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Thickened corneal nerves in multiple endocrine neoplasia type IIb

✍ Scribed by Brooke S. Kirby

Publisher
Elsevier Science
Year
1997
Tongue
English
Weight
598 KB
Volume
9
Category
Article
ISSN
0953-4431

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✦ Synopsis

Thickened cornea1 nerves are a hallmark of multiple endocrine neoplasia type IIb, a genetic syndrome characterized by medullary carcinoma of the thyroid, pheochromocytomas, and a distinctive phenotype. Both types of neoplasm may be lethal, but recognition of the ocular and non-ocular stigmata can lead to early diagnosis and successful treatment. Here, a presumptive case of multiple endocrine neoplasia type IIb is presented with background information concerning the phenotype, ocular manifestations, inheritance, diagnosis and management of the syndrome. 0 1997 Elsevier Science Ireland Ltd.

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## Abstract ## BACKGROUND Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal‐dominant syndrome associated with neoplasia of pituitary, pancreas, parathyroid, and foregut lineage neuroendocrine tissue. Although enteropancreatic carcinoid has been well described in patients with MEN 1, it w