[Epidemiology, clinical manifestations and efficiency of different methods of treatment of acromegaly according to the United Russian Registry of Patients with Pituitary Tumors].

BACKGROUND: The registry is the main source of information about patients with acromegaly for assessing the quality of medical care, effectiveness of treatment, determining the compliance of real clinical practice with existing standards and patient management protocols. AIMS: To evaluate epidemiological, demographic and clinical characteristics of acromegaly in Russian Federation and effectiveness of treatment modalities. MATERIALS AND METHODS: The object of the study was the database of the united Russian registry of patients with pituitary tumors with specific analysis of patients with acromegaly only. We analyzed the data of 4114 patients with acromegaly stored on the online system in February 2019. RESULTS: Based on the data 32% of patients had complete clinical and laboratory remission of acromegaly; the percentage of patients with no remission was 68%, among them 22.5% had significant improvements in clinical symptoms and a decrease in growth hormone (GH) and insulin-like growth factor-1 (IGF-1) without IGF-1 normalization. The average age of patients at the onset of the disease was 42.7 years and at diagnosis – 45.8 years. The ratio of men to women was 1:2.6. In patients with acromegaly hypopituitarism was registered in 14.7% of cases and among them hypothyroidism (66%) and hypogonadism (52%) were registered more often. Among other complications the leading were diabetes mellitus (15.7%) and acromegalic arthropathy (15%). The proportion of patients receiving neurosurgical treatment increased from 35.7% to 49.6% in 2012–2019; the portion of patients undergoing radiation therapy decreased significantly from 17.7% in 2012 to 0.8% in 2019. Remission was achieved in 40.47% after neurosurgery and 28.95% after medical treatment as a first line therapy p<0.01. The number of patients receiving medical treatment at the time of the study was 1209. Among them 51% of patients treated with long-acting lanreotide and 24% receiving long-acting octreotide achieved remission (p<0.0001) CONCLUSIONS: The remission rate of acromegaly remains suboptimal despite increased surgical activity, which corresponds to global trends. Long-acting lanreotide was significantly superior versus long-acting octreotide in the rate of acromegaly remission, which does not correspond with clinical trials.

The response of corticotropin and adrenal steroids to desmopressin stimulation in patients with various forms of hypercortisolism.

OBJECTIVE: The purpose of this study was to evaluate the direct action of desmopressin (agonist of vasopressin) on the hypophysis and the three zones of the adrenal cortex in patients with different forms of hypercortisolism. DESIGN: Forty-three patients with hypercortisolism-21 with Cushing's disease (14 females, 7 males), 11 with extrapituitary, ectopic tumours (5 females, 6 males), and 11 with ACTH-independent Cushing's syndrome (6 females, 5 males)-were evaluated. The response of the pituitary and adrenal glands was assessed by measuring plasma levels of adrenocorticotropic hormone (ACTH), cortisol, aldosterone, and dehydroepiandrosterone sulfate (DHEAS) at baseline and at 15, 30, 60, 90, and 120 min after the administration of desmopressin. RESULTS: We observed two main modes of secretory response: (1) elevation of the ACTH level followed by a rise of one, two or all three adrenal steroids, and (2) ACTH-independent elevation of adrenal steroids in various combinations. CONCLUSION: In a number of patients with hypercortisolism, the adrenal cortex responded to desmopressin administration by enhanced synthesis and secretion of glucocorticoids (cortisol), mineralocorticoids (aldosterone), and adrenal androgens (DHEAS) without a concomitant rise in ACTH. These findings suggest the presence of "ectopic" vasopressin receptors in the human adrenal cortex.