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[Endocrinologic disorders in deletion of chromosome 18].

Patients suffering from deletions of chromosome 18 (p-, q-) show regularly short stature. Endocrinological investigations were performed to prove if short stature is due to pituitary insufficiency. In three female patients with deletions of chromosome 18 and retarded bone age serum growth hormone was investigated after insulin induced hypoglycemia, after glucagon-propranolol and after stimulation with growth hormone releasing hormone. Thyroid function, gonadal function and adrenal function were investigated too. All three patients showed growth hormone deficiency. In one patient there were found in addition hypothyroidism and gonadotrophine deficiency as well. In conclusion growth failure in some patients with deletions of chromosome 18 seems to due to pituitary insufficiency. In these patients treatment with recombinant growth hormone may increase growth velocity.

Child, Preschool↗

[Adrenal incidentaloma as essential clinical problem in modern endocrinology].

Adrenal mass discovered during imaging for non-adrenal related causes is called incidentaloma. Adrenal masses can be asymptomatic or can have clinical manifestations related to hormone overproduction. The increasing number of discovered adrenal mass is by no doubt due to quick development of radiology procedures. The aim of study was determination of statistical frequency of different types of adrenal tumours and related clinical symptoms based on retrospective analysis of patients diagnosed in the Department of Endocrinology, Collegium Medicum, Jagiellonian University. There were 271 patients (184 female, 87 male) with incidentaloma confirmed in CT scan from January 2000 to June 2005. Mean patients' age was 56.8 +/- 12.7 years. The most common localisation was right adrenal gland (135 cases), bilaterally in 29 patients. Tumours less than 3 cm in diameter were observed in 58.5% of diagnosed persons, tumours from 3 to 6 cm in 33.3% of cases and tumours bigger than 6 cm in 8.2% patients. Hormone excess associated with adrenal mass was detected in 37 (13.6%) patients. Pheochromocytoma was diagnosed in 20 patients (7.4%). Subclinical hypercortisolism was found in 13 patients (4.8%). Primary hyperaldosteronism was diagnosed in 4 cases (1,4%). In patients with adrenal mass incidentaloma hormone tests should be performed before making therapeutic decisions despite no clinical symptoms.

Adrenal Gland Neoplasms↗

[Relation between endocrinology and psychiatry. Some recent developments].

Relations between Endocrinology and Psychiatry are developing in two main directions: Behavioural effects of hormones. After the effects of peripheral hormones, recent works are concerned with A.C.T.H. and Antidiuretic Hormone (which interfere with memory process) and with Hypothalamic Peptides (which might have psychotropic properties, independant of their endocrine regulation function). -Correlations between psychiatric syndroms and endocrine disorders. In some mentally depressed patients, disorders of the hypothalamic control of Coricotrophin, Growth Hormone and Thyreo-stimulating Hormone, have been observed. As many psychotropic drugs can influence the secretion of Prolactin, a systematic survey of this hormone in various mental illnesses, is suggested.

Adrenocorticotropic Hormone↗

[Incidentaloma--one of the greatest challenges of modern endocrinology].

Technological advance of the recent years has contributed to the development of the situation during which clinically inapparent masses are discovered in endocrine organs by high-resolution radiological imaging procedures that have been performed for other reasons. Most of these lesions, frequently referred to as incidentalomas, are clinically insignificant benign and hormonally inactive adenomas that neither pose a risk to a patient's health nor warrant the risks of further diagnosis and treatment. One of the greatest challenges of modern endocrinology is to distinguish the vast majority of clinically insignificant changes from other masses requiring further management such as hormone-secreting tumours and malignant lesions. Over the years a myriad of the diagnostic and therapeutic approaches has been recommended and presently there are still no uniform guidelines. The purpose of his article is to give an overview on the recent advances in diagnosis and management of pituitary and adrenal incidentalomas. We provide the reader with practical recommendations and underline areas in which further studies are required.

Adrenal Gland Neoplasms↗

SPECT-CT fusion: a new diagnostic tool for endocrinology.

INTRODUCTION: The introduction of the DICOM format in all diagnostic imaging devices allowed co-registering SPECT, CT, MR and other types of biomedical imaging. Fusion can be performed by dedicated hybrid devices or by means of software. The fusion algorithm consists of two steps: coregistration and simultaneous visualization. Our center gradually implemented SPECT-CT fusion in clinical diagnostic work-up of several endocrinologic and oncologic diseases more than 2 years ago. MATERIAL AND METHODS: An easy and fast algorithm in terms of computational complexity of image fusion was presented and applied to 81 consecutive cases. Thirty-two patients were scheduled to SPECT-CT fusion after thyroidectomy and (131)I treatment for thyroid cancer, twelve after somatostatin receptor scintigraphy, seven after 131I MIBG therapy, six after diagnostic MIBG scintigraphy with (123)I or (131)I, three after parathyroid scintigraphy and two after bone scan. The most common indication to the fusion was the need of metabolic characterization of suspected lesions detected on CT scan. The anatomic localization of a focal uptake seen on SPECT and the evaluation of the radiometabolic therapy effect folloved. RESULTS: A variance of error level observed was a result of human factor, decision on marker's placement, respiratory movements and marker's displacement between acquisitions. However, 74% of patients in our series have fusion results classified as "very good" or "good". CONCLUSIONS: The selection of patients, the training of the personel and the cooperation with radiologists are the most important factors for a correct application and interpretation of the SPECT-CT image fusion.

