The more severe mineralocorticoid manifestations in the ectopic ACTH syndrome compared to pituitary Cushing's disease have been attributed to hypersecretion of 11-deoxycorticosterone. Another difference between the two forms of ACTH-excess, however, is a more severe degree of hypercortisolism in the ectopic syndrome. Cortisol can become a potent mineralocorticoid if its ...

Pathogenesis of hypertension in Cushing's syndrome has remained controversial. A 56-year-old Japanese man with Cushing's syndrome due to adrenal carcinoma has been followed up for more than 6 years. During the followup period, left adrenalectomy and hemihepatectomy due to metastatic lesion were performed. Blood pressure, serum cortisol, and urinary excretion ...

The empty-sella syndrome is uncommon in pediatric patients. Hypothalamic-pituitary dysfunction is common in these patients but involvement of the posterior pituitary gland is very rare. We report a seven-year-old girl with empty-sella syndrome who first developed arginine-vasopressin deficient diabetes insipidus and then anterior pituitary gland deficiency. The empty-sella syndrome should ...

We report for the first time the development of Nelson's syndrome in a patient who had previously undergone unsuccessful pituitary microadenomectomy and subsequently bilateral adrenalectomy. The removal of a 3-mm portion of a microadenoma did not protect against the development of Nelson's syndrome within 3 years which was closely related ...

An unusual case of coexisting acromegaly and Cushing's syndrome is reported in a 34-yr-old female. There was no biochemical or morphological evidence to suggest the presence of other endocrinopathies. She did not have any family history to suggest a hereditary tendency to endocrine disorders. Her acromegaly and Cushing's syndrome were ...

Three patients were studied after transsphenoidal microsurgery for Cushing's disease because their symptoms and signs were slow to settle and/or because they had variable endocrine results. All were established as having cyclical Cushing's syndrome, first diagnosed post-operatively. This may be a much more common finding than previously realized and emphasizes ...

Three patients of Cushing's syndrome with severe disproportionate hypertrophy of the interventricular septum are reported. All three underwent adrenalectomy and the diagnosis of Cushing's syndrome was confirmed by the presence of adrenal adenoma. All three showed hypertension before the adrenalectomy and two remained hypertensive following adrenalectomy. Before the operations the ...

A 27-year-old man presented to the casualty department with visual failure. Clinically he demonstrated the Foster-Kennedy syndrome. Computed tomography revealed a large space-occupying lesion which was subsequently shown to be a pituitary adenoma. The literature is reviewed and possible mechanisms of the Foster-Kennedy syndrome are discussed.

Correlation analysis was used to investigate the interrelation between plasma ACTH and serum cortisol concentrations determined at 8:00, 12:00, 16:00 and 22:00 h in 48 patients bilaterally adrenalectomized for Cushing's disease, including 23 patients with a pituitary adenoma (Nelson's syndrome). In the patients without evidence of a pituitary adenoma a ...

Circadian profiles of the serum levels of cortisol and five androgens were studied in 20 females including 8 controls, 7 patients with ACTH-dependent Cushing's syndrome and 5 with hypercortisolism due to adrenal adenoma. A significant 24-h periodicity was found for each steroid in all groups. Besides hypercortisolaemia, a significant increase ...

The term 'Multiple Endocrine Neoplasia' (MEN) denotes a genetically determined syndrome characterized by the independent appearance of benign or malignant changes of several endocrine organs as well as occasional changes of neural, muscular and connective tissue. Three different forms have been identified: MEN 1 (Werner's syndrome) includes parathyroid hyperplasia in ...

A young Japanese female demonstrated unusual features of Cushing's syndrome, cardiac myxomas and mucocutaneous lentigines. At the age of 12 years she presented with growth failure and obesity. The dexamethasone suppression test, the metyrapone test and low corticotropin concentrations indicated a primary adrenal disorder. At surgery, the adrenal glands were ...

Unilateral cerebrospinal fluid (CSF) rhinorrhea as the only manifestation of the primary empty sella syndrome is a rare event. A case of a middle-aged male patient complaining for intermittent unilateral rhinorrhea, which started 5 months earlier, is reported. The persistence of this state was attributed to an allergic rhinitis. The ...

