WorldWideScience

Sample records for adrenal cortex diseases

  1. The adrenal cortex and life.

    Science.gov (United States)

    Vinson, Gavin P

    2009-03-05

    The template for our understanding of the physiological role of the adrenal cortex was set by Hans Selye, who demonstrated its key involvement in the response to stress, of whatever origin, and who also introduced the terms glucocorticoid and mineralocorticoid. Despite this, from the late 1940s on there was certainly general awareness of the multiple actions of glucocorticoids, including effects on the thymus and immune system, cardiovascular system, water balance, and the CNS. For these reasons, and perhaps because in the early studies of the actions of individual steroids there was less clear-cut difference between them, there was some initial resistance to the use of these terms. Today they are universal and unchallenged. It can be argued that, with respect to the glucocorticoids, this term colours our perception of their physiological importance, and may be misleading. By taking evidence from disease states, emphasis is placed on extreme conditions that do not necessarily reveal normal physiology. In particular, evidence for the role of glucocorticoid regulation of gluconeogenesis and blood glucose in the normal subject or animal is inconclusive. Similarly, while highly plausible theories explaining glucocorticoid actions on inflammation or the immune system as part of normal physiology have been presented, direct evidence to support them is hard to find. Under extreme conditions of chronic stress, the cumulative actions of glucocorticoids on insulin resistance or immunocompromise may indeed seem to be actually damaging. Two well-documented and long recognized situations create huge variation in glucocorticoid secretion. These are the circadian rhythm, and the acute response to mild stress, such as handling, in the rat. Neither of these can be adequately explained by the need for glucocorticoid action, as we currently understand it, particularly on carbohydrate metabolism or on the immune system. Perhaps we should re-examine other targets at the physiological

  2. [Lipid peroxidation in the adrenal cortex during exhausting stress].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Naumov, A V; Vinogradov, V V

    1990-05-01

    Under prolonged stress which is connected with exhaustion of functional resources of adrenal cortex the activation of lipid peroxidation processes in this gland was found. It is possible that the reason for such lipid peroxidation activation is the decrease in the content of adrenal cortex ascorbic acid and alpha-tocopherol.

  3. [Content of biological antioxidants and lipid peroxidation in the adrenal cortex in experimental circulatory insufficiency].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Nadol'nik, L I; Naumov, A V; Beluga, V B; Vinogradov, V V

    1991-01-01

    Thirteen-week experimental insufficiency in rats causes exhaustion of adrenal cortex function. The number of diene conjugates in the adrenals increased, the alpha-tocopherol level decreased. It is suggested that reduced level of antioxidant protection and the associated activation of lipid peroxidation processes underlie the disorders of adrenal cortex function.

  4. Quantification of the adrenal cortex hormones with radioimmunoassay

    Energy Technology Data Exchange (ETDEWEB)

    Badillo A, V.; Carrera D, A. A.; Ibarra M, C. M., E-mail: vbadillocren@hotmail.co [Universidad Autonoma de Zacatecas, Unidad Academica de Estudios Nucleares, Calle Cipres No. 10, Fracc. La Penuela, 98068 Zacatecas (Mexico)

    2010-10-15

    The pathologies of the adrenal cortex -adrenal insufficiency and Cushing syndrome- have their origin on the deficit or hypersecretion of some of the hormones that are secreted by the adrenal cortex, which is divided in three zones anatomically defined: the external zone, also called the zona glomerulosa, which is the main production site of aldosterone and mineralocorticoids; the internal zone, or zona reticularis, that produces androgens; and the external zone, or zone 1 orticotrop, which is responsible for producing glucocorticoids. In this work, a quantitative analysis of those hormones and their pathologic trigger was made; the quantification was made in the laboratory by means of highly sensitive and specific techniques, in this case, the radioimmunoassay, in which a radioisotope I-125 is used. This technique is based on the biochemical bond-type reaction, because it requires of a substance called the linker, which bonds to another called ligand. This reaction is also known as antigen-antibody (Ag-Ab), where the results of the reaction will depend on the quantity of antigen in the sample and on its affinity for the antibody. In this work, a 56 patients (of which 13 were men and 43 women) study was made. The cortisol, the ACTH, the androsterone and the DHEA values were very elevated in the majority of the cases corresponding to women, predominating cortisol; while in men, a notorious elevation of the 17 {alpha}-OH-PRG and of the DHEA-SO{sub 4} was observed. Based on that, we can conclude that 51 of them did not have mayor complications, because they just went to the laboratory once, while the remaining 5 had a medical monitoring, and they visited the laboratory more than one occasion, tell about a difficulty on their improvement. According to the results, an approximate relation of 8:2 women:men, respectively, becomes clear to the hormonal pathologies of the adrenal cortex. (Author)

  5. [Dynamics of lipid peroxidation and steroidogenesis in adrenal cortex during stress].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Vinogradov, V V

    1990-01-01

    The phase character of lipid peroxidation has been found in the rabbit adrenal cortex in the process of adaptation to extreme loads. Under acute stress the activation of lipid peroxidation is directly dependent on the hormonal synthesis processes. Under conditions of the prolonged stress factor an enhancement of the lipid peroxidation intensity in the adrenal cortex coincides with a decrease in the steroidogenesis rate.

  6. Aldo-keto reductases 1B in adrenal cortex physiology

    Directory of Open Access Journals (Sweden)

    Emilie PASTEL

    2016-07-01

    Full Text Available Aldose reductase proteins are cytosolic monomeric enzymes, belonging to the aldo-keto reductase (AKR superfamily. They perform oxidoreduction of carbonyl groups from a wide variety of substrates such as aliphatic and aromatic aldehydes or ketones. The Aldose reductase subgroup (AKR1B is one of the most characterized because of its involvement in human diseases such as diabetic complications resulting from the ability of its human archetype AKR1B1 to reduce glucose into sorbitol. However the issue of AKR1B function in non pathologic condition remains poorly resolved. Adrenal steroidogenesis is strongly associated with high production of endogenous harmful lipid aldehyde by-products including isocaproaldehyde (4-methylpentanal derived from cholesterol side chain cleavage (the first step of steroid synthesis and 4-hydroxynonenal (4- HNE that can both be reduced by AKR1B proteins. More recently, some AKR1B isoforms have been shown to be endowed with prostaglandin F synthase activity, suggesting that in addition to possible scavenger function, they could instigate paracrine signals. Interestingly, previous studies have established that the adrenal gland is one of the major site for human and murine AKR1B expression suggesting that their detoxifying/signaling activity could be specifically required for the correct handling of adrenal function. Moreover chronic effects of ACTH result in a coordinated regulation of genes encoding the steroidogenic enzymes and some AKR1B isoforms.This review presents the molecular mechanisms accounting for the adrenal specific expression of some AKR1B genes. Using data from recent mouse genetic models, we will try to connect their enzymatic properties and regulation with adrenal functions.

  7. Cushing's syndrome and fetal features resurgence in adrenal cortex-specific Prkar1a knockout mice.

    Directory of Open Access Journals (Sweden)

    Isabelle Sahut-Barnola

    2010-06-01

    Full Text Available Carney complex (CNC is an inherited neoplasia syndrome with endocrine overactivity. Its most frequent endocrine manifestation is primary pigmented nodular adrenocortical disease (PPNAD, a bilateral adrenocortical hyperplasia causing pituitary-independent Cushing's syndrome. Inactivating mutations in PRKAR1A, a gene encoding the type 1 alpha-regulatory subunit (R1alpha of the cAMP-dependent protein kinase (PKA have been found in 80% of CNC patients with Cushing's syndrome. To demonstrate the implication of R1alpha loss in the initiation and development of PPNAD, we generated mice lacking Prkar1a specifically in the adrenal cortex (AdKO. AdKO mice develop pituitary-independent Cushing's syndrome with increased PKA activity. This leads to autonomous steroidogenic genes expression and deregulated adreno-cortical cells differentiation, increased proliferation and resistance to apoptosis. Unexpectedly, R1alpha loss results in improper maintenance and centrifugal expansion of cortisol-producing fetal adrenocortical cells with concomitant regression of adult cortex. Our data provide the first in vivo evidence that loss of R1alpha is sufficient to induce autonomous adrenal hyper-activity and bilateral hyperplasia, both observed in human PPNAD. Furthermore, this model demonstrates that deregulated PKA activity favors the emergence of a new cell population potentially arising from the fetal adrenal, giving new insight into the mechanisms leading to PPNAD.

  8. Adrenal pathology in childhood: a spectrum of disease

    Energy Technology Data Exchange (ETDEWEB)

    Paterson, Anne [Royal Belfast Hospital for Sick Children, 180 Falls Road, Belfast BT12 6BE (United Kingdom)

    2002-10-01

    Adrenal lesions in children may present with an asymptomatic adrenal mass lesion, an endocrinopathy, a hypertensive or metabolic crisis or a paraneoplastic syndrome. Some of the underlying disease processes remain localised within the adrenal gland or may develop into disseminated disease. The role of the radiologist is to confirm the adrenal location of a lesion and to document any other foci of disease. Ultrasound, CT, MR imaging and metaiodobenzylguanidine scintigraphy are the most commonly utilised imaging modalities for examining the adrenal glands and their pathologies in children. This paper reviews the spectrum of adrenal disease in childhood and presents the information as a series of radiological appearances: anomalies of adrenal shape, location, number and size, cystic adrenal masses, solid adrenal mass lesions, and adrenal calcifications are all examined. The radiological findings must always be interpreted in conjunction with the patient's age, the clinical history, the findings on physical examination and the available biochemical data. (orig.)

  9. [Effect of alpha-tocopherol on adrenal cortex functions under stress].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Vinogradov, V V

    1991-01-01

    alpha-Tocopherol has been studied for its effect on lipid peroxidation and steroidogenesis in the adrenal cortices of rat and rabbit under stress. The vitamin is shown to exert an inhibitory effect on the lipid peroxidation developing under chronic stress. A biphasic pattern of the alpha-tocopherol effect on the steroidogenesis in the adrenal cortex is established: a decrease in the release of the steroids under the acute stress and maintaining of their levels under the chronic stress. A conclusion is drawn about a potential alpha-tocopherol application to correct the adrenal cortex function under stress.

  10. Down-regulation of the beacon gene expression in the regenerating rat adrenal cortex.

    Science.gov (United States)

    Ziolkowska, Agnieszka; Rucinski, Marcin; Tyczewska, Marianna; Belloni, Anna Sandra; Nowak, Magdalena; Nussdorfer, Gastone G; Malendowicz, Ludwik K

    2006-12-01

    Beacon, a hypothalamic peptide involved in the regulation of food intake, has been recently shown to be expressed in the adrenal cortex, and to inhibit its secretion and growth. To further characterize the role of beacon in the control of adrenal growth, we investigated the level of beacon gene expression in the regenerating rat adrenal cortex. Conventional reverse transcription-polymerase chain reaction (PCR) and immunocytochemistry demonstrated the expression of beacon mRNA and protein in the adrenals at both days 5 and 8 of regeneration after enucleation and contralateral adrenalectomy. Semiquantitative real time-PCR revealed a net down-regulation of beacon mRNA in the regenerating glands, as compared to the intact adrenal cortex of sham-operated animals. Beacon gene expression was higher at day 8 than at day 5 of regeneration. Mitotic index, as assayed by the stachmokinetic method with vincristin, was negligible in the intact adrenal, but greatly elevated in regenerating gland, with a higher index found at day 5 than at day 8 after surgery. Taken together our findings indicate that the level of beacon gene expression is inversely correlated with the proliferative activity of adrenocortical cells, and suggest that beacon might act as an endogenous inhibitor of adrenocortical growth in the rat.

  11. Aldo-Keto Reductases 1B in Adrenal Cortex Physiology.

    Science.gov (United States)

    Pastel, Emilie; Pointud, Jean-Christophe; Martinez, Antoine; Lefrançois-Martinez, A Marie

    2016-01-01

    Aldose reductase (AKR1B) proteins are monomeric enzymes, belonging to the aldo-keto reductase (AKR) superfamily. They perform oxidoreduction of carbonyl groups from a wide variety of substrates, such as aliphatic and aromatic aldehydes or ketones. Due to the involvement of human aldose reductases in pathologies, such as diabetic complications and cancer, AKR1B subgroup enzymatic properties have been extensively characterized. However, the issue of AKR1B function in non-pathologic conditions remains poorly resolved. Adrenal activities generated large amount of harmful aldehydes from lipid peroxidation and steroidogenesis, including 4-hydroxynonenal (4-HNE) and isocaproaldehyde (4-methylpentanal), which can both be reduced by AKR1B proteins. More recently, some AKR1B isoforms have been shown to be endowed with prostaglandin F synthase (PGFS) activity, suggesting that, in addition to possible scavenger function, they could instigate paracrine signals. Interestingly, the adrenal gland is one of the major sites for human and murine AKR1B expression, suggesting that their detoxifying/signaling activity could be specifically required for the correct handling of adrenal function. Moreover, chronic effects of ACTH result in a coordinated regulation of genes encoding the steroidogenic enzymes and some AKR1B isoforms. This review presents the molecular mechanisms accounting for the adrenal-specific expression of some AKR1B genes. Using data from recent mouse genetic models, we will try to connect their enzymatic properties and regulation with adrenal functions.

  12. Localization of Sonic hedgehog secreting and receiving cells in the developing and adult rat adrenal cortex.

    Science.gov (United States)

    Guasti, Leonardo; Paul, Alex; Laufer, Ed; King, Peter

    2011-04-10

    Sonic hedgehog signaling was recently demonstrated to play an important role in murine adrenal cortex development. The organization of the rat adrenal differs from that of the mouse, with the zona glomerulosa and zona fasciculata separated by an undifferentiated zone in the rat, but not in the mouse. In the present study we aimed to determine the mRNA expression patterns of Sonic hedgehog and the hedgehog signaling pathway components Patched-1 and Gli1 in the developing and adult rat adrenal. Sonic hedgehog expression was detected at the periphery of the cortex in cells lacking CYP11B1 and CYP11B2 expression, while signal-receiving cells were localized in the overlying capsule mesenchyme. Using combined in situ hybridization and immunohistochemistry we found that the cells expressing Sonic hedgehog lie between the CYP11B2 and CYP11B1 layers, and thus Sonic hedgehog expression defines one cell population of the undifferentiated zone.

  13. The effects of acute heat stress on proliferative and apoptotic processes in the rat adrenal cortex

    Directory of Open Access Journals (Sweden)

    Petrović-Kosanović Dragana

    2013-01-01

    Full Text Available Hyperthermia can cause significant structural and functional reorganization of tissues and organs. The proliferative and apoptotic processes of rat adrenal cortex were analyzed by light and electron microscopy after an acute exposure to high ambient temperature. Animals were divided in two groups. The first group consisted of intact controls. The rats from the second group were exposed to a high ambient temperature of 38°C for 60 min. Mitotic chromosomes and the largest number of immunoreactive nuclei for the Ki-67 were observed in the zona reticularis (ZR of the control animals. The relative number of mitoses after heat stress showed a significant decrease in the zona glomerulosa (ZG; 66.8%, zona fasciculata (ZF; 27.8% and ZR (86.7% (for all zones p<0.05, while in the whole adrenal cortex the after-treatment decrease was 61.9% (p<0.05 compared to the controls. Under heat stress numerous apoptotic nuclei were seen at the light and ultrastructural levels in all the zones of the adrenal cortex. Such dynamics of mitosis/apoptosis events seriously affect adrenal cortex morphology. [Projekat Ministarstva nauke Republike Srbije, br. 173023 i br. 173009

  14. Complexity of steroid hydroxylase gent expression in the adrenal cortex A microcosm of regulated transcription.

    Science.gov (United States)

    Waterman, M R; Lund, J; Simpson, E R

    1989-01-01

    Regulation of the expression of steroid hydroxylase genes in the adrenal cortex involves mechanisms required for maintenance of optimal steroidogenesis, tissue specificity, and ontogeny of the steroidogenic pathway. Evaluation of the molecular basis of this complexity promises to unfold new aspects of regulated eukaryotic gene expression.

  15. $sup 3$H-metyrapol as a tool for studies of interactions of deoxycorticosterone with adrenal cortex mitochondria

    Energy Technology Data Exchange (ETDEWEB)

    Satre, M.; Lunardi, J.; Vignais, P.V.

    1972-05-01

    From international conference on mechanism in bioenergetica; Bari, Italy (1 May 1972). /sup 3/H-metyrapol was prepared by reduction of metyrapone with tritiated sodium borohydride. Metyrapol behaves like metyrapone as an inhibitor of the 11 BETA -hydroxylation of deoxycorticosterone in adrenal cortex mitochondria and competes with metyrapone in binding tests. These results, and the ease of preparation of highly labeled /sup 3/H-metyrapol, recommend /sup 3/ Hmetyrapol as a probe of deoxycorticosterone interactions with adrenal cortex mitochondria. (auth)

  16. Principles and management of adrenal cancer

    Energy Technology Data Exchange (ETDEWEB)

    Javadpour, N. (ed.)

    1987-01-01

    Principles and Management of Adrenal Cancer is a comprehensive presentation of the medical and surgical management of neoplastic diseases of the adrenal glands. It consists of two parts. The first provides an overview of the embryology, anatomy, physiology, pathology, and advances in methods of diagnosis and imaging techniques. The second deals with specific diseases of the adrenal cortex and medulla. With 121 figs.

  17. Placental Estrogen Suppresses Cyclin D1 Expression in the Nonhuman Primate Fetal Adrenal Cortex*

    Science.gov (United States)

    Dumitrescu, Adina; Aberdeen, Graham W.; Pepe, Gerald J.

    2014-01-01

    We have previously shown that estrogen selectively suppresses growth of the fetal zone of the baboon fetal adrenal cortex, which produces the C19-steroid precursors, eg, dehydroepiandrosterone sulfate, which are aromatized to estrogen within the placenta. In the present study, we determined whether fetal adrenal expression of cell cycle regulators are altered by estrogen and thus provide a mechanism by which estrogen regulates fetal adrenocortical development. Cyclin D1 mRNA levels in the whole fetal adrenal were increased 50% (P < .05), and the number of cells in the fetal adrenal definitive zone expressing cyclin D1 protein was increased 2.5-fold (P < .05), whereas the total number of cells in the fetal zone and fetal serum dehydroepiandrosterone sulfate levels were elevated 2-fold (P < .05) near term in baboons in which fetal serum estradiol levels were decreased by 95% (P < .05) after maternal administration of the aromatase inhibitor letrozole and restored to normal by concomitant administration of letrozole plus estradiol throughout second half of gestation. However, fetal adrenocortical expression of cyclin D2, the cyclin-dependent kinase (Cdk)-2, Cdk4, and Cdk6, and Cdk regulatory proteins p27Kip1 and p57Kip2 were not changed by letrozole or letrozole plus estradiol administration. We suggest that estrogen controls the growth of the fetal zone of the fetal adrenal by down-regulating cyclin D1 expression and thus proliferation of progenitor cells within the definitive zone that migrate to the fetal zone. We propose that estrogen restrains growth and function of the fetal zone via cyclin D1 to maintain estrogen levels in a physiological range during primate pregnancy. PMID:25247468

  18. Placental estrogen suppresses cyclin D1 expression in the nonhuman primate fetal adrenal cortex.

    Science.gov (United States)

    Dumitrescu, Adina; Aberdeen, Graham W; Pepe, Gerald J; Albrecht, Eugene D

    2014-12-01

    We have previously shown that estrogen selectively suppresses growth of the fetal zone of the baboon fetal adrenal cortex, which produces the C19-steroid precursors, eg, dehydroepiandrosterone sulfate, which are aromatized to estrogen within the placenta. In the present study, we determined whether fetal adrenal expression of cell cycle regulators are altered by estrogen and thus provide a mechanism by which estrogen regulates fetal adrenocortical development. Cyclin D1 mRNA levels in the whole fetal adrenal were increased 50% (P < .05), and the number of cells in the fetal adrenal definitive zone expressing cyclin D1 protein was increased 2.5-fold (P < .05), whereas the total number of cells in the fetal zone and fetal serum dehydroepiandrosterone sulfate levels were elevated 2-fold (P < .05) near term in baboons in which fetal serum estradiol levels were decreased by 95% (P < .05) after maternal administration of the aromatase inhibitor letrozole and restored to normal by concomitant administration of letrozole plus estradiol throughout second half of gestation. However, fetal adrenocortical expression of cyclin D2, the cyclin-dependent kinase (Cdk)-2, Cdk4, and Cdk6, and Cdk regulatory proteins p27(Kip1) and p57(Kip2) were not changed by letrozole or letrozole plus estradiol administration. We suggest that estrogen controls the growth of the fetal zone of the fetal adrenal by down-regulating cyclin D1 expression and thus proliferation of progenitor cells within the definitive zone that migrate to the fetal zone. We propose that estrogen restrains growth and function of the fetal zone via cyclin D1 to maintain estrogen levels in a physiological range during primate pregnancy.

  19. A specific binding site recognizing a fragment of angiotensin II in bovine adrenal cortex membranes.

    Science.gov (United States)

    Bernier, S G; Fournier, A; Guillemette, G

    1994-12-12

    We have characterized a specific binding site for angiotensin IV in bovine adrenal cortex membranes. Pseudo-equilibrium studies at 37 degrees C for 2 h have shown that this binding site recognizes angiotensin IV with a high affinity (Kd = 0.24 +/- 0.03 nM). The binding site is saturable and relatively abundant (maximal binding capacity around 0.5 pmol/mg protein). Non-equilibrium kinetic analyses at 37 degrees C revealed a calculated kinetic Kd of 47 pM. The binding site is pharmacologically distinct from the classic angiotensin receptors AT1 or AT2. Competitive binding studies with bovine adrenal cortex membranes demonstrated the following rank order of effectiveness: angiotensin IV (Val-Tyr-Ile-His-Pro-Phe) = angiotensin II-(3-7) (Val-Tyr-Ile-His-Pro) > angiotensin III (Arg-Val-Tyr-Ile-His-Pro-Phe) > or = angiotensin II-(4-7) (Tyr-Ile-His-Pro) > angiotensin II (Asp-Arg-Val-Tyr-Ile-His-Pro-Phe) > angiotensin II-(1-6) (Asp-Arg-Val-Tyr-Ile-His) > angiotensin II-(4-8) (Tyr-Ile-His-Pro-Phe) > > > angiotensin II-(3-6) (Val-Tyr-Ile-His), angiotensin II-(4-6) (Tyr-Ile-His), L-158,809 (5,7-dimethyl-2-ethyl-3-[(2'(1-H-tetrazol-5-yl)[1,1'-biphenyl]-4-y l) methyl]-3-H-imidazo[4,5-beta]pyridine H2O) and PD 123319 (1-[4-(dimethylamino)3-methylphenyl]methyl-5-(diphenylacetyl)4,5,6 ,7- tetrahydro-1H-imidazo[4,5-c]pyridine-6-carboxylic acid). The divalent cations Mg2+ and Ca2+ were shown to diminish the binding of 125I-angiotensioffn IV to bovine adrenal cortex membranes.(ABSTRACT TRUNCATED AT 250 WORDS)

  20. [Activation of lipid peroxidation in the adrenal cortex by metal ions].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Vinogradov, V V

    1988-01-01

    The processes of lipid peroxidation have been studied in bovine adrenal cortex in vitro. The lipid peroxidation rate in this tissue is shown to be dependent on the content of metal ions. EDTA, deferroxamine and penicyllamine inhibit spontaneous lipid peroxidation by 25, 50 and 42%, respectively. The ability to activate the process permits arranging metal ions in the following sequence: Fe2+ greater than Fe3+ greater than Cu2+ greater than Mg2+ greater than Mn2+. The maximum activation of lipid peroxidation is observed at Fe2+ and Fe3+ concentrations within the range of 5 x 10(-6) x 10(-4) M.

  1. [Comparative determination of the potential for activating lipid peroxidation of the adrenal cortex, liver and heart in various animals].

    Science.gov (United States)

    Doroshkevich, N A; Antsulevich, S N; Vinogradov, V V

    1989-01-01

    Studies have been made on the rate of spontaneous, ascorbate- and NADP.H-dependent lipid peroxidation in the adrenal cortex, liver heart of rats, rabbits and cattle. It was shown that in spite of some species differences in predisposition of tissues to lipid peroxidation. The highest rate of this process is characteristic of the liver of all the animals investigated.

  2. [Quantitative histoenzymatic analysis of the adenohypophysis and adrenal cortex during the early stages of involution].

    Science.gov (United States)

    Prochukhanov, R A; Rostovtseva, T I

    1977-11-01

    A method of quantitative histenzymatic analysis was applied for determination of the involution changes of the neuroendocrine system. The activity of NAD- and NADP-reductases, acid and alkaline phosphatases, glucose-6-phosphoric dehydrogenase, 3-OH-steroid-dehydrogenase, 11-hydroxysteroid dehydrogenases was investigated in the adenohypophysis and in the adrenal cortex of rats aged 4 and 12 months. There were revealed peculiarities attending the structural-metabolic provision of physiological reconstructions of the neuro-endocrine system under conditions of the estral cycle at the early involution stages. An initial reduction of the cell ular-vascular transport with the retention of the functional activity of the intracellular organoids was demonstrated in ageing animals.

  3. [Reaction of the animal adrenal cortex to the action of a strong, constant magnetic field and to a hypomagnetic environment].

    Science.gov (United States)

    Shust, I V; Kostinik, I M

    1976-01-01

    Experiments were conducted on male albino rats; a study was made of the functional condition of the adrenal cortex following a single 30-minute (decapitation of the animals in 1, 8, 16, and 24 hours, and in 4, 8, 16, and 30 days) and repeated (for 30 minutes every day) prolonged (for 1 month) action of CMF (decapitation in 2, 4, 8, 16, and 30 days) and hypomagnetic field (for 8 and 30 days). Changes in the lipid content and in the alkaline phosphatase activity served as a morpho-histochemical criterion. A single brief CMF action proved to stimulate the adrenal function the first hours of the after-action; later the changes were oscillating in character. Prolonged repeated action of CMF depressed the functional activity of the adrenal cortex. Both in the first and in the second cases functional activity of the organism was almost completely restored by the end of the month, after the cessation of the CMF action. An 8-day stay of the animals in the hypomagnetic medium led to activation of the adrenal gland function, and a 30-day stay--to its reduction.

  4. Alterations in prealbumin concentration after adrenal autotransplantation for Parkinson's disease.

    Science.gov (United States)

    Abram, S R; Kruskal, J B; Allen, G S; Burns, R S; Parker, R; Tulipan, N

    1990-05-01

    The cerebrospinal fluid of eight patients with Parkinson's disease who underwent adrenal medullary autotransplantation was analyzed using SDS-polyacrylamide gel electrophoresis. A protein, subsequently identified as prealbumin, was noted to change in concentration over the intraoperative to 18-month postoperative time course. The qualitative changes observed on visual inspection were confirmed and quantified using laser densitometry. The concentration of prealbumin increased by an average of 90% when the intraoperative and 12-month samples were compared. This increase persisted at 18 months. The ratio of prealbumin to albumin also increased from intraoperative to 12 months by an average of 56%. This suggests that the increases in PA are the result of choroid plexus activation rather than a nonspecific breakdown of the blood-brain barrier. Given the association of prealbumin with other nervous system diseases, as well as its known ability to bind multiple substances, these findings may have important implications. Alterations in prealbumin may be responsible for the improvement seen in some patients who receive adrenal medullary autotransplants. Alternatively, prealbumin may be implicated in the pathophysiology of Parkinson's disease.

  5. Motor Cortex Stimulation in Parkinson's Disease

    OpenAIRE

    Marisa De Rose; Giusy Guzzi; Domenico Bosco; Mary Romano; Serena Marianna Lavano; Massimiliano Plastino; Giorgio Volpentesta; Rosa Marotta; Angelo Lavano

    2012-01-01

    Motor Cortex Stimulation (MCS) is less efficacious than Deep Brain Stimulation (DBS) in Parkinson's disease. However, it might be proposed to patients excluded from DBS or unresponsive to DBS. Ten patients with advanced PD underwent unilateral MCS contralaterally to the worst clinical side. A plate electrode was positioned over the motor cortex in the epidural space through single burr hole after identification of the area with neuronavigation and neurophysiological tests. Clinical assessment...

  6. The clinical usefulness of NP-59 scintigraphy in adrenal cortical diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Duk Kyu [College of Medicine, Donga Univ., Pusan (Korea, Republic of)

    1997-03-01

    {sup 131}I-6-{beta}-iodomethyl-19-norcholesterol (NP-59) has an advantage to assess adrenal dysfunction caused by adrenal cortical disorders. The aim of this study is to evaluate the clinical usefulness of NP-59 scintigraphy in each adrenal disease. Ten patients who did eleven NP-59 adrenal scintigraphies at Dong-A University Hospital from March 1990 to December 1996 were selected as the subject. Among the subject there were 5 cases of Cushing's syndrome, 2 cases of incidentaloma, 1 case of metastatic adrenal tumor, liver cirrhosis with hirsutism and hypertension respectively. Among 5 cases of Cushing's syndrome, there were 2 cases of Cushing's disease, 2 cases of adrenal adenoma and 1 case of adrenal carcinoma. There are no disagreement between clinical diagnosis and scan finding in Cushing's syndrome. In 2 incidentaloma cases, even though one is interpretated as a functioning tumor, both of 2 cases could avoid unnecessary biopsy according to scintigraphy result. One case of hirsutism, clinically adrenal originated, revealed the normal scintigraphic finding after dexamethasone suppression scan. It could suggest that the etiology of hirsutism was extra-adrenal origin. One case of hypertension took the study to exclude the possibility of primary aldosteronism. Normal suppression scan finding revealed that primary aldosteronism did not exist in this case. In conclusion, NP-59 scintigraphy was very useful in diagnosis and differential diagnosis of Cushing's syndrome and it could avoid unnecessary biopsy in the incidental adrenal tumor.

  7. [Oxidation of different substrates in mitochondria of bovine and rat adrenal cortex].

    Science.gov (United States)

    Mandrik, K A; Doroshkevich, N A; Vinogradov, V V

    1982-01-01

    The rate of oxygen uptake by mitochondria of bovine and rat adrenals was determined during oxidation of the Krebs cycle substrates. Succinate is established to be oxidized most actively under in vitro conditions.

  8. Lycopene ameliorates atrazine-induced oxidative damage in adrenal cortex of male rats by activation of the Nrf2/HO-1 pathway.

    Science.gov (United States)

    Abass, Marwa Ahmed; Elkhateeb, Shereen Ahmed; Abd El-Baset, Samia Adel; Kattaia, Asmaa Alhosiny; Mohamed, Eman Mosallam; Atteia, Hebatallah Husseini

    2016-08-01

    Atrazine (ATZ) is one of the most commonly used herbicides contaminating plants, soil and water resources. Several strategies have been used to counteract ATZ toxicity. Here, we tested the hypothesis that lycopene could ameliorate ATZ-induced toxicity in the adrenal cortex. For this purpose, 35 adult male albino rats were randomized into five equal groups: untreated control, vehicle control (received 0.5 mL corn oil/day), lycopene (treated with lycopene dissolved in 0.5 mL corn oil, 10 mg/kg b.w./day), ATZ (received ATZ dissolved in 0.5 mL corn oil 300 mg/kg b.w./day), and ATZ + lycopene (treated with ATZ and lycopene at the same previously mentioned doses). All treatments were given by oral gavage for 4 weeks. We found that ATZ exposure significantly increased relative adrenal weight, plasma ACTH levels, and adrenal oxidative stress as manifested by elevated malondialdehyde levels, decreased reduced glutathione content and depressed antioxidant enzyme activities in adrenal cortex tissues with respect to control groups. Furthermore, the transcription of adrenal cortex nuclear factor erythroid 2-related factor 2 (Nrf2), heme oxygenase-1 (HO-1), nuclear factor kappa B, and caspase-3 genes was increased significantly compared with the control groups. This was accompanied with DNA fragmentation and structural and ultrastructural changes in zona glomerulosa and zona fasiculata of the adrenal cortex. Notably, all these changes were partially ameliorated in rats treated concomitantly with ATZ and lycopene. Our results showed that lycopene exerts protective effects against ATZ-induced toxicity in rat adrenal cortex. These effects may be attributed to the antioxidative property of lycopene and its ability to activate the Nrf2/HO-1 pathway.

  9. [The sexual peculiarities of aging changes in circannual rhythms of pineal gland, hypophysis, adrenal cortex and thymus functions in healthy subjects].

    Science.gov (United States)

    Labunets, I F

    2013-01-01

    The interrelations of circannual rhythms of the functional state of pineal gland, hypophysis, adrenal cortex, thymus in healthy women and men from 20 to 79 years were studied. Fluctuations of melatonin, ACTH, cortisol and thymic serum factor, which were exchanged in aging (the season peaks of hormones and its acrophase) were found in blood of healthy 20-29 years old people. The changes of rhythmicity of indices were in male earlier (pineal gland and hypophysis over 30 years, thymus and adrenal cortex over 40 years) and more impressive than in women. The aging changes of pineal gland function's rhythm in healthy subjects have important role for changes of interrelations of circannual rhythms hypophysis, adrenal cortex and thymus.

  10. Spontaneous rupture of adrenal pheochromocytoma in a patient with Von Recklinghausen's disease

    OpenAIRE

    Azhough, Ramin; Barband, Ali Reza; Motayagheni, Negar; Niafar, Mitra; Pourfathi, Hojjat

    2009-01-01

    Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen's disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, irreversible hemodynamic shock, and death.

  11. Spontaneous rupture of adrenal pheochromocytoma in a patient with Von Recklinghausen′s disease

    Directory of Open Access Journals (Sweden)

    Azhough Ramin

    2009-01-01

    Full Text Available Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen ′ s disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, irreversible hemodynamic shock, and death.

  12. Spontaneous rupture of adrenal pheochromocytoma in a patient with Von Recklinghausen's disease

    Science.gov (United States)

    Azhough, Ramin; Barband, Ali Reza; Motayagheni, Negar; Niafar, Mitra; Pourfathi, Hojjat

    2009-01-01

    Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen's disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, irreversible hemodynamic shock, and death. PMID:19881191

  13. Cumene hydroperoxide supported demethylation of N,N-dimethylaniline by cytochrome P-450 from adrenal cortex mitochondria.

    Science.gov (United States)

    Akhrem, A A; Khatyleva SYu; Shkumatov, V M; Chashchin, V L; Kiselev, P A

    1982-01-01

    The interaction of highly purified cytochrome P-450 from bovine adrenal cortex mitochondria (cytochrome P-450scc) with N,N-dimethylaniline (DMA), aniline, N-dimethylcyclohexylamine and cumene hydroperoxide (CHP) has been investigated. The formation of complexes between cytochrome P-450scc and the above listed compounds could be demonstrated. The reaction of oxidative demethylation of DMA by cumene hydroperoxide involving cytochrome P-450scc has been carried out at 37 degrees C; the mechanism of this process is discussed. Incubation of cytochrome P-450scc with negatively charged phospholipids, phosphatidylglycerol (PG), and phosphatidylinosite (PI) exerts an inhibiting effect on the reaction of oxidative demethylation. The interaction of cytochrome P-450scc with CHP is accompanied by hemoprotein destruction in a complex biphasic way. The process of oxidative demethylation of DMA in the system of cytochrome P-450scc-CHP has been concluded to have a predominantly radical character.

  14. Zonal variation in the distribution of an alpha 1-acid glycoprotein glycoform receptor in human adrenal cortex

    DEFF Research Database (Denmark)

    Andersen, U O; Bøg-Hansen, T C; Kirkeby, S

    1999-01-01

    Using a histochemical technique with three different alpha 1-acid glycoprotein glycoform one glycoform specific receptor has been identified in human adrenal cortex. The receptor is associated to alpha 1-acid glycoprotein glycoform B and alpha 1-acid glycoprotein glycoform C. The glycoform specific...... specific receptor. The binding of alpha 1-acid glycoprotein glycoform B and alpha 1-acid glycoprotein glycoform C to the glycoform specific receptor is inhibited by the steroid hormones cortisone, aldosterone, estradiol and progesterone but not by testosterone. The pronounced changes in the distribution....... The binding activity was dependent on the presence of calcium ions and not on thiol reagents. Thus the lectin-like receptor may belong to the C-type lectin family. Using an antibody to alpha 1-acid glycoprotein the presence of alpha 1-acid glycoprotein was observed in the same location as the glycoform...

  15. Morphologic and morphometric analysis of adrenal gland cortex of sheep grazing on pastures with the ground of electrofilter ash

    Energy Technology Data Exchange (ETDEWEB)

    Juntes, P.; Pestevsek, U.; Pogacnik, M. [University of Ljubljana, Ljubljana (Slovenia)

    2007-07-01

    Electrofilter ash is a side product of power plants using coal as a fuel. Trials for transforming ash covered areas into cultivated landscape are ongoing e.g., efforts to form grasslands that would be usable for animal breeding and production. However the question of how safe grazing is on such surfaces in relation to animal health and the use of animal products, produced on such surfaces, for human consumption has not been truly resolved yet. The main goal of our work is a systematic search for the possible negative effects on animals grazing on such surfaces. In this work we present the results of morphologic and morphometric analyses that were made to evaluate the potential toxic or some other impact of electrofilter ash constituents on the adrenal gland cortex. No conclusive evidence of such effects was determined in this study.

  16. Adrenal gland and bone.

    Science.gov (United States)

    Hardy, Rowan; Cooper, Mark S

    2010-11-01

    The adrenal gland synthesizes steroid hormones from the adrenal cortex and catecholamines from the adrenal medulla. Both cortisol and adrenal androgens can have powerful effects on bone. The overproduction of cortisol in Cushing's disease leads to a dramatic reduction in bone density and an increase risk of fracture. Overproduction of adrenal androgens in congenital adrenal hyperplasia (CAH) leads to marked changes in bone growth and development with early growth acceleration but ultimately a significant reduction in final adult height. The role of more physiological levels of glucocorticoids and androgens on bone metabolism is less clear. Cortisol levels measured in elderly individuals show a weak correlation with measures of bone density and change in bone density over time with a high cortisol level associated with lower bone density and more rapid bone loss. Cortisol levels and the dynamics of cortisol secretion change with age which could also explain some age related changes in bone physiology. It is also now clear that adrenal steroids can be metabolized within bone tissue itself. Local synthesis of cortisol within bone from its inactive precursor cortisone has been demonstrated and the amount of cortisol produced within osteoblasts appears to increase with age. With regard to adrenal androgens there is a dramatic reduction in levels with aging and several studies have examined the impact that restoration of these levels back to those seen in younger individuals has on bone health. Most of these studies show small positive effects in women, not men, but the skeletal sites where benefits are seen varies from study to study.

  17. Synthesis of a new adrenal cortex imaging agent 6. beta. -/sup 131/I-iodomethyl-19-nor cholest-5(10)-en-3. beta. -ol (NP-59)

    Energy Technology Data Exchange (ETDEWEB)

    Basmadjian, G.P.; Hetzel, K.R.; Ice, R.D.; Beierwaltes, W.H.

    1975-04-10

    A new adrenal cortex imaging agent, 6..beta..-/sup 131/I-iodomethyl-19-nor-cholest-5(10)-en-3..beta..-ol (NP-59) (I) was synthesized by the homallylic rearrangement of 19-iodocholesterol or directly from cholest-5-ene-3..beta.., 19-diol-19-toluene-p-sulfonate via homoallylic rearrangement with the iodide ion as a nucleophile and subsequent exchange with Na/sup 131/I. NP-59 appears to concentrate 5 times better than 19-iodocholesterol in the rat adrenal and is currently being evaluated as a possible diagnostic agent in man.

  18. Effect of day-to-day variations in adrenal cortex hormone levels on abdominal symptoms

    Directory of Open Access Journals (Sweden)

    Tsumura Hideki

    2010-03-01

    Full Text Available Abstract Introduction The hypothalamic-pituitary-adrenal axis is known to be related to abdominal symptoms, and the relationship between abdominal pain and cortisol secretory patterns has been previously investigated using a cross-sectional approach. Here, we investigated the effect of day-to-day variations in salivary cortisol and dehydroepiandrosterone-sulfate levels on abdominal symptoms in healthy individuals. Methods Eleven college students (4 males and 7 females participated in this study. The participants were asked to collect their saliva immediately after awakening and before bedtime for eight consecutive days. They also completed a questionnaire about abdominal symptoms before bedtime. The linear mixed model was applied to analyze the effects of the day-by-day variability or the 8-day average adrenal hormone level (at awakening, before bedtime, slope from awakening to bedtime on abdominal symptoms. Results The day-to-day variability of cortisol levels before bedtime was negatively related with loose stool, while the day-to-day variability of the cortisol slope was positively correlated with loose stool. A low 8-day average dehydroepiandrosterone-sulfate level at awakening was positively related with frequent bowel movements, loose stool, and long bouts of severe abdominal pain. Likewise, a low 8-day average dehydroepiandrosterone-sulfate slope was positively related with long bouts of abdominal pain. Conclusions Low cortisol levels before bedtime and a steeper diurnal cortisol slope during the day may be related to bouts of diarrhea during the day.

  19. Adrenal insufficiency: diagnosis and management.

    Science.gov (United States)

    Munver, Ravi; Volfson, Ilya A

    2006-01-01

    Adrenal insufficiency is a disorder characterized by hypoactive adrenal glands resulting in insufficient production of the hormones cortisol and aldosterone by the adrenal cortex. This disorder may develop as a primary failure of the adrenal cortex or be secondary to an abnormality of the hypothalamic-pituitary axis. Patients with adrenal insufficiency often are asymptomatic or they may present with fatigue, muscle weakness, weight loss, low blood pressure, and sometimes darkening of the skin. The presentation of adrenal insufficiency varies dramatically and poses a major diagnostic dilemma. This review focuses on the diagnosis and treatment of primary and secondary adrenal insufficiency.

  20. Motor cortex stimulation in Parkinson's disease.

    Science.gov (United States)

    De Rose, Marisa; Guzzi, Giusy; Bosco, Domenico; Romano, Mary; Lavano, Serena Marianna; Plastino, Massimiliano; Volpentesta, Giorgio; Marotta, Rosa; Lavano, Angelo

    2012-01-01

    Motor Cortex Stimulation (MCS) is less efficacious than Deep Brain Stimulation (DBS) in Parkinson's disease. However, it might be proposed to patients excluded from DBS or unresponsive to DBS. Ten patients with advanced PD underwent unilateral MCS contralaterally to the worst clinical side. A plate electrode was positioned over the motor cortex in the epidural space through single burr hole after identification of the area with neuronavigation and neurophysiological tests. Clinical assessment was performed by total UPDRS, UPDRS III total, UPDRS III-items 27-31, UPDRS IV, and UPDRS II before implantation in off-medication and on-medication states and after surgery at 1, 3, 6, 12, 18, 24, and 36 months in on-medication/on-stimulation and off-medication/on-stimulation states. We assessed changes of quality of life, throughout the Parkinson's disease quality of life scale (PDQoL-39), and the dose of anti-Parkinson's disease medications, throughout the Ldopa equivalent daily dose (LEDD). During off-medication state, we observed moderate and transitory reduction of total UPDRS and UPDRS total scores and significant and long-lasting improvement in UPDRS III items 27-31 score for axial symptoms. There was marked reduction of UPDRS IV score and LEDD. PDQL-39 improvement was also significant. No important complications and adverse events occurred.

  1. Motor Cortex Stimulation in Parkinson's Disease

    Directory of Open Access Journals (Sweden)

    Marisa De Rose

    2012-01-01

    Full Text Available Motor Cortex Stimulation (MCS is less efficacious than Deep Brain Stimulation (DBS in Parkinson's disease. However, it might be proposed to patients excluded from DBS or unresponsive to DBS. Ten patients with advanced PD underwent unilateral MCS contralaterally to the worst clinical side. A plate electrode was positioned over the motor cortex in the epidural space through single burr hole after identification of the area with neuronavigation and neurophysiological tests. Clinical assessment was performed by total UPDRS, UPDRS III total, UPDRS III-items 27–31, UPDRS IV, and UPDRS II before implantation in off-medication and on-medication states and after surgery at 1, 3, 6, 12, 18, 24, and 36 months in on-medication/on-stimulation and off-medication/on-stimulation states. We assessed changes of quality of life, throughout the Parkinson's disease quality of life scale (PDQoL-39, and the dose of anti-Parkinson's disease medications, throughout the Ldopa equivalent daily dose (LEDD. During off-medication state, we observed moderate and transitory reduction of total UPDRS and UPDRS total scores and significant and long-lasting improvement in UPDRS III items 27–31 score for axial symptoms. There was marked reduction of UPDRS IV score and LEDD. PDQL-39 improvement was also significant. No important complications and adverse events occurred.

  2. Tumor necrosis factor alpha affect hydrocortisone expression in mice adrenal cortex cells mainly through tumor necrosis factor alpha-receptor 1

    Institute of Scientific and Technical Information of China (English)

    XIA Hai-ming; FANG Yuan; HUANG Pei-lin

    2011-01-01

    Background Tumor necrosis factor alpha (TNF-α) is important in promoting relative adrenal insufficiency (RAI) due to systemic inflammatory response syndrome (SIRS).We identified the TNF-α receptor involved in the inhibition of adrenal corticotrophin (ACTH)-stimulated hydrocortisone release by studying the expression of TNF-α receptors in adrenal cortex Y1 cells and the effect of downregulating TNF receptors on ACTH-stimulated hydrocortisone release.Methods We used real-time PCR and immunocytochemistry to evaluate the expression of TNF receptors on Y1 cells.TNF-receptor 1 (TNF-R1) DNA fragments corresponding to the short hairpin RNA (shRNA)-sequences were synthesized and cloned into pcDNATM 6.2-GW/EmGFP expression vector.Knockdown efficiency of TNF-R1 expression was evaluated in miRNA transfected and mock-miRNA transfected Y1 cells by quantitative real-time PCR (Q-PCR).Hydrocortisone expression levels were determined in TNF-R1-knockdown and control Y1 cells treated with TNF-α and ACTH.Results Mouse adrenal cortex Y1 cells were positive for type I TNF-R1,but not type Ⅱ TNF-receptor (TNF-R2).Blocking TNF-R1 expression resulted in loss of TNF-α-mediated inhibition of ACTH-stimulated hydrocortisone expression,suggesting a role for the TNF-R1 related signaling pathway in ACTH-stimulated hydrocortisone synthesis.Conclusion The inhibitory effect of TNF-α on ACTH-stimulated hydrocortisone synthesis was mediated via TNF-R1 in adrenal cortex.

  3. Intrinsic factors, adrenal gland morphology, and disease burden in captive cheetahs (Acinonyx jubatus) in South Africa.

    Science.gov (United States)

    Gillis-Germitsch, Nina; Vybiral, Pamela-Rose; Codron, Daryl; Clauss, Marcus; Kotze, Antoinette; Mitchell, Emily P

    2017-01-01

    Adrenal gland weight (AW) and corticomedullary ratio (ACMR) are used as indicators of stress in animals. Captive cheetahs (Acinonyx jubatus) have higher ACMRs than free-ranging ones and stress has been linked to gastritis, amyloidosis, glomerulosclerosis, and myocardial fibrosis. We reviewed age, sex, body weight (BW), kidney weight (KW), and left AW and ACMR with necropsy findings in 51 South African captive cheetahs. Eleven common histopathologic lesions were counted for each animal as measure of its disease burden. Adrenal corticomedullary hyperplasia was significantly correlated with left AW and ACMR. Males had significantly higher AWs than females; other parameters showed no difference between the sexes. Disease burden, gastritis, and myocardial fibrosis were moderately correlated with adrenal morphology supporting prior evidence that gastritis and myocardial fibrosis are linked to stress. Glomerulosclerosis was not correlated with adrenal morphology and neither kidney nor liver amyloidosis contributed significantly to variation in AW or ACMR on multivariate analyses. Interstitial nephritis showed much stronger correlations with kidney and liver amyloidosis than gastritis. All three adrenal parameters were correlated with age; age was the only significant variable affecting ACMR on the multivariate analyses; and disease burden as well as systemic amyloidosis and kidney disease (except for fibrosis) showed moderate correlations with age. Age may, therefore, be important in the pathogenesis of disease in captive cheetahs, particularly of amyloidosis and kidney disease. None of the intrinsic measurements or adrenal parameters were sufficiently closely linked to disease to be used as ante-mortem proxies for disease burden or specific diseases. Zoo Biol. 36:40-49, 2017. © 2016 Wiley Periodicals, Inc.

  4. Acupuncture Relieves the Excessive Excitation of Hypothalamic-Pituitary-Adrenal Cortex Axis Function and Correlates with the Regulatory Mechanism of GR, CRH, and ACTHR

    Directory of Open Access Journals (Sweden)

    Shao-Jun Wang

    2014-01-01

    Full Text Available It had been indicated in the previous studies that acupuncture relieved the excessive excitation of hypothalamic-pituitary-adrenal cortex axis (HPAA function induced by stress stimulation. But the changes in glucocorticoid receptor (GR induced by acupuncture have not been detected clearly. The objective of the study was to observe the impacts of acupuncture on the protein expressions of corticotrophin releasing hormone (CRH, adrenocorticotropic hormone receptor (ACTHR, and GR under the physiological and stress states. The results showed that under the stress state, acupuncture upregulated the protein expression of GR in the hippocampus, hypothalamic paraventricular nucleus (PVN, and pituitary gland, downregulated the protein expression of GR in the adrenal cortex, and obviously reduced the protein expressions of CRH and ACTHR. Under the physiological state, acupuncture promoted GR protein expression in the hippocampus and CRH protein expression in the hippocampus and PVN. The results explained that acupuncture regulated the stress reaction via promoting the combination of glucocorticoids (GC with GR, and GR protein expression. The increase of GR protein expression induced feedback inhibition on the overexpression of CRH and ACTHR, likely decreased GC level, and caused the reduction of GR protein expression in the adrenal cortex.

  5. Acupuncture Relieves the Excessive Excitation of Hypothalamic-Pituitary-Adrenal Cortex Axis Function and Correlates with the Regulatory Mechanism of GR, CRH, and ACTHR.

    Science.gov (United States)

    Wang, Shao-Jun; Zhang, Jiao-Jiao; Qie, Li-Li

    2014-01-01

    It had been indicated in the previous studies that acupuncture relieved the excessive excitation of hypothalamic-pituitary-adrenal cortex axis (HPAA) function induced by stress stimulation. But the changes in glucocorticoid receptor (GR) induced by acupuncture have not been detected clearly. The objective of the study was to observe the impacts of acupuncture on the protein expressions of corticotrophin releasing hormone (CRH), adrenocorticotropic hormone receptor (ACTHR), and GR under the physiological and stress states. The results showed that under the stress state, acupuncture upregulated the protein expression of GR in the hippocampus, hypothalamic paraventricular nucleus (PVN), and pituitary gland, downregulated the protein expression of GR in the adrenal cortex, and obviously reduced the protein expressions of CRH and ACTHR. Under the physiological state, acupuncture promoted GR protein expression in the hippocampus and CRH protein expression in the hippocampus and PVN. The results explained that acupuncture regulated the stress reaction via promoting the combination of glucocorticoids (GC) with GR, and GR protein expression. The increase of GR protein expression induced feedback inhibition on the overexpression of CRH and ACTHR, likely decreased GC level, and caused the reduction of GR protein expression in the adrenal cortex.

  6. Short-term effects of ACTH on protein synthesis in adrenal cortex cells of young rats.

    Science.gov (United States)

    Magalhães, M C; Magalhães, M M; Cimbra, A

    1975-11-19

    Two units of ACTH were administered intraperitoneally to young 20 gm-rats which received an intravenous injection of L-leucine-3H thirteen min later. ACTH-injected rats, and control rats which received the isotope alone, were killed at 2-, 10-, 30- and 60-min intervals. Electron microscope autoradiographs in control animals showed strong amino-acid uptake at pulse time (2-min) in the cytoplasm of adrenal zona fasciculata cells. Label was shared between the endoplasmic reticulum (ER) and mitochondria, and a lower but still considerable uptake was seen in nucleoli. At first chase time interval (10-min) cytoplasmic labelling declined, while nuclear and nucleolar labelling increased, both changing little thereafter, and there was a 10-30 min Golgi peak. ACTH administration provoked an overall increase in amino-acid incorporation into cytoplasm, nucleus and nucleolus at pulse time, with no changes in the distribution of the reactions among organelles. Intensification of labelling was most evident over nucleoli, the grain density of which was four-times as high as in controls. The short-term increase in ER and mitochondrial protein synthesis observed after ACTH injections was considered to be consistent with the hypothesis that most newly-formed proteins in these cells may be involved in the regulation of steroidogenesis. The marked increase in nucleolar labelling suggested the presence of proteins involved in RNA synthesis.

  7. Carcinoma of the Adrenal Cortex in Children%小儿肾上腺皮质癌

    Institute of Scientific and Technical Information of China (English)

    黄澄如; 贺荣友

    1982-01-01

    @@ 婴幼儿肾上腺皮质功能性病变,表现为使皮质醇症时,多系肾上腺皮质癌.我院1960~1979年共收治4例,均经手术切除.近期2例随访已超过2年,情况良好;1例术后死亡;1例未随访.今简要报导及讨论如下.%Adrenal carcinoma is often responsible for Cushing's syndrome complicated with virilization or isosexual precocity in a male.Usually the carcinoma is large enough to be palpable on examination and can be demonstrated by routine pyelography.Increased level of urinary 17-ketosteroids or 17-hydroxycorticoids and the failure to respond to the dexamethasone suppresion test strongly support the diagnosis of carcinoma.Four patients,two males and two females,with adrenal carcinoma were admitted to this hospital from 1960 to 19799 with the age ranging from 2 to 4 years.

  8. The Contribution of Online Peer-to-Peer Communication Among Patients With Adrenal Disease to Patient-Centered Care

    NARCIS (Netherlands)

    Kauw, D.; Repping-Wuts, H.; Noordzij, A.; Stikkelbroeck, N.; Hermus, A.R.; Faber, M.J.

    2015-01-01

    BACKGROUND: Addison's disease and Cushing's syndrome are rare. The Dutch Adrenal Society offers an online forum for Dutch adrenal patients to meet and communicate. However, little is known about the added value such a forum has for the delivery of patient-centered care. OBJECTIVE: Our aim was to ana

  9. Immunohistochemical evidence for the presence of a Vasoactive Intestinal Peptide, Neuropeptide Y, and Substance P, in rat adrenal cortex after acute heat stress

    Directory of Open Access Journals (Sweden)

    Petrović-Kosanović Dragana

    2013-01-01

    Full Text Available Immunohistochemistry revealed the presence of Vasoactive Intestinal Peptide (VIP, Neuropeptide Y (NPY, and the absence of Substance P (SP immunoreactivity in the rat adrenal cortex. VIP- and NPY-immunoreactivity were detected in nerve fibers around the small blood vessels projecting into the capsule and cortical zones surrounding blood vessels and cortical cells. After acute heat stress, VIP- and NPY-immunoreactivities in the nerve fibers were reduced, probably as a result of the release of these peptides. [Projekat Ministarstva nauke Republike Srbije, br. 173023

  10. [Adrenal mass and adrenal insufficiency].

    Science.gov (United States)

    Martínez Albaladejo, M; García López, B; Serrano Corredor, S; Alguacil García, G

    1996-12-01

    Primary adrenal insufficiency is a non frequent disease, that is declared in young adults and in the most of the cases is produced from an autoimmune mechanism or a tuberculous disease. The incidence of these forms in the different geographic areas is dependent of degree of irradication of the tuberculosis. We report the case of a patient with latent chronic adrenal insufficiency of tuberculous origin who was affected for an addisonian crisis during an intercurrent infectious disease, which permitted the diagnosis of the addisonian crisis, and Mal of Pott was moreover detected. Evolution with corticosteroid and specific treatment was very favorable.

  11. Principles and management of adrenal cancer

    Energy Technology Data Exchange (ETDEWEB)

    Javadpour, N.

    1987-01-01

    This book provides information on adrenal diseases of latest developments and guides the clinicians in the care of their patients. The book is divided into two parts. The first section gives an overview of the embryology, anatomy, physiology, markers, pathology, imaging and the current progress in the field. The second edition covers specific diseases of the adrenal cortex and medulla. The increasingly significant roles played by steroids, catecholamines, blockers, computed tomography and magnetic resonance are elucidated and discussed. The contents include: Overview of progress; current problems, and perspectives - embryology anatomy, physiology, and biologic markers; pathology; advances in diagnosis; imaging techniques; adrenal disorders in childhood; primary aldosteronism; Cushing's syndrome; carcinoma; pheochromocytoma; neuroblastoma; metastatic disease; surgical management; and subject index.

  12. Sex-related gene expression profiles in the adrenal cortex in the mature rat: microarray analysis with emphasis on genes involved in steroidogenesis.

    Science.gov (United States)

    Trejter, Marcin; Hochol, Anna; Tyczewska, Marianna; Ziolkowska, Agnieszka; Jopek, Karol; Szyszka, Marta; Malendowicz, Ludwik K; Rucinski, Marcin

    2015-03-01

    Notable sex-related differences exist in mammalian adrenal cortex structure and function. In adult rats, the adrenal weight and the average volume of zona fasciculata cells of females are larger and secrete greater amounts of corticosterone than those of males. The molecular bases of these sex-related differences are poorly understood. In this study, to explore the molecular background of these differences, we defined zone- and sex-specific transcripts in adult male and female (estrous cycle phase) rats. Twelve-week-old rats of both genders were used and samples were taken from the zona glomerulosa (ZG) and zona fasciculata/reticularis (ZF/R) zones. Transcriptome identification was carried out using the Affymetrix(®) Rat Gene 1.1 ST Array. The microarray data were compared by fold change with significance according to moderated t-statistics. Subsequently, we performed functional annotation clustering using the Gene Ontology (GO) and Database for Annotation, Visualization and Integrated Discovery (DAVID). In the first step, we explored differentially expressed transcripts in the adrenal ZG and ZF/R. The number of differentially expressed transcripts was notably higher in the female than in the male rats (702 vs. 571). The differentially expressed genes which were significantly enriched included genes involved in steroid hormone metabolism, and their expression levels in the ZF/R of adult female rats were significantly higher compared with those in the male rats. In the female ZF/R, when compared with that of the males, prevailing numbers of genes linked to cell fraction, oxidation/reduction processes, response to nutrients and to extracellular stimuli or steroid hormone stimuli were downregulated. The microarray data for key genes involved directly in steroidogenesis were confirmed by qPCR. Thus, when compared with that of the males, in the female ZF/R, higher expression levels of genes involved directly in steroid hormone synthesis were accompanied by lower

  13. Adrenal hypoplasia congenita: a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism

    Directory of Open Access Journals (Sweden)

    Marta Loureiro

    2015-09-01

    Full Text Available Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG, confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

  14. Disorders of adrenal development.

    Science.gov (United States)

    Ferraz-de-Souza, Bruno; Achermann, John C

    2008-01-01

    Human adrenal development is a complex and relatively poorly understood process. However, significant insight into some of the mechanisms regulating adrenal development and function is being obtained through the analysis of individuals and families with adrenal hypoplasia. Adrenal hypoplasia can occur: (1) secondary to defects in pituitary adrenocorticotropin (ACTH) synthesis, processing and release (secondary adrenal hypoplasia; e.g. HESX1, LHX4, SOX3, TPIT, pituitary POMC, PC1); (2) as part of several ACTH resistance syndromes (e.g. MC2R/ACTHR, MRAP, Alacrima, Achalasia, Addison disease), or as (3) a primary defect in the development of the adrenal gland itself (primary adrenal hypoplasia; e.g. DAX1/NR0B1 - dosage-sensitive sex reversal, adrenal hypoplasia congenita critical region on the X chromosome 1). Indeed, the X-linked form of primary adrenal hypoplasia due to deletions or mutations in the orphan nuclear receptor DAX1 occurs in around half of male infants presenting with a salt-losing adrenal crisis, where no obvious steroidogenic defect (e.g. 21-hydroxylase deficiency), metabolic abnormality (e.g. neonatal adrenoleukodystrophy) or physical cause (e.g. adrenal haemorrhage) is found. Establishing the underlying basis of adrenal failure can have important implications for investigating associated features, the likely long-term approach to treatment, and for counselling families about the risk of other children being affected.

  15. Hypopituitarism Presenting as Adrenal Insufficiency and Hypothyroidism in a Patient with Wilson's Disease: a Case Report.

    Science.gov (United States)

    Lee, Hae Won; Kang, Jin Du; Yeo, Chang Woo; Yoon, Sung Woon; Lee, Kwang Jae; Choi, Mun Ki

    2016-08-01

    Wilson's disease typically presents symptoms associated with liver damage or neuropsychiatric disturbances, while endocrinologic abnormalities are rare. We report an unprecedented case of hypopituitarism in a patient with Wilson's disease. A 40-year-old woman presented with depression, general weakness and anorexia. Laboratory tests and imaging studies were compatible with liver cirrhosis due to Wilson's disease. Basal hormone levels and pituitary function tests indicated secondary hypothyroidism and adrenal insufficiency due to hypopituitarism. Brain MRI showed T2 hyperintense signals in both basal ganglia and midbrain but the pituitary imaging was normal. She is currently receiving chelation therapy along with thyroid hormone and steroid replacement. There may be a relationship between Wilson's disease and hypopituitarism. Copper deposition or secondary neuronal damage in the pituitary may be a possible explanation for this theory.

  16. Adrenal medullary transplantation to the caudate nucleus in Parkinson's disease. Initial clinical results in 18 patients.

    Science.gov (United States)

    Allen, G S; Burns, R S; Tulipan, N B; Parker, R A

    1989-05-01

    Results from a pilot study of adrenal medullary autotransplantation for Parkinson's disease are presented. Eighteen patients were studied; 12 were followed up for 1 year, and 6 were followed up for 6 months. Four of 12 patients showed distinct improvement in the signs and symptoms of their disease, as assessed using the Columbia Rating Scale, at 1 year; none showed distinct deterioration. The 6 patients who were followed up for only 6 months were an average of 20 years older and generally more severely affected. None distinctly improved. Morbidity was considered to be minor and transient among the first 12 patients, while 4 of the last 6 patients experienced alteration in mental status lasting as long as several months. This problem has led us to conclude that older patients with preexisting cognitive impairment should not be included in future studies until the benefits are more clearly established. However, we believe that the distinct and persistent improvement seen in some of the younger patients warrants the initiation of a well-designed, randomized, and controlled trial of adrenal medullary autotransplantation for the purpose of confirming these results and assessing the effect of the procedure on the natural progression of Parkinson's disease.

  17. Aberrant functional connectivity differentiates retrosplenial cortex from posterior cingulate cortex in prodromal Alzheimer's disease.

    Science.gov (United States)

    Dillen, Kim N H; Jacobs, Heidi I L; Kukolja, Juraj; von Reutern, Boris; Richter, Nils; Onur, Özgür A; Dronse, Julian; Langen, Karl-Josef; Fink, Gereon R

    2016-08-01

    The posterior cingulate cortex (PCC) is a key hub of the default mode network, a resting-state network involved in episodic memory, showing functional connectivity (FC) changes in Alzheimer's disease (AD). However, PCC is a cytoarchitectonically heterogeneous region. Specifically, the retrosplenial cortex (RSC), often subsumed under the PCC, is an area functionally and microanatomically distinct from PCC. To investigate FC patterns of RSC and PCC separately, we used resting-state functional magnetic resonance imaging in healthy aging participants, patients with subjective cognitive impairment, and prodromal AD. Compared to the other 2 groups, we found higher FC from RSC to frontal cortex in subjective cognitive impairment but higher FC to occipital cortex in prodromal AD. Conversely, FC from PCC to the lingual gyrus was higher in prodromal AD. Furthermore, data indicate that RSC and PCC are characterized by differential FC patterns represented by hub-specific interactions with memory and attentions scores in prodromal AD compared to cognitively normal individuals, possibly reflecting compensatory mechanisms for RSC and neurodegenerative processes for PCC. Data thus confirm and extend previous studies suggesting that the RSC is functionally distinct from PCC.

  18. Spontaneous rupture of adrenal pheochromocytoma in a patient with Von Recklinghausen′s disease

    OpenAIRE

    Azhough Ramin; Barband Ali; Motayagheni Negar; Niafar Mitra; Pourfathi Hojjat

    2009-01-01

    Spontaneous rupture of an adrenal pheochromocytoma is extremely rare and can be lethal because of dramatic changes in the circulation. We describe a 35-year-old Iranian female with previously diagnosed von Recklinghausen ′ s disease who suffered spontaneous rupture of an adrenal pheochromocytoma, misdiagnosed as renal colic followed by an extensive retroperitoneal hematoma, irreversible hemodynamic shock, and death.

  19. Differential expression of somatostatin receptor subtype-related genes and proteins in non-functioning and functioning adrenal cortex adenomas.

