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PubMed1984

[Cushing syndrome and pregnancy].

Pregnancies in cases of Cushing syndrome are rare. When conception occurs the complication rate is high especially from hypertension and superimposed toxemia. Abortions, premature deliveries, and stillbirth occur more often. In the case described an adenoma of the adrenal gland was diagnosed early in pregnancy. Frequent prenatal care and treatment of the hypertension resulted in a pregnancy of 34 weeks gestation. At this time low HPL and estriol values and variable decelerations forced a Cesarean section. The premature infant was discharged in good condition after several weeks of cortico-steroid substitution. In the mother a well defined adrenal cortical tumour at right was found post-partum. The operative specimen showed an adenomatous nodular adrenal cortical hyperplasia. The described case shows that frequent prenatal care and early hospitalization permit an expectant treatment of pregnancy in cases of Cushing-Syndrome.

Adenoma↗
PubMed1985

[Corticotropin releasing factor as an aid in the diagnosis of Cushing syndrome].

6 patients with Cushing's syndrome were investigated with regard to the effect of synthetic ovine corticotropin-releasing factor (o-CRF), administered as an intravenous bolus of 100 micrograms, on peripheral plasma concentrations of ACTH and cortisol. The purpose of this study was to evaluate the usefulness of this "CRF test" in the differential diagnosis of Cushing's syndrome as compared with conventional diagnostic procedures. 100 micrograms CRF caused a rise in plasma ACTH and cortisol in patients with bilateral adrenal hyperplasia (n = 3). However, in patients with cortisol-producing adrenal adenoma (n = 2) and ectopic ACTH overproduction (n = 1), no increase in plasma cortisol and ACTH was induced by exogenous CRF. We conclude from these findings that the CRF test will prove a valuable diagnostic tool to differentiate pituitary from extrapituitary forms of endogenous hypercortisolism in patients with Cushing's syndrome.

Adolescent↗
PubMed1986

Pituitary and adrenal CT of Cushing syndrome.

Determination of the site of excessive hormone production in Cushing syndrome is possible with biochemical tests in 80% of cases. High-resolution CT of both the pituitary and adrenal glands was used to evaluate eight patients with surgically verified ACTH-secreting pituitary microadenomas and one patient with ectopic Cushing syndrome. Three ACTH-secreting microadenomas were demonstrated by CT. Adrenal CT was normal in six of the eight patients with pituitary tumors. The patient with ectopic ACTH production had mild unilateral adrenal gland enlargement and a normal pituitary CT scan. Normal adrenal or pituitary CT scans do not exclude Cushing syndrome.

Adenocarcinoma↗
PubMed2000

[Cushing syndromes due to aberrant expression of functional receptors other than ACTH. A new organic hypercorticism syndrome entity].

The diagnosis of Cushing's syndrome remains a challenge in clinical endocrinology. Cushing's syndromes are usually classified as dependent or independent from ACTH. In the first class are Cushing's disease, the ectopic corticotropin syndrome and the rare ectopic CRH syndrome. These ACTH-dependent Cushing's syndromes usually present diffusely hyperplastic adrenal glands. In the second class, are cortisol producing unilateral adrenocortical adenomas or carcinomas. New entities have recently emerged as bilateral adrenal hyperplasia not dependent from ACTH; their etiopathogenies are heterogeneous with illicit expressions at the adrenal level of functional receptors to various ligands: GIP, catecholamines, lutropin... The knowledge of such entities has to be taken into consideration in the diagnostic and management of ACTH independent Cushing syndromes.

Adrenal Gland Neoplasms↗
PubMed2001

Biochemical basis for the hypercoagulable state seen in Cushing syndrome; discussion 1006-7.