To determine the sensitivity of the overnight 1-mg dexamethasone suppression test in diagnosing Cushing's syndrome, we evaluated the cortisol responses of 55 subjects (25 non-obese individuals with body mass index less than 25 kg/m2, 20 obese individuals with body mass index greater than 30 kg/m2, and 10 patients with surgically ...

Tumors of the female genital tract may be associated with a variety of unusual clinical manifestations. Uncommon endocrine and paraendocrine syndromes include production of human chorionic gonadotropin by tumors other than those of germ cell origin, hyperthyroidism associated with struma ovarii and gestational trophoblastic disease, the carcinoid syndrome, the Zollinger-Ellison ...

We describe a female newborn infant with McCune-Albright syndrome. In addition to the cutaneous pigmentation, she had apparent manifestations of hyperthyroidism and Cushing syndrome since birth. X-ray examinations showed many scattered lucencies in multiple bones. Endocrinological findings were as follows: serum T 4 276 nmol/l; free T 4 125 pmol/l; ...

Acute hydrops of the gallbladder is a well-recognized complication of Kawasaki syndrome. We report a case of a child with this syndrome whose gallbladder hydrops slowly resolved after intravenous gamma-globulin therapy. However, he continued to experience postprandial right upper quadrant abdominal pain. Hepatobiliary scintigraphy revealed normal filling of the gallbladder ...

Pheochromocytoma can cause several paraneoplastic syndromes. We report a patient with pheochromocytoma who exhibited pyrexia and marked inflammatory signs along with an elevated serum interleukin-6 (IL-6) level. All of these abnormalities disappeared and serum IL-6 became undetectable by removal of the tumor. In addition, immunohistochemical analysis revealed the presence of ...

A 14-year-old girl with nevoid basal cell carcinoma syndrome presented with intermittent headache and nausea. Magnetic resonance imaging and a computed tomographic scan of the head revealed a colloid cyst of the third ventricle and mild dilatation of the lateral ventricle. The cyst was successfully removed by stereotaxic surgery. The ...

A wide spectrum of clinical and biochemical presentations characterize ectopic POMC syndrome. It is presently postulated that ectopic POMC production results from increased expression of the activity of a POMC gene normally occurring in a variety of tissues outside the pituitary gland. The syndrome is rapidly progressive and is characterized ...

The usefulness of spot determination of urinary cortisol in the screening of Cushing's syndrome was evaluated by measuring the cortisol concentration in randomly sampled urine in 68 normal subjects and in 9 patients with Cushing's syndrome. The urinary cortisol concentration in the morning was significantly higher in patients with Cushing's ...

We report 2 cases of unilateral, multilocular, cystic adrenal masses in neonates. Both patients demonstrated somatic hemihypertrophy, which is considered to have the same implications as the Beckwith-Wiedemann syndrome in terms of tumor risk. Pathological examination showed multiple, adrenal cysts without evidence of tumor or routine adrenal hemorrhage. This finding ...

A 34-year-old woman presented with her fourth occurrence of an atrial myxoma and a right lower lung field mass. Her surgical history was extensive. In addition to resection of three previous atrial myxomas, she had undergone resection of her adrenal glands as a teenager because of Cushing's syndrome, and a ...

The histological specimens from 12 patients with the Drash syndrome were identified from the National Wilms Tumor Study Group and reviewed for the presence of nephrogenic rests. Of 7 patients with the complete Drash syndrome 6 were evaluable for nephrogenic rests, including 5 (83%) who demonstrated intralobar nephrogenic rests. Of ...

The availability of CRH for clinical use and the data so far generated by clinical investigators interested in its application to the problem of Cushing's syndrome have resulted in a new approach to the differential diagnosis of Cushing's syndrome (Fig. 2). Once the diagnosis of Cushing's syndrome is made with ...

In order to investigate the effect of chronic hypercortisolaemia on endogenous natriuretic factors (atrial natriuretic hormone (ANH) and the Na+/K+ pump inhibitor) digitalis-like substance (DLS), and their relation to hypertension, 28 patients with pituitary- or adrenal-dependent Cushing's syndrome and six patients on high-dose prednisone treatment were studied. Plasma ANH levels ...