    Science.gov (United States)

    Pisarek, Hanna; Krupiński, Roman; Kubiak, Robert; Borkowska, Edyta; Pawlikowski, Marek; Winczyk, Katarzyna

    2011-01-01

    Adrenocortical adenomas display highly variable expressions of somatostatin receptor (SSTR) subtypes, whose expression is mandatory (although not always sufficient) to achieve the positive effects of somatostatin (SST) analog therapy. Immunohistochemistry (IHC) is the main method used to investigate receptor protein expression. The molecular biology method - polymerase chain reaction (PCR) - is also often used to investigate receptor expression. Nevertheless, the expression of receptor mRNA and the respective receptor protein is not always synchronized. The aim of this study was to investigate SSTR expression by IHC in adrenal adenomas, to compare the results to data obtained by real-time PCR and to determine whether hormonally functioning and non-functioning adenomas differ in this respect. Adrenocortical adenomas were removed surgically from 13 females and 2 males. The tissues were obtained from 9 non-functioning and 6 functioning adenomas. The intensity of IHC reaction was scored semiquantitatively by two independent observers. Real-time PCR was performed using pairs of primers in a reaction amplified along a gradient of temperatures. Amplified DNA was measured by monitoring SYBR-Green fluorescence. In non-functioning tumors, compatibility between IHC and PCR results was observed for SSTR 1 and 2 in 62.5% of the samples. Fifty percent of patients demonstrated compatibility for SSTR 4 and 5 and 37.5% for SSTR 3. In hormonally active adenomas, total compatibility of both methods was noted for SSTR 2 (100%). The compatibility obtained for SSTR 5 was 66.6%. We conclude that receptor gene and respective receptor protein expression are not always synchronized. Messenger RNA detection alone is not sufficient to predict the presence of the receptor protein acting as a target for SST and its analogs.

  20. Autologous Adrenal Medullary, Fetal Mesencephalic, and Fetal Adrenal Brain Transplantation in Parkinson's Disease: A Long-Term Postoperative Follow-Up

    Science.gov (United States)

    Madrazo, Ignacio; Franco-Bourland, Rebecca; Aguilera, Maricarmen; Ostrosky-Solis, Feggy; Madrazo, Mario; Cuevas, Carlos; Catrejon, Hugo; Guizar-Zahagun, Gabriel; Magallon, Eduardo

    1991-01-01

    We report on the clinical status of 5 patients with Parkinson's disease (PD) 3 years after autologous adrenal medullary (AM)-to-caudate nucleus (CN) implanfion, and of 2 PD patients, 2 years after fetal ventral mesencephalon (VM)- and fetal adrenal (A)-to-CN homotransplantation. Current clinical evaluation of 4 of the AM grafted patients revealed sustained bilateral amelioration of their PD signs, most notably of rgidity, postural imbalance and gait disturbances, resulting in a substantial improvement in their quality of life. the disease-related dystonia of one of them disappeared only 2 years after surgery. The levodopa requirements of 2 of these patients and the anticholinergic therapy of another have been reduced. In agreement with the satisfactory clinical evaluation of these 4 patients, their neuropsychological and electrophysiological improvements, initially registered 3 months after surgery, have been maintained for 3 years. After 1 year of significant recovery, the 5th patient of this group has almost returned to her preoperative state. The 2 homotransplanted patients also showed sustained bilateral improvement of their PD signs. Two years after surgery, the most improved signs of the fetal VM case were rigidity, bradykinesia, postural imbalance, gait disturbances and facial expression. The fetal A case has only shown amelioration of rigidity and bradykinesia. Neither of them has shown significant neuropsychological changes. Their current levodopa requirements are less than before surgery. The improvements shown here by PD patients after brain tissue grafts go beyond those obtained using any other therapeutic approach, when levodopa fails. Although more studies and the development of these procedures are obviously required, these initial human trials appear to be resisting the test of time. PMID:1782251

  1. 去势雄性大鼠肾上腺皮质束状带和网状带的变化及低雄激素对COX-2信号通路的影响%Changes in zona fasciculata and reticularis of adrenal cortex in castrated male rats and effect of low androgen on signal iathway of COX-2

    Institute of Scientific and Technical Information of China (English)

    施超; 陆颖理; 李艳香; 翟华玲; 姜博仁; 李影; 夏芳珍; 徐慧; 乔洁; 林东平

    2011-01-01

    ( P < 0.0S), while the expression of COX-2 mRNA in aorta tissues in castration group was significantly higher than that in control group and replacement group (P<0.05). Conclusion There are hyperplasia and hyperfunction of zona fasciculate and reticularis of adrenal cortex and lower expression of COX-2 mRNA in adrenal cortex tissues in castrated male rats, and the increased expression of COX-2 mRNA in aorta tissues may be related to the vascular disease induced by low androgen.%目的 观察去势雄性大鼠肾上腺皮质束状带和网状带的形态结构和功能的变化,探讨低雄激素对环氧化酶2( COX-2)信号通路的影响.方法 30只10周龄雄性SD大鼠随机分为对照组(n=8)、去势组(n=11)和替代组(n=11),对照组大鼠接受假去势手术,去势组大鼠接受去势手术,替代组大鼠在接受去势手术后给予肌肉注射睾酮(十一酸睾酮每月50 mg/kg)进行替代治疗.10周后采集各组大鼠血清,放射免疫分析法测定血清睾酮(T)、皮质酮(F)、促肾上腺皮质激素( ACTH)、黄体生成素(LH)和促卵泡激素( FSH)水平;取大鼠肾上腺皮质组织,HE染色后光学显微镜观察肾上腺皮质束状带和网状带形态学变化,透射电子显微镜观察超微结构改变;采用RT-PCR技术分别检测大鼠肾上腺皮质和胸腹主动脉组织中COX-2 mRNA表达.结果 与对照组和替代组比较,去势组大鼠血清T水平显著降低(P<0.01),血清F、ACTH、LH和FSH水平显著升高(P<0.05).去势组大鼠肾上腺皮质束状带和网状带细胞变大,细胞核增大且常染色质增多,线粒体和滑面内质网也较发达,脂滴明显减少;替代组大鼠肾上腺皮质束状带和网状带的细胞形态和超微结构与对照组相似.去势组大鼠肾上腺皮质组织中COX-2 mRNA表达显著低于对照组和替代组(P<0.05),而主动脉血管组织中COX-2 mRNA表达明显高于对照组和替代组(P<0.05).结论 去势雄性大鼠肾上腺皮质束

  2. [Potentialities of computed tomography and ultrasound in diagnosis of hormonally active adrenal diseases: results of comparison CT and US with operative adn histological data].

    Science.gov (United States)

    Denisova, L B; Vorontsova, S V; Emel'ianova, L N

    2000-01-01

    The data given in the paper suggest that X-ray computed tomography (CT) is highly effective in detecting all types of hormonally active adrenal abnormalities. CT used in hormonally active adrenal diseases yielded data on major quantitative and qualitative (primarily densitometric) criteria that could be used in assessing the images of the adrenal area in these patients. Ultrasound study (USS) made at the first stage of topical diagnostic searches was of informative value in detecting adrenal tumor lesions, the technique being highly sensitive in the diagnosis of adrenal pheochromocytomas and adenocarcinomas, but less informative in the detection of hormonally active adrenocortical adenomas (aldesterone-producing ones in particular) than CT. The diagnosis of various adrenocortical hyperplasies and the differentiation of hyperplastic and tumor forms of hypercorticoidism are a prerogative of CT that substantially supplements USS findings in such cases.

  3. Adrenal insufficiency.

    Science.gov (United States)

    Li-Ng, Melissa; Kennedy, Laurence

    2012-10-01

    Adrenocortical insufficiency may arise through primary failure of the adrenal glands or due to lack of ACTH stimulation as a result of pituitary or hypothalamic dysfunction. Prolonged administration of exogenous steroids will suppress the hypothalamic-pituitary-adrenal axis, and hence cortisol secretion. We review briefly the causes, investigation, and treatment of adrenal insufficiency, and highlight aspects of particular relevance to patients with adrenal tumors.

  4. Adrenocorticotrophic hormone (ACTH) stimulation of sheep fetal adrenal cortex can occur without increased expression of ACTH receptor (ACTH-R) mRNA

    DEFF Research Database (Denmark)

    Carter, A M; Petersen, Y M; Towstoless, M;

    2002-01-01

    In the present study, it was hypothesized that the adrenocorticotrophin hormone receptor (ACTH-R) would be up-regulated in the adrenal gland of the sheep fetus following infusion of physiological amounts of ACTH, as shown for adrenal cortical cells in culture. In chronically catheterized sheep, a...

  5. Imaging findings of neonatal adrenal disorders

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hye Kyung; Han, Bo Kyung; Lee, Min Hee [Sungkyunkwan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-01-01

    In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surrounded by a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenal gland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenal hemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenal hemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findings in neonates with various adrenal disorders.

  6. Imaging of Adrenal Masses with Emphasis on Adrenocortical Tumors

    Directory of Open Access Journals (Sweden)

    Anders Sundin

    2012-01-01

    Full Text Available Because of the more widespread and frequent use of cross-sectional techniques, mainly computed tomography (CT, an increasing number of adrenal tumors are detected as incidental findings (“incidentalomas”. These incidentaloma patients are much more frequent than those undergoing imaging because of symptoms related to adrenal disease. CT and magnetic resonance imaging (MRI are in most patients sufficient for characterization and follow-up of the incidentaloma. In a minor portion of patients, biochemical screening reveals a functional tumor and further diagnostic work-up and therapy need to be performed according to the type of hormonal overproduction. In oncological patients, especially when the morphological imaging criteria indicate an adrenal metastasis, biopsy of the lesion should be considered after pheochromocytoma is ruled out biochemically. In the minority of patients in whom CT and MRI fail to characterize the tumor and when time is of essence, functional imaging mainly by positron emission tomography (PET is available using various tracers. The most used PET tracer, [18F]fluoro-deoxy-glucose (18FDG, is able to differentiate benign from malignant adrenal tumors in many patients. 11C-metomidate (11C-MTO is a more specialized PET tracer that binds to the 11-beta-hydroxylase enzyme in the adrenal cortex and thus makes it possible to differ adrenal tumors (benign adrenocortical adenoma and adrenocortical cancer from those of non-adrenocortical origin.

  7. Primary adrenal sarcomatoid carcinoma

    Directory of Open Access Journals (Sweden)

    Aftab S. Shaikh

    2014-03-01

    Full Text Available Adrenal sarcomatoid carcinomas are extremely rare tumors presenting with extensive locoregional spread at the time of diagnosis. Patients succumb to metastases within a couple of months. As a result, very few cases are reported in the literature until now. We present a case of a 62-year old female with non-functional sarcomatoid carcinoma of the right adrenal gland. There was no radiological evidence of locoregional metastases. Patient underwent right adrenalectomy. Follow up after 3 months showed para-aortic lymphadenopathy and similar left adrenal mass on computed tomography. Patient refused further treatment and succumbed to the disease. A brief case report with review of literature is presented.

  8. NCI-H295R, a human adrenal cortex-derived cell line, expresses purinergic receptors linked to Ca²⁺-mobilization/influx and cortisol secretion.

    Directory of Open Access Journals (Sweden)

    Haruhisa Nishi

    Full Text Available Purinergic receptor expression and involvement in steroidogenesis were examined in NCI-H295R (H295R, a human adrenal cortex cell line which expresses all the key enzymes necessary for steroidogenesis. mRNA/protein for multiple P1 (A(2A and A(2B, P2X (P2X₅ and P2X₇, and P2Y (P2Y₁, P2Y₂, P2Y₆, P2Y₁₂, P2Y₁₃, and P2Y₁₄ purinergic receptors were detected in H295R. 2MeS-ATP (10-1000 µM, a P2Y₁ agonist, induced glucocorticoid (GC secretion in a dose-dependent manner, while other extracellular purine/pyrimidine agonists (1-1000 µM had no distinct effect on GC secretion. Extracellular purines, even non-steroidogenic ones, induced Ca²⁺-mobilization in the cells, independently of the extracellular Ca²⁺ concentration. Increases in intracellular Ca²⁺ concentration induced by extracellular purine agonists were transient, except when induced by ATP or 2MeS-ATP. Angiotensin II (AngII: 100 nM and dibutyryl-cyclic AMP (db-cAMP: 500 µM induced both GC secretion and Ca²⁺-mobilization in the presence of extracellular Ca²⁺ (1.2 mM. GC secretion by AngII was reduced by nifedipine (10-100 µM; whereas the Ca²⁺ channel blocker did not inhibit GC secretion by 2MeS-ATP. Thapsigargin followed by extracellular Ca²⁺ exposure induced Ca²⁺-influx in H295R, and the cells expressed mRNA/protein of the component molecules for store-operated calcium entry (SOCE: transient receptor C (TRPC channels, calcium release-activated calcium channel protein 1 (Orai-1, and the stromal interaction molecule 1 (STIM1. In P2Y₁-knockdown, 2MeS-ATP-induced GC secretion was significantly inhibited. These results suggest that H295R expresses a functional P2Y₁ purinergic receptor for intracellular Ca²⁺-mobilization, and that P2Y₁ is linked to SOCE-activation, leading to Ca²⁺-influx which might be necessary for glucocorticoid secretion.

  9. Testicular adrenal rest tumors (TARTs) as a male infertility factor. Case report.

    Science.gov (United States)

    Niedziela, Marek; Joanna, Talarczyk; Piotr, JedrzejczaK

    2012-09-01

    Since testes and adrenal cortex derive from the same urogenital ridge, adrenal tissue with descending gonads may migrate in early embryonic period. Although most often ectopic tissue undergoes atrophy in some cases, when adrenocorticotrophic (ACTH) overstimulation occurs, the adrenal remnants in the testes may become hypertrophic and form testicular adrenal rest tumors (TARTs). The growth of TARTs in the testes leads to obstruction of the seminiferous tubules which can mechanically impair the function of the gonads and cause irreversible azoospermia. We describe a patient suffering since neonatal period from congenital adrenal hyperplasia (CAH), disorder with defected pathway of cortisol production, which leads to increased ACTH production and to overstimulation of adrenal cortex. He had very poor disease control and therefore in late puberty he was diagnosed with TARTs. At the age of 19.5 he was diagnosed with azoospermia, most likely caused by TARTs. It is the first evidence of TARTs in Polish literature. Although not many cases have been published so far the incidence of TARTs seems to be highly underdiagnosed, so it seems reasonable to consider the disease in differential diagnosis of male infertility.

  10. Neurosteroid biosynthetic pathways changes in prefrontal cortex in Alzheimer's disease.

    Science.gov (United States)

    Luchetti, Sabina; Bossers, Koen; Van de Bilt, Saskia; Agrapart, Vincent; Morales, Rafael Ramirez; Frajese, Giovanni Vanni; Swaab, Dick F

    2011-11-01

    Expression of the genes for enzymes involved in neurosteroid biosynthesis was studied in human prefrontal cortex (PFC) in the course of Alzheimer's disease (AD) (n=49). Quantitative RT-PCR (qPCR) revealed that mRNA levels of diazepam binding inhibitor (DBI), which is involved in the first step of steroidogenesis and in GABAergic transmission, were increased, as were mRNA levels for several neurosteroid biosynthetic enzymes. Aromatase, 17β-hydroxysteroid dehydrogenase type 1 (HSD17B1) and aldo-keto reductase 1C2 (AKR1C2), were all increased in the late stages of AD. Several GABA-A subunits were significantly reduced in AD. Increased expression of aromatase in the PFC was confirmed by immunohistochemistry and was found to be localized predominantly in astrocytes. These data suggest a role for estrogens and allopregnanolone produced by astrocytes in the PFC in AD, possibly as part of a rescue program. The reduced gene expression of some synaptic and extra-synaptic GABA-A subunits may indicate a deficit of modulation of GABA-A receptors by neuroactive steroids, which may contribute to the neuropsychiatric characteristics of this disease.

  11. Implications of infectious diseases and the adrenal hypothesis for the etiology of childhood acute lymphoblastic leukemia

    Directory of Open Access Journals (Sweden)

    F. Azevedo-Silva

    2010-03-01

    Full Text Available Acute leukemia is the most frequent cancer in children. Recently, a new hypothesis was proposed for the pathogenesis of childhood acute lymphoblastic leukemia (ALL. The so-called "adrenal hypothesis" emphasized the role of endogenous cortisol in the etiology of B-cell precursor ALL. The incidence peak of ALL in children between 3 to 5 years of age has been well documented and is consistent with this view. The adrenal hypothesis proposes that the risk of childhood B-cell precursor ALL is reduced when early childhood infections induce qualitative and quantitative changes in the hypothalamus-pituitary-adrenal axis. It suggests that the increased plasma cortisol levels would be sufficient to eliminate all clonal leukemic cells originating during fetal life. Because Brazil is a continental and tropical country, the exposure to infections is diversified with endemic viral and regionally non-viral infections, with some characteristics that support the recent adrenal hypothesis. Here we discuss this new hypothesis in terms of data from epidemiological studies and the possible implications of the diversity of infections occurring in Brazilian children.

  12. Malignant peripheral nerve sheath tumor of adrenal gland with heterologus osseous differentiation in a case of Von Recklinghausen′s disease

    Directory of Open Access Journals (Sweden)

    Manas R Baisakh

    2014-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST of the adrenal gland is extremely rare. Most of them occur in association with neurofibromatosis, ganglioneuroma or as part of a composite tumor such as pheochromocytoma. Only seven cases of MPNST of the adrenal gland have been reported in the literature till date. Discriminating this entity from other soft tissue sarcomas and gastrointestinal stromal tumor of the adrenal gland has important diagnostic and therapeutic implications. Moreover, the tumor size and pattern of expression for certain immunohistochemical markers may serve as independent predictors of aggressiveness. Herein we present a 24-years-old male with features of Von Recklinghausen′s disease who presented with large left adrenal gland malignant peripheral nerve sheath tumor.

  13. Radiological findings of congenital lipoid adrenal hyperplasia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Jeong; Shin, Joo Yong; Lee, Hee Jung; Lee, Jin Hee; Sohn, Cheol Ho; Lee, Sung Moon; Kim, Hong; Woo, Seong Ku; Suh, Soo Ji [Keimyung Univ. School of Medicine, Taegu (Korea, Republic of)

    2001-05-01

    Congenital lipoid adrenal hyperplasia (CLAH) is a rare autosomal recessive disorder characterized by the marked accumulation of lipids and cholesterol in the adrenal cortex, and the failure of adrenal steroids to synthesise. We report the ultrasound (US), computed tomographic (CT), and magnetic resonance (MR) imaging findings in a four-day-old female neonate with CLAH.

  14. Ação da melatonina sobre a apoptose e fator de crescimento endotelial vascular no córtex da adrenal de ratas pinealectomizadas Melatonin action in apoptosis and vascular endothelial growth factor in adrenal cortex of pinealectomized female rats

    Directory of Open Access Journals (Sweden)

    Luiz Fernando Portugal Fuchs

    2010-08-01

    Full Text Available OBJETIVO: avaliar os efeitos da melatonina na apoptose e atividade celular no córtex da adrenal de ratas pinealectomizadas. MÉTODOS: foram utilizadas 40 ratas adultas, divididas randomicamente em 4 grupos com 10 animais cada: GCont - sem intervenção cirúrgica e com ministração do veículo; GSham - sem retirar a pineal com ministração do veículo; GExpV - pinealectomizado e tratado com veículo; GExpM - pinealectomizado e tratado com melatonina (10 µg/animal, por dia durante a noite. Após 60 dias de tratamento, todos os animais foram submetidos à eutanásia, as adrenais retiradas, fixadas em formol a 10% e processadas para inclusão em parafina. Nos cortes histológicos com 5 µm de espessura, foram realizados métodos imunoistoquímicos para detecção da apoptose (Caspase-3-clivada e da atividade celular (fator de crescimento endotelial vascular, VEGF-A. Para avaliar a percentagem de apoptose foram contadas as células reativas em 1.000 células de cada zona da região cortical da adrenal em cada animal; já o VEGF-A foi expresso em escores. Os dados quantitativos foram analisados pela análise de variância (ANOVA e pelo teste de comparações múltiplas de Tukey-Kramer (pPURPOSE: to evaluate the reactivity of VEGF-A and cleaved caspase-3 in the adrenal gland cortex of female pinealectomized rats treated with melatonin. METHODS: forty adult female rats were divided into 4 groups (G of 10 animals: GI - no surgical intervention, with vehicle administration; GII - sham pinealectomized with vehicle administration; GIII - pinealectomized with vehicle administration; GIV - pinealectomized with melatonin administration (10 µg/animal during the night. After 60 days of treatment, all animals were anesthetized, and the adrenal glands were removed and fixed in 10% formaldehyde (phosphate buffered for histological processing and paraffin embedding. Sections (5 µm thick were collected on silanized slides and submitted to imunnohistochemical

  15. Prefrontal cortex white matter tracts in prodromal Huntington disease

    Science.gov (United States)

    Matsui, Joy T.; Vaidya, Jatin G.; Wassermann, Demian; Kim, Regina Eunyoung; Magnotta, Vincent A.; Johnson, Hans J.; Paulsen, Jane S.

    2015-01-01

    Huntington disease (HD) is most widely known for its selective degeneration of striatal neurons but there is also growing evidence for white matter (WM) deterioration. The primary objective of this research was to conduct a large-scale analysis using multisite diffusion-weighted imaging (DWI) tractography data to quantify diffusivity properties along major prefrontal cortex WM tracts in prodromal HD. Fifteen international sites participating in the PREDICT-HD study collected imaging and neuropsychological data on gene-positive HD participants without a clinical diagnosis (i.e. prodromal) and gene-negative control participants. The anatomical prefrontal WM tracts of the corpus callosum (PFCC), anterior thalamic radiations (ATR), inferior fronto-occipital fasciculi (IFO), and uncinate fasciculi (UNC) were identified using streamline tractography of DWI. Within each of these tracts, tensor scalars for fractional anisotropy, mean diffusivity, radial diffusivity, and axial diffusivity coefficients were calculated. We divided prodromal HD subjects into three CAG-age product (CAP) groups having Low, Medium, or High probabilities of onset indexed by genetic exposure. We observed significant differences in WM properties for each of the four anatomical tracts for the High CAP group in comparison to controls. Additionally, the Medium CAP group presented differences in the ATR and IFO in comparison to controls. Furthermore, WM alterations in the PFCC, ATR, and IFO showed robust associations with neuropsychological measures of executive functioning. These results suggest long-range tracts essential for cross-region information transfer show early vulnerability in HD and may explain cognitive problems often present in the prodromal stage. PMID:26179962

  16. Prefrontal cortex white matter tracts in prodromal Huntington disease.

    Science.gov (United States)

    Matsui, Joy T; Vaidya, Jatin G; Wassermann, Demian; Kim, Regina Eunyoung; Magnotta, Vincent A; Johnson, Hans J; Paulsen, Jane S

    2015-10-01

    Huntington disease (HD) is most widely known for its selective degeneration of striatal neurons but there is also growing evidence for white matter (WM) deterioration. The primary objective of this research was to conduct a large-scale analysis using multisite diffusion-weighted imaging (DWI) tractography data to quantify diffusivity properties along major prefrontal cortex WM tracts in prodromal HD. Fifteen international sites participating in the PREDICT-HD study collected imaging and neuropsychological data on gene-positive HD participants without a clinical diagnosis (i.e., prodromal) and gene-negative control participants. The anatomical prefrontal WM tracts of the corpus callosum (PFCC), anterior thalamic radiations (ATRs), inferior fronto-occipital fasciculi (IFO), and uncinate fasciculi (UNC) were identified using streamline tractography of DWI. Within each of these tracts, tensor scalars for fractional anisotropy, mean diffusivity, radial diffusivity, and axial diffusivity coefficients were calculated. We divided prodromal HD subjects into three CAG-age product (CAP) groups having Low, Medium, or High probabilities of onset indexed by genetic exposure. We observed significant differences in WM properties for each of the four anatomical tracts for the High CAP group in comparison to controls. Additionally, the Medium CAP group presented differences in the ATR and IFO in comparison to controls. Furthermore, WM alterations in the PFCC, ATR, and IFO showed robust associations with neuropsychological measures of executive functioning. These results suggest long-range tracts essential for cross-region information transfer show early vulnerability in HD and may explain cognitive problems often present in the prodromal stage. Hum Brain Mapp 36:3717-3732, 2015. © 2015 Wiley Periodicals, Inc.

  17. Disassembled DJ-1 high molecular weight complex in cortex mitochondria from Parkinson's disease patients

    Directory of Open Access Journals (Sweden)

    Adler Charles

    2009-07-01

    Full Text Available Abstract Correction to Nural H, He P, Beach T, Sue L, Xia W, Shen Y. Disassembled DJ-1 high molecular weight complex in cortex mitochondria from Parkinson's disease patients Molecular Neurodegeneration 2009, 4:23.

  18. REPORT OF 285 PATIENTS WITH CONGENITAL ADRENAL HYPERPLASIA AND EVALUATION OF APPROXIMATE REVALENCE OF THE DISEASE IN IRAN

    Directory of Open Access Journals (Sweden)

    H. Moayeri

    1999-07-01

    Full Text Available In this study, 285 cases of congenital adrenal hyperplasia who were followed in the Tehran University Hospitals and Institute of Endocrinology and Metabolism arc reported. Among these cases, 165 (57.9% were female and 120 (42.1%, male. The most common type of congenital adrenal hyperplasia in these patients was the salt-losing type of 21-hydroxylase deficiency (57.9%; 11-hydroxylase deficiency was present in 13.68% of patients. There were only 3 cases with 3-beta hydroxysteroid dehydrogenase deficiency, 2 cases with 17-alphahydroxylase deficiency and one with 20, 22-desmolase deficiency. Presenting complaints were in decreasing order of frequency: ambiguous genitalia, vomiting and dehydration, precocious puberty, hypertension, failure to thrive, hirsutism and primary amenorrhea. The age of patients at the time of diagnosis was between 2 days to 17 years and the most common age was in the first two years of life especially in the neonatal period."nA positive family history of the same disease was present in 17 siblings of our patients. (21-OHD = 14 H-OHD=3. There were 27 cases of death among these patients (23 male and 4 female that 24 cases had 21-OHD and 2 cases had 3 beta HSD deficiency and one case had 20,22-desmolase deficiency .

  19. Adrenal Cortical and Medullar Hyperplasia-A Retrospective Analysis of 6 Cases

    Institute of Scientific and Technical Information of China (English)

    陈敏; 鲁功成; 张齐均

    2002-01-01

    Summary: The features of the symptoms, laboratory tests and pathological characteristics of a-drenal cortical and medullary hyperplasia were studied. In 6 cases of hypercatecholaminenia, plas-ma norepinephrine (NE), epinephrine (E), catecholamine (CA) and 24-h urinary vanillylmandelicacid (VMA), 17-hydroxycorticosteroid (OHCS) and 17-ketosteroid (KS) were determined. A-drenal glands were examined by CT scan and 131I-MIBG imaging. Pathological examination wasperformed after operation. The results showed that in 6 cases of hypercatecholaminenia (3 menand 3 women) aged from 34-50 years, the clinical features were just like "pheochromocytoma",for example, episodic headache, perspiration, palpitation, pallor, apprehension, nausea, tremor,anxiety and so on. Plasma levels of CA, NE and E were elevated in all 6 cases. 24-h urinary sam-ples obtained at the onset revealed elevated VMA in 1 case. 24-h urinary cortisol was obviously el-evated in all 6 cases. 24-h urinary 17-OHCS, 17-KS was normal. B-type ultrasound, CT, MRIand 13I-MIBG revealed 9 lateral adrenal gland diffuse or nodular enlargement in 6 cases. Patholog-ic examination showed adrenal cortical and medullary hyperplasia. Clinically, adrenal cortical andmedullary hyperplasia resembled "pheochromocytoma". The most significant feature of this dis-ease was both elevated plasma CA and 24-h urinary cortisol obviously. Pathologic examinationshowed adrenal cortex nodular hyperplasia and medullar diffuse or limit hyperplasia. Whether it isan independent disease or symptoms of the other disease has not final conclusion up till now

  20. [Rosai-Dorfman disease presented with involvement of the adrenal gland. A clinical case reported].

    Science.gov (United States)

    Flores-Carrillo, Víctor Manuel; Santaella-Torres, Félix; Sánchez-Martínez, Luis Carlos; Gómez-Lara, Miguel Humberto; Arellano-Poblete, Moisés; López Segura-Rueda, Eduardo; Villarroel-Noboa, Jorge

    2014-01-01

    INTRODUCCIÓN: la afectación extranodal de la glándula suprarrenal es una forma infrecuente de la histiocitosis sinusal con linfadenopatía masiva o enfermedad de Rosai-Dorfman. Aún se desconoce la etiopatogenia de esta enfermedad. Los síntomas y signos de presentación en la afectación adrenal son inciertos. Aunque es una enfermedad de buen pronóstico y curso clínico favorable, el bajo índice de sospecha y el difícil diagnóstico radiológico diferencial llevan a tratamiento radical en la mayoría de los casos. CASO CLÍNICO: se describe el caso de una paciente con enfermedad de Rosai-Dorfman en quien se identificó tumor adrenal derecho de 97 × 99 × 68 mm y tumor nodal infrahiliar izquierdo de 61 × 58 × 57 mm. El tratamiento incluyó adrenalectomía y vigilancia activa del tumor infrahilial mediante tomografía y gammagrama renal. La evolución de la paciente fue adecuada.

  1. 结直肠癌化疗对肾上腺皮质功能影响的研究%The Influence of Chemotherapy on Adrenal Cortex of Colorectal Cancer Patients

    Institute of Scientific and Technical Information of China (English)

    陆晓峰; 李曙光

    2011-01-01

    Objective To investigate the influence of chemotherapy on adrenal cortex among colorectal cancer patients. Methods 27 patients were involved into the study. Levels of serous cortisol and aldosterone were detected and compared before and after chemotherapy. Results There was significant decrease in serous cortisol and aldosterone after chemotherapy ( P 0. 05 ) . There was no significant decrease in serous cortisol and aldosterone between male group and female group ( P > 0. 05 ) . Conclusion Chemotherapy can reduce the level of serous cortisol and aldosterone in colorectal cancer patients, and can inhibit the function of adrenal cortex.%目的 探讨结直肠癌化疗对肾上腺皮质功能的影响.方法 选取结直肠癌患者27例,检测化疗前后血清皮质醇、醛固酮水平,对比检测结果.结果 化疗后血清皮质醇、醛固酮水平较化疗前均显著降低,差异有统计学意义(P<0.05).Folfox4方案化疗组与mFolfox6化疗方案组皮质醇与醛固酮差值比较,差异均无统计学意义(P>0.05).男性与女性皮质醇与醛固酮差值比较,差异均无统计学意义(P>0.05).结论 化疗使结直肠癌患者血清皮质醇、醛固酮水平下降,能抑制肾上腺皮质功能.

  2. Adrenal Gland Disorders

    Science.gov (United States)

    ... t live without, including sex hormones and cortisol. Cortisol helps you respond to stress and has many other important functions. With adrenal gland disorders, your glands make too much or not enough hormones. In Cushing's ... too much cortisol, while with Addison's disease, there is too little. ...

  3. Inflammatory airway features and hypothalamic-pituitary adrenal axis function in asthmatic rats combined with chronic obstructive pulmonary disease

    Institute of Scientific and Technical Information of China (English)

    CAI Cui; CAO Yu-xue; ZHANG Hong-ying; LE Jing-jing; DONG Jing-cheng; CUI Yan; XU Chang-qing; LIU Bao-jun; WU Jin-feng; DUAN Xiao-hong

    2010-01-01

    Background Bronchial asthma (BA) and chronic obstructive pulmonary disease (COPD) are both inflammatory airway diseases with different characteristics. However, there are many patients who suffer from both BA and COPD. This study was to evaluate changes of inflammatory airway features and hypothalamic-pituitary-adrenal (HPA) axis function in asthmatic rats combined with COPD.Methods Brown Norway (BN) rats were used to model the inflammatory airway diseases of BA, COPD and COPD+BA.These three models were compared and evaluated with respect to clinical symptoms, pulmonary histopathology, airway hyperresponsiveness (AHR), inflammatory cytokines and HPA axis function.Results The inflammatory airway features and HPA axis function in rats in the COPD+BA model group were greatly influenced. Rats in this model group showed features of the inflammatory diseases BA and COPD. The expression of inflammatory cytokines in this model group might be up or downregulated when both disease processes are present. The levels of corticotrophin releasing hormone mRNA and corticosterone in this model group were both significantly decreased than those in the control group (P <0.05).Conclusions BN rat can be used as an animal model of COPD+BA. By evaluating this animal model we found that the features of inflammation in rats in this model group seem to be exaggerated. The HPA axis functions in rats in this model group have been disturbed or impaired, which is prominent at the hypothalamic level.

  4. Cell-to-cell communication in bilateral macronodular adrenal hyperplasia causing hypercortisolism

    Directory of Open Access Journals (Sweden)

    Herve eLefebvre

    2015-04-01

    Full Text Available It has been well established that, in the human adrenal gland, cortisol secretion is not only controlled by circulating corticotropin but is also influenced by a wide variety of bioactive signals, including conventional neurotransmitters and neuropeptides, released within the cortex by various cell types such as chromaffin cells, neurons, cells of the immune system, adipocytes and endothelial cells. These different types of cells are present in bilateral macronodular adrenal hyperplasia, a rare etiology of primary adrenal Cushing’s syndrome, where they appear intermingled with adrenocortical cells in the hyperplastic cortex. In addition, the genetic events which cause the disease favor abnormal adrenal differenciation that results in illicit expression of paracrine regulatory factors and their receptors in adrenocortical cells. All these defects constitute the molecular basis for aberrant autocrine/paracrine regulatory mechanisms which are likely to play a role in the pathophysiology of bilateral macronodular adrenal hyperplasia-associated hypercortisolism. The present review summarizes the current knowledge on this topic as well as the therapeutic perspectives offered by this new pathophysiological concept.

  5. The angiotensin hexapeptide 3-8 fragment potently inhibits [125I]angiotensin II binding to non-AT1 or -AT2 recognition sites in bovine adrenal cortex.

    Science.gov (United States)

    Jarvis, M F; Gessner, G W; Ly, C Q

    1992-08-25

    In the present studies, ligand competition experiments were conducted to examine the ability of angiotensin II peptide agonists and nonpeptide AT1- and AT2-selective receptor antagonists to inhibit the binding of [125I]angiotensin II to bovine adrenal cortical membranes. Angiotensin II, angiotensin III, the All-(3-8) hexapeptide fragment of angiotensin II, and the AT1-selective receptor antagonist L-158,809, inhibited [125I]angiotensin II binding in a biphasic fashion indicative of a ligand interaction at more than one recognition site. Approximately 20% of low affinity [125I]angiotensin II binding was inhibited only by high micromolar concentrations of L-158,809. RG 13647 (1(-1,4-benzodioxan-2-methyl)-5-diphenylacetyl-4,5,6,7-tetra hydro-1H-imidazo- [4,5,c]-pyridine-6-carboxylic acid) represents a potent and AT2-selective analog of PD 123177 and showed weak activity in competing for [125I]angiotensin II binding with an IC50 value of 100 microM. When subsequent competition studies were conducted in the presence of 1 microM L-158,809 to block [125I]angiotensin II to the AT1 receptor subtype, the angiotensin II agonists produced monophasic inhibition curves with AII-(3-8) showing the greatest activity (IC50 = 6 nM) followed by angiotensin III (IC50 = 15 nM) much greater than angiotensin II (IC50 = 110 nM). RG 13647 was not found to significantly inhibit this portion of [125I]angiotensin II binding. These data demonstrate that bovine adrenal cortex contains both the AT1 receptor subtype, as well as, a novel class of [125I]angiotensin II recognition sites which may be analogous to the recently described angiotensin IV (AT4) receptor.

  6. Giant Adrenal Myelolipoma Masquerading as Heart Failure

    Directory of Open Access Journals (Sweden)

    Parijat S. Joy

    2014-03-01

    Full Text Available Adrenal myelolipomas are rare benign tumors of the adrenal cortex composed of adipose and hematopoietic cells. They have been postulated to arise from repeated stimulation by stress, inflammation and ACTH oversecretion. Myelolipomas are usually detected incidentally on imaging and do not require any active intervention besides regular follow-up by imaging. However, myelolipomas may insidiously grow to large sizes and cause mass effects and hemorrhage. Timely diagnosis and surgical resection are curative and lifesaving.

  7. Giant adrenal myelolipoma masquerading as heart failure.

    Science.gov (United States)

    Joy, Parijat S; Marak, Creticus P; Nashed, Nadia S; Guddati, Achuta K

    2014-01-01

    Adrenal myelolipomas are rare benign tumors of the adrenal cortex composed of adipose and hematopoietic cells. They have been postulated to arise from repeated stimulation by stress, inflammation and ACTH oversecretion. Myelolipomas are usually detected incidentally on imaging and do not require any active intervention besides regular follow-up by imaging. However, myelolipomas may insidiously grow to large sizes and cause mass effects and hemorrhage. Timely diagnosis and surgical resection are curative and lifesaving.

  8. Attention and sentence processing deficits in Parkinson's disease: the role of anterior cingulate cortex.

    Science.gov (United States)

    Grossman, M; Crino, P; Reivich, M; Stern, M B; Hurtig, H I

    1992-01-01

    Parkinson's disease (PD) is a complex neurodegenerative condition involving a motor disorder that is related to reduced dopaminergic input to the striatum. Intellectual deficits are also seen in PD, but the pathophysiology of these difficulties is poorly understood. Regional cerebral blood flow (rCBF) was studied in neurologically intact subjects during the performance of attention-demanding, sentence processing tasks using positron emission tomography (PET). The results demonstrated significantly increased rCBF in a distributed set of cerebral regions during the detection of an adjective or a particular agent in a sentence, including anterior cingulate cortex, left inferior and middle frontal cortex, left inferior temporo-occipital cortex, posterolateral temporal cortex, left caudate, and left thalamus. We identified defects in this cerebral network by studying PD patients with two PET techniques. Resting PET studies revealed a significant correlation between regional cerebral glucose metabolism in anterior cingulate cortex and deficits in attending to subtle grammatical aspects of sentences. Studies of PD patients with the PET activation technique revealed little change in anterior cingulate and left frontal CBF during performance of the adjective detection or agent detection tasks. These data suggest that a defect in anterior cingulate cortex contributes to the cognitive impairments observed in PD.

  9. Widespread heterogeneous neuronal loss across the cerebral cortex in Huntington's disease.

    Science.gov (United States)

    Nana, Alissa L; Kim, Eric H; Thu, Doris C V; Oorschot, Dorothy E; Tippett, Lynette J; Hogg, Virginia M; Synek, Beth J; Roxburgh, Richard; Waldvogel, Henry J; Faull, Richard L M

    2014-01-01

    Huntington's disease is an autosomal dominant neurodegenerative disease characterized by neuronal degeneration in the basal ganglia and cerebral cortex, and a variable symptom profile. Although progressive striatal degeneration is known to occur and is related to symptom profile, little is known about the cellular basis of symptom heterogeneity across the entire cerebral cortex. To investigate this, we have undertaken a double blind study using unbiased stereological cell counting techniques to determine the pattern of cell loss in six representative cortical regions from the frontal, parietal, temporal, and occipital lobes in the brains of 14 Huntington's disease cases and 15 controls. The results clearly demonstrate a widespread loss of total neurons and pyramidal cells across all cortical regions studied, except for the primary visual cortex. Importantly, the results show that cell loss is remarkably variable both within and between Huntington's disease cases. The results also show that neuronal loss in the primary sensory and secondary visual cortices relate to Huntington's disease motor symptom profiles, and neuronal loss across the associational cortices in the frontal, parietal and temporal lobes is related to both Huntington's disease motor and to mood symptom profiles. This finding considerably extends a previous study (Thu et al., Brain, 2010; 133:1094-1110) which showed that neuronal loss in the primary motor cortex was related specifically to the motor symptom profiles while neuronal loss in the anterior cingulate cortex was related specifically to mood symptom profiles. The extent of cortical cell loss in the current study was generally related to the striatal neuropathological grade, but not to CAG repeat length on the HTT gene. Overall our findings show that Huntington's disease is characterized by a heterogeneous pattern of neuronal cell loss across the entire cerebrum which varies with symptom profile.

  10. [Congenital Adrenal Hyperplasia in Adults].

    Science.gov (United States)

    Vrbíková, Jana

    2016-01-01

    Congenital adrenal hyperplasia is a life-long disease requiring an integrated therapy. It may negatively influence the quality of life. In childhood, the main problems of the care of these patients involve sex determination and ensuring optimum growth and puberty. The therapeutic goals for adults are the prevention of Addisonian crisis and ensuring the best possible quality of life, including fertility.Key words: androgens - cardiovascular risk - congenital adrenal hyperplasia - bone density - testicular rest tumors.

  11. Budesonide-related adrenal insufficiency.

    Science.gov (United States)

    Arntzenius, Alexander; van Galen, Louise

    2015-10-01

    Iatrogenic adrenal insufficiency is a potential harmful side effect of treatment with corticosteroids. It manifests itself when an insufficient cortisol response to biological stress leads to an Addisonian crisis: a life-threatening situation. We describe a case of a patient who developed an Addisonian crisis after inappropriate discontinuation of budesonide (a topical steroid used in Crohn's disease) treatment. Iatrogenic adrenal insufficiency due to budesonide use has been rarely reported. Prescribers should be aware of the resulting risk for an Addisonian crisis.

  12. Effects of ACTH on RNA synthesis and migration in the adrenal cortex cells of the young rat, as shown by radioautography

    Energy Technology Data Exchange (ETDEWEB)

    Magalhaes, M.C.; Vitor, A.B.; Magalhaes, M.M.

    1986-01-01

    The effect of ACTH on the RNA synthesis in adrenal zona fasciculata cells of the young rat were studied by light and electron microscope radioautography. Two units of ACTH were administered sc to animals and immediately followed by an iv injection of (/sup 3/)uridine. ACTH-injected and control rats, which received the isotope alone, were sacrificed at various time intervals. Labelling over extranucleolar areas was higher in the ACTH-treated animals at 20 min, then becoming lower than in the controls at 60 min and 24 h. Nucleolar radioactivity, however, was consistently decreased by ACTH at all experimental times. Apart from these changes in the rate of synthesis, the over-all curves of labelling were similar to those in the control animals with a striking peak at 1 h. The short-term increase in extranucleolar RNA synthesis observed after ACTH injection was considered to be consistent with the hypothesis that an enhanced extranucleolar synthesis of mRNA takes place early in stimulated animals and is associated with the synthesis of steroidogenic proteins. On the other hand, the relatively decreased uridine uptake of the label by the nucleolus in ACTH-treated animals, suggests an inhibition of nucleolar transcription with diminished pre-rRNA formation in treated animals.

  13. Role of voltage-gated calcium channels in the regulation of aldosterone production from zona glomerulosa cells of the adrenal cortex.

    Science.gov (United States)

    Barrett, Paula Q; Guagliardo, Nick A; Klein, Peter M; Hu, Changlong; Breault, David T; Beenhakker, Mark P

    2016-10-15

    Zona glomerulosa cells (ZG) of the adrenal gland constantly integrate fluctuating ionic, hormonal and paracrine signals to control the synthesis and secretion of aldosterone. These signals modulate Ca(2+) levels, which provide the critical second messenger to drive steroid hormone production. Angiotensin II is a hormone known to modulate the activity of voltage-dependent L- and T-type Ca(2+) channels that are expressed on the plasma membrane of ZG cells in many species. Because the ZG cell maintains a resting membrane voltage of approximately -85 mV and has been considered electrically silent, low voltage-activated T-type Ca(2+) channels are assumed to provide the primary Ca(2+) signal that drives aldosterone production. However, this view has recently been challenged by human genetic studies identifying somatic gain-of-function mutations in L-type CaV 1.3 channels in aldosterone-producing adenomas of patients with primary hyperaldosteronism. We provide a review of these assumptions and challenges, and update our understanding of the state of the ZG cell in a layer in which native cellular associations are preserved. This updated view of Ca(2+) signalling in ZG cells provides a unifying mechanism that explains how transiently activating CaV 3.2 channels can generate a significant and recurring Ca(2+) signal, and how CaV 1.3 channels may contribute to the Ca(2+) signal that drives aldosterone production.

  14. Prefrontal cortex and striatal activation by feedback in Parkinson's disease

    NARCIS (Netherlands)

    Keitz, Martijn; Koerts, Janneke; Kortekaas, Rudie; Renken, Remco; de Jong, Bauke M.; Leenders, Klaus L.

    2008-01-01

    Positive feedbacks reinforce goal-directed behavior and evoke pleasure. in Parkinson's disease (PD) the striatal dysfunction impairs motor performance, but also may lead to decreased positive feedback (reward) processing. This study investigates two types of positive feedback processing (monetary fe

  15. Adrenal cysts

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    @@ Ture cysts of the adrenal gland are lined with endothelium or epithe lium.Most lesions are asympomatic and are discovered incidentally.They may produce s ymptoms because of hemorrhage.CT findings of cysts include(Fig 1): ① Cyst are well-marginated, nonenhancing, homogeneous, fluid-cont aining masses; ② The wall may have thin peripheral calcification if previous hemor rhage has occurred.③ Cyst contents have characteristics of simple fluids(<20 HU)unle ss hemorrhage has occurred.

  16. Interneurons, tau and amyloid-β in the piriform cortex in Alzheimer's disease.

    Science.gov (United States)

    Saiz-Sanchez, Daniel; De la Rosa-Prieto, Carlos; Ubeda-Banon, Isabel; Martinez-Marcos, Alino

    2015-07-01

    Impaired olfaction has been described as an early symptom of Alzheimer's disease. Neuroanatomical changes underlying this deficit in the olfactory system are largely unknown. Interestingly, neuropathology begins in the transentorhinal cortex and extends to the neighboring limbic system and basal telencephalic structures that mediate olfactory processing, including the anterior olfactory nucleus and olfactory bulb. The human piriform cortex has been described as a crucial area in odor quality coding; disruption of this region mediates early olfactory deficits in Alzheimer's disease. Most neuropathological investigations have focused on the entorhinal cortex and hippocampus, whereas the piriform cortex has largely been neglected. This work aims to characterize the expression of the neuropathological amyloid-β peptide, tau protein and interneuron population markers (calretinin, parvalbumin and somatostatin) in the piriform cortex of ten Alzheimer-diagnosed (80.4 ± 8.3 years old) and five control (69.6 ± 11.1) cases. Here, we examined the distribution of different interneuronal markers as well as co-localization of interneurons and pathological markers. Results indicated preferential vulnerability of somatostatin- (p = 0.0001 < α = 0.05) and calretinin-positive (p = 0.013 < α = 0.05) cells that colocalized with amyloid-β peptide, while the prevalence of parvalbumin-positive cells was increased (p = 0.045 < α = 0.05) in the Alzheimer's cases. These data may help to reveal the neural basis of olfactory deficits linked to Alzheimer's disease as well as to characterize neuronal populations preferentially vulnerable to neuropathology in regions critically involved in early stages of the disease.

  17. Visual cortex in aging and Alzheimer's disease: changes in visual field maps and population receptive fields.

    Science.gov (United States)

    Brewer, Alyssa A; Barton, Brian

    2014-01-01

    Although several studies have suggested that cortical alterations underlie such age-related visual deficits as decreased acuity, little is known about what changes actually occur in visual cortex during healthy aging. Two recent studies showed changes in primary visual cortex (V1) during normal aging; however, no studies have characterized the effects of aging on visual cortex beyond V1, important measurements both for understanding the aging process and for comparison to changes in age-related diseases. Similarly, there is almost no information about changes in visual cortex in Alzheimer's disease (AD), the most common form of dementia. Because visual deficits are often reported as one of the first symptoms of AD, measurements of such changes in the visual cortex of AD patients might improve our understanding of how the visual system is affected by neurodegeneration as well as aid early detection, accurate diagnosis and timely treatment of AD. Here we use fMRI to first compare the visual field map (VFM) organization and population receptive fields (pRFs) between young adults and healthy aging subjects for occipital VFMs V1, V2, V3, and hV4. Healthy aging subjects do not show major VFM organizational deficits, but do have reduced surface area and increased pRF sizes in the foveal representations of V1, V2, and hV4 relative to healthy young control subjects. These measurements are consistent with behavioral deficits seen in healthy aging. We then demonstrate the feasibility and first characterization of these measurements in two patients with mild AD, which reveal potential changes in visual cortex as part of the pathophysiology of AD. Our data aid in our understanding of the changes in the visual processing pathways in normal aging and provide the foundation for future research into earlier and more definitive detection of AD.

  18. Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    S. Al-Bahri

    2014-01-01

    Full Text Available Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH. We report the case of a 39-year-old male with a history of CAH secondary to 21-α hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing’s syndrome, Addison’s disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.

  19. CT diagnosis of adrenal abnormalities in patients with primary non-adrenal malignancies

    Energy Technology Data Exchange (ETDEWEB)

    Hussain, S.; Seltzer, S.E.; Abrams, H.L.; Belldegrun, A.; Richie, J.P.

    1986-05-01

    Fifty-seven patients with primary non-adrenal malignancy were found to have unsuspected adrenal abnormality on CT. In 33, comparison of histophatologic findings and/or the patients' hospital course or follow-up lead to the diagnosis of adrenal metastases (23), benign non-functioning adenomas (7), metastasis with hyperplasia (1), benign hyperplasia (1), and fatty infiltration (1). The analysis of CT findings indicated that: I) A heterogenous adrenal mass showing contrast enhancement was always metastatic, II) Nonfunctioning adenomas were always 3 cm or smaller in diameter, III) Bilateral adrenal masses and growth of adrenal mass on follow-up CT or regression on treatment indicated metastases, and IV) metastatic disease could not be excluded purely on the basis of the size of the adrenal mass.

  20. New onset Graves' disease as a cause of an adrenal crisis in an individual with panhypopituitarism: brief report

    Directory of Open Access Journals (Sweden)

    Lewandowski Krzysztof C

    2008-11-01

    Full Text Available Abstract 46 year old patient was admitted as an emergency with vomiting, hypotension and serum cortisol of 0,940 μg/dl (26 nmol/l indicative of adrenal failure. Despite previous history of panhypopituitarism he was found to be hyperthyroid [free T4 6.32 ng/dl (ref. range: 0.93–1.7, free T3 22.21 pg/ml (ref. range: 1.8–4.6]. He was fit and well till the age of 45. Eight months prior to this hospitalisation he presented with diabetes insipidus and was found to have a large cystic tumour in the area of the pituitary gland. Surgery was only partially successful and histologically the tumour was diagnosed as craniopharyngioma. Endocrine assessment revealed deficiency in ACTH-cortisol, growth hormone, and gonadotropin, as well as low-normal free T4. On the day of his emergency admission he looked ill and dehydrated, though was fully conscious and cooperative. Heart rate was 120 beats/min (sinus rhythm, blood pressure 85/40 mm Hg. There were no obvious features of infection, but there was marked tremor and thyroid bruit. He received treatment with intravenous fluids and hydrocortisone. L-thyroxine was stopped. Administration of large dose of methimazole (60 mg/day resulted in gradual decrease in free T4 and free T3 (to 1.76 ng/ml, and 5.92 pg/ml, respectively over a 15-day period. The patient was found to have increased titre of antithyroperoxidase (anti-TPO and anti-TSH receptor (anti-TSHR antibodies [2300 IU/l (ref. range Conclusion Our case illustrates coexistence of hypopituitarism and clinically significant autoimmune thyroid disease. The presence of hypopituitarism does not preclude the development of autoimmune thyrotoxicosis.

  1. Detection of disease-associated prion protein in the optic nerve and the adrenal gland of cattle with bovine spongiform encephalopathy by using highly sensitive immunolabeling procedures.

    Science.gov (United States)

    Okada, Hiroyuki; Iwamaru, Yoshifumi; Fukuda, Shigeo; Yokoyama, Takashi; Mohri, Shirou

    2012-04-01

    A sensitive immunohistochemical procedure, the tyramide signal amplification (TSA) system, was applied to detect the localization of immunolabeled disease-associated prion protein (PrP(Sc)) in cattle affected with bovine spongiform encephalopathy (BSE). In this procedure, immunolabeling could be visualized in the optic nerve and the adrenal medulla. In the optic nerve, the dual immunofluorescent technique showed that the granular PrP(Sc) was occasionally detected in the astrocytes, microglia, and myelin sheath adjacent to the axon. Clustered PrP(Sc) was also scattered in association with microglial cells and astrocytes of the optic nerve. In the adrenal gland, PrP(Sc) immunolabeling was confined within the sympathetic nerve fibers and endings. The results suggest that (1) PrP(Sc) might centrifugally spread within and between glial cells and/or the non-axonal (also known as ad-axonal) region of nerve fibers, rather than the axonal and/or extracellular space pathway in the optic nerve, and (2) the sympathetic innervations might be important for the trafficking of BSE agent in the adrenal glands of cattle. This study also suggests that tyramide-based immunochemical analysis should be performed to detect immunolabeled PrP(Sc) in the extracerebral tissues of BSE-affected cattle.

  2. PKA regulatory subunit 1A inactivating mutation induces serotonin signaling in primary pigmented nodular adrenal disease.

    Science.gov (United States)

    Bram, Zakariae; Louiset, Estelle; Ragazzon, Bruno; Renouf, Sylvie; Wils, Julien; Duparc, Céline; Boutelet, Isabelle; Rizk-Rabin, Marthe; Libé, Rossella; Young, Jacques; Carson, Dennis; Vantyghem, Marie-Christine; Szarek, Eva; Martinez, Antoine; Stratakis, Constantine A; Bertherat, Jérôme; Lefebvre, Hervé

    2016-09-22

    Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent hypercortisolism. The disease is primarily caused by germline mutations of the protein kinase A (PKA) regulatory subunit 1A (PRKAR1A) gene, which induces constitutive activation of PKA in adrenocortical cells. Hypercortisolism is thought to result from PKA hyperactivity, but PPNAD tissues exhibit features of neuroendocrine differentiation, which may lead to stimulation of steroidogenesis by abnormally expressed neurotransmitters. We hypothesized that serotonin (5-HT) may participate in the pathophysiology of PPNAD-associated hypercortisolism. We show that PPNAD tissues overexpress the 5-HT synthesizing enzyme tryptophan hydroxylase type 2 (Tph2) and the serotonin receptors types 4, 6, and 7, leading to formation of an illicit stimulatory serotonergic loop whose pharmacological inhibition in vitro decreases cortisol production. In the human PPNAD cell line CAR47, the PKA inhibitor H-89 decreases 5-HT4 and 5-HT7 receptor expression. Moreover, in the human adrenocortical cell line H295R, inhibition of PRKAR1A expression increases the expression of Tph2 and 5-HT4/6/7 receptors, an effect that is blocked by H-89. These findings show that the serotonergic process observed in PPNAD tissues results from PKA activation by PRKAR1A mutations. They also suggest that Tph inhibitors may represent efficient treatments of hypercortisolism in patients with PPNAD.

  3. PKA regulatory subunit 1A inactivating mutation induces serotonin signaling in primary pigmented nodular adrenal disease

    Science.gov (United States)

    Bram, Zakariae; Louiset, Estelle; Renouf, Sylvie; Duparc, Céline; Boutelet, Isabelle; Rizk-Rabin, Marthe; Libé, Rossella; Young, Jacques; Carson, Dennis; Vantyghem, Marie-Christine; Szarek, Eva; Martinez, Antoine; Stratakis, Constantine A.; Bertherat, Jérôme

    2016-01-01

    Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of ACTH-independent hypercortisolism. The disease is primarily caused by germline mutations of the protein kinase A (PKA) regulatory subunit 1A (PRKAR1A) gene, which induces constitutive activation of PKA in adrenocortical cells. Hypercortisolism is thought to result from PKA hyperactivity, but PPNAD tissues exhibit features of neuroendocrine differentiation, which may lead to stimulation of steroidogenesis by abnormally expressed neurotransmitters. We hypothesized that serotonin (5-HT) may participate in the pathophysiology of PPNAD-associated hypercortisolism. We show that PPNAD tissues overexpress the 5-HT synthesizing enzyme tryptophan hydroxylase type 2 (Tph2) and the serotonin receptors types 4, 6, and 7, leading to formation of an illicit stimulatory serotonergic loop whose pharmacological inhibition in vitro decreases cortisol production. In the human PPNAD cell line CAR47, the PKA inhibitor H-89 decreases 5-HT4 and 5-HT7 receptor expression. Moreover, in the human adrenocortical cell line H295R, inhibition of PRKAR1A expression increases the expression of Tph2 and 5-HT4/6/7 receptors, an effect that is blocked by H-89. These findings show that the serotonergic process observed in PPNAD tissues results from PKA activation by PRKAR1A mutations. They also suggest that Tph inhibitors may represent efficient treatments of hypercortisolism in patients with PPNAD. PMID:27699247

  4. Unilateral adrenal hemorrhagic infarction in essential thrombocythemia.

    Science.gov (United States)

    Burnet, G; Lambert, M; Annet, L; Lefebvre, C

    2015-12-01

    Adrenal hemorrhage is a rare disease associated with various conditions. We report a case of a 68-year-old woman with abdominal and back pain. The diagnostic work-up showed a left adrenal gland infarction associated with essential thrombocythemia. Treatment consisted in painkillers and treating the underlying condition in order to prevent further thrombotic events.

  5. A brief history of adrenal research: steroidogenesis - the soul of the adrenal.

    Science.gov (United States)

    Miller, Walter L

    2013-05-22

    The adrenal is a small gland that escaped anatomic notice until the 16th century, and whose essential role in physiology was not established until the mid 19th century. Early studies were confounded by failure to distinguish the effects of the cortex from those of the medulla, but advances in steroid chemistry permitted the isolation, characterization and synthesis of many steroids by the mid 20th century. Knowledge of steroid structures, radiolabeled steroid conversions, and the identification of accumulated urinary steroids in diseases of steroidogenesis permitted a generally correct description of the steroidogenic pathways, but one confounded by the failure to distinguish species-specific differences. The advent of cloning technologies and molecular genetics rapidly corrected and clarified the understanding of steroidogenic processes. Our laboratory in San Francisco was one of several contributing to this effort, focusing on human steroidogenic enzymes, the genetic disorders in their biosynthesis and the transcriptional and post-translational mechanisms regulating enzyme activity.

  6. 依托咪酯复合骶管麻醉对小儿皮质醇的影响%Effects of Etomidate on Pedo-adrenal Cortex Function in General Anesthesia

    Institute of Scientific and Technical Information of China (English)

    朱平增; 焦岩; 韩雪萍

    2012-01-01

    Objective:To study the effects of Etomidate on pedo-adrenal cortex function.Metheods:60 children scheduled for hypospadia-neoplasty,aged 5~6, ASA I ~ II, randomly arranged into etomidate group(group A),propofol group (group B) with 30 exaples in either group. After intravenous injiection ketamine 2 mg/kg ,all children were did caudal anesthesia. Anesthesia was maintained with etomidate (22~25) μ g/kg·min injection in group A orpropofol (8~10) mg/kg·h in Group B. Blood sample were taken before induction ( I ), immediately after 30 minutes pump-injected of Etomidate/Propofol(II) immediately, after operation(III) , 12hr (IV),24hr(V) and 48 hr(VI) after operation to measure plasma concentration of cortisol.Results:The concentration of cortisol of group B was significant higher than group B(P<0.05) at II ~-V, and restored to preoperative level in both groups at VI. Conclusion: Etomidate has some certain inhabit effects to adrenal cortex,it can be restored to baseline level at 48 hr after operation. Continuous infusion of etomidate was effective and safefor pedo-hypospadia neoplasty.%目的:观察持续输注依托咪酯复合骶管麻醉对小儿血清皮质醇浓度的影响.方法:60例择期行尿道下裂修补术患儿,年龄5~6岁,ASAⅠ~Ⅱ级,随机分为依托咪酯组(A组),丙泊酚组(B组),各30例.入室后均静脉注射氯胺酮2 mg/kg,入睡后常规骶管阻滞,术中麻醉维持:A组泵注依托咪酯脂肪乳(22~25)μg/kg·min,B组泵注丙泊酚(8~10)mg/kg·h.分别在麻醉前(Ⅰ)、泵注依托咪酯脂肪乳/丙泊酚30min(Ⅱ)、术后即刻(Ⅲ)、术后12h(Ⅳ)、术后24h(Ⅴ)、术后48h(Ⅵ)抽静脉血,测量血清皮质醇水平.结果:Ⅱ、Ⅲ、Ⅳ、Ⅴ时点A组血清皮质醇浓度低于B组(P<0.05),两组血清皮质醇浓度于Ⅵ时点均恢复至麻醉前水平.结论:依托咪酯脂肪乳对小儿肾上腺皮质功能的抑制作用短暂,术后48h恢复至麻醉前水平,可以应用于小儿静脉麻醉维持.

  7. A case of human intramuscular adrenal gland transplantation as a cure for chronic adrenal insufficiency.

    Science.gov (United States)

    Grodstein, E; Hardy, M A; Goldstein, M J

    2010-02-01

    Intramuscular endocrine gland transplantation has been well described as it pertains to parathyroid autotransplantation; however, transplantation of the adrenal gland is less well characterized. While adrenal autotransplantation in the setting of Cushing's disease has been described, intramuscular adrenal allotransplantation as a cure for adrenal insufficiency to our knowledge has not been previously carried out. Current treatment for adrenal insufficiency leaves patients without diurnal variation in cortisol release and susceptible to the detrimental effects of chronic hypercortisolism. We describe here the case of a 5-year-old girl with renal failure who had adrenal insufficiency following fulminant meningococcemia that led to requirements for both stress-dose steroid and mineralocorticoid replacement. Ten months after the onset of her disease, she received a simultaneous renal and adrenal gland transplant from her mother. The adrenal gland allograft was morselized into 1 mm(3) segments and implanted into three 2 cm pockets created in her rectus abdominis muscle. Three years after surgery, her allograft remains fully functional, responding well to adrenocorticotropin hormone stimulation and the patient does not require any steroid or mineral-corticoid supplementation. We believe this case represents the first description of successful functional intramuscular adrenal allograft transplantation with long-term follow up as a cure for adrenal insufficiency.

  8. Primary Bilateral Non-Hodgkin’s Lymphoma of the Adrenal Gland Presenting as Incidental Adrenal Masses

    Directory of Open Access Journals (Sweden)

    Christopher Rizzo

    2015-01-01

    Full Text Available Although lymphoma may occasionally involve the adrenal glands as part of a generalized disease process, primary adrenal lymphoma (PAL is a rare disease. We present a case of a 62-year-old woman with a history of mild/moderate hereditary spherocytosis with a well-compensated baseline haemoglobin, who presented with rapidly progressive symptomatic anaemia. During the diagnostic workup, imaging revealed bilateral large adrenal masses and she was later diagnosed with diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL, with the adrenal glands being the dominant site of the disease. The patient was started on systemic chemotherapy, but her disease progressed with neurological involvement which responded to second-line therapy. Her adrenal disease however was refractory to further therapy.

  9. Primary adrenal lymphoma with paraneoplastic syndrome

    Directory of Open Access Journals (Sweden)

    Radhika Dasararaju

    2013-01-01

    Full Text Available Context: The adrenal gland is a common site for neoplastic diseases and primary adrenal lymphoma (PAL is a rare tumor with around 120 cases reported so far. Case Report: We present a rare case of 76-year-old male who presented with headache, confusion, inappropriate body movements and abdominal pain. Adrenal biopsy revealed PAL and he has had an excellent neurologic outcome to date with chemotherapy and involved field radiation. Conclusion: The majority of cases of PAL are B cell lymphomas with diffuse large cell in 70% of cases. Clinical symptoms are variable and patients may present with abdominal pain, fever, anorexia, weight loss, fatigue or symptoms of adrenal insufficiency. Therapeutic modalities for PAL include surgery, chemotherapy and radiotherapy and corticosteroid replacement. With this case report, we hope to raise awareness about this rare disease and to include lymphoma in the differential of adrenal masses.

  10. A case of non-Hodgkin's lymphoma primary arising in both adrenal glands associated with adrenal failure.

    Science.gov (United States)

    Nishiuchi, Takamasa; Imachi, Hitomi; Fujiwara, Mako; Murao, Koji; Onishi, Hiroaki; Kiguchi, Tohru; Takimoto, Hidetaka; Kushida, Yoshio; Haba, Reiji; Ishida, Toshihiko

    2009-02-01

    It is known that adrenal insufficiency is one of the complications in primary adrenal lymphoma, especially those with bilateral adrenal involvement. A 73-year-old man was referred for general fatigue and high fever to the nearest hospital. The patient was transferred to our hospital for evaluation of bilateral adrenal tumors and hyponatremia. He was diagnosed as having non-Hodgkin's lymphoma (NHL) with primaries arising in both adrenal glands. Primary adrenal lymphoma (PAL) is a rare extra-nodal NHL. Although an appropriate treatment of this disease has not been established, our case has demonstrated that the combination of rituximab and THP-COP chemotherapy could be administered, and that it improved clinical manifestations. This case raises the suggestion that malignant lymphoma should be suspected in patients with bilateral adrenal tumors that present with progressive adrenal insufficiency.

  11. Role of cerebral cortex in the neuropathology of Huntington´s disease

    Directory of Open Access Journals (Sweden)

    Ana María Estrada-Sánchez

    2013-02-01

    Full Text Available An expansion of glutamine repeats in the N-terminal domain of the huntingtin protein leads to Huntington´s disease (HD, a neurodegenerative condition characterized by the presence of involuntary movements, dementia, and psychiatric disturbances. Evaluation of postmortem HD tissue indicates that the most prominent cell loss occurs in cerebral cortex and striatum, forebrain regions in which cortical pyramidal neurons (CPNs and striatal medium spiny neurons (MSNs are the most affected. Subsequent evidence obtained from HD patients and especially from transgenic mouse models of HD indicates that long before neuronal death, patterns of communication between CPNs and MSNs become dysfunctional. In fact, electrophysiological signaling in transgenic HD mice is altered even before the appearance of the HD behavioral phenotype, suggesting that dysfunctional cortical input to the striatum sets the stage for the emergence of HD neurological signs. Striatal MSNs, moreover, project back to cortex via multi-synaptic connections, allowing for even further disruptions in cortical processing. An effective therapeutic strategy for HD, therefore, may lie in understanding the synaptic mechanisms by which it dysregulates the corticostriatal system. Here, we review literature evaluating the molecular, morphological, and physiological alterations in the cerebral cortex, a key component of brain circuitry controlling motor behavior, as they occur in both patients and transgenic HD models.

