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GTR Home > Conditions/Phenotypes > Islet cell adenomatosis

Islet cell adenomatosis

Summary

Insulinomatosis and diabetes mellitus syndrome is an autosomal dominant disorder in which affected individuals within a family present with either hyperinsulinemic hypoglycemia secondary to pancreatic neuroendocrine tumors, or a noninsulin-dependent form of diabetes mellitus. A few affected individuals show only impaired glucose tolerance. Some patients also exhibit congenital cataract and/or congenital glaucoma (Iacovazzo et al., 2018). [from OMIM]

Available tests

2 tests are in the database for this condition.

Clinical tests (2 available)

Molecular Genetics Tests

Genes See tests for all associated and related genes

  • Also known as: INSDM, RIPE3b1, hMafA, MAFA
    Summary: MAF bZIP transcription factor A

Clinical features

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Imported from Human Phenotype Ontology (HPO)

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