Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.4/234
Título: Síndroma de Cushing ACTH-dependente: estudo retrospectivo
Outros títulos: ACTH-dependent Cushing's syndrome: a revision of 43 cases
Autor: Paiva, I
Ribeiro, C
Gomes, L
Baptista, C
Gomes, F
Rito, M
Rebelo, O
Marnoto, D
Moura, C
Leitão, F
Carvalheiro, M
Palavras-chave: Síndrome de Cushing
Data: 2004
Editora: Centro Editor Livreiro da Ordem dos Médicos
Citação: Acta Med Port. 2004 Sep-Oct;17(5):367-74
Resumo: AIM: 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.
URI: http://hdl.handle.net/10400.4/234
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