A Patient with Multiple Endocrine Neoplasia Type 1 Presented with Precocious Puberty

Nese Akcan; Ruveyde Bundak

Volume : VII, Issue : XII, December - 2018

A Patient with Multiple Endocrine Neoplasia Type 1 Presented with Precocious Puberty

Nese Akcan, Ruveyde Bundak

Abstract :

Multiple Endocrine Neoplasia Type 1 (MEN1) is an autosomal dominant inherited tumour syndrome. Mean age at onset of MEN1 is rare before and during puberty. Although there are two case reports about MEN1 and delay puberty, early and rapidly progressive puberty with MEN1 has no reported. We presented a case whose father has MEN1, applied with central precocious puberty. In pituitary MR, hypointense region which was stabil in size during the follow up, was seen in intermediate lob but it was not interpreted in favor of the adenoma. On admission, serum calcium level was high whereas serum phosphorus, parathormone levels were normal. In follow up, PTH increased, and patient was diagnosed as MEN1. The diagnosis confirmed by moleculer analysis. This case emphasize relevance of early screening of endocrine disorders in members of families with MEN1. Precocious puberty can also be detected in the follow up of patients with MEN1