A case of cyclical Cushing's disease associated with corticosteroidbinding globulin deficiency: a rare pitfall in the diagnosis of Cushing's disease

1995 ◽  
Vol 133 (3) ◽  
pp. 317-319 ◽  
Author(s):  
Hajime Watanobe ◽  
Takeshi Nigawara ◽  
Ryo Nasushita ◽  
Shinsuke Sasaki ◽  
Kazuo Takebe
Keyword(s):  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Plasma Acth ◽  
School Of Medicine ◽  
Corticosteroid Binding Globulin ◽  
High Urinary Excretion ◽  
Cortisol Levels ◽  
Cyclical Cushing’S Disease ◽  
Free Plasma ◽  
Periodic Secretion

Watanobe H, Nigawara T, Nasushita R, Sasaki S, Takebe K. A case of cyclical Cushing's disease associated with corticosteroid-binding globulin deficiency: a rare pitfall in the diagnosis of Cushing's disease. Eur J Endocrinol 1995;133:317–9. ISSN 0804–4643 We experienced an extremely unusual combination of Cushing's disease and corticosteroid-binding globulin (CBG) deficiency that has been reported in only one similar case to date. A 53-year-old woman presented at a medical clinic with clinical Cushing's disease. However, her plasma levels of adrenocorticotropin (ACTH) and cortisol were in the normal range. Six months later, during a second visit, a high urinary excretion of 17-hydroxycorticosteroids was found, but plasma ACTH and cortisol levels were normal again. Further investigation revealed a decreased CBG concentration. Free plasma cortisol levels were clearly elevated. Furthermore, the Cushing's disease of our patient was complicated by periodic secretion of ACTH and cortisol, with high or normal outputs of corticosteroids occurring alternately every 1–3 days, which explained the occasionally normal plasma ACTH and cortisol levels. A combination of a decreased serum CBG concentration and periodic secretion of ACTH can be an important pitfall in the diagnosis of Cushing's disease. Hajime Watanobe, Third Department of Internal Medicine, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki, Aomori 036, Japan

Vasoactive intestinal polypeptide enhances ACTH levels in some patients with adrenocorticotropin - secreting pituitary adenomas

1987 ◽  
Vol 116 (2) ◽  
pp. 216-220 ◽  
Author(s):  
Bruno Ambrosi ◽  
Domenico Bochicchio ◽  
Alessandro Sartorio ◽  
Francesco Morabito ◽  
Giovanni Faglia
Keyword(s):  
Vasoactive Intestinal Polypeptide ◽  
Cushing’S Disease ◽  
Normal Subjects ◽  
Significant Rise ◽  
Cushing's Disease ◽  
Plasma Acth ◽  
Nelson’S Syndrome ◽  
Nelson's Syndrome ◽  
Cortisol Levels ◽  
Intestinal Polypeptide

Abstract. Vasoactive intestinal polypeptide (VIP) was administered (75 μg iv over 12 min) to 14 patients with Cushing's disease, 1 patient with Nelson's syndrome, and 8 normal subjects. VIP induced a significant rise of plasma ACTH levels in 6 patients with Cushing's disease, from a baseline of 13.2 pmol/l (9.9–18.5 pmol/l) to a peak of 24.5 pmol/l (7.7–18.9 pmol/l), median and range (P < 0.05), and in the patient with Nelson's syndrome, from a baseline of 260.9 to 461.3 pmol/l. A significant elevation of cortisol levels was also observed, from a baseline of 567 nmol/l (185–842 nmol/l) to a peak of 727 nmol/l (364–1029 nmol/l); P < 0.05. No modifications in plasma ACTH and cortisol levels were noticed in the other 8 patients with Cushing's disease, or in the normal subjects. In the responsive patients, the median plasma ACTH level reached after VIP was found to be less than that induced by CRH administration. In 2 of the responsive patients, VIP was injected again after successful microadenomectomy and did not then cause changes in ACTH and cortisol concentration. These data demonstrate that VIP specifically stimulates ACTH release in some patients with corticotropinomas but not in normal subjects; the disappearance of such abnormal ACTH responses after successful adenomectomy suggests the presence of specific VIP receptors only on the adenomatous corticotropes.

Effect of ovine corticotrophin releasing factor, bromocriptine, and dopamine on release of ACTH and β-endorphin in a patient with Cushing's disease

1985 ◽  
Vol 109 (1) ◽  
pp. 7-12 ◽  
Author(s):  
Hiroko Nakashima ◽  
Yukio Hirata ◽  
Masahito Uchihashi ◽  
Masahiro Tomita ◽  
Takuo Fujita
Keyword(s):  
Pituitary Adenoma ◽  
Cushing’S Disease ◽  
Plasma Cortisol ◽  
Cushing's Disease ◽  
Intermediate Lobe ◽  
Plasma Acth ◽  
Corticotrophin Releasing Factor ◽  
Predominant Component ◽  
Cortisol Levels

Abstract. Release of immunoreactive ACTH and β-endorphin (β-EP) in response to corticotrophin-releasing factor (CRF) and dopaminergic agents was studied in vivo and in vitro in a patient with Cushing's disease. Iv administration of synthetic ovine (o) CRF significantly stimulated plasma ACTH release, accompanied by increase of plasma cortisol levels. Oral administration of bromocriptine significantly suppressed plasma cortisol levels. Although reduced responses of plasma ACTH and cortisol to o-CRF was observed 1 month after removal of the pituitary adenoma, these normalized 6 months after operation. In vitro perifusion of the pituitary adenoma obtained by surgery revealed that o-CRF also stimulated ACTH and β-EP release in a dose-responsive manner (10−9m–10−5 m) and that dopamine suppressed their basal secretion. Gel exclusion chromatography of the perfusates showed that the predominant component of ACTH and β-EP before and after o-CRF stimulation coeluted with standard ACTH and β-EP, respectively. The present data suggest that o-CRF is a potent secretagogue for ACTH and β-EP release from the human pituitary adenoma causing Cushing's disease and that ACTH secretion from certain adenomas, possibly originating from the intermediate lobe of the pituitary gland, is partly regulated by a dopaminergic mechanism.

Pediatric cyclical Cushing’s disease: a case report and literature review

2020 ◽  
Vol 0 (1) ◽  
pp. 47-56
Author(s):  
A. V. Solntsava ◽  
N. V. Volkava ◽  
K. A. Beliayeva ◽  
V. A. Zhurauliou
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Cyclical Cushing’S Disease

scholarly journals Dynamics of Serum Cortisol Levels after Transsphenoidal Surgery in a Cohort of Patients with Cushing’s Disease

2004 ◽  
Vol 89 (3) ◽  
pp. 1131-1139 ◽  
Author(s):  
G. A. F. S. Rollin ◽  
N. P. Ferreira ◽  
M. Junges ◽  
J. L. Gross ◽  
M. A. Czepielewski
Keyword(s):  
Cushing’S Disease ◽  
Transsphenoidal Surgery ◽  
Serum Cortisol ◽  
Cushing's Disease ◽  
Cortisol Levels

How early can one diagnose Cushing’s disease? An early diagnosis in a case of prepubertal Cushing’s disease

2014 ◽  
Vol 27 (11-12) ◽  
pp. 1043-1047 ◽  
Author(s):  
Julia Hoppmann ◽  
Isabel V. Wagner ◽  
Gudrun Junghans ◽  
Stefan A. Wudy ◽  
Michael Buchfelder ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Cushing’S Disease ◽  
Dexamethasone Suppression Test ◽  
Cushing's Disease ◽  
High Dose ◽  
Thyroid Function Tests ◽  
Free Cortisol ◽  
Suppression Test ◽  
Cortisol Levels ◽  
Dexamethasone Suppression

Abstract Background: Cushing’s disease is very rare in children, and the diagnosis is frequently delayed by several years. Objective: We report a case of prepubertal Cushing’s disease with a medical history of only 9 months. This case illustrates the difficulties involved in diagnosing children at the early stage of the disease. Case presentation: An 8-year-old prepubertal boy presented with rapid weight gain accompanied by a decreasing growth velocity and hirsutism. Thyroid function tests and growth factor levels were normal, thus excluding hypothyroidism and growth hormone deficiency. Cushing’s syndrome was confirmed by elevated 24-h urinary free cortisol levels, increased diurnal cortisol levels, and a lack of cortisol suppression in the low-dose dexamethasone suppression test. Further tests to investigate the source of the hypercortisolism showed the following results: Basal morning adrenocorticotropic hormone (ACTH) was normal. The high-dose dexamethasone suppression test led to a 51% decrease in cortisol level. In the corticotropin-releasing hormone (CRH) test, ACTH and cortisol increased only by 28%. Repeated magnetic resonance imaging (MRI) finally revealed a microadenoma in the anterior pituitary, thus establishng the diagnosis of Cushing’s disease. Upon diagnosis, the patient underwent transsphenoidal surgery. Histological analysis confirmed an ACTH-secreting pituitary adenoma. Conclusion: This case illustrates the difficulties associated with the clinical, biochemical, and radiological diagnoses of Cushing’s disease in children. Early diagnosis remains a challenge because test results often do not match standard diagnostic criteria.

scholarly journals SAT-266 Accelerated Osteoporosis - a Rare Presentation of Cushing’s Disease

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Vijaykumar Sekar ◽  
Joseph Hughes ◽  
Sugam Gouli
Keyword(s):  
Cushing’S Disease ◽  
Low Dose ◽  
Incidental Finding ◽  
Serum Cortisol ◽  
Cushing's Disease ◽  
Adrenal Hyperplasia ◽  
Bilateral Adrenal Hyperplasia ◽  
Cortisol Levels ◽  
Post Menopausal ◽  
Dexamethasone Suppression

Abstract Background: Osteoporosis in post-menopausal women is usually due to bone loss from estrogen deficiency and/or age. Secondary osteoporosis (SO) is less common. Up to 30% of postmenopausal women and 50% of men with osteoporosis may have an underlying cause. Cushing’s disease (CD) is one cause of SO but rarely the presenting symptoms. The prevalence of osteoporosis (69.6% vs 37.8%) is significantly higher in patients with adrenal rather than pituitary CD. Diagnosing CD remains a challenge to physicians in spite of advances in diagnostic techniques. We report a case of CD in a post-menopausal woman presenting as accelerated osteoporosis. Clinical Case A 63-year-old Caucasian female with a history of hypertension and hysterectomy in her 50s on transdermal estrogen was referred to our Endocrine clinic for evaluation of osteoporosis and incidental finding of bilateral adrenal hyperplasia on CT spine. The patient rapidly developed kyphoscoliosis within the past 2 years. She was debilitated by pain and decreased mobility from compression fractures and spinal stenosis, and underwent thoracic and lumbar fusion surgery. On physical examination, her heart rate was 64 beats per minute, blood pressure 130/92 mmHg, weight 188 lbs. and Height 5.1 ft; a year ago it was 5.5 ft. Her face appeared round but not red. Buffalo hump and supraclavicular pad were noted. No striae or bruises noted. Healing surgical scars over the thoracic and lumbar spine were violaceous. The patient’s urine free cortisol levels, tested a month apart, were 190 mcg and 132 mcg (n 3.5-45 mcg/24h). Midnight salivary cortisol levels taken consecutive nights were 160 ng/dL and 513 ng/dL (n &lt; 100 ng/dL). Morning Serum Cortisol and ACTH were 20.2 ug/dL and 14 pg/mL (n Cortisol 6.0-27.0 ug/dL and ACTH 7.2-63 pg/mL). Following low dose (1mg) dexamethasone suppression testing, her serum cortisol were 12.6 ug/dL, ACTH levels were 32 pg/mL and dexamethasone were 187 ng/dL (n &lt; 30 ng/dL). 25-Hydroxy D total, TSH, Free T4, PTH intact, calcium, renin, aldosterone and SPEP levels were normal. Renal and liver functions were normal except alkaline phosphatase was 142 U/L (n 34-104 U/L). Pituitary MRI with contrast showed pituitary microadenoma. The patient was referred to a neurosurgeon and is planned for an inferior petrosal sinus sampling prior to transsphenoidal resection. Conclusion Cushing’s disease as a cause of osteoporosis is rare. Diagnosis of Cushing’s can be challenging in patients without obvious signs, as in our patient who was referred to an endocrinologist due to incidental finding of bilateral adrenal hyperplasia. A high degree of clinical suspicion is needed when investigating CD, as initial test results can be indecisive. As in our patient, initial ACTH and cortisol levels were normal, the low dose dexamethasone suppression test helped us direct our diagnosis towards CD.

