Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: An Underestimated Entity.

Background: The diagnosis of Cushing disease is based on endocrinological pa-rameters, with no single test being specific. In some patients, dynamic thin-slice sellar magnetic resonance imaging fails to detect a pituitary tumor.

Objective: The purpose of this study is to investigate the role of ectopic pituitary adenoma in this situation.

Sympathetic activity is reduced by nCPAP in hypertensive obstructive sleep apnoea patients.

There is increasing evidence that nasal continuous positive airway pressure (nCPAP) lowers blood pressure in obstructive sleep apnoea (OSA) patients, not only during sleep but also in the daytime. However, both the mechanisms of blood pressure reduction and the considerable differences in the magnitude of the effect in the studies presented to date are not fully understood. Therefore, the authors prospectively studied the effect of nCPAP on noradrenaline plasma levels (NApl), blood pressure and heart rate (HR) in 10 normotensive and eight hypertensive OSA patients before and after 41.

Familial ACTH-independent Cushing's syndrome with bilateral macronodular adrenal hyperplasia clinically affecting only female family members.

Primary adrenal hyperplasia, which may occur as a familial disorder, is a rare cause of ACTH-independent Cushing's syndrome. In most of these cases the underlying pathology is primary adrenocortical micronodular dysplasia. Very few cases of familial Cushing's syndrome due to primary macronodular adrenal hyperplasia have been described.

Obstructive sleep apnoea in acromegaly: the role of craniofacial changes.

Obstructive sleep apnoea (OSA) is due to craniofacial changes and acromegaly. The question addressed by this study was whether growth hormone (GH) induced craniofacial changes might explain persisting OSA despite endocrine inactivity in acromegaly. Nineteen patients treated for acromegaly were examined cephalometrically for craniofacial changes and polysomnographically for OSA.

Atrial natriuretic peptide levels and pulmonary artery pressure awake, at exercise and asleep in obstructive sleep apnoea syndrome.

Elevated nocturnal plasma atrial natriuretic peptide (ANP) levels were found in patients with obstructive sleep apnoea (OSA). The purpose of our study was to examine the secretion of ANP during the night and to measure changes in oxygen saturation, pulmonary artery pressure and intrathoracic pressure swings in patients with OSA. Moreover, we analysed the secretion of ANP and the pulmonary artery pressure in different behavioural states, e.

[Reactive thymus dysplasia following therapy for ACTH-producing tumors].

Unlabelled: Surgical or conservative treatment of ACTH-producing tumors results in acute drop of the previously excessively high cortisol levels. The following associated pathophysiological changes also occur in the organism's recovery from stress, such as trauma, operation or chemotherapy of tumors. Both cases result in a regeneration of the immune system, which might even be exalted.

High levels of circulating adrenomedullin in severe illness: correlation with C-reactive protein and evidence against the adrenal medulla as site of origin.

Adrenomedullin (AM) is a novel vasorelaxing peptide which was originally isolated from the extracts of human pheochromocytoma. It is produced by a number of organs among which the adrenal gland exhibits by far the highest concentrations. The peptide circulates in blood and its plasma levels have been reported to be increased in several diseases such as renal failure and sepsis.

Impact of profile haemodialysis on intra-/extracellular fluid shifts and the release of vasoactive hormones in elderly patients on regular dialysis treatment.

In 15 patients with end-stage renal failure and proven coronary heart disease, profile haemodialysis with decreasing ultrafiltration rate and hyperionic, decreasing dialysate solute concentration was compared with conventional, extracorporeal bicarbonate haemodialysis (Na+D = 138 mmol/l). Body fluid distribution and the release of vasoactive hormones (plasma renin activity, aldosterone, norepinephrine, epinephrine, and atrial natriuretic peptide) were investigated. Haemodialysis with constant ultrafiltration rate and constant dialysate composition (A) was followed by two dialysis profiles: decreasing ultrafiltration rate (B) and additional hyperionic, decreasing dialysate sodium concentration (C).

Radiommunological measurement of leptin in plasma of obese and diabetic human subjects.

Studies in mice have identified the ob gene product, leptin, as a signaling factor regulating body weight homeostasis and energy balance. Defective production of the encoded protein may be one of the causes for the development of obesity. Using a high affinity antibody, that in immunohistochemical studies specifically stained human adipocytes, a radioimmunoassay was established and leptin immunoreactivity was quantified in plasma of lean and obese human subjects.

Successful treatment of osteoporosis in systemic mastocytosis with interferon alpha-2b.

Osteoporosis is frequently seen in systemic mastocytosis. Although diphosphonate therapy has been shown to be transiently effective, therapy options for this form of osteopenia are very limited. We have treated three patients with systemic mastocytosis and osteopenia successfully with interferon alpha-2b.

[Plasma endothelin concentration during cold provocation in primary Raynaud's syndrome].

Plasma endothelin concentration was measured separately in each hand before and after unilateral whole-hand cooling in 23 patients (5 men, 18 women; mean age 35.2 [19-52] years) thought to have primary Raynaud's syndrome (Raynaud's disease). The diagnosis was confirmed, after excluding other causes, by strain-gauge plethysmography demonstrating cold-induced vasospasm in 8, disproved in 15.

Regulation of blood volume-implications for cardiovascular pathophysiology in sleep apnoea.

Volume homeostasis plays an important role in the regulation of the cardiovascular system and maintenance of haemodynamics. The heart-kidney axis represents the central part of the volume regulating system: the heart senses changes in the volume status and influences renal function via neural and humoral pathways in order to compensate for disturbances in volume homeostasis and prevent under- or overfilling of the heart. An undisturbed circadian rhythmicity of volume homeostasis, renal function and secretory pattern of volume regulating hormones may be of physiological importance.