HYPOTHESIS: Cushing syndrome (CS) is associated with a hypercoagulable state that results in a 4-fold increase in the incidence of pulmonary embolism, deep venous thrombosis, and a 4-fold mortality rate compared with the general population. The incidence of CS in humans is approximately 2 to 5 per million per year, whereas in dogs it is much higher. The clinical complications of CS in humans are also manifested in dogs. We used a dog model of CS to better define the biochemical basis for the hypercoagulable state seen in the disease. DESIGN: A consecutive sample of dogs with CS and a cohort of healthy control dogs identified at a "well-dog check" were enrolled. All dogs underwent blood assays to identify the levels of procoagulant factors, natural antithrombotics, and the degree of ongoing activation of the coagulation cascade. SETTING: University veterinary medical teaching hospital. RESULTS: A total of 86 dogs were enrolled, 56 with CS and 30 control dogs. Levels of procoagulation factors II, V, VII, IX, X, XII, and fibrinogen were significantly increased in dogs with CS (P<.05). The natural antithrombotic antithrombin was significantly decreased in dogs with CS (P<.02). Thrombin-antithrombin complexes, a marker of subclinical thrombosis, were significantly increased in dogs with CS (P<.05). CONCLUSIONS: The hypercoagulable state of CS is demonstrated by an increase in thrombin-antithrombin complexes. This hypercoagulable state may be caused in part by (1) an elevation of procoagulant factors, and (2) a decrease in antithrombin. Because of the similar clinical and biochemical changes between dogs with CS and humans, this canine model may be a useful tool for the future study of the hypercoagulable state in CS.

Animals↗
PubMed2005

A case of Cushing syndrome secondary to ectopic adrenocorticotropic hormone producing carcinoid of the duodenum.

Cushing syndrome caused by adrenocorticotropic hormone (ACTH) production from solid tumors can result in life-threatening hypercortisolemia. Ectopic ACTH production is most commonly associated with bronchial carcinoids and squamous cell carcinoma of the lung. We report a case of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum. The patient presented to an outside hospital in hypertensive crisis and diabetic ketoacidosis. After stabilization, diagnostic studies including a serum cortisol level, and computed tomography (CT) scans of the head, chest, abdomen, and pelvis revealed hypercortisolemia and a large mass in the head of the pancreas. Pancreaticoduodenectomy was performed. Pathologic investigation revealed a 1-cm carcinoid of the duodenum with two large metastatic lymph nodes near the head of the pancreas. This is the first reported case in the English literature of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum.

ACTH Syndrome, Ectopic↗
PubMed1992

The Cushing syndrome induced by bronchial carcinoid tumors.

OBJECTIVES: To define the clinical spectrum of bronchial carcinoid tumors in patients presenting with the Cushing syndrome, to evaluate the role of chest radiographs and computed tomography in their diagnosis, to review the characteristic histologic features, and to determine patient outcome. DESIGN: Retrospective analysis of consecutive patients. SETTING: A referral-based medical center. PATIENTS: Fifteen consecutive patients with the Cushing syndrome evaluated at the Mayo Clinic with histologically proven bronchial carcinoid tumors. MEASUREMENTS: Clinical, radiographic, and histologic features. RESULTS: The Cushing syndrome was the initial clinical presentation, and bronchial carcinoid tumors were found later in all 15 patients. The diagnosis of carcinoid tumor was proved histologically in all cases. Ten biopsies showed typical carcinoid tumors, three were histologically atypical, and three were metastatic. Corticotropin was detected by immunostaining in seven of these tumors. Biochemical analysis showed marked elevations of circulating corticotropin with a mean serum value of 156 +/- 58 pmol/L (normal, 4 to 22 pmol/L). Additional clinical features included hypokalemia in six patients and glucocorticoid response to either high-dose dexamethasone or metyrapone in 6 of 13. These hormonally active carcinoid tumors were frequently radiographically occult, with 10 of 15 patients initially having normal chest radiographs. Computed tomography was successful in locating carcinoid tumors in five patients with negative chest radiographs evaluated after 1980. All five remaining patients with normal chest radiographs evaluated before 1980 eventually developed nodular lesions on standard chest radiography from 1 to 10 years later. Ten patients achieved complete remission and two patients, partial remission of the Cushing syndrome after surgical resection. Three patients continued to have symptomatic glucocorticoid excess due to metastatic disease. CONCLUSIONS: Although uncommon, the Cushing syndrome may be the initial clinical manifestation of an otherwise indolent bronchial carcinoid tumor. Radiographic imaging of occult lesions can be successfully accomplished with computed tomography. Surgical resection is curative in most patients with this disorder.

