Browsing by Author "Marnoto, D"
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- Neurorradiologia da síndroma de KallmannPublication . Melo Freitas, P; Carvalho, S; Ribeiro, F; Marnoto, D; Martins, FThe present study refers to the magnetic resonance characteristics observed in a group of three patients with clinical and laboratory evidence of Kallmann's Syndrome. This disease, also named olfactogenital dysplasia, affects the normal gonadal and olfactory development and is clinically characterized by the association of hypogonadotrophic hypogonadism and anosmia/hyposmia. The study protocol, which included the attainment of sagittal and coronal T1-weighted MPRAGE (Multiplanar Rapid Acquisition Gradient Echo) volumetric sequences, showed defective rhinocephalon development in all patients, expressed by the aplasia/hypoplasia of the olfactory bulbs/tracts and/or of the olfactory sulci. The changes observed corroborate the predictable imaging of this genetic disorder and have been mainly visible in the coronal slices obtained. The results shown are more clear evidence that magnetic resonance is an effective method to show the abnormalities in the olfactory region that are invariably found in this syndrome.
- Síndroma de Cushing ACTH-dependente: estudo retrospectivoPublication . Paiva, I; Ribeiro, C; Gomes, L; Baptista, C; Gomes, F; Rito, M; Rebelo, O; Marnoto, D; Moura, C; Leitão, F; Carvalheiro, MAIM: To evaluate the characteristics and outcomes of the patients diagnosed as ACTH-dependent Cushing syndrome, registered in the department. MATERIAL AND METHODS: We reviewed the files of forty-three patients followed from 1974 to 2002. RESULTS: A progressive rise in the number of patients diagnosed was found, being 80% females. Clinical suspicion was based mostly on the typical fat distribution; hirsutism and amenorrhoea were important in women. The more reliable diagnostic tests were: 11 pm cortisol, day curve of ACTH and cortisol, and dexamethasone suppression tests. The ACTH response to CRH during inferior petrosal sinus sampling permitted the diagnosis of ectopic source. In thirty-seven patients a pituitary adenoma was diagnosed. The three patients diagnosed before 1985 went for bilateral adrenalectomy (Nelson's syndrome in two); the others were submitted to transsphenoidal pituitary adenomectomy, obtaining remission in twenty six at the first operation and in two others at the second. Three patients had a recidive. Of the six patients with persistent disease (all treated with metyrapone or ketoconazole), three were submitted to radiotherapy, two to bilateral adrenalectomy, and one was waiting for surgery. Four patients had a bronchial carcinoid, successfully removed in three. One patient was lost to follow-up and another was still being evaluated. CONCLUSIONS: We found a positive evolution in the capacity to diagnose and treat these patients. Neurosurgical ability to achieve remission was 80% in the operated cases. More effective technical methods and drugs, as well as a multidisciplinary and dedicated medical team, lead to long lasting remissions in most of the patients.