  12. Adrenal Gland Tumors: Statistics

    Science.gov (United States)

    ... Gland Tumor: Statistics Request Permissions Adrenal Gland Tumor: Statistics Approved by the Cancer.Net Editorial Board , 03/ ... primary adrenal gland tumor is very uncommon. Exact statistics are not available for this type of tumor ...

  13. Adrenal Gland Cancer

    Science.gov (United States)

    ... either benign or malignant. Benign tumors aren't cancer. Malignant ones are. Most adrenal gland tumors are ... and may not require treatment. Malignant adrenal gland cancers are uncommon. Types of tumors include Adrenocortical carcinoma - ...

  14. Symptomatic Adrenal Insufficiency due to Bilateral Adrenal Non-Hodgkin’s Lymphoma

    Directory of Open Access Journals (Sweden)

    Şafak Akın

    2016-09-01

    Full Text Available Secondary involvement of the adrenal gland with non-Hodgkin’s lymphoma has been reported to occur in up to 25% of patients during the course of the disease. A 45-year-old man presented with a several month history of nausea, fatigue and weight loss. His medical history was unremarkable. Abdominal computed tomography (CT was performed and showed bilateral adrenal massive masses measuring 10x7.5 cm on the left and 4.8x4 cm on the right. He developed adrenal insufficiency in the follow-up period. The patient was started on replacement dose of prednisolone. A positron emission tomography-CT scan was acquired for further staging of the disease and showed intense fluorodeoxyglucose accumulation in both adrenal glands, additionally a slight fluorodeoxyglucose accumulation was observed in the ileocecal site. He did not accept adrenal biopsy or surgery. Histopathological examination of the ileocecal site revealed diffuse large B-cell lymphoma. He was administered rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. Bilateral adrenal lymphoma is a rare entity when compared with the incidence of adenoma and adrenal metastases of other cancers. Adrenal insufficiency may be the primary symptom of presentation, especially with bilateral involvement as in bilateral adrenal lymphoma.

  15. Altered regional homogeneity of prefrontal cortex in Parkinson's disease with mild cognitive impairment

    Institute of Scientific and Technical Information of China (English)

    DeZhi Kang; FuXiang Chen; FuYong Chen; Ying Liu; Gang Wu; LiangHong Yu; YuanXiang Lin

    2016-01-01

    Background:Mild cognitive impairment (MCI) is a common non-motor symptom of early Parkinson's disease (PD),but the neural mechanisms underlying it remain poorly understood.The aim of the present study was to investigate the characteristics of cognition-related brain activities in the PD patients with MCI.Methods:The brain fMRIs and cognition tests were acquired in 39 PD patients and 22 healthy controls (HC) from September 2013 to January 2015.The patients were divided into two groups:PD-MCI (n--18) and PD with normal cognition (PDNC,n =19).we used resting state fMRI and a regional homogeneity (ReHo) method to explore patterns of intrinsic brain activity in patients with PD-MCI as compared with PDNC subjects and HC.Results:Compared with the PDNC group,the PD-MCI group exhibited significantly increased ReHo in parts of the prefrontal cortex regions (e.g.right superior frontal gyrus,right middle frontal gyrus and orbitofrontal cortex).Compared to the HC group,a decrease of ReHo value in left thalamus was found in PD-MCI.However,this reduction was not found in the left thalamus of PDNC group,but in the above prefrontal regions (p < 0.05,with Bonferroni correction).Conclusions:These results demonstrate that the ReHo of prefrontal cortex in resting state is changed in PD patients with MCI.The presence of MCI in PD may be attributed to abnormal regional activity in prefrontal cortex regions.

  16. Entorhinal cortex disruption causes memory deficit in early Alzheimer's disease as shown by PET.

    Science.gov (United States)

    Eustache, F; Desgranges, B; Giffard, B; de la Sayette, V; Baron, J C

    2001-03-26

    Voxel-based mapping of the correlations between cognitive scores and resting-state brain glucose utilization measured by PET has recently emerged as a novel way to reveal in living patients with Alzheimer's disease (AD) the neural systems whose disruption underlies particular neuropsychological, especially mnemonic, deficits. We have now applied this approach using a novel cognitive paradigm designed to selectively assess verbal episodic memory, and show that in early AD disruption of the left entorhinal cortex underlies this memory deficit, consistent with post mortem data showing that this brain area is affected earliest and most severely by tau pathology in AD.

  17. Adrenal invovement in histoplasmosis

    OpenAIRE

    Norasyikin, A. Wahab; Nor Azmi, Kamaruddin; Rozita, Mohd; Suehazlyn, Zainudin

    2013-01-01

    Histoplasmosis infection is endemic in Asia and disseminated histoplasmosis (DH) is one form of its presentation (Benevides et al., 2007). DH commonly affects both adrenal glands. We describe a case of disseminated histoplasmosis complicated with hypercalcaemia in a 75-year-old immunosuppressed patient who presented with bilateral adrenal masses. The fine needle aspiration cytology of the adrenal mass was positive for Histoplasma capsulatum.

  18. Functional connectivity of primary motor cortex is dependent on genetic burden in prodromal Huntington disease.

    Science.gov (United States)

    Koenig, Katherine A; Lowe, Mark J; Harrington, Deborah L; Lin, Jian; Durgerian, Sally; Mourany, Lyla; Paulsen, Jane S; Rao, Stephen M

    2014-09-01

    Subtle changes in motor function have been observed in individuals with prodromal Huntington disease (prHD), but the underlying neural mechanisms are not well understood nor is the cumulative effect of the disease (disease burden) on functional connectivity. The present study examined the resting-state functional magnetic resonance imaging (rs-fMRI) connectivity of the primary motor cortex (M1) in 16 gene-negative (NEG) controls and 48 gene-positive prHD participants with various levels of disease burden. The results showed that the strength of the left M1 connectivity with the ipsilateral M1 and somatosensory areas decreased as disease burden increased and correlated with motor symptoms. Weakened M1 connectivity within the motor areas was also associated with abnormalities in long-range connections that evolved with disease burden. In this study, M1 connectivity was decreased with visual centers (bilateral cuneus), but increased with a hub of the default mode network (DMN; posterior cingulate cortex). Changes in connectivity measures were associated with worse performance on measures of cognitive-motor functioning. Short- and long-range functional connectivity disturbances were also associated with volume loss in the basal ganglia, suggesting that weakened M1 connectivity is partly a manifestation of striatal atrophy. Altogether, the results indicate that the prodromal phase of HD is associated with abnormal interhemispheric interactions among motor areas and disturbances in the connectivity of M1 with visual centers and the DMN. These changes may, respectively, contribute to increased motor symptoms, visuomotor integration problems, and deficits in the executive control of movement as individuals approach a manifest diagnosis.

  19. Editing for an AMPA receptor subunit RNA in prefrontal cortex and striatum in Alzheimer's disease, Huntington's disease and schizophrenia

    Science.gov (United States)

    Akbarian, S.; Smith, M. A.; Jones, E. G.; Bloom, F. E. (Principal Investigator)

    1995-01-01

    Animal studies and cell culture experiments demonstrated that posttranscriptional editing of the transcript of the GluR-2 gene, resulting in substitution of an arginine for glutamine in the second transmembrane region (TM II) of the expressed protein, is associated with a reduction in Ca2+ permeability of the receptor channel. Thus, disturbances in GluR-2 RNA editing with alteration of intracellular Ca2+ homeostasis could lead to neuronal dysfunction and even neuronal degeneration. The present study determined the proportions of edited and unedited GluR-2 RNA in the prefrontal cortex of brains from patients with Alzheimer's disease, in the striatum of brains from patients with Huntington's disease, and in the same areas of brains from age-matched schizophrenics and controls, by using reverse transcriptase-polymerase chain reaction, restriction endonuclease digestion, gel electrophoresis and scintillation radiometry. In the prefrontal cortex of controls, 99.9% were edited; in the prefrontal cortex both of schizophrenics and of Alzheimer's patients approximately 1.0% of all GluR-2 RNA molecules were unedited and 99% were edited. In the striatum of controls and of schizophrenics, approximately 0.5% of GluR-2 RNA molecules were unedited and 99.5% were edited; in the striatum of Huntington's patients nearly 5.0% of GluR-2 RNA was unedited. In the prefrontal white matter of controls, approximately 7.0% of GluR-2 RNA was unedited. In the normal human prefrontal cortex and striatum, the large majority of GluR-2 RNA molecules contains a CGG codon for arginine in the TMII coding region; this implies that the corresponding AMPA receptors have a low Ca2+ permeability, as previously demonstrated for the rat brain. The process of GluR-2 RNA editing is compromised in a region-specific manner in schizophrenia, in Alzheimer's disease and Huntington's Chorea although in each of these disorders there is still a large excess of edited GluR-2 RNA molecules. Disturbances of GluR-2 RNA

  20. Stem cells in the development and differentiation of the human adrenal glands.

    Science.gov (United States)

    Terada, Tadashi

    2015-01-01

    There are no studies on stem cells (SCs) and development and differentiation (DD) of the human adrenal glands. The SCs in DD of the adrenal glands were herein investigated histochemically and immunohistochemically in 18 human embryonic adrenal glands at gestational week (GW) 7-40. At 7 GW, the adrenal glands were present, and at 7 GW, numerous embryonic SCs (ESCs) are seen to create the adrenal cortex. The ESCs were composed exclusively of small cells with hyperchromatic nuclei without nucleoli. The ESCs were positive for neural cell adhesion molecule, KIT, neuron-specific enolase, platelet-derived growth factor receptor-α, synaptophysin, and MET. They were negative for other SC antigens, including chromogranin, ErbB2, and bcl-2. They were also negative for lineage antigens, including cytokeratin (CK)7, CK8, CK18, and CK19, carcinoembryonic antigen, carbohydrate antigen 19-9, epithelial membrane antigen, HepPar1, mucin core apoprotein (MUC)1, MUC2, MUC5AC, and MUC6, and cluster differentiation (CD)3, CD45, CD20, CD34, and CD31. The Ki-67 labeling index (LI) was high (Ki-67 LI = around 20%). α-Fetoprotein was positive in the ESCs and adrenal cells. The ESC was first seen in the periphery of the adrenal cortex at 7-10 GW. The ESC migrates into the inner part of the adrenal cortex. Huge islands of ESC were present near the adrenal, and they appeared to provide the ESC of the adrenal. At 16 GW, adrenal medulla appeared, and the adrenal ESCs were present in the periphery or the cortex, in the cortical parenchyma, corticomedullary junctions, and in the medulla. The adrenal essential architecture was established around 20 GW; however, there were still ESCs. At term, there are a few ESCs. These data suggest that the adrenal glands were created by ESCs.

  1. The structural involvement of the cingulate cortex in premanifest and early Huntington's disease.

    Science.gov (United States)

    Hobbs, Nicola Z; Pedrick, Amy V; Say, Miranda J; Frost, Chris; Dar Santos, Rachelle; Coleman, Allison; Sturrock, Aaron; Craufurd, David; Stout, Julie C; Leavitt, Blair R; Barnes, Josephine; Tabrizi, Sarah J; Scahill, Rachael I

    2011-08-01

    The impact of Huntington's disease neuropathology on the structure of the cingulate is uncertain, with evidence of both cortical enlargement and atrophy in this structure in early clinical disease. We sought to determine differences in cingulate volume between premanifest Huntington's disease and early Huntington's disease groups compared with controls using detailed manual measurements. Thirty controls, 30 subjects with premanifest Huntington's disease, and 30 subjects with early Huntington's disease were selected from the Vancouver site of the TRACK-HD study. Subjects underwent 3 Tesla magnetic resonance imaging and motor, cognitive, and neuropsychiatric assessment. The cingulate was manually delineated and subdivided into rostral, caudal, and posterior segments. Group differences in volume and associations with performance on 4 tasks thought to utilize cingulate function were examined, with adjustment for appropriate covariates. Cingulate volumes were, on average, 1.7 mL smaller in early Huntington's disease (P=.001) and 0.9 mL smaller in premanifest Huntington's disease (P=.1) compared with controls. Smaller volumes in subsections of the cingulate were associated with impaired recognition of negative emotions (P=.04), heightened depression (P=.009), and worse visual working memory performance (P=.01). There was no evidence of associations between volume and ability on a performance-monitoring task. This study disputes previous findings of enlargement of the cingulate cortex in Huntington's disease and instead suggests that the cingulate undergoes structural degeneration during early Huntington's disease with directionally consistent, nonsignificant differences seen in premanifest Huntington's disease. Cingulate atrophy may contribute to deficits in mood, emotional processing, and visual working memory in Huntington's disease.

  2. Temporal cortex direct current stimulation enhances performance on a visual recognition memory task in Alzheimer disease.

    Science.gov (United States)

    Boggio, P S; Khoury, L P; Martins, D C S; Martins, O E M S; de Macedo, E C; Fregni, F

    2009-04-01

    Several studies have reported that transcranial direct current stimulation (tDCS), a non-invasive method of neuromodulation, enhances some aspects of working memory in healthy and Parkinson disease subjects. The aim of this study was to investigate the impact of anodal tDCS on recognition memory, working memory and selective attention in Alzheimer disease (AD). Ten patients with diagnosis of AD received three sessions of anodal tDCS (left dorsolateral prefrontal cortex, left temporal cortex and sham stimulation) with an intensity of 2 mA for 30 min. Sessions were performed in different days in a randomised order. The following tests were assessed during stimulation: Stroop, Digit Span and a Visual Recognition Memory task (VRM). The results showed a significant effect of stimulation condition on VRM (p = 0.0085), and post hoc analysis showed an improvement after temporal (p = 0.01) and prefrontal (p = 0.01) tDCS as compared with sham stimulation. There were no significant changes in attention as indexed by Stroop task performance. As far as is known, this is the first trial showing that tDCS can enhance a component of recognition memory. The potential mechanisms of action and the implications of these results are discussed.

  3. Reduced pCREB in Alzheimer's disease prefrontal cortex is reflected in peripheral blood mononuclear cells

    Science.gov (United States)

    Bartolotti, N; Bennett, D A; Lazarov, O

    2016-01-01

    Cyclic-AMP response element-binding protein (CREB) signaling has a critical role in the formation of memories. CREB signaling is dysfunctional in the brains of mouse models of Alzheimer's disease (AD), and evidence suggests that CREB signaling may be disrupted in human AD brains as well. Here, we show that both CREB and its activated form pCREB-Ser133 (pCREB) are reduced in the prefrontal cortex of AD patients. Similarly, the transcription cofactors CREB-binding protein (CBP) and p300 are reduced in the prefrontal cortex of AD patients, indicating additional dysfunction of CREB signaling in AD. Importantly, we show that pCREB expression is reduced in peripheral blood mononuclear cells (PBMC) of AD subjects. In addition, pCREB levels in PBMC positively correlated with pCREB expression in the postmortem brain of persons with AD. These results suggest that pCREB expression in PBMC may be indicative of its expression in the brain, and thus offers the intriguing possibility of pCREB as a biomarker of cognitive function and disease progression in AD. PMID:27480489

  4. [Pediatric emergency: adrenal insufficiency and adrenal crisis].

    Science.gov (United States)

    Martínez, Alicia; Pasqualini, Titania; Stivel, Mirta; Heinrich, Juan Jorge

    2010-04-01

    Adrenal insufficiency is defined by impaired secretion of adrenocortical hormones. It is classified upon the etiology in primary and secondary. Rapid recognition and therapy of adrenocortical crisis are critical to survival. Patients often have nonspecific symptoms: anorexia, vomiting, weakness, fatigue and lethargy. They are followed by hypotension, shock, hypoglicemia, hyponatremia and hyperkalemia. All patients with adrenal insufficiency require urgent fluid reposition, correction of hypoglycemia and glucocorticoid replacement, in order to avoid serious consequences of adrenal crisis. After initial crisis treatment, maintenance dose of corticoids should be indicated. Mineralocorticoids replacement, if necessary, should also be initiated.

  5. Adrenal insufficiency and adrenal replacement therapy. Current status in Spain.

    Science.gov (United States)

    Aulinas, Anna; Casanueva, Felipe; Goñi, Fernando; Monereo, Susana; Moreno, Basilio; Picó, Antonio; Puig-Domingo, Manel; Salvador, Javier; Tinahones, Francisco J; Webb, Susan M

    2013-03-01

    Adrenal insufficiency (AI) is a rare endocrine disease, associated to increased mortality if left untreated. It can be due to a primary failure of the adrenal glands (primary AI) or malfunctioning of the hypothalamic-pituitary-adrenal axis (HPA) (secondary AI). The lack of data on incidence/prevalence of adrenal insufficiency in Spain complicates any evaluation of the magnitude of the problem in our country. Initial symptoms are non-specific, so often there is a delay in diagnosis. Current therapy with available glucocorticoids is associated with decreased quality of life in patients with treated AI, as well as with increased mortality and morbidity, probably related to both over-treatment and lack of hydrocortisone, associated with non-physiological peaks and troughs of the drug over the 24 hours. The availability of a new drug with a modified dual release (immediate and retarded), that requires one only daily dose, improves and simplifies the treatment, increases compliance as well as quality of life, morbidity and possibly mortality. This revision deals with the knowledge on the situation both globally and in Spain, prior to the availability of this new drug.

  6. IMAGe association: report of two cases in siblings with adrenal hypoplasia and review of the literature.

    Science.gov (United States)

    Phillips, Katherine; Arroyo, May R; Duckworth, Lizette Vila

    2014-01-01

    We report the postmortem findings of two siblings with gross and microscopic features consistent with IMAGe association (Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital anomalies) with an emphasis on the histopathology of the adrenal gland in this rare syndrome. The first sibling was an 8-week old male diagnosed postnatally with primary adrenal insufficiency. There was no deletion of the DAX1 gene by FISH. Examination at autopsy revealed dysmorphic features including frontal bossing, epicanthal folds, flat philtrum, cryptorchidism, penile chordee, overriding fourth toe, and height and weight below 3rd percentile. Grossly, the adrenal glands were not identified; however, microscopic examination of the suprarenal soft tissue revealed a 3 mm focus of disorganized fetal adrenal cortex with distended "cytomegalic" cells with abundant pink eosinophilic cytoplasm, vesicular nuclei, and cytoplasmic vacuolization. A minute focus of permanent adult cortex was also seen, but no adrenal medulla was identified. An autopsy of the sibling, who died 12 years previously at day 9 of life, revealed dysmorphic facial features with cryptorchidism and a large phallus. The adrenal glands were grossly hypoplastic (11 mm). Histologically, the adrenal glands showed disorganized fetal cortex with cytomegalic cells, a larger amount of permanent adult cortex, and bizarre nuclei with numerous pseudoinclusions. While there is currently limited information regarding the histopathologic adrenal findings in IMAGe association, our small case series suggests overlapping features between X-linked recessive congenital adrenal hypoplasia (cytomegalic cells with lack of permanent adult cortex) and autosomal recessive congenital adrenal hypoplasia (diminished permanent adult cortex without cytomegalic cells).

  7. Adrenal adrenoceptors in heart failure

    Directory of Open Access Journals (Sweden)

    Claudio ede Lucia

    2014-07-01

    Full Text Available Heart failure (HF is a chronic clinical syndrome characterized by the reduction in left ventricular (LV function and it represents one of the most important causes of morbidity and mortality worldwide. Despite considerable advances in pharmacological treatment, HF represents a severe clinical and social burden. Sympathetic outflow, characterized by increased circulating catecholamines (CAs biosynthesis and secretion, is peculiar in HF and sympatholytic treatments (as β-blockers are presently being investigated for the treatment of this disease. Adrenal gland secretes Epinephrine (80% and Norepinephrine (20% in response to acetylcholine stimulation of nicotinic cholinergic receptors on the chromaffin cell membranes. This process is regulated by adrenergic receptors (ARs: α2ARs inhibit CA release through coupling to inhibitory Gi-proteins, and βARs (mainly β2ARs stimulate CA release through coupling to stimulatory Gs-proteins. All ARs are G-protein-coupled receptors (GPCRs and GPCR kinases (GRKs regulate their signaling and function. Adrenal GRK2-mediated α2AR desensitization and downregulation are increased in HF and seem to be a fundamental regulator of CA secretion from the adrenal gland. Consequently, restoration of adrenal a2AR signaling through the inhibition of GRK2 is a fascinating sympatholytic therapeutic strategy for chronic HF. This strategy could have several significant advantages over existing HF pharmacotherapies (antiadrenergic, such as bAR-blockers minimizing side-effects on extra-cardiac tissues and reducing the chronic activation of the renin–angiotensin–aldosterone and endothelin systems.The role of adrenal ARs in regulation of sympathetic hyperactivity opens interesting perspectives in understanding pathophysiology of HF and identifying new potential therapeutic targets.

  8. Characterization of L-[3H]nicotine binding in human cerebral cortex: comparison between Alzheimer's disease and the normal.

    Science.gov (United States)

    Flynn, D D; Mash, D C

    1986-12-01

    Putative nicotine receptors in the human cerebral cortex were characterized with L-[3H]nicotine, L-[3H]Nicotine binding was enhanced by the addition of Ca2+ and abolished in the presence of Na3EDTA. Association and dissociation of the ligand were rapid at 25 degrees C with t1/2 values of 2 and 3 min, respectively. Saturation binding analysis revealed an apparent single class of sites with a dissociation constant of 5.6 nM and a Hill coefficient of 1.05. There was no effect of postmortem interval on the density of binding sites assayed up to 24 h in rat frontoparietal cortex. Nicotine binding in human cortical samples was also unaltered by increasing sampling delay. In human cortical membranes, binding site density decreased with normal aging. Receptor affinity and concentration in samples of frontal cortex (Brodmann area 10) from patients with Alzheimer's disease were comparable to age-matched control values. Samples of infratemporal cortex (Brodmann area 38) from patients with Alzheimer's disease had a 50% reduction in the number of L-[3H]nicotine sites. Choline acetyltransferase activity was significantly decreased in both cortical areas. Enzyme activities in the temporal pole were reduced to 20% of control values. These data indicate that postsynaptic nicotine receptors are spared in the frontal cortex in Alzheimer's disease. In the infratemporal cortex, significant numbers of receptors remain despite the severe reduction in choline acetyltransferase activity. Replacement therapy directed at these sites may be warranted in Alzheimer's disease.

  9. EXPRESSION OF ENDOGENOUS OUABAIN IN MULTIPLE ADRENAL TUMORS

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective To explore expression of endogenous ouabain(EO) in multiple adrenal tumors.Methods Thirty-one cases of adrenal tumors and 6 cases of healthy adrenal tissues were selected. The expression of EO in the adrenal tiss ue was detected with immunohistochemical streptavidin peroxadase conjugated(SP) method.Results Most of EO positive products were localized in cy toplasm of the zona reticularis of human adrenal cortex, and positive products s howed to be fine granular. There was no positive signal in the medulla. EO showe d on diffused positive in patients with pheochromocytoma accompanied high blood pressure[SBP:(165.22±7.61) mmHg, DBP:(105.52±4.26) mmHg], but there were neg ative in ones with normative blood pressure[SBP:(118.52±4.58) mmHg, DBP:(83±3.60) m mHg]. The expression of EO was positive in all adrenocortical hyperplasic, aden oma an d carcinoma, no matter its high or normative blood pressure. The degree of expre ssion of EO in adrenal tissues was related to the level of BP.Conclusion Expression of endogenous ouabain(EO) in health y adrenal tissue and adrenal tumors was a valuable morphological and pathophysio logical clue for the research on ouabain.

  10. Novel insights into adrenal insufficiency in childhood.

    Science.gov (United States)

    Levy-Shraga, Y; Pinhas-Hamiel, O

    2014-12-01

    Adrenal insufficiency (AI) is a rare disease in childhood and adolescence that results from disruption in the hypothalamic-pituitary-adrenal axis. Pediatricians should be familiar with this entity since acute adrenal crisis is a life-threatening condition that requires immediate treatment. In the early stages of AI, the clinical manifestations may be subtle and non-specific; thus, they are frequently unrecognized. The main therapeutic approach in all forms of adrenal insufficiency is glucocorticoid replacement; the dose should be titrated appropriately to avoid under or overtreatment. Patient and family education is particularly important, to enable adjustment of dosage replacement therapy during stress and to prevent crisis. This article summarizes the current knowledge of AI and provides new insights on its management in children.

  11. Differential damage in the frontal cortex with aging, sporadic and familial Alzheimer's disease.

    Science.gov (United States)

    Leuba, Geneviève; Vernay, André; Zimmermann, Vincent; Saini, Krishan; Kraftsik, Rudolf; Savioz, Armand

    2009-10-28

    In order to understand relationships between executive and structural deficits in the frontal cortex of patients within normal aging or Alzheimer's disease, we studied frontal pathological changes in young and old controls compared to cases with sporadic (AD) or familial Alzheimer's disease (FAD). We performed a semi-automatic computer assisted analysis of the distribution of beta-amyloid (Abeta) deposits revealed by Abeta immunostaining as well as of neurofibrillary tangles (NFT) revealed by Gallyas silver staining in Brodman areas 10 (frontal polar), 12 (ventro-infero-median) and 24 (anterior cingular), using tissue samples from 5 FAD, 6 sporadic AD and 10 control brains. We also performed densitometric measurements of glial fibrillary acidic protein, principal compound of intermediate filaments of astrocytes, and of phosphorylated neurofilament H and M epitopes in areas 10 and 24. All regions studied seem almost completely spared in normal old controls, with only the oldest ones exhibiting a weak percentage of beta-amyloid deposit and hardly any NFT. On the contrary, all AD and FAD cases were severely damaged as shown by statistically significant increased percentages of beta-amyloid deposit, as well as by a high number of NFT. FAD cases (all from the same family) had statistically more beta-amyloid and GFAP than sporadic AD cases in both areas 10 and 24 and statistically more NFT only in area 24. The correlation between the percentage of beta-amyloid and the number of NFT was significant only for area 24. Altogether, these data suggest that the frontal cortex can be spared by AD type lesions in normal aging, but is severely damaged in sporadic and still more in familial Alzheimer's disease. The frontal regions appear to be differentially vulnerable, with area 12 having the less amyloid burden, area 24 the less NFT and area 10 having both more amyloid and more NFT. This pattern of damage in frontal regions may represent a strong neuroanatomical support for the

  12. What is normal in normal aging? Effects of aging, amyloid and Alzheimer's disease on the cerebral cortex and the hippocampus

    OpenAIRE

    Fjell, Anders M.; McEvoy, Linda; Holland, Dominic; Dale, Anders M; Walhovd, Kristine B.

    2014-01-01

    What can be expected in normal aging, and where does normal aging stop and pathological neurodegeneration begin? With the slow progression of age-related dementias such as Alzheimer's disease (AD), it is difficult to distinguish age-related changes from effects of undetected disease. We review recent research on changes of the cerebral cortex and the hippocampus in aging and the borders between normal aging and AD. We argue that prominent cortical reductions are evident in fronto-temporal reg...

  13. Visual cortex in aging and Alzheimer’s disease: Changes in visual field maps and population receptive fields

    Directory of Open Access Journals (Sweden)

    Alyssa A. Brewer

    2014-02-01

    Full Text Available Although several studies have suggested that cortical alterations underlie such age-related visual deficits as decreased acuity, little is known about what changes actually occur in visual cortex during healthy aging. Two recent studies showed changes in primary visual cortex (V1 during normal aging; however, no studies have characterized the effects of aging on visual cortex beyond V1, important measurements both for understanding the aging process and for comparison to changes in age-related diseases. Similarly, there is almost no information about changes in visual cortex in Alzheimer’s disease (AD, the most common form of dementia. Because visual deficits are often reported as one of the first symptoms of AD, measurements of such changes in the visual cortex of AD patients might improve our understanding of how the visual system is affected by neurodegeneration as well as aid early detection, accurate diagnosis and timely treatment of AD. Here we use fMRI to first compare the visual field map (VFM organization and population receptive fields (pRFs between young adults and healthy aging subjects for occipital VFMs V1, V2, V3, and hV4. Healthy aging subjects do not show major VFM organizational deficits, but do have reduced surface area and increased pRF sizes in the foveal representations of V1, V2, and hV4 relative to healthy young control subjects. These measurements are consistent with behavioral deficits seen in healthy aging. We then demonstrate the feasibility and first characterization of these measurements in two patients with mild AD, which reveal potential changes in visual cortex as part of the pathophysiology of AD. Our data aid in our understanding of the changes in the visual processing pathways in normal aging and provide the foundation for future research into earlier and more definitive detection of AD.

  14. Ontogeny of innervation of rat and ovine fetal adrenals.

    Science.gov (United States)

    Engeland, W C; Wotus, C; Rose, J C

    1998-01-01

    The formation of adrenocortical zonation occurs in rats during late gestation. Since adult cortical function is modulated by neural mediators, it is possible that the development of differentiated function is dependent on cortical innervation. The goal of this study was to compare the pattern and timing of rodent and ovine adrenal innervation during late organogenesis by staining with antibodies directed against the neuropeptides vasoactive intestinal peptide (VIP), calcitonin gene-related peptide (CGRP) and neuropeptide tyrosine (NPY) and the catecholamine biosynthetic enzyme, tyrosine hydroxylase (TOH). Rat adrenals were collected from fetal days 17-21 (term=21 days) and ovine adrenals from fetal days 101-136 (term=145 days). Adrenals were fixed, cryosectioned at 100 microns and immunostained using Cy3-conjugated secondary antibodies. In both species, staining of VIP, CGRP, NPY and TOH fibers was observed in the capsule and subcapsular layers of the cortex during gestation. In late gestation, VIP- and NPY-positive ganglions cells were observed near the medulla extending processes toward the outer cortex; in ovine adrenals, fibers from ganglion cells appeared to surround nests of outer cortical (presumably, zona glomerulosa) cells. These data show that phenotypically distinct neural elements appear at different stages of adrenocortical development. The presence of neural elements in contact with adrenal cortical cells supports the possibility for neural control of adrenocortical development.

  15. Adrenal lesions encountered in current medical practice − a review of their radiological imaging

    Directory of Open Access Journals (Sweden)

    Vanesha Naidu

    2013-11-01

    Full Text Available Modern radiological technology has transformed the way that adrenal lesions are currently investigated. The contemporary radiologist has been catapulted to the forefront in the management of adrenal disease. With the increasing use of cross-sectional imaging, adrenal lesions are being serendipitously discovered in radiological studies undertaken for non-adrenal-related conditions – the so-called adrenal ‘incidentaloma’. This review discusses the imaging modalities available for characterising these lesions, highlighting current concepts and controversies in differentiating benign from malignant pathology. The article also provides a brief overview of the spectrum of adrenal pathology commonly encountered in the adult population.

  16. [Methylmercury causes diffuse damage to the somatosensory cortex: how to diagnose Minamata disease].

    Science.gov (United States)

    Ekino, Shigeo; Ninomiya, Tadashi; Imamura, Keiko; Susa, Mari

    2007-01-01

    The first acute case of methylmercury (MeHg) poisoning by the consumption of fish arose in Minamata, Japan, in 1953. It was officially recognized and called Minamata disease (MD) in 1956. There are still arguments about the definition of MD in terms of its associated clinical symptoms and lesions even 50 years after the initial recognition of MD. Studies on this MD epidemic are reviewed along with its historical background. Since MeHg dispersed from Minamata to the Shiranui Sea, residents living around the sea had been exposed to low-dose MeHg through fish consumption for about 20 years (at least from 1950 to 1968). These chronic MeHg poisoning patients complained of paresthesia at the distal parts of their extremities and around the lips even 30 years after the cessation of exposure to MeHg of anthropogenic origin. The persisting somatosensory disorders after the discontinuation of exposure to MeHg were induced by diffuse damage to the somatosensory cortex, but not by damage to the peripheral nervous system, as previously believed. Based on these findings, symptoms and lesions in MeHg poisoning are reappraised.

  17. GATA transcription factors in adrenal development and tumors.

    Science.gov (United States)

    Parviainen, Helka; Kiiveri, Sanne; Bielinska, Malgorzata; Rahman, Nafis; Huhtaniemi, Ilpo T; Wilson, David B; Heikinheimo, Markku

    2007-02-01

    Of the six GATA transcription factors, GATA-4 and GATA-6 are expressed in the mouse and human adrenal with distinct developmental profiles. GATA-4 is confined to the fetal cortex, i.e. to the less differentiated proliferating cells, while GATA-6 is expressed both in the fetal and adult adrenal. In vitro, GATA-4 regulates inhibin-alpha and steroidogenic factor-1 implicated in normal adrenal function. GATA-6 probably has roles in the development and differentiation of adrenocortical cells, and in the regulation of steroidogenesis. GATA-4 expression is dramatically upregulated and GATA-6 downregulated in gonadotropin dependent mouse adrenocortical tumors. This is accompanied by the appearance of luteinizing hormone receptor (LHR). In vitro, GATA-4 transactivates LHR promoter, and gonadotropins upregulate GATA-4 levels. Human adrenal tumors occasionally express GATA-4, whereas GATA-6 levels are usually lower than normal.

  18. Dexamethasone-suppression adrenal scintigraphy in hyperandrogenism: concise communication

    Energy Technology Data Exchange (ETDEWEB)

    Gross, M.D.; Freitas, J.E.; Swanson, D.P.; Woodbury, M.C.; Schteingart, D.E.; Beierwaltes, W.H.

    1981-01-01

    To assess the contribution of adrenal-derived androgens in women with hirsutism, adrenal scintigrams under dexamethasone suppression (DS) were performed on 35 women with increasing facial or body hair and irregular or absent menses. Based upon the DS regimen chosen (8 mg/d for 2 days or 4 md/d for 7 days before the injection of 6..beta..-(/sup 131/I)iodomethylnorcholesterol), three imaging patterns were identified. The first was the absence of uptake before 3 days (8-mg DS) or before 5 days (4-mg DS) after injection. This imaging pattern was seen in 17 of the 35 patients studied and was considered normal. The second pattern was bilateral uptake earlier than 3 days (8-mg DS regimen) or 5 days (4-mg DS) after injection. This was seen in 13 of the 35 patients and was interpreted as bilateral early visualization. Adrenal-vein catheterization performed on six patients with this pattern showed increased adrenal-vein testosterone. The third pattern, observed in five patients, was unilateral early visualization, which in four cases investigated to date was the result of an adrenocortical adenoma. This study confirms the adrenal cortex as a source of androgens in women with hirsutism and hyperandrogenism and demonstrates that DS adrenal scintigraphy can be utilized to identify those women in whom adrenal-derived androgens contribute to their hyperandrogenism.

  19. Comparison of the effect of high-dose inhaled budesonide and fluticasone on adrenal function in patients with severe chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Ahmed Fahim

    2012-01-01

    Conclusion: Inhaled budesonide and fluticasone have no significantly different effect on adrenal function in moderate to severe COPD. The adverse event profile of high-dose inhaled steroids should not influence the choice of medication.

  20. Iatrogenic Cushing's Disease in a Boy after Misdiagnosis of Salt-Losing Virilizing Adrenal Hyperplasia: Impaired Metyrapone Response with Failure of Catch-Up Growth

    Science.gov (United States)

    Mendilaharzu, Hernan; And Others

    1973-01-01

    A boy misdiagnosed as having the sodium-losing form of virilizing adrenal hyperplasia was treated with large doses of glucocorticoids and mineralocorticoids from the newborn period until he was more than 4 years of age. (Author)

  1. Congenital adrenal hyperplasia

    Science.gov (United States)

    ... their genitalia during infancy. Steroids used to treat congenital adrenal hyperplasia do not usually cause side effects such as obesity or weak bones, because the doses replace the hormones that the ...

  2. [Adrenal neoplasms in childhood. Description of a clinical case and review of the literature].

    Science.gov (United States)

    Calzi, P; Bertanza, C; Gargantini, L; Bianchi, C

    1987-01-01

    Hyperadrenocorticism caused by tumors of adrenal cortex is uncommon in children; it occurs more frequently in girls and on the left side. The clinical manifestations depend upon age and sex of patients; virilization is the predominant sign in about two thirds of the cases, and Cushing's syndrome in the others. Diagnosis is made by hormonal and instrumental evaluations. Therapy is surgical eradication. Histology has not proved to be useful because pleiomorphism and capsular invasion have been found in clinically benign tumors. High mortality rate in older literature may have been due to post-operative complications and inadequate steroid replacement; in more recent issues good prognosis is referred in pediatric patients. Early diagnosis and therapy are important to prevent adverse effect on growth and development. After an up-to-date review of the literature, a case of adrenocortical tumor is reported. We present a 3 years old boy with pseudoprecocious puberty (accelerated growth and bone age, sexual hair, facial acne, penile enlargement, hoarse voice) and a diagnosis of testosterone-producing adrenal tumor was confirmed by endocrine and radiological investigations. Removal of functioning adrenal tumor was followed by rapid regression of most of the clinical signs of the disease; plasma and urinary steroids returned to normal values. Twelve months after eradication of the tumor the patient is in good health; long-term-follow-up is necessary to exclude any relapse.

  3. Bilateral adrenal histoplasmosis in an immunocompetent man Histoplasmose adrenal bilateral em um homem imunocompetente

    Directory of Open Access Journals (Sweden)

    Carlos Frederico Lopes Benevides

    2007-04-01

    Full Text Available Histoplasmosis is a fungal disease that is endemic in Brazil. It may present as chronic pulmonary infection or in disseminated form. Disseminated histoplasmosis frequently affects the adrenal gland; however, unilateral involvement in immunosuppressed patients is the usual presentation. We report a case of an elderly immunocompetent male with history of weight loss, fever and bilateral adrenal mass who was successfully treated with itraconazole.Histoplasmose é uma doença fúngica endêmica no Brasil que pode se apresentar como infecção pulmonar crônica ou na forma disseminada. A histoplasmose disseminada freqüentemente acomete a glândula adrenal; entretanto, ocorre mais em pacientes imunossuprimidos e de forma unilateral. Relatamos um caso de um homem idoso imunocompetente com história de perda de peso, febre e massa adrenal bilateral que foi tratada com itraconazol, com sucesso.

  4. Synchronous laparoscopic resection of colorectal and renal/adrenal neoplasms.

    Science.gov (United States)

    Ng, Simon S M; Lee, Janet F Y; Yiu, Raymond Y C; Li, Jimmy C M; Leung, Ka Lau

    2007-08-01

    Synchronous laparoscopic resections of coexisting abdominal diseases are shown to be feasible without additional postoperative morbidity. We report our experience with synchronous laparoscopic resection of colorectal carcinoma and renal/adrenal neoplasms with an emphasis on surgical and oncologic outcomes. Five patients diagnosed to have synchronous colorectal carcinoma and renal/adrenal neoplasms (renal cell carcinoma in 2 patients, adrenal cortical adenoma in 2 patients, and adrenal metastasis in 1 patient) underwent synchronous laparoscopic resection. The median operative time was 420 minutes and the median operative blood loss was 1000 mL. Three patients developed minor complications, including wound infection in 2 patients and retention of urine in 1 patient. There was no operative mortality. The median duration of hospital stay was 11 days. At a median follow-up of 17.6 months, no patient developed recurrence of disease. Synchronous laparoscopic resection of colorectal and renal/adrenal neoplasms is technically feasible and safe.

  5. Scene construction impairments in Alzheimer's disease - A unique role for the posterior cingulate cortex.

    Science.gov (United States)

    Irish, Muireann; Halena, Stephanie; Kamminga, Jody; Tu, Sicong; Hornberger, Michael; Hodges, John R

    2015-12-01

    Episodic memory dysfunction represents one of the most prominent and characteristic clinical features of patients with Alzheimer's disease (AD), attributable to the degeneration of medial temporal and posterior parietal regions of the brain. Recent studies have demonstrated marked impairments in the ability to envisage personally relevant events in the future in AD. It remains unclear, however, whether AD patients can imagine fictitious scenes free from temporal constraints, a process that is proposed to rely fundamentally upon the integrity of the hippocampus. The objective of the present study was to investigate the capacity for atemporal scene construction, and its associated neural substrates, in AD. Fourteen AD patients were tested on the scene construction task and their performance was contrasted with 14 age- and education-matched healthy older Control participants. Scene construction performance was strikingly compromised in the AD group, with significant impairments evident for provision of contextual details, spatial coherence, and the overall richness of the imagined experience. Voxel-based morphometry analyses based on structural MRI revealed significant associations between scene construction capacity and atrophy in posterior parietal and lateral temporal brain structures in AD. In contrast, scene construction performance in Controls was related to integrity of frontal, parietal, and medial temporal structures, including the parahippocampal gyrus and posterior hippocampus. The posterior cingulate cortex (PCC) emerged as the common region implicated for scene construction performance across participant groups. Our study highlights the importance of regions specialised for spatial and contextual processing for the construction of atemporal scenes. Damage to these regions in AD compromises the ability to construct novel scenes, leading to the recapitulation of content from previously experienced events.

  6. Is the subcallosal medial prefrontal cortex a common site of atrophy in Alzheimer’s disease and frontotemporal lobar degeneration?

    Directory of Open Access Journals (Sweden)

    Olof eLindberg

    2012-11-01

    Full Text Available Regions affected late in neurodegenerative disease are thought to be anatomically connected to regions affected earlier. The subcallosal medial prefrontal cortex (SMPC has connections with the dorsolateral prefrontal cortex (DLPFC, orbitofrontal cortex (OFC and hippocampus (HC, which are regions that may become atrophic in frontotemporal lobar degeneration (FTLD and Alzheimer’s disease (AD. We hypothesized that the SMPC is a common site of frontal atrophy in the FTLD subtypes and in AD. The volume of the SMPC, DLPFC, OFC, HC and entorhinal cortex were manually delineated for 12 subjects with frontotemporal dementia (FTD, 13 with semantic dementia (SD, 9 with progressive nonfluent aphasia (PNFA, 10 AD cases and 13 controls. Results revealed significant volume loss in the left SMPC in FTD, SD and PNFA, while the right SMPC was also atrophied in SD and FTD. In AD a non significant tendency of volume loss in the left SMPC was found (p=0.08, with no volume loss on the right side. Results indicated that volume loss reflected the degree of brain connectivity. In SD and AD temporal regions displayed most atrophy. Among the frontal regions, the SMPC (which receives the strongest temporal projections demonstrated most volume loss, the OFC (which receives less temporal projections less volume loss, while the DLPFC (which is at multisynaptic distance from the temporal regions demonstrated no volume loss. In PNFA, the left SMPC was atrophic, possibly reflecting progression from the left anterior insula, while FTD patients may have had SMPC atrophy at the initial stages of the disease. Atrophy of the SMPC may thus be affected by either initial temporal or initial frontal atrophy, making it a common site of frontal atrophy in the dementia subtypes investigated.

  7. 5-HT2A Receptor Binding in the Frontal Cortex of Parkinson's Disease Patients and Alpha-Synuclein Overexpressing Mice

    DEFF Research Database (Denmark)

    Rasmussen, Nadja Bredo; Olesen, Mikkel Vestergaard; Brudek, Tomasz;

    2016-01-01

    function are also steadily being associated with this disease. Not much is known about the pathophysiology behind this. The aim of this study was thereby twofold: (1) to investigate receptor binding levels in Parkinson’s brains and (2) to investigate whether PD associated pathology, alpha-synuclein (AS...... by increased receptor binding in PD brains. In a separate study, we looked for changes in receptors in the prefrontal cortex in 52-week-old transgenic mice overexpressing human AS. We performed region-specific receptor binding measurements followed by gene expression analysis. The transgenic mice showed lower...... binding in the frontal association cortex that was not accompanied by changes in gene expression levels. This study is one of the first to look at differences in serotonin receptor levels in PD and in relation to AS overexpression....

  8. Imaging of the adrenal gland lesions

    Energy Technology Data Exchange (ETDEWEB)

    Herr, Keith [Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA (United States); Muglia, Valdair F. [Universidade de Sao Paulo (FMRP/USP), Ribeirao Preto, SP (Brazil). Faculdade de Medicina; Koff, Walter Jose [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Faculdade de Medicina. Dept. de Cirurgia; Westphalen, Antonio Carlos, E-mail: antonio.westphalen@ucsf.edu [Departments of Radiology and Biomedical Imaging and Urology, School of Medicine, University of California, San Francisco, CA (United States)

    2014-07-15

    With the steep increase in the use of cross-sectional imaging in recent years, the incidentally detected adrenal lesion, or 'incidentaloma', has become an increasingly common diagnostic problem for the radiologist, and a need for an approach to classifying these lesions as benign, malignant or indeterminate with imaging has spurred an explosion of research. While most incidentalomas represent benign disease, typically an adenoma, the possibility of m alignant involvement of the adrenal gland necessitates a reliance on imaging to inform management decisions. In this article, we review the literature on adrenal gland imaging, with particular emphasis on computed tomography, magnetic resonance imaging, and photon-emission tomography, and discuss how these findings relate to clinical practice. Emerging technologies, such as contrast-enhanced ultrasonography, dual-energy computed tomography, and magnetic resonance spectroscopic imaging will also be briefly addressed. (author)

  9. Imaging of the adrenal gland lesions

    Directory of Open Access Journals (Sweden)

    Keith Herr

    2014-08-01

    Full Text Available With the steep increase in the use of cross-sectional imaging in recent years, the incidentally detected adrenal lesion, or "incidentaloma", has become an increasingly common diagnostic problem for the radiologist, and a need for an approach to classifying these lesions as benign, malignant or indeterminate with imaging has spurred an explosion of research. While most incidentalomas represent benign disease, typically an adenoma, the possibility of malignant involvement of the adrenal gland necessitates a reliance on imaging to inform management decisions. In this article, we review the literature on adrenal gland imaging, with particular emphasis on computed tomography, magnetic resonance imaging, and photon-emission tomography, and discuss how these findings relate to clinical practice. Emerging technologies, such as contrast-enhanced ultrasonography, dual-energy computed tomography, and magnetic resonance spectroscopic imaging will also be briefly addressed.

  10. Delay in Diagnosis of Adrenal Insufficiency Is a Frequent Cause of Adrenal Crisis

    Directory of Open Access Journals (Sweden)

    Lucyna Papierska

    2013-01-01

    Full Text Available Delay of diagnosis of primary adrenal insufficiency (PAI leads to adrenal crisis which is potentially lethal complication. The objective of our work was an assessment whether the establishment of diagnosis of adrenocortical insufficiency in Poland is so much delayed as assessed in the past. We have analysed data from 60 patients with diagnosis of PAI established in our department during the past 12 years and who are still under our care. We found that the time to diagnosis of primary adrenal insufficiency in Poland exceeds 3 months in every patient and 6 months in patients admitted with symptoms of adrenal crisis. Forty-four percent of patients were diagnosed only just after the hospitalisation due to crisis, despite the evident signs and symptoms of PAI. Lack of appetite and loss of body weight occurred in all patients and for that reason a diagnosis of chronic gastric and duodenal ulcer disease was the most often incorrect diagnosis. After the proper diagnosis and treatment, in the course of 1–11 years of observation, there was only 6 imminent adrenal crises in 5 patients. Our results indicated that training of primary care physicians in the field of recognising and treatment of adrenal insufficiency is still essential.

  11. Evidence of adrenal failure in aging Dax1-deficient mice.

    Science.gov (United States)

    Scheys, Joshua O; Heaton, Joanne H; Hammer, Gary D

    2011-09-01

    Dosage-sensitive sex reversal, adrenal hypoplasia congenita (AHC) critical region on the X chromosome, gene 1 (Dax1) is an orphan nuclear receptor essential for development and function of the mammalian adrenal cortex and gonads. DAX1 was cloned as the gene responsible for X-linked AHC, which is characterized by adrenocortical failure necessitating glucocorticoid replacement. Contrary to these human data, young mice with genetic Dax1 knockout (Dax1(-/Y)) exhibit adrenocortical hyperfunction, consistent with the historic description of Dax1 as a transcriptional repressor that inhibits steroidogenic factor 1-dependent steroidogenesis. This paradox of molecular function and two apparently opposite phenotypes associated with Dax1 deficiency in mice and humans is compounded by the recent observations that under certain circumstances, Dax1 can serve as a transcriptional activator of steroidogenic factor 1. The recently revealed role of Dax1 in embryonic stem cell pluripotency, together with the observation that its expression in the adult adrenal is restricted to the subcapsular cortex, where presumptive undifferentiated progenitor cells reside, has led us to reexamine the phenotype of Dax1(-/Y) mice in order to reconcile the conflicting mouse and human data. In this report, we demonstrate that although young Dax1(-/Y) mice have enhanced steroidogenesis and subcapsular adrenocortical proliferation, as these mice age, they exhibit declining adrenal growth, decreasing adrenal steroidogenic capacity, and a reversal of their initial enhanced hormonal sensitivity. Together with a marked adrenal dysplasia in aging mice, these data reveal that both Dax1(-/Y) mice and patients with X-linked AHC exhibit adrenal failure that is consistent with adrenocortical subcapsular progenitor cell depletion and argue for a significant role of Dax1 in maintenance of these cells.

  12. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  13. Imaging of adrenal disorders

    Energy Technology Data Exchange (ETDEWEB)

    Fukuchi, S. (Fukushima Medical Coll. (Japan))

    1982-03-01

    Adrenal scintillation scanning, CT and ultrasonography are compared with the conventional imaging methods. The accuracy of retroperitoneal pneumography and adrenal venography are not high, and they detected only large tumors such as Cushing's syndrome and pheochromocytoma. Scintillation scanning is highly effective for the diagnoses of primary aldosteronism and Cushing's syndrome. However, this technique does not visualize pheochromocytoma or hypopituitarism. CT is noninvasive and of high diagnostic value. It is impossible to diagnose tumors by ultrasonography unless the size is more than 3 cm.

  14. Exposure to an Extremely-Low-Frequency Magnetic Field Stimulates Adrenal Steroidogenesis via Inhibition of Phosphodiesterase Activity in a Mouse Adrenal Cell Line.

    Science.gov (United States)

    Kitaoka, Kazuyoshi; Kawata, Shiyori; Yoshida, Tomohiro; Kadoriku, Fumiya; Kitamura, Mitsuo

    2016-01-01

    Extremely low-frequency magnetic fields (ELF-MFs) are generated by power lines and household electrical devices. In the last several decades, some evidence has shown an association between ELF-MF exposure and depression and/or anxiety in epidemiological and animal studies. The mechanism underlying ELF-MF-induced depression is considered to involve adrenal steroidogenesis, which is triggered by ELF-MF exposure. However, how ELF-MFs stimulate adrenal steroidogenesis is controversial. In the current study, we investigated the effect of ELF-MF exposure on the mouse adrenal cortex-derived Y-1 cell line and the human adrenal cortex-derived H295R cell line to clarify whether the ELF-MF stimulates adrenal steroidogenesis directly. ELF-MF exposure was found to significantly stimulate adrenal steroidogenesis (p steroidogenesis via an increase in intracellular cAMP caused by the inhibition of phosphodiesterase activity in Y-1 cells. The same mechanism may trigger the increase in adrenal steroid secretion in mice observed in our previous study.

  15. Preliminary evidence of altered steroidogenesis in women with Alzheimer's disease: Have the patients "OLDER" adrenal zona reticularis?

    Science.gov (United States)

    Vaňková, Markéta; Hill, Martin; Velíková, Marta; Včelák, Josef; Vacínová, Gabriela; Dvořáková, Kateřina; Lukášová, Petra; Vejražková, Daniela; Rusina, Robert; Holmerová, Iva; Jarolímová, Eva; Vaňková, Hana; Kancheva, Radmila; Bendlová, Běla; Stárka, Luboslav

    2016-04-01

    Alzheimer's disease (AD) represents more than half of total dementias. Various factors including altered steroid biosynthesis may participate in its pathophysiology. We investigated how the circulating steroids (measured by GC-MS and RIA) may be altered in the presence of AD. Sixteen women with AD and 22 age- and BMI-corresponding controls aged over 65 years were enrolled in the study. The steroid levels (47 steroids and steroid polar conjugates) and their ratios in AD female patients indicated increased CYP11A1 activity, weakened activity of the CYP17A1C17,20 lyase metabolic step and attenuated sulfotransferase SULT2A1 activity at higher activity of the CYP17A1 17-hydroxylase step. The patients showed diminished HSD3B2 activity for C21 steroids, abated conversion of 17-hydroxyprogesterone to cortisol, and significantly elevated cortisol. The women with AD had also attenuated steroid 7α-hydroxylation forming immunoprotective Δ(5)-C19 steroids, attenuated aromatase activity forming estradiol that induces autoimmunity and a shift from the 3β-hydroxy-5α/β-reduced C19 steroids to their neuroinhibitory and antiinflammatory GABAergic 3α-hydroxy- counterparts and showed higher levels of the 3α-hydroxy-5α/β-reduced C21 steroids and pregnenolone sulfate (improves cognitive abilities but may be both protective and excitotoxic). Our preliminary data indicated functioning of alternative "backdoor" pathway in women with AD showing higher levels of both 5α/β-reduced C21 steroids but reduced levels of both 5α/β-reduced C21 steroids, which implied that the alternative "backdoor" pathway might include both 5α- and 5β-reduced steroids. Our study suggested relationships between AD status in women based on the age of subjects and levels of 10 steroids measured by GC-MS.

  16. Lymphocytic adrenal medullitis and lymphocytic thyroiditis in a laboratory beagle dog.

    Science.gov (United States)

    Doi, Takuya; Tomonari, Yuki; Kawasako, Kazufumi; Yamada, Naoaki; Tsuchitani, Minoru

    2017-02-04

    Lymphocytic adrenal medullitis characterized by inflammation and atrophy in the medulla of the bilateral adrenal glands was observed in an 18-month-old male laboratory beagle dog. It might be that the present lymphocytic adrenal medullitis is an autoimmune-mediated disease as the histological characteristics are consistent with an autoimmune pathogenesis. However, the actual cause remains unclear as the existence of serum autoantibodies against the adrenal medulla could not be confirmed. Although this dog also contracted lymphocytic thyroiditis along with serum thyroglobulin autoantibodies, indicating that the thyroiditis occurred with an autoimmune basis; the relation between the adrenal medullitis and thyroiditis is unknown.

  17. [Hypoplasia adrenal congenita of anencephalic type: two cases with pituitary abnormalities and review of literature].

    Science.gov (United States)

    Folligan, K; Roume, J; Razavi, F; Sepaniak, S; Bouvier, R; Morel, Y; Trouillas, J

    2011-03-01

    Hypoplasia adrenal congenita is an extremely uncommon disease of early onset. This condition can be lethal in the absence of treatment. Some forms are due to the congenital adrenal hypoplasia of anencephalic type whose origin is even unknown. Here, we present two cases of congenital adrenal hypoplasia of anencephalic type with pituitary abnormalities. The two male newborns died because adrenal insufficiency in the neonatal period. The adrenal glands were hypoplastic with a histological structure of anencephalic type Immunocytochemical study of the pituitary revealed an absence of the gonadotrophs. No mutation of DAX 1 and SF-1 was found.

  18. Levodopa reinstates connectivity from prefrontal to premotor cortex during externally paced movement in Parkinson's disease

    DEFF Research Database (Denmark)

    Herz, Damian M; Siebner, Hartwig R; Hulme, Oliver J

    2014-01-01

    Dopamine deficiency affects functional integration of activity in distributed neural regions. It has been suggested that lack of dopamine induces disruption of neural interactions between prefrontal and premotor areas, which might underlie impairment of motor control observed in patients...... to examine oscillatory coupling between prefrontal and premotor areas during respectively low and high levels of dopamine. In 10 patients and 12 control participants multiple source beamformer analysis yielded task-related activation of a contralateral cortical network comprising prefrontal cortex (PFC...

  19. Bilateral Adrenal Incidentalomas: A Rare Presentation of Lung Cancer

    Directory of Open Access Journals (Sweden)

    Halit Diri

    2015-01-01

    Full Text Available Adrenal incidentalomas are found incidentally during a radiologic examination performed for indications other than an adrenal disease, and 15% of them are bilateral adrenal masses. This study describes a 51-year-old male smoker patient admitted with diabetes mellitus. An abdominal ultrasonography performed due to his anemia revealed bilateral adrenal masses. His chest X-ray showed abnormal 10 cm opacity at the right upper lung, and brain, thorax, and abdomen CT scans showed multiple lesions compatible with lung cancer metastases. The pathological examination of the transthoracic lung biopsy specimen was consistent with lung adenocarcinoma. Findings in this patient indicate that, in middle aged patients with bilateral adrenal mass and a history or finding of any malignancy, the first diagnosis which should be considered is adrenal metastasis, and confirming the diagnosis by adrenal biopsy may be useless. Furthermore, screening all smoking patients by chest X-ray or thoracic CT for lung cancer may not be accepted as a routine procedure, but in smokers admitted to a hospital due to signs and symptoms attributed to a pulmonary disease, at least a chest X-ray should be requested.

  20. [Isolated bilateral adrenal metastasis from renal cancer. Case report].

    Science.gov (United States)

    Rabii, R; Joual, A; Naciri, K; Guessous, H; el Mrini, M; Benjelloun, S

    1999-01-01

    The authors report an uncommon case of bilateral synchronous adrenal gland metastases from left renal cell carcinoma. The diagnosis was established by abdominal ultrasound and computed tomography. The surgical approach initially consisted of left radical nephrectomy and ipsilateral adrenalectomy. Histologically, the tumor of the left adrenal gland was identical to the left renal cell carcinoma. Subsequent contralateral adrenalectomy showed an adrenal metastasis identical to the left renal cell carcinoma. Patient follow-up was good with no recurrence of the disease after one year. This is an uncommon case for renal cancer. The treatment and prognosis are discussed.

  1. [Phenotype B primitive adrenal lymphoma, diagnosed by percutaneous aspiration biopsy].

    Science.gov (United States)

    Dahami, Z; Debbagh, A; Dakir, M; Hafiani, M; Joual, A; Bennani, S; el Mrini, M; Benjelloun, S

    2001-01-01

    The authors report a case of primary adrenal lymphoma in a 30-year old-female who complained of lumbar pain and was in poor general condition. Ultrasonography and CT scan revealed a heterogeneous mass with necrosis in the left adrenal gland. Non-Hodgkin's lymphoma of B-cell origin was determined by ultrasound-guided aspiration biopsy of the adrenal mass. Taking this case and the findings in the literature into consideration, the features of this disease have been reviewed and the problem of diagnosis, treatment and prognosis have been examined.

  2. Expression and localization of pChAT as a novel method to study cholinergic innervation of rat adrenal gland.

    Science.gov (United States)

    Elnasharty, Mohamed A; Sayed-Ahmed, Ahmed

    2014-10-01

    Cholinergic innervation of the rat adrenal gland has been analyzed previously using cholinergic markers including acetylcholinesterase (AChE), choline acetyltransferase (ChAT) and vesicular acetylcholine transporter (VAChT). In the present study, we demonstrate putative cholinergic neurons in the rat adrenal gland using an antibody to pChAT, which is the product of a splice variant of ChAT mRNA that is preferentially localized in peripheral cholinergic nerves. Most of the ganglionic neurons as well as small single sporadic neurons in the adrenal gland were stained intensely for pChAT. The density of pChAT-immunoreactive (IR) fibers was distinct in the adrenal cortex and medulla. AChE-, cChAT- and VAChT-immunoreactivities were also observed in some cells and fibers of the adrenal medulla, while the cortex had few positive nerve fibers. These results indicate that ganglionic neurons of the adrenal medulla and nerve fibers heterogeneously express cholinergic markers, especially pChAT. Furthermore, the innervation of the adrenal gland, cortex and medulla, by some cholinergic fibers provides additional morphological evidence for a significant role of cholinergic mechanisms in adrenal gland functions.

  3. Adrenal Insufficiency and Addison's Disease

    Science.gov (United States)

    ... in Endocrinology, Diabetes and Obesity. 2010;(3):217–223. Clinical Trials The National Institute of Diabetes and ... la salud en español Health Statistics Healthy Moments Radio Broadcast Clinical Trials For Health Care Professionals Community ...

  4. Genetic disorders involving adrenal development.

    Science.gov (United States)

    Lin, Lin; Ferraz-de-Souza, Bruno; Achermann, John C

    2007-01-01

    The past decade has seen significant advances in our understanding of the genetic aetiology of several forms of adrenal failure that present in infancy or childhood. Several of these disorders affect adrenal development and are termed 'adrenal hypoplasia'. These conditions can be broadly divided into: (1) secondary forms of adrenal hypoplasia due to panhypopituitarism (e.g. HESX1, LHX4, SOX3) or abnormalities in ACTH synthesis (TPIT) or processing (e.g. POMC or PC1); (2) adrenal hypoplasia as part of an ACTH resistance syndrome [MC2R/ACTH receptor, MRAP, AAAS (triple A syndrome)], and (3) primary defects in the development of the adrenal gland itself (primary adrenal hypoplasia). Primary adrenal hypoplasia most commonly occurs in an X-linked form due to mutations in the nuclear receptor DAX1 (NR0B1) but can occur in a poorly understood recessive form or as part of the IMAGe (intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia, genitourinary anomalies) syndrome. Defining the molecular basis of these conditions can have significant clinical implications for management, counselling and presymptomatic diagnosis, as well as providing fascinating insight into normal and abnormal mechanisms of adrenal development in humans.

  5. [Hyperprogesteronism due to bilateral adrenal carcinomas in a cat with diabetes mellitus].

    Science.gov (United States)

    Quante, S; Sieber-Ruckstuhl, N; Wilhelm, S; Favrot, C; Dennler, M; Reusch, C

    2009-09-01

    An 8 year old male castrated Russian Blue cat with polyuria, polydipsia, polyphagia, abdominal enlargement, unkempt and easily epilated hair coat and abdominal alopecia is described. As a first step diabetes mellitus was diagnosed. Further work-up by ultrasonography revealed severe bilateral enlargement of the adrenal glands. Hypercortisolism was suspected and therefore ACTH stimulation test and dexamethasone suppression test were performed. In all samples cortisol concentrations were below the detection limit of the assay used. Various precursor hormones were measured and high progesterone concentrations were found. Histologically, the adrenal masses were characterised as bilateral adrenal carcinomas of the adrenal cortex. The case report demonstrates that adrenal gland tumors are also capable to secrete sex hormones instead of cortisol. Clinical signs of hyperprogesteronism are identical to those of hypercortisolism.

  6. Temporal and spatial distribution of mast cells and steroidogenic enzymes in the human fetal adrenal.

    Science.gov (United States)

    Naccache, Alexandre; Louiset, Estelle; Duparc, Céline; Laquerrière, Annie; Patrier, Sophie; Renouf, Sylvie; Gomez-Sanchez, Celso E; Mukai, Kuniaki; Lefebvre, Hervé; Castanet, Mireille

    2016-10-15

    Mast cells are present in the human adult adrenal with a potential role in the regulation of aldosterone secretion in both normal cortex and adrenocortical adenomas. We have investigated the human developing adrenal gland for the presence of mast cells in parallel with steroidogenic enzymes profile and serotonin signaling pathway. RT-QPCR and immunohistochemical studies were performed on adrenals at 16-41 weeks of gestation (WG). Tryptase-immunopositive mast cells were found from 18 WG in the adrenal subcapsular layer, close to 3βHSD- and CYP11B2-immunoreactive cells, firstly detected at 18 and 24 WG, respectively. Tryptophan hydroxylase and serotonin receptor type 4 expression increased at 30 WG before the CYP11B2 expression surge. In addition, HDL and LDL cholesterol receptors were expressed in the subcapsular zone from 24 WG. Altogether, our findings suggest the implication of mast cells and serotonin in the establishment of the mineralocorticoid synthesizing pathway during fetal adrenal development.

  7. COMPARATIVE MORPHOFUNCTIONAL CHARACTERISTIC OF ADRENAL GLANDS IN ARTERIAL HYPERTENSION

    Directory of Open Access Journals (Sweden)

    S. Yu. Alyabyeva

    2015-01-01

    Full Text Available The article is devoted to the study of morphological changes of the adrenal glands in arterial hyperten-sion. Adrenals investigated persons who do not suffer in life and suffering from hypertension during the life of hypertension and died from various causes – is incompatible with the life of a mechanical trauma, cerebral hemorrhage, and acute left ventricular failure. In each case, each of the adrenal glands were assessed: the presence and severity of focal and diffuse mononuclear infiltration, the number of lympho-cytes, monocytes, plasma cells and fibroblasts arranged in the respective zones of the cortex and medulla, the severity of hyperemia and edema of various structural parts, the number of zones cortex and medulla endocrinocytes with pycnotic nuclei in the reticular zone evaluated the distribution of secretory cells with lipofuscin and the degree of saturation of the cytoplasm of the secretory cells, in secretory cells of medul-la evaluated nuclear-cytoplasmic ratio, the severity of vacuolization and basophils cytoplasm revealed a number of regularities of morphological changes depending from the various clinical manifestations of hypertension, defining the ultimate option of dying. At the heart tanatogenesis version are more pro-nounced morphological features hyperfunctions glomerular zone – namely delipidization left and right glands. The beam and netted areas contralateral glands embodiment of dying of a heart compared to the brain, is more pronounced hyperemia. When cardiac variant tanatogenesis more pronounced focal lym-phoid infiltration and vacuolization in secretory cells of left adrenal medulla.

