A case of ACTH-independent bilateral macronodular adrenal hyperplasia and severe congestive heart failure

D. Suri, M. Alonso, Roy E Weiss

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Cortisol secretion in ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH) can be regulated by aberrant adrenal receptors. We describe a patient with Cushing's syndrome (CS) due to AIMAH and concomitant Class IV congestive heart failure (CHF). Clinical testing for the presence of aberrant receptors revealed a pronounced serum cortisol (257%) and aldosterone response (212%) to the administration of ACTH and a partial serum cortisol (35%) and aldosterone (106%) response to upright posture. This suggested the possible presence of aberrant hormone receptors for ACTH [melanocortin 2 receptor (MC2-R)], vasopressin, catecholamines or angiotensin II (AT-II) on the patient's adrenal glands. Adrenal tissue from the patient demonstrated an eight-fold increased expression of MC2-R compared to normal adrenal tissue. This increased expression was consistent with the increase in cortisol and aldosterone seen in response to exogenous ACTH. We propose that the severe CHF resulted in activation of the renin-angiotensin system, with an increased production of AT-II. The elevated circulating levels of AT-II may have led to increased expression of MC2-R on the patient's adrenal glands and increased responsiveness to ACTH. This unusual case of CS may elucidate a heretofore unknown mechanism for the development of AIMAH.

Original languageEnglish (US)
Pages (from-to)940-946
Number of pages7
JournalJournal of Endocrinological Investigation
Volume29
Issue number10
StatePublished - Nov 2006
Externally publishedYes

Fingerprint

Adrenocorticotropic Hormone
Hyperplasia
Receptor, Melanocortin, Type 2
Heart Failure
Hydrocortisone
Aldosterone
Angiotensin II
Cushing Syndrome
Adrenal Glands
Corticotropin Receptors
Renin-Angiotensin System
Serum
Vasopressins
Posture
Catecholamines
Hormones

Keywords

  • ACTH receptor
  • CRF
  • Cushing's syndrome
  • Insulin resistance
  • Taqman

ASJC Scopus subject areas

  • Endocrinology

Cite this

A case of ACTH-independent bilateral macronodular adrenal hyperplasia and severe congestive heart failure. / Suri, D.; Alonso, M.; Weiss, Roy E.

In: Journal of Endocrinological Investigation, Vol. 29, No. 10, 11.2006, p. 940-946.

Research output: Contribution to journalArticle

@article{ebf722934f3b49539b5ca4ea56171b4f,
title = "A case of ACTH-independent bilateral macronodular adrenal hyperplasia and severe congestive heart failure",
abstract = "Cortisol secretion in ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH) can be regulated by aberrant adrenal receptors. We describe a patient with Cushing's syndrome (CS) due to AIMAH and concomitant Class IV congestive heart failure (CHF). Clinical testing for the presence of aberrant receptors revealed a pronounced serum cortisol (257{\%}) and aldosterone response (212{\%}) to the administration of ACTH and a partial serum cortisol (35{\%}) and aldosterone (106{\%}) response to upright posture. This suggested the possible presence of aberrant hormone receptors for ACTH [melanocortin 2 receptor (MC2-R)], vasopressin, catecholamines or angiotensin II (AT-II) on the patient's adrenal glands. Adrenal tissue from the patient demonstrated an eight-fold increased expression of MC2-R compared to normal adrenal tissue. This increased expression was consistent with the increase in cortisol and aldosterone seen in response to exogenous ACTH. We propose that the severe CHF resulted in activation of the renin-angiotensin system, with an increased production of AT-II. The elevated circulating levels of AT-II may have led to increased expression of MC2-R on the patient's adrenal glands and increased responsiveness to ACTH. This unusual case of CS may elucidate a heretofore unknown mechanism for the development of AIMAH.",
keywords = "ACTH receptor, CRF, Cushing's syndrome, Insulin resistance, Taqman",
author = "D. Suri and M. Alonso and Weiss, {Roy E}",
year = "2006",
month = "11",
language = "English (US)",
volume = "29",
pages = "940--946",
journal = "Journal of Endocrinological Investigation",
issn = "0391-4097",
publisher = "Editrice Kurtis s.r.l.",
number = "10",

}

TY - JOUR

T1 - A case of ACTH-independent bilateral macronodular adrenal hyperplasia and severe congestive heart failure

AU - Suri, D.

AU - Alonso, M.

AU - Weiss, Roy E

PY - 2006/11

Y1 - 2006/11

N2 - Cortisol secretion in ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH) can be regulated by aberrant adrenal receptors. We describe a patient with Cushing's syndrome (CS) due to AIMAH and concomitant Class IV congestive heart failure (CHF). Clinical testing for the presence of aberrant receptors revealed a pronounced serum cortisol (257%) and aldosterone response (212%) to the administration of ACTH and a partial serum cortisol (35%) and aldosterone (106%) response to upright posture. This suggested the possible presence of aberrant hormone receptors for ACTH [melanocortin 2 receptor (MC2-R)], vasopressin, catecholamines or angiotensin II (AT-II) on the patient's adrenal glands. Adrenal tissue from the patient demonstrated an eight-fold increased expression of MC2-R compared to normal adrenal tissue. This increased expression was consistent with the increase in cortisol and aldosterone seen in response to exogenous ACTH. We propose that the severe CHF resulted in activation of the renin-angiotensin system, with an increased production of AT-II. The elevated circulating levels of AT-II may have led to increased expression of MC2-R on the patient's adrenal glands and increased responsiveness to ACTH. This unusual case of CS may elucidate a heretofore unknown mechanism for the development of AIMAH.

AB - Cortisol secretion in ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH) can be regulated by aberrant adrenal receptors. We describe a patient with Cushing's syndrome (CS) due to AIMAH and concomitant Class IV congestive heart failure (CHF). Clinical testing for the presence of aberrant receptors revealed a pronounced serum cortisol (257%) and aldosterone response (212%) to the administration of ACTH and a partial serum cortisol (35%) and aldosterone (106%) response to upright posture. This suggested the possible presence of aberrant hormone receptors for ACTH [melanocortin 2 receptor (MC2-R)], vasopressin, catecholamines or angiotensin II (AT-II) on the patient's adrenal glands. Adrenal tissue from the patient demonstrated an eight-fold increased expression of MC2-R compared to normal adrenal tissue. This increased expression was consistent with the increase in cortisol and aldosterone seen in response to exogenous ACTH. We propose that the severe CHF resulted in activation of the renin-angiotensin system, with an increased production of AT-II. The elevated circulating levels of AT-II may have led to increased expression of MC2-R on the patient's adrenal glands and increased responsiveness to ACTH. This unusual case of CS may elucidate a heretofore unknown mechanism for the development of AIMAH.

KW - ACTH receptor

KW - CRF

KW - Cushing's syndrome

KW - Insulin resistance

KW - Taqman

UR - http://www.scopus.com/inward/record.url?scp=33846470141&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33846470141&partnerID=8YFLogxK

M3 - Article

C2 - 17185906

AN - SCOPUS:33846470141

VL - 29

SP - 940

EP - 946

JO - Journal of Endocrinological Investigation

JF - Journal of Endocrinological Investigation

SN - 0391-4097

IS - 10

ER -