A case of advanced cervical carcinoma of the uterus with ectopic adrenocorticotrophic hormone (ACTH) syndrome is described. The patient was seen for general malaise 21 months after surgical treatment of the primary lesion whose histology was undifferentiated small cell carcinoma of the uterine cervix. She had extensive metastases in the ...

We describe the occurrence of a liver adenoma in a young patient with Klinefelter's syndrome, diagnosed by classic 47,XXY karyotype in all investigated cells and a sex hormone imbalance. To our knowledge, this is the first report of such an association, which might suggest a simple coincidence. However, a pathogenetic ...

Described is the case of a 73-yr-old woman with metastatic pancreatic islet carcinoma that manifested initially as Zollinger-Ellison syndrome followed by onset of endogenous Cushing's syndrome, who developed Pneumocystis carinii pneumonia while on therapy with a long-acting somatostatin analog. Although P. carinii pneumonia has been observed in patients with Cushing's ...

We report an unusual case of pregnancy associated osteoporosis and empty Sella Syndrome with normal pituitary function. Pregnancy associated osteoporosis has been described rarely in the literature. Primary empty Sella Syndrome may occur in 5.5% of all autopsies. Primary empty Sella Syndrome with normal pituitary function has not previously been ...

A 70-year-old woman with Cogan's syndrome first presented with central diabetes insipidus and then developed secondary hypothyroidism. Magnetic resonance imaging revealed a diffuse pituitary swelling without evidence of tumor. High-dose glucocorticoid therapy administered to treat Cogan's syndrome was very effective in suppressing the inflammatory process, and resulted in the reversal ...

Adrenocorticotrophic hormone (ACTH)-secreting adenomas of patients with Cushing's disease (undifferentiated and well-differentiated ACTH-cell adenomas) were studied ultrastructurally and analysed morphometrically by a computer-supported quantitative image-analysing system. They were compared with identically prepared ACTH tumours (undifferentiated and well-differentiated ACTH-cell adenomas) of pituitaries from bilateral adrenalectomised patients with Nelson's syndrome. The aim ...

Ketoconazole, an imidazole derivative which inhibits adrenal steroidogenesis, has been used with success for the metabolic control of Cushing's disease. Few data are available about the use of ketoconazole in the management of the ectopic ACTH syndrome. We have used ketoconazole in eight patients: four patients with Cushing's disease, two ...

Cushing's syndrome caused by ectopic secretion of adrenocorticotropic hormone (ACTH) is often a serious disease and a diagnostic dilemma. In the reported patient, the source of ACTH proved to be a benign pulmonary carcinoid tumor. The patient describes his trying experiences through the six months from initial diagnosis to definitive ...

Pituitary apoplexy describes the clinical syndrome characterised by headache, visual impairment and ophthalmoplegia caused by sudden enlargement of a pituitary adenoma. This is usually due to extensive tumour infarction or haemorrhage. Pituitary apoplexy is rare, mimics spontaneous intracranial bleeding, and thus may be easily mistaken for acute subarachnoid haemorrhage. Urgent ...

Subacute thyroiditis is a benign, self-limited disorder characterized by hyperthyroidism that resolves spontaneously. Although cortisol metabolism is accelerated in hyperthyroidism, cortisol levels are usually normal. We describe a child with subacute thyroiditis whose presenting complaints and preliminary evaluation suggested a diagnosis of Cushing's syndrome. There was spontaneous improvement in the ...

Disturbed function of the hypothalamic-pituitary axis at adrenal and thyroid levels was found during an episode of hypersomnia in the idiopathic recurring hypersomnia syndrome. These endocrinological abnormalities, abolished ACTH and cortisol responses to insulin-induced hypoglycemia and absent TSH response to TRH, normalised thereafter in the symptom-free interval. These data support ...

