Aims: this study evaluates the most common associations of symptoms and complications in patients with Cushing’s syndrome (CS) in order to choose a potential population to be screened for CS and estimates the diagnostic accuracy of first line screening tests (cortisol, ACTH) to differentiate ACTH-ectopic CS from Cushing’s disease. Materials and Methods: The clinical data of 259 patients with proven CS during 2001–2011 was analyzed. The clinical presentations of 197 patients (159 Cushing’s disease, 28 ACTH-ectopic CS and 10 cases of benign cortisol-secreting adrenal adenoma) were compared according to the cause of hypercortisolism. ROC-analysis was performed to estimate the diagnostic accuracy of the first line tests (cortisol, ACTH) to suggest ACTH-ectopic CS. A threshold for the test with the highest area under the curves was chosen based on the maximum sum of the sensitivity and specificity. Results: The most frequent complaints were related to fatigue, muscle weakness, weight gain and changes in appearance (facial plethora and fullness, striae). Among the complications of CS the most frequent were being overweight or obese (71%), hypertension (63%), dislipoproteinemia (41%), low traumatic fractures (43%) and steroid-induced diabetes (31%). In women, 16% were older than 50, in those who were younger amenorrhea was registered in 43%. The patients with ACTH-ectopic CS had higher rate of low traumatic fractures (p=0.04), increased serum late-night cortisol, 24 hours urinary free cortisol, morning and evening ACTH and lower levels of potassium (p0.01 for all parameters). Plasma late-night ACTH measurements showed the highest AUC (0,811 (95% CI 0,712–0,909)) to differentiate ACTH-ectopic CS from Cushing’s disease. A cut off value of 108.9 pg/ml for late-night ACTH yielded a sensitivity of 60,7% and a specificity of 79%. Conclusions: patients with a coexistence of obesity, muscle weakness, fatigue, some components of metabolic syndrome and especially low traumatic fractures should be screened for CS. High plasma late night ACTH values in patients with proven CS value suggest ACTH-ectopic syndrome.
Background: The registry is the main source of information about patients with acromegaly for assessing the quality of medical care, treatment effectiveness, determining the compliance of real clinical practice with existing standards and patient management. Aims: To analyze epidemiological, demographic and clinical characteristics of acromegaly in the Russian Federation and the effectiveness of various treatment methods. Materials and methods: The object of the study was the database of 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; percentage of patients with no remission was 68%, among them 22.5% had significant improvements in clinical symptoms and a decrease in GH and IGF1 without IGF1 normalization. The average age of patients at the onset of the disease was 42.7 years and at diagnosis 45.8 years. Men to women ratio 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 percentage 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 p0.01. 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 (p0.0001) 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 and can be explained by the usage of different generic forms of octreotide, regional differences in medical supply and difficulties in long-acting octreotide injection vs lanreotide.
The term ~empty" sella turcica (EST) was first introduced by Bush in 1951, when in describing the data of 788 autopsies, the diaphragm of the sella was completely lacking in 5.5% of cases, and the hypophysis was spread out on the floor in the form of a thin lamina. This being the case, the sella appeared empty. As a disease, the EST was described in 1962 in patients subjected to surgical or radiation therapy because of endosellar adenoma of the hypophysis. It was later related to the syndrome of secondary EST. Primary EST is characterized by the presence of insufficiency of the diaphragm of the sella turcica with a decrease in the volume of the hypophysis and expansion of the intrasellar subarachnoid space. There are reports in the literature touching on the anatomical, roentgenological, and clinical manifestations of this pathological state [4,7,8]. However, the diagnosis of EST is complicated, since the clinical-roentgenological changes are insufficiently specific and may imitate an endosellar tumor of the hypophysis. The pneumocisternography and computerized tomography with preliminary contrasting that are used today [7,9] are invasive methods and are associated with a substantial radiation burden.One of the most promising methods in solving the problem of the recognition of EST is the new noninvasive method of endoscopic diagnosis, tomography based on the phenomenon of nuclear magnetic resonance, magnetic resonance tomography (MRT). The method makes it possible to carry out an investigation without the use of ionizing radiation or the introduction of iodide or any other contrast agents; the investigation can be carried out multiply, over the course of time, in the three mutually perpendicular planes; it has the Department of Endocrinology, I. M. Sechenov Moscow Medical Academy; Laboratory of Magnetic Resonance Tomography of the Cardiological Scientific Center, Russian Academy Medical Sciences, Moscow. capacity to differentiate soft tissue, and makes it possible to simultaneously obtain images of various organs and systems [1-3, 6, 11]. There are only individual reports relative to the use of MRT for the diagnosis of EST, which, in addition, are based on a small number of clinical observations, have highly contradictory data, relate mainly to secondary 9 EST following operative treatment of adenomas of the hypophysis, or include descriptions of patients with EST out of relationship with endocrinological symptomatology [5].The aim of this study was the determination of the capacities of MRT in the diagnosis of EST. 229 MATERIALS AND METHODSOne hundred and ten patients with the most prevalent diseases of the hypothalamohypophyseal system were examined. The distribution of the patients with the respect to nosologic forms is presented in Table 1.The diagnostic formulations were based on a comprehensive clinical-laboratory investigation, including x-ray craniography in two projections, investigation of the level of hormonal markers specific for the particular diseases (prolactin, TI'H, ACTH, STH, thyroid and adrena...
The authors analyze the results of many-year application of magnetic imaging to the diagnosis of volume diseases of the adrenals. The method may be effectively used for topical diagnosis of such conditions. Due to some of its characteristics, such as clear differentiation of soft tissues, possibility of examination in 3 and more projections, safety, etc., magnetic imaging may be considered a method of choice in the diagnosis of adrenal tumors. It provides valuable information on the size, shape, localization, and dissemination of adrenal tumors and in some cases differentiates the variants of anatomical norm of the adjacent organs simulating adrenal tumors shown by other topographic methods. Features of magnetic image of tumors different by their hormonal activity: aldosteroma, pheochromocytoma, corticosteroma, "incidentaloma", and malignant tumors are described. Use of paramagnetic contrast agents appreciably improves the quality of magnetic resonance tomographic diagnosis of adrenal tumors, for it permits a more reliable conclusion on the tumor structure and its position amidst the adjacent vascular structures (abdominal aorta and inferior vena cava) and the parenchymatous organs (liver and kidneys), which is particularly valuable for planning surgery.
Sagliker syndrome is a rare complication of renal osteodystrophy, characterized by severe skeletal and cranium deformities, neurologic and soft tissue abnormalities in patients with chronic renal failure (CRF) and untreated secondary hyperparathyroidism. This article reports a 29-year-old female patient with end-stage CRF after 9 years of hemodialysis. She had severe secondary hyperparathyroidism, hyperplasia of three parathyroid glands and cranium and skeletal bone structure deformation. The first changes appeared after 4 years of therapy with peritoneal dialysis. They included uglifying face appearances, short stature, severe maxillary changes, chest deformity. During the examination we revealed severe tomographical and X-ray changes: maxillary and mandibular hyperplasia, temporomandibular articulation changes, affected cheekbones, sphenoid bone and bones of the cranial vault, fingertip changes, vertebral body compression. Although surgical parathyroidectomy was effective at biochemical abnormalities, severe bone deformities were not regressed. This case highlights the importance of clinicians’ attention for early monitoring and appropriate treatment of secondary hyperparathyroidism in patients with end-stage CRF.
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