scholarly journals Adrenal Cushing’s syndrome during pregnancy

2017 ◽  
Vol 177 (5) ◽  
pp. K13-K20 ◽  
Author(s):  
C E Andreescu ◽  
R A Alwani ◽  
J Hofland ◽  
L H J Looijenga ◽  
W W de Herder ◽  
...  
Keyword(s):  
Luteinizing Hormone ◽  
Cushing’S Syndrome ◽  
Adrenal Tumor ◽  
Cushing Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Receptor Expression ◽  
Aberrant Expression ◽  
Cortisol Responses

Cushing syndrome (CS) during pregnancy is a rare condition with only a few cases reported in the literature. Misdiagnosis of CS is common because of overlapping features like fatigue, weight gain, striae and emotional changes that can occur during normal pregnancy. Changes in maternal hormones and their binding proteins complicate assessment of glucocorticoid hormone levels during gestation. CS during pregnancy is most frequently due to an adrenal adenoma and to a lesser degree to adrenocorticotropic hormone (ACTH) hypersecretion by a pituitary adenoma. Furthermore, aberrant expression of luteinizing hormone (LH) receptors in the adrenal cortex has been suggested to be involved in the pathogenesis of adrenal CS during pregnancy. We report three pregnant women with ACTH-independent Cushing’s syndrome and an adrenal tumor. After uncomplicated delivery, patient 1 underwent in vivo testing for aberrant hormone receptor expression by the adenoma. Cortisol responses were found after administration of luteinizing hormone-releasing hormone (LHRH), human chorionic gonadotropin (hCG), glucagon, vasopressin and a standard mixed meal. All patients were treated with laparoscopic adrenalectomy. Adrenal tumor tissue of two patients showed positive immunohistochemical staining of LH receptors. Considering the cortisol responses to LHRH and hCG, and the development of CS during pregnancy in these patients, it is likely that ACTH-independent hypercortisolism was induced by the pregnancy-associated rise in hCG levels that activated aberrantly expressed LH receptors in the adrenal adenoma. Remarkably, adrenal adenomas may simultaneously express multiple aberrant receptors and individual ligands may play a role in the regulation of cortisol production in CS during pregnancy.

scholarly journals Aberrant cortisol regulations in bilateral macronodular adrenal hyperplasia: a frequent finding in a prospective study of 32 patients with overt or subclinical Cushing's syndrome

2010 ◽  
Vol 163 (1) ◽  
pp. 129-138 ◽  
Author(s):  
Rossella Libé ◽  
Joël Coste ◽  
Laurence Guignat ◽  
Frédérique Tissier ◽  
Hervé Lefebvre ◽  
...  
Keyword(s):  
Functional Status ◽  
Cushing’S Syndrome ◽  
Hormone Receptors ◽  
Cushing's Syndrome ◽  
Receptor Expression ◽  
Adrenal Hyperplasia ◽  
Cortisol Responses ◽  
A Prospective Study ◽  
Underlying Pathophysiology

ContextACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare and heterogeneous condition characterized by abnormal steroid production. Cortisol secretion can be regulated by aberrant hormone receptors.ObjectiveA large series of patients with AIMAH were evaluated to provide information on the prevalence and profile of aberrant regulations, in relation with the functional status.Design and patientsThirty-two consecutive patients with AIMAH were prospectively studied: 10 had a Cushing's syndrome (CS), and 22 had a subclinical CS (SCS).MethodsA baseline endocrine evaluation was followed by an in vivo protocol in search of aberrant cortisol responses (seven provocative tests). An acute inhibition test with the somatostatin analog octreotide was also performed.ResultsAt least one aberrant cortisol response was identified in 28 of 32 (87%) patients. The overall prevalence of aberrant responses was independent of the functional status. Responses to the upright posture and to metoclopramide were frequently observed (67 and 56% respectively). A glucagon response was frequently observed in the SCS group (58%). A cortisol inhibition by octreotide was specifically found in the three CS patients who positively responded to the mixed meal, and was observed also in 12 of 13 (92%) patients with SCS.ConclusionsCortisol responses indicative of aberrant receptor expression were highly prevalent in AIMAH. Thorough phenotyping of AIMAH may help uncover the underlying pathophysiology.