  8. Adrenal incidentaloma in neurofibromatosis type 1

    Directory of Open Access Journals (Sweden)

    Tančić-Gajić Milina

    2008-01-01

    Full Text Available INTRODUCTION Neurofibromatosis type 1 is one of the most common genetically transmitted diseases with a high index of spontaneous mutations and extremely varied and unpredictable clinical manifestations. It is diagnosed by the existence of certain clinical criteria. The presence of numerous localised cutaneous neurofibromas or a plexiform neurofibroma is virtually pathognomonic of neurofibromatosis type 1. The incidence of pheochromocytoma in neurofibromatosis type 1 is 0.1-5.7%. CASE OUTLINE A 56-year old female patient was admitted for further evaluation of incidental adrenal tumour previously diagnosed on computerized tomography (CT. She had previously unrecognized neurofibromatosis type 1 and a clinical picture which could remind of pheochromocytoma. None of the catecholamine samples in 24 hr urine indicated functionally active pheochromocytoma. Chromogranin A was moderately increased. Decision for operation was made after performing the image techniques. Adrenal incidentaloma had features of pheochromocytoma on abdominal magnetic resonance imaging (MRI, with positive 131I-MIBG (iodine 131-labelled metaiodobenzylguanidine scintigraphy. After being treated with phenoxybenzamine and propranolol, she was operated on. The pathohistological finding showed the case of left adrenal pheochromocytoma. CONCLUSION Detailed diagnostic procedure for pheochromocytoma should be performed with patients having neurofibromatosis type 1 and adrenal incidentaloma. Pheochromocytomas are rare tumours with fatal outcome if not duly recognized and cured.

  9. Adrenal Insufficiency - Aetiology, Diagnosis and Treatment

    Directory of Open Access Journals (Sweden)

    Nazma Akter

    2015-03-01

    Full Text Available Adrenal insufficiency is caused by either primary adrenal failure or by hypothalamic-pituitary impairment of the corticotropic axis. Adrenal insufficiency, first codified in 1855 by Thomas Addison, remains relevant in 2014 because of its lethal nature. Though, it is a rare disease but is life threatening when overlooked. Main presenting symptoms such as fatigue, anorexia and weight loss are nonspecific, thus diagnosis is often delayed. The diagnostic work-up is well established but some pitfalls remain. The diagnosis is adequately established by the 250 μg ACTH (adrenocorticotropic hormone stimulation test in most cases. Glucocorticoids provide life saving treatment but long-term quality of life is impaired, perhaps because therapy is not given in a physiologic way. Dehydroepiandrosterone-replacement therapy has been introduced that could help to restore quality of life. It may be useful in pubertal girls, but not in adults. Monitoring of glucocorticoid-replacement is difficult due to lack of objective methods of assessment and is therefore largely based on clinical grounds. Thus, long-term management of patients with adrenal insufficiency remains a challenge, requiring an experienced specialist.

  10. Adrenal insufficiency in patients with decompensatedcirrhosis

    Institute of Scientific and Technical Information of China (English)

    2015-01-01

    Adrenal reserve depletion and overstimulation of thehypothalamus-pituitary-adrenal (HPA) axis are causesfor adrenal insufficiency (AI) in critically ill individuals.Cirrhosis is a predisposing condition for AI in cirrhotics aswell. Both stable cirrhotics and liver transplant patients(early and later after transplantation) have been reportedto present AI. The mechanisms leading to reducedcortisol production in cirrhotics are the combination oflow cholesterol levels (the primary source of cortisol), theincreased cytokines production that overstimulate andexhaust HPA axis and the destruction of adrenal glandsdue to coagulopathy. AI has been recorded in 10%-82%cirrhotics depending on the test used to evaluate adrenalfunction and in 9%-83% stable cirrhotics. The similarityof those proportions support the assumption that AI isan endogenous characteristic of liver disease. However,the lack of a gold standard method for AI assessmentand the limitation of precise thresholds in cirrhoticsmake difficult the recording of the real prevalence of AI.This review aims to summarize the present data overAI in stable, critically ill cirrhotics and liver transplantrecipients. Moreover, it provides information about thecurrent knowledge in the used diagnostic tools and thepossible effectiveness of corticosteroids administration incritically ill cirrhotics with AI.

  11. Adrenal crisis in a patient with acute myeloid leukaemia

    OpenAIRE

    Li, Wang; Okwuwa, Ikemefuna; Toledo-Frazzini, Karla; Alhomosh, Alaaedin

    2013-01-01

    Adrenal crisis is a group of clinical manifestation predominantly with hypotensive shock, electrolyte imbalance in a patient with adrenal insufficiency or in a patient who was abruptly withdrawn from glucocorticoid treatment acute myeloid leukaemia (AML) is one of the most common acute leukaemia in adults. Though the above diseases are commonly seen in individual patients, the coexistence of both conditions in the same patient is rare. We reported a 64-year-old African-American man with a his...

  12. Diffusion-Weighted MRI in Creutzfeldt-Jakob Disease: Focus on the Cerebral Cortex and Chronologic Change

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jeong Eun; Song, Chang Joon; Lee, In Ho [Chungnam National University, Daejeon (Korea, Republic of); Yu, In Kyu [Eulji University Hospital, Seoul (Korea, Republic of); Choi, See Sung [Wonkwang University Hospital, Iksan (Korea, Republic of)

    2010-08-15

    To evaluate high cortical signal intensity and chronologic changes for diffusion-weighted MR imaging (DWI) in sporadic Creutzfeldt-Jakob disease. We retrospectively analyzed the DWI results of 16 patients with probable CJD (according to WHO criteria) and evaluated the distribution, extent and bilaterality of the lesions in the cortex, basal ganglia and thalamus. We also reviewed the chronologic changes of the lesions by evaluating the followup MR examination results in 8 of 16 patients. Cortical abnormalities were present in 15 (94%) of 16 patients. Isolated cortical involvement was present in 6 patients (40%), while the combined involvement of the cortex and basal ganglia was present in 9 patients (60%). The distribution of the lesions was bilateral in 12 patients and predominantly on the right side in 8 patients. Upon follow-up MR imaging, the cortical lesions showed progress in terms of extent and signal intensity. Basal ganglia abnormalities were present in 9 of 15 patients. Moreover, 4 of 6 patients who had no abnormal signal intensity in the basal ganglia on the initial MR imaging results, showed abnormally high signal intensity upon follow-up MR imaging. The characteristically high cortical signal intensities on DWI in an elderly patient with rapidly progressive dementia should point to the diagnosis of early phase CJD and might be useful for the differential diagnosis.

  13. What is normal in normal aging? Effects of aging, amyloid and Alzheimer's disease on the cerebral cortex and the hippocampus.

    Science.gov (United States)

    Fjell, Anders M; McEvoy, Linda; Holland, Dominic; Dale, Anders M; Walhovd, Kristine B

    2014-06-01

    What can be expected in normal aging, and where does normal aging stop and pathological neurodegeneration begin? With the slow progression of age-related dementias such as Alzheimer's disease (AD), it is difficult to distinguish age-related changes from effects of undetected disease. We review recent research on changes of the cerebral cortex and the hippocampus in aging and the borders between normal aging and AD. We argue that prominent cortical reductions are evident in fronto-temporal regions in elderly even with low probability of AD, including regions overlapping the default mode network. Importantly, these regions show high levels of amyloid deposition in AD, and are both structurally and functionally vulnerable early in the disease. This normalcy-pathology homology is critical to understand, since aging itself is the major risk factor for sporadic AD. Thus, rather than necessarily reflecting early signs of disease, these changes may be part of normal aging, and may inform on why the aging brain is so much more susceptible to AD than is the younger brain. We suggest that regions characterized by a high degree of life-long plasticity are vulnerable to detrimental effects of normal aging, and that this age-vulnerability renders them more susceptible to additional, pathological AD-related changes. We conclude that it will be difficult to understand AD without understanding why it preferably affects older brains, and that we need a model that accounts for age-related changes in AD-vulnerable regions independently of AD-pathology.

  14. Testicular adrenal rest tumours in congenital adrenal hyperplasia

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Hermus, A.R.M.M.; Otten, B.J.

    2009-01-01

    In adult patients with congenital adrenal hyperplasia (CAH), the presence of testicular adrenal rest tumours (TART) is an important complication leading to gonadal dysfunction and infertility. These tumours can be already found in childhood and puberty. In this paper, we review the embryological, hi

  15. Transcriptional network analysis in frontal cortex in Lewy body diseases with focus on dementia with Lewy bodies.

    Science.gov (United States)

    Santpere, Gabriel; Garcia-Esparcia, Paula; Andres-Benito, Pol; Lorente-Galdos, Belen; Navarro, Arcadi; Ferrer, Isidro

    2017-03-21

    The present study investigates global transcriptional changes in frontal cortex area 8 in incidental Lewy Body disease (iLBD), Parkinson disease (PD) and Dementia with Lewy bodies (DLB). We identified different co-expressed gene sets associated with disease stages, and gene ontology categories enriched in gene modules and differentially expressed genes including modules or gene clusters correlated to iLBD comprising upregulated dynein genes and taste receptors, and down-regulated innate inflammation. Focusing on DLB, we found modules with genes significantly enriched in functions related to RNA and DNA production, mitochondria and energy metabolism, purine metabolism, chaperone and protein folding system, and synapses and neurotransmission (particularly the GABAergic system). The expression of more than fifty selected genes was assessed with RT-qPCR. Our findings provide, for the first time, evidence of molecular cortical alterations in iLBD and involvement of several key metabolic pathways and gene hubs in DLB which may underlie cognitive impairment and dementia. This article is protected by copyright. All rights reserved.

  16. Double-hit primary unilateral adrenal lymphoma with good outcome

    Directory of Open Access Journals (Sweden)

    Marković Olivera

    2014-01-01

    Full Text Available Introduction. Primary adrenal non-Hodgkin’s lymphoma (NHL is a rare neoplasm with poor prognosis. On the other side, double-hit lymphomas with BCL2 and MYC translocation are characterized by advanced disease stage, extranodal and central nervous system involvements at presentation or disease progression. Case report. We reported a 73-year-old male patient with double-hit primary adrenal lymphoma and preserved adrenal function, showing a favorable clinical course. Computed tomography of abdomen showed a 9 7 cm mass of the left adrenal gland. Laparatomy with left adrenalectomy was done and histological examination revealed diagnosis of a diffuse large B-cell NHL (DLBCL, non-GCB subtype. The patient was treated with 6 cycles of R-CHOP chemotherapy with reduced doses of doxorubicin because of the decreased left verticle ejection fraction. The patient was followed up regularly for 20 months with no evidence of tumor recurrence despite the inherently poor prognostic profile and double-hit phenotype of the disease. Conclusion. R-CHOP chemotherapy in combination with adrenalectomy can be an effective first-line regimen for primary adrenal DLBCL, despite the inherently poor prognostic profile (non-GCB subtype, bulky disease, elevated lactate dehydrogenase and double-hit phenotype of the disease.

  17. 脓毒症早期大鼠肾上腺皮质功能变化及地塞米松的干预作用%Changes in adrenal cortex function and protective effect of dexamethasone in septic rats during early phases

    Institute of Scientific and Technical Information of China (English)

    胡振杰; 田惠玉; 王澜涛; 田慧艳

    2009-01-01

    phases of sepsis and its probable mechanisms,and to study the optimal time for glucocorticoid replacement therapy.Methods A total of 260 healthy male Wistar rats were randomized into five groups(each group n=52),including normal control group,sham operation group,cecal ligation and puncture(CLP)group,dexamethasone prevention group(with 10 mg/kg dexamethasone injection into the abdominal cavity before CLP) and dexamethasone treatment group(with 10 mg/kg dexamethasone injection into the abdominal cavity 7 hours after CLP).Each group was subdivided into five subgroups according to five time points:2,4,6,8 and 12 hours. Adrenocorticotrophic hormone(ACTH)test was conducted at 8 hours and 12 hours,and before and after 30 minutes of ACTH administration。the cortisol content in serum was determined with radioimmunoassay(RIA)and the expressions of Toll-like receptor 4(TLR4),tumor necrosis factor-α (TNF-α) mRNA in adrenal glands were detected with semiquantitative reverse transcription-polymerase chain reaction(RT-PCR).UItrastructure of adrenal cortex was observed with transmission electron microscope.The survival rats was recorded in all groups.Results ① The levels of cortisol in CLP group,dexamethasone prevention group and dexamethasone treatment group were respectively higher than those of normal control and sham operation groups(all P<0.05).But there was no marked change in the levels of eortisol between pre-and post-ACTH in rats with sepsis in the early phases. ② The expressions of TLR4,TNF-α mRNA in adrenal both were significantly increased in CLP group,and they were higher than those in sham operation group(P<0.05 or P<0.01).The expressions of TLR4,TNF-α mRNA in dexamethasone prevention group and treatment group were significantly lower than those in CLP group,and those in the dexamethasone prevention group were lower than those in sham operation group. ③ In the groups of CLP,dexamethasone prevention and treatment,ultrastructure changes were observed in the

  18. [Adrenal failure caused by primary adrenal non-Hodgkin lymphoma: a case report and review of the literature].

    Science.gov (United States)

    Hernández Marín, B; Díaz Muñoz de la Espada, V M; Alvarez Alvarez, R; Encinas García, S; Khosravi Shahi, P; Pérez Fernández, R; Pérez Manga, G

    2008-03-01

    We report a case of 78-year old man who presented with symptoms of adrenal insufficiency. The computed tomography (CT) scan showed the presence of bilateral adrenal masses. A CT-scan guided needle biopsy revealed diffuse large- B cell lymphoma. The absence of pathological findings in clinical, bone marrow and CT scan examinations supported the diagnosis of primary non-Hodgkin Lymphoma of the adrenal glands. The patient was treated with four cycles of R-CHOP chemotherapy with Rituximab, liposomal Doxorubicin, Cyclophosphamide, Vincristine and Prednisolone. At the end of fourth cycle there was radiological improvement but the chemotherapy was stopped because of IV grade toxicity. He completed treatment with radiotherapy of right adrenal mass. Few days after finishing radiation therapy the patient died due to a disseminated infection. No progressive disease was founded.

  19. Primary bilateral adrenal non-Hodgkin's lymphoma associated with normal adrenal function.

    Science.gov (United States)

    Gu, Bin; Ding, Qiang; Xia, Guowei; Fang, Zujun; Fang, Jie; Jiang, Haowen; Yao, Mengshu

    2009-04-01

    Primary bilateral adrenal non-Hodgkin's lymphoma is rare. Adrenal insufficiency or adrenal failure as a result of tumor destruction is the main pathophysiological change of most cases. Normal adrenal function despite bulky bilateral adrenal masses is extremely rare. We present a case of primary bilateral adrenal non-Hodgkin's lymphoma associated with normal adrenal function. Positron emission tomography-computed tomography is helpful to the diagnosis.

  20. Estrogen deficiency leads to telomerase inhibition,telomere shortening and reduced cell proliferation in the adrenal gland of mice

    Institute of Scientific and Technical Information of China (English)

    Sharyn Bayne; Margaret EE Jones; He Li; Alex R Pinto; Evan R Simpson; Jun-Ping Liu

    2008-01-01

    Estrogen deficiency mediates aging, but the underlying mechanism remains to be fully determined. We report here that estrogen deficiency caused by targeted disruption of aromatase in mice results in significant inhibition oftelomerase activity in the adrenal gland in vivo. Gene expression analysis showed that, in the absence of estrogen, telomerase reverse transcriptase (TERT) gene expression is reduced in association with compromised cell proliferation in the adrenal gland cortex and adrenal atrophy. Stem cells positive in c-kit are identified to populate in the parenchyma of adrenal cortex. Analysis of telomeres revealed that estrogen deficiency results in significantly shorter telomeres in the adrenal cortex than that in wild-type (WT) control mice. To further establish the causal effects of estrogen, we conducted an estrogen replacement therapy in these estrogen-deficient animals. Administration of estrogen for 3 weeks restores TERT gene expression, telomerase activity and cell proliferation in estrogen-deficient mice. Thus, our data show for the first time that estrogen deficiency causes inhibitions of TERT gene expression, telomerase activity, telomere maintenance, and cell proliferation in the adrenal gland of mice in vivo, suggesting that telomerase inhibition and telomere shortening may mediate cell proliferation arrest in the adrenal gland, thus contributing to estrogen deficiency-induced aging under physiological conditions.

  1. ADRENAL INCIDENTALOMAS: ANALYSIS OF 126 CASES

    Institute of Scientific and Technical Information of China (English)

    李汉忠; 严维刚; 曾正陪; 肖河; 冯超; 王惠君

    2003-01-01

    Purpose. To evaluate the diagnosis and treatment of adrenal incidentalomas. Methods. One hundred and twenty-six patients with incidentalomas were analyzed, among them 98 underwent operation. Results. Eighty-eight of the adrenal incidentalomas were discovered by ultrasound. Of all the types of adrenal incidentalomas, 52 (41.3%) of them were adenomas; 43 (34.1%) were hypersecretory adrenal tumors, including 29 pheochromocytomas, 9 primary aldosteronisms, 1 adrenogenitol syndrome combined with adrenal adenoma, 2 Cushing's syndrome combined with adenomas and 2 Cushing's syndrome combined with nodular hyperplasias. All nonhypersecretory adrenal adenomas were under 6 cm, and all adrenal carcinomas were above 6 cm.Conclusions. To search for hypersecretory adrenal tumors and to detect malignant adrenal tumors are quite essential in the process of diagnosing adrenal incidentalomas. For nonhypersecretory adrenal adenomas, the size of tumor is the most important index in determining whether the tumor is benign or malignant and whether the tumor needs to be treated with operation.

  2. Clinical value of isotope methods in adrenal morphology investigations

    Energy Technology Data Exchange (ETDEWEB)

    Jakubowski, W.; Feltynowski, T.; Graban, W. (Akademia Medyczna, Warsaw (Poland))

    1981-01-01

    In the years 1976-1980 in 105 patients 124 adrenal scintigrams were obtained, including 50 in patients with primary hyperaldosteronism syndrome, 27 with Cushings syndrome, 8 after bilateral total adrenalectomy, 4 with adrenal virilizing tumours, 6 with phaeochromocytoma, 10 with normal adrenal function. The results of scintigraphy were compared with those of arteriography, phlebography, computer tomography and postoperative findings. The clinical value of scintigraphy was found to be greatest in cases of adrenocortical disease. In cases of phaeochromocytoma isotope angioscintigraphy is a useful method for localizing the tumour since it makes possible demonstration of the vascular bed of the tumour. A close correlation was demonstrated between the results of scintigraphy and those of computer tomography. In the group of 27 patients treated surgically for adrenocortical disease in 87% of cases an agreement was found between the results of scintigraphy and the result of the operation. For different adrenocortical diseases this per cent was: 77% for Cushings syndrome, 91% for primary hyperaldosteronism, 100% for adrenal virilizing tumours. The study showed that adrenal scintigraphy is a very valuable diagnostic method of high clinical usefulness in adrenocortical diseases.

  3. Differential DNA Methylation of MicroRNA Genes in Temporal Cortex from Alzheimer’s Disease Individuals

    Directory of Open Access Journals (Sweden)

    Darine Villela

    2016-01-01

    Full Text Available This study investigated for the first time the genomewide DNA methylation changes of noncoding RNA genes in the temporal cortex samples from individuals with Alzheimer’s disease (AD. The methylome of 10 AD individuals and 10 age-matched controls were obtained using Illumina 450 K methylation array. A total of 2,095 among the 15,258 interrogated noncoding RNA CpG sites presented differential methylation, 161 of which were associated with miRNA genes. In particular, 10 miRNA CpG sites that were found to be hypermethylated in AD compared to control brains represent transcripts that have been previously associated with the disease. This miRNA set is predicted to target 33 coding genes from the neuregulin receptor complex (ErbB signaling pathway, which is required for the neurons myelination process. For 6 of these miRNA genes (MIR9-1, MIR9-3, MIR181C, MIR124-1, MIR146B, and MIR451, the hypermethylation pattern is in agreement with previous results from literature that shows downregulation of miR-9, miR-181c, miR-124, miR-146b, and miR-451 in the AD brain. Our data implicate dysregulation of miRNA methylation as contributor to the pathogenesis of AD.

  4. Brain banks as key part of biochemical and molecular studies on cerebral cortex involvement in Parkinson's disease.

    Science.gov (United States)

    Ravid, Rivka; Ferrer, Isidro

    2012-04-01

    Exciting developments in basic and clinical neuroscience and recent progress in the field of Parkinson's disease (PD) are partly a result of the availability of human specimens obtained through brain banks. These banks have optimized the methodological, managerial and organizational procedures; standard operating procedures; and ethical, legal and social issues, including the code of conduct for 21st Century brain banking and novel protocols. The present minireview focuses on current brain banking organization and management, as well as the likely future direction of the brain banking field. We emphasize the potentials and pitfalls when using high-quality specimens of the human central nervous system for advancing PD research. PD is a generalized disease in which α-synuclein is not a unique component but, instead, is only one of the players accounting for the complex impairment of biochemical/molecular processes involved in metabolic pathways. This is particularly important in the cerebral cortex, where altered cognition has a complex neurochemical substrate. Mitochondria and energy metabolism impairment, abnormal RNA, microRNA, protein synthesis, post-translational protein modifications and alterations in the lipid composition of membranes and lipid rafts are part of these complementary factors. We have to be alert to the possible pitfalls of each specimen and its suitability for a particular study. Not all samples qualify for the study of DNA, RNA, proteins, post-translational modifications, lipids and metabolomes, although the use of carefully selected samples and appropriate methods minimizes pitfalls and errors and guarantees high-quality reserach.

  5. Primary bilateral adrenal B-cell lymphoma associated with EBV and JCV infection

    Directory of Open Access Journals (Sweden)

    Guzzardo Vincenza

    2009-01-01

    Full Text Available Abstract Primary lymphoma of the adrenal gland is a rare and highly aggressive disease, with only a few reports in the literature. The pathogenesis is unknown, but detection of Epstein Barr virus (EBV genome sequences and gene expression in some cases of primary adrenal lymphomas suggested the virus might be a causative agent of the malignancy. While investigating the presence of genome sequences of oncogenic viruses in a large series of adrenal tumors, both EBV and JC polyomavirus (JCV DNA sequences were detected in a diffuse large primary bilateral B-cell non-Hodgkin lymphoma of the adrenal gland, which was diagnosed only at postmortem examination in a 77 year-old woman with incidentally discovered adrenal masses and primary adrenal insufficiency. The presence of both EBV and JCV genome sequences suggests the relevance of EBV and JCV coinfection in the pathogenesis of this rare form of B-cell lymphoma.

  6. Guidelines regarding management of adrenal insufficiency in the Holy month of Ramadan

    Directory of Open Access Journals (Sweden)

    Sheelu S Siddiqi

    2012-01-01

    Full Text Available Adrenal insufficiency is a life-threatening event, so it is recommended for patients with known adrenal insufficiency to be properly educated regarding sick-day management. In the month of Ramadan, people refrain from eating and drinking during daylight hours. It is very important for patients with adrenal insufficiency, who wish to keep a fast, to be well aware of the disease, the suitable drug to be used for that particular period, warning signs, sick-day management, physical activity, and dietary limits. This article describes guidelines for the sick-day management of patients with adrenal insufficiency, in the month of Ramadan.

  7. Acupuncture on GB34 activates the precentral gyrus and prefrontal cortex in Parkinson’s disease

    NARCIS (Netherlands)

    Yeo, Sujung; Choe, I.H.; Noort, M.W.M.L. van den; Bosch, M.P.C.; Jahng, G.H.; Rosen, B.; Kim, S.H.; Lim, S.

    2014-01-01

    Background Acupuncture is increasingly used as an additional treatment for patients with Parkinson’s disease (PD). Methods In this functional magnetic resonance imaging study, brain activation in response to acupuncture in a group of 12 patients with PD was compared with a group of 12 healthy partic

  8. Oxidant/antioxidant effects of chronic exposure to predator odor in prefrontal cortex, amygdala, and hypothalamus.

    Science.gov (United States)

    Mejia-Carmona, G E; Gosselink, K L; Pérez-Ishiwara, G; Martínez-Martínez, A

    2015-08-01

    The incidence of anxiety-related diseases is increasing these days, hence there is a need to understand the mechanisms that underlie its nature and consequences. It is known that limbic structures, mainly the prefrontal cortex and amygdala, are involved in the processing of anxiety, and that projections from prefrontal cortex and amygdala can induce activity of the hypothalamic-pituitary-adrenal axis with consequent cardiovascular changes, increase in oxygen consumption, and ROS production. The compensatory reaction can include increased antioxidant enzymes activities, overexpression of antioxidant enzymes, and genetic shifts that could include the activation of antioxidant genes. The main objective of this study was to evaluate the oxidant/antioxidant effect that chronic anxiogenic stress exposure can have in prefrontal cortex, amygdala, and hypothalamus by exposition to predator odor. Results showed (a) sensitization of the HPA axis response, (b) an enzymatic phase 1 and 2 antioxidant response to oxidative stress in amygdala, (c) an antioxidant stability without elevation of oxidative markers in prefrontal cortex, (d) an elevation in phase 1 antioxidant response in hypothalamus. Chronic exposure to predator odor has an impact in the metabolic REDOX state in amygdala, prefrontal cortex, and hypothalamus, with oxidative stress being prevalent in amygdala as this is the principal structure responsible for the management of anxiety.

  9. Proteomic profiling of brain cortex tissues in a Tau transgenic mouse model of Alzheimer's disease

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Seong-Hun; Jung, In-Soo; Han, Gi-Yeon; Kim, Nam-Hee; Kim, Hyun-Jung [School of Life Sciences and Biotechnology, Korea University, Seoul 136-701 (Korea, Republic of); Kim, Chan-Wha, E-mail: cwkim@korea.ac.kr [School of Life Sciences and Biotechnology, Korea University, Seoul 136-701 (Korea, Republic of)

    2013-01-11

    Highlights: Black-Right-Pointing-Pointer A transgenic mouse model expressing NSE-htau23 was used. Black-Right-Pointing-Pointer 2D-gel electrophoresis to analyze the cortex proteins of transgenic mice was used. Black-Right-Pointing-Pointer Differentially expressed spots in different stages of AD were identified. Black-Right-Pointing-Pointer GSTP1 and CAII were downregulated with the progression of AD. Black-Right-Pointing-Pointer SCRN1 and ATP6VE1 were up regulated and down regulated differentially. -- Abstract: Alzheimer's disease (AD) involves regionalized neuronal death, synaptic loss, and an accumulation of intracellular neurofibrillary tangles and extracellular senile plaques. Although there have been numerous studies on tau proteins and AD in various stages of neurodegenerative disease pathology, the relationship between tau and AD is not yet fully understood. A transgenic mouse model expressing neuron-specific enolase (NSE)-controlled human wild-type tau (NSE-htau23), which displays some of the typical Alzheimer-associated pathological features, was used to analyze the brain proteome associated with tau tangle deposition. Two-dimensional electrophoresis was performed to compare the cortex proteins of transgenic mice (6- and 12-month-old) with those of control mice. Differentially expressed spots in different stages of AD were identified with ESI-Q-TOF (electrospray ionization quadruple time-of-flight) mass spectrometry and liquid chromatography/tandem mass spectrometry. Among the identified proteins, glutathione S-transferase P 1 (GSTP1) and carbonic anhydrase II (CAII) were down-regulated with the progression of AD, and secerin-1 (SCRN1) and V-type proton ATPase subunit E 1 (ATP6VE1) were up-regulated only in the early stages, and down-regulated in the later stages of AD. The proteins, which were further confirmed by RT-PCR at the mRNA level and with western blotting at the protein level, are expected to be good candidates as drug targets for AD. The

  10. A Case of Congenital Adrenal Hyperplasia Mimicking Cushing's Syndrome

    Science.gov (United States)

    Kim, Hye Jeong; Kang, Mira; Kim, Jae Hyeon; Kim, Sun Wook; Chung, Jae Hoon; Min, Yong-Ki; Lee, Moon-Kyu; Kim, Kwang-Won

    2012-01-01

    Congenital adrenal hyperplasia (CAH) is characterized by decreased adrenal hormone production due to enzymatic defects and subsequent rise of adrenocorticotrophic hormone that stimulates the adrenal cortex to become hyperplastic, and sometimes tumorous. As the pathophysiology is basically a defect in the biosynthesis of cortisol, one may not consider CAH in patients with hypercortisolism. We report a case of a 41-yr-old man with a 4 cm-sized left adrenal tumorous lesion mimicking Cushing's syndrome who was diagnosed with CAH. He had central obesity and acanthosis nigricans involving the axillae together with elevated 24-hr urine cortisol level, supporting the diagnosis of Cushing's syndrome. However, the 24-hr urine cortisol was suppressed by 95% with the low dose dexamethasone suppression test. CAH was suspected based on the history of precocious puberty, short stature and a profound suppression of cortisol production by dexamethasone. CAH was confirmed by a remarkably increased level of serum 17-hydroxyprogesterone level. Gene mutation analysis revealed a compound heterozygote mutation of CYP21A2 (I173N and R357W). PMID:23166432

  11. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors.

    Science.gov (United States)

    Fassnacht, Martin; Arlt, Wiebke; Bancos, Irina; Dralle, Henning; Newell-Price, John; Sahdev, Anju; Tabarin, Antoine; Terzolo, Massimo; Tsagarakis, Stylianos; Dekkers, Olaf M

    2016-08-01

    : By definition, an adrenal incidentaloma is an asymptomatic adrenal mass detected on imaging not performed for suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas, but may also represent conditions requiring therapeutic intervention (e.g. adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis). The purpose of this guideline is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with adrenal incidentalomas based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. We predefined four main clinical questions crucial for the management of adrenal incidentaloma patients, addressing these four with systematic literature searches: (A) How to assess risk of malignancy?; (B) How to define and manage low-level autonomous cortisol secretion, formerly called 'subclinical' Cushing's syndrome?; (C) Who should have surgical treatment and how should it be performed?; (D) What follow-up is indicated if the adrenal incidentaloma is not surgically removed? SELECTED RECOMMENDATIONS: (i) At the time of initial detection of an adrenal mass establishing whether the mass is benign or malignant is an important aim to avoid cumbersome and expensive follow-up imaging in those with benign disease. (ii) To exclude cortisol excess, a 1mg overnight dexamethasone suppression test should be performed (applying a cut-off value of serum cortisol ≤50nmol/L (1.8µg/dL)). (iii) For patients without clinical signs of overt Cushing's syndrome but serum cortisol levels post 1mg dexamethasone >138nmol/L (>5µg/dL), we propose the term 'autonomous cortisol secretion'. (iv) All patients with '(possible) autonomous cortisol' secretion should be screened for hypertension and type 2 diabetes mellitus, to ensure these are appropriately treated. (v) Surgical treatment should be considered in an individualized approach in patients with

  12. Acupuncture on GB34 activates the precentral gyrus and prefrontal cortex in Parkinson’s disease

    OpenAIRE

    Yeo, Sujung; Choe, I.H.; Noort, M.W.M.L. van den; Bosch, M.P.C.; Jahng, G H; Rosen, B.; Kim, S. H.; S. Lim

    2014-01-01

    Background: Acupuncture is increasingly used as an additional treatment for patients with Parkinson’s disease (PD). Methods: In this functional magnetic resonance imaging study, brain activation in response to acupuncture in a group of 12 patients with PD was compared with a group of 12 healthy participants. Acupuncture was conducted on a specific acupoint, the right GB 34 (Yanglingquan), which is a frequently used acupoint for motor function treatment in the oriental medical field. Results: ...

  13. Endoscopic Retroperitoneal Adrenalectomy for Adrenal Metastases

    Directory of Open Access Journals (Sweden)

    Gintaras Simutis

    2014-01-01

    Full Text Available Objectives. To evaluate whether retroperitoneal approach for adrenalectomy is a safe and effective treatment for adrenal metastases (AM. Methods. From June 2004 to January 2014, nine consecutive patients with AM were treated with endoscopic retroperitoneal adrenalectomy (ERA. A retrospective study was conducted, and clinical data, tumor characteristics, and oncologic outcomes were acquired and analyzed. Results. Renal cancer was the primary site of malignancy in 44.4% of cases. The mean operative time was 132 ± 10.4 min. There were 5 synchronous and 4 metachronous AM. One patient required conversion to transperitoneal laparoscopic procedure. No mortality or perioperative complications were observed. The median overall survival was 11 months (range: 2–42 months. Survival rates of 50% and 25% were identified at 1 and 3 years, respectively. At the end of the study, 4 patients were alive with a mean observed follow-up of 20 months. No patients presented with local tumor relapse or port-site metastases. Conclusions. This study shows that ERA is a safe and effective procedure for resection of AM and advances the surgical treatment of adrenal disease. The use of the retroperitoneal approach for adrenal tumors less than 6 cm can provide very favorable surgical outcomes.

  14. Radioimmunoassay of renin-angiotensin-aldosterone in patients with adrenal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Slavnov, V.N.; Yakovlev, A.A.; Yugrinov, O.G.; Gandzha, T.I. (Kievskij Nauchno-Issledovatel' skij Inst. Ehndokrinologii i Obmena Veshchestv (Ukrainian SSR))

    1983-02-01

    The results are presented of a study of the renin-angiotensin-aldosterone system in 89 patients with aldosteronoma, corticosteroma, pheochromocytoma and hypertension. Radioimmunoassay was used to measure aldosterone concentration and renin activity in the peripheral blood and blood from vena cava inferior, the renal and adrenal veins, the circadian cycle of their content and the responsiveness of the glomerular zone of the adrenal cortex and the juxtaglomerular renal system under the influence of lasix intake and the change over from a horizontal into vertical position. Patients with adrenal tumors have shown disorders of renin-angiotensin-aldosterone function. Radioimmunoassay of the renin-angiotensin-aldosterone system promotes early detection of adrenal tumors in the general population of patients with hypertension and can be used for control over therapeutic efficacy.

  15. Comparative effect of ACTH and related peptides on proliferation and growth of rat adrenal gland

    Directory of Open Access Journals (Sweden)

    Claudimara Ferini Pacicco Lotfi

    2016-05-01

    Full Text Available Pro-opiomelanocortin (POMC is a polypeptide precursor known to yield biologically active peptides related to a range of functions. These active peptides include the adrenocorticotropic hormone (ACTH, which is essential for maintenance of adrenal growth and steroidogenesis, and the alpha-melanocyte stimulation hormone, which plays a key role in energy homeostasis. However, the role of the highly conserved N-terminal region of POMC peptide fragments has begun to be unraveled only recently. Here we review the cascade of events involved in regulation of proliferation and growth of murine adrenal cortex triggered by ACTH and other POMC-derived peptides. Key findings regarding signaling pathways and modulation of genes and proteins required for the regulation of adrenal growth are summarized. We have outlined the known mechanisms as well as future challenges for research on the regulation of adrenal proliferation and growth triggered by these peptides.

  16. Ectopic Thyroid in the Adrenal Presenting as an Adrenal Incidentaloma

    Directory of Open Access Journals (Sweden)

    Banu Aktaş Yılmaz

    2016-12-01

    Full Text Available Adrenal incidentalomas are clinical dilemmas for the clinicians. The work up, to differentiate between malignant and benign lesions, and hyperfunctioning and nonfunctioning lesions is mandatory before the consideration of surgical resection. Ectopic thyroid tissue located in the adrenal gland (ETTAG is a very rare condition. We report a case of ETTAG presenting with adrenal incidentaloma. A 57-year-old woman was admitted with incidental right adrenal mass. Hormone evaluation showed no hormonal activity. Magnetic resonance imaging revealed a 20x17 mm lobulated solid mass, which contained millimetric hypointense nodular areas consistent with calcifications. Loss of signal intensity on out-of-phase could not be evaluated because of the calcifications. Right adrenalectomy was performed to establish the histopathological diagnosis and to rule out malignancy. Histopathological diagnosis revealed ETTAG. Her medical history was positive for multinodular goiter and bilateral subtotal thyroidectomy 32 years ago. Thyroid ultrasonography showed residual thyroid tissue in both the right and left lobes, and colloid thyroid nodules. Fine needle aspiration biopsy from the nodules revealed benign nodules. The patient has been followed up for six years, and no change in thyroid nodule sizes and no evidence of metastatic foci have been detected. ETTAG would be considered in the differential diagnosis of adrenal mass showing no hormonal activity, especially when magnetic resonance images are not consistent with adrenal adenoma. Long follow-up duration of this case suggests that it was a benign condition.

  17. Entorhinal Cortex dysfunction can be rescued by inhibition of microglial RAGE in an Alzheimer’s disease mouse model

    Science.gov (United States)

    Criscuolo, Chiara; Fontebasso, Veronica; Middei, Silvia; Stazi, Martina; Ammassari-Teule, Martine; Yan, Shirley ShiDu; Origlia, Nicola

    2017-01-01

    The Entorhinal cortex (EC) has been implicated in the early stages of Alzheimer’s disease (AD). In particular, spreading of neuronal dysfunction within the EC-Hippocampal network has been suggested. We have investigated the time course of EC dysfunction in the AD mouse model carrying human mutation of amyloid precursor protein (mhAPP) expressing human Aβ. We found that in mhAPP mice plasticity impairment is first observed in EC superficial layer and further affected with time. A selective impairment of LTP was observed in layer II horizontal connections of EC slices from 2 month old mhAPP mice, whereas at later stage of neurodegeneration (6 month) basal synaptic transmission and LTD were also affected. Accordingly, early synaptic deficit in the mhAPP mice were associated with a selective impairment in EC-dependent associative memory tasks. The introduction of the dominant-negative form of RAGE lacking RAGE signalling targeted to microglia (DNMSR) in mhAPP mice prevented synaptic and behavioural deficit, reducing the activation of stress related kinases (p38MAPK and JNK). Our results support the involvement of the EC in the development and progression of the synaptic and behavioural deficit during amyloid-dependent neurodegeneration and demonstrate that microglial RAGE activation in presence of Aβ-enriched environment contributes to the EC vulnerability. PMID:28205565

  18. RNA-Seq analysis of the parietal cortex in Alzheimer's disease reveals alternatively spliced isoforms related to lipid metabolism.

    Science.gov (United States)

    Mills, James D; Nalpathamkalam, Thomas; Jacobs, Heidi I L; Janitz, Caroline; Merico, Daniele; Hu, Pingzhao; Janitz, Michael

    2013-03-01

    The parietal cortex of the human brain plays a unique role in the coordination of movement and in the integration of signals from the other cortices. Because of its extensive connections and involvement in many higher-order cognitive functions, neurodegenerative changes in the parietal lobe are believed to be crucial in the early symptoms of Alzheimer's disease (AD). Little is known about the transcriptome of this part of the human brain or how it is perturbed by the neurodegenerative process. To that end, we performed mRNA sequencing using the Illumina RNA-Seq technique on samples derived from normal and AD parietal lobes. Gene expression analysis evaluating alternatively spliced isoform expression and promoter usage revealed surprisingly elevated transcriptome activity in the AD condition. This phenomenon was particularly apparent in the alternative usage of transcriptional start sites. A Gene Ontology analysis of the differentially expressed genes revealed enrichment in the functional pathways related to lipid metabolism, thus highlighting the importance of astrocyte activity in the neurodegenerative process. We also identified an upregulation of the diazepam-binding inhibitor (DBI) gene in AD, as the result of a splicing switch toward shorter, intron-retaining isoforms driven by alternative promoters and was coupled with a simultaneous decrease in the abundance of protein-coding transcripts. These two DBI isoforms have not been described previously.

  19. [Pheochromocytomas as adrenal gland incidentalomas].

    Science.gov (United States)

    Cerović, Snezana; Cizmić, Milica; Milović, Novak; Ajdinović, Boris; Brajusković, Goran

    2002-07-01

    Adrenal incidentalomas are a heterogeneous group of pathological entities, including benign or malignant adrenocortical or medullary tumors, hormonally active or inactive lesions, which are identified incidentally during the examination of nonadrenal-related abdominal complaints. About 1.5% to 23% of adrenal incidentalomas are pheochromocytomas. Composite pheochromocytoma is a rare tumour of adrenal medulla with divergente clinical course. This type of pheochromocytoma is designated "composite" or "mixed," depending on whether pheochromocytoma and nonpheochromocytoma components show the same embryologic origin. Nonpheochromocytoma components found in the composite pheochromocytoma include ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and malignant schwannoma. The biologic behavior of composite pheochromocytomas may be as difficult to predict as more traditional pheochromocytomas; based on the number of cases reported to date the presence of areas resembling ganglioneuroblastoma or neuroblastoma does not necessary indicate a poor prognosis. Some may behave in a malignant fashion with metastasis by a component of the tumour which has neural features. Pheochromocytomas and paragangliomas are well-defined entities. Some of their nonsporadic associations and unusual morphological appearances are not universally appreciated. We report on a rare association of left adrenal CP, with typical right adrenal phochromocytoma and retroperitoneal paraganglioma, and a review of literature. We analyzed the clinical and immunohistochemical features in a 24-year-old woman with composite pheochromocytoma localized in the left adrenal gland and associated with blood pressure of 200/140 mmHg. Abdominal computed tomography and 131-J MIBG revealed a 65 x 60 mm mass in the right adrenal gland, but no revealed 45 x 40 mm retroperitoneal mass and 20 x 20 mm mass in the left adrenal region. Serum and urinary adrenaline levels were high, and catecholamine levels in the blood sample of

  20. Influence of Adrenal Cortex Function of Respiratory Failure Patients on Noninvasive Mechanical Ventilation Treatment ;Outcome%呼吸衰竭患者肾上腺皮质功能对无创机械通气治疗效果的影响研究

    Institute of Scientific and Technical Information of China (English)

    廖卫平; 朱丽阳; 周立新; 傅艳妮

    2016-01-01

    Objective To investigate the influence of adrenal cortex function of respiratory failure patients on noninvasive mechanical ventilation treatment outcome. Methods From February 2010 to August 2015,120 respiratory failure patients in intensive care units( ICU)of the 4th People's Hospital of Foshan City were selected in this study. The selected patients were divided into the normal adrenal cortex function group〔plasma cortisol( COR) ﹥3. 0 μg/dl,n =80〕and the adrenocortical insufficiency group(plasma COR≤3. 0 μg/dl,n=40)according to functional status of adrenal cortex of them at admission. 14 days after treatment,patients' rehabilitation condition,and plasma COR level,pulmonary function test index, heart rate and mean arterial pressure pre and posttreatment were also compared. Results There was no significant difference in gender,average age,BMI,acute physiology and chronic health evaluation Ⅱ score( APACHEⅡ),and protopathy between the two groups(P﹥0. 05). The standard time of weaning for the first time,duration of mechanical ventilation and time of ICU stay of the adrenocortical insufficiency group were significantly longer than those in normal group(P﹤0. 05). The plasma COR level of the adrenocortical insufficiency group pre and posttreatment was significantly lower than that of normal group(P﹤0. 05);the plasma COR level of the two groups posttreatment was significantly higher than that of pretreatment(P﹤0. 05). The forced vital capacity( FVC)and the ratio of forced expiratory volume to FVC( FEV1/FVC)between two groups pretreatment showed no significant differences ( P ﹥ 0. 05 );FVC and FEV1/FVC of the adrenocortical insufficiency group posttreatment were significantly lower than those of normal group ( P ﹤ 0. 05 );FVC and FEV1/FVC between two groups posttreatment were significantly higher than those of pretreatment(P﹤0. 05). The heart rate and average arterial pressure of the two groups pre and posttreatment showed no significant

  1. Incidentally Detected Kaposi Sarcoma of Adrenal Gland with Anaplastic Features in an HIV Negative Patient

    Directory of Open Access Journals (Sweden)

    Zeliha Esin Celik

    2016-01-01

    Full Text Available Kaposi sarcoma (KS, a vascular tumor caused by infection with human herpesvirus 8 (HHV8, is a systemic disease that can present with cutaneous lesions with or without visceral involvement. Very few cases of KS, most of which were associated with AIDS, have been reported in the adrenal gland. Anaplastic transformation of KS is a rare clinical presentation known as an aggressive disease with local recurrence and metastatic potential. We report here a 47-year-old HIV negative male presented with extra-adrenal symptoms and had an incidentally detected anaplastic adrenal KS exhibited aggressive clinical course. To the best of our knowledge, this is the first case of anaplastic primary adrenal KS without mucocutaneous involvement but subsequently developed other side adrenal metastases in an HIV negative patient.

  2. Incidentally Detected Kaposi Sarcoma of Adrenal Gland with Anaplastic Features in an HIV Negative Patient

    Science.gov (United States)

    Celik, Murat; Sen, Erdem; Cebeci, Hakan; Ata, Ozlem; Yavas, Cagdas

    2016-01-01

    Kaposi sarcoma (KS), a vascular tumor caused by infection with human herpesvirus 8 (HHV8), is a systemic disease that can present with cutaneous lesions with or without visceral involvement. Very few cases of KS, most of which were associated with AIDS, have been reported in the adrenal gland. Anaplastic transformation of KS is a rare clinical presentation known as an aggressive disease with local recurrence and metastatic potential. We report here a 47-year-old HIV negative male presented with extra-adrenal symptoms and had an incidentally detected anaplastic adrenal KS exhibited aggressive clinical course. To the best of our knowledge, this is the first case of anaplastic primary adrenal KS without mucocutaneous involvement but subsequently developed other side adrenal metastases in an HIV negative patient.

  3. Changes in motor cortex excitability associated with muscle fatigue in patients with Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Milanović Slađan

    2013-01-01

    Full Text Available Background/Aim. Transcranial magnetic stimulation (TMS is a standard technique for noninvasive assessment of changes in central nervous system excitability. The aim of this study was to examine changes in responses to TMS in patients suffering from Parkinson’s disease (PD during sustained submaximal isometric voluntary contraction [60% of maximal voluntary contraction (MVC] of the adductor pollicis muscle, as well as during a subsequent recovery period. Methods. Cortical excitability was tested by single TMS pulses of twice of the motor threshold intensity applied over the vertex. Testing was carried out during the sustained contraction phase every 10 s before and every 5 s after the endurance point, as well as at rest and during brief 60% MVC contractions before (control, immediately after the sustained contraction, and at 5 min intervals during the recovery period. Results. Although the PD patients could sustain the contraction at the required level for as long period of time as the healthy subjects (though contraction level subsided more rapidly after the endurance point, effects of muscle fatigue on the responses to TMS were different. In contrast to the findings observed in the healthy people where motor evoked potentials (MEP and EMG silent period (SP in fatigued muscle gradually diminished during contraction up to the endurance point, and increased thereafter, in the majority of patients no changes occurred in MEP size (peak and area of the adductor pollicis muscle, either before or after the endurance point. On the other hand, changes in the SP of this muscle differed among the subjects, showing a gradual increase, a decrease or no changes in duration. The trends of changes in both MEP size and SP duration in the musculus brachioradialis varied among the tested PD patients, without any consistent pattern, which was in contrast with the findings in the healthy people where both measures showed a gradual increase from the beginning of

  4. Cystic adrenal lesions: focus on pediatric population (a review)

    Science.gov (United States)

    CARSOTE, MARA; GHEMIGIAN, ADINA; TERZEA, DANA; GHEORGHISAN-GALATEANU, ANCUTA AUGUSTINA; VALEA, ANA

    2017-01-01

    Background and aim The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. General data Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks. Conclusion Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented

  5. Hypercortisolism and hyperandrogenism as manifestations of adrenal adenoma. Report on a Mexican girl and review of the literature

    Directory of Open Access Journals (Sweden)

    Paola de Baro-Álvarez

    2014-07-01

    Full Text Available Background: Adrenal cortex tumors (ACT are extremely rare in children and adolescents. Only 0.2% of all the new cases of cancer diagnosed in the United Stated and Europe are adrenal cortex tumors. The international incidence is not well defined, since it differs demographically. Due to the rarity of these tumors in children, little is known about their natural history. Case report: 11 months old female patient who developed, at the age of nine months,acne in forehead and thorax, also, weight gain, facial hair, apocrine activity, appearance of pubic hair, and hirsutism, increase of appetite, altered sleep-vigil cycle, increased height with accelerated growth velocity. From a biochemical standpoint, alterations in cortisol, showed an increase in morning and evening cortisol levels, as well as androgens. CT scan showed an adrenal mass on the right side not dependent of the kidney, which was completely resected. The  histopathology report was of adenoma of the adrenal cortex. Currently, the patient is asymptomatic and is followed at the Endocrinology Service with dose reduction of steroid. Conclusion: Because adrenal cortex tumors are extremely rare in children, a small percentage appears with virilization data and Cushing’s syndrome. For this reason, we should be aware of their existence in order to make the diagnosis and treat them as soon as possible.

  6. Determination of Adrenal Volume by MRI in Healthy Children

    DEFF Research Database (Denmark)

    Mouritsen, Annette; Lindhardt Johansen, Marie; Wohlfart-Veje, Christine;

    2014-01-01

    Adrenal disorders like Congenital Adrenal Hyperplasia result in abnormal adrenal size and morphology, but little is known about the clinical value of magnetic resonance imaging (MRI) in determining adrenal volume....

  7. [Adrenal cortex insufficiency in children due to inhaled corticosteroids

    NARCIS (Netherlands)

    Eijkemans, M.C.J.; Otten, B.J.; Yntema, J.L.

    2011-01-01

    A 3-year-old boy was treated for asthmatic symptoms with fluticasone inhalations. Due to a flattening growth curve Cushing's syndrome was suspected and the dosage of fluticasone was gradually decreased after which the boy became less active and his appetite decreased. Another patient, a 7-year-old b

  8. Expression of the IGF and the aromatase/estrogen receptor systems in human adrenal tissues from early infancy to late puberty: implications for the development of adrenarche.

    Science.gov (United States)

    Belgorosky, Alicia; Baquedano, María Sonia; Guercio, Gabriela; Rivarola, Marco A

    2009-03-01

    Adrenarche is a process of postnatal sexual maturation occurring in higher primates, in which there is an increase in the secretion of adrenal androgens. It is the consequence of a process of postnatal organogenesis characterized by the development of a new zone in the adrenal cortex, the zona reticularis (ZR). The mechanism of this phenomenon remains poorly understood, suggesting that it might be a multifactorial event. A relationship between circulating IGF-I, insulin sensitivity, and adrenal androgens has been postulated. Boys and girls have different patterns of changes in insulin sensitivity at puberty, perhaps secondary to differences in the estrogen milieu. Estrogen effects may also play a role in premature adrenarche. Peripheral or local IGF-1 actions could regulate adrenal progenitor cell proliferation and migration. Since adrenal progenitor cells as well as IGF-I and the IGF-R1 are located in the outer zone of the adrenal cortex during childhood and adolescence, this peripheral cell layer, below the capsule, may contain undifferentiated progenitor cells. Therefore, the IGF-R1 signaling pathway might positively modulate the proliferation and migration of adrenal progenitor cell to stimulate the development of adrenal zones, including ZR. However, no evidence of a direct action of IGF-I on ZR was found. In addition, a role for estrogens in the ontogenesis of ZR is suggested by the presence of aromatase (CYP19) in the subcapsular zona glomerulosa and in the adrenal medulla. Estrogens produced locally could act on ZR by interacting with estrogen receptor beta (ERbeta), but not alpha, and membrane estrogen receptor GPR-30. An estradiol-induced increase in DHEA/cortisol ratio was indeed seen in cultures of adrenocortical cells from post-adrenarche adrenals. In summary, several lines of evidence point to the action of multiple factors, such as local adrenal maturational changes and peripheral metabolic signals, on postnatal human adrenal gland ZR formation.

  9. Hypercortisolism and hyperandrogenism as manifestations of adrenal adenoma. Report on a Mexican girl and review of the literature

    OpenAIRE

    Paola de Baro-Álvarez; Lizbeth García-García; Olinka Reyes-Posada; Jorge Cortes-Sauza; Pedro A Sánchez-Márquez

    2014-01-01

    Background: Adrenal cortex tumors (ACT) are extremely rare in children and adolescents. Only 0.2% of all the new cases of cancer diagnosed in the United Stated and Europe are adrenal cortex tumors. The international incidence is not well defined, since it differs demographically. Due to the rarity of these tumors in children, little is known about their natural history. Case report: 11 months old female patient who developed, at the age of nine months,acne in forehead and thorax, also, we...

  10. Comparison of miRNA expression profiles in pituitary-adrenal axis between Beagle and Chinese Field dogs after chronic stress exposure.

    Science.gov (United States)

    Luo, Wei; Fang, Meixia; Xu, Haiping; Xing, Huijie; Fu, Jiangnan; Nie, Qinghua

    2016-01-01

    MicoRNAs (miRNAs), usually as gene regulators, participate in various biological processes, including stress responses. The hypothalamus-pituitary-adrenal axis (HPA axis) is an important pathway in regulating stress response. Although the mechanism that HPA axis regulates stress response has been basically revealed, the knowledge that miRNAs regulate stress response within HPA axis, still remains poor. The object of this study was to investigate the miRNAs in the pituitary and adrenal cortex that regulate chronic stress response with high-throughput sequencing. The pituitary and adrenal cortex of beagles and Chinese Field dogs (CFD) from a stress exposure group (including beagle pituitary 1 (BP1), CFD pituitary 1 (CFDP1), beagle adrenal cortex 1 (BAC1), CFD adrenal cortex 1 (CFDAC1)) and a control group (including beagle pituitary 2 (BP2), CFD pituitary 2 (CFDP2), beagle adrenal cortex 2 (BAC2), CFD adrenal cortex 2 (CFDAC2)), were selected for miRNA-seq comparisons. Comparisons, that were made in pituitary (including BP1 vs. BP2, CFDP1 vs. CFDP2, BP1 vs. CFDP1 and BP2 vs. CFDP2) and adrenal cortex (including BAC1 vs. BAC2, CFDAC1 vs. CFDAC2, BAC1 vs. CFDAC1 and BAC2 vs. CFDAC2), showed that a total of 39 and 18 common differentially expressed miRNAs (DE-miRNAs) (Total read counts > 1,000, Fold change > 2 & p-value 2 & p-value < 0.05) from those target genes in pituitary and adrenal cortex were obtained separately, in combination with our previous corresponding transcriptome study. Meanwhile, in line with that miRNAs usually negatively regulated their target genes and the dual luciferase reporter assay, we finally identified cfa-miR-205 might play an important role by upregulating MMD in pituitary and hippocampus, thus enhancing the immune response, under chronic stress exposure. Our results shed light on the miRNA expression profiles in the pituitary and adrenal cortex with and without chronic stress exposure, and provide a new insight into miR-205 with its

  11. Scrotal hematoma as a sign of adrenal hemorrhage in newborns

    Directory of Open Access Journals (Sweden)

    Renata Gonçalves

    Full Text Available CONTEXT: Bluish discoloration and swelling of the scrotum in newborns can arise from a number of diseases, including torsion of the testes, orchitis, scrotal or testicular edema, hydrocele, inguinal hernia, meconium peritonitis, hematocele, testicular tumor and traumatic hematoma. Forty-two cases of scrotal abnormalities as signs of neonatal adrenal hemorrhage were found in the literature. CASE REPORT: We present a case of scrotal hematoma due to adrenal hemorrhage in a newborn. Conservative treatment with clinical follow-up was adopted, with complete resolution within 10 days. The possible differential diagnoses are reviewed and discussed.

  12. Severe Psychotic Disorder as the Main Manifestation of Adrenal Insufficiency

    Science.gov (United States)

    Farah, Julia de Lima; Lauand, Carolina Villar; Chequi, Lucas; Fortunato, Enrico; Pasqualino, Felipe; Bignotto, Luis Henrique; Batista, Rafael Loch; Aprahamian, Ivan

    2015-01-01

    We describe a case of severe psychotic disorder as the only manifestation of primary adrenal insufficiency. A 63-year-old man presented with psychotic symptoms without any prior psychiatric history. During the clinical and laboratorial investigation, exams revealed a normovolemic hyponatremia. The patient showed no other clinical signs or symptoms compatible with adrenal insufficiency but displayed very high ACTH and low serum cortisol concentrations. Brain magnetic resonance imaging showed no significant changes, including the pituitary gland. The patient was initially treated with intravenous corticosteroids, resulting in rapid remission of the psychotic symptoms. The association between adrenal insufficiency and neuropsychiatric symptoms is rare but these symptoms can often be the first clinical presentation of the disease. PMID:25954562

  13. Severe Psychotic Disorder as the Main Manifestation of Adrenal Insufficiency

    Directory of Open Access Journals (Sweden)

    Julia de Lima Farah

    2015-01-01

    Full Text Available We describe a case of severe psychotic disorder as the only manifestation of primary adrenal insufficiency. A 63-year-old man presented with psychotic symptoms without any prior psychiatric history. During the clinical and laboratorial investigation, exams revealed a normovolemic hyponatremia. The patient showed no other clinical signs or symptoms compatible with adrenal insufficiency but displayed very high ACTH and low serum cortisol concentrations. Brain magnetic resonance imaging showed no significant changes, including the pituitary gland. The patient was initially treated with intravenous corticosteroids, resulting in rapid remission of the psychotic symptoms. The association between adrenal insufficiency and neuropsychiatric symptoms is rare but these symptoms can often be the first clinical presentation of the disease.

  14. Postnatal expression and distribution of Refsum disease gene associated protein in the rat retina and visual cortex: effect of binocular visual deprivation.

    Science.gov (United States)

    Ahn, Kyu Youn; Nam, Kwang Il; Kim, Baik Yoon; Cho, Chul Woong; Jeong, Sang Ki; Yang, Kun Jin; Kim, Kyung Keun

    2002-04-01

    Previously, phytanoyl-CoA alpha-hydroxylase-associated protein 1 (PAHX-AP1) was isolated as a novel neuron-specific protein to interact with Refsum disease (RfD) gene PAHX. Its expression in the brain increased after eyelid opening, and the elevated level was maintained through adulthood. In this report, to verify the hypothesis that light could trigger this increase, we have examined the developmental distribution pattern of PAHX-AP1 in rat retina and visual cortex, and changes of its expression by binocular deprivation. Northern blot analyses demonstrated PAHX-AP1 expression reached its highest level in the visual cortex and eyeball at 4 weeks after birth, and these levels were maintained through adult life. Two weeks after visual deprivation, its expression in the eyeball and visual cortex decreased compared with the control. In situ hybridization analyses of the retina showed that PAHX-AP1 expression was limited to the ganglionic cell layer at 10 days after birth, but expressed in the inner nuclear cell layer and extended to the outer nuclear cell layer at 2 and 3 weeks after birth, respectively. Two weeks after visual deprivation, however, it decreased in the ganglionic and inner nuclear cell layer, and disappeared in the rod and cone cell layers. In the visual cortex, strong signals of PAHX-AP1 were detected in layers IV and VI, and II-VI at 10 days and 2 weeks after birth, respectively. Its expression decreased after 2 weeks of visual deprivation. These results indicate that visual stimulation is essential for the maintenance of PAHX-AP1 expressions in the retina, especially in the rod and cone cell layers, and visual cortex, and suggest that PAHX-AP1 may be involved in the developmental regulation of the photoreceptor's function.

  15. Cushing syndrome due to adrenal tumor

    Science.gov (United States)

    Adrenal tumor - Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the ... or cancerous (malignant). Noncancerous tumors that can cause ... Adrenal adenomas Micronodular hyperplasia Cancerous tumors that ...

  16. Genetics Home Reference: primary macronodular adrenal hyperplasia

    Science.gov (United States)

    ... Genetics Home Health Conditions PMAH primary macronodular adrenal hyperplasia Enable Javascript to view the expand/collapse boxes. ... Open All Close All Description Primary macronodular adrenal hyperplasia ( PMAH ) is a disorder characterized by multiple lumps ( ...

  17. Spontaneous bilateral adrenal hemorrhage following cholecystectomy.

    Science.gov (United States)

    Dahan, Meryl; Lim, Chetana; Salloum, Chady; Azoulay, Daniel

    2016-06-01

    Postoperative bilateral adrenal hemorrhage is a rare but potentially life-threatening complication. This diagnosis is often missed because the symptoms and laboratory results are usually nonspecific. We report a case of bilateral adrenal hemorrhage associated with acute primary adrenal insufficiency following laparoscopic cholecystectomy. The knowledge of this uncommon complication following any abdominal surgery allows timey diagnosis and rapid treatment.

  18. Adrenal Hypoplasia Congenita Presenting as Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Jennifer L. Flint

    2013-01-01

    Full Text Available We report on a patient with genetically confirmed adrenal hypoplasia congenita (AHC whose presentation and laboratory abnormalities were consistent with the more common condition, congenital adrenal hyperplasia (CAH. The patient presented with failure to thrive and salt wasting. General appearance showed marked hyperpigmentation and normal male genitalia. He displayed mildly elevated 17-hydroxyprogesterone and markedly elevated 11-deoxycortisol levels at baseline and with ACTH stimulation testing. Results were consistent with 11β-hydroxylase deficiency. He required glucocorticoids and high doses of mineralocorticoids. The marked elevation in 11-deoxycortisol directed our clinical reasoning away from a hypoplastic condition and towards a hyperplasic adrenal condition. Sequencing of the DAX1 gene (named for dosage-sensitive sex reversal (DSS locus and the AHC locus on the X chromosome revealed a missense mutation. A review of the literature revealed that elevated 11-deoxycortisol levels have been noted in kindreds with DAX1 mutations, but only when measured very early in life. A mouse model has recently been described that displays elevated 11-deoxycorticosterone levels and evidence for hyperplasia of the zona glomerulosa of the adrenal gland. We conclude that DAX1 testing may be considered in patients with laboratory evidence of 11β-hydroxylase deficiency, especially in those with severe salt wasting.

  19. Primary antiphospholipid antibody syndrome with adrenal hemorrhage in a child : a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun; Lee, Soo Hyun; Kim, Hyun Joo; Yoo, Han Wook; Yoon, Chong Hyun [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-11-01

    Primary antiphospholipid antibody syndrome is a disease that is clinically diagnosed if a patient suffers recurrent thromboses, stroke, recurrent fetal loss, livedo reticularis, and thrombocytopenia, without evidence of systemic lupus erythematosus or other connective diseases. Adrenal hemorrhage in a patient with primary antiphospholipid antibody syndrome is a rarely recognized, but potentially catastrophic disorder. We recently encountered bilateral adrenal hemorrhaging in a child with antiphospholipid antibody syndrome and casem as well as reviewing the literature.

  20. Computed tomography of the adrenals in patients with tuberculosis; Emprego da tomografia computadorizada na deteccao de alteracoes das adrenais em doentes com tuberculose

    Energy Technology Data Exchange (ETDEWEB)

    Yamashita, Seizo; Machado, Jussara Marcondes; Morceli, Jose [UNESP, Botucatu, SP (Brazil). Faculdade de Medicina. Dept. de Doencas Tropicais e Diagnostico por Imagem]. E-mail: seizo_eid@uol.com.br

    2004-06-01

    We evaluated the adrenals of individuals without tuberculosis (group 1 - G1) and with tuberculosis (group 2 - G2) using computed tomography. The antero-posterior length, the thickness, and the longitudinal length of the adrenals were compared in G1 and G2. The duration of the disease, the occurrence of morphologic abnormalities in G2, the distribution according to sex, age and skin color were also studied. There was difference in the antero-posterior length and thickness of right adrenal between G1 and G2. A higher prevalence of white skin male individuals was observed in G2. There was no association between duration of the disease and the occurrence of morphologic abnormalities in G2. A higher occurrence of adrenal enlargement was observed in G2. The main computed tomography findings in patients with tuberculosis were enlargement of the adrenal without calcification, enlargement of the adrenal with punctiform calcifications, and residual calcification of the adrenal. (author)

  1. Adrenal morpho-functional alterations in patients with acromegaly.

    Science.gov (United States)

    Scaroni, C; Selice, R; Benedini, S; De Menis, E; Arosio, M; Ronchi, C; Gasperi, M; Manetti, L; Arnaldi, G; Polenta, B; Boscaro, M; Albiger, N; Martino, E; Mantero, F

    2008-07-01

    Acromegaly is associated with a greater morbidity and higher incidence of tumors, possibly due to the permissive role of elevated GH and IGF-I levels. In the general population, adrenal masses are frequently discovered (prevalence 1-5%) at computed tomography (CT). We evaluated the prevalence of adrenal lesions in patients with acromegaly. We studied 94 acromegalic patients, 54 females (mean age 55.0+/-16.0 yr) and 40 males (mean age 50+/-14 yr) referred to 5 Endocrinology Units between 2001-2003; 49 had active disease and 45 had been treated with surgery and/or were controlled with medical therapy. Abdominal CT showed adrenal lesions in 27 patients; 9 of them had unilateral masses (10%) with benign features (diameter 0.5-3 cm) and 18 had hyperplasia (14 monolateral and 4 bilateral), with no significant differences between patients with active vs controlled disease, and with no correlation between prevalence of masses and duration of disease, GH and IGF-I levels. Hormone study (urinary free cortisol, catecholamines/metanephrines, upright plasma renin activity and aldosterone, morning plasma ACTH and low-dose dexamethasone suppression test) disclosed no major endocrine alterations. During a 1-yr follow-up, the adrenal masses increased in size in 3 cases and 1 patient also developed subclinical Cushing's syndrome. Adrenal lesions seem more frequent in acromegaly than in the general population, but no single factor (GH/IGF-I levels or disease duration) predicts them. The masses appear to be benign and nonhypersecreting, but a longer follow-up is recommended to disclose any changes in their morphofunctional state.

