Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos.
To evaluate the characteristics and outcomes of the patients diagnosed as ACTH-dependent Cushing syndrome, registered in the department.We reviewed the files of forty-three patients followed from 1974 to 2002.A progressive rise in the number of patients diagnosed was found, being 80% females. Clinic...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Ordem dos Médicos
2004-10-01
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| Series: | Acta Médica Portuguesa |
| Online Access: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1111 |
| _version_ | 1811242418528321536 |
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| author | Isabel Paiva Cristina Ribeiro Leonor Gomes Carla Baptista Fernando Gomes Manuel Rito Olinda Rebelo Dina Marnoto Cristina Moura Fátima Leitão Manuela Carvalheiro |
| author_facet | Isabel Paiva Cristina Ribeiro Leonor Gomes Carla Baptista Fernando Gomes Manuel Rito Olinda Rebelo Dina Marnoto Cristina Moura Fátima Leitão Manuela Carvalheiro |
| author_sort | Isabel Paiva |
| collection | DOAJ |
| description | To evaluate the characteristics and outcomes of the patients diagnosed as ACTH-dependent Cushing syndrome, registered in the department.We reviewed the files of forty-three patients followed from 1974 to 2002.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.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. |
| first_indexed | 2024-04-12T13:51:01Z |
| format | Article |
| id | doaj.art-4f873c52d44545bf82bb4b5561e8e790 |
| institution | Directory Open Access Journal |
| issn | 0870-399X 1646-0758 |
| language | English |
| last_indexed | 2024-04-12T13:51:01Z |
| publishDate | 2004-10-01 |
| publisher | Ordem dos Médicos |
| record_format | Article |
| series | Acta Médica Portuguesa |
| spelling | doaj.art-4f873c52d44545bf82bb4b5561e8e7902022-12-22T03:30:31ZengOrdem dos MédicosActa Médica Portuguesa0870-399X1646-07582004-10-0117510.20344/amp.1111Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos.Isabel Paiva0Cristina RibeiroLeonor GomesCarla BaptistaFernando GomesManuel RitoOlinda RebeloDina MarnotoCristina MouraFátima LeitãoManuela CarvalheiroServiço de Endocrinologia, Diabetes e Metabolismo, Hospitais da Universidade de Coimbra, Coimbra.To evaluate the characteristics and outcomes of the patients diagnosed as ACTH-dependent Cushing syndrome, registered in the department.We reviewed the files of forty-three patients followed from 1974 to 2002.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.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.https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1111 |
| spellingShingle | Isabel Paiva Cristina Ribeiro Leonor Gomes Carla Baptista Fernando Gomes Manuel Rito Olinda Rebelo Dina Marnoto Cristina Moura Fátima Leitão Manuela Carvalheiro Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos. Acta Médica Portuguesa |
| title | Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos. |
| title_full | Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos. |
| title_fullStr | Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos. |
| title_full_unstemmed | Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos. |
| title_short | Síndroma de cushing ACTH-dependente: estudo retrospectivo de 43 casos. |
| title_sort | sindroma de cushing acth dependente estudo retrospectivo de 43 casos |
| url | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1111 |
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