Therapy for canine pituitary-dependent Cushing's syndrome (PDC) has primarily been medical management with o,p'-DDD. This drug therapy does not directly affect the underlying pituitary pathology, and may have undesirable side effects. Pituitary-dependent Cushing's syndrome may be caused by pituitary microadenomas or macroadenomas. Advances in diagnostic imagery have allowed the antemortem ...

A 74-year old female taking medroxyprogesterone acetate (MPA) presented with Cushing's syndrome together with low serum cortisol and plasma ACTH and an impaired response to synthetic ACTH (Synacthen, CIBA). When the medroxyprogesterone therapy was ceased the cushingoid features resolved and serum cortisol, ACTH and ACTH responsiveness all returned to normal. ...

Nelson's syndrome is generally regarded as an unusual sequela of primary bilateral adrenalectomy when performed for Cushing's disease. It is classically defined by cutaneous hyperpigmentation, considerably elevated adrenocorticotropic hormone (ACTH) levels, and an enlarged sella turcica. In this report, we present three cases initially treated by transsphenoidal sellar exploration for ...

Cycles of excessive cortisol secretion have been reported interposed with phases of remission lasting from few days to several months. In this study we report a ten year follow up on a patient who had four episodes of hypercortisolism and Cushing's syndrome associated with hypokalemia and edema and three long ...

A 59-year-old Caucasian housewife presented with a 2-year history of marked loss of tissue substance from the finger and toe pulps and the heel pads. There was no clinical evidence or history of urticaria or other inflammatory change. Investigations demonstrated a raised plasma cortisol secondary to a left adrenal adenoma. ...

A twenty-one-year-old virilized woman with Cushing syndrome due to a huge adrenocortical carcinoma was successively treated with trilostane (3 beta-hydroxysteroid dehydrogenase inhibitor), subsequent adrenalectomy, and postoperative cis-platinum. Clinical or biochemical abnormalities peculiar to Cushing syndrome gradually subsided, and three and one-half years after the adrenal surgery, the patient delivered a ...

Cushing's syndrome due to ectopic production of adrenocorticotropic hormone (corticotropin) has been recognized for many years. Traditionally, clinicians have thought that most cases were due to lung carcinomas and that the clinical manifestations differed from those for pituitary-dependent Cushing's syndrome. We report two cases of corticotropin-producing bronchial carcinoid tumors that ...

Cushing syndrome can recur following an adrenalectomy. One of the primary causes is recurrence of adrenal carcinoma either locally or from metastases. Hyperplasia and hyperfunction of adrenal remnants may also occur if there is pituitary stimulation. We have a patient in whom recurrent Cushing syndrome developed from small nonmalignant deposits ...

The case of a 35-year-old woman with Albright's syndrome, acromegaly and Hashimoto's thyroiditis is presented. She had noted deformity of the left mandible and chest from childhood. She developed persistent galactorrhea and amenorrhea after the delivery of her second child. X ray of the skull, and a head CT, revealed ...

We have reported the case of a 30-year-old woman with Cushing's disease who died of massive pulmonary thromboembolism 5 weeks after successful transsphenoidal hypophysectomy. Glucocorticoid excess appears to cause a hypercoagulable state, and consideration of this thromboembolic propensity and its potential duration after cure is indicated in all patients with ...

Establishment of the precise cause in a patient with Cushing's syndrome remains a major clinical challenge. The following case of a female patient with cyclical Cushing's syndrome illustrates how recent advances such as the introduction of the corticotropin-releasing hormone (CRH) test (in determining the cause of ACTH production) and high-resolution ...

Adrenal histoplasmosis in Cushing's syndrome of adrenal origin is rare. A patient with Cushing's disease with bilateral nodular hyperplasia and histoplasmosis of both the adrenal glands is described. The diagnosis of histoplasmosis was only made post operatively as the constitutional manifestations, besides being partially masked by hypercortisolism also resemble those ...

It may sometimes be difficult to distinguish Cushing's disease from ectopic ACTH syndrome. A case is described here of a patient with a Cushing's syndrome and diagnostic difficulties. Initial features were consistent with a Cushing's disease (in particular metopirone test was positive). Because of relapse of hypercortisolism after mitotane therapy, ...