Algorithms↗

Reversibility of age associated NK defect by endocrinological and/or nutritional intervention.

Several abnormalities arise at the level of NK cell function in both rodents and humans with increasing age. The age-related changes are not irreversible alterations since they can be reversed either by endocrinological or nutritional approaches, suggesting that age-related microenvironmental changes may play a relevant role in the age-related immune deterioration. Whether endocrine and nutritional factors have an additive effect or act through the same intracellular mechanisms remains to be established, though the first possibility seems more likely since the action of TSH and thyroid hormones is specifically directed towards lymphokine-boosted NK activity, while AL are able to prevent age-associated defect of basal NK cytotoxicity.

Aging↗

[Endocrinologic function following cranial irradiation in acute lymphoblastic leukemia in childhood].

Endocrinological function was evaluated in 31 children after successful treatment of acute lymphoblastic leukaemia. All patients had received combination chemotherapy and 12-24Gy of cranial irradiation according to the German therapy protocols BFM-81, BFM-83 and BFM-86. Height, weight, bone age and pubertal development, as well as hypothalamic-pituitary function were measured. Long-term linear growth was unaffected in all patients. However, 9 patients showed subnormal serum growth hormone levels in response to pharmacological stimulation of the pituitary. All patients had normal levels of T3 and T4, but one patient showed an increased response of thyrotropin to thyrotropin releasing hormone. All prepubertal and postpubertal children demonstrated appropriate secretion of follicle-stimulation hormone (FSH) and luteinizing hormone (LH) after stimulation with LH-releasing hormone (LH-RH). 3 pubertal girls showed adequate oestradiol levels, but abnormally high levels of gonadotropins in response to LH-RH. Sexual development was normal in two of them, but the third had a late menarche and irregular menses. The significance of these findings is discussed in the context of recommendations possibly to further reduce or completely delete prophylactic cranial irradiation.

Adolescent↗

Studies on varicocele. 1. Submicroscopical and endocrinological features.

In this study we have selected a group of patients affected by a more or less severe condition of varicocele. After the evaluation of spermatogenesis and sperm function by electron microscopy we have demonstrated that the sperm malformations are mostly due to immaturity. Subsequently we have observed low FSH levels in the blood, concomitant with inhibin high contents, and we have studied Sertoli cells at submicroscopical level. In conclusion we suggest the following mode of action of varicocele in endocrinologically and spermatologically altered patients: varicocele----Sertoli cells----increased inhibin----hypophysis----decreased FSH----decreased testosterone----aberrant spermatogenesis----immature spermatozoa. The research will continue.

Adult↗

[Endocrinology 1990-1991].

The author presents an account of selected important findings in endocrinology during the last year. The mediator of action of STH, IGF-I, was tested as a protein anabolic in a child with Laron's nanism. STH is about to be tested as a geriatric drug. A non-peptide vasopressin antagonist was described. Melatonin is being tested in sleep disorders. A combination of methimazol and thyroxine is more suitable for treatment of Graves-Basedow's disease than the goitrogen alone. Dermal vasoconstriction can be an indicator of the effectiveness of glucocorticoids in asthma. Significant advances were made in the sphere of the new hormone, NO: it is not only an effective vasorelaxing agent but also a neuromodulator and participates in the natural lymphocytic cytotoxicity. Marked advances were made as regards knowledge of the endogenous ligand for benzodiazepine receptors.

Endocrine System Diseases↗

[Radioimmunological measurement methods in ambulatory endocrinology].

Measurements of plasma-hormone concentrations can be helpful for differential diagnosis in clinically suspect endocrine disorders. By means of radioimmunological methods a series of hormones can be estimated in small plasma volumes. The assay technique differs from chemical methods routinely used in a clinical laboratory. Reagents and details of immunoassay procedures vary between laboratories, and hence hormone estimations from different institutes cannot be directly compared. The plasma half-life of many hormones is 10-30 minutes. The regulation of hormone secretion is complex. Dynamic tests using a known stimulation or suppression provide a better estimate of the function of an endocrine axis than a single random measurement. Hormone assays should be required and interpreted in close collaboration with the endocrine laboratory, which should provide competent counseling with respect to methodologic and endocrinologic aspects.

