Thymic carcinoid in the setting of a multiple endocrine neoplasia syndrome (MEN 1). Prophylactic thymectomy?

ar enlargement, and no other signs of sexual development; ome mechanisms proposed for this phenomenon impliate the NR0B1 gene in the prepubertal control mechanism f the gonadal axis, ACTH-mediated stimulus of testicuar steroidogenesis, or autonomous hyperplasia of Leydig ells.2 In the pubertal period, these patients will require estosterone replacement for the development of secndary sexual characteristics. Since DAX1 abnormalities may ffect testicular development and spermatogenesis, fertility reatment using pulsatile GnRH and gonadotropins is often neffective.4 Mental disability (motor, speech, and social ehavior) may also occur. To our knowledge, there are no ases reported in the literature of an association between AX1 and GH deficiency as seen in case 1. patients with congenital adrenal hypoplasia. J Clin Endocrinol Metab. 2010 [Epub ahead of print]. 2. Landau Z, Hanukoglu A, Sack J, Goldstein N, Weintrob N, Eliakim A, et al. Clinical and genetic heterogeneity of congenital adrenal hypoplasia due to NR0B1 gene mutations. Clin Endocrinol (Oxf). 2010;72:448--54. 3. Wiltshire E, Couper J, Rodda C, Jameson JL, Achermann JC. Variable presentation of X-linked adrenal hypoplasia congenita. J Pediatr Endocrinol Metab. 2001;14: 1093--6. 4. Mantovani G, Ozisik G, Achermann JC, Romoli R, Borretta G, Persani L, et al. Hypogonadotropic hypogonadism as a presenting feature of late-onset X-linked adrenal hypoplasia congenita. J Clin Endocrinol Metab. 2002;87:44--8. 5. Lin L, Gu WX, Ozisik G, To WS, Owen CJ, Jameson JL, et al. Analysis of DAX1 (NR0B1) and steroidogenic factor-1 aded from http://www.elsevier.es, day 25/03/2018. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited.