BIOSYNTHESIS OF CORTICOIDS AND ANDROGENS IN AN ADENOMA FROM A CUSHING'S SYNDROME PATIENT

1963 ◽  
Vol 42 (2) ◽  
pp. 187-194
Author(s):  
Menek Goldstein ◽  
Marcel Gut ◽  
Ralph I. Dorfman ◽  
Louis J. Soffer ◽  
J. Lester Gabrilove

ABSTRACT Incubation of cholesterol-4-14C and pregnenolone-7-3H with a homogenate of adenomatous adrenal tissue from a patient with Cushing's disease yielded 14C and 3H labelled, cortisol, cortisone, 11-deoxycortisol, deoxycorticosterone, dehydroepiandrosterone, and 11β-hydroxy-androst-4-3,17-dione.

1995 ◽  
Vol 41 (1) ◽  
pp. 23-24
Author(s):  
S. Yu. Serpuhovitin ◽  
Ye. I. Marova ◽  
E. N. Bazarova ◽  
V. G. Mamaeva ◽  
I. V. Panteleyev

It is known a surgeon operating on the adrenal glands, should be aware of the possibility of the presence of ectopic adrenal tissue. According to the literature, there are relapses of Cushing's disease due to hyperfunction of adrenal accessory tissue. Its location may be the perinephric tissue, testicles, mediastinum, kidneys, urinary tract, broad ligament of the uterus, vagina, scrotum. The inclusion of adrenal tissue in the ovaries, liver, pancreas is also described. There is some evidence that additional adrenal tissue can be a source of tumors, including hormonal-active ones. O. I. Nikolaev (1970) cites a case of ectopia of a tumor in the kidney, 4 cases of ectopia in one of the ovaries, which, in addition to symptoms of hypercorticism, was accompanied by severe virilization. However, this pathology is extremely rare, therefore, there are few publications in the literature. In this regard, it seems appropriate to us to make such an observation.


2015 ◽  
Vol 38 (2) ◽  
pp. E4 ◽  
Author(s):  
Vivek Bansal ◽  
Nadine El Asmar ◽  
Warren R. Selman ◽  
Baha M. Arafah

Despite many recent advances, the management of patients with Cushing's disease continues to be challenging. Cushing's syndrome is a complex metabolic disorder that is a result of excess glucocorticoids. Excluding the exogenous causes, adrenocorticotropic hormone–secreting pituitary adenomas account for nearly 70% of all cases of Cushing's syndrome. The suspicion, diagnosis, and differential diagnosis require a logical systematic approach with attention paid to key details at each investigational step. A diagnosis of endogenous Cushing's syndrome is usually suspected in patients with clinical symptoms and confirmed by using multiple biochemical tests. Each of the biochemical tests used to establish the diagnosis has limitations that need to be considered for proper interpretation. Although some tests determine the total daily urinary excretion of cortisol, many others rely on measurements of serum cortisol at baseline and after stimulation (e.g., after corticotropin-releasing hormone) or suppression (e.g., dexamethasone) with agents that influence the hypothalamic-pituitary-adrenal axis. Other tests (e.g., measurements of late-night salivary cortisol concentration) rely on alterations in the diurnal rhythm of cortisol secretion. Because more than 90% of the cortisol in the circulation is protein bound, any alteration in the binding proteins (transcortin and albumin) will automatically influence the measured level and confound the interpretation of stimulation and suppression data, which are the basis for establishing the diagnosis of Cushing's syndrome. Although measuring late-night salivary cortisol seems to be an excellent initial test for hypercortisolism, it may be confounded by poor sampling methods and contamination. Measurements of 24-hour urinary free-cortisol excretion could be misleading in the presence of some pathological and physiological conditions. Dexamethasone suppression tests can be affected by illnesses that alter the absorption of the drug (e.g., malabsorption, celiac disease) and by the concurrent use of medications that interfere with its metabolism (e.g., inducers and inhibitors of the P450 enzyme system). In this review, the authors aim to review the pitfalls commonly encountered in the workup of patients suspected to have hypercortisolism. The optimal diagnosis and therapy for patients with Cushing's disease require the thorough and close coordination and involvement of all members of the management team.