  2. ACTH-Independent Cushing’s Syndrome with Bilateral Micronodular Adrenal Hyperplasia and Ectopic Adrenocortical Adenoma

    Science.gov (United States)

    Louiset, Estelle; Gobet, Françoise; Libé, Rossella; Horvath, Anelia; Renouf, Sylvie; Cariou, Juliette; Rothenbuhler, Anya; Bertherat, Jérôme; Clauser, Eric; Grise, Philippe; Stratakis, Constantine A.; Kuhn, Jean-Marc; Lefebvre, Hervé

    2010-01-01

    Context: Bilateral micronodular adrenal hyperplasia and ectopic adrenocortical adenoma are two rare causes of ACTH-independent Cushing’s syndrome. Objective: The aim of the study was to evaluate a 35-yr-old woman with ACTH-independent hypercortisolism associated with both micronodular adrenal hyperplasia and ectopic pararenal adrenocortical adenoma. Design and Setting: In vivo and in vitro studies were performed in a University Hospital Department and academic research laboratories. Intervention: Mutations of the PRKAR1A, PDE8B, and PDE11A genes were searched for in leukocytes and adrenocortical tissues. The ability of adrenal and adenoma tissues to synthesize cortisol was investigated by immunohistochemistry, quantitative PCR, and/or cell culture studies. Main Outcome Measure: Detection of 17α-hydroxylase and 21-hydroxylase immunoreactivities, quantification of CYP11B1 mRNA in adrenal and adenoma tissues, and measurement of cortisol levels in supernatants by radioimmunological assays were the main outcomes. Results: Histological examination of the adrenals revealed nonpigmented micronodular cortical hyperplasia associated with relative atrophy of internodular cortex. No genomic and/or somatic adrenal mutations of the PRKAR1A, PDE8B, and PDE11A genes were detected. 17α-Hydroxylase and 21-hydroxylase immunoreactivities as well as CYP11B1 mRNA were detected in adrenal and adenoma tissues. ACTH and dexamethasone activated cortisol secretion from adenoma cells. The stimulatory action of dexamethasone was mediated by a nongenomic effect involving the protein kinase A pathway. Conclusion: This case suggests that unknown molecular defects can favor both micronodular adrenal hyperplasia and ectopic adrenocortical adenoma associated with Cushing’s syndrome. PMID:19915020

  3. Adrenal Disorders and the Paediatric Brain: Pathophysiological Considerations and Clinical Implications

    Directory of Open Access Journals (Sweden)

    Vincenzo Salpietro

    2014-01-01

    Full Text Available Various neurological and psychiatric manifestations have been recorded in children with adrenal disorders. Based on literature review and on personal case-studies and case-series we focused on the pathophysiological and clinical implications of glucocorticoid-related, mineralcorticoid-related, and catecholamine-related paediatric nervous system involvement. Childhood Cushing syndrome can be associated with long-lasting cognitive deficits and abnormal behaviour, even after resolution of the hypercortisolism. Exposure to excessive replacement of exogenous glucocorticoids in the paediatric age group (e.g., during treatments for adrenal insufficiency has been reported with neurological and magnetic resonance imaging (MRI abnormalities (e.g., delayed myelination and brain atrophy due to potential corticosteroid-related myelin damage in the developing brain and the possible impairment of limbic system ontogenesis. Idiopathic intracranial hypertension (IIH, a disorder of unclear pathophysiology characterised by increased cerebrospinal fluid (CSF pressure, has been described in children with hypercortisolism, adrenal insufficiency, and hyperaldosteronism, reflecting the potential underlying involvement of the adrenal-brain axis in the regulation of CSF pressure homeostasis. Arterial hypertension caused by paediatric adenomas or tumours of the adrenal cortex or medulla has been associated with various hypertension-related neurological manifestations. The development and maturation of the central nervous system (CNS through childhood is tightly regulated by intrinsic, paracrine, endocrine, and external modulators, and perturbations in any of these factors, including those related to adrenal hormone imbalance, could result in consequences that affect the structure and function of the paediatric brain. Animal experiments and clinical studies demonstrated that the developing (i.e., paediatric CNS seems to be particularly vulnerable to alterations induced by

  4. High grade primary adrenal intravascular large B-cell lymphoma manifesting as Addison disease Linfoma intravascular de alto grado de células B grandes y origen suprarrenal que se manifiesta en forma de enfermedad de Addison

    Directory of Open Access Journals (Sweden)

    J. Venizelos

    2007-08-01

    Full Text Available We report a rare case of a 68 aged male who presented with adrenal failure and was diagnosed of high grade large B-cell lymphoma primarily arising in the adrenal glands. The patient was administrated with additional chemotherapy but he passed away 7 months later due to infection in the lungs. Intravascular lymphoma should be suspected in patients with bilateral adrenal masses who present with rapidly progressive adrenal insufficiency.Publicamos el caso poco frecuente de un varón de 68 años de edad que debutó con insuficiencia adrenal y fue diagnosticado de linfoma de alto grado de células B grandes ubicado principalmente en las glándulas suprarrenales. Al paciente le administraron quimioterapia adicional, pero falleció 7 meses después de infección pulmonar. El linfoma intravascular debe sospecharse en los pacientes con masas suprarrenales bilaterales que presenten insuficiencia adrenal rápidamente progresiva.

  5. Adrenal hemorrhage in a newborn.

    Science.gov (United States)

    Abdu, Arebu T; Kriss, Vesna M; Bada, Henrietta S; Reynolds, Eric W

    2009-09-01

    Sometimes in the course of care in a neonatal intensive care unit, there may be a rush to intervene in cases where limited intervention is actually the correct course. One such example is that of neonatal adrenal hemorrhage. We present the case of a male term neonate with shock, metabolic acidosis, distended abdomen, and falling hematocrit. His prenatal and delivery histories were uneventful except for a nuchal cord. Apgar scores were 9 and 9. Because of his dramatic presentation, certain members of the medical team suggested immediate surgical intervention. However, a calm and careful evaluation revealed the true diagnosis and course of action. Ultrasound of the abdomen showed a mass between the liver and kidney, but the origin was difficult to identify. A computed tomography scan supported the diagnosis of right adrenal hemorrhage. His serum cortisol level was normal. The patient was managed conservatively and discharged home after a 1-week stay in the hospital. Subsequent abdominal ultrasound showed resolving adrenal hemorrhage with minimal calcification. A review of the pertinent literature is presented. Physicians should remember adrenal hemorrhage when evaluating a newborn infant with shock, acidosis, abdominal distention, and falling hematocrit and that conservative management is usually indicated.

  6. Adrenal cryptococcosis in an immunosuppressed patient showing intensely increased metabolic activity on (18)F-FDG PET/CT.

    Science.gov (United States)

    Papadakis, Georgios Z; Holland, Steven M; Quezado, Martha; Patronas, Nicholas J

    2016-12-01

    Disseminated cryptococcosis most commonly occurs in immunosuppressed patients and can rarely affect the adrenal glands. We report on a patient with biopsy proven bilateral adrenal cryptococcosis resulting in primary adrenal insufficiency, which was evaluated with whole-body positron emission tomography/computed tomography scan using (18)F-FDG. Both enlarged adrenal glands presented intensely increased (18)F-FDG activity in the periphery, while central necrotic regions were photopenic. Although diagnosis was established by adrenal gland biopsy, (18)F-FDG positron emission tomography/computed tomography scan can significantly contribute to the assessment of disease activity and monitoring of treatment response. Furthermore, fungal infections should always be considered when encountering hypermetabolic adrenal masses, especially in the setting of immunodeficient patients.

  7. [Adrenal injury in blunt abdominal trauma].

    Science.gov (United States)

    Abakumov, M M; Smoliar, A N; Barmina, T G; Boĭko, A V; Shalimova, I G

    2009-01-01

    10 patients with adrenal damage were observed during 2.5 years. It amounted 0.93% of all patients with closed abdominal injuries. The right adrenal gland was traumatized in all cases evidently due to it's compression between right lobe of liver and vertebral column. Adrenal damage is observed quite often in combination with injuries of right liver lobe, right kidney and retroperitoneal hematoma formation. 5 patients underwent laparotomy on account of intra-abdominal bleeding, but adrenal damage was never revealed. Ultrasound and tomographic semiotics of adrenal damage was worked out, which allowed ascertaining diagnosis in 80% on application of ultrasound study and in 100% at computer tomography. Injury of one adrenal gland was not accompanied by adrenal failure and did not require hormonal replacement therapy.

  8. Intraoperative identification of adrenal-renal fusion

    Science.gov (United States)

    Boll, Griffin; Rattan, Rishi; Yilmaz, Osman; Tarnoff, Michael E

    2015-01-01

    Adrenal - renal fusion is a rare entity defined as incomplete encapsulation of the adrenal gland and kidney with histologically adjacent functional tissue. This report describes the first published intraoperative identification of this anomaly during laparoscopic adrenalectomy. The patient was a 59-year-old man with chronic hypertension refractory to multiple antihypertensives found to be caused by a right-sided aldosterone-producing adrenal adenoma in the setting of bilateral adrenal hyperplasia. During laparoscopic adrenalectomy, the normal avascular plane between the kidney and adrenal gland was absent. Pathologic evaluation confirmed adrenal - renal fusion without adrenal heterotopia. Identified intraoperatively, this may be misdiagnosed as invasive malignancy, and thus awareness of this anomaly may help prevent unnecessarily morbid resection. PMID:26195881

  9. Age-related changes of brain iron load changes in the frontal cortex in APPswe/PS1ΔE9 transgenic mouse model of Alzheimer's disease.

    Science.gov (United States)

    Xian-hui, Dong; Wei-juan, Gao; Tie-mei, Shao; Hong-lin, Xie; Jiang-tao, Bai; Jing-yi, Zhao; Xi-qing, Chai

    2015-04-01

    Alzheimer's disease (AD) as a neurodegenerative brain disorder is a devastating pathology leading to disastrous cognitive impairments and dementia, associated with major social and economic costs to society. Iron can catalyze damaging free radical reactions. With age, iron accumulates in brain frontal cortex regions and may contribute to the risk of AD. In this communication, we investigated the age-related brain iron load changes in the frontal cortex of 6- and 12-month-old C57BL/6J (C57) and APPswe/PS1ΔE9 (APP/PS1) double transgenic mouse by using graphite furnace atomic absorption spectrometry (GFAAS) and Perls' reaction. In the present study, we also evaluated the age-related changes of DMT1 and FPN1 by using Western blot and qPCR. We found that compared with 6-month-old APP/PS1 mice and the 12-month-old C57 mice, the 12-month-old APP/PS1 mice had increased iron load in the frontal cortex. The levels of DMT1 were significantly increased and the FPN1 were significantly reduced in the frontal cortex of the 12-month-old APP/PS1 mice than that in the 6-month-old APP/PS1 mice and 12-month-old C57 mice. We conclude that in AD damage occurs in conjunction with iron accumulation, and the brain iron load associated with loss control of the brain iron metabolism related protein DMT1 and FPN1 expressions.

  10. Silent intravascular lymphoma initially manifesting as a unilateral adrenal incidentaloma.

    Science.gov (United States)

    Takahashi, Yoshiko; Iida, Keiji; Hino, Yasuhisa; Ohara, Takeshi; Kurahashi, Toshifumi; Tashiro, Takashi; Chihara, Kazuo

    2012-01-01

    Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of malignant lymphoma. Although the involvement of adrenal glands in IVLBCL is often observed, primary adrenal IVLBCL is rare. Most reported cases of adrenal IVLBCL showed bilateral lesions resulting in rapidly progressive adrenal failure and poor prognosis. Here, we report a case of slowly progressive primary adrenal IVLBCL manifesting initially with unilateral adrenal incidentaloma. This case is a silent IVLBCL and shows that the enlargement of both adrenal glands can be followed.

  11. Refractory hypoglycemia in a patient with functional adrenal cortical carcinoma

    Directory of Open Access Journals (Sweden)

    Katia Regina Marchetti

    2016-11-01

    Full Text Available Adrenacarcinomas are rare, and hypoglycemic syndrome resulting from the secretion of insulin-like growth factor II (IGF-II by these tumors have been described infrequently. This study describes the case of a young woman with severe persistent hypoglycemia and a large adrenal tumor and discusses the physiopathological mechanisms involved in hypoglycemia. The case is described as a 21-year-old woman who presented with 8 months of general symptoms and, in the preceding 3 months, with episodes of mental confusion and visual blurring secondary to hypoglycemia. A functional assessment of the adrenal cortex revealed ACTH-independent hypercortisolism and hyperandrogenism. Hypoglycemia, hypoinsulinemia, low C-peptide and no ketones were also detected. An evaluation of the GH–IGF axis revealed GH blockade (0.03; reference: up to 4.4 ng/mL, greatly reduced IGF-I levels (9.0 ng/mL; reference: 180–780 ng/mL, slightly reduced IGF-II levels (197 ng/mL; reference: 267–616 ng/mL and an elevated IGF-II/IGF-I ratio (21.9; reference: ~3. CT scan revealed a large expansive mass in the right adrenal gland and pulmonary and liver metastases. During hospitalization, the patient experienced frequent difficult-to-control hypoglycemia and hypokalemia episodes. Octreotide was ineffective in controlling hypoglycemia. Due to unresectability, chemotherapy was tried, but after 3 months, the patient’s condition worsened and progressed to death. In conclusion, our patient presented with a functional adrenal cortical carcinoma, with hyperandrogenism associated with hypoinsulinemic hypoglycemia and blockage of the GH–IGF-I axis. Patient’s data suggested a diagnosis of hypoglycemia induced by an IGF-II or a large IGF-II-producing tumor (low levels of GH, greatly decreased IGF-I, slightly decreased IGF-II and an elevated IGF-II/IGF-I ratio.

  12. Inclusões intracitoplasmáticas hialinas na medular da adrenal de bovinos

    Directory of Open Access Journals (Sweden)

    L.P Mesquita

    2011-02-01

    Full Text Available Cytoplasmic inclusion bodies in adrenal medullary chromaffin cells have been described in various species including humans. These inclusions are believed to be related to certain infectious, toxic and neurodegenerative diseases. No reports concerning such adrenal inclusions have been described in bovines. Adrenal glands from twenty bovines were evaluated in a retrospective study. Seven of these exhibited inclusions - three cases of rabies, two cases of chronic suppurative bronchopneumonia, one case of chronic suppurative peritonitis, and one case of gangrenous mastitis. The inclusions were present in higher numbers especially in cases of rabies and also in one case of chronic suppurative bronchopneumonia. The inclusions were intracytoplasmic, eosinophilic, rounded, single or multiple, of various sizes, strongly stained by PAS and were present in higher numbers in the external layer of the adrenal medulla. The inclusions were negative when subjected to immunohistochemistry for detection of viral antigens in the cases of rabies. Although inclusion bodies were present in adrenal glands devoid of other histological alterations, they were more abundant in cases in which the adrenal gland had other alterations. The correlation between certain diseases and the development of inclusion bodies is not known, which highlights the importance of further studies on these inclusions in adrenal glands of bovines.

  13. Extra-adrenal glucocorticoid synthesis: immune regulation and aspects on local organ homeostasis.

    Science.gov (United States)

    Talabér, Gergely; Jondal, Mikael; Okret, Sam

    2013-11-05

    Systemic glucocorticoids (GCs) mainly originate from de novo synthesis in the adrenal cortex under the control of the hypothalamus-pituitary-adrenal (HPA)-axis. However, research during the last 1-2 decades has revealed that additional organs express the necessary enzymes and have the capacity for de novo synthesis of biologically active GCs. This includes the thymus, intestine, skin and the brain. Recent research has also revealed that locally synthesized GCs most likely act in a paracrine or autocrine manner and have significant physiological roles in local homeostasis, cell development and immune cell activation. In this review, we summarize the nature, regulation and known physiological roles of extra-adrenal GC synthesis. We specifically focus on the thymus in which GC production (by both developing thymocytes and epithelial cells) has a role in the maintenance of proper immunological function.

  14. Influence of magnetic field onnitric oxide and neuropeptideY in rat adrenal gland

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    We have investigated the effects of magnetic fields on nitric acid (NO) and neuropeptide Y (NPY) on adrenal glands of the rat using NO nitric acid reductase-spec- trophotometry and histochemistry techniques. We found that all cellular layers of the adrenal cortex, including zona glomerulosa, zona fasciculata and zona reticularis were stained by NADPH-d, and some chromaffin cells of the medulla were positive for NPY. Furthermore, magnetic fields increased NO so strongly that high NO levels could be maintained for several hours, as well as some neuroganglion cells in medulla that were double-stained for NPY and NADPH-d. Our data showed that the magnetic field can regulate endocrine and neuroendocrine directly by some action on parenchyma cells, or indirectly by action to NO-ergic, NPY-ergic neurons in the adrenal gland.

  15. Physiological Basis for the Etiology, Diagnosis, and Treatment of Adrenal Disorders: Cushing’s Syndrome, Adrenal Insufficiency, and Congenital Adrenal Hyperplasia

    OpenAIRE

    Raff, Hershel; Sharma, Susmeeta T.; Nieman, Lynnette K.

    2014-01-01

    The hypothalamic-pituitary-adrenal (HPA) axis is a classic neuroendocrine system. One of the best ways to understand the HPA axis is to appreciate its dynamics in the variety of diseases and syndromes that affect it. Excess glucocorticoid activity can be due to endogenous cortisol overproduction (spontaneous Cushing’s syndrome) or exogenous glucocorticoid therapy (iatrogenic Cushing’s syndrome). Endogenous Cushing’s syndrome can be subdivided into ACTH-dependent and ACTH-independent, the latt...

  16. Physical, social and societal functioning of children with congenital adrenal hyperplasia (CAH) and their parents, in a Dutch population.

    NARCIS (Netherlands)

    Sanches, S.A.; Wiegers, T.A.; Otten, B.J.; Claahsen-van der Grinten, H.L.

    2012-01-01

    BACKGROUND: Most research concerning congenital adrenal hyperplasia (CAH) and related conditions caused by primary adrenal insufficiency, such as Addison's or Cushing's disease, has focused on medical aspects rather than on patients' quality of life. Therefore, our objective was to investigate the p

  17. Primary bilateral adrenal intravascular large B-cell lymphoma associated with adrenal failure.

    Science.gov (United States)

    Fukushima, Ayumi; Okada, Yosuke; Tanikawa, Takahisa; Onaka, Takashi; Tanaka, Aya; Higashi, Takehiro; Tsukada, Junichi; Tanaka, Yoshiya

    2003-07-01

    We report a rare case of bilateral primary adrenal non-Hodgkin's lymphoma with adrenal failure. A 66-year-old woman developed symptoms of adrenal failure. The cause of adrenal failure was suspected to be malignant lymphoma based on the high levels of serum soluble interleukin-2 receptor and LDH. Bilateral adrenalectomy was performed and pathological examination showed intravascular large B-cell lymphoma (IVL). Although complete remission was achieved, recurrence occurred three months later with brain metastases. IVL should be suspected in patients with bilateral adrenal tumors who present with rapidly progressive adrenal failure.

  18. Surgical resection of adrenal metastasis from primary liver tumors:a report of two cases

    Institute of Scientific and Technical Information of China (English)

    Durgatosh Pandey; Kai-Chah Tan

    2008-01-01

    BACKGROUND: Although the treatment of extrahepatic metastases from primary liver tumors is essentially palliative, solitary metastasis from such tumors offers a possibility of cure by surgical resection. The adrenal gland is an uncommon site for metastasis from primary liver tumors. METHOD: We report two cases of adrenalectomy for solitary adrenal metastasis: one from intrahepatic cholangiocarcinoma and the other from hepatocellular carcinoma. RESULTS: The patient with intrahepatic cholangiocar-cinoma had a synchronous adrenal metastasis and underwent simultaneous liver resection and adrenalectomy. However, he developed recurrent disease 17 months following surgery for which he is presently on palliative chemotherapy. The other patient underwent adrenalectomy for adrenal metastasis 3 months following liver transplantation for hepatocellular carcinoma. He is presently alive and disease-free 27 months after adrenalectomy. CONCLUSION: Carefully selected patients with solitary metastasis from primary liver tumors may be considered for resection.

  19. Isolated hydatid cyst of adrenal gland with hypertension mimicking Conn's syndrome: a very rare case

    Directory of Open Access Journals (Sweden)

    Tarun Chaudhary

    2016-07-01

    Full Text Available Hydatid cyst of the adrenal gland is one of the rare conditions caused by the larval stage of Echinococcus granulosus. The incidence of adrenal gland involvement is less than 1% of all hydatid disease in humans and isolated adrenal involvement is extremely rare. Hydatid disease is frequent in endemic regions and sheep farming areas with equal sex distribution. Here, a case of 23 year old female with isolated adrenal gland hydatid cyst is presented, that was evaluated clinically, investigated radiographicaly and by blood investigations and finally histopathology confirmed the diagnosis. No complications occurred at peri and postoperative period. The patient was given 6 cycles of albendazole (10 mg/kg in two divided doses, each for a period of four weeks followed by a week's rest. The patient is on regular follow-up without recurrence in last 1 year. [Int J Res Med Sci 2016; 4(7.000: 3071-3073

  20. Analyses of the similarity and difference of global gene expression profiles in cortex regions of three neurodegenerative diseases: sporadic Creutzfeldt-Jakob disease (sCJD), fatal familial insomnia (FFI), and Alzheimer's disease (AD).

    Science.gov (United States)

    Tian, Chan; Liu, Di; Xiang, Wei; Kretzschmar, Hans A; Sun, Qing-Lan; Gao, Chen; Xu, Yin; Wang, Hui; Fan, Xue-Yu; Meng, Ge; Li, Wei; Dong, Xiao-Ping

    2014-10-01

    Neurodegenerative disease is a general designation for the disorders that are progressive loss of structure or function and final death of neurons, including Alzheimer's, Parkinson's, Huntington's, prion diseases, etc. In this study, we comparatively analyzed 21 individual microarray data sets of the cortex tissues from 11 sporadic Creutzfeldt-Jakob disease (sCJD), 3 fatal familial insomnia (FFI), 3 Alzheimer's disease (AD), and 4 normal controls. After normalization, a collection of 730 differently expressed sets (DESets) were obtained by comparison of the data of three diseases with their original controls. Principal component analysis (PCA) showed a background-related distribution within the groups of FFI, AD, and normal control, but two apparently different subgroups within the group of sCJD were observed. Review of the clinical materials of 11 sCJD patients identified the difference in brain PrP(Sc) deposits between two subgroups. Hierarchical cluster analysis illustrated the relatively independent clusters of normal controls, FFIs, six sCJD cases (subgroup 1) with more PrP(Sc) deposits, respectively, while an overlapped cluster of five cases of sCJD2 (subgroup 2) with less PrP(Sc) deposits and AD patients. Despite of the presence of special gene expressions, many common features were found among those neurodegenerative diseases. The most commonly changed biological processes (BPs) were signal transduction, synaptic transmission, and neuropeptide signaling pathway. The most commonly changed pathways were MAPK signaling pathway, Parkinson's disease, and oxidative phosphorylation. Our data here provide the similarity and difference in global gene expressions among the patients with sCJD, FFI, and AD, which may help to understand the common mechanism of neurodegenerative diseases.

  1. What Are Some Types of Adrenal Gland Disorders?

    Science.gov (United States)

    ... Adrenal Gland Tumors Most adrenal gland tumors—abnormal growths on the adrenal glands—are not cancerous. They often do not cause symptoms or require treatment. However, adrenal gland tumors can produce a variety of different hormones, leading hormone levels to get too high. Adrenal ...

  2. [Morphological studies of rat adrenal glands after space flight on "Kosmos-1667"].

    Science.gov (United States)

    Prodan, N G; Bara'nska, V

    1989-01-01

    Histological and histomorphometric examinations of rat adrenals after a 7-day flight revealed the following changes: blood congestion in the cortex and medulla, progressive delipoidization of the cortex, slight enlargement of the nuclear volume of glomerular and fascicular zones, vacuolization of the cytoplasm of medulla cells, reduction of the area of noradrenocyte islets and cell nuclei of the medulla; the adrenal weight remained however unchanged. It is concluded that an early period of adaptation to microgravity was accompanied by a weak stress-reaction. Upon return to Earth the rats developed an acute gravitational stress. From the morphological point of view the stress manifested as: increased volume of nuclei in fascicular cells, decreased content of lipids in them, and greater vacuolization of the cytoplasm of medulla cells. The lack of medulla hypertrophy, reduction of the area of noradrenocyte islets and nuclei of medulla cells suggest that 7-day exposure to microgravity did not exert of stimulating effect on the sympathetic system of rats.

  3. Intermittent Theta-Burst Stimulation of the Right Dorsolateral Prefrontal Cortex to Promote Metaphor Comprehension in Parkinson Disease: A Case Study.

    Science.gov (United States)

    Tremblay, Christina; Monetta, Laura; Langlois, Mélanie; Schneider, Cyril

    2016-01-01

    This single-case research-designed study explored whether intermittent theta-burst stimulation (iTBS) of the right dorsolateral prefrontal cortex (DLPFC) could improve metaphor comprehension in people with Parkinson disease (PD) and language impairments. A right-handed participant with PD diagnosed 9 years ago, receiving long-term treatment with levodopa, and with metaphor comprehension impairment was recruited to undergo 10 sessions of sham stimulation (in 2wk), a washout period (6wk), and then 10 sessions of iTBS (in 2wk). Clinical scores of metaphor comprehension and motor evaluation (Unified Parkinson Disease Rating Scale part III) and transcranial magnetic stimulation to test the excitability of the primary motor cortex (M1) were used at baseline, postsham, post-iTBS, and at 3 follow-ups (8, 14, and 20wk post-iTBS). Metaphor comprehension was improved after iTBS, and the highest scores were obtained 8 weeks later (P=.01). This improvement was correlated with the increase of the right M1 excitability (r=-.86, P=.03) and with the decrease of transcallosal inhibition latency from the left to the right hemisphere (r=-.88, P=.02). Sham yielded no effect (P>.05). Administration of iTBS over the right DLPFC improved metaphor comprehension likely by a long-term influence on brain synaptic plasticity, including improvement of interhemispheric dialogue. More studies are warranted to confirm these findings in larger samples of participants with PD.

  4. Evaluation of Iron Deposition in the Adrenal Glands of β Thalassemia Major Patients Using 3-Tesla MRI

    Directory of Open Access Journals (Sweden)

    Guzelbey

    2016-05-01

    Full Text Available Background Beta-thalassemia major (β-TM patients need blood transfusions, which result in iron deposition. To regulate chelation therapy, iron load has to be measured. With MRI, the amount of signal loss and T2* decay time shortening are used for iron quantification. Objectives The aim was to measure adrenal iron load with T2* relaxometry using MRI, and to compare it with liver and cardiac iron and serum ferritin, and to find out whether adrenal iron could be predicted from those parameters. Patients and Methods Between October 2014 and March 2015, MRI was performed in 21 patients with β-TM, recieving blood transfusions and chelation therapy. The control group (n = 11 included healthy volunteers with no known history of adrenal, hematologic, chronic disease, and blood transfusion. Results Among patients, there was no significant correlation between plasma ferritin and adrenal T2*. Significant difference was detected among T2* values of adrenals between the patient and control groups. There was no significant correlation between adrenal gland and liver T2* in β-TM patients, moderate correlation was detected between adrenal T2* and cardiac T2*. Conclusion Adrenal iron in β-TM can be reliably measured in 3 Tesla MRI. The results highlight the absence of correlation between adrenal iron deposition both with serum ferritin and hepatic iron.

  5. Congenital adrenal hyperplasia: Treatment and outcomes.

    Science.gov (United States)

    Kamoun, Mahdi; Feki, Mouna Mnif; Sfar, Mohamed Habib; Abid, Mohamed

    2013-10-01

    Congenital adrenal hyperplasia (CAH) describes a group of autosomal recessive disorders where there is impairment of cortisol biosynthesis. CAH due to 21-hydroxylase deficiency accounts for 95% of cases and shows a wide range of clinical severity. Glucocorticoid and mineralocorticoid replacement therapies are the mainstays of treatment of CAH. The optimal treatment for adults with CAH continues to be a challenge. Important long-term health issues for adults with CAH affect both men and women. These issues may either be due to the disease or to steroid treatment and may affect final height, fertility, cardiometabolic risk, bone metabolism, neuro-cognitive development and the quality-of-life. Patients with CAH should be regularly followed-up from childhood to adulthood by multidisciplinary teams who have knowledge of CAH. Optimal replacement therapy, close clinical and laboratory monitoring, early life-style interventions, early and regular fertility assessment and continuous psychological management are needed to improve outcome.

  6. Congenital adrenal hyperplasia: Treatment and outcomes

    Directory of Open Access Journals (Sweden)

    Mahdi Kamoun

    2013-01-01

    Full Text Available Congenital adrenal hyperplasia (CAH describes a group of autosomal recessive disorders where there is impairment of cortisol biosynthesis. CAH due to 21-hydroxylase deficiency accounts for 95% of cases and shows a wide range of clinical severity. Glucocorticoid and mineralocorticoid replacement therapies are the mainstays of treatment of CAH. The optimal treatment for adults with CAH continues to be a challenge. Important long-term health issues for adults with CAH affect both men and women. These issues may either be due to the disease or to steroid treatment and may affect final height, fertility, cardiometabolic risk, bone metabolism, neuro-cognitive development and the quality-of-life. Patients with CAH should be regularly followed-up from childhood to adulthood by multidisciplinary teams who have knowledge of CAH. Optimal replacement therapy, close clinical and laboratory monitoring, early life-style interventions, early and regular fertility assessment and continuous psychological management are needed to improve outcome.

  7. Papillary Thyroid Carcinoma Metastases Presenting as Ipsilateral Adrenal Mass and Renal Cyst

    Directory of Open Access Journals (Sweden)

    Serge Ginzburg

    2015-11-01

    Full Text Available Metastatic spread of differentiated thyroid cancer to genitourinary organs is rare. Synchronous presentation of renal and adrenal thyroid metastasis is even less common, this case being only the 3rd reported. We describe a case of a 60-year-old male with oligometastatic thyroid cancer, where adrenal and renal metastases were the only extracervical sites of disease and triggered the patient's presentation.

  8. Use of adrenal arterial embolization in severe ACTH-dependent Cushing's syndrome.

    OpenAIRE

    Blunt, S B; Pirmohamed, M.; Chatterjee, V K; Burrin, J. M.; Allison, D J; Joplin, G. F.

    1989-01-01

    The management of a patient with severe Cushing's syndrome due to ectopic ACTH produced by a medullary carcinoma of the thyroid is described. Initial treatment with maximal adrenolytic medical therapy and two attempts at bilateral adrenal venous infarction had failed to control the disease, and she was at that time unfit for surgery. Subsequent use of bilateral adrenal arterial embolization enabled medical therapy to produce sufficient control of the Cushing's syndrome to allow bilateral adre...

  9. Proteins in human brain cortex are modified by oxidation, glycoxidation, and lipoxidation. Effects of Alzheimer disease and identification of lipoxidation targets.

    Science.gov (United States)

    Pamplona, Reinald; Dalfó, Esther; Ayala, Victòria; Bellmunt, Maria Josep; Prat, Joan; Ferrer, Isidre; Portero-Otín, Manuel

    2005-06-03

    Diverse oxidative pathways, such as direct oxidation of amino acids, glycoxidation, and lipoxidation could contribute to Alzheimer disease pathogenesis. A global survey for the amount of structurally characterized probes for these reactions is lacking and could overcome the lack of specificity derived from measurement of 2,4-dinitrophenylhydrazine reactive carbonyls. Consequently we analyzed (i) the presence and concentrations of glutamic and aminoadipic semialdehydes, N(epsilon)-(carboxymethyl)-lysine, N(epsilon)-(carboxyethyl)-lysine, and N(epsilon)-(malondialdehyde)-lysine by means of gas chromatography/mass spectrometry, (ii) the biological response through expression of the receptor for advanced glycation end products, (iii) the fatty acid composition in brain samples from Alzheimer disease patients and age-matched controls, and (iv) the targets of N(epsilon)-(malondialdehyde)-lysine formation in brain cortex by proteomic techniques. Alzheimer disease was associated with significant, although heterogeneous, increases in the concentrations of all evaluated markers. Alzheimer disease samples presented increases in expression of the receptor for advanced glycation end products with high molecular heterogeneity. Samples from Alzheimer disease patients also showed content of docosahexaenoic acid, which increased lipid peroxidizability. In accordance, N(epsilon)-(malondialdehyde)-lysine formation targeted important proteins for both glial and neuronal homeostasis such as neurofilament L, alpha-tubulin, glial fibrillary acidic protein, ubiquinol-cytochrome c reductase complex protein I, and the beta chain of ATP synthase. These data support an important role for lipid peroxidation-derived protein modifications in Alzheimer disease pathogenesis.

  10. Large bilateral adrenal metastases in non-small cell lung cancer

    Directory of Open Access Journals (Sweden)

    Karanikiotis Charisios

    2004-11-01

    Full Text Available Abstract Background The adrenal gland is one of the common sites of metastasis from primary lung cancer. Adrenal metastases are usually unilateral however bilateral adrenal metastases are seen in 10% of all lung cancer patients; of these 2–3% occurs at the initial presentation of non-small cell lung cancer. Secondary tumors can disrupt the structure and function of the adrenal. This can lead to adrenal hemorrhage, which constitutes a life threatening hazard for the patient. Case presentation A 59-year-old male presented with persisting abdominal pain. His initial work-up revealed significant anemia, an invasive process in the right upper lobe of the lung and large masses of heterogeneous texture, with hemorrhagic and necrotic elements in both adrenal glands. A biopsy confirmed it to be a large-cell carcinoma of the lungs. The patient developed severe leukocytosis akin to the paraneoplastic syndrome and died suddenly five days after the administration of chemotherapy. Conclusion Intratumoral hemorrhage is a rare but life threatening complication of adrenal metastases and should be treated as soon as it has been diagnosed. If adrenalectomy is not feasible, combination chemotherapy should be applied as in metastatic disease. For choosing the appropriate chemotherapeutic regimen it is important to accurately achieve the diagnosis.

  11. X-linked congenital adrenal hypoplasia associated with hypospadias in an Egyptian baby: a case report

    Directory of Open Access Journals (Sweden)

    Metwalley Kotb

    2012-12-01

    Full Text Available Abstract Introduction X-linked congenital adrenal hypoplasia is a rare developmental disorder of the human adrenal cortex and is caused by deletion or mutation of the dosage-sensitive sex reversal adrenal hypoplasia congenita critical region of the X chromosome, gene 1 (DAX-1 gene. Most affected children present with failure to thrive, salt wasting and hypoglycemic convulsions in the first months of life. Hypospadias affects approximately one in 250 live male births. Mutations in the mastermind-like domain-containing 1 (MAMLD1 gene have been implicated as one of the causes of hypospadias in children. To the best of our knowledge, an association between congenital adrenal hypoplasia due to a DAX-1 mutation and hypospadias due to mutation of the MAMLD1 gene has not previously been reported in the literature. Case presentation A 35-day-old male Egyptian baby was referred to our institution for the evaluation of a two-week history of recurrent vomiting associated with electrolyte imbalance. On examination, our patient was found to have hypotension and dehydration. A genital examination showed distal penile hypospadias with chordee and normal testes. He had hyponatremia, hyperkalemia, hypoglycemia and metabolic acidosis. Endocrinological investigations revealed low levels of cortisol, 17-hydroxyprogesterone and aldosterone, with a high level of adrenocorticotrophic hormone. A provisional diagnosis of congenital adrenal hypoplasia associated with hypospadias was made. A molecular genetics study confirmed the diagnosis of X-linked congenital adrenal hypoplasia due to DAX-1 mutations and hypospadias due to MAMLD1 mutation. He was started on hydrocortisone and fludrocortisone treatment. After three weeks of treatment, his symptoms improved and his blood sugar, sodium, potassium and cortisol levels normalized. Conclusions We report the case of an Egyptian baby with an association of congenital adrenal hypoplasia due to DAX-1 mutation and hypospadias due

  12. Laparoscopic Management of a Complex Adrenal Cyst

    Directory of Open Access Journals (Sweden)

    Koichi Kodama

    2015-01-01

    Full Text Available Adrenal cysts are rare, and their clinical management remains controversial. We report a case involving an adrenal cyst with a complicated appearance on radiological studies. Unenhanced computed tomography revealed a unilocular, noncalcified, hypoattenuating mass with a thin wall in the left adrenal gland. The lesion gradually increased in size from 10 to 50 mm at two-year follow-up. On contrast-enhanced magnetic resonance imaging, a mural nodule with contrast enhancement was observed. The entire adrenal gland was excised en bloc via a lateral transperitoneal laparoscopic approach without violating the principles of surgical oncology. The pathological diagnosis was an adrenal pseudocyst. Laparoscopic adrenalectomy is a safe option for the treatment of complex adrenal cysts, while maintaining the benefits of minimal invasiveness.

  13. Laparoscopic Resection of an Adrenal Schwannoma

    Science.gov (United States)

    Konstantinos, Toutouzas G.; Panagiotis, Kekis B.; Nikolaos, Michalopoulos V.; Ioannis, Flessas; Andreas, Manouras; Geogrios, Zografos

    2012-01-01

    Background and Objectives: Schwannomas are tumors originating from Schwann cells of the peripheral nerve sheath (neurilemma) of the neuroectoderm. Rarely, schwannomas can arise from the retroperitoneum and adrenal medulla. We describe a case of a 71-y-old woman who presented with an incidentally discovered adrenal tumor. Methods: Ultrasound and computed tomography scans revealed a lesion with solid and cystic areas originating from the left adrenal gland. The patient underwent complete laparoscopic resection of the tumor and the left adrenal gland. Results: Histopathological examination and immunohistochemical staining of the excised specimen revealed a benign schwannoma measuring 5.5×5×3.7 cm. To our knowledge, few other cases of laparoscopic resection of adrenal schwannomas have been reported. Conclusion: Because preoperative diagnosis of adrenal tumors is inconclusive, complete laparoscopic excision allows for definitive diagnosis with histological evaluation and represents the treatment of choice. PMID:23484583

  14. Adrenal insufficiency in a child with MELAS syndrome.

    Science.gov (United States)

    Afroze, Bushra; Amjad, Nida; Ibrahim, Shahnaz H; Humayun, Khadija Nuzhat; Yakob, Yusnita

    2014-11-01

    Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) are established subgroups of mitochondrial encephalomyopathy. m.3243A>G a common point mutation is detected in tRNA in majority of patients with MELAS phenotype whereas m.8344A>G point mutation in tRNA is observed, in MERRF phenotype. Adrenal insufficiency has not been reported in mitochondrial disease, except in Kearns-Sayre Syndrome (KSS), which is a mitochondrial deletion syndrome. We report an unusual presentation in a five year old boy who presented with clinical phenotype of MELAS and was found to have m.8344A>G mutation in tRNA. Addison disease was identified due to hyperpigmentation of lips and gums present from early childhood. This is the first report describing adrenal insufficiency in a child with MELAS phenotype.

  15. 5-HT2A Receptor Binding in the Frontal Cortex of Parkinson's Disease Patients and Alpha-Synuclein Overexpressing Mice

    DEFF Research Database (Denmark)

    Rasmussen, Nadja Bredo; Olesen, Mikkel Vestergaard; Brudek, Tomasz;

    2016-01-01

    The receptor is highly involved in aspects of cognition and executive function and seen to be affected in neurodegenerative diseases like Alzheimer’s disease and related to the disease pathology. Even though Parkinson’s disease (PD) is primarily a motor disorder, reports of impaired executive fun...

  16. Paraganglioma funcional extra-adrenal

    Directory of Open Access Journals (Sweden)

    Laura Arroyo-Martínez

    2006-03-01

    Full Text Available Los paragangliomas funcionales son tumores raros, se originan del tejido cromafín extraadrenal productor de catecolaminas, con frecuencia son malignos y tienen alta incidencia de enfermedad persistente o recurrente¹. Se les conoce como: glomus, quemodectomas, paragangliomas cromafines y glomerulocitomas. La localización es diversa y refleja la distribución paraganglionar en el cuerpo, desde la base del cráneo hasta el piso pélvico. Los paragangliomas se encuentran en donde hay ganglios del sistema autónomo, sin embargo, aproximadamente el 90% de estos tumores aparecen en las glándulas suprarrenales (y constituyen los feocromocitomas y el 10% restante tienen una ubicación extraadrenal, mas se ha dicho que su incidencia puede ser subestimada, variando del 18% al 22% en adultos, y en niños hasta un 30%. Los extra-adrenales se originan con mayor frecuencia en el abdomen (85%, otros en el tórax (12% y más raramente en la cabeza y el cuello (3% ². Los estudios de imágenes y la medición de la producción no fisiológica de catecolaminas pueden ayudar en el diagnóstico de esta entidad. La cirugía es el tratamiento de elección. Presentamos aquí el caso de una paciente de 32 años, primigesta con HTAIE que requirió cesárea, quien tuvo un postparto tórpido y pese a múltiples tratamientos antihipertensivos su patología fue de difícil manejo, con complicaciones oftálmicas. Tiempo después la paciente se estudia por hiperhidrosis, se solicitan exámenes de laboratorio e imágenes y se le documenta incidentalmente, una tumoración retroperitoneal izquierda, se le amplían los estudios, y se llega al diagnóstico correcto. La tumoración requirió resección quirúrgica. Tuvo un postoperatorio satisfactorio y la paciente egresó con control en la Consulta Externa.Functioning paragangliomas are rare tumors that produce catecholamines. They originate from extra-adrenal chromaffin cells. They are frequentIy malignant and are associated

  17. Adrenal Failure due to Adrenal Metastasis of Lung Cancer: A Case Report

    Science.gov (United States)

    Faulhaber, Gustavo Adolpho Moreira; Borges, Flavia Kessler; Ascoli, Aline Maria; Seligman, Renato; Furlanetto, Tania Weber

    2011-01-01

    We report a case of a patient with adrenal failure due to bilateral adrenal metastasis of lung cancer. This is a rare presentation of lung cancer. We review the differential diagnosis of weight loss and how to make diagnosis of adrenal insufficiency. PMID:22606443

  18. Adrenal Failure due to Adrenal Metastasis of Lung Cancer: A Case Report

    Directory of Open Access Journals (Sweden)

    Gustavo Adolpho Moreira Faulhaber

    2011-01-01

    Full Text Available We report a case of a patient with adrenal failure due to bilateral adrenal metastasis of lung cancer. This is a rare presentation of lung cancer. We review the differential diagnosis of weight loss and how to make diagnosis of adrenal insufficiency.

  19. Adrenal Myelolipoma- A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Vijayalaxmi V. Suranagi

    2009-05-01

    Full Text Available Adrenal myelolipoma (AML is a rare benign tumour composed of mature adipose tissue and hematopoietic tissue. Very few cases have been reported. Most of these patients are asymptomatic. We present a rare case of Adrenal Myelolipoma where the patient presented with hypertension and a clinical suspicion of Pheochromocytoma, which turned out to be an Adrenal myelolipoma. Adrenal myelolipoma is a rare entity, not encountered frequently and can occur as an incidental finding. Awareness regarding this entity is very much essential to exclude surgical exploration or extensive surgery.

  20. Genetics Home Reference: X-linked adrenal hypoplasia congenita

    Science.gov (United States)

    ... X-linked adrenal hypoplasia congenita X-linked adrenal hypoplasia congenita Enable Javascript to view the expand/collapse ... Open All Close All Description X-linked adrenal hypoplasia congenita is a disorder that mainly affects males. ...

  1. 5-HT2A Receptor Binding in the Frontal Cortex of Parkinson's Disease Patients and Alpha-Synuclein Overexpressing Mice

    DEFF Research Database (Denmark)

    Rasmussen, Nadja Bredo; Olesen, Mikkel Vestergaard; Brudek, Tomasz;

    2016-01-01

    The 5-HT2A receptor is highly involved in aspects of cognition and executive function and seen to be affected in neurodegenerative diseases like Alzheimer's disease and related to the disease pathology. Even though Parkinson's disease (PD) is primarily a motor disorder, reports of impaired...... executive function are also steadily being associated with this disease. Not much is known about the pathophysiology behind this. The aim of this study was thereby twofold: (1) to investigate 5-HT2A receptor binding levels in Parkinson's brains and (2) to investigate whether PD associated pathology, alpha...

  2. Emotional exhaustion and overcommitment to work are differentially associated with hypothalamus-pituitary-adrenal (HPA) axis responses to a low-dose ACTH1-24 (Synacthen) and dexamethasone-CRH test in healthy school teachers.

    Science.gov (United States)

    Wolfram, Maren; Bellingrath, Silja; Feuerhahn, Nicolas; Kudielka, Brigitte M

    2013-01-01

    Evidence for a detrimental impact of chronic work stress on health has accumulated in epidemiological research. Recent studies indicate altered hypothalamus-pituitary-adrenal (HPA) axis regulation as a possible biological pathway underlying the link between stress and disease. However, the direction of dysregulation remains unclear, with reported HPA hyper- or hyporeactivity. To disentangle potential effects on different functional levels in the HPA axis, we examined responses using two pharmacological stimulation tests in 53 healthy teachers (31 females, 22 males; mean age: 49.3 years; age range: 30-64 years): a low-dose adrenocorticotrophic hormone (ACTH(1-24), Synacthen) test was used to assess adrenal cortex sensitivity and the combined dexamethasone-corticotropin releasing hormone (DEX-CRH) test to examine pituitary and adrenal cortex reactivity. Blood and saliva samples were collected at - 1,+15,+30,+45,+60,+90,+120 min. Emotional exhaustion (EE), the core dimension of burnout, was measured with the Maslach Burnout Inventory. Overcommitment (OC) was assessed according to Siegrist's effort-reward-imbalance model. We found a significant association between EE and higher plasma cortisol profiles after Synacthen (p = 0.045). By contrast, OC was significantly associated with attenuated ACTH (p = 0.045), plasma cortisol (p = 0.005), and salivary cortisol (p = 0.023) concentrations following DEX-CRH. Results support the notion of altered HPA axis regulation in chronically work-stressed teachers, with differential patterns of hyper- and hyporeactivity depending on individual stress condition and the tested functional level of the HPA axis.

  3. Adrenal haemorrhage with cholestasis and adrenal crisis in a newborn of a diabetic mother.

    Science.gov (United States)

    Koklu, Esad; Kurtoglu, Selim; Akcakus, Mustafa; Koklu, Selmin

    2007-03-01

    The large hyperaemic foetal adrenal gland is vulnerable to vascular damage. This may occur in the neonatal period as a consequence of difficult labour, or its aetiology may not be apparent. The spectrum of presentation is considerable, ranging from asymptomatic to severe life-threatening intra-abdominal haemorrhage. The presentation of adrenal insufficiency may be delayed but the regenerative capacity of the adrenal is great, and most adrenal haemorrhage is not associated with significantly impaired function. Some reports showed that cholestatic hepatopathy with congenital hypopituitarism reversed by hydrocortisone treatment is considered in the context of the endocrine syndrome, probably as a consequence of the adrenal failure. We describe a case of bilateral adrenal haemorrhage with hepatitis syndrome and persistent hypoglycaemia in a newborn male with striking features of neonatal cholestasis and adrenal crisis.

  4. Puberty and fertility in congenital adrenal hyperplasia.

    NARCIS (Netherlands)

    Otten, B.J.; Stikkelbroeck, M.M.L.; Claahsen-van der Grinten, H.L.; Hermus, A.R.M.M.

    2005-01-01

    Congenital adrenal hyperplasia (CAH) is a disorder of adrenal steroid synthesis. The symptoms and signs of CAH depend on the degree of enzyme deficiency; severe salt-wasting (SW) form, less severe simple virilizing (SV) form and mild nonclassic (NC) form. In this paper, puberty and fertility in CAH

  5. Severe Hyperkalemia and Bilateral Adrenal Metastasis

    Directory of Open Access Journals (Sweden)

    Michael Nagler

    2009-01-01

    Full Text Available Adrenal metastases are a common finding in metastatic lung and breast cancer. Often there are no clinical symptoms suggesting them. In this paper, we present a case of a 66-year-old man with metastatic lung cancer suffering from severe hyperkaliemia due to hypoaldosteronism as a result of bilateral adrenal metastasis.

  6. Quantitative and qualitative evaluation of CART-containing cells in adrenal glands of male rats with hypertension.

    Science.gov (United States)

    Kasacka, I; Piotrowska, Ż; Knaś, M; Lewandowska, A

    2014-10-01

    Adrenal activity is stimulated and secretion of stress hormones is increased during advanced stages of renovascular hypertension. The literature suggests that the neuropeptide, cocaine and amphetamine regulated transcript (CART), might regulate adrenal secretory function and thus could influence its activity. We assessed potential quantitative and qualitative changes in the cells that contained CART in the adrenal glands of rats with renovascular hypertension. The renal arteries of ten rats were subjected to a clipping procedure, i.e., two-kidney one-clip (2K1C) model of arterial hypertension, and after 6 weeks each rat developed stable hypertension. CART was localized using immunohistochemistry. CART was detected in a large population of cells in the medulla, sparse nerve fibers in the cortex and the capsule of the adrenal gland. The population of CART-positive cells in adrenal glands of two kidney-one clip (2K1C) treated rats was greater and their immunoreactivity was increased compared to controls. Similarly, the length, width, area and diameter of CART-immunoreactive cells were significantly greater in the hypertensive rats than in controls. We demonstrated that renovascular hypertension alters the number and immunoreactivity of CART-containing cells in adrenal glands.

  7. Development of CYP11B1 and CYP11B2 assays utilizing homogenates of adrenal glands: Utility of monkey as a surrogate for human.

    Science.gov (United States)

    Cerny, Matthew A; Csengery, Alexander; Schmenk, Jennifer; Frederick, Kosea

    2015-11-01

    Elevated levels of aldosterone are associated with arterial hypertension, congestive heart failure, chronic kidney disease, and obesity. Aldosterone is produced predominantly in the zona glomerulosa of the cortex of the adrenal gland by the enzyme aldosterone synthase (CYP11B2). Treatment of the above indications by decreasing production of aldosterone is thought to be of therapeutic benefit by lessening the deleterious effects of aldosterone mediated through both the mineralocorticoid receptor and also through so called non-genomic pathways. However, inhibition of the highly similar enzyme, CYP11B1, which is responsible for the production of cortisol, must be avoided in the development of clinically useful aldosterone synthase inhibitors due to the resulting impairment of the cortisol-induced stress response. In efforts to assess the interactions of compounds with the CYP11B enzymes, a variety of cell-based inhibitor screening assays for both CYP11B1 and CYP11B2 have been reported. Herein we report details of assays employing both cynomolgus monkey adrenal homogenate (CAH) and human adrenal homogenate (HAH) as sources of CYP11B1 and CYP11B2 enzymes. Utilizing both CAH and HAH, we have characterized the kinetics of the CYP11B1-mediated conversion of 11-deoxycortisol to cortisol and the CYP11B2-mediated oxidation of corticosterone to aldosterone. Inhibition assays for both CYP11B1 and CYP11B2 were subsequently developed. Based on a comparison of human and monkey amino acid sequences, kinetics data, and inhibition values derived from the HAH and CAH assays, evidence is provided in support of using cynomolgus monkey tissue-derived cell homogenates as suitable surrogates for the human enzymes.

  8. A Case of Long-term Survival after Curative Resection for Synchronous Solitary Adrenal Metastasis from Rectal Cancer.

    Science.gov (United States)

    Chen, Linlin; Wang, Da; Mao, Weifang; Huang, Xuefeng; He, Chao

    2014-01-01

    Clinically curable adrenal metastasis is rare. We report a case of synchronous solitary adrenal metastasis from rectal cancer in a 51-year-old man who underwent curative resection. A right adrenal mass was found by ultrasonography during his routine physical examination and this was confirmed by computed tomography (CT). His serum carcinoembryonic antigen (CEA) level was found elevated, and colonoscopy revealed a rectal tumor located 10cm from anal verge. A simultaneous laparoscopic right adrenalectomy and anterior resection for rectal carcinoma was performed. Histopathological examination revealed well-differentiated rectal adenocarcinoma with adrenal metastasis. The patient is still alive and free from disease 6 years after the surgery. A review in the literature showed that synchronous solitary adrenal metastasis from colorectal carcinoma is very rare. Surgical resection and for selected patients, laparoscopic procedure may provide survival benefit and potential surgical cure for a solitary metastasis.

  9. Diagnosis and management of adrenal insufficiency.

    Science.gov (United States)

    Bancos, Irina; Hahner, Stefanie; Tomlinson, Jeremy; Arlt, Wiebke

    2015-03-01

    Adrenal insufficiency continues to be a challenge for patients, their physicians, and researchers. During the past decade, long-term studies have shown increased mortality and morbidity and impaired quality of life in patients with adrenal insufficiency. These findings might, at least partially, be due to the failure of glucocorticoid replacement therapy to closely resemble physiological diurnal secretion of cortisol. The potential effect of newly developed glucocorticoid drugs is a focus of research, as are the mechanisms potentially underlying increased morbidity and mortality. Adrenal crisis remains a threat to lives, and awareness and preventative measures now receive increasing attention. Awareness should be raised in medical teams and patients about adrenal insufficiency and management of adrenal crisis to improve clinical outcome.

  10. Bone mineral status in children with congenital adrenal hyperplasia.

    Science.gov (United States)

    Fleischman, Amy; Ringelheim, Julie; Feldman, Henry A; Gordon, Catherine M

    2007-02-01

    Congenital adrenal hyperplasia (CAH) is caused by a deficiency in an adrenal enzyme resulting in alterations in cortisol and aldosterone production. Bone status is affected by chronic glucocorticoid therapy and excess androgen exposure in children with CAH. This cross-sectional study enrolled participants with 21-hydroxylase deficiency from a pediatric referral center. Bone mineral density in the participants was normal when compared to age, gender and ethnicity adjusted standards, with respect to chronological age or bone age. Lean body mass was positively correlated with bone mineral content (BMC), independent of fat mass (p bone status may be explained by the differential effects of glucocorticoids on growing bone, beneficial androgen effects, or other disease specific factors.

  11. Role of DAX-1 (NR0B1) and steroidogenic factor-1 (NR5A1) in human adrenal function.

    Science.gov (United States)

    El-Khairi, Ranna; Martinez-Aguayo, Alejandro; Ferraz-de-Souza, Bruno; Lin, Lin; Achermann, John C

    2011-01-01

    The nuclear receptor transcription factors DAX-1 (NR0B1) and SF-1 (NR5A1) regulate many aspects of adrenal and reproductive development and function. Disruption of the genes encoding these factors can be associated with pediatric adrenal disease. DAX-1 mutations are classically associated with X-linked adrenal hypoplasia congenita, hypogonadotropic hypogonadism and impaired spermatogenesis. However, other phenotypes are also being reported, such as isolated mineralocorticoid insufficiency, premature sexual development, primary adrenal insufficiency in a 46, XX patient and late-onset X-linked adrenal hypoplasia congenita and/or hypogonadotropic hypogonadism. SF-1 mutations have also been associated with primary adrenal insufficiency, together with 46, XY disorders of sex development. However it is emerging that SF-1 changes are a relatively rare cause of primary adrenal failure in humans, and most individuals with SF-1 mutations have a spectrum of 46, XY disorders of sex development phenotypes. These conditions range from 46, XY females with streak gonads and müllerian structures, through children with ambiguous genitalia and inguinal testes, to severe penoscrotal hypospadias with undescended testes. Therefore, the human gonad appears to be more sensitive than the adrenal gland to loss of SF-1 function. This review will focus on the expanding range of phenotypes associated with DAX-1 and SF-1 mutations.

  12. Leucine-rich α2-glycoprotein is a novel biomarker of neurodegenerative disease in human cerebrospinal fluid and causes neurodegeneration in mouse cerebral cortex.

    Directory of Open Access Journals (Sweden)

    Masakazu Miyajima

    Full Text Available Leucine-rich α2-glycoprotein (LRG is a protein induced by inflammation. It contains a leucine-rich repeat (LRR structure and easily binds with other molecules. However, the function of LRG in the brain during aging and neurodegenerative diseases has not been investigated. Here, we measured human LRG (hLRG concentration in the cerebrospinal fluid (CSF and observed hLRG expression in post-mortem human cerebral cortex. We then generated transgenic (Tg mice that over-expressed mouse LRG (mLRG in the brain to examine the effects of mLRG accumulation. Finally, we examined protein-protein interactions using a protein microarray method to screen proteins with a high affinity for hLRG. The CSF concentration of hLRG increases with age and is significantly higher in patients with Parkinson's disease with dementia (PDD and progressive supranuclear palsy (PSP than in healthy elderly people, idiopathic normal pressure hydrocephalus (iNPH patients, and individuals with Alzheimer's disease (AD. Tg mice exhibited neuronal degeneration and neuronal decline. Accumulation of LRG in the brains of PDD and PSP patients is not a primary etiological factor, but it is thought to be one of the causes of neurodegeneration. It is anticipated that hLRG CSF levels will be a useful biomarker for the early diagnosis of PDD and PSP.

  13. Early Alzheimer's disease-type pathology in the frontal cortex of wild mountain gorillas (Gorilla beringei beringei).

    Science.gov (United States)

    Perez, Sylvia E; Sherwood, Chet C; Cranfield, Michael R; Erwin, Joseph M; Mudakikwa, Antoine; Hof, Patrick R; Mufson, Elliott J

    2016-03-01

    Amyloid beta (Aβ) and tau pathology have been described in the brains of captive aged great apes, but the natural progression of these age-related pathologies from wild great apes, including the gorilla, is unknown. In our previous study of Western lowland gorillas (Gorilla gorilla gorilla) who were housed in American Zoos and Aquariums-accredited facilities, we found an age-related increase in Aβ-positive plaques and vasculature, tau-positive astrocytes, oligodendrocyte coiled bodies, and neuritic clusters in the neocortex as well as hippocampus in older animals. Here, we demonstrate that aged wild mountain gorillas (Gorilla beringei beringei), who spent their entire lives in their natural habitat, also display an age-related increase in amyloid precursor protein (APP) and/or Aβ-immunoreactive blood vessels and plaques, but very limited tau pathology, in the frontal cortex. These results indicate that Aβ and tau lesions are age-related events that occur in the brain of gorillas living in captivity and in the wild.

  14. An ectopic ACTH-producing small cell lung carcinoma associated with enhanced corticosteroid biosynthesis in the peritumoral areas of adrenal metastasis.

    Science.gov (United States)

    Satoh, Hironori; Saito, Ryoko; Hisata, Shu; Shiihara, Jun; Taniuchi, Shinji; Nakamura, Yasuhiro; Nukiwa, Toshihiro; Ebina, Masahito; Sasano, Hironobu

    2012-06-01

    A 60-year-old Japanese male presented with swelling of bilateral cervical lymph nodes was subsequently diagnosed as the late stage of primary small cell lung carcinoma (SCLC). He was then treated with cisplatin and irinotecan as first-line chemotherapy, but hypokalemia with muscle weakness of the bilateral legs became gradually noticeable following two months of effective chemotherapy. A computed tomography (CT) scan revealed enlargement of bilateral adrenal glands and abdominal and mediastinal lymph nodes, though primary lung tumor remained the same in size. An ectopic ACTH-producing syndrome (EAS) was subsequently revealed by the following endocrinological studies. Hypokalemia was clinically improved by the treatment with metyrapone and the second-line chemotherapy with amrubicin for SCLC was started, but the patient died 12 days after the second-line chemotherapy. Post-mortem examination revealed ACTH immunoreactivity in tumor cells of all the metastatic lesions. Non-neoplastic adrenal cortex demonstrated hyperplasia associated with lipid depletion and marked expression of steroidogenic enzymes, especially in cortical cells around tumor infiltration, suggestive of paracrine ACTH stimulation of cortisol production. This is the first report evaluating expression of steroidogenic enzymes in adrenal cortex especially adjacent to the adrenal metastasis in the patients with EAS due to SCLC. These findings suggest that ACTH producing adrenal metastasis can induce EAS more frequently and severely, and that the symptoms and examination of EAS should be monitored carefully in the patients with adrenal metastasis of SCLC.

  15. Clinical features of congenital adrenal insufficiency including growth patterns and significance of ACTH stimulation test.

    Science.gov (United States)

    Koh, Ji Won; Kim, Gu Hwan; Yoo, Han Wook; Yu, Jeesuk

    2013-11-01

    Congenital adrenal insufficiency is caused by specific genetic mutations. Early suspicion and definite diagnosis are crucial because the disease can precipitate a life-threatening hypovolemic shock without prompt treatment. This study was designed to understand the clinical manifestations including growth patterns and to find the usefulness of ACTH stimulation test. Sixteen patients with confirmed genotyping were subdivided into three groups according to the genetic study results: congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH, n=11), congenital lipoid adrenal hyperplasia (n=3) and X-linked adrenal hypoplasia congenita (n=2). Bone age advancement was prominent in patients with CAH especially after 60 months of chronologic age (n=6, 67%). They were diagnosed in older ages in group with bone age advancement (Pcongenital adrenal insufficiency. ACTH stimulation test played an important role to support the diagnosis and serum 17-hydroxyprogesterone levels were significantly elevated in all of the CAH patients. The test will be important for monitoring growth and puberty during follow up of patients with congenital adrenal insufficiency.

  16. [Parasitism of the vena cava and adrenal veins in chronic chagasic patients].

    Science.gov (United States)

    Teixeira, V de P; Almeida, H de O; dos Reis, M A; Silveira, S A; Araujo, M B

    1989-01-01

    The occurrence of nest of trypanosoma cruzi in the smooth muscle cells of the central veins of the adrenal glands and the inferior vena cava was studied in 21 chronic Chagas' patients, by serial sections. Nine patients presented parasites in the adrenal veins, with a total of 24 nests, and only one nest was found in the inferior vena cava. The area of muscular tissue examined was of 2944.3 mm2 for the adrenal veins and of 47808.6 mm2 for the vena cava, giving a relation of 0.815 nests/100 mm2 for the adrenal vein and of 0.002 for the vena cava. Thus the smooth muscle of the adrenal vein was 407 times more parasited than the muscular wall of the inferior vena cava. This higher parasitism of the adrenal vein by trypanosoma cruzi in chronic Chagas' disease may be determined by its greater corticosteroid concentration, which can reduce the anti-trypanosomal defenses.

  17. [Adrenal tumors as a cause of hypertension].

    Science.gov (United States)

    Grimaldi, Franco

    2010-01-01

    Hypertension is one of the main risk factors for cardiovascular diseases, which today are the primary cause of mortality in developed countries. To decrease the mortality and morbidity due to cardiovascular disease, it is necessary to treat hypertension correctly on the basis of an accurate diagnosis. A secondary cause of hypertension must be suspected if it is severe or resistant to treatment, if there is a sudden increase in blood pressure in a patient hitherto well controlled, or if it develops in childhood or in a person under 30 years of age, especially if not obese and without a family history of hypertension. In these instances the frequency of hypertension varies from 1-5% for mild to moderate to 10-20% for severe or refractory hypertension. One of the principal causes of secondary hypertension is related to adrenal gland tumors or dysfunctions such as Cushing's syndrome, primary aldosteronism (Conn's syndrome) and pheochromocytoma. This paper will discuss the clinical presentation and diagnostic tests pertinent to these neoplasms, organ damage assessment, utilization of imaging techniques, and the medical and surgical options.

  18. 以Addison’s病首诊的肾上腺淋巴瘤临床特点分析--附5例报告并文献复习%Clinical features of adrenal lymphoma presenting as Addison’s disease:5 case report and literature review

    Institute of Scientific and Technical Information of China (English)

    谷秀莲; 王先令; 吕朝晖; 巴建明; 母义明; 陆菊明; 李江源; 潘长玉; 谷伟军; 窦京涛; 杨国庆; 杜锦; 陈康; 臧丽; 金楠; 杨丽娟

    2015-01-01

    目的:肾上腺淋巴瘤在临床上少见,以Addison’s病首诊者更少见,本文拟探讨以Addison’s病首诊的肾上腺淋巴瘤临床特征、诊治要点及预后,以提高临床医师对其认识。方法回顾性地分析解放军总医院1995年至2014年间收治的以Addison’s病首诊并经病理确诊的5例肾上腺淋巴瘤患者病例资料,统计其症状、体征、病程、肿瘤大小、病理类型及实验室检查结果,并结合国内外文献进行分析。结果5例均为老年患者,年龄(66.4±6.6)岁,男女比例为4∶1。5例患者均有典型Addison’s病的表现,如皮肤变黑、消瘦、乏力、食欲减退。均诊断为原发性肾上腺非霍奇金淋巴瘤,病理类型为B细胞来源,4例明确为弥漫大B细胞型。术后给予R-CHOP方案化疗,其中2例目前健在,发病至今已分别存活了10个月和20个月,随访效果尚可,1例存活3年,余2例存活时间均不足1年。结论以Addison’s病首诊的肾上腺淋巴瘤多发生于老年男性患者,双侧肾上腺受累多见,确诊需依赖穿刺病理诊断,病理类型主要为弥漫大B细胞型,预后较差。%Objective Adrenal lymphoma is a rare entity in clinical practice, especially when presenting as Addison’s disease. Hence, we summarized the clinical features and outcomes of this rare disease in order to raise awareness in clinicians. Methods Clinical data of 5 cases of adrenal lymphoma admitted to our hospital from 1995 to 2014 were retrospectively analyzed. All of them were firstly diagnosed as Addison’s disease and then identified as adrenal lymphoma according to the results of pathology. Their signs, symptoms, tumor size, pathological types and results of laboratory examinations were analyzed, considering literatures review. Results The 5 patients were at an age of (66.4±6.6) years, with a male-to-female ratio of 4∶1. All of them manifested as typical signs and symptoms of

  19. Amylin Treatment Reduces Neuroinflammation and Ameliorates Abnormal Patterns of Gene Expression in the Cerebral Cortex of an Alzheimer’s Disease Mouse Model

    Science.gov (United States)

    Wang, Erming; Zhu, Haihao; Wang, Xiaofan; Gower, Adam C.; Wallack, Max; Blusztajn, Jan Krzysztof; Kowall, Neil; Qiu, Wei Qiao

    2017-01-01

    Our recent study has demonstrated that peripheral amylin treatment reduces the amyloid pathology in the brain of Alzheimer’s disease (AD) mouse models, and improves their learning and memory. We hypothesized that the beneficial effects of amylin for AD was beyond reducing the amyloids in the brain, and have now directly tested the actions of amylin on other aspects of AD pathogenesis, especially neuroinflammation. A 10-week course of peripheral amylin treatment significantly reduced levels of cerebral inflammation markers, Cd68 and Iba1, in amyloid precursor protein (APP) transgenic mice. Mechanistic studies indicated the protective effect of amylin required interaction with its cognate receptor because silencing the amylin receptor expression blocked the amylin effect on Cd68 in microglia. Using weighted gene co-expression network analysis, we discovered that amylin treatment influenced two gene modules linked with amyloid pathology: 1) a module related to proinflammation and transport/vesicle process that included a hub gene of Cd68, and 2) a module related to mitochondria function that included a hub gene of Atp5b. Amylin treatment restored the expression of most genes in the APP cortex toward levels observed in the wild-type (WT) cortex in these two modules including Cd68 and Atp5b. Using a human dataset, we found that the expression levels of Cd68 and Atp5b were significantly correlated with the neurofibrillary tangle burden in the AD brain and with their cognition. These data suggest that amylin acts on the pathological cascade in animal models of AD, and further supports the therapeutic potential of amylin-type peptides for AD. PMID:27911303

  20. Task-related activity in sensorimotor cortex in Parkinson’s disease and essential tremor: changes in beta and gamma bands

    Directory of Open Access Journals (Sweden)

    Nathan C Rowland

    2015-09-01

    Full Text Available In Parkinson’s disease patients in the OFF medication state, basal ganglia local field potentials exhibit changes in beta and gamma oscillations that correlate with reduced voluntary movement, manifested as rigidity and akinesia. However, magnetoencephalography and low-resolution electrocorticography (ECoG studies in Parkinson’s patients suggest that changes in sensorimotor cortical oscillations differ from those of the basal ganglia. To more clearly define the role of sensorimotor cortex oscillatory activity in Parkinson’s, we performed intraoperative, high-resolution (4 mm spacing ECoG recordings in 10 Parkinson’s patients (2 females, ages 47-72 undergoing deep brain stimulation (DBS lead placement in the awake, OFF medication state. We analyzed ECoG potentials during a computer-controlled reaching task designed to separate movement preparation from movement execution and compared findings to similar invasive recordings in eight patients with essential tremor (3 females, ages 59-78, a condition not associated with rigidity or akinesia. We show that 1 cortical beta spectral power at rest does not differ between Parkinson’s and essential tremor patients (p = 0.85, 2 early motor preparation in Parkinson’s patients in the OFF medication state is associated with a larger beta desynchronization compared to patients with essential tremor (p = 0.0061, and 3 cortical broadband gamma power is elevated in Parkinson’s patients compared to essential tremor patients during both rest and task recordings (p = 0.004. Our findings suggest an oscillatory profile in sensorimotor cortex of Parkinson’s patients that, in contrast to the basal ganglia, may act to promote movement to oppose the anti-kinetic bias of the dopamine-depleted state.