ACTH Syndrome, Ectopic↗
PubMed1995

Differential diagnosis in cushing syndrome. Use of corticotropin-releasing hormone.

Testing with ovine corticotropin-releasing hormone (CRH) has facilitated the differential diagnosis of Cushing syndrome, which is often not straightforward. We provide our experience between January 1989 and August 1993 with 30 patients with Cushing syndrome and describe 4 cases in detail to illustrate how CRH testing can be successfully applied to some of the difficulties encountered in the evaluation. CRH testing proved to be particularly useful in distinguishing cases of Cushing syndrome of adrenal etiology from those of Cushing disease with low or undetectable adrenocorticotropin (ACTH) levels. CRH testing during petrosal sinus sampling was also found to help distinguish the ectopic ACTH syndrome from pituitary-dependent Cushing syndrome. Our cases illustrate the need for careful biochemical evaluation before proceeding to imaging studies. Using CRH testing to evaluate cases of Cushing syndrome in which standard testing was inconclusive can provide useful information and lead to a more rapid determination of etiology and definitive therapy than previously possible.

ACTH Syndrome, Ectopic↗
PubMed1989

[Metabolism of calcium and phosphorus in Cushing syndrome with osteoporosis].

52 patients with Cushing syndrome were studied. According to the appearance of X-ray film of bones, they were divided into three groups: (I) Without osteoporosis, 12 cases (23.1%), (II) Mild osteoporosis, 17 cases (32.7%), (III) Severe osteoporosis with fractured ribs and/or wedge-shaped vertebrae, 23 cases (44.2%). It was found that the mean level of serum calcium in the patients was significantly lower than that in 94 normal subjects (mean +/- S 2.3 +/- 0.2 versus 2.4 +/- 0.1 mmol/L P less than 0.001). The mean concentrations of blood alkaline phosphatase and parathyroid hormone in the patients were much higher than those in normal subjects (65.0 +/- 27.6 versus 42.6 +/- 15.6 IU/L P less than 0.001, 44.6 +/- 22.4 versus 20.6 +/- 8.0 pg/ml P less than 0.001 respectively). The mean level of serum 25 (OH)-D in the patients was significantly lower than that in controls (10.9 +/- 5.6 versus 16.2 +/- 4.6 ng/ml P less than 0.001). Urinary calcium excretion increased in the patients as compared with that in controls (P less than 0.01). The urinary calcium excretion correlated well with the blood total cortisol and urinary free cortisol. It is suggested that hypercalciuria might cause decrease of serum calcium. Thus the parathyroid glands were stimulated and the parathyroid hormone (PTH) secretion was increased. Excess of PTH stimulates bone resorption. All of these factors are involved in the pathogenesis of osteopenia in Cushing's syndrome.

Adolescent↗
PubMed2004

Cyclical Cushing syndrome presenting in infancy: an early form of primary pigmented nodular adrenocortical disease, or a new entity?

