Vol 59, No 3 (2013)

The scheme of preoperative preparation of the patients presenting with pheochromocytoma
Bel'tsevich D.G., Lysenko M.A., Kuznetsov N.S., Kats L.E., Vanushko V.É., Soldatova T.V., Iukina M.I., Gazizova D.O., Mel'nichenko G.A.
Abstract
The results of analysis of mortality and probability of the development of "uncontrollable hemodynamics" syndrome were used to estimate the necessity of preoperative preparation for the patients with pheochromocytoma to be exposed to different treatment strategies at different time periods. The scheme of preoperative preparation described in this paper is based on the experience with the application of two different regimens of preoperative prescription of alpha-adrenoblockers to 106 patients.
Problems of Endocrinology. 2013;59(3):3-7
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The levels of leptin, adiponectin, and free fatty acids in the patients of different body weight presenting with myocardial infarction and elevated ST segment
Kvitkova L.V., Borodkina D.A., Gruzdeva O.V., Barbarash O.L., Silonova A.A., Terletskaia O.S.
Abstract
The present study involed the patients (n=100) presenting with myocardial infarction (MI). Based on the body mass index, they were allocated to three groups: those with normal body mass index (BMI) (18.5≤BMI< 25 kg/m2; n=32; group 1), overweight patients (25≤BMI <30 kg/m2; n=42; group 2), and obese patients (BMI≤30 kg/m2; n=27; group 3). The laboratory studies included the measurement of serum adipocytokine levels (leptin, adiponectin, and free fatty acids (FFA)) in conjunction with the evaluation of insulin resistance (IR). All the patients regardless of BMI had the waist circumference in excess of the upper limit of the normal gender-specific values. Deviations from the reference values of leptin, adiponectin, and FFA levels were observed in 65.6% of the patients with normal BWI, in 69.0% of the overweight patients, and in 70.3% of the obese patients. In the patients of all the three groups, significant correlation was documented between waist circumference and the levels of leptin (group 1: r=0.3100, p=0.00; group 2: r=0.32, p=0.00; group 3: r=0.37, p=0.03) and adiponectin (group 1: r=-0.43, p=0.00; group 2: r=-0.35, p=0.04; group 3: r=-0.18, p=0.01). Moreover, the waist circumference significantly correlated with the occurrence of IR (group 1: r=0.11, p=0.04; group 2: r=0.45, p=0.00, group 3: r=0.34, p=0.03). It is concluded that the observed deviations of the parameters of interest from the respective reference values suggest disturbances in the metabolic and secretory functions of the visceral adipose tissue associated with the enlargement of its volume).
Problems of Endocrinology. 2013;59(3):8-12
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Quality of life indices and psychoemotional status of the patients presenting with diffuse toxic goiter during the long-term follow-up after iodine radiotherapy
Dreval' A.V., Nechaeva O.A., Garbuzov P.I., Mamedova T.R., Chikh I.D., Krylov V.V.
Abstract
A total of 60 patients given substitution treatment with levothyroxin for diffuse toxic goiter were examined within 3-7 years after iodine radiotherapy. The patients showing different TSH levels were allocated to three groups: (group 1: low normal TSH level: 0.4-2.25 mcIU/ml; group 2: high normal TSH level: 2.6-4.0 mcIU/ml; group 3, decompensated hypothyroidism: above 4.0 mcIU/ml but lower than 10 mcIU/ml). The quality of life was assessed with the use of the abridged version of the SF-36 questionnaire and the psychoemotional status based on the Beck depression inventory and the Spilberg-Khanin test. The patients presenting with compensated hypothyroidism showed better characteristics of the quality of life compared with the patients of other groups. Moreover, they were less frequently subjected to anxiety and depressive disorders. It is concluded that replacement therapy with levothyroxin designed to maintain the TSH level within the low normal values ensures a higher quality of life and lower incidence of depressive disorders than in the patients having TSH levels closer to the upper normal limit.
Problems of Endocrinology. 2013;59(3):13-18
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Pheochromocytoma/paraganglioma: clinical and genetic aspects
Iukina M.I., Troshina E.A., Bel'tsevich D.G., Tiul'pakov A.N., Lysenko M.A.
Abstract
The present retrospective descriptive and analytical study included 167 patients divided into three groups. Two groups were comprised of the patients with genetically predetermined pheochromocytoma (type 2 MEN syndrome and von Hippel-Lindau disease respectively), the third croup consisted of the patients presenting with sporadic pheochromocytoma. The median age at which pheochromocytoma was diagnosed was 25 years in group 1, 18 years in group 2, and 47 years in group 3. Adrenalin-type secretion was predominated in group 1 in contrast to the noradrenalin-type and mixed-type secretion in the patients of groups 2 and 3 respectively. The patients of group 1 showed positive correlation between the tumour size and excretion of methylated catecholamines. 82% of these patients exhibited bilateral adrenal lesions and 58% suffered multifocal lesions; no cases of extra-adrenal localization of the tumour were documented. The extra-adrenal localization of pheochromocytomas was observed in 16.4% of the patients in group 2, where 6.6% of the patients had metastases. No metastases of pheochromocytoma were found in the patients of groups 1 and 3. It is concluded that the patients under the age of 40 presenting with bilateral and multicentral pheochromocytoma with the aggravated familial history need the genetic predetermination of the disease to be excluded taking into account syndromal symptoms. Examination for the detection of type 2 MEN is not required in the patients showing isolated normetanephrin excretion. Nor is the search for metastases of pheochromocytoma and its extra-adrenal localization is necessary in the patients presenting with type 2 MEN syndrome and/or exhibiting isolated metanephrin excretion.
Problems of Endocrinology. 2013;59(3):19-26
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Proliferative activity of the epithelial sheet of the mucous membrane in the lower airways during experimental diabetes mellitus
Pivovarova O.A., Man'kovskiĭ B.N.