A study of hypothalamic-pituitary-adrenal suppression following curative surgery for Cushing's syndrome due to adrenal adenoma

1987 ◽  
Vol 114 (2) ◽  
pp. 166-170 ◽  
Author(s):  
D. Gordon ◽  
C. G. Semple ◽  
G. H. Beastall ◽  
J. A. Thomson
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Hypothalamic Pituitary Adrenal Axis ◽  
Curative Surgery ◽  
Unilateral Adrenalectomy ◽  
Hypothalamic Pituitary Adrenal ◽  
Adrenal Axis ◽  
Cortisol Responses ◽  
Pituitary Adrenal Axis

Abstract. The hypothalamic-pituitary-adrenal axis was investigated in all six patients requiring glucocorticoid replacement 2.5–11 years after unilateral adrenalectomy for adrenal adenomas causing Cushing's syndrome. The hypothalamic-pituitary-adrenal axis was assessed by insulin induced hypoglycaemia and CRF testing in each patient. Two patients showed normal cortisol and ACTH responses to hypoglycaemia. Two patients showed subnormal cortisol responses to hypoglycaemia in the presence of high or normal basal ACTH concentrations. ACTH concentrations increased with both hypoglycaemia and CRF. Two patients showed subnormal cortisol responses to hypoglycaemia and CRF. One of these patients showed an ACTH rise following hypoglycaemia but not CRF. Defects at either hypothalamic-pituitary or adrenal levels were demonstrated and recovery of the axis appears to commence at the hypothalamic-pituitary level.

scholarly journals Cushing syndrome secondary to late onset adrenal hyperplasia: presentation and challenges of management

2018 ◽  
Vol 1 (1) ◽  
Author(s):  
Muzzammil Abdullahi ◽  
Mamuda Atiku ◽  
Imam Mohammed Ibrahim
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Late Onset ◽  
Adrenal Adenoma ◽  
Protein Structures ◽  
Cushing's Syndrome ◽  
Response To Treatment ◽  
Adrenal Tumors ◽  
Adrenal Hyperplasia ◽  
Supraclavicular Fossa

Cushing’s syndrome is a clinical disorder caused by overproduction of cortisol. Adrenal adenoma is the cause in 5% of cases of Cushing syndrome. ACTH-independent Cushing’s syndrome in 90% is caused by unilateral adrenal tumors. Of these, adenomas are the cause in 80% of the cases, while the others are adrenocortical carcinoma. Rare causes of Cushing’s syndrome include adrenal hyperplasia. Overproduction of cortisol results in weakened protein structures leading to protuberant abdomen and poor wound healing, glucose is converted to fat and deposited in the abdomen, supraclavicular fossa and cheeks. Other presentations are diabetes, hypertension, osteoporosis, fractures, impaired immune function, glucose intolerance, and psychosis. We report a 21-year-old female who presented with clinical symptoms of newly diagnosed hypertension and diabetes mellitus which after poor response to treatment she was evaluated and diagnosed to have Cushing syndrome. She subsequently had left adrenalectomy and did very well with resolution of patients’ symptoms. The histology came out to be diffuse adrenal hyperplasia.

Incidentally discovered ACTH-dependent adrenal adenoma presenting as 'Pre-Cushing's syndrome'

1986 ◽  
Vol 111 (1) ◽  
pp. 89-92 ◽  
Author(s):  
U. Bogner ◽  
U. Eggens ◽  
J. Hensen ◽  
W. Oelkers
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenal Mass ◽  
Adrenal Adenoma ◽  
Clinical Signs ◽  
Cushing's Syndrome ◽  
Cortisol Secretion ◽  
Adrenal Tumour ◽  
Urinary Cortisol