Cushing syndrome is uncommon in childhood and rare in infancy. We report the case of a 3-yr-old child who presented with symptoms of Cushing syndrome beginning shortly after birth. Her hypercortisolemia was cyclical, causing relapsing and remitting symptoms, which eventually led to suspicions of possible Munchausen syndrome by proxy. Investigation at the National Institutes of Health excluded exogenous administration of glucocorticoids and indicated ACTH-independent Cushing syndrome. Paradoxical response to dexamethasone stimulation (Liddle's test) suggested a diagnosis of primary pigmented nodular adrenocortical disease (PPNAD). After bilateral adrenalectomy, both glands showed micronodular adrenocortical hyperplasia, but histology was not consistent with typical PPNAD. DNA analysis of the coding sequences of the PRKAR1A gene (associated with PPNAD and Carney complex) and the GNAS gene (associated with McCune-Albright syndrome) showed no mutations. We conclude that hypercortisolemia in infancy may be caused by micronodular adrenocortical hyperplasia, which can be cyclical and confused with exogenous Cushing syndrome. A paradoxical rise of glucocorticoid excretion during Liddle's test may delineate these patients. Infantile micronodular disease has some features of PPNAD and may represent its early form; however, at least in the case of the patient reported here, micronodular hyperplasia was not caused by coding mutations of the PRKAR1A or GNAS genes or associated with typical histology or any other features of Carney complex or McCune-Albright syndrome and may represent a distinct entity.

Adrenal Cortex Diseases↗
PubMed1994

The metyrapone and dexamethasone suppression tests for the differential diagnosis of the adrenocorticotropin-dependent Cushing syndrome: a comparison.

OBJECTIVE: To develop criteria for interpreting results of the metyrapone test for the differential diagnosis of the adrenocorticotropin (ACTH)-dependent Cushing syndrome and to compare its diagnostic accuracy with that of the high-dose dexamethasone suppression test. DESIGN: Retrospective cohort study. SETTING: Inpatient research ward. PATIENTS: 186 patients believed to have the ACTH-dependent Cushing syndrome who had the metyrapone test, the dexamethasone test, and a surgical procedure to remove the source of excessive ACTH. MEASUREMENTS: The sensitivity, specificity, and diagnostic accuracy were determined for the metyrapone test using urine excretion of hydroxysteroid and plasma levels of 11-deoxycortisol. For the dexamethasone suppression test, urine excretions of 17-hydroxysteroid (17-OHS) and free cortisol were used. MAIN RESULTS: 156 patients had pituitary disease, 15 had ectopic ACTH secretion, and 15 had no diagnosis after pituitary surgery. Of those 15 patients, 14 were ultimately classified as having pituitary disease on the basis of follow-up, and 1 was found to have ACTH-independent Cushing syndrome. After administration of metyrapone, stimulation of 17-OHS excretion greater than 70% or of a plasma 11-deoxycortisol level greater than 400-fold did not result in the misclassification of any of the patients with surgically confirmed cases of ectopic ACTH secretion. When these criteria were combined, the percentage of correct predictions (122 of 186 [71%; 95% CI, 62% to 79%]) was higher than that for either steroid alone (116 of 186 [62%; CI, 52% to 71%]) for excretion of 17-OHS and that for plasma 11-deoxycortisol (82 of 186 [44%; CI, 34% to 54%]). When the criteria for both the metyrapone test and the dexamethasone test were combined, the percentage of correct predictions (153 of 186 [82%; CI, 75% to 87%]) was higher than that obtained when the criteria for either test alone were used (P = 0.001). Similar results were found when the 15 patients with indeterminate surgery were assigned to the appropriate group on the basis of follow-up. When the criteria for both the metyrapone and dexamethasone tests were combined to identify patients with the pituitary Cushing syndrome, the sensitivity and diagnostic accuracy improved to 88% and 89%, respectively. CONCLUSIONS: The metyrapone test, which can be done in 48 hours, has a sensitivity and specificity for the diagnosis of the Cushing syndrome identical to that of the standard 6-day high-dose dexamethasone suppression test. Combining both tests results in greater accuracy than that obtained with either test alone.

Adrenocorticotropic Hormone↗
PubMedDate not supplied

CT and MR imaging of the adrenal glands in ACTH-independent cushing syndrome.

