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Vol. 54. Núm. 7.
Páginas 379-389 (Agosto 2007)
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Vol. 54. Núm. 7.
Páginas 379-389 (Agosto 2007)
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Síndrome de Cushing por receptores corticosuprarrenales aberrantes
Cushing’s syndrome caused by aberrant adrenal receptors
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9505
Elena Outeiriñoa, Manuel Penína, Fernando Cordidoa,b,
Autor para correspondencia
fernando_cordido@canalejo.org

Correspondencia: Dr. F. Cordido. Servicio de Endocrinología. Complexo HospitalarioUniversitário Juan Canalejo. Xubias de Arriba, 84. 15006 A Coruña. España.
a Servicio de Endocrinología. Complexo Hospitalario Universitário Juan Canalejo. A Coruña. España
b Departamento de Medicina. Universidade Da Coruña. A Coruña. España
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En investigaciones recientes se ha demostrado que la producción de cortisol en algunos casos de síndrome de Cushing no dependiente de la corticotropina (previamente descritos como “autónomos”) está regulada por la existencia de receptores de membrana aberrantes que producen un estímulo crónico de las células corticosuprarrenales, no regulado de forma negativa por glucocorticoides, y que conduce a un incremento crónico de la esteroidogénesis y (posiblemente) a la proliferación celular en la glándula. Se han descrito receptores de este tipo en casos de síndrome de Cushing para varias hormonas, entre ellas el péptido inhibidor gástrico (GIP), la arginina vasopresina (AVP), las catecolaminas, la lutropina/gonadotropina coriónica humana, la serotonina y otras. Los mecanismos moleculares que conducen a la aparición de este tipo de receptores en la corteza suprarrenal todavía son desconocidos. Esta nueva variante etiológica del síndrome de Cushing no dependiente de la corticotropina dará lugar (como así está ocurriendo) a la utilización de tratamientos farmacológicos alternativos a la adrenalectomía. Son probables la identificación futura de nuevos receptores aberrantes capaces de inducir la esteroidogénesis que causa el síndrome de Cushing y la descripción de receptores aberrantes en otros órganos endocrinos y no endocrinos.

Palabras clave:
Síndrome de Cushing
Receptores aberrantes
Corticotropina

Recent studies have shown that cortisol production in some cases of adrenocorticotropic hormone (ACTH)- independent Cushing’s syndrome (previously suspected as being “autonomous”) is actually regulated by aberrant membrane receptors. These receptors produce a chronic stimulus on adrenal cells unregulated by the usual glucocorticoid negative feedback, thus increasing chronic steroidogenesis and possibly stimulating cellular hyperplasia. Receptors of this type have been described in cases of Cushing’s syndrome caused by a several hormones: gastric inhibitor peptide (GIP), arginine-vasopressin (AVP), catecholamines, LH/hCG, serotonin (5-HT) and others.

The molecular mechanisms leading to expression of this type of receptors in the adrenal cortex are still unknown. This new etiological variant of ACTH-independent Cushing’s syndrome will lead (as is already happening) to the use of new pharmacological alternatives to adrenalectomy. Potentially, further studies will identify other aberrant receptors that induce steroidogenesis leading to Cushing’s syndrome, as well as the presence of aberrant receptors in other endocrine and non-endocrine organs.

Key words:
Cushing’s syndrome
Aberrant receptors
Adrenocorticotropic hormone
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