Abstract. An adrenal tumour was incidentally discovered with no clinical signs of Cushing's syndrome. The endocrine evaluation revealed the unique hormonal constellation of an increased urinary cortisol excretion rate, unequivocal suppressibility of plasma and urinary cortisol by dexamethasone, but only to a residual level in the low normal range which probably reflected ACTH-independent 'autonomous' cortisol secretion. After removal of the adrenal mass, urinary cortisol secretion and dexamethasone suppressibility were normalized. In vitro, the tumour cells were as sensitive towards ACTH as 'normal' human adrenal cells, but showed a reduced cortisol production rate per cell. We suppose that the adrenal mass participated in the diurnal rhythm of ACTH-mediated cortisol secretion in vivo, which resulted in an increased cortisol secretion. During the night, when ACTH levels were low, the cortisol production decreased and the hormone levels were probably too low to suppress ACTH. We regard the hormonal findings in our patients as 'Pre-Cushing's syndrome', although the absence of clinical features of Cushing's syndrome remains unclear. We suggest that every patient with an incidentally discovered adrenal mass should have an endocrinological evaluation because the results may help to decide whether or not the adrenal tumour should be removed.

scholarly journals Unilateral nodular adrenocortical hyperplasia presenting as Cushing’s syndrome: a case report

2021 ◽  
Vol 12 (1) ◽  
pp. 78-82
Author(s):  
Lubna Naznin ◽  
Susane Giti ◽  
SK Md Jaynul Islam ◽  
Khandaker Rokshana Akhter ◽  
Shamoli Yasmin ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Histopathological Examination ◽  
Case Reports ◽  
Adrenal Adenoma ◽  
Final Diagnosis ◽  
Cushing's Syndrome ◽  
Uncontrolled Hypertension ◽  
Secondary Hypothyroidism ◽  
Adrenocortical Hyperplasia

Cushing syndrome results from chronic exposure to excess cortisol. Nodular adrenal hyperplasia is usually bilateral and has only a few case reports of unilateral presentation. Biochemically it is presented as hyperaldosteronism or as Cushing’s syndrome. Here, we are reporting a 17-year-old female presenting with weight gain over 5 months and uncontrolled hypertension. Biochemically she was found to have diabetes mellitus, secondary hypothyroidism and hypogonadotrophic hypogonadism due to Cushing’s syndrome of adrenal origin. Unilateral adrenal adenoma/hyperplasia in right adrenal gland was evident by radiology. Histopathological examination was done after laparoscopic adrenalectomy showed nodular adrenocortical hyperplasia in right adrenal mass. Following surgery, clinical features of the patient improved notably. Cushing syndrome due to unilateral nodular adrenocortical hyperplasia is a rare entity. Biochemical evaluation of hypothalamo-pituitary-adrenal axis, radiological evidence and histopathology are the important armaments can guide to final diagnosis. BIRDEM Med J 2022; 12(1): 78-82

Adrenocortical adenoma in a six-year-old Egyptian boy

2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Eman B Kamaleldeen ◽  
◽  
Shimaa Kamal Mohamed ◽  
Kotb Abbass Metwalley ◽  
Hamdy M Ibrahim ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Histopathological Examination ◽  
Adrenal Adenoma ◽  
Time Lag ◽  
Laboratory Data ◽  
Clinical Signs ◽  
Cushing's Syndrome ◽  
Additional Treatment ◽  
Adrenocortical Adenoma

Although adrenal cortical tumors are rare in children, pediatricians should be very cautious about it. Neoplasm is a rare but significant cause of Cushing’s syndrome. Unfortunately, they can be missed very easily, as there is often a time lag between the onset of the symptoms and final diagnosis. Here, we present a case of adrenal adenoma in a six-year - old Egyptian boy who regrettably misdiagnosed twice as exogenous Cushing syndrome and simple obesity. Diagnosis of an adrenal cortical tumor was confirmed by laboratory data, MRI and histopathological examination after more than 2 years of the onset of his symptoms. After resection of the tumor, serum steroids normalized, and clinical signs receded. The child received no additional treatment and remains disease-free after 18 months of close observation. The possibility of neoplasms should always be considered early to avoid delayed diagnosis and treatment of Cushing’s syndrome.

scholarly journals Aberrant expression of multiple hormone receptors in ACTH-independent macronodular adrenal hyperplasia causing Cushing's syndrome

2010 ◽  
Vol 163 (2) ◽  
pp. 293-299 ◽  
Author(s):  
J W B de Groot ◽  
T P Links ◽  
A P N Themmen ◽  
L H Looijenga ◽  
R R de Krijger ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Hormone Receptors ◽  
Immunohistochemical Analysis ◽  
Cushing's Syndrome ◽  
Adrenal Hyperplasia ◽  
Aberrant Expression ◽  
Adrenal Tissue ◽  
The Individual

