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[ACTH-dependent Cushing's syndrome: a revision of 43 cases].
- Source :
-
Acta medica portuguesa [Acta Med Port] 2004 Sep-Oct; Vol. 17 (5), pp. 367-74. Date of Electronic Publication: 2004 Dec 20. - Publication Year :
- 2004
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Abstract
- Aim: To evaluate the characteristics and outcomes of the patients diagnosed as ACTH-dependent Cushing syndrome, registered in the department.<br />Material and Methods: We reviewed the files of forty-three patients followed from 1974 to 2002.<br />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.<br />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.
Details
- Language :
- Portuguese
- ISSN :
- 1646-0758
- Volume :
- 17
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- Acta medica portuguesa
- Publication Type :
- Academic Journal
- Accession number :
- 16197843