  1. Distinct transcriptome expression of the temporal cortex of the primate Microcebus murinus during brain aging versus Alzheimer's disease-like pathology.

    Directory of Open Access Journals (Sweden)

    Ronza Abdel Rassoul

    Full Text Available Aging is the primary risk factor of neurodegenerative disorders such as Alzheimer's disease (AD. However, the molecular events occurring during brain aging are extremely complex and still largely unknown. For a better understanding of these age-associated modifications, animal models as close as possible to humans are needed. We thus analyzed the transcriptome of the temporal cortex of the primate Microcebus murinus using human oligonucleotide microarrays (Affymetrix. Gene expression profiles were assessed in the temporal cortex of 6 young adults, 10 healthy old animals and 2 old, "AD-like" animals that presented ß-amyloid plaques and cortical atrophy, which are pathognomonic signs of AD in humans. Gene expression data of the 14,911 genes that were detected in at least 3 samples were analyzed. By SAM (significance analysis of microarrays, we identified 47 genes that discriminated young from healthy old and "AD-like" animals. These findings were confirmed by principal component analysis (PCA. ANOVA of the expression data from the three groups identified 695 genes (including the 47 genes previously identified by SAM and PCA with significant changes of expression in old and "AD-like" in comparison to young animals. About one third of these genes showed similar changes of expression in healthy aging and in "AD-like" animals, whereas more than two thirds showed opposite changes in these two groups in comparison to young animals. Hierarchical clustering analysis of the 695 markers indicated that each group had distinct expression profiles which characterized each group, especially the "AD-like" group. Functional categorization showed that most of the genes that were up-regulated in healthy old animals and down-regulated in "AD-like" animals belonged to metabolic pathways, particularly protein synthesis. These data suggest the existence of compensatory mechanisms during physiological brain aging that disappear in "AD-like" animals. These results open

  2. Effects of adrenal steroids on the bone metabolism of children with congenital adrenal hyperplasia.

    Science.gov (United States)

    Lin-Su, Karen; New, Maria I

    2007-11-01

    The primary treatment for patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is glucocorticoid replacement therapy, which at supraphysiologic levels can result in diminished bone accrual and lead to osteopenia and osteoporosis. Unlike other diseases treated with chronic glucocorticoid therapy, previous studies of patients with 21OHD have not demonstrated a detrimental effect of glucocorticoid treatment on bone mineral density (BMD). It has been postulated that the elevated androgens typically found in these patients have a protective effect on bone integrity, but the precise mechanism remains unknown. We propose that the inhibitory effect of corticosteroid therapy on bone formation is counteracted by estrogen's effect on bone resorption through the RANK-L/osteoprotegerin (OPG) system. A better understanding of the mechanism by which patients with 21OHD are protected against bone loss may lead to novel therapeutic measures to prevent or treat osteopenia and osteoporosis in other conditions, including postmenopausal women.

  3. Silent Intravascular Lymphoma Initially Manifesting as a Unilateral Adrenal Incidentaloma

    Directory of Open Access Journals (Sweden)

    Yoshiko Takahashi

    2012-01-01

    Full Text Available Intravascular large B-cell lymphoma (IVLBCL is a rare subtype of malignant lymphoma. Although the involvement of adrenal glands in IVLBCL is often observed, primary adrenal IVLBCL is rare. Most reported cases of adrenal IVLBCL showed bilateral lesions resulting in rapidly progressive adrenal failure and poor prognosis. Here, we report a case of slowly progressive primary adrenal IVLBCL manifesting initially with unilateral adrenal incidentaloma. This case is a silent IVLBCL and shows that the enlargement of both adrenal glands can be followed.

  4. Adrenal Collision Tumor: Coexistence of Pigmented Adrenal Cortical Oncocytoma and Ganglioneuroma

    Directory of Open Access Journals (Sweden)

    Hye Seung Lee

    2016-01-01

    Full Text Available Background. Adrenal collision tumors (ACTs, in which distinct tumors coexist without intermingling in the same adrenal gland, are rare and their actual prevalence is unknown. ACTs commonly consist of adrenal cortical adenoma, pheochromocytoma, or metastatic malignant tumor. Case Report. A 32-year-old woman who had been experiencing gastric discomfort for one month was referred to our hospital with abnormal imaging findings. The physical examination and the laboratory data including endocrine studies were unremarkable. Abdomen computed tomography (CT and magnetic resonance imaging (MRI showed two adjacent masses in the left suprarenal fossa, and a laparoscopic left adrenalectomy was done. Histological and immunohistochemical (IHC examinations revealed two distinct tumors: a pigmented adrenal cortical oncocytoma (ACO and a ganglioneuroma, respectively. Conclusion. Both tumors are rare in the adrenal gland and exist as ACTs only exceptionally rarely. This is the first reported case of coexisting oncocytoma and ganglioneuroma in the same adrenal gland to our knowledge.

  5. Lipid peroxidation in adrenal and testicular microsomes.

    OpenAIRE

    1981-01-01

    Studies were carried out to determine the actions of and interactions between ascorbate, NADPH, Fe2+, and Fe3+ on lipid peroxidation in adrenal and testicular microsomes. Ascorbate-induced malonaldehyde production was maximal in adrenal and testicular microsomes at an ascorbate concentration of 1 X 10(-4)M. Fe2+, at levels between 10(-6) and 10(-3)M, produced concentration-dependent increases in lipid peroxidation in adrenal and testicular microsomes; Fe2+ had a far greater effect than Fe3+ i...

  6. Effects of Erythropoietin Administration on Adrenal Glands of Landrace/Large White Pigs after Ventricular Fibrillation.

    Science.gov (United States)

    Faa, Armando; Faa, Gavino; Papalois, Apostolos; Obinu, Eleonora; Locci, Giorgia; Pais, Maria Elena; Lelovas, Pavlos; Barouxis, Dimitrios; Pantazopoulos, Charalampos; Vasileiou, Panagiotis V; Iacovidou, Nicoletta; Xanthos, Theodoros

    2016-01-01

    Aim. To evaluate the effects of erythropoietin administration on the adrenal glands in a swine model of ventricular fibrillation and resuscitation. Methods. Ventricular fibrillation was induced via pacing wire forwarded into the right ventricle in 20 female Landrace/Large White pigs, allocated into 2 groups: experimental group treated with bolus dose of erythropoietin (EPO) and control group which received normal saline. Cardiopulmonary resuscitation (CPR) was performed immediately after drug administration as per the 2010 European Resuscitation Council (ERC) guidelines for Advanced Life Support (ALS) until return of spontaneous circulation (ROSC) or death. Animals who achieved ROSC were monitored, mechanically ventilated, extubated, observed, and euthanized. At necroscopy, adrenal glands samples were formalin-fixed, paraffin-embedded, and routinely processed. Sections were stained with hematoxylin-eosin. Results. Oedema and apoptosis were the most frequent histological changes and were detected in all animals in the adrenal cortex and in the medulla. Mild and focal endothelial lesions were also detected. A marked interindividual variability in the degree of the intensity of apoptosis and oedema at cortical and medullary level was observed within groups. Comparing the two groups, higher levels of pathological changes were detected in the control group. No significant difference between the two groups was observed regarding the endothelial changes. Conclusions. In animals exposed to ventricular fibrillation, EPO treatment has protective effects on the adrenal gland.

  7. Chronic Stress and Limbic-Hypothalamopituitary-Adrenal Axis (LHPA Response in Female Reproductive system

    Directory of Open Access Journals (Sweden)

    Farideh Zafari Zangeneh

    2009-12-01

    Full Text Available The hypothalamo-pituitary-adrenocortical (HPA axis is a critical adaptive system that maximizes survival potential in the face of physical or psychological challenge. The principal end products of the HPA axis, glucocorticoid hormones, act on multiple organ systems, including the brain, to maintain homeostatic balance. The brain is a target of stress, and the hippocampus is the first brain region, besides the hypothalamus, to be recognized as a target of glucocorticoids. These anatomical areas in brain are limbic system, and in particular the hippocampus, medial prefrontal cortex (mPFC and amigdal that have multiple control points in regulation of the hypothalamic–pituitary–adrenal (HPA axis. The studies show the prefrontal cortex (PFC plays an important role in the regulation of stress-induced hypothalamic–pituitary–adrenal (HPA activity and regulation of gonadal function in men and women is under the control of the HPA. This regulation is complex and sex steroids are important regulators of GnRH and gonadotropin release through classic feedback mechanisms in the hypothalamus and pituitary gland. Chronic stress can have a deleterious effect on the reproductive axis that, for females, is manifested in reduced pulsatile gonadotropin secretion and increased incidence of ovulatory abnormalities and infertility. The limbic–hypothalamic–pituitary–adrenal (LHPA axis suggests a functional role for gonadal steroids in the regulation of a female’s response to stress.

  8. PET studies of parkinsonian patients treated with autologous adrenal implants.

    Science.gov (United States)

    Guttman, M; Burns, R S; Martin, W R; Peppard, R F; Adam, M J; Ruth, T J; Allen, G; Parker, R A; Tulipan, N B; Calne, D B

    1989-08-01

    Transplantation of autologous adrenal medulla tissue into the striatum has recently been proposed as a treatment for Parkinson's disease. We report the use of positron emission tomography (PET) to evaluate patients who had adrenal implants placed into the right caudate. 6-[18F] fluoro-L-dopa (6-FD) scans were performed to study the integrity and activity of the implant, and the nigrostriatal dopamine system before and six weeks after transplantation surgery. [68Ga] Gallium-ethylenediaminetetraacetate (Ga) scans were also performed to assess the blood brain barrier. The Ga scans performed on two patients showed increased permeability of the blood brain barrier at the surgical site. 6-FD PET scans in five patients did not show a consistent change in striatal uptake following adrenal medullary implantation after six weeks. Further assessment of implant viability with 6-FD PET scans after longer follow up may provide useful information if the blood-brain barrier becomes re-established with the passage of time.

  9. Stereotactic Radiotherapy for Adrenal Gland Metastases: University of Florence Experience

    Energy Technology Data Exchange (ETDEWEB)

    Casamassima, Franco, E-mail: f.casamassima@dfc.unifi.it [Clinical Radiobiological Institute, University of Florence, Florence (Italy); Livi, Lorenzo [Department of Radiation-Oncology, University of Florence, Florence (Italy); Masciullo, Stefano; Menichelli, Claudia; Masi, Laura [Clinical Radiobiological Institute, University of Florence, Florence (Italy); Meattini, Icro [Department of Radiation-Oncology, University of Florence, Florence (Italy); Bonucci, Ivano [Clinical Radiobiological Institute, University of Florence, Florence (Italy); Agresti, Benedetta; Simontacchi, Gabriele [Department of Radiation-Oncology, University of Florence, Florence (Italy); Doro, Raffaela [Clinical Radiobiological Institute, University of Florence, Florence (Italy)

    2012-02-01

    Purpose: To evaluate a retrospective single-institution outcome after hypofractionated stereotactic body radiotherapy (SBRT) for adrenal metastases. Methods and Materials: Between February 2002 and December 2009, we treated 48 patients with SBRT for adrenal metastases. The median age of the patient population was 62.7 years (range, 43-77 years). In the majority of patients, the prescription dose was 36 Gy in 3 fractions (70% isodose, 17.14 Gy per fraction at the isocenter). Eight patients were treated with single-fraction stereotactic radiosurgery and forty patients with multi-fraction stereotactic radiotherapy. Results: Overall, the series of patients was followed up for a median of 16.2 months (range, 3-63 months). At the time of analysis, 20 patients were alive and 28 patients were dead. The 1- and 2-year actuarial overall survival rates were 39.7% and 14.5%, respectively. We recorded 48 distant failures and 2 local failures, with a median interval to local failure of 4.9 months. The actuarial 1-year disease control rate was 9%; the actuarial 1- and 2-year local control rate was 90%. Conclusion: Our retrospective study indicated that SBRT for the treatment of adrenal metastases represents a safe and effective option with a control rate of 90% at 2 years.

  10. Does somatostatin have a role in the regulation of cortisol secretion in primary pigmented nodular adrenocortical disease (PPNAD)? A clinical and in vitro investigation

    NARCIS (Netherlands)

    Z. Bram (Zakariae); P. Xekouki (Paraskevi); E. Louiset (Estelle); M. Keil (Mark); D. Avgeropoulos (Dimitrios); C. Giatzakis (Christoforos); M. Nesterova (Maria); N. Sinaii (Ninet); L.J. Hofland (Leo); R. Cherqaoui (Rabia); H. Lefebvre (Hervé); C.A. Stratakis (Constantine)

    2014-01-01

    textabstractContext: Somatostatin (SST) receptors (SSTRs) are expressed in a number of tissues, including the adrenal cortex, but their role in cortisol secretion has not been well characterized. Objectives: The objective of the study was to investigate the expression of SSTRs in the adrenal cortex

  11. Persistence of histoplasma in adrenals 7 years after antifungal therapy

    Directory of Open Access Journals (Sweden)

    Deepak Kothari

    2013-01-01

    Full Text Available Adrenal histoplasmosis is an uncommon cause for adrenal insufficiency. The duration of treatment for adrenal histoplasmosis is not clear. Existing treatment regimens advocate antifungals given for periods ranging from 6 months to 2 years. We report here a rare case who showed persistence of histoplasma in adrenal biopsy 7 years after being initially treated with itraconazole for 9 months. This calls for a prolonged therapy with regular review of adrenal morphology and histology in these patients.

  12. Bilateral adrenal histoplasmosis in an immunocompetent man from Texas

    Directory of Open Access Journals (Sweden)

    Colin J. Rog

    2016-12-01

    Full Text Available Disseminated histoplasmosis affecting the adrenal gland(s of immunocompetent adults is a very rare infection. Here, we present a case of bilateral adrenal histoplasmosis in an immunocompetent, 62-year-old gentleman from Texas along with a brief review of the published literature. Given the risk of patient decompensation secondary to adrenal insufficiency and the wide availability of effective treatments, adrenal histoplasmosis must be considered even in immunocompetent adults who acquire adrenal masses.

  13. Insuficiência adrenal na criança com choque séptico Adrenal insufficiency in children with septic shock

    Directory of Open Access Journals (Sweden)

    Carlos H. Casartelli

    2003-11-01

    for diagnosing and treating adrenal insufficiency in patients with septic shock. SOURCES OF DATA: Articles published in Brazilian and foreign journals selected through these publications' websites and Medline, as well as references cited in key articles. SUMMARY OF THE FINDINGS: The literature reports a range betwen 17 and 54 % for the finding of adrenal insufficiency in patients with septic shock. There is no consensus for diagnosing adrenal insufficiency in patients suffering from critical diseases, particularly in patients with septic shock. The presence of volume-refractory and catecholamine-resistant septic shock suggests this condition, while basal cortisol under 25 µg/dl is a diagnostic criterion indicating adrenal insufficiency. The adrenal stimulation test is a useful resource for identifying patients with relative adrenal insufficiency. Our testing option for adrenal stimulation in children is the use of corticotropin in low doses (0.5 µg/1,73 m². An increase of less than 9 µg/dl in the value of postcorticotropin-stimulated cortisol suggests the presence of occult (relative adrenal insufficiency. In patients with septic shock presenting adrenal insufficiency, either suspected or confirmed, the administration of hydrocortisone in shock or stress doses can be vital for a favorable clinical outcome. CONCLUSIONS: The existing data, although controversial, already provides a basis to determine when to begin hormone replacement therapy, the serum level of cortisol accepted as adequate, and the choice of corticotropin doses for performing the adrenal stimulation test and diagnosing occult or relative adrenal insufficiency in patients with septic shock.

  14. The anterior cingulate cortex

    Directory of Open Access Journals (Sweden)

    Pavlović D.M.

    2009-01-01

    Full Text Available The anterior cingulate cortex (ACC has a role in attention, analysis of sensory information, error recognition, problem solving, detection of novelty, behavior, emotions, social relations, cognitive control, and regulation of visceral functions. This area is active whenever the individual feels some emotions, solves a problem, or analyzes the pros and cons of an action (if it is a right decision. Analogous areas are also found in higher mammals, especially whales, and they contain spindle neurons that enable complex social interactions. Disturbance of ACC activity is found in dementias, schizophrenia, depression, the obsessive-compulsive syndrome, and other neuropsychiatric diseases.

  15. A case of adrenal Cushing’s syndrome with bilateral adrenal masses

    Directory of Open Access Journals (Sweden)

    Ya-Wun Guo

    2016-05-01

    Full Text Available A functional lesion in corticotrophin (ACTH-independent Cushing’s syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and 131I-6β-iodomethyl-19-norcholesterol (131I-NP-59 scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL. No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2μg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and 131I-NP-59 scintigraphy are good diagnostic methods for Cushing’s syndrome presenting with bilateral adrenal masses.

  16. Development of adrenal zonation in fetal rats defined by expression of aldosterone synthase and 11beta-hydroxylase.

    Science.gov (United States)

    Wotus, C; Levay-Young, B K; Rogers, L M; Gomez-Sanchez, C E; Engeland, W C

    1998-10-01

    The adult rat adrenal cortex is comprised of three concentric steroidogenic zones that are morphologically and functionally distinguishable: the zona glomerulosa, zona intermedia, and the zona fasciculata/reticularis. Expression of the zone-specific steroidogenic enzymes, cytochrome P450 aldosterone synthase (P450aldo), and P450 11beta hydroxylase (P45011beta), produced by the zona glomerulosa and zona fasciculata/reticularis, respectively, can be used to define the adrenal cortical cell phenotype of these two zones. In this study, immunohistochemistry and in situ hybridization were used to determine the ontogeny of expression of P450aldo and P45011beta to monitor the pattern of development of the rat adrenal cortex. RIA was used to measure adrenal content of aldosterone and corticosterone, the resulting products of the two enzymatic pathways. Double immunofluorescent staining for both enzymes at gestational day 16 (E16) showed P45011beta protein expressed in cells distributed throughout most of the adrenal intermixed with a separate, but smaller, population of cells expressing P450aldo protein. Whereas expression of P45011beta protein retained a similar pattern of distribution from E16 to adulthood (ignoring distribution of SA-1 positive, presumptive medullary cells), P450aldo protein changed its pattern of distribution by E19, becoming localized in a discontinuous ring of cells adjacent to the capsule. By postnatal day 1, P450aldo protein distribution was similar to that observed in adult glands; P450aldo-positive cells formed a continuous zone underlying the capsule. In situ hybridization showed that the pattern of P45011beta messenger RNA expression paralleled protein expression at all times, whereas P450aldo messenger RNA paralleled protein at E19 and after, but was undetectable before E19. However, adrenal aldosterone and corticosterone, as measured by RIA, were detected by E16, supporting the functional capacity of both phenotypes for all ages studied. These

  17. Precerebellin-related genes and precerebellin 1 peptide in the adrenal gland of the rat: expression pattern, localization, developmental regulation and effects on corticosteroidogenesis.

    Science.gov (United States)

    Rucinski, Marcin; Ziolkowska, Agnieszka; Szyszka, Marta; Malendowicz, Ludwik K

    2009-03-01

    Precerebellin (Cbln)-related peptides are known to modulate the secretory activity and growth of the adrenal gland. However, precise expression of the Cbln-related genes and Cbln1 peptide in the adrenal remains unclear. Therefore, we investigated, using RT-PCR, QPCR, Western blotting, immunohistochemistry and hormonal assays, their expression in the adrenals of adult rats and in the course of postnatal ontogenesis. Of the 4 known Cblns, Cbln(1-3) mRNAs were found in the adrenal gland of the adult male rats. Expression patterns of Cbln1 and 3 were similar to each other and different from that of Cbln2. Highest expression of the Cbln1 and 3 genes was observed in the zona glomerulosa (ZG), lower expression was noted in the fasciculata/reticularis and lowest expression was observed in the adrenal medulla. Expression of these genes was also present in freshly isolated rat adrenocortical cells. On the contrary, by means of classic RT-PCR, we demonstrated the presence of mRNAs of CBLN(1-4) in the human adrenal gland. In the rat, highest expression of the Cbln1 and 3 genes was found at postnatal day 2 and was somewhat lower at day 90. On the contrary, expression of the Cbln2 gene was low in adrenals of 2-day-old rats and notably higher at the remaining time points studied (up to day 360). Cerebellin (CER)-like immunoreactivity was observed in the membranes of the adrenal ZG cells, while in the medulla, immunoreactive substances were localized primarily in the cytoplasm of chromaffin cells. Cbln1-like immunoreactivity was present mainly in the cortex of the gland, and reaction products were noted both in the membranes and cytoplasm of adrenocortical cells. Semiquantitative evaluation of Cbln1 protein expression in compartments of the adrenal gland of the adult rat revealed a higher concentration of Cbln1 protein in the cortex than in the medulla of studied rats. We also found that both CER and desCER stimulated basal aldosterone secretion by freshly isolated ZG cells. Thus

  18. Activities against hemostatic proteins and adrenal gland ultrastructural changes caused by the brown widow spider Latrodectus geometricus (Araneae: Theridiidae) venom.

    Science.gov (United States)

    Guerrero, Belsy; Finol, Hector J; Reyes-Lugo, Matias; Salazar, Ana M; Sánchez, Elda E; Estrella, Amalid; Roschman-González, Antonio; Ibarra, Carlos; Salvi, Ivan; Rodríguez-Acosta, Alexis

    2010-01-01

    Brown widow spider (BrWS) (Latrodectus geometricus) venom produces intense systemic reactions such as cramps, harsh muscle nociceptive, nauseas, vomiting and hypertension. The proposed pathogenic mechanisms resulting in these accidents have principally been damages occurring at the nervous system. However, it is suspected that there is also damage of the adrenal glands, as a result of the experimental animal's clinical manifestations, which developed symptoms compatible with acute adrenal insufficiency. We have currently found that the adrenal gland is damaged by this venom gland homogenates (VGH) producing severe alterations on cortex cells resulting in death by acute adrenal insufficiency. In general, the ultrastructural study on the glands of mice under transmission electronic microscopy observations showed alterations in the majority of the intracellular membranes within 3 to 24h. BrWSVGH also showed specific actions on extracellular matrix proteins such as fibronectin, laminin and fibrinogen. In addition, zymogram experiments using gelatin as substrates detected gelatinolytic activity. The molecular exclusion fractionation of crude BrWSVGH resulted in 15 fractions, of which F1 and F2 presented alpha/beta-fibrinogenase and fibronectinolytic activities. Fractions F6, F14 and F15 showed only alpha-fibrinogenase activity; in contrast, the gelatinolytic action was only observed in fraction F11. Only metalloproteinase inhibitors abolished all these proteolytic activities. Our results suggest that adrenal cortex lesions may be relevant in the etiopathogenesis of severe brown widow spider envenoming. To our knowledge, this is the first report on adrenal gland damages, fibrinogenolytic activity and interrelations with cell-matrix adhesion proteins caused by L.geometricus VGH. The venom of this spider could be inducing hemostatic system damages on envenomed patients.

  19. Ultrasound diagnosis of adrenal hemorrhage in meningococcemia

    Energy Technology Data Exchange (ETDEWEB)

    Sarnaik, A.P.; Sanfilippo, D.J.K.; Slovis, T.L.

    1988-07-01

    Adrenal hemorrhage (AH) is a well-described complication of the neonatal period, anticoagulant therapy, and overwhelming bacterial infection especially with N. meningitis. Until recently the diagnosis of acute AH was based predominantly on autopsy findings. Ultrasound and computed tomography examinations have been successfully used for antemortem detection of AH in neonates and anticoagulated patients. We report two patients with fulminant meningococcal infection who demonstrated bilateral adrenal hemorrhages on ultrasonography.

  20. Immunologic, hemodynamic, and adrenal incompetence in cirrhosis

    DEFF Research Database (Denmark)

    Risør, Louise Madeleine; Bendtsen, Flemming; Møller, Søren

    2015-01-01

    dysfunction, but is not responsive to volume expansion. Recent research indicates that development of hepatic nephropathy represents a continuous spectrum of functional and structural dysfunction and may be precipitated by the inherent immunologic, adrenal, and hemodynamic incompetence in cirrhosis. New...... research explores several new markers of renal dysfunction that may replace serum creatinine in the future and give new insight on the hepatic nephropathy. Our understanding of the pathophysiological mechanisms causing the immunologic, adrenal, and hemodynamic incompetence, and the impact on renal...

  1. Chronic Heroin Dependence Leading to Adrenal Insufficiency

    Directory of Open Access Journals (Sweden)

    Gautam Das

    2014-01-01

    Full Text Available Opioids have been the mainstay for pain relief and palliation over a long period of time. They are commonly abused by drug addicts and such dependence usually imparts severe physiologic effects on multiple organ systems. The negative impact of opioids on the endocrine system is poorly understood and often underestimated. We describe a patient who developed severe suppression of the hypothalamic-pituitary adrenal (HPA axis leading to secondary adrenal insufficiency due to long standing abuse of opioids.

  2. Metabolism of adrenal cholesterol in man

    Science.gov (United States)

    Borkowski, Abraham; Delcroix, Claude; Levin, Sam

    1972-01-01

    The synthesis of adrenal cholesterol, its esterification and the synthesis of the glucocorticosteroid hormones were studied in vitro on human adrenal tissue. It was found that the synthesis of adrenal cholesterol may normally be small in the zona “fasciculata,” particularly when compared with the synthesis of the glucocorticosteroid hormones, that it is several times higher in the zona “reticularis” where esterified cholesterol is less abundant, and that under ACTH stimulation it increases strikingly and proportionally to the degree of esterified adrenal cholesterol depletion. On the other hand, the relative rate of esterification as well as the concentration of free adrenal cholesterol are remarkably stable: they do not differ according to the adrenal zonation and are unaffected by ACTH. Furthermore, from a qualitative point of view, the relative proportions of Δ1 and Δ2 cholesteryl esters formed in situ are similar to those anticipated from their relative concentrations, suggesting that the characteristic fatty acid distribution of the adrenal cholesteryl esters results from an in situ esterification rather than from a selective uptake of the plasma cholesteryl esters. Besides, the in vitro esterification reveals a propensity to the formation of the most unsaturated cholesteryl esters. Regarding hydrocortisone and corticosterone, their synthesis tends to be more elevated in the zona “fasciculata.” Despite its higher cholesterol concentration the zona “fasciculata” should not therefore be viewed as a quiescent functional complement to the zona “reticularis” and the cortical distribution of glucocorticosteroid hormone synthesis is quite distinct from that of adrenal cholesterol synthesis. PMID:4338120

  3. Metabolic control in patients with type 1 diabetes mellitus at the onset of primary adrenal insufficiency

    Directory of Open Access Journals (Sweden)

    A A Larina

    2013-06-01

    Full Text Available Primary adrenal insufficiency is a rare disorder but it is more common in patients with type 1 diabetes mellitus (T1DM or autoimmune thyroid diseases than in the general population. T1DM may precede the development of adrenocortical insufficiency. Addison’s disease can influence the glycemic control in patients with T1DM worsening glucose metabolism. It causes the decrease of gluconeogenesis, reduction in total insulin requirement and rather often «unexplained» recurrent hypoglycemia. Glucocorticoid replacement therapy in patients with primary adrenal insufficiency and T1DM increases the insulin requirement. The article presents the development of primary adrenal insufficiency in a patient with type 1 DM and autoimmune hypothyroidism, accompanied with incidents of recurrent hypoglycemia.

  4. Human prefrontal cortex phospholipids containing docosahexaenoic acid increase during normal adult aging, whereas those containing arachidonic acid decrease.

    Science.gov (United States)

    Norris, Sarah E; Friedrich, Michael G; Mitchell, Todd W; Truscott, Roger J W; Else, Paul L

    2015-04-01

    Membrane phospholipids make up a substantial portion of the human brain, and changes in their amount and composition are thought to play a role in the pathogenesis of age-related neurodegenerative disease. Nevertheless, little is known about the changes that phospholipids undergo during normal adult aging. This study examined changes in phospholipid composition in the mitochondrial and microsomal membranes of human dorsolateral prefrontal cortex over the adult life span. The largest age-related changes were an increase in the abundance of both mitochondrial and microsomal phosphatidylserine 18:0_22:6 by approximately one-third from age 20 to 100 years and a 25% decrease in mitochondrial phosphatidylethanolamine 18:0_20:4. Generally, increases were seen with age in phospholipids containing docosahexaenoic acid across both membrane fractions, whereas phospholipids containing either arachidonic or adrenic acid decreased with age. These findings suggest a gradual change in membrane lipid composition over the adult life span.

  5. Idiopathic Adrenal Hematoma Masquerading as Neoplasm

    Directory of Open Access Journals (Sweden)

    Kazuki Sasaki

    2012-04-01

    Full Text Available We report herein a case of idiopathic adrenal hematoma. A 59-year-old Japanese man was referred to our hospital for evaluation of a 7.0 cm mass in the right upper abdominal cavity. The tumor was suspected to originate from either the posterior segment of the liver or the right adrenal gland. His chief complaint was weight loss of 8 kg over the previous 6 months. He had no past medical history and took no medications, including no anticoagulants. Laboratory data were almost normal except for a slight elevation of PIVKA-II. The origin of the tumor was found to be the adrenal gland, as angiography revealed the blood supply to the mass to derive from the right superior and inferior adrenal arteries. A fine needle biopsy of the lesion was unable to confirm the diagnosis. Open right adrenalectomy was performed. The histopathological findings of the surgical specimen revealed a hematoma with normal adrenal tissue. In the absence of any obvious etiology, the diagnosis was idiopathic adrenal hematoma.

  6. Adrenal gland volume, intra-abdominal and pericardial adipose tissue in major depressive disorder.

    Science.gov (United States)

    Kahl, Kai G; Schweiger, Ulrich; Pars, Kaweh; Kunikowska, Alicja; Deuschle, Michael; Gutberlet, Marcel; Lichtinghagen, Ralf; Bleich, Stefan; Hüper, Katja; Hartung, Dagmar

    2015-08-01

    Major depressive disorder (MDD) is associated with an increased risk for the development of cardio-metabolic diseases. Increased intra-abdominal (IAT) and pericardial adipose tissue (PAT) have been found in depression, and are discussed as potential mediating factors. IAT and PAT are thought to be the result of a dysregulation of the hypothalamus-pituitary-adrenal axis (HPAA) with subsequent hypercortisolism. Therefore we examined adrenal gland volume as proxy marker for HPAA activation, and IAT and PAT in depressed patients. Twenty-seven depressed patients and 19 comparison subjects were included in this case-control study. Adrenal gland volume, pericardial, intraabdominal and subcutaneous adipose tissue were measured by magnetic resonance imaging. Further parameters included factors of the metabolic syndrome, fasting cortisol, fasting insulin, and proinflammatory cytokines. Adrenal gland and pericardial adipose tissue volumes, serum concentrations of cortisol and insulin, and serum concentrations tumor-necrosis factor-α were increased in depressed patients. Adrenal gland volume was positively correlated with intra-abdominal and pericardial adipose tissue, but not with subcutaneous adipose tissue. Our findings point to the role of HPAA dysregulation and hypercortisolism as potential mediators of IAT and PAT enlargement. Further studies are warranted to examine whether certain subtypes of depression are more prone to cardio-metabolic diseases.

  7. Adrenal Insufficiency as a Cause of Acute Liver Failure: A Case Report

    Directory of Open Access Journals (Sweden)

    Jamshid Vafaeimanesh

    2013-01-01

    Full Text Available Introduction. Many diseases and conditions can contribute to elevated liver enzymes. Common causes include viral and autoimmune hepatitis, fatty liver, and bile duct diseases, but, in uncommon cases like liver involvement in endocrine disorders, liver failure is also seen. Adrenal insufficiency is the rarest endocrine disorder complicating the liver. In the previously reported cases of adrenal insufficiency, mild liver enzymes elevation was seen but we report a case with severe elevated liver enzymes and liver failure due to adrenal insufficiency. Based on our knowledge, this is the first report in this field. Case Report. A 39-year-old woman was referred to emergency ward due to drowsiness and severe fatigue. Her laboratory tests revealed prothrombin time: 21 sec, alanine aminotransferase (ALT: 2339 IU/L, aspartate aminotransferase (AST: 2002 IU/L, and ALP: 90 IU/L. No common cause of liver involvement was discovered, and eventually, with diagnosis of adrenal insufficiency and corticosteroid therapy, liver enzymes and function became normal. Finally, the patient was discharged with good general condition. Conclusion. With this report, we emphasize adrenal insufficiency (primary or secondary as a reason of liver involvement in unexplainable cases and recommend that any increase in the liver enzymes, even liver failure, in these patients should be observed.

  8. Glucocorticoids and the regulation of memory in health and disease

    NARCIS (Netherlands)

    de Quervain, Dominique J. -F; Aerni, Amanda; Schelling, Gustav; Roozendaal, Benno

    2009-01-01

    Over the last decades considerable evidence has accumulated indicating that glucocorticoids - stress hormones released from the adrenal cortex - are crucially involved in the regulation of memory. Specifically, glucocorticoids have been shown to enhance memory consolidation of emotionally arousing e

  9. Update on adrenal insufficiency in patients with liver cirrhosis.

    Science.gov (United States)

    Trifan, Anca; Chiriac, Stefan; Stanciu, Carol

    2013-01-28

    Liver cirrhosis is a major cause of mortality worldwide, often with severe sepsis as the terminal event. Over the last two decades, several studies have reported that in septic patients the adrenal glands respond inappropriately to stimulation, and that the treatment with corticosteroids decreases mortality in such patients. Both cirrhosis and septic shock share many hemodynamic abnormalities such as hyperdynamic circulatory failure, decreased peripheral vascular resistance, increased cardiac output, hypo-responsiveness to vasopressors, increased levels of proinflammatory cytokines [interleukine(IL)-1, IL-6, tumor necrosis factor-alpha] and it has, consequently, been reported that adrenal insufficiency (AI) is common in critically ill cirrhotic patients. AI may also be present in patients with stable cirrhosis without sepsis and in those undergoing liver transplantation. The term hepato-adrenal syndrome defines AI in patients with advanced liver disease with sepsis and/or other complications, and it suggests that it could be a feature of liver disease per se, with a different pathogenesis from that of septic shock. Relative AI is the term given to inadequate cortisol response to stress. More recently, another term is used, namely "critical illness related corticosteroid insufficiency" to define "an inadequate cellular corticosteroid activity for the severity of the patient's illness". The mechanisms of AI in liver cirrhosis are not completely understood, although decreased levels of high-density lipoprotein cholesterol and high levels of proinflammatory cytokines and circulatory endotoxin have been suggested. The prevalence of AI in cirrhotic patients varies widely according to the stage of the liver disease (compensated or decompensated, with or without sepsis), the diagnostic criteria defining AI and the methodology used. The effects of corticosteroid therapy on cirrhotic patients with septic shock and AI are controversial. This review aims to summarize the

  10. The agonistic adrenal: melatonin elicits female aggression via regulation of adrenal androgens.

    Science.gov (United States)

    Rendon, Nikki M; Rudolph, Lauren M; Sengelaub, Dale R; Demas, Gregory E

    2015-11-22

    Classic findings have demonstrated an important role for sex steroids as regulators of aggression, but this relationship is lacking within some environmental contexts. In mammals and birds, the adrenal androgen dehydroepiandrosterone (DHEA), a non-gonadal precursor of biologically active steroids, has been linked to aggression. Although females, like males, use aggression when competing for limited resources, the mechanisms underlying female aggression remain understudied. Here, we propose a previously undescribed endocrine mechanism regulating female aggression via direct action of the pineal hormone melatonin on adrenal androgens. We examined this in a solitary hamster species, Phodopus sungorus, in which both sexes are highly territorial across the seasons, and display increased aggression concomitant with decreased serum levels of sex steroids in short 'winter-like' days. Short- but not long-day females had increased adrenal DHEA responsiveness co-occurring with morphological changes in the adrenal gland. Further, serum DHEA and total adrenal DHEA content were elevated in short days. Lastly, melatonin increased DHEA and aggression and stimulated DHEA release from cultured adrenals. Collectively, these findings demonstrate that DHEA is a key peripheral regulator of aggression and that melatonin coordinates a 'seasonal switch' from gonadal to adrenal regulation of aggression by direct action on the adrenal glands.

  11. Ovarian adrenal rest tissue in congenital adrenal hyperplasia--a patient report.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Hulsbergen- van de Kaa, C.A.; Otten, B.J.

    2006-01-01

    We report a young girl who died in an Addisonian crisis due to previously undiagnosed congenital adrenal hyperplasia (CAH), in whom ovarian adrenal rest tissue was detected at postmortem histopathological examination. This is a very rare complication in female patients with CAH with only two previou

  12. Adrenal Cavernous Hemangioma: A Case Report with Review of the Literature

    Directory of Open Access Journals (Sweden)

    Joseph J. Noh

    2014-05-01

    Full Text Available Context Adrenal cavernous hemangioma is a rare type of tumor that is usually diagnosed post-operatively. There have only been approximately 63 cases reported in the literature to date. Case report We report a case of adrenal cavernous hemangioma in a 27-year-old pregnant woman. The mass was discovered on ultrasonography when she visited a gastroenterologist for vague epigastric discomfort and vomiting. The laboratory tests were within normal limits and did not show any features suggestive of adrenal endocrinologic dysfunction. Computed Tomography (CT revealed a well-defined 7.8 × 7.8 oval mass in the right adrenal gland with speckled calcifications. The mass was removed by transabdominal laparoscopic surgery. Strong positive immunostaining for CD31 and CD34 with weakly positive staining for podoplanin/D2-40 confirmed the diagnosis of cavernous hemangioma. Conclusions We reviewed 52 case reports of adrenal cavernous hemangioma in an attempt to identify tumor characteristics. More than half of the patients reviewed showed a heterogeneous internal structure of the mass with peripheral patchy enhancement on CT. They also showed focal or speckled calcifications either on X-ray or CT. Nevertheless, many of these characteristics overlap with the imaging phenotypes of other common diseases of the adrenal gland and therefore do not seem to provide definite evidence for differential diagnosis. Laparoscopic approach is a feasible and safe modality to remove adrenal cavernous hemangiomas because they seem to form a rigid fibrotic capsule; hence the risk of bleeding due to surgical manipulation is relatively low.

  13. Oleoyl-estrone increases adrenal corticosteroid synthesis gene expression in overweight male rats.

    Science.gov (United States)

    Romero, María del Mar; Vilà, Ruth; Fernández-López, José Antonio; Esteve, Montserrat; Alemany, Marià

    2010-01-01

    Oleoyl-estrone (OE) induces a marked loss of body fat in rats by maintaining energy expenditure, body protein and blood glucose despite decreasing food intake. OE increases glucocorticoids, but they arrest OE lipid-mobilization. We studied here whether OE induces a direct effect on adrenal glands function as part of this feedback regulation. Dietary overweight male rats were given oral 10nmol/g OE gavages for ten days. A group (PF) of pair-fed to OE rats, and controls received vehicle-only gavages. OE rats lost slightly more body than PF, but had larger adrenal glands. Tissue corticosterone levels, and gene expressions for glucocorticoid-synthesizing enzymes were increased in OE versus controls and PF; thus, we assumed that adrenal growth affected essentially its cortex since OE also lowered the expression of the medullar catecholamine synthesis enzyme genes. Serum corticosterone was higher in PF than in OE and controls, but liver expression of corticosteroid-disposing steroid 5alpha-reductase was 3x larger in OE than PF and controls. Circulating glucocorticoids changed little under OE, in spite of higher adrenal gland and liver content, hinting at modulation of glucocorticoid turnover as instrumental in their purported increased activity. In conclusion, we have observed that OE considerable enhanced the expression of the genes controlling the synthesis of glucocorticoids from cholesterol in the rat and increasing the adrenal glands' corticosterone, size and cellularity, but also the liver disposal of corticosteroids, suggesting that OE increases corticosterone synthesis and degradation (i.e. serum turnover), a process not driven by limited energy availability but directly related to the administration of OE.

  14. Repetitive transcranial magnetic stimulation of the primary motor cortex in the treatment of motor signs in Parkinson's disease: A quantitative review of the literature.

    Science.gov (United States)

    Zanjani, Anosha; Zakzanis, Konstantine K; Daskalakis, Zafiris J; Chen, Robert

    2015-05-01

    Parkinson's disease (PD) is a progressive disorder characterized by the emergence of motor deficits. In light of the voluminous and conflicting findings in the literature, the aim of the present quantitative review was to examine the effects of repetitive transcranial magnetic stimulation (rTMS) targeting the primary motor cortex (M1) in the treatment of motor signs in PD. Studies meeting inclusion criteria were analyzed using meta-analytic techniques and the Unified Parkinson's Disease Rating Scale (UPDRS) sections II and III were used as outcome measures. In order to determine the treatment effects of rTMS, the UPDRS II and III scores obtained at baseline, same day, to 1 day post rTMS treatment (short-term follow-up) and 1-month post stimulation (long-term follow-up) were compared between the active and sham rTMS groups. Additionally, the placebo effect was evaluated as the changes in UPDRS III scores in the sham rTMS groups. A placebo effect was not demonstrated, because sham rTMS did not improve motor signs as measured by UPDRS III. Compared with sham rTMS, active rTMS targeting the M1 significantly improved UPDRS III scores at the short-term follow-up (Cohen's d of 0.27, UPDRS III score improvement of 3.8 points). When the long-term follow-up UPDRS III scores were compared with baseline scores, the standardized effect size between active and sham rTMS did not reach significance. However, this translated into a significant nonstandardized 6.3-point improvement on the UPDRS III. No significant improvement in the UPDRS II was found. rTMS over the M1 may improve motor signs. Further studies are needed to provide a definite conclusion.

  15. Hypoactivation of the primary sensorimotor cortex in de novo Parkinson's disease. A motor fMRI study under controlled conditions

    Energy Technology Data Exchange (ETDEWEB)

    Tessa, Carlo; Vignali, Claudio [Versilia Hospital, AUSL Versilia, Division of Radiology, Camaiore (Italy); Lucetti, Claudio [Versilia Hospital, AUSL Versilia, Division of Neurology, Camaiore (Italy); Diciotti, Stefano; Paoli, Lorenzo; Ginestroni, Andrea; Mascalchi, Mario [University of Florence, Radiodiagnostic Section, Department of Clinical Physiopathology, Florence (Italy); Cecchi, Paolo; Baldacci, Filippo [University of Pisa, Department of Neuroscience, Pisa (Italy); Giannelli, Marco [Azienda Ospedaliero-Universitaria Pisana, Unit of Medical Physics, Pisa (Italy); Bonuccelli, Ubaldo [Versilia Hospital, AUSL Versilia, Division of Neurology, Camaiore (Italy); University of Pisa, Department of Neuroscience, Pisa (Italy)

    2012-03-15

    Nuclear medicine studies in Parkinson's disease (PD) indicate that nigrostriatal damage causes a widespread cortical hypoactivity assumed to be due to reduced excitatory thalamic outflow. However, so far, functional MRI (fMRI) studies have provided controversial data about this ''functional deafferentation'' phenomenon. To further clarify this issue, we assessed, with fMRI, de novo drug-naive PD patients using a relatively complex motor task under strictly controlled conditions. Nineteen de novo PD patients with right-predominant or bilateral symptoms and 13 age-matched healthy volunteers performed continuous writing of ''8'' figures with the right-dominant hand using a MR-compatible device that enables identification of incorrectly performed tasks and measures the size and the frequency of the ''8''s. The data were analyzed with FSL software and correlated with the clinical severity rated according to the Hoehn and Yahr (HY) staging system. Fifteen (89%) of 19 PD patients and 12 (92%) of 13 controls correctly executed the task. PD patients showed significant hypoactivation of the left primary sensorimotor cortex (SM1) and cerebellum and no hyperactive areas as compared to controls. However, activation in SM1 and supplementary motor area bilaterally, in left supramarginal, parietal inferior, parietal superior and frontal superior gyri as well as in right parietal superior and angular gyri paralleled increasing disease severity as assessed with the HY stage. In line with the ''deafferentation hypothesis'', fMRI demonstrates hypoactivation of the SM1 in the early clinical stage of PD. (orig.)

  16. Impact of molecular genetics on congenital adrenal hyperplasia management.

    Science.gov (United States)

    Balsamo, A; Baldazzi, L; Menabò, S; Cicognani, A

    2010-09-01

    Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders caused by mutations in genes encoding the enzymes involved in one of the 5 steps of adrenal steroid synthesis or the electron donor P450 oxidoreductase (POR) enzyme. Steroid 21-hydroxylase deficiency (21-OHD), the principal focus of this review, accounts for about 90-95% of all CAH cases, and its biochemical and clinical severity depends on the underlying CYP21A2 gene disruption. Molecular genetic advancements have been achieved in recent years, and the aim of this review is to attempt to highlight its contribution to the comprehension and management of the disease. When possible, we will try to achieve this goal also by providing some results from our personal experience regarding: some aspects of CYP21A2 gene analysis, with basic genotype/phenotype relationships; its crucial role in both genetic counselling and in prenatal diagnosis and treatment in families at risk for 21-OHD; its help in the comprehension of the severity of the disease in patients diagnosed by neonatal screening and possibly treated before an evident salt-loss crisis or before performing adequate blood sampling; its usefulness in the definition of post ACTH 17-hydroxyprogesterone values, discriminating between non-classic, heterozygote and normal subjects; and finally the contribution of genes other than CYP21A2 whose function or dysfunction could influence 21-hydroxylase activity and modify the presentation or management of the disease.

  17. Chronic physical stress changes gene expression of catecholamine biosynthetic enzymes in the adrenal medulla of adult rats

    Directory of Open Access Journals (Sweden)

    Gavrilović Ljubica

    2012-01-01

    Full Text Available In this study we examined how chronic forced running (CFR affects the expression of catecholamine biosynthetic enzymes and cAMP response element-binding (CREB in the adrenal medulla and the weight of adrenal glands of rats. Also, we examined how CFR and additional acute immobilization stress affect the expression of catecholamine biosynthetic enzymes in the adrenal medulla and the concentration of catecholamines and corticosterone (CORT in the blood plasma. In this experiment we used as a model forced exercise in rats (treadmill running. We used the most advanced method for determining the level of gene expression, Real-time PCR with TaqMan probes, as well as Western blot analysis (ECL. We found that CFR decreases tyrosine hydroxylase (TH, and dopamine-β-hydroxylase (DBH mRNA and protein levels in the adrenal medulla. The decreased TH and DBH mRNA levels coincide with the reduced expression of CREB in the adrenal medulla and with the reduced plasma CORT level. Additionally, CFR reduces the level of phenylethanolamine N-methyltransferase (PNMT mRNA, but elevates its protein level in the adrenal medulla and increases the concentration of adrenaline (A in the plasma. Reduced level of PNMT mRNA in the adrenal medulla coincides with reduced plasma CORT level. The additional acute immobilization stress increases gene expression of catecholamine biosynthetic enzymes in the adrenal medulla, as well as catecholamines and CORT levels in the plasma. The increased synthesis of PNMT enzyme in the adrenal medulla may result in an increased biosynthesis of A under chronic stress conditions. Additionally, increased level of catecholamines in the plasma after chronic physical stress is the allostatic load that may induce numerous diseases and pathological conditions.

  18. Early correlation of microglial activation with enhanced tumor necrosis factor-alpha and monocyte chemoattractant protein-1 expression specifically within the entorhinal cortex of triple transgenic Alzheimer's disease mice

    Directory of Open Access Journals (Sweden)

    LaFerla Frank M

    2005-10-01

    Full Text Available Abstract Background Alzheimer's disease is a complex neurodegenerative disorder characterized pathologically by a temporal and spatial progression of beta-amyloid (Aβ deposition, neurofibrillary tangle formation, and synaptic degeneration. Inflammatory processes have been implicated in initiating and/or propagating AD-associated pathology within the brain, as inflammatory cytokine expression and other markers of inflammation are pronounced in individuals with AD pathology. The current study examines whether inflammatory processes are evident early in the disease process in the 3xTg-AD mouse model and if regional differences in inflammatory profiles exist. Methods Coronal brain sections were used to identify Aβ in 2, 3, and 6-month 3xTg-AD and non-transgenic control mice. Quantitative real-time RT-PCR was performed on microdissected entorhinal cortex and hippocampus tissue of 2, 3, and 6-month 3xTg-AD and non-transgenic mice. Microglial/macrophage cell numbers were quantified using unbiased stereology in 3xTg-AD and non-transgenic entorhinal cortex and hippocampus containing sections. Results We observed human Aβ deposition at 3 months in 3xTg-AD mice which is enhanced by 6 months of age. Interestingly, we observed a 14.8-fold up-regulation of TNF-α and 10.8-fold up-regulation of MCP-1 in the entorhinal cortex of 3xTg-AD mice but no change was detected over time in the hippocampus or in either region of non-transgenic mice. Additionally, this increase correlated with a specific increase in F4/80-positive microglia and macrophages in 3xTg-AD entorhinal cortex. Conclusion Our data provide evidence for early induction of inflammatory processes in a model that develops amyloid and neurofibrillary tangle pathology. Additionally, our results link inflammatory processes within the entorhinal cortex, which represents one of the earliest AD-affected brain regions.

  19. Incidentally detected hydatid cyst of the adrenal gland: A case report.

    Science.gov (United States)

    Akbulut, Sami

    2016-09-16

    Hydatid cysts are a zoonotic disease that can involve many organs and tissues in the human body but primarily involve the liver and lungs. Of the main organs, adrenal glands are those seldom affected by hydatid cysts. The purpose of this study was to present a case with an incidentally detected hydatid cyst of the right adrenal gland on computed tomography, and a positive echincoccus IgG enzyme-linked immunosorbent assay test on top of a toxic multinodular thyroid goiter for which thyroidectomy was indicated.

  20. Incidentally detected hydatid cyst of the adrenal gland: A case report

    OpenAIRE

    Akbulut, Sami

    2016-01-01

    Hydatid cysts are a zoonotic disease that can involve many organs and tissues in the human body but primarily involve the liver and lungs. Of the main organs, adrenal glands are those seldom affected by hydatid cysts. The purpose of this study was to present a case with an incidentally detected hydatid cyst of the right adrenal gland on computed tomography, and a positive echincoccus IgG enzyme-linked immunosorbent assay test on top of a toxic multinodular thyroid goiter for which thyroidecto...

  1. Progressive abnormalities in the brain scan in adrenal leukodystrophy. [/sup 99m/Tc tracer technique

    Energy Technology Data Exchange (ETDEWEB)

    Chatterton, B.E.

    1977-11-01

    A case report is presented of a 10-yr-old boy with restless movements and deteriorated mental ability. A brain scan was performed using /sup 99m/Tc pertechnetate. A faint area of uptake appeared; 6 months later the brain scan showed more intense uptake and neurologic symptoms increased; the patient died soon after and autopsy showed leukodystrophy of the brain and adrenal atrophy. A study of previous cases indicates that adrenal leukodystrophy is a sex-linked hereditary disease in which progressive demyelination leads to dementia, cortical blindness, and spasticity. In all reported cases abnormal areas on the brain scan corresponded with pathologic changes. (HLW)

  2. Prednisolone is associated with a worse lipid profile than hydrocortisone in patients with adrenal insufficiency

    NARCIS (Netherlands)

    Quinkler, Marcus; Ekman, Bertil; Marelli, Claudio; Uddin, Sharif; Zelissen, Pierre; Murray, Robert

    2017-01-01

    OBJECTIVE: Prednisolone is used as glucocorticoid replacement therapy for adrenal insufficiency (AI). Recent data indicate that its use in AI is associated with low bone mineral density. Data on risk factors for cardiovascular disease in patients with AI treated with prednisolone are scarce, despite

  3. Influence of fetal growth velocity and smallness at birth on adrenal function in adolescence

    DEFF Research Database (Denmark)

    Beck Jensen, Rikke; vielwerth, Signe; Larsen, Torben;

    2011-01-01

    The hypothalamic-pituitary-adrenal axis is susceptible to programming during fetal development and may be linked to risk of disease later in life. In a former prospective study the cohort was divided into those born appropriate for gestational age (AGA) or small for gestational age (SGA; birth...

  4. What Are the Treatments for Congenital Adrenal Hyperplasia (CAH)?

    Science.gov (United States)

    ... Publications What are the treatments for congenital adrenal hyperplasia (CAH)? Skip sharing on social media links Share ... sees many CAH cases. 6 The Congenital Adrenal Hyperplasia Research, Education & Support (CARES) Foundation strongly recommends delaying ...

  5. Study of morphological alterations of the adrenal glands in the neoplastic cachexia Estudo das alterações morfológicas da glândula adrenal na caquexia neoplásica

    Directory of Open Access Journals (Sweden)

    Tânia Longo Mazzuco

    2009-01-01

    Full Text Available Advanced cancer occurs with nutritional and metabolic alterations that characterize neoplastic cachexia. When homeostasis is compromised, the adrenal glands have a fundamental role in the neuroendocrine response. Our purpose in this research was to study morphological alterations of the adrenal glands in the development of cancer associated to cachexia. Cachexia experimental model induced by Walker 256 tumor in Wistar rats, was used. Animals were sacrificed 12 days after tumor cells inoculation and adrenal glands removal for histopathologic analysis by means of hematoxylin and eosin stain. Nutritional parameters, cachexia index and adrenal glands weight, were evaluated. Animals with tumor presented cachexia index of 16,6 ± 4%. Adrenal glands average weight was significantly higher in the tumor group (40 mg ± 10 than in the control group (25 mg ± 3. Adrenal cortex of animals with cachexia showed hypertrophy of the zona fasciculata and reticular layer, with voluminous spongiocytes; vascular congestion and stasis were observed in the medullar region. Results were similar in the pair and ad libitum-fed groups. Animals with cancer cachexia showed compromised morphology of the adrenal glands which showed alterations related to stress response, suggesting increased cathecolamine secretion and activation of the hypothalamus-pituitary-adrenal axis.   Advanced cancer occurs with nutritional and metabolic alterations that characterize neoplastic cachexia. When homeostasis is compromised, the adrenal glands have a fundamental role in the neuroendocrine response. Our purpose in this research was to study morphological alterations of the adrenal glands in the development of cancer associated to cachexia. Cachexia experimental model induced by Walker 256 tumor in Wistar rats, was used. Animals were sacrificed 12 days after tumor cells inoculation and adrenal glands removal for histopathologic analysis by means of hematoxylin and eosin stain. Nutritional

  6. Astrocytes and Glutamate Homoeostasis in Alzheimer's Disease: A Decrease in Glutamine Synthetase, But Not in Glutamate Transporter-1, in the Prefrontal Cortex

    Directory of Open Access Journals (Sweden)

    Magdalena Kulijewicz-Nawrot

    2013-09-01

    Full Text Available Astrocytes control tissue equilibrium and hence define the homoeostasis and function of the CNS (central nervous system. Being principal homoeostatic cells, astroglia are fundamental for various forms of neuropathology, including AD (Alzheimer's disease. AD is a progressive neurodegenerative disorder characterized by the loss of cognitive functions due to specific lesions in mnesic-associated regions, including the mPFC (medial prefrontal cortex. Here, we analyzed the expression of GS (glutamine synthetase and GLT-1 (glutamate transporter-1 in astrocytes in the mPFC during the progression of AD in a triple-transgenic mouse model (3xTg-AD. GS is an astrocyte-specific enzyme, responsible for the intracellular conversion of glutamate into glutamine, whereas the removal of glutamate from the extracellular space is accomplished mainly by astroglia-specific GLT-1. We found a significant decrease in the numerical density (Nv, cells/mm3 of GS-positive astrocytes from early to middle ages (1–9 months; at the age of 1 month by 17%, 6 months by 27% and 9 months by 27% when compared with control animals in parallel with a reduced expression of GS (determined by Western blots, which started at the age of 6 months and was sustained up to 12 months of age. We did not, however, find any changes in the expression of GLT-1, which implies an intact glutamate uptake mechanism. Our results indicate that the decrease in GS expression may underlie a gradual decline in the vital astrocyte-dependent glutamate–glutamine conversion pathway, which in turn may compromise glutamate homoeostasis, leading towards failures in synaptic connectivity with deficient cognition and memory.

  7. Astrocytes and glutamate homoeostasis in Alzheimer's disease: a decrease in glutamine synthetase, but not in glutamate transporter-1, in the prefrontal cortex

    Directory of Open Access Journals (Sweden)

    Alexei Verkhratsky

    2013-10-01

    Full Text Available Astrocytes control tissue equilibrium and hence define the homoeostasis and function of the CNS (central nervous system. Being principal homoeostatic cells, astroglia are fundamental for various forms of neuropathology, including AD (Alzheimer's disease. AD is a progressive neurodegenerative disorder characterized by the loss of cognitive functions due to specific lesions in mnesic-associated regions, including the mPFC (medial prefrontal cortex. Here, we analyzed the expression of GS (glutamine synthetase and GLT-1 (glutamate transporter-1 in astrocytes in the mPFC during the progression of AD in a triple-transgenic mouse model (3xTg-AD. GS is an astrocyte-specific enzyme, responsible for the intracellular conversion of glutamate into glutamine, whereas the removal of glutamate from the extracellular space is accomplished mainly by astroglia-specific GLT-1. We found a significant decrease in the numerical density (Nv, cells/mm3 of GS-positive astrocytes from early to middle ages (1–9 months; at the age of 1 month by 17%, 6 months by 27% and 9 months by 27% when compared with control animals in parallel with a reduced expression of GS (determined by Western blots, which started at the age of 6 months and was sustained up to 12 months of age. We did not, however, find any changes in the expression of GLT-1, which implies an intact glutamate uptake mechanism. Our results indicate that the decrease in GS expression may underlie a gradual decline in the vital astrocyte-dependent glutamate–glutamine conversion pathway, which in turn may compromise glutamate homoeostasis, leading towards failures in synaptic connectivity with deficient cognition and memory.

  8. Bilateral adrenal hemorrhage in polycythemia vera

    Directory of Open Access Journals (Sweden)

    Shruti Bhandari

    2016-09-01

    Full Text Available Bilateral adrenal hemorrhage (BAH is a rare complication typically seen in critically ill patients, which can lead to acute adrenal insufficiency and death unless it is recognized promptly and treated appropriately. We describe the case of a 64-year-old man with polycythemia vera found to be unresponsive with fever, hypotension, tachycardia, and hypoglycemia. Electrocardiogram showed ST-elevation with elevated troponin, hemoglobin, prothrombin time, and partial thromboplastin time. He required aggressive ventilator and vasopressor support. Despite primary coronary intervention, he remained hypotensive. Random cortisol level was low. He received stress dose hydrocortisone with immediate hemodynamic stability. BAH was highly suspected and was confirmed by non-contrast abdominal computed tomography. Prompt recognition and timely initiated treatment remain crucial to impact the mortality associated with acute adrenal insufficiency.

  9. THE RESULTS OF SURGICAL TREATMENT OF PATIENTS WITH ADRENAL TUMORS

    Directory of Open Access Journals (Sweden)

    A. A. Kostin

    2016-01-01

    Full Text Available Malignant adrenal tumors such as adrenocortical cancer (ACC and malignant pheochromocytoma (MPCC have a particularly aggressive course, leading to higher mortality in these patients, especially in the later stages of the disease. In this regard, there is a statistically significant difference in survival of patients, depending on the time of detection and initiation of treatment.Purpose. Analysis of the results of surgical treatment of patients with adrenal tumors.Patients and methods. 55 patients aged from 17 to 75 years (median 50 ± 13 years with primary tumor neoplasms of the adrenal gland underwent surgical treatment in the period from 1999 to 2014 in the Department of Onco-urology, P. Hertsen MORI. 18 adrenalectomy (32,7% completed laparoscopy, 36 (65,5% – open access, 1 (1,8% – videoassistance. Among them, retroperitoneal lymph node dissection with ipsilateral hand made in 14 (25.5%, combined operations with resection of adjacent organs – 7 (12,7%, thrombectomy with resection of the inferior Vena cava – 3 (5,5% cases. The median time was 100 ± 73 minutes in open surgical procedures – 183 ± 55 minutes, laparoscopic – 60 ± 30 minutes. The volume of blood loss – 300 ± 1136 ml. According to the routine morphological studies, 24 (43,6% patients the tumor were benign, the remaining 31 (56,4% – malignant.Results. Long-term results have been traced in 84% of patients in group with ACC. The observation time for patients varied from 2 to 167 months, with a median of 64.5 (IQR 22–111 months. Median survival without progression and 5-year overall and tumor survival was: I stage – 93 months and 67 ± 13%, II stage – 30 months and 75 ± 21%, III stage– 18 months and 67 ± 27%, IV stage – 10 months and 25 ± 21%. The observation time for patients with MPCC varies from 12 to 102 months, with a median of 60 (IQR 18–102 months. Median survival without progression was 26 (IQR 15 to 38 months, 5-year overall and tumor survival

  10. A success story in congenital adrenal hyperplasia.

    Science.gov (United States)

    Kriplani, Alka; Lunkad, Amol; Agarwal, Nutan; Kulshreshtha, Bindu; Ariachery, C Aminni

    2012-12-01

    Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders characterized by enzyme defects in adrenal steroidogenic pathways. CAH due to 21-hydroxylase deficiency accounts for 95 % of cases. This case was diagnosed to have simple virilizing type of CAH and started on dexamethasone, and underwent genitoplasty and clitoroplasty at 25 years of age, then was married 3 years after surgery and conceived spontaneously 2 years after marriage, to deliver a healthy male baby. Thus, proper diagnosis and treatment with steroids and genitoplasty can give females with CAH a normal sexual, normal menstrual, and reproductive function.