Adrenocorticotropic hormone (ACTH)-independent hypercortisolism accounts for 15%-20% of cases of Cushing syndrome and always arises from primary adrenal disease. Computed tomographic (CT) and magnetic resonance (MR) imaging findings in 37 patients with primary adrenal Cushing syndrome were analyzed and correlated with pathologic findings. Hyperfunctioning adenomas (n = 24), together with functioning carcinomas (n = 10), accounted for 92% of cases. Adenomas had a significantly smaller mean size (3.5 vs 14.5 cm) and lower mean unenhanced CT attenuation value (11 vs 28 HU) than did carcinomas. The presence of necrosis, hemorrhage, and calcification favored a diagnosis of carcinoma. Six of 10 carcinoma patients had metastases at presentation. Two adenomas were seen within a myelolipoma, which was recognized at both CT and MR imaging due to its fat content, and two adenomas were of uncertain malignant potential. Bilateral disease--primary pigmented nodular adrenal dysplasia (PPNAD) (n = 2) and ACTH-independent macronodular adrenal hyperplasia (AIMAH) (n = 1)--had characteristic imaging features. In PPNAD, multiple tiny (2-5-mm) nodules were visible bilaterally, with no overall glandular enlargement and normal intervening adrenal tissue. In AIMAH, both glands were grossly enlarged and contained nodules up to 3 cm in diameter. Familiarity with the range of imaging appearances of the adrenal glands in primary adrenal Cushing syndrome may help establish the underlying diagnosis.

Adenocarcinoma↗
PubMed1987

Cushing syndrome: pituitary CT scanning.

Proper treatment of patients with Cushing syndrome depends on knowledge of whether the condition derives from a pituitary or adrenal adenoma or an ectopic tumor that secretes adrenocorticotropic hormone (ACTH). Fifty-seven patients with Cushing syndrome were prospectively evaluated to determine whether computed tomography (CT) of the pituitary gland is useful in diagnosing or excluding an ACTH-producing adenoma and in predicting the site of the adenoma within the pituitary gland. Findings on CT scans were normal in 35 of 50 patients with surgically confirmed Cushing disease and in seven patients with ectopic ACTH-producing tumors. Of the 15 patients with Cushing disease for whom CT results were abnormal, eight had macroadenomas and seven had microadenomas. This study demonstrates a low sensitivity (30%) and low diagnostic accuracy (39%) of CT in the detection of ACTH-producing pituitary tumors and indicates the need for alternative methods of confirming the pituitary origin of excessive ACTH secretion in most patients.

Adenoma↗
PubMedDate not supplied

[Gonadotropic function in female Cushing syndrome (author's transl)].

A study of 38 female Cushing syndromes showed decreased levels of FSH and LH, with normal LH-RH stimulation in most adrenal hyperplasia and benign adenoma, and absent stimulation in carcinoma. The levels were normalized by suppression of hypercorticism. This suggests a blunted gonadotropic function, in female Cushing syndrome, probably at the hypothalamic level. The predominant respective roles of hyperandrogeny in carcinoma and of hypercortisolism in adrenal hyperplasia and benign adenoma seem probable.

Adenoma↗
PubMed1975

Cushing syndrome, sexual precocity, and polyostotic fibrous dysplasia (Albright syndrome) in infancy.

The sexual precocity of polyostotic fibrous dysplasia is occasionally accompanied by other endocrine disorders, but in only two previous instances has Cushing syndrome been reported. The history of a 6-month-old girl is presented, in whom this syndrome was complicated by congenital Cushings syndrome. Although endocrinopathies of polyostotic fibrous dysplasia have usually been ascribed to a central (hypothalamic) origin, the findings in this patient suggest autonomous hyperfunction of the peripheral endocrine glands, with the Cushing syndrome caused by hyperplastic nodules in the adrenal glands and the precocity by luteinized follicular cysts of the ovary.

Adrenal Glands↗
PubMed2003

Pseudo-Cushing syndrome caused by fenofibrate interference with urinary cortisol assayed by high-performance liquid chromatography.