2015 ◽  
Vol 38 (2) ◽  
pp. E7 ◽  
Author(s):  
Vivien Bonert ◽  
Namrata Bose ◽  
John D. Carmichael

Diagnosing Cushing's syndrome is challenging and is further hampered when investigations are performed in a patient with cyclic Cushing's syndrome. A subset of patients with Cushing's syndrome exhibit periods of abnormal cortisol secretion with interspersed normal secretion. Patients can have periods of clinical improvement during these quiescent phases or remain symptomatic. Initial diagnostic testing can be challenging because of the unpredictable durations of the peak and trough phases, and it is especially challenging when the diagnosis of cyclic Cushing's syndrome has not yet been determined. Here, the authors present the case of a patient with Cushing's disease with a pathology-proven adrenocorticotropic hormone (ACTH)–secreting pituitary adenoma and whose initial inferior petrosal sinus sampling (IPSS) results were deemed indeterminate; further studies elucidated the diagnosis of cyclic Cushing's syndrome. Repeat IPSS was diagnostic of a central source for ACTH secretion, and the patient was treated successfully with transsphenoidal resection. Literature concerning the diagnosis and management of cyclic Cushing's syndrome is also reviewed.


1986 ◽  
Vol 79 (5) ◽  
pp. 262-269 ◽  
Author(s):  
R Fahlbusch ◽  
M Buchfelder ◽  
O A Müller

A series of 101 patients with Cushing's disease underwent transsphenoidal surgery. Diagnosis was fundamentally based on dynamic testing, mainly on the dosage-dependent suppression of cortisol after dexamethasone. The effect of surgery was monitored by intraoperative ACTH measurements. In 96 out of 101 patients a microadenoma of the pituitary was identified and removed selectively. In 74% of patients there was a clinical and endocrinological remission of Cushing's disease. Four ‘operative failures’ after selective adenomectomy underwent hypophysectomy in a second operation and each remitted. Thus the overall remission rate was 77%. In general, bilateral adrenalectomy was performed in patients who had failed to remit after selective adenomectomy. Although there is a considerable mortality and morbidity in patients with Cushing's syndrome, complications attributed to surgery were low. Two patients died postoperatively. In general, an improvement of disturbed pituitary function was noted after selective adenomectomy.


2006 ◽  
Vol 91 (10) ◽  
pp. 3746-3753 ◽  
Author(s):  
James W. Findling ◽  
Hershel Raff

Abstract Context: The diagnosis, differential diagnosis, and treatment of Cushing’s syndrome are challenging problems in clinical endocrinology. We focus on critical questions addressing screening for Cushing’s syndrome, differentiation of Cushing’s subtypes, and treatment options. Evidence Acquisition: Ovid’s MEDLINE (1996 through April 2006) was used to search the general literature. We also relied on previously published reviews and a recent monograph and cite a mix of primary articles and recent reviews. Evidence Synthesis: Although this article represents our opinion, it draws heavily on a recent consensus statement from experts in the field and a recent monograph on Cushing’s syndrome. Conclusions: We concluded that: 1) measurement of late-night or bedtime salivary cortisol is a useful approach to screen for Cushing’s syndrome; 2) measurement of suppressed plasma ACTH by immunometric assay is useful to differentiate ACTH-dependent and -independent Cushing’s syndrome; 3) inferior petrosal sinus sampling for ACTH should be performed in patients with ACTH-dependent hypercortisolism in whom a pituitary magnetic resonance imaging is normal or equivocal (in the absence of a pituitary ACTH gradient, prolactin levels should be measured to confirm the integrity of venous sampling); 4) computed tomography of the chest and abdomen and somatostatin receptor scintigraphy should be performed in patients with the occult ectopic ACTH syndrome; and 5) patients with Cushing’s disease should be referred to a neurosurgeon with extensive experience operating on corticotroph microadenomas. Bilateral laparoscopic adrenalectomy should be considered in patients with Cushing’s disease who fail therapies directed at the pituitary.


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