  11. NonClassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Azziz Ricardo

    2010-05-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and "on-time" puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  12. Cystic Pheochromocytoma Presenting as Adrenal Cyst

    Science.gov (United States)

    Abdulsalam, Mohammed Shafi; Satish, Priyanka; Janakiraman, Raghunath Keddy; Singh, Shivshankar

    2016-01-01

    Pheochromocytomas are usually solid tumours. But it can present as cystic lesions in the adrenal gland. Cystic lesions in adrenal gland with hypertension needs attention to rule out pheochromocytoma. If ignored, it may lead to hypertensive emergency, multisystem crisis and death. Early diagnosis with biochemistry, Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) of abdomen, proper functional imaging like Meta Iodo Benzyl Guanidine (MIBG) scan is essential. Proper preoperative preparation is important to prevent hypertensive crisis during and after surgery. We are reporting a case of cystic pheochromocytoma in a young male. PMID:28050427

  13. Analysis of 10 cases died from the acute infections disease with the severe adrenalitis, necrosis and hemorrhage%急性重症感染合并出血坏死性肾上腺炎死亡十例分析

    Institute of Scientific and Technical Information of China (English)

    李品玉; 于晓军; 徐小虎; 刘卯阳

    2009-01-01

    Objective To discuss the potential mechanisms and the role of adrenaliris with extensive hemorrhage and necrosis in acute critical infectious diseases.Methods Forensic autopsy cases from 1998 to 2007 were retrospectively analyzed.And 10 cases of them were involved in the primary and critical infecfion with acute infiammation,hemorrhage and necrosis of adrenal glands.Results Clinical diagnoses of 10 Cases were respiratory tract infection(n=6),septic shock(n=2),acute esophagitis(n=1),and undefined causes(n=1),and the quickly aggravatinng conditions resulted in death ultimately.However,systematic autopsy confirmed that 7 cages died of acute lymphocytic pancarditis,2 cases acute and lymphocytic interstitial pneumonia and 1 cKse epidemic cerebrospinal meningitis with septicemia.And severe complications of all cases included hemorrhagic necmtizing adrenalitis,systemic inflammatory response syndrome and multiple organ failure(MOF).Conclusions Hemorrhagic necrotizing adrenalitis should be considered as one of the important pathological changes in fatal MOF resulting from severe infection and trauma etc.More attention should be paid in clinical treatment.%目的 探讨出血坏死性肾上腺炎在急性生症感染性疾病死亡中的作用.方法 回顾性分析1998至2007年系统法医病理学解剖案例中10例原发性重症感染性疾病合并严重出血坏死性肾上腺炎案例的临床病理资料.结果 10例中临床诊断上呼吸道感染6例,感染性休克2例,急性食管炎1例,诊断不明1例.均病情迅速恶化而死亡.尸检7例为急性淋巴细胞性全心炎,2例为急性淋巴细胞性间质性肺炎,1例为暴发性脑膜炎球菌败血症,均合并严重的出血坏死性肾上腺炎、全身炎症反应综合征和多器官衰竭.结论 肾上腺炎性出血坏死应属于常见的致死性多器官功能衰竭的重要器官病变之一,临床应予重视.

  14. A Rare Presentation of Primary Hyperparathyroidism with Concurrent Aldosterone-Producing Adrenal Carcinoma

    Directory of Open Access Journals (Sweden)

    Mario Molina-Ayala

    2015-01-01

    Full Text Available Aldosterone-producing adrenocortical carcinomas are an extremely rare cause of hyperaldosteronism (<1%. Coexistence of different endocrine tumors warrants additional screening for multiple endocrine neoplasia syndromes, especially in young patients with large or malignant masses. We present the case of a 40-year-old man with a history of hypertension that presented with an incidental left adrenal tumor during an ultrasound performed for nephrolithiasis. Biochemical assessment showed a mildly elevated calcium (11.1 mg/dL, high parathyroid hormone, and a plasma aldosterone concentration/plasma renin activity ratio of 124.5 (normal < 30, compatible with primary hyperparathyroidism with a concomitant primary hyperaldosteronism. A Tc99m-MIBI scintigraphy showed an abnormally increased tracer uptake in the right superior parathyroid and abdominal computed tomography confirmed a left adrenal tumor of 20 cm. The patient underwent parathyroidectomy and adrenalectomy with final pathology reports of parathyroid hyperplasia and adrenal carcinoma with biochemical remission of both endocrinopathies. He was started on chemotherapy, but the patient developed a frontal cortex and an arm metastasis and finally died less than one year later.

  15. Analysis of endocrine disruption effect of Roundup® in adrenal gland of male rats

    Directory of Open Access Journals (Sweden)

    Aparamita Pandey

    2015-01-01

    Full Text Available The effect of Roundup® on adrenal gland steroidogenesis and signaling pathway associated with steroid production was investigated. Doses of 10, 50, 100 and 250 mg/kg bw/d Roundup® were administered for two weeks to adult male rats. The 10 mg/kg bw/d dose which reduced circulatory corticosterone levels, but did not change food consumption and body weight, was selected for further study. The expression of cholesterol receptor (low density lipoprotein receptor, de novo cholesterol synthesis enzyme (3-hydroxy-3-methylglutaryl-coenzyme A synthase, hormone-sensitive lipase, steroidogenic acute regulatory protein (StAR mRNA and phosphorylated form was decreased. Adrenocorticotropic hormone receptor (ACTH, melanocortin-2 receptor, expression was not changed but circulatory ACTH levels and adrenal cortex protein kinase A (PKA activity were reduced. Surprisingly, exogenous ACTH treatment rescued steroidogenesis in Roundup®-treated animals. Apoptosis was evident at 250 mg/kg bw/d, but not at 10 mg/kg bw/d dose. These results suggest that Roundup® may be inhibitory to hypothalamic–pituitary axis leading to reduction in cyclic adenosine monophosphate (cAMP/PKA pathway, StAR phosphorylation and corticosterone synthesis in the adrenal tissue.

  16. A Rare Presentation of Primary Hyperparathyroidism with Concurrent Aldosterone-Producing Adrenal Carcinoma

    Science.gov (United States)

    Molina-Ayala, Mario; Ramírez-Rentería, Claudia; Manguilar-León, Analleli; Paúl-Gaytán, Pedro; Ferreira-Hermosillo, Aldo

    2015-01-01

    Aldosterone-producing adrenocortical carcinomas are an extremely rare cause of hyperaldosteronism (<1%). Coexistence of different endocrine tumors warrants additional screening for multiple endocrine neoplasia syndromes, especially in young patients with large or malignant masses. We present the case of a 40-year-old man with a history of hypertension that presented with an incidental left adrenal tumor during an ultrasound performed for nephrolithiasis. Biochemical assessment showed a mildly elevated calcium (11.1 mg/dL), high parathyroid hormone, and a plasma aldosterone concentration/plasma renin activity ratio of 124.5 (normal < 30), compatible with primary hyperparathyroidism with a concomitant primary hyperaldosteronism. A Tc99m-MIBI scintigraphy showed an abnormally increased tracer uptake in the right superior parathyroid and abdominal computed tomography confirmed a left adrenal tumor of 20 cm. The patient underwent parathyroidectomy and adrenalectomy with final pathology reports of parathyroid hyperplasia and adrenal carcinoma with biochemical remission of both endocrinopathies. He was started on chemotherapy, but the patient developed a frontal cortex and an arm metastasis and finally died less than one year later. PMID:26161274

  17. Ultrasound follow up of testicular adrenal rest tumors with congenital adrenal hyperplasia: Report of three cases

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Jeong Yeon; Kim, Dong Won; Yoon, Seong Kuk; Nam, Kyung Jin [Dept. of Radiology, Dong-A University Hospital, Busan (Korea, Republic of)

    2014-12-15

    While testicular adrenal rest tumor is generally a rare intratesticular tumor, it is frequent in patients with congenital adrenal hyperplasia. The tumors are diagnosed and followed up by ultrasound examination because these tumors are non-palpable and symptomless in most cases and always benign. Ultrasound imaging features change depending on how congenital adrenal hyperplasia is controlled. We herein report three cases of testicular adrenal rest tumors with different usual and unusual imaging findings and follow-up imaging. Patient 1 was a 14-year-old boy who presented with poor compliance to medication. Patient 2 and 3 were a 10-year-old and 13-year-old boy who presented with precocious puberty and short stature, respectively. Ultrasound examinations demonstrated oval hypoechoic masses and irregular speculated hyperechoic masses in the testes and different serial imaging findings.

  18. Insular cortex and neuropsychiatric disorders: a review of recent literature.

    Science.gov (United States)

    Nagai, M; Kishi, K; Kato, S

    2007-09-01

    The insular cortex is located in the centre of the cerebral hemisphere, having connections with the primary and secondary somatosensory areas, anterior cingulate cortex, amygdaloid body, prefrontal cortex, superior temporal gyrus, temporal pole, orbitofrontal cortex, frontal and parietal opercula, primary and association auditory cortices, visual association cortex, olfactory bulb, hippocampus, entorhinal cortex, and motor cortex. Accordingly, dense connections exist among insular cortex neurons. The insular cortex is involved in the processing of visceral sensory, visceral motor, vestibular, attention, pain, emotion, verbal, motor information, inputs related to music and eating, in addition to gustatory, olfactory, visual, auditory, and tactile data. In this article, the literature on the relationship between the insular cortex and neuropsychiatric disorders was summarized following a computer search of the Pub-Med database. Recent neuroimaging data, including voxel based morphometry, PET and fMRI, revealed that the insular cortex was involved in various neuropsychiatric diseases such as mood disorders, panic disorders, PTSD, obsessive-compulsive disorders, eating disorders, and schizophrenia. Investigations of functions and connections of the insular cortex suggest that sensory information including gustatory, olfactory, visual, auditory, and tactile inputs converge on the insular cortex, and that these multimodal sensory information may be integrated there.

  19. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

    Science.gov (United States)

    Yu, Hyeoh Won; Cho, Won Im; Choi, Keun Hee; Yun, Sumi; Cho, Hwan Seong; Shin, Choong Ho; Yang, Sei Won

    2016-01-01

    Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases. PMID:27104180

  20. Bilateral adrenal [corrected] nodules due to histoplasmosis in an elderly.

    Science.gov (United States)

    Carvalho, Flávio Pedreira de Freitas de; Curiati, José Antônio Esper; Mauad, Thaís; Incerti, Milena Mendes; Jacob Filho, Wilson

    2007-02-01

    We report a case history of an 84-year-old elderly male patient that presented with a clinical picture suggestive of adrenal failure and bilateral adrenal nodules detected by abdominal computed tomography. A fine needle-guided biopsy was inconclusive for achieving a final diagnosis. The patient died due to septic shock and the autopsy disclosed histoplasmosis with extensive bilateral necrosis of the adrenal glands. Although the adrenal involvement in chronic disseminated histoplasmosis has been described, there have been few reports of the infection being associated with adrenal insufficiency.

  1. A case of congenital lipoid adrenal hyperplasia

    Directory of Open Access Journals (Sweden)

    Mahin Hashemipour

    2012-01-01

    Full Text Available Lipoid congenital adrenal hyperplasia (lipoid CAH, a rare disorder of steroid biosynthesis, is the most severe form of CAH. In this disorder the synthesis of glucocorticoids, mineralocorticoids and sex steroids is impaired which result in adrenal failure, severe salt wasting crisis and hyperpigmentation in phenotypical female infants irrespective of genetic sex. In this report, we presented a 28-day-old phenotypic female infant, which referred with lethargy, failure to thrive and electrolyte abnormalities. Considering the clinical and biochemical findings, lipoid CAH was diagnosed and replacement therapy with standard doses of glucocorticoid and mineralocorticoid and sodium chloride was initiated. During follow-up, she had good clinical condition, but at 6 years of age, she refers with hypertension and adrenal insufficiency because of arbitrary drug discontinuation by mother. In ultrasonography an abdominal mass (the testicles was reported. Chromosome study showed 46XY pattern. Orchiectomy was performed. We recommended that in cases with clinical presentation of adrenal insufficiency if there is not the facility to determine the karyotype, repeated ultrasonography perform during follow-up. In addition, investigating the genetic bases of the disorder would help us to determine the pathogenesis of lipoid CAH in our community. It would be helpful in prenatal diagnosis and treatment of the disorder to prevent its related comorbidities.

  2. Imaging features of benign adrenal cysts

    Energy Technology Data Exchange (ETDEWEB)

    Sanal, Hatice Tuba [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey)]. E-mail: tubasanal@yahoo.com; Kocaoglu, Murat [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Yildirim, Duzgun [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Bulakbasi, Nail [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Guvenc, Inanc [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Tayfun, Cem [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey); Ucoz, Taner [Department of Radiology, Gulhane Military Medical Academy, Ankara (Turkey)

    2006-12-15

    Benign adrenal gland cysts (BACs) are rare lesions with a variable histological spectrum and may mimic not only each other but also malignant ones. We aimed to review imaging features of BACs which can be helpful in distinguishing each entity and determining the subsequent appropriate management.

  3. Mielolipoma adrenal bilateral em um cão Bilateral adrenal myelolipoma in a dog

    Directory of Open Access Journals (Sweden)

    Silvia de Araújo França

    2008-08-01

    Full Text Available O presente relato descreve um caso de mielolipoma adrenal bilateral em uma cadela Poodle de 13 anos de idade. O cão morreu por peritonite séptica aguda, decorrente de ulceração e perfuração intestinal por corpo estranho linear. O neoplasma foi achado incidentalmente durante a necropsia. Macroscopicamente, as adrenais estavam redondas e com volume e aumentados. Ao corte elas eram friáveis, vermelho-amarronzadas, intercaladas por áreas branco-amareladas levemente proeminentes. Histologicamente, extensas áreas do parênquima continham adipócitos bem diferenciados, células hematopoéticas e macrófagos com hemossiderina, achados característicos de mielolipoma adrenal.A case of bilateral adrenal myelolipoma in a 13-year-old female Poodle is reported. Acute septic peritonitis due to intestinal ulceration and perforation caused by a linear foreign body was the cause of death. The adrenal neoplasm was an incidental necropsy finding. The adrenal glands were grossly rounded, enlarged and friable, and had red-brownish parenchyma interwoven by slightly prominent white-yellowish foci. Histologically, the findings of differentiated adipocytes in large areas of the parenchyma, in association to hematopoietic cells and macrophages with hemosiderin were consistent with the diagnosis of adrenal myelolipoma.

  4. Histoplasmosis of the adrenal glands studied by CT

    Energy Technology Data Exchange (ETDEWEB)

    Wilson, D.A.; Muchmore, H.G.; Tisdal, R.G.; Fahmy, A.; Pitha, J.V.

    1984-03-01

    Computed tomography (CT) of the adrenal glands was performed on seven patients who had histologically proved disseminated histoplasmosis. All seven patients showed some degree of adrenal gland abnormality. The range of CT findings included minimal enlargement with faint flecks of calcium, moderate enlargement with focal low attenuation nodules, and massive enlargement with large areas of necrosis or dense calcification. The changes in each patient were bilateral and symmetrical. Adrenal gland shape was usually preserved. Finding of percutaneous adrenal biopsy, which was performed under CT guidance, made the diagnosis in one patient. Five of seven patients had adrenal insufficiency. It is concluded that the diagnosis of disseminated histoplasmosis should be considered in any patient who has bilateral adrenal gland enlargement and who resides in an endemic area, especially if there is evidence of adrenal insufficiency.

  5. Laparoscope resection of ectopic corticosteroid-secreting adrenal adenoma.

    Science.gov (United States)

    Wang, Xian-Ling; Dou, Jing-Tao; Gao, Jiang-Ping; Zhong, Wen-Wen; Jin, Du; Hui, Lüzhao; Lu, Ju-Ming; Mu, Yi-Ming

    2012-01-01

    Tumors originating from ectopic adrenal tissue are relatively rare. In this article, we describe a case with Cushing's syndrome caused by an ectopic adrenal adenoma. A 38 year-old male patient presenting with cushingoid appearance for 2 years was diagnosed to have ACTH-independent Cushing's syndrome based on endocrinological evaluation. Mutiple radiological examinations detected bilateral adrenal atrophy. When the images were investigated in a more expanded scope, a 3.0×3.5×5.3 cm mass was detected in the anterior of left renal hilum and left renal vein. The mass was successfully resected with intraoperative endoscopy and pathological evaluation revealed an ectopic adrenal tumor. It is suggested that when the endocrinlogically confirmed adrenal neoplasm could not be well and definitely localized, the possibility of ectopic adrenal should be presumed and further radiography examinations should extend to the field where ectopic adrenal usually presents.

  6. Blunt adrenal gland trauma in the pediatric population.

    Science.gov (United States)

    Roupakias, Stylianos; Papoutsakis, Marinos; Mitsakou, Paraskevi

    2011-07-01

    A retrospective review of the literature was performed to determine the natural history, prevalence, prognosis and management of adrenal injury associated with blunt abdominal trauma in pediatric population. Blunt adrenal injury in children is uncommon, rarely isolated, and typically present as part of a multi organ trauma. Adrenal hemorrhage is being diagnosed more frequently since the emergence of computed tomography in modern emergency rooms. Obstetric birth trauma during vaginal delivery of a macrosomic fetus may result in neonatal adrenal hemorrhage. In children appear to be an incidental finding that resolves on follow-up imaging. Most of these injuries are self-limited and do not require intervention. The differential diagnosis of an adrenal neoplasm, especially in children with an isolated adrenal hemorrhage, must be considered. The presence of adrenal hemorrhage in the absence of a trauma history should alert to the possibility of pediatric inflicted injury.

  7. Adrenal Lymphangioma Masquerading as a Catecholamine Producing Tumor

    Directory of Open Access Journals (Sweden)

    Israel Hodish

    2015-01-01

    with an adrenal cystic lesion and biochemical testing concerning for pheochromocytoma. The pertinent diagnostic and imaging features of adrenal lymphangiomas are reviewed. Methods. We describe a 59-year-old patient who presented with hyperhidrosis and a 2.2 by 2.2 cm left adrenal nodule. Biochemical evaluation revealed elevated plasma-free normetanephrine, urine normetanephrine, urine vanillylmandelic acid, and urine norepinephrine levels. Elevated plasma norepinephrine levels were not suppressed appropriately with clonidine administration. Results. Given persistent concern for pheochromocytoma, the patient underwent adrenalectomy. The final pathology was consistent with adrenal lymphangioma. Conclusions. Lymphangiomas are benign vascular lesions that can very rarely occur in the adrenal gland. Imaging findings are generally consistent with a cyst but are nonspecific. Excluding malignancy in patients presenting with adrenal cysts can be difficult. Despite its benign nature, the diagnosis of adrenal lymphangioma may ultimately require pathology.

  8. Executive functioning in children with congenital adrenal hyperplasia.

    Science.gov (United States)

    Agoston, A Monica; Gonzalez-Bolanos, Maria Teresa; Semrud-Clikeman, Margaret; Vanderburg, Nancy; Sarafoglou, Kyriakie

    2017-01-01

    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is a disorder characterized by impaired cortisol synthesis leading to excessive production of adrenal androgens. Prenatal and postnatal exposure to excess androgens may increase neural vulnerability to insult and affect cognitive functions, particularly dopamine-dependent neural circuits responsible for executive functioning (EF). Our study aimed to investigate relationship between more pronounced androgen exposure and EF-related behaviors in children with CAH, as well as sex differences in these associations. Parents of patients with CAH (n=41, boys=17, girls=24; age: M=8.41, SD=4.43) completed the Behavior Rating Inventory of Executive Function (BRIEF), a measure assessing behavioral manifestations of EF. Assessments of bone age advancement, a proxy of cumulative androgen exposure, were analyzed. Advanced bone age predicted more inhibition difficulties in boys but not in girls, and more difficulties in all other BRIEF domains in the total sample. Excessive androgen production affected EF such that more advanced bone age led to more EF-related difficulties. Sex differences in inhibition may result from estrogen exposure moderating the impact of androgens in girls but not in boys. Future interventions may include targeting EF in patients with CAH to enhance quality of life and reduce cognitive consequences associated with this disease.

  9. Behavioral Effects of Deep Brain Stimulation of the Anterior Nucleus of Thalamus, Entorhinal Cortex and Fornix in a Rat Model of Alzheimer's Disease

    Institute of Scientific and Technical Information of China (English)

    Chao Zhang; Wen-Han Hu; De-Long Wu; Kai Zhang; Jian-Guo Zhang

    2015-01-01

    Background:Recent clinical and preclinical studies have suggested that deep brain stimulation (DBS) can be used as a tool to enhance cognitive functions.The aim of the present study was to investigate the impact of DBS at three separate targets in the Papez circuit,including the anterior nucleus of thalamus (ANT),the entorhinal cortex (EC),and the fornix (FX),on cognitive behaviors in an Alzheimer's disease (AD) rat model.Methods:Forty-eight rats were subjected to an intrahippocampal injection ofamyloid peptides 1-42 to induce an AD model.Rats were divided into six groups:DBS and sham DBS groups of ANT,EC,and FX.Spatial learning and memory were assessed by the Morris water maze (MWM).Recognition memory was investigated by the novel object recognition memory test (NORM).Locomotor and anxiety-related behaviors were detected by the open field test (OF).By using two-way analysis of variance (ANOVA),behavior differences between the six groups were analyzed.Results:In the MWM,the ANT,EC,and FX DBS groups performed differently in terms of the time spent in the platform zone (F(2.23) =6.04,P < 0.01),the frequency of platform crossing (F(2,23) =11.53,P < 0.001),and the percent time spent within the platform quadrant (F(2,23) =6.29,P < 0.01).In the NORM,the EC and FX DBS groups spent more time with the novel object,although the ANT DBS group did not (F(2,23) =10.03,P < 0.001).In the OF,all of the groups showed a similar total distance moved (F(1.42) =1.14,P =0.29)and relative time spent in the center (F(2,42) =0.56,P =0.58).Conclusions:Our results demonstrated that DBS of the EC and FX facilitated hippocampus-dependent spatial memory more prominently thanANT DBS.In addition,hippocampus-independent recognition memory was enhanced by EC and FX DBS.None of the targets showed side-effects of anxiety or locomotor behaviors.

  10. Histological and histomorphometric alterations in thyroid and adrenals of CD rat pups exposed in utero to methyl thiophanate.

    Science.gov (United States)

    Maranghi, Francesca; Macrí, Caterina; Ricciardi, Claudio; Stazi, Anna Velia; Rescia, Michele; Mantovani, Alberto

    2003-01-01

    Pregnant CD rats were treated with an initial dose of 0, 310 or 560 mg/kg bw per day of the fungicide methyl thiophanate (MT) on gestational days 10-14, corresponding to formation of thyroid and adrenal primordia; newborns were sacrificed on postnatal days (PNDs) 10 and 23. No apparent maternal toxicity and no effects on litter size, viability or weight gain were present. Delayed ear pinna detachment and eye opening were present at top dose level. Thyroid histology showed increased irregular nuclei and/or mitoses (PND 10-both doses), cells with necrotic or hydropic changes (PND 23-top dose). The adrenal cortex showed increased karyomegaly and hydropic degeneration (PND 23-both doses). Thyroid histomorphometry showed reduced follicular density, moderately increased follicular cell height and number of nuclei/follicle (PND 10-top dose and PND 23-both doses), suggesting retarded follicular maturation. The adrenal cortex relative area was slightly decreased (PND 10-top dose and PND 23-both doses).MT may act as weak endocrine disrupter, suggesting that attention should be paid to delayed endocrine alterations elicited by agrochemicals.

  11. Hippocampus, caudate nucleus and entorhinal cortex volumetric MRI measurements in discrimination between Alzheimer’s disease, mild cognitive impairment, and normal aging

    Directory of Open Access Journals (Sweden)

    Rasha Elshafey

    2014-06-01

    Conclusion: Semi-automated MR volumetric measurements can be used to determine atrophy in hippocampus, caudate nucleus and entorhinal cortex which aided in discrimination of healthy elderly control subjects from subjects with AD and MCI and predict clinical decline of MCI leading to increase the efficiency of clinical treatments, delay institutionalization and improve cognition and behavioral symptoms.

  12. Transcriptome comparison in the pituitary-adrenal axis between Beagle and Chinese Field dogs after chronic stress exposure.

    Science.gov (United States)

    Luo, Wei; Fang, Meixia; Xu, Haiping; Xing, Huijie; Nie, Qinghua

    2015-10-01

    Chronic stress can induce a series of maladjustments, and the response to stress is partly regulated by the hypothalamus-pituitary-adrenal axis. The aim of this study was to investigate the genetic mechanisms of this axis regulating stress responsiveness. The pituitary and adrenal cortex of Beagle and Chinese Field Dog (CFD) from a stress exposure group [including Beagle pituitary 1 (BP1), CFD pituitary 1 (CFDP1), Beagle adrenal cortex 1 (BAC1), CFD adrenal cortex 1 (CFDAC1)] and a control group [including Beagle pituitary 2 (BP2), CFD pituitary 2 (CFDP2), Beagle adrenal cortex 2 (BAC2), CFD adrenal cortex 2 (CFDAC2)], selected to perform RNA-seq transcriptome comparisons, showed that 40, 346, 376, 69, 70, 38, 57 and 71 differentially expressed genes were detected in BP1 vs. BP2, CFDP1 vs. CFDP2, BP1 vs. CFDP1, BP2 vs. CFDP2, BAC1 vs. BAC2, CFDAC1 vs. CFDAC2, BAC1 vs. CFDAC1 and BAC2 vs. CFDAC2 respectively. NPB was a gene common to BAC1 vs. BAC2 and CFDAC1 vs. CFDAC2, indicating it was a potential gene affecting response to chronic stress, regardless of the extent of chronic stress induced. PLP1 was a gene common to BP1 vs. CFDP1 and BP2 vs. CFDP2, suggesting its important roles in affecting the stress-tolerance difference between the two breeds, regardless of whether there was stress exposure or not. Pathway analysis found 12, 4, 11 and 1 enriched pathway in the comparisons of BP1 vs. CFDP1, BP2 vs. CFDP2, CFDP1 vs. CFDP2 and BAC1 vs. BAC2 respectively. Glutamatergic synapse, neuroactive ligand-receptor interaction, retrograde endocannabinoid signaling, GABAergic synapse, calcium signaling pathway and dopaminergic synapse were the most significantly enriched pathways in both CFDP1 vs. CFDP2 and BP1 vs. CFDP1. GO, KEGG pathway and gene network analysis demonstrated that GRIA3, GRIN2A, GRIN2B and NPY were important in regulating the stress response in CFD. Nevertheless, ADORA1, CAMK2A, GRM1, GRM7 and NR4A1 might be critical genes contributing to the stress

  13. Precuneus and Cingulate Cortex Atrophy and Hypometabolism in Patients with Alzheimer’s Disease and Mild Cognitive Impairment: MRI and 18F-FDG PET Quantitative Analysis Using FreeSurfer

    Directory of Open Access Journals (Sweden)

    Matthieu Bailly

    2015-01-01

    Full Text Available Objective. The objective of this study was to compare glucose metabolism and atrophy, in the precuneus and cingulate cortex, in patients with Alzheimer’s disease (AD and mild cognitive impairment (MCI, using FreeSurfer. Methods. 47 individuals (17 patients with AD, 17 patients with amnestic MCI, and 13 healthy controls (HC were included. MRI and PET images using 18F-FDG (mean injected dose of 185 MBq were acquired and analyzed using FreeSurfer to define regions of interest in the hippocampus, amygdala, precuneus, and anterior and posterior cingulate cortex. Regional volumes were generated. PET images were registered to the T1-weighted MRI images and regional uptake normalized by cerebellum uptake (SUVr was measured. Results. Mean posterior cingulate volume was reduced in MCI and AD. SUVr were different between the three groups: mean precuneus SUVr was 1.02 for AD, 1.09 for MCI, and 1.26 for controls (p<0.05; mean posterior cingulate SUVr was 0.96, 1.06, and 1.22 for AD, MCI, and controls, respectively (p<0.05. Conclusion. We found graduated hypometabolism in the posterior cingulate cortex and the precuneus in prodromal AD (MCI and AD, whereas atrophy was not significant. This suggests that the use of 18F-FDG in these two regions could be a neurodegenerative biomarker.

  14. Cerebellin in the rat adrenal gland: gene expression and effects of CER and [des-Ser1]CER on the secretion and growth of cultured adrenocortical cells.

    Science.gov (United States)

    Rucinski, Marcin; Albertin, Giovanna; Spinazzi, Raffaella; Ziolkowska, Agnieszka; Nussdorfer, Gastone G; Malendowicz, Ludwick K

    2005-03-01

    Cerebellin (CER) is a regulatory peptide, originally isolated from rat cerebellum, which derives from the cleavage of precerebellin (Cbln), three types of which (Cbln1-3) have been identified in humans and rats. CER is also expressed in several extra-cerebellar tissues, including adrenal gland, and evidence has been provided that CER exerts a modulatory action on human and rat adrenal gland. Hence, we have investigated the expression of Cbln1-3 mRNAs and CER protein-immunoreactivity (IR) in the various zones of rat adrenal glands, and the effects of CER and its metabolite [des-Ser(1)]CER (des-CER) on the secretion and growth of cultured rat adrenocortical cells. Reverse transcription-polymerase chain reaction showed high and low expression of Cbln2 mRNA in zona glomerulosa (ZG) and zona fasciculata-reticularis, respectively. Cbln1 was not expressed, and Cbln3 mRNA was detected only in ZG. No Cbln expression was found in adrenal medulla. Immunocytochemistry demonstrated the presence of CER-IR exclusively in the adrenal cortex, the reaction being more intense in ZG. As expected, ACTH (10(-8) M) markedly enhanced corticosterone secretion and lowered proliferation rate of cultured adrenocortical cells. CER was ineffective, while des-CER exerted an ACTH-like effect, but only at the lowest concentration (10(-10) M). Taken together, these findings allow us to conclude that CER is expressed in rat adrenal cortex, and to suggest that CER conversion to des-CER by endopeptidases is needed for CER to exert its autocrine-paracrine regulatory functions.

  15. Results by motor cortex stimulation in treatment of focal dystonia, Parkinson's disease and post-ictal spasticity. The experience of the Italian Study Group of the Italian Neurosurgical Society.

    Science.gov (United States)

    Pagni, C A; Albanese, A; Bentivoglio, A; Broggi, G; Canavero, S; Cioni, B; Rose, M D; Simone, C D; Franzini, A; Lavano, A; Landi, A; Meglio, M; Modugno, M; Romanelli, L; Romito, L M; Sturiale, C; Valzania, F; Zeme, S; Zenga, F

    2008-01-01

    Extradural motor cortex stimulation has been employed in cases of Parkinson's disease (PD), fixed dystonia (FD) and spastic hemiparesis (SH) following cerebral stroke. Symptoms of PD are improved by EMCS: results were evaluated on the basis of the UPDRS and statistically analysed. In PD EMCS is less efficacious than bilateral subthalamic nucleus (STN) stimulation, but it may be safely employed in patients not eligible for deep brain stimulation (DBS). The most rewarding effect is the improvement, in severely affected patients, of posture and gait. FD, unresponsive to bilateral pallidal stimulation, has been relieved by EDMS. In SH reduction of spasticiy by EMCS allows improvement of the motor function.

  16. Reversible brain atrophy and cognitive impairment in an adolescent Japanese patient with primary adrenal Cushing’s syndrome

    Directory of Open Access Journals (Sweden)

    Ohara N

    2014-09-01

    Full Text Available Nobumasa Ohara,1 Hiroshi Suzuki,1 Akiko Suzuki,1 Masanori Kaneko,1 Masahiro Ishizawa,1 Kazuo Furukawa,1 Takahiro Abe,1 Yasuhiro Matsubayashi,1 Takaho Yamada,1 Osamu Hanyu,1 Takayoshi Shimohata,2 Hirohito Sone1 1Department of Hematology, Endocrinology and Metabolism, Faculty of Medicine, Niigata University, Niigata, Japan; 2Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan Abstract: Endogenous Cushing’s syndrome is an endocrine disease resulting from chronic exposure to excessive glucocorticoids produced in the adrenal cortex. Although the ultimate outcome remains uncertain, functional and morphological brain changes are not uncommon in patients with this syndrome, and generally persist even after resolution of hypercortisolemia. We present an adolescent patient with Cushing’s syndrome who exhibited cognitive impairment with brain atrophy. A 19-year-old Japanese male visited a local hospital following 5 days of behavioral abnormalities, such as money wasting or nighttime wandering. He had hypertension and a 1-year history of a rounded face. Magnetic resonance imaging (MRI revealed apparently diffuse brain atrophy. Because of high random plasma cortisol levels (28.7 µg/dL at 10 AM, he was referred to our hospital in August 2011. Endocrinological testing showed adrenocorticotropic hormone-independent hypercortisolemia, and abdominal computed tomography demonstrated a 2.7 cm tumor in the left adrenal gland. The patient underwent left adrenalectomy in September 2011, and the diagnosis of cortisol-secreting adenoma was confirmed histologically. His hypertension and Cushingoid features regressed. Behavioral abnormalities were no longer observed, and he was classified as cured of his cognitive disturbance caused by Cushing’s syndrome in February 2012. MRI performed 8 months after surgery revealed reversal of brain atrophy, and his subsequent course has been uneventful. In summary, the young age at onset and the

  17. Adrenal-Derived Hormones Differentially Modulate Intestinal Immunity in Experimental Colitis

    Science.gov (United States)

    de Souza, Patrícia Reis; Basso, Paulo José; Nardini, Viviani; Silva, Angelica; Banquieri, Fernanda

    2016-01-01

    The adrenal glands are able to modulate immune responses through neuroimmunoendocrine interactions and cortisol secretion that could suppress exacerbated inflammation such as in inflammatory bowel disease (IBD). Therefore, here we evaluated the role of these glands in experimental colitis induced by 3% dextran sulfate sodium (DSS) in C57BL/6 mice subjected to adrenalectomy, with or without glucocorticoid (GC) replacement. Mice succumbed to colitis without adrenals with a higher clinical score and augmented systemic levels of IL-6 and lower LPS. Furthermore, adrenalectomy negatively modulated systemic regulatory markers. The absence of adrenals resulted in augmented tolerogenic lamina propria dendritic cells but no compensatory local production of corticosterone and decreased mucosal inflammation associated with increased IFN-γ and FasL in the intestine. To clarify the importance of GC in this scenario, GC replacement in adrenalectomized mice restored different markers to the same degree of that observed in DSS group. Finally, this is the first time that adrenal-derived hormones, especially GC, were associated with the differential local modulation of the gut infiltrate, also pointing to a relationship between adrenalectomy and the modulation of systemic regulatory markers. These findings may elucidate some neuroimmunoendocrine mechanisms that dictate colitis outcome. PMID:27403034

  18. Long-term Results after CT-Guided Percutaneous Ethanol Ablation for the Treatment of Hyperfunctioning Adrenal Disorders

    Directory of Open Access Journals (Sweden)

    Nathan Elie Frenk

    Full Text Available OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.

  19. Cushing syndrome associated with an adrenal tumour.

    Science.gov (United States)

    Vieira, Helena; Brain, Caroline

    2012-08-27

    Cushing syndrome (CS) in children is a rare disorder that is most frequently caused by an adrenal tumour or a pituitary corticotrophin-secreting adenoma. The management is challenging and requires an individualised approach and multidisciplinary care. We present the case of a 23-month-old female child with a history of excessive weight gain, growth failure, hirsutism, acne and behavioural difficulties. Investigations revealed elevated serum midnight cortisol and 24 h urinary free cortisol. Overnight dexamethasone suppression testing showed no suppression of cortisol levels. Abdominal imaging revealed a right-sided suprarenal mass. She underwent right adrenalectomy and the histology showed an adrenal cortical carcinoma. There was clinical improvement with catch-up growth and weight normalisation. Despite being rare in clinical practice, in a child with weight gain, hirsuitism and growth failure the diagnosis must be considered. The overall prognosis of CS in childhood is good, but challenges remain to ensure normal growth and body composition.

  20. Functional ectopic adrenal carcinoma in a dog

    Science.gov (United States)

    Taylor, Jim A.; Lee, Maris S.; Nicholson, Matthew E.; Justin, Robert B.

    2014-01-01

    An 11-year-old spayed female pit bull terrier was presented with a 2-month history of polyuria, polydipsia, polyphagia, and panting. Serum chemistry, blood and urine analysis, and tests for hyperadrenocorticism suggested an adrenal tumor. Abdominal ultrasound identified a mass caudal to the right kidney. The mass was completely excised and histopathology was consistent with endocrine carcinoma. Three years later there was no evidence of recurrence or metastasis. PMID:25183891

  1. Renal infarction associated with adrenal pheochromocytoma.

    Science.gov (United States)

    Thewjitcharoen, Yotsapon; Atikankul, Taywin; Sunthornyothin, Sarat

    2013-09-01

    The coexistence of pheochromocytoma and renal artery stenosis had been reported occasionally from the possible mechanism of catecholoamine-induced vasospasm and extrinsic compression of renal artery in some reported cases. However, renal infarction caused by pheochromocytoma is an uncommon phenomenon. Herein, we report an interesting case of adrenal pheochromocytoma associated with renal artery thrombosis, which should be included in the differential diagnosis of pheochromocytoma patients who present with abdominal pain.

  2. Dysregulation of Autophagy, Mitophagy, and Apoptotic Genes in the Medial Temporal Lobe Cortex in an Ischemic Model of Alzheimer’s Disease

    Science.gov (United States)

    Ułamek-Kozioł, Marzena; Kocki, Janusz; Bogucka-Kocka, Anna; Petniak, Alicja; Gil-Kulik, Paulina; Januszewski, Sławomir; Bogucki, Jacek; Jabłoński, Mirosław; Furmaga-Jabłońska, Wanda; Brzozowska, Judyta; Czuczwar, Stanisław J.; Pluta, Ryszard

    2016-01-01

    Ischemic brain damage is a pathological incident that is often linked with medial temporal lobe cortex injury and finally its atrophy. Post-ischemic brain injury associates with poor prognosis since neurons of selectively vulnerable ischemic brain areas are disappearing by apoptotic program of neuronal death. Autophagy has been considered, after brain ischemia, as a guardian against neurodegeneration. Consequently, we have examined changes in autophagy (BECN 1), mitophagy (BNIP 3), and apoptotic (caspase 3) genes in the medial temporal lobe cortex with the use of quantitative reverse-transcriptase PCR following transient 10-min global brain ischemia in rats with survival 2, 7, and 30 days. The intense significant overexpression of BECN 1 gene was noted on the 2nd day, while on days 7–30 the expression of this gene was still upregulated. BNIP 3 gene was downregulated on the 2nd day, but on days 7–30 post-ischemia, there was a significant reverse tendency. Caspase 3 gene, associated with apoptotic neuronal death, was induced in the same way as BNIP 3 gene after brain ischemia. Thus, the demonstrated changes indicate that the considerable dysregulation of expression of BECN 1, BNIP 3, and caspase 3 genes may be connected with a response of neuronal cells in medial temporal lobe cortex to transient complete brain ischemia. PMID:27472881

  3. Management considerations for the adult with congenital adrenal hyperplasia.

    Science.gov (United States)

    Auchus, Richard J

    2015-06-15

    The congenital adrenal hyperplasias (CAH) are a group of genetic defects in cortisol biosynthesis, most commonly steroid 21-hydroxylase deficiency (21OHD). With the advent of cortisone therapy in the 1960s and newborn screening in the 1990s, most children with 21OHD now reach adulthood. The needs and concerns of adults with 21OHD overlap with those of children, but the focus and approach shift as these patients reach adulthood. Cohort studies suggest that adults with 21OHD experience significant health concerns such as infertility, obesity, short stature, neoplasia, and bone loss, as well as reduced quality of life. Nevertheless, the spectrum of health status and disease severity is broad, but only some of the reasons for these disparities are known. This review will summarize the current state of knowledge and suggested approaches to management adults with classic 21OHD, plus a few major considerations for adults with nonclassic 21OHD.

  4. 单孔后腹腔镜手术在肾及肾上腺疾病中的应用(附33例报告)%Single-site Retroperitoneal Laparoscopy for Kidney and Adrenal Gland Diseases: Report of 33 Cases

    Institute of Scientific and Technical Information of China (English)

    陈志军; 李庆文; 薛胜; 常睿; 汪盛; 张青川; 刘贝贝; 王成勇

    2013-01-01

    目的 探讨利用自制单孔多通道装置及常规腹腔镜器械完成单孔后腹腔镜肾及肾上腺手术的可行性.方法 2011年8月~ 2012年3月,采用切口保护器+橡胶手套自制单孔多通道装置,利用常规腹腔镜器械完成33例单孔后腹腔镜手术,其中肾上腺肿瘤切除10例,肾癌根治性肾切除6例,肾囊肿去顶减压术12例,肾蒂淋巴管结扎术4例,肾盂成形术1例.结果 33例手术均顺利完成,其中1例肾盂成形术因术中缝合困难另增加-5 mm通道完成手术,无中转开放手术.住院时间4~9d,平均6d.术中术后未出现明显并发症.结论 自制单孔多通道后腹腔镜手术在治疗肾及肾上腺疾病方面安全可行,具有创伤小、切口美观等优点.自制单孔多通道装置制作使用方便,价格低廉.%Objective To summarize the feasibility of home-made single-site retroperitoneal laparoscopic surgery for kidney and adrenal gland diseases. Methods From August 2011 to March 2012, 33 patients underwent retroperitoneal laparoendoseopic single-site surgery ( LESS) by using a home-made single-port multichannel device and standard laparoscopic instruments. The procedures included adrenalectomy (10 cases) , radical nephrectomy (6 cases) , renal cyst unroofing (12 cases) , ligation of renal lymphatic vessels (4 cases) , and pyeloplasty (1 case). Results All the operations were successfully completed without conversion to open surgery, but a additional 5-mm trocar was used to assist the procedure in one case ( pyeloplasty) ; no intraoperative or postoperative complications occurred. The mean hospital stay was 6 days (4-9 days). Conclusions Retroperitoneal LESS is feasible, effective and safe for kidney and adrenal gland diseases, with good cosmetic outcomes. Home-made port is convenient to make and use while its cost is low.

  5. Left-sided giant adrenal myelolipoma secreting catecholamine

    Directory of Open Access Journals (Sweden)

    Sujatha Udupa

    2012-01-01

    Full Text Available Adrenal myelolipoma (AML is a rare benign tumor composed of mature adipose and hematopoietic tissue. Most of these patients are asymptomatic and the tumors are non-secreting. We present a case with a large functional adrenal myelolipoma, wherein the patient was hypertensive and biochemistry revealed increase in 24 hours urinary Vanillylmandelic Acid (VMA, a metabolite of catecholamine. The mass was removed surgically and diagnosed as adrenal myelolipoma on histopathological examination. Both his blood pressure and urinary VMA returned to normal following surgery, which suggested that the mass was functioning and was secreting catecholamine. To the best of our knowledge, a catecholamine secreting adrenal myelolipoma has been reported in the literature only once previously. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine, as seen in our case. We also review the literature on functioning adrenal myelolipoma.

  6. Curative surgical management of isolated adrenal recurrence of oesophageal adenocarcinoma.

    LENUS (Irish Health Repository)

    O'Sullivan, K E

    2013-01-01

    Adrenal metastases of oesophageal adenocarcinoma are rarely detected in the clinical setting, more frequently being found as an incidental postmortem finding in the presence of widespread metastases. With improvements in the sensitivity of radiological diagnostic modalities, the incidence of adrenal tumour detection is on the rise. We report herein a particularly rare case of primary operative management by adrenalectomy for an isolated right-sided adrenal metastasis secondary to oesophageal adenocarcinoma, with a long-term survival.

  7. Curative surgical management of isolated adrenal recurrence of oesophageal adenocarcinoma.

    Science.gov (United States)

    O'Sullivan, K E; Moriarty, A R; Larkin, J O; Reynolds, J V

    2013-04-29

    Adrenal metastases of oesophageal adenocarcinoma are rarely detected in the clinical setting, more frequently being found as an incidental postmortem finding in the presence of widespread metastases. With improvements in the sensitivity of radiological diagnostic modalities, the incidence of adrenal tumour detection is on the rise. We report herein a particularly rare case of primary operative management by adrenalectomy for an isolated right-sided adrenal metastasis secondary to oesophageal adenocarcinoma, with a long-term survival.

  8. Rifampicin induced adrenal crisis in an uncommon setting

    Science.gov (United States)

    Ray, Animesh; Suri, J. C.; Gupta, Mansi

    2013-01-01

    Adrenal crisis occurs when there is decreased secretions of steroid hormones (mainly cortisol) from the adrenal glands due to varied reasons. It may arise due to a primary adrenal condition or due to decreased hormonal signals from the pituitary secondary to a hypofunctioning pituitary. Hypopituitarism may result due to direct causes like trauma, tumour, infection or it may be due to some vascular insult as seen in Sheehan syndrome. We report an unusual presentation of Sheehan syndrome in the form of life-threatening adrenal crisis precipitated by the usage of rifampicin. PMID:24339502

  9. Rifampicin induced adrenal crisis in an uncommon setting

    Directory of Open Access Journals (Sweden)

    Animesh Ray

    2013-01-01

    Full Text Available Adrenal crisis occurs when there is decreased secretions of steroid hormones (mainly cortisol from the adrenal glands due to varied reasons. It may arise due to a primary adrenal condition or due to decreased hormonal signals from the pituitary secondary to a hypofunctioning pituitary. Hypopituitarism may result due to direct causes like trauma, tumour, infection or it may be due to some vascular insult as seen in Sheehan syndrome. We report an unusual presentation of Sheehan syndrome in the form of life-threatening adrenal crisis precipitated by the usage of rifampicin.

  10. Ativação adrenérgica intramiocárdica na cardiomiopatia chagásica e doença arterial coronariana Activación adrenérgica intramiocárdica en la cardiomiopatía chagásica y enfermedad arterial coronaria Intramyocardial adrenergic activation in chagasic cardiomyopathy and coronary artery disease

    Directory of Open Access Journals (Sweden)

    Luciano Nastari

    2011-02-01

    BACKGROUND: Myocardial norepinephrine is altered in left ventricular impairment. In patients with Chagas' cardiomyopathy (CC, this issue has not been addressed. OBJECTIVE: To determine the level of myocardial norepinephrine in patients with CC and compare it in patients with coronary artery disease, and to relate myocardial norepinephrine to left ventricular ejection fraction (LVEF. METHODS: We studied 39 patients with CC, divided into group 1: 21 individuals with normal LVEF and group 2: 18 individuals with decreased LVEF. Seventeen patients with coronary artery disease were divided into group 3: 12 individuals with normal LVEF and group 4: 5 individuals with decreased LVEF. Two-dimensional echocardiography was used to measure LVEF. Myocardial norepinephrine was determined by high-performance liquid chromatography. RESULTS: Myocardial norepinephrine in CC with and without ventricular dysfunction was 1.3±1.3 and 6.1±4.2 pg/μg noncollagen protein, respectively (p<0.0001; in coronary artery disease with and without ventricular dysfunction, it was 3.3±3.0 and 9.8±4.2 pgμg noncollagen protein, respectively (p<0.0001. A positive correlation was found between LVEF and myocardial norepinephrine concentration in the patients with Chagas' cardiomyopathy (p<0.01, r = 0.57 and also in those with coronary artery disease (p<0.01, r=0.69. A significant difference was demonstrated between norepinephrine concentrations in patients with normal LVEF (groups 1 and 3; p = 0.0182, but no difference was found in patients with decreased LVEF (groups 2 and 4; p = 0.1467. CONCLUSION: In patients with Chagas' cardiomyopathy and normal global ejection fraction there is an early cardiac denervation, when compared to coronary artery disease patients.

  11. Ultrasound diagnosis of adrenal gland hemorrhage in newborn; Diagnostyka ultrasonograficzna krwawien do nadnerczy u noworodkow

    Energy Technology Data Exchange (ETDEWEB)

    Bieganowska-Klamut, Z.; Dybiec, E.; Wieczorek, P.; Charanicz-Bartler, H. [Zaklad Radiologii Pediatrycznej, Akademia Medyczna, Lublin (Poland)

    1995-12-31

    4 cases of adrenal gland hemorrhage in the newborns diagnosed by ultrasound examination were described. In 3 cases the hemorrhage was in one adrenal gland and in one the hemorrhage was in both. In 1 newborn control ultrasound and CT examination performed after 7 weeks demonstrated pseudocyst of right adrenal gland. In 2 other cases control ultrasound examination showed normalization of adrenal glands. The authors suggest differential diagnosis between the adrenal hemorrhage and neuroblastoma of adrenal gland. (author) 7 refs, 2 figs

  12. Mechanisms Mediating Environmental Chemical-Induced Endocrine Disruption in the Adrenal Gland

    Science.gov (United States)

    Martinez-Arguelles, Daniel B.; Papadopoulos, Vassilios

    2015-01-01

    Humans are continuously exposed to hundreds of man-made chemicals that pollute the environment in addition to multiple therapeutic drug treatments administered throughout life. Some of these chemicals, known as endocrine disruptors (EDs), mimic endogenous signals, thereby altering gene expression, influencing development, and promoting disease. Although EDs are eventually removed from the market or replaced with safer alternatives, new evidence suggests that early-life exposure leaves a fingerprint on the epigenome, which may increase the risk of disease later in life. Epigenetic changes occurring in early life in response to environmental toxicants have been shown to affect behavior, increase cancer risk, and modify the physiology of the cardiovascular system. Thus, exposure to an ED or combination of EDs may represent a first hit to the epigenome. Only limited information is available regarding the effect of ED exposure on adrenal function. The adrenal gland controls the stress response, blood pressure, and electrolyte homeostasis. This endocrine organ therefore has an important role in physiology and is a sensitive target of EDs. We review herein the effect of ED exposure on the adrenal gland with particular focus on in utero exposure to the plasticizer di(2-ethylehyl) phthalate. We discuss the challenges associated with identifying the mechanism mediating the epigenetic origins of disease and availability of biomarkers that may identify individual or population risks. PMID:25788893

  13. Mechanisms mediating environmental chemical-induced endocrine disruption in the adrenal gland

    Directory of Open Access Journals (Sweden)

    Daniel B Martinez-Arguelles

    2015-03-01

    Full Text Available Humans are continuously exposed to hundreds of man-made chemicals that pollute the environment in addition to multiple therapeutic drug treatments administered throughout life. Some of these chemicals, known as endocrine disruptors (EDs, mimic endogenous signals, thereby altering gene expression, influencing development, and promoting disease. Although EDs are eventually removed from the market or replaced with safer alternatives, new evidence suggests that early life exposure leaves a fingerprint on the epigenome, which may increase the risk of disease later in life. Epigenetic changes occurring in early life in response to environmental toxicants have been shown to affect behavior, increase cancer risk, and modify the physiology of the cardiovascular system. Thus, exposure to an ED or combination of EDs may represent a first hit to the epigenome. Only limited information is available regarding the effect of ED exposure on adrenal function. The adrenal gland controls the stress response, blood pressure, and electrolyte homeostasis. This endocrine organ therefore has an important role in physiology and is a sensitive target of EDs. We review herein the effect of ED exposure on the adrenal gland with particular focus on in utero exposure to the plasticizer di(2-ethylehyl phthalate. We discuss the challenges associated with identifying the mechanism mediating the epigenetic origins of disease and availability of biomarkers that may identify individual or population risks.

  14. Nebennierenkrisen und Hypoglykämien bei Kindern mit klassischem adrenogenitalem Syndrom (AGS // Adrenal crisis and hypoglycemia in children with congenital adrenal hyperplasia (CAH

    Directory of Open Access Journals (Sweden)

    Bonfig W

    2016-01-01

    Full Text Available Patients with classic congenital adrenal hyperplasia (CAH have a life-long risk for potentially leathal adrenal crisis. Toddlers and infants are also at risk for hypoglycemia. Especially infants with CAH have a higher seizure risk as a consequence of hyponatremia and hypoglycemia. Typical trigger situations for adrenal crisis are infectious diseases, especially gastroenteritis. Patients with CAH and their family members should be educated in terms of compliance with glucocorticoid medication, adequate stress dosing (usually triple to five fold elevated oral hydrocortisone dosage and intramuscular injection of hydrocortisone or prednisone. p bKurzfassung: /bPatienten mit klassischem adrenogenitalem Syndrom (AGS sind lebenslang für potentiell letale Nebennierenkrisen gefährdet. Bei Kleinkindern können außerdem Hypoglykämien auftreten. Als Folge von Elektrolytentgleisungen und Hypoglykämien haben besonders Kleinkinder auch ein erhöhtes Krampfanfallrisiko, wie erstmalig in einer deutschen Langzeitstudie berichtet wurde. Typische Triggersituationen für Nebennierenkrisen sind Infektionserkrankungen, vor allem Gastroenteritiden. Patienten mit AGS und deren Angehörige sollten im Umgang mit der Erkrankung regelmäßig geschult werden. Dies betrifft die Notwendigkeit der regelmäßigen Medikamenteneinnahme, die adäquate Glukokortikoiddosiserhöhung bei Stress (in der Regel dreifache bis fünffache Hydrokortison-Dosiserhöhung sowie die parenterale Verabreichung von Hydrokortison oder Prednison.

  15. A substitution in the ligand binding domain of the porcine glucocorticoid receptor affects activity of the adrenal gland.

    Directory of Open Access Journals (Sweden)

    Eduard Murani

    Full Text Available Glucocorticoids produced in the adrenal cortex under the control of the hypothalamic-pituitary axis play a vital role in the maintenance of basal and stress-related homeostasis and influence health and well-being. To identify loci affecting regulation of the hypothalamic-pituitary-adrenal (HPA axis in the pig we performed a genome-wide association study for two parameters of acute and long-term adrenal activity: plasma cortisol level and adrenal weight. We detected a major quantitative trait locus at the position of the glucocorticoid receptor gene (NR3C1 - a key regulator of HPA axis activity. To determine the causal variant(s, we resequenced the coding region of NR3C1 and found three missense single nucleotide polymorphisms (SNPs. SNP c.1829C>T, leading to a p.Ala610Val substitution in the ligand binding domain, showed large (about 0.6× and 1.2× phenotypic standard deviations for cortisol level and adrenal weight, respectively, and highly significant (2.1E-39≤log10(1/p≤1.7E+0 negative effects on both traits. We were able to replicate the association in three commercial pig populations with different breed origins. We analyzed effects of the p.Ala610Val substitution on glucocorticoid-induced transcriptional activity of porcine glucocorticoid receptor (GR in vitro and determined that the substitution introduced by SNP c.1829C>T increased sensitivity of GR by about two-fold. Finally, we found that non-coding polymorphisms in linkage disequilibrium with SNP c.1829C>T have only a minor effect on the expression of NR3C1 in tissues related to the HPA axis. Our findings provide compelling evidence that SNP c.1829C>T in porcine NR3C1 is a gain-of-function mutation with a major effect on the activity of the adrenal gland. Pigs carrying this SNP could provide a new animal model to study neurobiological and physiological consequences of genetically based GR hypersensitivity and adrenal hypofunction.

  16. Hypercortisolism and hyperandrogenism as manifestions of adrenal adenoma. Report of a mexican girl and review of the literature

    OpenAIRE

    de Baro Álvarez Paola; García García Lizbeth; Reyes Posada Olinka

    2014-01-01

    Background: The adrenal cortex tumors (ACT) are ex- tremely rare in children and adolescents. Only 0.2% of all the new cases of cancer in the USA and Europe are ACT. The international incidence is not well defined, since it differs demographically. Due to the rarity of ACT, little is known about their natural history. Case report: 11 months old female patient who de- veloped, at the age of nine months, acne in forehead and thorax, weight gain, facial hair, apocrine activity, appearance of ...

  17. Apparent diffusion coefficient of normal adrenal glands

    Directory of Open Access Journals (Sweden)

    Sara Reis Teixeira

    Full Text Available Abstract Objective: To assess the feasibility and reliability of apparent diffusion coefficient (ADC measurements of normal adrenal glands. Materials and methods: This was a retrospective study involving 32 healthy subjects, divided into two groups: prepubertal (PreP, n = 12, aged from 2 months to 12.5 years (4 males; 8 females; and postpubertal (PostP, n = 20, aged from 11.9 to 61 years (5 males; 15 females. Diffusion-weighted magnetic resonance imaging (DW-MRI sequences were acquired at a 1.5 T scanner using b values of 0, 20, 500, and 1000 s/mm2. Two radiologists evaluated the images. ADC values were measured pixel-by-pixel on DW-MRI scans, and automatic co-registration with the ADC map was obtained. Results: Mean ADC values for the right adrenal glands were 1.44 × 10-3 mm2/s for the PreP group and 1.23 × 10-3 mm2/s for the PostP group, whereas they were 1.58 × 10-3 mm2/s and 1.32 × 10-3 mm2/s, respectively, for the left glands. ADC values were higher in the PreP group than in the PostP group (p < 0.05. Agreement between readers was almost perfect (intraclass correlation coefficient, 0.84-0.94; p < 0.05. Conclusion: Our results demonstrate the feasibility and reliability of performing DW-MRI measurements of normal adrenal glands. They could also support the feasibility of ADC measurements of small structures.

  18. Apparent diffusion coefficient of normal adrenal glands*

    Science.gov (United States)

    Teixeira, Sara Reis; Elias, Paula Condé Lamparelli; Leite, Andrea Farias de Melo; de Oliveira, Tatiane Mendes Gonçalves; Muglia, Valdair Francisco; Elias Junior, Jorge

    2016-01-01

    Objective To assess the feasibility and reliability of apparent diffusion coefficient (ADC) measurements of normal adrenal glands. Materials and methods This was a retrospective study involving 32 healthy subjects, divided into two groups: prepubertal (PreP, n = 12), aged from 2 months to 12.5 years (4 males; 8 females); and postpubertal (PostP, n = 20), aged from 11.9 to 61 years (5 males; 15 females). Diffusion-weighted magnetic resonance imaging (DW-MRI) sequences were acquired at a 1.5 T scanner using b values of 0, 20, 500, and 1000 s/mm2. Two radiologists evaluated the images. ADC values were measured pixel-by-pixel on DW-MRI scans, and automatic co-registration with the ADC map was obtained. Results Mean ADC values for the right adrenal glands were 1.44 × 10-3 mm2/s for the PreP group and 1.23 × 10-3 mm2/s for the PostP group, whereas they were 1.58 × 10-3 mm2/s and 1.32 × 10-3 mm2/s, respectively, for the left glands. ADC values were higher in the PreP group than in the PostP group (p < 0.05). Agreement between readers was almost perfect (intraclass correlation coefficient, 0.84-0.94; p < 0.05). Conclusion Our results demonstrate the feasibility and reliability of performing DW-MRI measurements of normal adrenal glands. They could also support the feasibility of ADC measurements of small structures. PMID:28057963

  19. Neuropsychology of prefrontal cortex

    OpenAIRE

    2008-01-01

    The history of clinical frontal lobe study is long and rich which provides valuable insights into neuropsychologic determinants of functions of prefrontal cortex (PFC). PFC is often classified as multimodal association cortex as extremely processed information from various sensory modalities is integrated here in a precise fashion to form the physiologic constructs of memory, perception, and diverse cognitive processes. Human neuropsychologic studies also support the notion of different funct...

  20. Renal Cell Carcinoma Mimicking Adrenal Tumor

    Directory of Open Access Journals (Sweden)

    Mohammad Kazem Moslemi

    2010-01-01

    Full Text Available There are a variety of causes of adrenal pseudotumors on computerized tomography (CT scan, including upper-pole renal mass, gastric diverticulum, prominent splenic lobulation, pancreatic mass, hepatic mass, and periadrenal varices. We present a case of a large subhepatic mass that discrimination of its origin from neighborhood organs was difficult preoperatively. Our patient was a 58 years old man, that three months after an unsuccessful operation in another center for a pseudoadrenal mass underwent a very difficult subcapsular tumorectomy in our center.

  1. Extra-adrenal Pheochromocytoma in an Adolescent

    Directory of Open Access Journals (Sweden)

    Abdullah, Ibrahim

    2011-05-01

    Full Text Available A 17-year-old male with symptoms of headache and diaphoresis presented to the emergency department. He had eight months of noted hypertension attributed to medications. On arrival his blood pressure was 229/117mmHg, and he was ill-appearing. His blood pressure was managed aggressively, and he was diagnosed with extra-adrenal pheochromocytoma by computed tomography. He eventually underwent resection of the mass. Children with severe, symptomatic hypertension should be evaluated for pheochromocytoma. Although rare, it is curable. Failure to diagnose carries a high risk of morbidity and mortality. [West J Emerg Med. 2011;12(2:258-261.

  2. Incidence and Cause of Hypertension During Adrenal Radiofrequency Ablation

    Energy Technology Data Exchange (ETDEWEB)

    Yamakado, Koichiro, E-mail: yama@clin.medic.mie-u.ac.jp; Takaki, Haruyuki [Mie University School of Medicine, Department of Interventional Radiology (Japan); Yamada, Tomomi [Mie University School of Medicine, Department of Translational Medicine (Japan); Yamanaka, Takashi; Uraki, Junji; Kashima, Masataka; Nakatsuka, Atsuhiro; Takeda, Kan [Mie University School of Medicine, Department of Interventional Radiology (Japan)

    2012-12-15

    Purpose: To evaluate the incidence and cause of hypertension prospectively during adrenal radiofrequency ablation (RFA). Methods: For this study, approved by our institutional review board, written informed consent was obtained from all patients. Patients who received RFA for adrenal tumors (adrenal ablation) and other abdominal tumors (nonadrenal ablation) were included in this prospective study. Blood pressure was monitored during RFA. Serum adrenal hormone levels including epinephrine, norepinephrine, dopamine, and cortisol levels were measured before and during RFA. The respective incidences of procedural hypertension (systolic blood pressure >200 mmHg) of the two patient groups were compared. Factors correlating with procedural systolic blood pressure were evaluated by regression analysis.ResultsNine patients underwent adrenal RFA and another 9 patients liver (n = 5) and renal (n = 4) RFA. Asymptomatic procedural hypertension that returned to the baseline by injecting calcium blocker was found in 7 (38.9%) of 18 patients. The incidence of procedural hypertension was significantly higher in the adrenal ablation group (66.7%, 6/9) than in the nonadrenal ablation group (11.1%, 1/9, P < 0.0498). Procedural systolic blood pressure was significantly correlated with serum epinephrine (R{sup 2} = 0.68, P < 0.0001) and norepinephrine (R{sup 2} = 0.72, P < 0.0001) levels during RFA. The other adrenal hormones did not show correlation with procedural systolic blood pressure. Conclusion: Hypertension occurs frequently during adrenal RFA because of the release of catecholamine.

  3. Adrenal incidentaloma in thalassemia: a case report and literature review.

    Science.gov (United States)

    Gamberini, Maria Rita; Prandini, Napoleone; Chiodi, Elisabetta; Farneti, Carlotta; Garani, Maria Chiara

    2011-03-01

    In the last 30 years the development and widespread use of modern imaging techniques has caused a 20-time increase in the diagnosis of adrenal incidentaloma (AI). Among AIs myelolipoma (ML) is reported with a frequency up to 10%. In the literature 8 patients with adrenal masses in thalassaemia or chronic haemolytic anaemia have been reported: five cases were shown to have extramedullary haematopoiesis (EH) and 3 ML. We describe here a case of an adult male affected by beta thalassaemia intermedia and large bilateral lipomatous adrenal masses. The patient was referred to our ward at the age of 55 and underwent hormonal testing, MRI, and SPECT/CT scans. Adrenal masses were hormonally inactive, and fat-containing on MRI and CT scans. SPECT/CT examination with 99mTccolloid demonstrated the presence of marrow tissue. ML and EH are the only two tumours with marrow tissue among lipomatous adrenal masses. In our patient a brown nodular mass was resected and histologically classified as ML. In benign adrenal masses, radiological follow-up is indicated; in case of large bilateral masses adrenal function tests are suggested periodically in order to detect possible adrenal hypofunction.

  4. Volume of the adrenal and pituitary glands in depression

    DEFF Research Database (Denmark)

    Kessing, Lars Vedel; Willer, Inge Stoel; Knorr, Ulla

    2011-01-01

    Numerous studies have shown that the hypothalamic-pituitary-adrenal (HPA) axis is hyperactive in some depressed patients. It is unclear whether such hyperactivity results in changed volumes of the adrenal glands, pituitary gland and hypothalamus. We systematically reviewed all controlled studies ...

  5. Synchronous Bilateral Adrenal Metastases from Papillary Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Kaan Gokcen

    2014-12-01

    Full Text Available We report a case of synchronous bilateral adrenal metastasis of renal cell carcinoma. The contralateral metastatic adrenal mass was treated by the laparoscopic transperitoneal approach. The renal mass and its huge ipsilateral metastatic adrenal gland were removed en bloc with open procedure. A 54-year-old man presented to our clinic with left-sid renal cell carcinoma synchronously bilateral adrenal metastases. The primary tumor was localized in the upper-mid pole of the kidney. The diagnosis was established preoperatively by computed tomography. The size of the contralateral adrenal mass was 65 x 45 mm, but the ipsilateral metastatic adrenal mass was huge (140 x 65 mm. After all analysis and other scannings for any metastasis, a contralateral lapararoscopic transperitoneal adrenalectomy and a left open nephroadrenalectomy were performed simultaneously. Synchronous bilateral adrenal metastases from primary renal cell carcinoma without another metastasis is very rare. The optimal surgical procedure should be selected according to the metastatic adrenal masses size and the patient%u2019s status.