ObjectiveAberrant adrenal expression of various hormone receptors has been identified in ACTH-independent macronodular adrenal hyperplasia (AIMAH) causing cortisol hypersecretion regulated by hormones other than ACTH. We aimed to determine aberrant expression of multiple hormone receptors in vivo and in vitro in adrenal tissue of a patient with AIMAH.DesignThe design of the study includes clinical case description, and biochemical and immunohistochemical analysis to demonstrate aberrant expression of multiple hormone receptors in AIMAH.MethodsThe subject of the study is a male diagnosed with Cushing's syndrome because of AIMAH. Directly after laparoscopic removal of the adrenals, adrenal tissue was incubated with and without test substances (ACTH, forskolin, arginine vasopressin (AVP), desmopressin, epinephrine, norepinephrine, purified human chorionic gonadotropin (hCG), metoclopramide and the combinations of AVP with ACTH, epinephrine and metoclopramide). LH/hCG-receptor (hCG-R) immunohistochemistry and RT-PCR analyses were performed to demonstrate aberrant expression of LH/hCG-R and V1–3-AVPR.ResultsAIMAH was characterized by in vivo cortisol responsiveness to AVP and in vitro cortisol responses to AVP, hCG, epinephrine, and norepinephrine suggesting aberrant adrenal expression of the receptors for AVP (the V1–3-AVPRs), catecholamines (the β-AR), and LH (the LH/hCG-R). Incubation with combinations of AVP and ACTH and of AVP with epinephrine induced a stronger cortisol response compared with incubation with the individual agents. Moreover, we demonstrated adrenal V1–3-AVPR and LH/hCG-R expression.ConclusionsAIMAH tissue may simultaneously express multiple aberrant hormone receptors, and individual ligands may potentiate each other regarding cell proliferation and cortisol production.

scholarly journals Long term control of hypercortisolism with fluconazole: case report and in vitro studies

2006 ◽  
Vol 154 (4) ◽  
pp. 519-524 ◽  
Author(s):  
Michaela Riedl ◽  
Christina Maier ◽  
Georg Zettinig ◽  
Peter Nowotny ◽  
Wolfgang Schima ◽  
...  
Keyword(s):  
Case Report ◽  
Cell Line ◽  
Cushing’S Syndrome ◽  
Adrenal Adenoma ◽  
Cushing's Syndrome ◽  
Unilateral Adrenalectomy ◽  
Cortisol Excretion ◽  
Glucocorticoid Production

Objectives: To evaluate the efficacy of fluconazole as an alternative treatment for controlling hypercortisolism in Cushing’s syndrome and to determine its effect on glucocorticoid production in vitro. Design: Case report and in vitro study in a University Clinic. Case: An 83 year old patient presented with recurrence of Cushing’s syndrome due to pulmonary metastases three years after unilateral adrenalectomy. During a near fatal episode of sepsis she was started on fluconazole 200 mg/day intravenously which normalised cortisol excretion. The therapy was continued orally for 18 months. Upon temporary discontinuation and reintroduction of treatment, cortisol levels increased and normalized, respectively. At month 16, fluconazole had to be increased to a dose of 400 mg/day to keep cortisol excretion in the normal range. Disease progression was slow and no side effects occurred. In vitro results: Fluconazole in a concentration of 500 μM nearly abolished corticosterone production over 24 h from the adrenal adenoma cell line Y-1 (8.6 ± 0.5% compared with control, P < 0.0001) and significantly reduced corticosterone production in concentrations of 50 μM (48.3 ± 1.9% vs. control, P < 0.0001) and 5 μM (80.5 ± 8.5% vs. control, P < 0.05). Conclusion: These results demonstrate for the first time that fluconazole normalises cortisol concentrations in vivo in a patient with Cushing’s syndrome with adrenal carcinoma and inhibit glucocorticoid production in vitro in a cell line. Thus, fluconazole might be useful in controlling glucocorticoid excess in Cushing’s syndrome and because of its lower toxicity might be preferable to ketoconazole.

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