Abstract
Objective: ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare and unusual cause of Cushing's syndrome, characterized by bilateral nodular adrenocortical hyperplasia and hypersecretion of cortisol. Familial AIMAH has rarely been reported. Recently, the aberrant expression of adrenal receptors for various ligands in AIMAH patients has become important in explaining the pathogenesis of AIMAH. In this study, we present the cases of two sisters who were affected with AIMAH. Patients and Measurements: Two sisters, aged 46 and 58, respectively, at the time of diagnosis, were found to have Cushing's syndrome due to AIMAH. We performed provocation tests with various materials and reverse transcription polymerase chain reaction (RT-PCR) with their resected adrenal tissues to examine the hyperresponsiveness to steroidogenesis and the overexpression of related receptors, respectively. Results: Provocation tests in vivo indicated that arginine vasopressin (AVP) promoted cortisol secretion through vasopressin V1a as well as V1b and V2 receptors. RT-PCR analysis revealed an abnormal cDNA expression of vasopressin V1b and V2 receptors, none of which is known to be normally expressed in the adrenal glands. Conclusion: The expression of ectopic vasopressin V1b and V2 receptors may be involved in the aetiology of AIMAH, at least in the case of the sibling patients presented here.
| Original language | English |
|---|---|
| Pages (from-to) | 625-630 |
| Number of pages | 6 |
| Journal | Clinical Endocrinology |
| Volume | 63 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - 2005 Dec 1 |
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All Science Journal Classification (ASJC) codes
- Endocrinology, Diabetes and Metabolism
- Endocrinology
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Ectopic expression of vasopressin V1b and V2 receptors in the adrenal glands of familial ACTH-independent macronodular adrenal hyperplasia. / Lee, Sihoon; Hwang, Ranjoo; Lee, Junho; Rhee, Yumie; Kim, Dae Jung; Chung, Ung Il; Lim, Sungkil.
In: Clinical Endocrinology, Vol. 63, No. 6, 01.12.2005, p. 625-630.Research output: Contribution to journal › Article
TY - JOUR
T1 - Ectopic expression of vasopressin V1b and V2 receptors in the adrenal glands of familial ACTH-independent macronodular adrenal hyperplasia
AU - Lee, Sihoon
AU - Hwang, Ranjoo
AU - Lee, Junho
AU - Rhee, Yumie
AU - Kim, Dae Jung
AU - Chung, Ung Il
AU - Lim, Sungkil
PY - 2005/12/1
Y1 - 2005/12/1
N2 - Objective: ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare and unusual cause of Cushing's syndrome, characterized by bilateral nodular adrenocortical hyperplasia and hypersecretion of cortisol. Familial AIMAH has rarely been reported. Recently, the aberrant expression of adrenal receptors for various ligands in AIMAH patients has become important in explaining the pathogenesis of AIMAH. In this study, we present the cases of two sisters who were affected with AIMAH. Patients and Measurements: Two sisters, aged 46 and 58, respectively, at the time of diagnosis, were found to have Cushing's syndrome due to AIMAH. We performed provocation tests with various materials and reverse transcription polymerase chain reaction (RT-PCR) with their resected adrenal tissues to examine the hyperresponsiveness to steroidogenesis and the overexpression of related receptors, respectively. Results: Provocation tests in vivo indicated that arginine vasopressin (AVP) promoted cortisol secretion through vasopressin V1a as well as V1b and V2 receptors. RT-PCR analysis revealed an abnormal cDNA expression of vasopressin V1b and V2 receptors, none of which is known to be normally expressed in the adrenal glands. Conclusion: The expression of ectopic vasopressin V1b and V2 receptors may be involved in the aetiology of AIMAH, at least in the case of the sibling patients presented here.
AB - Objective: ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare and unusual cause of Cushing's syndrome, characterized by bilateral nodular adrenocortical hyperplasia and hypersecretion of cortisol. Familial AIMAH has rarely been reported. Recently, the aberrant expression of adrenal receptors for various ligands in AIMAH patients has become important in explaining the pathogenesis of AIMAH. In this study, we present the cases of two sisters who were affected with AIMAH. Patients and Measurements: Two sisters, aged 46 and 58, respectively, at the time of diagnosis, were found to have Cushing's syndrome due to AIMAH. We performed provocation tests with various materials and reverse transcription polymerase chain reaction (RT-PCR) with their resected adrenal tissues to examine the hyperresponsiveness to steroidogenesis and the overexpression of related receptors, respectively. Results: Provocation tests in vivo indicated that arginine vasopressin (AVP) promoted cortisol secretion through vasopressin V1a as well as V1b and V2 receptors. RT-PCR analysis revealed an abnormal cDNA expression of vasopressin V1b and V2 receptors, none of which is known to be normally expressed in the adrenal glands. Conclusion: The expression of ectopic vasopressin V1b and V2 receptors may be involved in the aetiology of AIMAH, at least in the case of the sibling patients presented here.
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UR - http://www.scopus.com/inward/citedby.url?scp=28844465395&partnerID=8YFLogxK
U2 - 10.1111/j.1365-2265.2005.02387.x
DO - 10.1111/j.1365-2265.2005.02387.x
M3 - Article
C2 - 16343095
AN - SCOPUS:28844465395
VL - 63
SP - 625
EP - 630
JO - Clinical Endocrinology
JF - Clinical Endocrinology
SN - 0300-0664
IS - 6
ER -