Cyclical Cushing's Disease: Two Distinct Rhythms in a Patient with a Basophil Adenoma

1985 ◽  
Vol 60 (2) ◽  
pp. 328-332 ◽  
Author(s):  
A.B. ATKINSON ◽  
A. CHESTNUTT ◽  
E. CROTHERS ◽  
R. WOODS ◽  
J.A. WEAVER ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Cyclical Cushing’S Disease

scholarly journals A putative role for the aryl hydrocarbon receptor (AHR) gene in a patient with cyclical Cushing’s disease

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Sunita M. C. De Sousa ◽  
Jim Manavis ◽  
Jinghua Feng ◽  
Paul Wang ◽  
Andreas W. Schreiber ◽  
...  
Keyword(s):  
Aryl Hydrocarbon Receptor ◽  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Aryl Hydrocarbon ◽  
Putative Role ◽  
Ahr Gene ◽  
Cyclical Cushing’S Disease

Octreotide exerts different effects in vivo and in vitro in Cushing's disease

1994 ◽  
Vol 130 (2) ◽  
pp. 125-131 ◽  
Author(s):  
Günter K Stalla ◽  
Steffi J Brockmeier ◽  
Ulrich Renner ◽  
Chris Newton ◽  
Michael Buchfelder ◽  
...  
Keyword(s):  
Cell Cultures ◽  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Dependent Manner ◽  
Acth Secretion ◽  
Adenoma Cell ◽  
Cortisol Levels ◽  
Corticotropic Adenoma

Stalla GK, Brockmeier SJ, Renner U, Newton C, Buchfelder M, Stalla J, Müller OA. Octreotide exerts different effects in vivo and in vitro in Cushing's disease. Eur J Endocrinol 1994;130:125–31. ISSN 0804–4643 The effect of the long-acting somatostatin analog octreotide (SMS 201-995) on adrenocorticotropin (ACTH) secretion was studied in five patients with untreated Cushing's disease in vivo and in six human corticotropic adenoma cell cultures in vitro. For the in vivo study, 100 μg of octreotide sc was given 30 and 180 min after cannulation of the cubital vein and 100 μg of corticotropin-releasing hormone (CRH) was injected iv at 210 min. Serum ACTH and cortisol levels were measured for 390 min. In vivo, octreotide had no significant effect either on basal or CRH-stimulated ACTH levels and did not influence cortisol levels. The in vitro studies were conducted with corticotropic adenoma cell cultures derived from adenoma tissue obtained from six patients with Cushing's disease. In four of six cell cultures, octreotide (1 nmol/l–1 μmol/l) inhibited basal ACTH secretion in a dose-dependent manner. The inhibition ranged from 70 to 92% for 1 nmol/l octreotide to 14–46% for 1 μmol/l octreotide as compared to controls (100%). In three of three octreotide-responsive adenoma cell cultures investigated, CRH-stimulated ACTH secretion was suppressed by octreotide. Hydrocortisone pretreatment in vitro abolished the inhibitory effect of octreotide on ACTH secretion in one octreotide-responsive corticotropic adenoma cell culture. In conclusion, we showed that octreotide in most cases could inhibit the ACTH release from human corticotropic adenoma cells in vitro but had no suppressive effect on ACTH levels of patients with Cushing's disease in vivo. This discrepancy could be due to a somatostatin receptor down-regulation by cortisol at the hypercortisolemic state in vivo. Günter K Stalla, Max-Planck-Institute of Psychiatry, Clinical Institute, Kraepelinstr. 10, D-80804 Munich, Germany

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