Urinary free cortisol (UFC) excretion over 24 h reflects the production rate of cortisol and is used commonly in the diagnosis of Cushing syndrome. We report on two patients evaluated for Cushing syndrome who had elevated UFC when analyzed by HPLC but normal values for the analysis performed by RIA and HPLC-mass spectrometry/mass spectrometry (HPLC-MS/MS). Other laboratory testing was inconsistent with the diagnosis of Cushing syndrome and raised doubts about the diagnosis. We identified a probable cause of analytical interference as coming from fenofibrate (Tricor), medication taken by the patients. Fenofibrate peak overlapped with the HPLC peak of cortisol and produced an MS/MS transition overlapping the major transition of cortisol. A second MS/MS transition was free from interference. In summary, fenofibrate administration may cause false elevation of UFC values determined by HPLC or HPLC-MS/MS in patients evaluated for Cushing syndrome. An HPLC-MS/MS method using multiple mass transitions, rather than a single transition, allows accurate quantitation of urinary cortisol in patients taking fenofibrate.

Adult↗
PubMed2001

Expression of adrenocorticotropin receptor gene in adrenocortical adenomas from patients with Cushing syndrome: possible contribution for the autonomous production of cortisol.

OBJECTIVE: To examine whether inhibition of endogenous adrenocorticotropin (ACTH) secretion in patients with Cushing syndrome affects the expression of the ACTH receptor (ACTH-R) gene in adrenocortical adenoma and attached atrophic normal gland. SUMMARY BACKGROUND DATA: ACTH increases adrenal cell growth and steroidogenesis by means of ACTH-R. In vivo and in vitro studies have shown that expression of ACTH-R is upregulated by its own ligand ACTH in several species. In patients with Cushing syndrome resulting from adrenocortical adenoma, there is autonomous production of cortisol from the adenoma. This strongly inhibits endogenous ACTH secretion, giving rise to the speculation that the expression of the ACTH-R gene in these patients is also suppressed. However, previous studies have shown that administration of exogenous ACTH to these patients leads to a further increase in the production of cortisol, suggesting the expression of functional ACTH-R in the adenoma. The authors, therefore, examined the expression of the ACTH-R gene in these patients. METHODS: Fourteen patients with Cushing syndrome were studied. Glucocorticoid excess resulting from autonomous production from the adenomas was ascertained, and unilateral adrenalectomy was performed. The levels of ACTH-R and cytochrome P450 side chain cleavage enzyme (P450scc) mRNAs were determined by Northern blot analysis. The entire coding region of the ACTH-R gene in these patients was sequenced. RESULTS: ACTH-R mRNA abundance in the attached atrophic normal adrenals was suppressed and invariably less than that in the normal gland obtained from a patient with renal cancer. However, the expression of ACTH-R mRNA was not suppressed in any of the adenomas. Expression of ACTH-R mRNA in the adenomas was four- to sixfold greater than that in the attached atrophic gland. No mutation in the coding sequence of the ACTH-R gene in the adenoma was detected in any of the patients. The mRNA in the adenomas appeared to be translated into functionally active receptor because intramuscular administration of ACTH resulted in significant increases in plasma cortisol before surgery but not 3 months after surgery. In addition, there was a positive linear correlation between the expressions of ACTH-R and P450scc mRNAs in the adenoma tissue. CONCLUSIONS: Suppressed ACTH secretion in patients with Cushing syndrome results in reduction of the ACTH-R mRNA expression in nonneoplastic adrenocortical cells. However, the regulatory mechanism of ACTH-R expression might be different in adenoma. Persistent expression in the adenoma of ACTH-R alone, even in the absence of ACTH, might result in increased basal adenyl cyclase activity, as observed in the case of thyroid-stimulating hormone receptor, and thereby might play a role in the autonomous production of cortisol.

Adenoma↗