvash Disease.

Robbins J, Halegoua-DeMarzio D, Basu Mallick A, Vijayvergia N, Ganetzky R, Lavu H, Giri VN, Miller J, Maley W, Shah AP, DiMeglio M, Ambelil M, Yu R, Sato T, Lefler DS
N Engl J Med 2023 Nov 23;389(21):1972-1978. doi: 10.1056/NEJMoa2303226. PMID: 37991855

Pancreatic α-cell hyperplasia: facts and myths.

Yu R
J Clin Endocrinol Metab 2014 Mar;99(3):748-56. Epub 2013 Nov 27 doi: 10.1210/jc.2013-2952. PMID: 24285676

See all (2)