Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia

Detalhes bibliográficos
Autor(a) principal: Costa,Marcia Helena Soares
Data de Publicação: 2007
Outros Autores: Lacroix,André
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Arquivos Brasileiros de Endocrinologia & Metabologia (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302007000800008
Resumo: ACTH-Independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of endogenous Cushing's syndrome (CS), in which clinical features usually become apparent only after several decades of life. This form of adrenal hyperplasia typically produces excess cortisol with overt or subclinical CS, but concurrent secretion of mineralocorticoids or sexual steroids can also occur. The diagnosis is suspected by bilateral adrenal nodules larger than 1 cm on incidental imaging studies or following the demonstration of ACTH-independent hormonal hypersecretion. The pathophysiology of this entity is heterogeneous and has been intensely explored in recent years. Several G-protein coupled receptors aberrantly expressed in the adrenal cortex have been implicated in the regulation of steroidogenesis and in the initial cell proliferation in AIMAH. Several familial cases of AIMAH have been recently described with the same pattern of aberrant hormone receptors in all affected members of the family. It is probable that additional somatic genetic events related to cell cycle regulation, adhesion and transcription factors occur in addition over time in the various nodules; other mechanisms, as Gsp or ACTH receptor mutations and paracrine adrenal hormonal secretion have been rarely identified as the molecular mechanism in some cases. When systematically screened, most patients with AIMAH exhibit an in vivo aberrant cortisol response to one or various ligands suggesting the presence of aberrant adrenal receptors. The identification of these receptors creates the possibility of a specific pharmacological treatment isolated or associated with adrenalectomy.
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spelling Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasiaACTH-independent macronodular adrenal hyperplasia (AIMAH)Aberrant adrenal receptorsCushing's syndromeACTH-Independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of endogenous Cushing's syndrome (CS), in which clinical features usually become apparent only after several decades of life. This form of adrenal hyperplasia typically produces excess cortisol with overt or subclinical CS, but concurrent secretion of mineralocorticoids or sexual steroids can also occur. The diagnosis is suspected by bilateral adrenal nodules larger than 1 cm on incidental imaging studies or following the demonstration of ACTH-independent hormonal hypersecretion. The pathophysiology of this entity is heterogeneous and has been intensely explored in recent years. Several G-protein coupled receptors aberrantly expressed in the adrenal cortex have been implicated in the regulation of steroidogenesis and in the initial cell proliferation in AIMAH. Several familial cases of AIMAH have been recently described with the same pattern of aberrant hormone receptors in all affected members of the family. It is probable that additional somatic genetic events related to cell cycle regulation, adhesion and transcription factors occur in addition over time in the various nodules; other mechanisms, as Gsp or ACTH receptor mutations and paracrine adrenal hormonal secretion have been rarely identified as the molecular mechanism in some cases. When systematically screened, most patients with AIMAH exhibit an in vivo aberrant cortisol response to one or various ligands suggesting the presence of aberrant adrenal receptors. The identification of these receptors creates the possibility of a specific pharmacological treatment isolated or associated with adrenalectomy.Sociedade Brasileira de Endocrinologia e Metabologia2007-11-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302007000800008Arquivos Brasileiros de Endocrinologia & Metabologia v.51 n.8 2007reponame:Arquivos Brasileiros de Endocrinologia & Metabologia (Online)instname:Sociedade Brasileira de Endocrinologia e Metabologia (SBEM)instacron:SBEM10.1590/S0004-27302007000800008info:eu-repo/semantics/openAccessCosta,Marcia Helena SoaresLacroix,Andréeng2008-01-14T00:00:00Zoai:scielo:S0004-27302007000800008Revistahttps://www.aem-sbem.com/ONGhttps://old.scielo.br/oai/scielo-oai.php||abem-editoria@endocrino.org.br1677-94870004-2730opendoar:2008-01-14T00:00Arquivos Brasileiros de Endocrinologia & Metabologia (Online) - Sociedade Brasileira de Endocrinologia e Metabologia (SBEM)false
dc.title.none.fl_str_mv Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
title Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
spellingShingle Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
Costa,Marcia Helena Soares
ACTH-independent macronodular adrenal hyperplasia (AIMAH)
Aberrant adrenal receptors
Cushing's syndrome
title_short Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
title_full Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
title_fullStr Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
title_full_unstemmed Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
title_sort Cushing's syndrome secondary to ACTH-Independent macronodular adrenal hyperplasia
author Costa,Marcia Helena Soares
author_facet Costa,Marcia Helena Soares
Lacroix,André
author_role author
author2 Lacroix,André
author2_role author
dc.contributor.author.fl_str_mv Costa,Marcia Helena Soares
Lacroix,André
dc.subject.por.fl_str_mv ACTH-independent macronodular adrenal hyperplasia (AIMAH)
Aberrant adrenal receptors
Cushing's syndrome
topic ACTH-independent macronodular adrenal hyperplasia (AIMAH)
Aberrant adrenal receptors
Cushing's syndrome
description ACTH-Independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of endogenous Cushing's syndrome (CS), in which clinical features usually become apparent only after several decades of life. This form of adrenal hyperplasia typically produces excess cortisol with overt or subclinical CS, but concurrent secretion of mineralocorticoids or sexual steroids can also occur. The diagnosis is suspected by bilateral adrenal nodules larger than 1 cm on incidental imaging studies or following the demonstration of ACTH-independent hormonal hypersecretion. The pathophysiology of this entity is heterogeneous and has been intensely explored in recent years. Several G-protein coupled receptors aberrantly expressed in the adrenal cortex have been implicated in the regulation of steroidogenesis and in the initial cell proliferation in AIMAH. Several familial cases of AIMAH have been recently described with the same pattern of aberrant hormone receptors in all affected members of the family. It is probable that additional somatic genetic events related to cell cycle regulation, adhesion and transcription factors occur in addition over time in the various nodules; other mechanisms, as Gsp or ACTH receptor mutations and paracrine adrenal hormonal secretion have been rarely identified as the molecular mechanism in some cases. When systematically screened, most patients with AIMAH exhibit an in vivo aberrant cortisol response to one or various ligands suggesting the presence of aberrant adrenal receptors. The identification of these receptors creates the possibility of a specific pharmacological treatment isolated or associated with adrenalectomy.
publishDate 2007
dc.date.none.fl_str_mv 2007-11-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
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status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302007000800008
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-27302007000800008
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/S0004-27302007000800008
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dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Sociedade Brasileira de Endocrinologia e Metabologia
publisher.none.fl_str_mv Sociedade Brasileira de Endocrinologia e Metabologia
dc.source.none.fl_str_mv Arquivos Brasileiros de Endocrinologia & Metabologia v.51 n.8 2007
reponame:Arquivos Brasileiros de Endocrinologia & Metabologia (Online)
instname:Sociedade Brasileira de Endocrinologia e Metabologia (SBEM)
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reponame_str Arquivos Brasileiros de Endocrinologia & Metabologia (Online)
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