Adrenocorticotropic Hormone↗

[Endocrinology of the chiasma syndromes (author's transl)].

A wealth of new knowledge about the function of the hypothalamo-hypophyseal system has been accumulated over the past ten years and progress keeps going at a fast pace. Several factors have contributed to this explosive growth: Pituitary hormones can now be measured by radio-immunoassay in body fluids, hypothalamic stimulating and inhibiting factors have been identified and are available for clinical studies and dynamic tests have been developed for detailed investigations of minor disturbances in the pituitary control system. A tremendous gain in accuracy of clinical diagnosis and endocrine follow-up is one of the consequences. Furthermore, new clinical pictures have emerged such as several variants of the hyperprolactinemia-hypogonadism syndrome or the concept of pituitary microadenoma. At the same time, neuropharmacology is also developing at a tremendous pace and exceedingly interesting perspectives linking neurotransmission with endocrinology and with various diseases of the brain are emerging. One highlight is the discovery of bromoergocryptine as a dopaminergic agonist, active in suppressing prolactin secretion as well as in treating Parkinsonism. In a second part of this presentation 5 cases with pituitary disease are presented. They were selected to cover part of the wide range of clinical signs and symptoms produced by tumours of the pituitary region. There is indeed no good relationship between ophthalmo-neurologic symptoms, destruction of the bony sella and endocrine disturbances.

Adenoma, Acidophil↗

[Diagnosis of adrenal diseases focusing on morphological and endocrinological viewpoints].

The recent advances in the diagnosis of adrenal diseases owe much to the rapid progress in radiological and endocrinological areas. The former is ascribed to the progress of medical electronics. The latter has been realized by the development, improvement and spread of the measurement of a very small amount of hormones or hormone-like substances in blood, urine or tissues, supported by the improvement of measurement instruments as well as the immunoassay or chemical assay techniques. This paper reviews approaches to diagnosis of adrenal diseases causing hypertension and asymptomatic, incidentally discovered adrenal tumors. Incidentally discovered adrenal tumors have increased owing to the spread of medical electronic instruments such as abdominal ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI). Discrimination of malignancy is an important clinical concern. More than 90% of the tumors reported in the Japanese were larger than 3 cm in diameter. The diagnosis of primary aldosteronism has been made by the measurement of plasma renin and aldosterone, CT and 131I-cholesterol scintigraphy. However the differential diagnosis of adrenal adenoma from bilateral adrenal hyperplasia has remained as a problem. There were a few patients in whom adrenal adenomas appeared after 2-3 years' follow-up period. In Cushing's syndrome, tumors are found with ease by US, CT and MRI. The diagnosis of pheochromocytoma is made mainly by urinary catecholamines and metabolites including metanephrine, normetanephrine and VMA. US, CT and MRI are very useful. Diagnosis and discovery of metastasis will be more reliably made when 131I-MIBG comes to the clinical stage.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Gland Diseases↗

[Thyroid irradiation doses and organization of endocrinological monitoring of the inhabitants of the Ukrainian S.S.R. after the accident at the Chernobyl AES].

The paper is devoted to one of the most important aspects of the accident at the Chernobyl Nuclear Power Station: thyroid irradiation in the residents of 8 areas in the Ukrainian SSR and the town of Pripyat, affected with radioactive iodine. Irradiation doses, prognosis of medical effects of the accident, methods and practical measures for the organization of endocrinological monitoring of the affected population were thoroughly considered. The authors proposed a scheme of health care of the population in these areas. They also discussed the structure of data bases for analysis of medico-dosimetric information on "iodine" effects of the accident. The main trends in medico-dosimetric problems of the effects of thyroid irradiation were outlined.

Accidents↗

[Endocrinological analysis of climacteric symptoms and gonadal dysfunction by CRF test].

Endocrinological studies on stressed climacteric women and some cases of gonadal dysfunction were carried out by analysing blood levels of adrenocorticotropic hormone (ACTH), beta-lipotropin (beta-LPH) and beta-endorphin (beta-EP) after the administration of synthetic corticotropin releasing factor (CRF, CRF test). Our results can be summarized as follows: 1. The responsiveness to CRF in perimenopausal and ovariectomized women rose and it corresponded with those of post gonadectomy in testicular feminization. 2. Responsiveness to CRF was decreased as estrogen levels decreased with the patient's age, and a similar tendency was observed in gonadal dysfunction. 3. Climacteric women with non-specific complaints have a higher responsiveness to CRF than that of postmenopausal women without complaints. Subjects with a high K.I score and with severe hot flush showed higher responsiveness to CRF than other subjects. These data suggest that CRF and its related hormones may be correlative with stress and hot flushes in climacteric periods and endogenous CRF may play an important psychosomatic role by regulating the hypothalamo-pituitary-adrenal function where there is decreased estrogen.