Abstract
The present study was designed to develop the experimental model of diabetes mellitus based on 5-6 month-old Wistar rats weighing 234.00±2.64 g (n=47). Diabetes was induced by a single intraperitoneal administration of streptozotocin (60 mg/kg, "Sigma", USA) in a 0.1 M citrate buffer solution, pH 4.5. The control group was comprised of 43 intact male rats. The animals with experimental diabetes had a reduced number of secretory cell nuclei per unit area of the epithelial sheet of the bronchial tree; the area of epitheliocyte nuclei also decreased.
Problems of Endocrinology. 2013;59(3):27-29
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Familial isolated glucocorticoid deficiency: three clinical cases and a brief overview of the current literature
Orlova E.M., Kareva M.A., Melikian M.A., Tiul'pakov A.N., Peterkova V.A.
Abstract
Familial isolated glucocorticoid deficiency (FIGD) is a rare form of primary adrenal insufficiency associated with the resistance of the adrenal cortex to the action of ACTH. This pathology manifests itself in the form of cortisol deficiency despite the normal mineralocorticoid function. Approximately 50% of all FIGD cases are attributable to mutations in two genes (melanocortin 2 receptor gene, MC2R, and melanocortin 2 receptor accessory protein gene, MRAP). In addition, the relationship between FIGD and mutations in nicotinamide nucleotide transhydrogenase gene, NNT, minichromosome maintenance gene, MCM4, and STAR is documented. No genetic defect was found in almost 25% of the cases of isolated deficiency of gucocorticoids. The clinical descriptions of the patients presenting with FIGD including its molecular-genetic diagnostics are of great interest for the understanding of this rare disease.
Problems of Endocrinology. 2013;59(3):30-35
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The non-classical form of congenital adrenal cortical hyperplasia due to 11Β-hydroxylase deficiency: the description of three clinical cases
Ionova T.A., Zubkova N.A., Tiul'pakov A.N., Nizhnik A.N., Savel'eva L.V.
Abstract
The non-classical form of congenital adrenal cortical hyperplasia due to 11Β-hydroxylase deficiency (11Β-OH CAH) is the second most frequent non-classical form of CAH. Mutations in the CYP11B1 gene characteristic of this pathology are responsible for the slight reduction in the 11Β-hydroxylase activity manifested as the "soft" phenotype. They make it difficult to distinguish 11Β-OH CAH from similar clinical conditions. There are occasional publications in the world literature describing patients with genetically confirmed 11Β-OH CAH. No such data can be found in the Russian-language literature. We have described three clinical cases of 11Β-OH CAH caused by mutations in the CYP11B1 gene. Our observations showed that the presence of the signs of hyperandrogenism (regardless of the patients' age and sex) in combination with arterial hypertension or without it as well as the marked increase in the baseline and/or stimulated levels of 11-deoxycortisol gives reason to suspect non-classical variant of 11Β-hydroxylase deficiency. The molecular-genetic study revealed mutations in the CYP11B1 gene in all the examined patients.
Problems of Endocrinology. 2013;59(3):36-44
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The risk of development of perioperative myocardial lesions and acute left ventricular insufficiency in the patients presenting with pheochromocytoma
Kuznetsov A.B., Bel'tsevich D.G., Lysenko M.A., Kats L.E., Gomzhin I.S., Iukina M.I.
Abstract
Differential diagnostics of coronary heart disease (CHD) and catecholamine-induced cardiomyopathy was undertaken in 19 of the 106 patients presenting with pheochromocytoma. Group 1 included 13 patients having the history of acute myocardial infarction. Diagnostics of CHD was performed following the adequate preoperative alpha-blockade (supplemented by Β-blockade whenever indicated). Group 2 was comprised of 6 patients suffering pulmonary oedema (normotensive, 10-70 min after removal of the tumour) and showing the elevated levels of biomarkers of myocardial necrosis and/or ischemic-type changes of ECG in the early postoperative period. The clinically significant myocardial ischemia requiring correction was documented only in one of the 19 patients. The two groups were not significantly different in terms of the mean tumour size and duration of surgery, the quality of preoperative compensation, and intraoperative fluctuations of arterial pressure. The mean age of the patients in group 1 (47 years) was much higher than that in group 2 (29 years). All patients in group 2 exhibited the predominantly adrenalin-type secretion whereas 10 patients of group 1 had the noradrenalin-type secretion and two others mixed-type secretion with the predominance of noradrenalin release. It was shown that the patients with the predominantly adrenaline-type secretion by the tumour were at higher risk of pulmonary oedema. The possibility of application of alpha-methylparatyrosine having beneficial effect on contractile activity of myocardium as the main agent for preoperative preparation is discussed.
Problems of Endocrinology. 2013;59(3):45-50
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Risk factors of impaired fertility in the patients presenting with congenital adrenal cortical hyperplasia
Kopylova I.V., Kareva M.A.
Abstract
The analysis of publications concerning the impairment of fertility in the patients presenting with congenital adrenal cortical hyperplasia was focused on the reduction of the degree of estrogenization. It included the data on the course of sexual development and the outcomes of feminizing plastic procedures in the girls suffering this disease depending on the degree of its compensation and the date of onset of the treatment.
Problems of Endocrinology. 2013;59(3):51-56
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Glibenclamide: proven facts, perspectives, discussions
Chepliaeva N.I.
Abstract
Despite the discussion about cardiovascular risk glibenclamide has proved its efficacy and safety by 40 years of clinical practice. Basing on numerous experimental publications and continued clinical investigations, perspectives of glibenclamide's use in treatment of traumatic and ischemic injuries of CNS are presented.
Problems of Endocrinology. 2013;59(3):57-62
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