[Oral contraceptive-induced pancreatitis in the hyperchylomicronemia syndrome].

A now 24-year-old woman was found at the age of 2 years to have an hyperchylomicronaemia syndrome due to lipoprotein lipase deficiency: the triglyceride level was then 6000 mg/dl. But in subsequent years it had been reduced to between 550 and 2600 mg/dl by dieting. There were no xanthomas or abdominal symptoms during those years.

Influence of somatostatin to biochemical parameters in patients with primary hyperparathyroidism.

Somatostatin (SRIF) is effective in the nonoperative management of a variety endocrine tumors. A potential role of SRIF for treatment of patients with primary hyperparathyroidism (pHPT) has been suggested. In a controlled, prospective, triple-blinded, randomized clinical trial, the somatostatin analogue octreotide (SMS 201-995, Sandostatin) was evaluated in 40 patients with well documented pHPT.

Parathyroid function following ligation of the inferior thyroid arteries during bilateral subtotal thyroidectomy.

A randomized controlled trial was performed to compare two techniques of bilateral subtotal thyroidectomy for non-toxic nodular goitre with regard to postoperative parathyroid function. The 50 patients in group 1 underwent ligation of the trunks of the inferior thyroid arteries. In group 2 (50 patients) the branches of these arteries were suture-ligated at the thyroid capsule.

[Iodine-induced hyperthyroidism in metastatic thyroid carcinoma].

A 75-year-old man with nodular goitre (for the preceding 2 years treated with 75 micrograms/dl L-thyroxine) complained of pain over the left hip: on auscultation an arterial flow murmur was audible over the hip. The radiograph demonstrated extensive osteolysis in the flat part of the ilium. Search for the primary tumour, including two pelvic angiographs, was unsuccessful.

[Adrenomyeloneuropathy. A frequent cause of Addison's disease].

Adrenomyeloneuropathy (AMN) is a "milder form" of adrenoleukodystrophy with a X-linked inheritance. Abnormal catabolism of the very long-chain fatty acids (VLCFA) results in Addison's disease and spastic paraparesis. The VLCFA concentration was measured in 23 of 26 patients with Addison's disease (mean age 48.

Lipoprotein (a), low-density, intermediate-density lipoprotein, and blood pressure in a young male population.

Both hypercholesterolemia and hypertension are risk factors for atherosclerotic vascular disease, and elevated cholesterol levels occur more frequently than expected in patients with hypertension. Elevated levels of intermediate-density lipoproteins (IDL) and low-density lipoproteins (LDL) were shown to be atherogenic, and LDL, comprising the major cholesterol-carrying fraction in human plasma, are structurally related to lipoprotein (a)[Lp(a)], a further risk factor for atherosclerosis. In the present study we investigated 200 male employees (mean age 26 +/- 7 years) to determine whether the relationship of IDL and Lp(a) to systemic blood pressure is similar to the reported correlations between total and LDL cholesterol and systemic blood pressure.

[Osteoporotic vertebral fractures in systemic mastocytosis without skin involvement].

Radiological investigation of the vertebral column in two patients with low-back pain (53-year-old woman and a 52-year-old man) revealed unusually marked osteoporosis and sintering fractures of the 3rd, and 1st and 4th lumbar vertebrae, respectively. Biochemical tests failed to provide any evidence about metabolic or endocrinological abnormality. Iliac crest biopsy showed mastocytosis.

Reduction of nocturnal diuresis and natriuresis during treatment of obstructive sleep apnea (OSA) with nasal continuous positive air pressure (nCPAP) correlates to cGMP excretion.

In ten patients with severe obstructive sleep apnea (OSA) profound changes in renal function could be demonstrated at night during nCPAP therapy. Natriuresis and diuresis decreased by about 50% while creatinine excretion rate and urinary osmolality did not change. We found parallel changes in the excretion of ANP's second messenger cyclic guanosine monophosphate (cGMP) in a dose-response-related manner to natriuresis respectively diuresis.

Disturbances in volume regulating hormone system--a key to the pathogenesis of hypertension in obstructive sleep apnea syndrome?

Recent studies about renal function and volume regulating hormones in obstructive sleep apnea (oSAS) indicate complex disturbances in volume homeostasis. Increased nocturnal secretion of atrial natriuretic peptide (ANP) and decreased renin secretion during apnea looks similar to a situation seen during hypervolemia or increased cardiac volume load. Increased venous return induced by pathologically high negative intrathoracic pressure during obstructive apnea may be the cause.

[Adrenomyeloneuropathy as a cause of Addison's disease].

A 25-year old male, with a 3-year history of spastic paraparesis and a disturbance of bladder emptying, developed Addison's disease (plasma ACTH 1250 pg/ml, depressed cortisol values with loss of diurnal rhythm, potassium 6.8 mmol/l, sodium 123 mmol/l) associated with a urinary tract infection. The suspected diagnosis of adrenomyeloneuropathy was confirmed by the finding of raised plasma long-chain fatty acid concentrations.

[Serum endothelin in patients with Raynaud syndrome before and following cold provocation].

To determine wether there is a pathogenetic effect of endothelin (ET) in vasoconstriction in Raynaud's phenomenon, plasma-endothelin and it's association to extent of vasoconstriction was measured before and after digital cooling. Only in cases with massive vasoconstriction ET rose significantly after cooling. In conclusion ET seems to be involved in the pathogenesis of RS, whereas the exact pathomechanism remains unclear.