Adrenocorticotropic Hormone↗

[The role of insulin in reproductive endocrinology and perinatal medicine].

Soskin, in his 1946 textbook, stated that insulin may be regarded as the dominant instrument in the symphony of endocrine action that results in normal carbohydrate metabolism. After almost half a century, great progress in the medical field has revealed that insulin plays more than even he described. Some aspects of important actions of insulin in our field as investigated in our laboratory are summarized below. 1. Role of insulin in reproductive endocrinology. (1) Correlation of insulin and testosterone in normal young women and patients with polycystic ovary syndrome (PCO). The sum of serum insulin values during 75g OGTT and serum testosterone values were positively correlated in normal women and patients with PCO. Glucose transport activities in isolated adipocytes from a typical PCO patient were decreased, but insulin binding activities were not, which indicates that insulin resistance in this patients is due to some post-receptor defects. (2) Insulin may be a risk factor of endometrial carcinoma. It is well-recognized that several diseases associate with hyperinsulinemia, such as obesity, PCO, diabetes mellitus, and hypertension are risk factors for endometrial carcinoma. The sum of the insulin values during OGTT was significantly higher in patients with endometrial carcinoma than in those without. 2. Role of insulin in perinatal medicine. (1) Increase in insulin secretion during pregnancy. High serum insulin concentration during OGTT, increased secretion of urinary C-peptide, and enhanced staining of insulin in B cells by the PAP method suggest that insulin secretion is enhanced during pregnancy. (2) Insulin resistance during pregnancy. Glucose utilization rate in both pregnant and progesterone-treated rats, as assessed by a glucose clamp technique, is significantly decreased as compared to nonpregnant rats. The technique of 2-deoxyglucose injection revealed that whole body insulin resistance is due to insulin resistance in individual insulin-sensitive tissues. The activities of 3-0-methyl-D-glucose transport in isolated rat skeletal muscle and human adipocytes were found to decrease during late pregnancy, but insulin binding activities were not. These results suggest that insulin resistance during pregnancy is due to some post-receptor mechanisms. (3) Physiological meaning of insulin in fetal growth.(ABSTRACT TRUNCATED AT 250 WORDS)

Embryonic and Fetal Development↗

Endocrinologic and metabolic manifestations of the acquired immunodeficiency syndrome.

The endocrine abnormalities associated with acquired immunodeficiency syndrome (AIDS) are reviewed. These include adrenal insufficiency, hyporeninemic hypoaldosteronism, panhypopituitarism, hypogonadism, and alterations in thyroid function tests. AIDS-related infections or neoplasms may lead to hypercalcemia, whereas malabsorption may cause hypocalcemia. The possibility that AIDS-associated cachexia and hypertriglyceridemia may be caused by cachectin (tumor necrosis factor) is discussed, along with possible therapy for cachexia with megestrol acetate. Ketoconazole, sulfonamides, and pentamidine have specific, potentially deleterious metabolic effects when used in AIDS patients. Because treatment of endocrinological abnormalities of AIDS is often effective, improved diagnosis and appropriate therapy of these abnormalities will result in improved quality of life and, possibly, longer survival of patients with AIDS.

Acquired Immunodeficiency Syndrome↗

[The nuclear magnetic resonance in endocrinology. Spectroscopy and imaging, present and future].

The first part, after a brief historical reminder, gives an oversimplified and non-rigorous approach to the Nuclear Magnetic Resonance (NMR) phenomenon; this "physical" introduction leads the clinician to know the essential about the spectral and imaging techniques in NMR, just to understand what is done... The second part of this paper shows some medical applications of these two aspects of NMR, with help of some examples, taken from the literature, according to what is concerned with endocrinology. Pure bio chemical applications (enzymology), or those concerning isolated (and perfused) organs, or living animals, and even human beings, are numerous and complementary, and there is no doubt that the applications of NMR technologies to medicine will know a promising future...

Animals↗

[Transitory precocious puberty caused by autonomous ovarian cysts. Clinical, endocrinologic and sonographic course].

Precocious pseudopuberty in girls may be due to autonomous ovarian cysts. The spontaneous course in two girls aged 3.5 and 5.75 years is described. For differential diagnosis of the precocious pubertal development the following parameters were particularly helpful: growth curves, bone age determinations, repeated LHRH tests and serial sonographic examinations of ovaries and uterus. Since spontaneous regression of pubertal signs could be seen after a few weeks with complete resolution of the ovarian cysts and endocrinological parameters returning to normal after a few months, laparotomy and ovariectomy could be avoided in these two patients.

Body Height↗