Our data suggest that, in most normal infants, the development of hypothalamic-pituitary-adrenal axis circadian maturation may occur at a much earlier age than previously described.

Our results support the possibility that altered miRNA expression profile might be involved in corticotrophic tumorigenesis. However, the lack of knowledge about miRNA target genes postpones full understanding of the biological functions of down-regulated or up-regulated miRNAs in corticotropinomas.

We have identified novel inherited MKRN3 defects in children with apparently sporadic CPP, supporting a fundamental role of this peptide in the suppression of the reproductive axis.

Together, these data demonstrate that NLRP3 inflammasome, through activation of IL-1R, is critically involved in the deleterious vascular effects of aldosterone, placing NLRP3 as a potential target for therapeutic interventions in conditions with high aldosterone levels.

No abstract

Objective: This study is an updated review of a Southeast Brazilian experience NFPA, emphasizing clinical features, laboratorial and imaging assessment, therapeutic management and outcome. Design and Methods: Retrospective study, in which 104 patients with NFPA were evaluated by the same team of endocrinologists and neurosurgeon. Patients underwent biochemical evaluation, radiological studies and visual field assessment. Results: Hypopituitarism and neuro-ophthalmological defects were observed in 89%. We observed GH deficiency (81.4%), hypogonadism (63.3%), adrenal hypofunction (59.5%), hypothyroidism (20.4%), high (38.5%) and low (16.7%) prolactin levels. Preoperative imaging classified 93% of the tumors as macroadenomas. Extra-sellar expansion was observed in 83.8%. Varying degrees of visual disturbance were observed in 74%. Primary treatment was transsphenoidal surgery (75%). Clinical control was achieved with one surgery in 37.5 % of patients. The majority of patients needed a second therapeutic approach, radiotherapy or other surgeries. Immunohistochemistry resulted negative for pituitary hormones in 43%. Improvement of neuro-ophthalmological symptoms was observed in 61% of the patients after treatment. Conclusions: Our data confirm elevated prevalence of mass effect and hypopituitarism in patients harboring NFPA. Recurrence due to invasion or incomplete resection of the tumor is quite common, which frequently leads to a second therapeutic option. Arq Bras endocrinol metab. 2009;53(1):31-39. KeywordsPituitary adenomas; hypopituitarism; neuro-ophthalmological defects; extra-sellar expansion; transsphenoidal surgery resuMo Objetivos: Esse estudo é uma revisão atualizada em adenomas hipofisários não-secretores (AHNS), enfatizando características clínicas, avaliações laboratorial e neuro-radiológica, manejo terapêutico e desfecho. Métodos: Estudo retrospectivo, em que 104 pacientes com AHNS foram avaliados pela mesma equipe de endocrinologistas e neurocirurgião. Os pacientes foram submetidos à avaliação bioquímica, a estudos radiológicos e à avaliação de campo visual. Resultados: Hipopituitarismo e distúrbios neuro-oftalmológicos foram observados em 89%. Observou-se deficiência de GH (81,4%), hipogonadismo (63,3%), hipofunção adrenal (59,5%), hipotireoidismo (20,4%), concentrações elevadas (38,5%) e baixas (16,7%) de prolactina. Imagens pré-operatórias classificaram os tumores em sua maioria como macroadenomas (93%). Expansão extra-selar foi observada em 83,8%. Graus variados de distúrbios visuais foram observados em 74%. O tratamento de escolha foi a cirurgia transesfenoidal (75%). O controle clínico foi alcançado com cirurgia em 37,5% dos pacientes. A maioria dos pacientes precisou de segunda abordagem terapêutica, radioterapia ou outras cirurgias. A imunoistoquímica resultou negativa para hormônios hipofisários em 43%. Melhora de sintomas neuro-oftalmológicos foi observada em 61% dos pacientes após o tratamento. Conclusões: Os dados do estudo confirmam elevada prevalência de efeitos de massa e hipopi...

The combination of salivary cortisol determination at 2300 hours and after-Dexamethasone is an easily performed and noninvasive method with high specificity and sensitivity for diagnosing Cushing's syndrome in children.

CTNNB1 mutations are not common in childhood ACT but appear to associate with poor prognosis. Nevertheless, most ACT exhibit increased expression of β-catenin and WISP2 and reduced expression of Wnt inhibitor genes (DKK3, SFRP1, and AXIN1). Thus, in addition to CTNNB1 mutations, other genetic events affecting the Wnt/β-catenin pathway may be involved in childhood adrenocortical tumorigenesis.