  6. Early Hormonal Influences on Cognitive Functioning in Congenital Adrenal Hyperplasia.

    Science.gov (United States)

    Resnick, Susan M.; And Others

    1986-01-01

    Reports the results of cognitive test performance and early childhood activities in individuals with congenital adrenal hyperplasia, an autosomal recessive disorder associated with elevated prenatal adrenal androgen levels, demonstrating the effects of early exposure to excess androgenizing hormones on sexually dimorphic cognitive functioning.…

  7. Wnt signaling inhibits adrenal steroidogenesis by cell-autonomous and non-cell-autonomous mechanisms.

    Science.gov (United States)

    Walczak, Elisabeth M; Kuick, Rork; Finco, Isabella; Bohin, Natacha; Hrycaj, Steven M; Wellik, Deneen M; Hammer, Gary D

    2014-09-01

    Wnt/β-catenin (βcat) signaling is critical for adrenal homeostasis. To elucidate how Wnt/βcat signaling elicits homeostatic maintenance of the adrenal cortex, we characterized the identity of the adrenocortical Wnt-responsive population. We find that Wnt-responsive cells consist of sonic hedgehog (Shh)-producing adrenocortical progenitors and differentiated, steroidogenic cells of the zona glomerulosa, but not the zona fasciculata and rarely cells that are actively proliferating. To determine potential direct inhibitory effects of βcat signaling on zona fasciculata-associated steroidogenesis, we used the mouse ATCL7 adrenocortical cell line that serves as a model system of glucocorticoid-producing fasciculata cells. Stimulation of βcat signaling caused decreased corticosterone release consistent with the observed reduced transcription of steroidogenic genes Cyp11a1, Cyp11b1, Star, and Mc2r. Decreased steroidogenic gene expression was correlated with diminished steroidogenic factor 1 (Sf1; Nr5a1) expression and occupancy on steroidogenic promoters. Additionally, βcat signaling suppressed the ability of Sf1 to transactivate steroidogenic promoters independent of changes in Sf1 expression level. To investigate Sf1-independent effects of βcat on steroidogenesis, we used Affymetrix gene expression profiling of Wnt-responsive cells in vivo and in vitro. One candidate gene identified, Ccdc80, encodes a secreted protein with unknown signaling mechanisms. We report that Ccdc80 is a novel βcat-regulated gene in adrenocortical cells. Treatment of adrenocortical cells with media containing secreted Ccdc80 partially phenocopies βcat-induced suppression of steroidogenesis, albeit through an Sf1-independent mechanism. This study reveals multiple mechanisms of βcat-mediated suppression of steroidogenesis and suggests that Wnt/βcat signaling may regulate adrenal homeostasis by inhibiting fasciculata differentiation and promoting the undifferentiated state of progenitor

  8. Localization and production of peptide endocannabinoids in the rodent CNS and adrenal medulla.

    Science.gov (United States)

    Hofer, Stefanie C; Ralvenius, William T; Gachet, M Salomé; Fritschy, Jean-Marc; Zeilhofer, Hanns Ulrich; Gertsch, Jürg

    2015-11-01

    The endocannabinoid system (ECS) comprises the cannabinoid receptors CB1 and CB2 and their endogenous arachidonic acid-derived agonists 2-arachidonoyl glycerol and anandamide, which play important neuromodulatory roles. Recently, a novel class of negative allosteric CB1 receptor peptide ligands, hemopressin-like peptides derived from alpha hemoglobin, has been described, with yet unknown origin and function in the CNS. Using monoclonal antibodies we now identified the localization of RVD-hemopressin (pepcan-12) and N-terminally extended peptide endocannabinoids (pepcans) in the CNS and determined their neuronal origin. Immunohistochemical analyses in rodents revealed distinctive and specific staining in major groups of noradrenergic neurons, including the locus coeruleus (LC), A1, A5 and A7 neurons, which appear to be major sites of production/release in the CNS. No staining was detected in dopaminergic neurons. Peptidergic axons were seen throughout the brain (notably hippocampus and cerebral cortex) and spinal cord, indicative of anterograde axonal transport of pepcans. Intriguingly, the chromaffin cells in the adrenal medulla were also strongly stained for pepcans. We found specific co-expression of pepcans with galanin, both in the LC and adrenal gland. Using LC-MS/MS, pepcan-12 was only detected in non-perfused brain (∼ 40 pmol/g), suggesting that in the CNS it is secreted and present in extracellular compartments. In adrenal glands, significantly more pepcan-12 (400-700 pmol/g) was measured in both non-perfused and perfused tissues. Thus, chromaffin cells may be a major production site of pepcan-12 found in blood. These data uncover important areas of peptide endocannabinoid occurrence with exclusive noradrenergic immunohistochemical staining, opening new doors to investigate their potential physiological function in the ECS. This article is part of the Special Issue entitled 'Fluorescent Tools in Neuropharmacology'.

  9. Surgical technique: Retroperitoneoscopic approach for adrenal masses in children.

    Science.gov (United States)

    Yankovic, F; Undre, S; Mushtaq, I

    2014-04-01

    Laparoscopic adrenalectomy is considered to be the standard of care for the surgical excision of adrenal masses. The transperitoneal laparoscopic and retroperitoneoscopic approaches are described. Both are safe and as effective as open adrenalectomy, with the added benefit of the minimally invasive approach. It can be utilized for patients requiring surgery for a phaeochromocytoma, adrenal adenoma, adrenal adenocarcinoma, Cushing's syndrome, neuroblastoma, and an incidentaloma. Relative contraindications include previous surgery of the liver or kidney, large tumours (>8-10 cm in diameter) or coagulation disorders. Although the transperitoneal route is used more widely, the retroperitoneal approach provides direct access to the adrenal gland and easy visualization of the adrenal vein. It avoids also colonic mobilization, minimizes the risk of injury to hollow viscera, and the potential risk of adhesion formation. However, the reversed orientation of the kidney and hilum, combined with a significantly smaller working space, may make this approach difficult to master.

  10. Adrenal vein sampling in the diagnosis of aldosteronism

    Directory of Open Access Journals (Sweden)

    Deipolyi AR

    2015-06-01

    Full Text Available Amy R Deipolyi,1 Rahmi Oklu2 1Vascular and Interventional Radiology, NYU Langone Medical Center, New York, NY, USA; 2Interventional Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA Abstract: Primary aldosteronism causes 15%–25% of cases of drug-resistant hypertension. Adrenal vein sampling (AVS is a procedure entailing the measurement of aldosterone from both adrenal veins, to diagnose an adrenal source of excess aldosterone secretion. Because unilateral adrenal etiologies of primary aldosteronism may be surgically resected, identifying these sources by venous sampling is critical. Technical aspects of the procedure are reviewed, with emphasis on strategies to avoid common difficulties during AVS. Keywords: primary aldosteronism, hypertension, venous sampling, adrenal adenoma

  11. Prenatal xenobiotic exposure and intrauterine hypothalamus-pituitary-adrenal axis programming alteration.

    Science.gov (United States)

    Zhang, Chong; Xu, Dan; Luo, Hanwen; Lu, Juan; Liu, Lian; Ping, Jie; Wang, Hui

    2014-11-05

    The hypothalamic-pituitary-adrenal (HPA) axis is one of the most important neuroendocrine axes and plays an important role in stress defense responses before and after birth. Prenatal exposure to xenobiotics, including environmental toxins (such as smoke, sulfur dioxide and carbon monoxide), drugs (such as synthetic glucocorticoids), and foods and beverage categories (such as ethanol and caffeine), affects fetal development indirectly by changing the maternal status or damaging the placenta. Certain xenobiotics (such as caffeine, ethanol and dexamethasone) may also affect the fetus directly by crossing the placenta into the fetus due to their lipophilic properties and lower molecular weights. All of these factors probably result in intrauterine programming alteration of the HPA axis, which showed a low basal activity but hypersensitivity to chronic stress. These alterations will, therefore, increase the susceptibility to adult neuropsychiatric (such as depression and schizophrenia) and metabolic diseases (such as hypertension, diabetes and non-alcoholic fatty liver disease). The "over-exposure of fetuses to maternal glucocorticoids" may be the main initiation factor by which the fetal HPA axis programming is altered. Meantime, xenobiotics can directly induce abnormal epigenetic modifications and expression on the important fetal genes (such as hippocampal glucocorticoid receptor, adrenal steroidogenic acute regulatory protein, et al) or damage by in situ oxidative metabolism of fetal adrenals, which may also be contributed to the programming alteration of fetal HPA axis.

  12. [Correlations between the hypothalamo-pituitary-adrenal axis and the metabolic syndrome].

    Science.gov (United States)

    Góth, Miklós; Hubina, Erika; Korbonits, Márta

    2005-01-09

    The metabolic syndrome has several similarities with Cushing's syndrome (impaired glucose tolerance, hypertension, dyslipidemia, central obesity) suggesting that abnormalities in the regulation of the hypothalamic-pituitary-adrenal axis may have a link with the metabolic syndrome. Several studies suggested an association between the clinical signs of the metabolic syndrome and the increased hypothalamic-pituitary-adrenal axis activity based on increased cortisol concentration at 09.00 a.m. and increased cortisol response to corticotropin. According to the Barker hypothesis the fetal malnutrition could determine adult cardiovascular diseases (coronary heart disease, hypertension), some endocrine and metabolic disorders (obesity, type 2 diabetes and hyperlipidemia). The suggested mechanism of the phenomenon is that the suboptimal fetal nutrition results in glucocorticoid overproduction. The 11beta-hydroxysteroid dehydrogenase (converts biological inactive cortisone to cortisol and vice versa) is an important enzyme in cortisol metabolism. The increased expression of 11beta-hydroxysteroid dehydrogenase type 1 in fat tissue could lead to central obesity and impaired glucose tolerance. The hypothesis that increased corticotropin-releasing hormone production drives the overactive hypothalamo-pituitary-adrenal axis was not proven. Further investigations are needed to identify additional pathogenetic factors and to find new therapeutic possibilities.

  13. Giant adrenal cyst - a case report and classification; Cisto gigante da adrenal - relato de caso e classificacao

    Energy Technology Data Exchange (ETDEWEB)

    Pitaki, Sergio Augusto Munhoz; Louveira, Maria Helena [Clinica Radiologica Pitaki, Curitiba, PR (Brazil). Alphasonic; Rorato, Luciana; Antonik, Gustavo; Andrade, Andre Luis Lugnani de; Teixeira, Ana Maria Gonzaga [Faculdade Evangelica de Medicina do Parana (FEMPAR), Curitiba, PR (Brazil)

    1999-12-01

    Cystic lesions of the adrenal gland are relatively rare, but they must be considering in the differential diagnosis of abdominal masses. The authors report a case of a giant adrenal cyst assessed by ultrasonography, computed tomography and magnetic resonance imaging, and outline their features and classification. (author)

  14. Prevalence of testicular adrenal rest tumours in male children with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Sweep, C.G.J.; Blickman, J.G.; Hermus, A.R.M.M.; Otten, B.J.

    2007-01-01

    OBJECTIVE: Testicular adrenal rest tumours (TART) are a well-known complication in adult male patients with congenital adrenal hyperplasia (CAH), with a reported prevalence of up to 94%. In adulthood, the tumours are associated with gonadal dysfunction most probably due to longstanding obstruction o

  15. A morphometric study of the adrenal cortex of the female DDD mouse.

    Science.gov (United States)

    Tsujio, Masashi; Mizorogi, Toshihiro; Nishijima, Kazutoshi; Kuwahara, Sachi; Aoyama, Hiroaki; Ohno, Tamio; Tanaka, Shin

    2009-02-01

    The aim of this study was to determine whether the thickness of the adrenocortical zone is associated with age in virgin and parous female DDD mice. The zona reticularis and zona glomerulosa of parous mice tended to be thicker than those of virgin mice at all ages. The zona fasciculata lactating parous mice was significantly thicker than that of virgin mice at 20 weeks of age (PDDD mice less than one year of age.

  16. Adrenal gland infection by serotype 5 adenovirus requires coagulation factors.

    Directory of Open Access Journals (Sweden)

    Lucile Tran

    Full Text Available Recombinant, replication-deficient serotype 5 adenovirus infects the liver upon in vivo, systemic injection in rodents. This infection requires the binding of factor X to the capsid of this adenovirus. Another organ, the adrenal gland is also infected upon systemic administration of Ad, however, whether this infection is dependent on the cocksackie adenovirus receptor (CAR or depends on the binding of factor X to the viral capsid remained to be determined. In the present work, we have used a pharmacological agent (warfarin as well as recombinant adenoviruses lacking the binding site of Factor X to elucidate this mechanism in mice. We demonstrate that, as observed in the liver, adenovirus infection of the adrenal glands in vivo requires Factor X. Considering that the level of transduction of the adrenal glands is well-below that of the liver and that capsid-modified adenoviruses are unlikely to selectively infect the adrenal glands, we have used single-photon emission computed tomography (SPECT imaging of gene expression to determine whether local virus administration (direct injection in the kidney could increase gene transfer to the adrenal glands. We demonstrate that direct injection of the virus in the kidney increases gene transfer in the adrenal gland but liver transduction remains important. These observations strongly suggest that serotype 5 adenovirus uses a similar mechanism to infect liver and adrenal gland and that selective transgene expression in the latter is more likely to be achieved through transcriptional targeting.

  17. Bilateral Adrenal Metastasis in Bronchogenic Carcinoma: Case Report

    Directory of Open Access Journals (Sweden)

    Nargess Afzali

    2009-01-01

    Full Text Available "nAdrenal metastases from bronchogenic carcinoma are found in approximately one third of patients at autopsy. Tumors that commonly metastasize to the adrenal gland include bronchogenic carcinoma, breast carcinoma and melanoma and small cell lung cancer, accounting for 6% of the adrenal metastasis. Routine preoperative upper abdominal CT scanning reveals an adrenal mass in approximately 10% of the patients. In selected patients, excision of the primary lung tumor and the isolated adrenal metastasis may improve survival. "nHere a 82-year-old female is presented with a history of 3 months pleuritic chest pain, chronic cough, dyspnea and also abdominal distention and discomfort. Bronchoscopy revealed tumoral vegetation in the posterior segment bronchus of the right lung upper lobe. It was diagnosed as an undifferentiated lung carcinoma pathologically. Abdominal CT scan revealed bilateral large heterogeneous masses in the adrenal glands. Biopsy of the adrenal masses revealed their metastatic nature. The patient was expired before excision of the abdominal masses because of lung cancer with pericardial invasion.   

  18. Acute adrenal crisis after orthopedic surgery for pathologic fracture

    Directory of Open Access Journals (Sweden)

    Hashimoto Nobuyuki

    2007-03-01

    Full Text Available Abstract Background Adrenal crisis after surgical procedure is a rare but potentially catastrophic life-threatening event. Its manifestations, such as hypotension, tachycardia, hypoxia, and fever mimic the other more common postoperative complications. Clinical outcome is dependent upon early recognition of the condition and proper management with exogenous steroid administration. Case presentation We report a 75-year-old man who presented with shock immediately after surgery for a femoral fracture from lung cancer metastasis. Anemia and severe hyponatremia were detected. Despite adequate fluid resuscitation, nonspecific symptoms including hypotension, tachycardia, hypoxia, fever and confusion occurred. Emergent CT revealed enlarged bilateral adrenal glands. Under the diagnosis of adrenal crisis due to metastatic infiltration of adrenal glands, the patient was treated with appropriate steroid replacement resulting in rapid improvement and recovery. Conclusion We describe a case of adrenal crisis caused by the lack of adrenal reserve based on metastatic involvement and surgical stress, the first published case of adrenal crisis after surgery for a pathologic fracture from lung cancer metastasis. Surgeons treating pathologic fractures should be aware of this complication and familiar with its appropriate therapy because of increasing opportunity to care patients with metastatic bone tumors due to recent advances in cancer treatment.

  19. The Age of Human Cerebral Cortex Neurons

    Energy Technology Data Exchange (ETDEWEB)

    Bhardwaj, R D; Curtis, M A; Spalding, K L; Buchholz, B A; Fink, D; Bjork-Eriksson, T; Nordborg, C; Gage, F H; Druid, H; Eriksson, P S; Frisen, J

    2006-04-06

    The traditional static view of the adult mammalian brain has been challenged by the realization of continuous generation of neurons from stem cells. Based mainly on studies in experimental animals, adult neurogenesis may contribute to recovery after brain insults and decreased neurogenesis has been implicated in the pathogenesis of neurological and psychiatric diseases in man. The extent of neurogenesis in the adult human brain has, however, been difficult to establish. We have taken advantage of the integration of {sup 14}C, generated by nuclear bomb tests during the Cold War, in DNA to establish the age of neurons in the major areas of the human cerebral cortex. Together with the analysis of the cortex from patients who received BrdU, which integrates in the DNA of dividing cells, our results demonstrate that whereas non-neuronal cells turn over, neurons in the human cerebral cortex are not generated postnatally at detectable levels, but are as old as the individual.

  20. Approach to the handling of adrenal insufficiency Manejo de la insuficiencia adrenal

    Directory of Open Access Journals (Sweden)

    Federico Uribe Londoño

    2005-03-01

    Full Text Available The term adrenal insufficiency refers to the hypofunction of this gland. From the etiologic point of view it may be either primary or secondary. This insufficiency manifests as inadequate serum levels of cortisol and adrenal androgens in the secondary form and of these and mineralocorticoids in the primary one. Clinical manifestations are often nonspecific and, consequently, diagnosis may be difficult to establish and treatment may be delayed with increased morbidity and mortality. This article on adrenal insufficiency includes its definition, epidemiology, etiology, physiopathology, classification, clinical presentation, diagnostic criteria and treatment guidelines. Besides, some special situations like critically ill patients and pregnant women are given special consideration. Emphasis is done on a diagnostic algorithm to make it easier for general practitioners the approach to patients with this endocrine disorder. La insuficiencia adrenal (ia se refiere a la hipofunción de dicha glándula debida a causas tanto primarias como secundarias, que resultan en niveles plasmáticos inadecuados de cortisol, andrógenos adrenales y adicionalmente, en la falla primaria de mineralocorticoides. Sus manifestaciones inespecíficas dificultan o retrasan con frecuencia el diagnóstico y tratamiento oportuno, lo cual incrementa la morbilidad y eventualmente la mortalidad de estos pacientes. Se incluyen en este artículo: definición, epidemiología, causas, fisiopatología, clasificación, manifestaciones clínicas, diagnóstico y tratamiento de la IA. Además se consideran algunas situaciones especiales como la IA en el paciente críticamente enfermo y en la mujer gestante. Finalmente se hace especial énfasis en un algoritmo diagnóstico, con la finalidad de facilitarle al médico general un enfoque ágil y oportuno de los pacientes con este problema endocrino.

  1. Laparoscopic Nephrectomy with Adrenalectomy for Synchronous Adrenal Myelolipoma and Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Kallappan Senthil

    2015-01-01

    Full Text Available Introduction. Adrenal myelolipomas are uncommon nonfunctioning tumors of the adrenal. Synchronous renal cell carcinomas with adrenal myelolipomas are very rare. We present the case report of adrenal myelolipoma with synchronous RCC managed laparoscopically. Case Report. A 60-year-old old gentleman presented with incidental right upper polar mass with right adrenal mass. Metastatic work-up was negative. Laparoscopic radical nephrectomy with adrenalectomy was done under general anesthesia. The biopsy report was right kidney clear cell adenocarcinoma (T1b with right adrenal myelolipoma. Conclusion. This is the first case report of laparoscopic adrenalectomy with nephrectomy for ipsilateral synchronous renal cell carcinoma with adrenal myelolipoma.

  2. Paraneoplastic (non-metastatic) adrenal insufficiency preceded the onset of primary lung cancer by 12 weeks.

    Science.gov (United States)

    Shantha, Ghanshyam Palamaner Subash; Kumar, Anita A; Jeyachandran, Vijay; Rajamanickam, Deepan; Bhaskar, Emmanuel; Paniker, Vinod K; Abraham, Georgi

    2009-01-01

    Clinically evident adrenal insufficiency associated with lung cancer is a rare entity. Among reported cases, adrenal insufficiency has occurred with or succeeded the primary lung cancer. Adrenal insufficiency has also been secondary to metastasis to the adrenal gland. The present report concerns a 61-year-old man, a chronic smoker, who presented to us with symptomatic adrenal insufficiency. He had no evidence of lung cancer during this visit. The primary lung cancer was only identified 12 weeks later. Additionally, his adrenals showed no evidence of metastasis. Hence his adrenal insufficiency had been a paraneoplastic manifestation of the lung cancer, and it had also preceded the primary by 12 weeks.

  3. The brain's code and its canonical computational motifs. From sensory cortex to the default mode network: A multi-scale model of brain function in health and disease.

    Science.gov (United States)

    Turkheimer, Federico E; Leech, Robert; Expert, Paul; Lord, Louis-David; Vernon, Anthony C

    2015-08-01

    A variety of anatomical and physiological evidence suggests that the brain performs computations using motifs that are repeated across species, brain areas, and modalities. The computational architecture of cortex, for example, is very similar from one area to another and the types, arrangements, and connections of cortical neurons are highly stereotyped. This supports the idea that each cortical area conducts calculations using similarly structured neuronal modules: what we term canonical computational motifs. In addition, the remarkable self-similarity of the brain observables at the micro-, meso- and macro-scale further suggests that these motifs are repeated at increasing spatial and temporal scales supporting brain activity from primary motor and sensory processing to higher-level behaviour and cognition. Here, we briefly review the biological bases of canonical brain circuits and the role of inhibitory interneurons in these computational elements. We then elucidate how canonical computational motifs can be repeated across spatial and temporal scales to build a multiplexing information system able to encode and transmit information of increasing complexity. We point to the similarities between the patterns of activation observed in primary sensory cortices by use of electrophysiology and those observed in large scale networks measured with fMRI. We then employ the canonical model of brain function to unify seemingly disparate evidence on the pathophysiology of schizophrenia in a single explanatory framework. We hypothesise that such a framework may also be extended to cover multiple brain disorders which are grounded in dysfunction of GABA interneurons and/or these computational motifs.

  4. 60 YEARS OF POMC: Adrenal and extra-adrenal functions of ACTH.

    Science.gov (United States)

    Gallo-Payet, Nicole

    2016-05-01

    The pituitary adrenocorticotropic hormone (ACTH) plays a pivotal role in homeostasis and stress response and is thus the major component of the hypothalamo-pituitary-adrenal axis. After a brief summary of ACTH production from proopiomelanocortin (POMC) and on ACTH receptor properties, the first part of the review covers the role of ACTH in steroidogenesis and steroid secretion. We highlight the mechanisms explaining the differential acute vs chronic effects of ACTH on aldosterone and glucocorticoid secretion. The second part summarizes the effects of ACTH on adrenal growth, addressing its role as either a mitogenic or a differentiating factor. We then review the mechanisms involved in steroid secretion, from the classical Cyclic adenosine monophosphate second messenger system to various signaling cascades. We also consider how the interaction between the extracellular matrix and the cytoskeleton may trigger activation of signaling platforms potentially stimulating or repressing the steroidogenic potency of ACTH. Finally, we consider the extra-adrenal actions of ACTH, in particular its role in differentiation in a variety of cell types, in addition to its known lipolytic effects on adipocytes. In each section, we endeavor to correlate basic mechanisms of ACTH function with the pathological consequences of ACTH signaling deficiency and of overproduction of ACTH.

  5. Caffeine-induced activated glucocorticoid metabolism in the hippocampus causes hypothalamic-pituitary-adrenal axis inhibition in fetal rats.

    Science.gov (United States)

    Xu, Dan; Zhang, Benjian; Liang, Gai; Ping, Jie; Kou, Hao; Li, Xiaojun; Xiong, Jie; Hu, Dongcai; Chen, Liaobin; Magdalou, Jacques; Wang, Hui

    2012-01-01

    Epidemiological investigations have shown that fetuses with intrauterine growth retardation (IUGR) are susceptible to adult metabolic syndrome. Clinical investigations and experiments have demonstrated that caffeine is a definite inducer of IUGR, as children who ingest caffeine-containing food or drinks are highly susceptible to adult obesity and hypertension. Our goals for this study were to investigate the effect of prenatal caffeine ingestion on the functional development of the fetal hippocampus and the hypothalamic-pituitary-adrenal (HPA) axis and to clarify an intrauterine HPA axis-associated neuroendocrine alteration induced by caffeine. Pregnant Wistar rats were intragastrically administered 20, 60, and 180 mg/kg · d caffeine from gestational days 11-20. The results show that prenatal caffeine ingestion significantly decreased the expression of fetal hypothalamus corticotrophin-releasing hormone. The fetal adrenal cortex changed into slight and the expression of fetal adrenal steroid acute regulatory protein (StAR) and cholesterol side-chain cleavage enzyme (P450scc), as well as the level of fetal adrenal endogenous corticosterone (CORT), were all significantly decreased after caffeine treatment. Moreover, caffeine ingestion significantly increased the levels of maternal and fetal blood CORT and decreased the expression of placental 11β-hydroxysteroid dehydrogenase-2 (11β-HSD-2). Additionally, both in vivo and in vitro studies show that caffeine can downregulate the expression of fetal hippocampal 11β-HSD-2, promote the expression of 11β-hydroxysteroid dehydrogenase 1 and glucocorticoid receptor (GR), and enhance DNA methylation within the hippocampal 11β-HSD-2 promoter. These results suggest that prenatal caffeine ingestion inhibits the development of the fetal HPA axis, which may be associated with the fetal overexposure to maternal glucocorticoid and activated glucocorticoid metabolism in the fetal hippocampus. These results will be beneficial in

  6. POD-1/Tcf21 overexpression reduces endogenous SF-1 and StAR expression in rat adrenal cells

    Directory of Open Access Journals (Sweden)

    M. M. França

    2015-12-01

    Full Text Available During gonad and adrenal development, the POD-1/capsulin/TCF21transcription factor negatively regulates SF-1/NR5A1expression, with higher SF-1 levels being associated with increased adrenal cell proliferation and tumorigenesis. In adrenocortical tumor cells, POD-1 binds to the SF-1 E-box promoter region, decreasing SF-1 expression. However, the modulation of SF-1 expression by POD-1 has not previously been described in normal adrenal cells. Here, we analyzed the basal expression of Pod-1 and Sf-1 in primary cultures of glomerulosa (G and fasciculata/reticularis (F/R cells isolated from male Sprague-Dawley rats, and investigated whether POD-1 overexpression modulates the expression of endogenous Sf-1 and its target genes in these cells. POD-1 overexpression, following the transfection of pCMVMycPod-1, significantly decreased the endogenous levels of Sf-1 mRNA and protein in F/R cells, but not in G cells, and also decreased the expression of the SF-1 target StAR in F/R cells. In G cells overexpressing POD-1, no modulation of the expression of SF-1 targets, StAR and CYP11B2, was observed. Our data showing that G and F/R cells respond differently to ectopic POD-1 expression emphasize the functional differences between the outer and inner zones of the adrenal cortex, and support the hypothesis that SF-1 is regulated by POD-1/Tcf21 in normal adrenocortical cells lacking the alterations in cellular physiology found in tumor cells.

  7. POD-1/Tcf21 overexpression reduces endogenous SF-1 and StAR expression in rat adrenal cells.

    Science.gov (United States)

    França, M M; Abreu, N P; Vrechi, T A M; Lotfi, C F

    2015-12-01

    During gonad and adrenal development, the POD-1/capsulin/TCF21transcription factor negatively regulates SF-1/NR5A1expression, with higher SF-1 levels being associated with increased adrenal cell proliferation and tumorigenesis. In adrenocortical tumor cells, POD-1 binds to the SF-1 E-box promoter region, decreasing SF-1 expression. However, the modulation of SF-1 expression by POD-1 has not previously been described in normal adrenal cells. Here, we analyzed the basal expression of Pod-1 and Sf-1 in primary cultures of glomerulosa (G) and fasciculata/reticularis (F/R) cells isolated from male Sprague-Dawley rats, and investigated whether POD-1 overexpression modulates the expression of endogenous Sf-1 and its target genes in these cells. POD-1 overexpression, following the transfection of pCMVMycPod-1, significantly decreased the endogenous levels of Sf-1 mRNA and protein in F/R cells, but not in G cells, and also decreased the expression of the SF-1 target StAR in F/R cells. In G cells overexpressing POD-1, no modulation of the expression of SF-1 targets, StAR and CYP11B2, was observed. Our data showing that G and F/R cells respond differently to ectopic POD-1 expression emphasize the functional differences between the outer and inner zones of the adrenal cortex, and support the hypothesis that SF-1 is regulated by POD-1/Tcf21 in normal adrenocortical cells lacking the alterations in cellular physiology found in tumor cells.

  8. Adaptations in Basal and Hypothalamic–Pituitary–Adrenal-Activated Deoxycorticosterone Responses Following Ethanol Self-administration in Cynomolgus Monkeys

    Science.gov (United States)

    Jimenez, Vanessa A.; Porcu, Patrizia; Morrow, A. Leslie; Grant, Kathleen A.

    2017-01-01

    Acute ethanol activates the hypothalamic–pituitary–adrenal (HPA) axis, while long-term exposure results in a blunted neuroendocrine state, particularly with regards to the primary endpoint, cortisol, the primary glucocorticoid produced in the adrenal cortex. However, it is unknown if this dampened neuroendocrine status also influences other adrenocortical steroids. Plasma concentration of the mineralocorticoid and neuroactive steroid precursor deoxycorticosterone (DOC) is altered by pharmacological challenges of the HPA axis in cynomolgus monkeys. The present study investigated HPA axis regulation of circulating DOC concentration over the course of ethanol (4% w/v) induction and self-administration in non-human primates (Macaca fasciculata, n = 10). Plasma DOC, measured by radioimmunoassay, was compared at baseline (ethanol naïve), during schedule-induced polydipsia, and following 6-months of 22 h/day access to ethanol and water. The schedule induction of ethanol drinking did not alter basal DOC levels but selectively dampened the DOC response to pharmacological challenges aimed at the anterior pituitary (ovine corticotrophin-releasing hormone) and adrenal gland (post-dexamethasone adrenocorticotropin hormone), while pharmacological inhibition of central opioid receptors with naloxone greatly enhanced the DOC response during induction. Following 6 months of ethanol self-administration, basal DOC levels were increased more than twofold, while responses to each of the challenges normalized somewhat but remained significantly different than baseline. These data show that HPA axis modulation of the neuroactive steroid precursor DOC is markedly altered by the schedule induction of ethanol drinking and long-term voluntary ethanol self-administration. The consequences of chronic ethanol consumption on HPA axis regulation of DOC point toward allostatic modification of hypothalamic and adrenal function. PMID:28220108

  9. Adrenal Function Status in Patients with Paracoccidioidomycosis after Prolonged Post-Therapy Follow-Up

    Science.gov (United States)

    Tobón, Angela M.; Agudelo, Carlos A.; Restrepo, Carlos A.; Villa, Carlos A.; Quiceno, William; Estrada, Santiago; Restrepo, Angela

    2010-01-01

    This study assessed adrenal function in patients with paracoccididioidomycosis who had been treated to determine a possible connection between high antibody titers and adrenal dysfunction attributable to persistence of the fungus in adrenal gland. Adrenal gland function was studied in 28 previously treated patients, 2 (7.1%) of whom were shown to have adrenal insufficiency and 7 (259%) who showed a below normal response to stimuli by adrenocorticotropic hormone. Paracoccidioides brasiliensis was detected in the adrenal gland from one of the patients with adrenal insufficiency. Although the study failed to demonstrate a significant difference between high antibody titers and low cortisol levels, the proportion of adrenal insufficiency detected and the subnormal response to adrenocorticotropic hormone confirmed that adrenal damage is an important sequela of paracoccidioidomycosis. Studies with a larger number of patients should be conducted to confirm the hypothesis of persistence of P. brasiliensis in adrenal gland after therapy. PMID:20595488

  10. Histologic and Immunohistochemical classification of 41 bovine adrenal gland neoplasms

    DEFF Research Database (Denmark)

    Grossi, Anette Blak; Leifsson, Páll S.; Jensen, Henrik Elvang;

    2013-01-01

    Tumors of the adrenal glands are among the most frequent tumors in cattle; however, few studies have been conducted to describe their characteristics. The aim of this study was to classify 41 bovine adrenal neoplasms from 40 animals based on macroscopic and histologic examination, including....... An immunohistochemistry panel consisting of antibodies against melan A, synaptophysin, and CNPase was considered most useful to classify bovine adrenal tumors. However, the distinction between benign and malignant adrenocortical tumors was based on histologic features as in human medicine....

  11. Right adrenal abscess -- an unusual complication of acute apendicitis.

    Science.gov (United States)

    Dimofte, Gabriel; Dubei, Liviu; Lozneanu, Lili-Gabriela; Ursulescu, Corina; Grigora Scedil, Mihai

    2004-09-01

    Acute appendicitis represents one of the most frequent abdominal emergencies encountered in everyday surgical practice. Local infectious complications are not unusual and retroperitoneal abscesses after acute retrocaecal appendicitis have been previously described. The authors present the case of a 22-years-young female patient, admitted for a right iliac fossa abscess, secondary to gangrenous appendicitis. A right adrenal mass 35/40 mm was revealed during preoperative ultrasound evaluation, which evolved in an adrenal abscess that spontaneously drained 10 days after appendectomy and retrocecal drainage. Adrenal abscesses are exceptionally rare, with only a few cases being reported in the literature, but none of these after acute appendicitis.

  12. Early intervention and management of adrenal insufficiency in children.

    LENUS (Irish Health Repository)

    Moloney, Sinéad

    2012-09-01

    The endocrine disorder adrenal insufficiency includes inadequate production of the steroid hormone cortisol. This results in poor physiological responses to illness, trauma or other stressors and risk of adrenal crisis. Management is based on administration of hydrocortisone. It is important to avoid under- or over-treatment and increase the dosage during times of physiological stress. To reduce morbidity, hospital admissions and mortality, the education and empowerment of parents and carers, and prompt intervention when necessary are essential. A steroid therapy card for adrenal insufficiency containing personal information on a patient\\'s condition was developed for use by families and their specialist centres.

  13. Intense Adrenal Enhancement: A CT Feature of Cardiogenic Shock

    Energy Technology Data Exchange (ETDEWEB)

    Hrabak-Paar, Maja, E-mail: maja.hrabak.paar@mef.hr [University of Zagreb School of Medicine, Department of Diagnostic and Interventional Radiology, University Hospital Center Zagreb (Croatia)

    2016-02-15

    In this report, images of intense adrenal enhancement in a 79-year-old female patient with right-sided heart failure and severe tricuspid insufficiency are presented. Only two cases of intense adrenal enhancement as a sign of cardiogenic shock were previously reported in the literature. Intense adrenal enhancement could be one of the earliest CT signs of cardiogenic shock. Its presence should be immediately reported to the referring physician as a sign of significant hemodynamic instability warranting early critical-care management.

  14. Intense Adrenal Enhancement: A CT Feature of Cardiogenic Shock.

    Science.gov (United States)

    Hrabak-Paar, Maja

    2016-02-01

    In this report, images of intense adrenal enhancement in a 79-year-old female patient with right-sided heart failure and severe tricuspid insufficiency are presented. Only two cases of intense adrenal enhancement as a sign of cardiogenic shock were previously reported in the literature. Intense adrenal enhancement could be one of the earliest CT signs of cardiogenic shock. Its presence should be immediately reported to the referring physician as a sign of significant hemodynamic instability warranting early critical-care management.

  15. Oxytocin and hypothalamo-pituitary- adrenal axis

    Directory of Open Access Journals (Sweden)

    Berrak Ç. Yeğen

    2010-05-01

    Full Text Available BSTRACT: Upon exposure to different types of stressors, neuroendocrine and behavioral responses that include the activation of the hypothalamus-pituitary-adrenal (HPA axis are given to allow the individuals to cope with stress conditions. It was proven that oxytocin, anonapeptide released from the posterior pituitary, has behavioral and stress-attenuating effects by dampening HPA activity. On the other hand, the neuropeptide was also shown to exert anti-inflammatory effects through the modulation of immune and inflammatory processes in several experimental models of tissue injury. The findings of recent studies suggest that the anti-inflammatory effect of oxytocin depends on its role on HPA axis activity and subsequent release of cortisol. Thus, oxytocin seems to restrain the activity within the HPA-axis, which becomes overactive during many inflammatory processes

  16. Sphingosine-1-phosphate lyase mutations cause primary adrenal insufficiency and steroid-resistant nephrotic syndrome

    Science.gov (United States)

    Prasad, Rathi; Hadjidemetriou, Irene; Meimaridou, Eirini; Buonocore, Federica; Saleem, Moin; Hurcombe, Jenny; Bierzynska, Agnieszka; Barbagelata, Eliana; Bergadá, Ignacio; Cassinelli, Hamilton; Das, Urmi; Krone, Ruth; Hacihamdioglu, Bulent; Sari, Erkan; Yesilkaya, Ediz; Storr, Helen L.; Clemente, Maria; Fernandez-Cancio, Monica; Camats, Nuria; Ram, Nanik; Achermann, John C.; Van Veldhoven, Paul P.; Guasti, Leonardo; Braslavsky, Debora; Guran, Tulay; Metherell, Louise A.

    2017-01-01

    Primary adrenal insufficiency is life threatening and can present alone or in combination with other comorbidities. Here, we have described a primary adrenal insufficiency syndrome and steroid-resistant nephrotic syndrome caused by loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1). SGPL1 executes the final decisive step of the sphingolipid breakdown pathway, mediating the irreversible cleavage of the lipid-signaling molecule sphingosine-1-phosphate (S1P). Mutations in other upstream components of the pathway lead to harmful accumulation of lysosomal sphingolipid species, which are associated with a series of conditions known as the sphingolipidoses. In this work, we have identified 4 different homozygous mutations, c.665G>A (p.R222Q), c.1633_1635delTTC (p.F545del), c.261+1G>A (p.S65Rfs*6), and c.7dupA (p.S3Kfs*11), in 5 families with the condition. In total, 8 patients were investigated, some of whom also manifested other features, including ichthyosis, primary hypothyroidism, neurological symptoms, and cryptorchidism. Sgpl1–/– mice recapitulated the main characteristics of the human disease with abnormal adrenal and renal morphology. Sgpl1–/– mice displayed disrupted adrenocortical zonation and defective expression of steroidogenic enzymes as well as renal histology in keeping with a glomerular phenotype. In summary, we have identified SGPL1 mutations in humans that perhaps represent a distinct multisystemic disorder of sphingolipid metabolism. PMID:28165343

  17. A Case Report of Adrenocortical Adenoma Mimicking Congenital Adrenal Hyperplasia in a Young Girl.

    Science.gov (United States)

    Sheng, Qingfeng; Lv, Zhibao; Xu, Weijue; Liu, Jiangbin; Wu, Yibo; Xi, Zhengjun

    2015-06-01

    Adrenal cortical tumors are rare in children. Secondary tumors associated with untreated congenital adrenal hyperplasia (CAH) have also been reported in pediatric population. It is difficult for pediatricians to differentiate these 2 lesions.We described a 4.5-year-old girl who presented with symptoms and signs of virilization. Bone age was 9.5 years. Genetic analysis of CYP21A2 and CYP11B1 revealed a heterozygous mutation of CYP11B1 at c.1157C>T (A386V). No germline p53 gene mutation including R337H was detected.The patient was first misdiagnosed as CAH and treated with hydrocortisone for 3 months. Diagnosis of an adrenal cortical tumor was confirmed by laboratory data and abdominal computed tomography. After resection of the tumor, serum steroids normalized and clinical signs receded. The child received no additional treatment and remains disease free after 12 months of close observation. Histological examination showed neoplasia cells with predominantly eosinophilic cytoplasm and few atypical mitotic figures. The proliferation-associated Ki-67 index was <1% detected by immunohistochemistry.Neoplasm is a rare but significant cause of precocious puberty (PP). The possibility of neoplasms should always be considered early to avoid delayed cancer diagnosis and treatment in cases of PP.

  18. Recent advances in diagnosis, treatment, and outcome of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

    Science.gov (United States)

    Riepe, Felix G; Sippell, Wolfgang G

    2007-12-01

    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is an autosomal-recessive disease causing cortisol deficiency, aldosterone deficiency and hyperandrogenism. Diagnosis of 21-OHD is confirmed by steroid analysis in newborn screening or later on. Standard medical treatment consists of oral glucocorticoid and mineralocorticoid administration in order to suppress adrenal androgens and to compensate for adrenal steroid deficiencies. However, available treatment is far from ideal, and not much is known about the long-term outcome in CAH as trials in patients in adulthood or old age are rare. Here we briefly describe the pathophysiology, clinical picture, genetics and epidemiology of 21-OHD. This is followed by a comprehensive review of the recent advances in diagnosis, treatment and outcome. Novel insights have been gained in the fields of newborn screening, specific steroid measurement utilizing mass spectrometry, genetics, glucocorticoid stress dosing, additive medical therapy, prenatal treatment, side-effects of medical treatment, adrenomedullary involvement, metabolic morbidity, fertility and gender identity. However, many issues are still unresolved, and novel questions, which will have to be answered in the future, arise with every new finding.

  19. Complications of extrahepatic echinococcosis:Fistulization of an adrenal hydatid cyst into the intestine

    Institute of Scientific and Technical Information of China (English)

    Juan Francisco Ruiz-Rabelo; Manuel Gomez-Alvarez; Joaquin Sanchez-Rodriguez; Sebastian Rufian Pe(n)a

    2008-01-01

    Echinococcal cysts are usually found in liver and lungs,but any other organ can potentially be involved.Extrahepatic disease due to hydatid cyst may develop in the abdominal and pelvic cavity,aside from in other less common locations,which may make both diagnosis and treatment more complex.We present a rare case of extrahepatic echinococcosis in a 70-year old patient with a 4-d history of dull abdominal pain,anemia within the transfusion range and fever.She underwent surgery for left renal hydatid cysts 30 years ago.After non operative treatment,imaging studies showed a calcified hydatid cyst in a retrogastric location communicating with a proximal jejunal loop.En-block resection of the mass together with the adrenal gland was performed including closure of the enteric fistula.Anatomic pathology confirmed the diagnosis of a calcified hydatid cyst of left adrenal origin.Surgery is the treatment of choice and most authorsrecommend removal of cyst and adrenal gland.

  20. Cyberknife fractionated radiotherapy for adrenal metastases: Preliminary report from a multispecialty Indian cancer care center

    Directory of Open Access Journals (Sweden)

    Trinanjan Basu

    2015-03-01

    Full Text Available Purpose: Metastasis to adrenal gland from lung, breast, and kidney malignancies are quite common. Historically radiotherapy was intended for pain palliation. Recent studies with stereotactic body radiotherapy (SBRT including Cyberknife robotic radiosurgery aiming at disease control brings about encouraging results. Here we represent the early clinical experience with Cyberknife stereotactic system from an Indian cancer care center. The main purpose of this retrospective review is to serve as a stepping stone for future prospective studies with non- invasive yet effective technique compared to surgery. Methods: We retrospectively reviewed four cases of adrenal metastases (three: lung and one: renal cell carcinoma treated with Cyberknife SBRT. X sight spine tracking was employed for planning and treatment delivery. Patients were evaluated for local response clinically as well as with PETCT based response criteria.Results: With a median gross tumor volume of 20.5 cc and median dose per fraction of 10 Gy, two patients had complete response (CR and two had partial response (PR when assessed 8-12 weeks post treatment as per RECIST. There was no RTOG grade 2 or more acute adverse events and organs at risk dosage were acceptable. Till last follow up all the patients were locally controlled and alive. Conclusion: Cyberknife SBRT with its unique advantages like non- invasive, short duration outpatient treatment technique culminating in similar local control rates in comparison to surgery is an attractive option. World literature of linear accelerator based SBRT and our data with Cyberknife SBRT with small sample size and early follow up are similar in terms of local control in adrenal metastases. Future prospective data would reveal more information on the management of adrenal metastases.

  1. Paraneoplastic (non-metastatic) adrenal insufficiency preceded the onset of primary lung cancer by 12 weeks

    OpenAIRE

    Shantha, Ghanshyam Palamaner Subash; Kumar, Anita A; Jeyachandran, Vijay; Rajamanickam, Deepan; Bhaskar, Emmanuel; Paniker, Vinod K; Abraham, Georgi

    2009-01-01

    Clinically evident adrenal insufficiency associated with lung cancer is a rare entity. Among reported cases, adrenal insufficiency has occurred with or succeeded the primary lung cancer. Adrenal insufficiency has also been secondary to metastasis to the adrenal gland. The present report concerns a 61-year-old man, a chronic smoker, who presented to us with symptomatic adrenal insufficiency. He had no evidence of lung cancer during this visit. The primary lung cancer was only identified 12 wee...

  2. Prefrontal cortex glutamate and extraversion.

    Science.gov (United States)

    Grimm, Simone; Schubert, Florian; Jaedke, Maren; Gallinat, Jürgen; Bajbouj, Malek

    2012-10-01

    Extraversion is considered one of the core traits of personality. Low extraversion has been associated with increased vulnerability to affective and anxiety disorders. Brain imaging studies have linked extraversion, approach behaviour and the production of positive emotional states to the dorsolateral prefrontal cortex (DLPFC) and glutamatergic neurotransmission. However, the relationship between extraversion and glutamate in the DLPFC has not been investigated so far. In order to address this issue, absolute glutamate concentrations in the DLPFC and the visual cortex as a control region were measured by 3-Tesla proton magnetic resonance spectroscopy (1H-MRS) in 29 subjects with high and low extraversion. We found increased glutamate levels in the DLPFC of introverts as compared with extraverts. The increased glutamate concentration was specific for the DLPFC and negatively associated with state anxiety. Although preliminary, results indicate altered top-down control of DLPFC due to reduced glutamate concentration as a function of extraversion. Glutamate measurement with 1H-MRS may facilitate the understanding of biological underpinnings of personality traits and psychiatric diseases associated with dysfunctions in approach behaviour and the production of positive emotional states.

  3. The novel multi-target iron chelator, M30 modulates HIF-1α-related glycolytic genes and insulin signaling pathway in the frontal cortex of APP/PS1 Alzheimer's disease mice.

    Science.gov (United States)

    Mechlovich, Danit; Amit, Tamar; Bar-Am, Orit; Mandel, Silvia; Youdim, Moussa B H; Weinreb, Orly

    2014-02-01

    Increasing evidence suggests that dysregulation of brain insulin/insulin receptor (InsR) and insulin signaling cascade are associated with the pathogenesis of Alzheimer's disease (AD). Our group has designed and synthesized a series of multi-target iron chelating, brain permeable compounds for AD. One leading multi-target compound, M30 possesses the neuroprotective N-propargyl moiety of the anti-Parkinsonian, monoamine oxidase (MAO)-B inhibitor, rasagiline (Azilect®) and the antioxidant-iron chelating moiety of an 8-hydroxyquinoline derivative of the iron chelator, VK28. Positive outcomes for the behavioral/cognitive and neuroprotective effects of M30 were recently obtained in preclinical experimental studies, regarding pathological aspects relevant to ageing and AD. We report that chronic treatment with M30 (1 and 5 mg/kg p.o; three times a week for 9 months) significantly elevated cortical insulin and InsR transcript and protein expression, respectively and increased the phosphorylated form of glycogen synthase kinase-3β in the frontal cortex of amyloid precursor protein (APP) and presenilin 1 (PS1) double transgenic mice. In addition, M30 treatment upregulated the levels of hypoxia-inducible factor (HIF)-1α and expression of its target genes involved in glycolysis including, aldolase A, enolase-1 and glucose transporter-1 (Glut-1), in the frontal cortex of APP/PS1 mice. Treatment with M30 also lead to an increase in the hepatic protein expression levels of InsR and Glut-1 and lowered the increase in blood glucose levels following glucose tolerance test. The present findings indicate that the multifunctional iron chelating drug, M30 regulates major brain glucose metabolism parameters and thus, might be beneficial for AD, in which impaired neuronal insulin signaling and Glut expression have been implicated.

  4. Testicular adrenal rest tumors in adult males with congenital adrenal hyperplasia: evaluation of pituitary-gonadal function before and after successful testis-sparing surgery in eight patients.

    NARCIS (Netherlands)

    Claahsen-van der Grinten, H.L.; Otten, B.J.; Takahashi, S.; Meuleman, E.J.H.; Hulsbergen- van de Kaa, C.A.; Sweep, C.G.J.; Hermus, A.R.M.M.

    2007-01-01

    CONTEXT: In male patients with congenital adrenal hyperplasia (CAH), testicular adrenal rest tumors (TART) are frequently present. These tumors can interfere with testicular function. Intensifying glucocorticoid therapy does not always lead to tumor regression and improvement of testicular function.

  5. Acute adrenal insufficiency due to primary antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Kishore Kumar Behera

    2013-01-01

    Full Text Available Introduction: We report a case of acute adrenal insufficiency (AAI in a patient with antiphospholipid syndrome (APS. Case Report: A 44-year-old female patient presented to us with acute abdominal pain associated with recurrent vomiting and giddiness. On examination, her blood pressure was 80/50 mm Hg. Systemic examination was normal. Further evaluation revealed hypocortisolemia with elevated plasma adrenocorticotropin hormone indicative of primary adrenal insufficiency. Her abdominal computed tomography scan showed features of evolving bilateral adrenal infarction. Etiological work-up revealed prolonged activated thromboplastin time, which didn′t correct with normal plasma, her anti-cardiolipin antibody and lupus anticoagulant were also positive. She was diagnosed to have APS with adrenal insufficiency and she was started on intravenous steroids and heparin infusion. Conclusion: AAI due to the APS can present with acute abdominal pain followed by hypotension. A high index of suspicion is needed to make the correct diagnosis and to initiate appropriate treatment.

  6. The role of imaging in congenital adrenal hyperplasia

    Energy Technology Data Exchange (ETDEWEB)

    Teixeira, Sara Reis; Andrade, Marco Tulio Soares; Melo, Andrea Farias; Elias Junior, Jorge, E-mail: jejunior@fmrp.usp.br [Department of Internal Medicine, Division of Radiology, Clinical Hospital, Ribeirao Preto Medical School, University of Sao Paulo (FMRP-USP), Ribeirao Preto, SP (Brazil); Elias, Paula Condé Lamparelli [Department of Internal Medicine, Division of Endocrinology, Clinical Hospital, FMRP-USP, Ribeirao Preto, SP (Brazil)

    2014-10-15

    Congenital adrenal hyperplasia (CAH) is an autossomic recessive disorder caused by impaired steroidogenesis. Patients with CAH may present adrenal insufficiency with or without salt-wasting, as well as various degrees of virilization and fertility impairment, carrying a high incidence of testicular adrenal rest tumors and increased incidence of adrenal tumors. The diagnosis of CAH is made based on the adrenocortical profile hormonal evaluation and genotyping, in selected cases. Follow-up is mainly based on hormonal and clinical evaluation. Utility of imaging in this clinical setting may be helpful for the diagnosis, management, and follow-up of the patients, although recommendations according to most guidelines are weak when present. Thus, the authors aimed to conduct a narrative synthesis of how imaging can help in the management of patients with CAH, especially focused on genitography, ultrasonography, computed tomography, and magnetic resonance imaging. (author)

  7. Unilateral pulmonary edema during laparoscopic resection of adrenal tumor

    Science.gov (United States)

    Prakash, Smita; Nayar, Pavan; Virmani, Pooja; Bansal, Shipra; Pawar, Mridula

    2015-01-01

    Despite technological, therapeutic and diagnostic advancements, surgical intervention in pheochromocytoma may result in a life-threatening situation. We report a patient who developed unilateral pulmonary edema during laparoscopic resection of adrenal tumor. PMID:26330724

  8. Unilateral pulmonary edema during laparoscopic resection of adrenal tumor

    Directory of Open Access Journals (Sweden)

    Smita Prakash

    2015-01-01

    Full Text Available Despite technological, therapeutic and diagnostic advancements, surgical intervention in pheochromocytoma may result in a life-threatening situation. We report a patient who developed unilateral pulmonary edema during laparoscopic resection of adrenal tumor.

  9. Development of the adrenal axis in the neonatal rat

    Energy Technology Data Exchange (ETDEWEB)

    Guillet, Ronnie

    1977-01-01

    Plasma corticosterone and ACTH concentrations were determined in neonatal rats 1, 7, 14, and 21 days old, under a variety of experimental conditions, to obtain more information on the postnatal development of the rat hypothalamo-adrenal (HHA) axis. The results indicate that: (1) there is a diminution followed by an increase in responsiveness of the adrenal gland, but the pituitary response to direct hormonal stimulation is unchanged during the first three postnatal weeks; (2) continued stimulation of the adrenal by ACTH or of the central nervous system (CNS) or hypothalamus by corticosterone is necessary during early postnatal development to allow normal maturation of the HHA axis; and (3) feedback inhibition is operative by birth, at least to a moderate degree. Taken together, the studies suggest that both the adrenal and pituitary glands are potentially functional at birth, but that the hypothalamic and CNS mediators of the stress response are not mature until at least the second or third postnatal week. (ERB)

  10. Volume of the adrenal and pituitary glands in depression

    DEFF Research Database (Denmark)

    Kessing, Lars Vedel; Willer, Inge Stoel; Knorr, Ulla

    2011-01-01

    Numerous studies have shown that the hypothalamic-pituitary-adrenal (HPA) axis is hyperactive in some depressed patients. It is unclear whether such hyperactivity results in changed volumes of the adrenal glands, pituitary gland and hypothalamus. We systematically reviewed all controlled studies...... on the adrenal or pituitary glands or hypothalamus volume in unipolar depressive disorder published in PubMed 1966 to December 2009. We identified three studies that investigated the volume of the adrenal glands and eight studies that examined the volume of the pituitary gland, but no studies on hypothalamus...... identified such as small population samples, different subtypes of depression and insufficient matching of patients and controls. Due to large heterogeneity of study designs and data, it was futile to make a meta-analysis. It is concluded that it remains unclear whether hyperactivity of the HPA axis results...

  11. [Neuroanatomy of Frontal Association Cortex].

    Science.gov (United States)

    Takada, Masahiko

    2016-11-01

    The frontal association cortex is composed of the prefrontal cortex and the motor-related areas except the primary motor cortex (i.e., the so-called higher motor areas), and is well-developed in primates, including humans. The prefrontal cortex receives and integrates large bits of diverse information from the parietal, temporal, and occipital association cortical areas (termed the posterior association cortex), and paralimbic association cortical areas. This information is then transmitted to the primary motor cortex via multiple motor-related areas. Given these facts, it is likely that the prefrontal cortex exerts executive functions for behavioral control. The functional input pathways from the posterior and paralimbic association cortical areas to the prefrontal cortex are classified primarily into six groups. Cognitive signals derived from the prefrontal cortex are conveyed to the rostral motor-related areas to transform them into motor signals, which finally enter the primary motor cortex via the caudal motor-related areas. Furthermore, it has been shown that, similar to the primary motor cortex, areas of the frontal association cortex form individual networks (known as "loop circuits") with the basal ganglia and cerebellum via the thalamus, and hence are extensively involved in the expression and control of behavioral actions.

  12. First evidence of overlaps between HIV-Associated Dementia (HAD and non-viral neurodegenerative diseases: proteomic analysis of the frontal cortex from HIV+ patients with and without dementia

    Directory of Open Access Journals (Sweden)

    Kapur Amit

    2010-06-01

    Full Text Available Abstract Background The pathogenesis of HIV-associated dementia (HAD is poorly understood. To date, detailed proteomic fingerprinting directly from autopsied brain tissues of HAD and HIV non-dementia patients has not been performed. Result Here, we have analyzed total proteins from the frontal cortex of 9 HAD and 5 HIV non-dementia patients. Using 2-Dimensional differential in-gel electrophoresis (2-DIGE to analyze the brain tissue proteome, 76 differentially expressed proteins (p 1.25 were identified between HAD and HIV non-dementia patients, of which 36 protein spots (based on 3D appearance of spots on the images were chosen for the mass spectrometry analysis. The large majority of identified proteins were represented in the energy metabolic (mitochondria and signal transduction pathways. Furthermore, over 90% of the protein candidates are common to both HAD and other non-viral neurodegenerative disease, such as Alzheimer's disease. The data was further validated using specific antibodies to 4 proteins (CA2, GS, CKMT and CRMP2 by western blot (WB in the same samples used for 2D-DIGE, with additional confirmation by immunohistochemitsry (IHC using frontal lobe tissue from different HAD and HIV+ non-dementia patients. The validation for all 4 antibodies by WB and IHC was in concordance with the DIGE results, lending further credence to the current findings. Conclusion These results suggest not only convergent pathogenetic pathways for the two diseases but also the possibility of increased Alzheimer's disease (AD susceptibility in HAD patients whose life expectancy has been significantly increased by highly active antiretroviral therapy.

  13. Stage 4S Bilateral Adrenal Neuroblastoma in a Newborn

    Directory of Open Access Journals (Sweden)

    Rahul Gupta

    2014-02-01

    Full Text Available Stage 4S bilateral adrenal neuroblastoma presenting in the neonatal period is extremely rare. A 1-day-old male with 4S bilateral adrenal neuroblastoma complicated by marked hepatomegaly managed by chemotherapy is being reported. The provisional diagnosis of neuroblastoma was made in the fetal life during the last trimester of pregnancy. Cardiomyopathy due to doxorubicin cytotoxicity developed over ensuing years, which is being treated.

  14. Simultaneous adrenal pheochromocytoma and carotid body paraganglioma in a woman

    Energy Technology Data Exchange (ETDEWEB)

    Han, Eun Ji; Lee, Sang Hoon; Song, In Uk; Chung, Yong An; Maeng, Lee So [The Catholic Univ. of Korea, Incheon (Korea, Republic of)

    2012-03-15

    Simultaneous occurrence of carotid body tumor and pheochromocytoma is rare. Most pheochromocytomas have grown on adrenal medulla, but some of the pheochromocytoma patients have multifocal paragangliomas arising from extraaderenal tissues. Pheochromocytomas and paragangliomas occur as sporadic tumors or they can be associated with several hereditary syndromes such as (1) multiple endocrine neoplasia type 2 (MEN 2), (2) Von Hippel Lindau disease (VHL) and (3) neurofibromatosis type 1 as an unusual genetic cause of pheochromocytomas. Genetic testing is recommended for patients with an apparently sporadic pheochromocytoma under the age of 20 years with a family history or features suggestive of hereditary pheochromocytoma or for patients with sympathetic paragangliomas. For individuals who do not meet these criteria, genetic testing is optional. Discovery of pheochromocytoma or paraganglioma in a patient should lead to a careful search to rule out multifocal lesions and/or hereditary syndromes. The diagnosis of pheochromocytoma and paraganglioma is made by biochemical testing, and imaging is done to localize the tumor for surgical planning. F 18 FDG PET has proved to be an effective tool in the localization of pheochromocytomas and paragangliomas.

  15. Assembling of ({beta}LPH) beta-lypothrophine radioimmunoassay. Plasma levels standardization in normal individuals and patients with hypophysis and adrenals diseases; Montagem do radioimunoensaio da beta-lipotrofina ({beta}LPH). Padronizacao dos niveis plasmaticos em individuos normais e em pacientes com doencas hipofisarias e adrenais

    Energy Technology Data Exchange (ETDEWEB)

    Castro, Margaret de

    1988-12-31

    The present study investigates the extraction and radioimmunoassay (RIA) conditions of plasma {beta}LPH. It was extracted by the activated silicic acid method, with a mean extraction efficiency of 31.6% and a mean intra-extraction variation coefficient of 8.1%. Radioiodination was performed by the chloramine-T method and {beta}LPH {sup 125} I was purified by gel chromatography on Sephadex G100. Estimated specific activity ranged from 100 to 192.8 {mu}Ci/{mu}g, with a mean incorporation percentage of 66.6%. The titer of the first antibody was 1:50.000/100 {mu}l. The assay was performed under non-equilibrium conditions, with a pre-incubation period of 24 hours and incubation of 4 hours. Mean immunoreactivity (Bo/Total) was 21.1%, with a mean Blank/Total ratio of 2.3%. Sensitivity, expressed as the mean minimum detectable dose, was 40 pg/tube, equivalent to 56 pg/ml plasma. Intra-assay variation coefficients were 6.5%, 3.8% and 6.8%, respectively, at B/Bo levels of 0.8, 0.6 and 0.4 of the standard curve. At B/Bo equal to 0.5, the intra-assay variation coefficient was 20.9%. Replicates of 14 plasma samples showed a correlation coefficient of r 0.99, (p< 0.05). Parallelism between the curve obtained with different volumes of an extract with a high {beta}LPH value and the standard curve was found. The method was controlled biologically by the presence of correlation between the plasma {beta}LPH levels and determined pathological states and with clinical functional studies. Twenty seven normal individuals, 10 patients with Cushing`s disease to a tumor of the hypophysis, 4 patients with Cushing syndrome due to an adrenal tumor, 10 patients Addison disease, and 8 patients with hypopituitarism were studied. (author). 119 refs., 28 figs., 2 tabs.

  16. Endogenous Cushing's Syndrome with Precocious Puberty in an 8-Year-Old Boy due to a Large Unilateral Adrenal Adenoma

    Science.gov (United States)

    Hossain, Muhammad Rajib; Alam, Md. Mashiul; Nabi, Junaid; Kibria, Mahzabin

    2013-01-01

    Adrenocortical tumors (ACTs) causing Cushing's syndrome are extremely rare in children and adolescents. Bilateral macronodular adrenocortical disease which is a component of the McCune-Albright syndrome is the most common cause of endogenous Cushing's syndrome. We report the case of a boy with Cushing's syndrome who presented with obesity and growth retardation. The child was hypertensive. The biochemical evaluation revealed that his serum cortisol levels were 25.80 μg/dL, with a concomitant plasma ACTH level of 10.0 pg/mL and nonsuppressed serum cortisol on high-dose dexamethasone suppression test (HDDST) to be 20.38 μg/dL. Computed tomography of the abdomen demonstrated a 8 × 6 × 5 cm left adrenal mass with internal calcifications. Following preoperative stabilization, laparotomy was carried out which revealed a lobulated left adrenal mass with intact capsule weighing 120 grams. Histopathological examination revealed a benign cortical neoplastic lesion, suggestive of adrenal adenoma; composed of large polygonal cells with centrally placed nuclei and prominent nucleoli without capsular and vascular invasion. On the seventh postoperative day, cortisol levels were within normal range indicating biochemical remission of Cushing's syndrome. On followup after three months, the patient showed significant clinical improvement and had lost moderate amount of weight and adrenal imaging was found to be normal. PMID:23533838

  17. Current practice of glucocorticoid replacement therapy and patient-perceived health outcomes in adrenal insufficiency - a worldwide patient survey

    Directory of Open Access Journals (Sweden)

    Forss M

    2012-06-01

    Full Text Available Abstract Background The aim was to survey current practice in glucocorticoid replacement therapy and self-perceived health outcomes in patients with adrenal insufficiency. Methods Participants were recruited via patient organizations to respond anonymously to a web-based survey developed by clinical experts. Unique entries were set up for each patient organization enabling geographical localization of the entries. Results 1245 participants responded (primary adrenal insufficiency: 84%; secondary adrenal insufficiency: 11%; unsure: 5%. Therapies included hydrocortisone (75%, prednisone/prednisolone (11%, cortisone acetate (6% and dexamethasone (4%. Dosing regimens were once daily (10%, twice daily (42%, thrice daily (32% or other (17%. Compromised subjective health necessitating changes to physical activity or social-, work- or family life was reported by 64% of the participants. 40% of the participants reported absence from work/school in the last 3 months. Irrespective of diagnosis, 76% were concerned about long-term side-effects of therapy, mainly osteoporosis (78%, obesity (64% and cardiovascular morbidity (46%. 38% of the participants had been hospitalized in the last year. Conclusions Glucocorticoid replacement therapy among the respondents consisted primarily of hydrocortisone administered twice or thrice daily. A majority reported impact of their disease or treatment on subjective health requiring alterations in e.g. physical activity or family life. Three quarters reported concerns about long-term side-effects of the treatment. These data demonstrate - from the patients' perspective - a need for improvement in the management of adrenal insufficiency.

  18. Neural plasticity occurs in the adrenal medulla of asthmatic rats

    Institute of Scientific and Technical Information of China (English)

    FENG Jun-tao; LI Xiao-zhao; HU Cheng-ping; WANG Jun; NIE Hua-ping

    2010-01-01

    Background Airway symptoms in asthma are related to decrease of epinephrine secretion, which may be ascribed to elevated nerve growth factor (NGF) in the organism.The aim of this study was to monitor the neuroendocrine alteration in the adrenal medulla of asthmatic rats.Methods Sixteen rats were randomly divided into two groups (n=8), control group and asthma group, and the asthmatic rats were sensitized and challenged with ovalbumin (OVA).The levels of NGF, epinephrine and norepinephrine in serum were detected by enzyme linked immunosorbent assay (ELISA), the NGF expression in adrenal medulla was detected by immunohistochemistry, and the changes in the ultrastructure of the adrenal medulla was observed by electron microscopy.Results The NGF expression was increased in asthmatic rats compared with control rats.Compared with control rats,the results indicated that the epinephrine level was decreased in asthmatic rats, but no significant difference was found in norepinephrine levels.We found more ganglion cells in the adrenal medulla of asthmatic rats than in control rats, with NGF immunostaining mainly located in these ganglion cells.Electron microscopic images showed the density of chromaffin granula decreased and there was shrunken nucleolemma in the adrenal medullary cells of asthmatic rats.Conclusion The innervation of the adrenal medulla is changed in asthmatic rats, and it may contribute to the epinephrine decrease in asthma.

  19. Acute adrenal insufficiency: an aide-memoire of the critical importance of its recognition and prevention.

    Science.gov (United States)

    Gargya, A; Chua, E; Hetherington, J; Sommer, K; Cooper, M

    2016-03-01

    Adrenal crisis is a life-threatening emergency that causes significant excess mortality in patients with adrenal insufficiency. Delayed recognition by medical staff of an impending adrenal crisis and failure to give timely hydrocortisone therapy within the emergency department continue to be commonly encountered, even in metropolitan teaching hospitals. Within the authors' institutions, several cases of poorly handled adrenal crises have occurred over the last 2 years. Anecdotal accounts from members of the Addison's support group suggest that these issues are common in Australia. This manuscript is a timely reminder for clinical staff on the critical importance of the recognition, treatment and prevention of adrenal crisis. The manuscript: (i) outlines a case and the clinical outcome of sub-optimally managed adrenal crisis, (ii) summarises the clinical features and acute management of adrenal crisis, (iii) provides recommendations on the prevention of adrenal crisis and (iv) provides guidance on the management of 'sick days' in patients with adrenal insufficiency.

  20. Adiponectin and adiponectin receptor system in the rat adrenal gland: ontogenetic and physiologic regulation, and its involvement in regulating adrenocortical growth and steroidogenesis.

    Science.gov (United States)

    Paschke, Lukasz; Zemleduch, Tomasz; Rucinski, Marcin; Ziolkowska, Agnieszka; Szyszka, Marta; Malendowicz, Ludwik K

    2010-09-01

    Adiponectin (ADN) is a regulatory peptide secreted mostly by adipose tissue and acting via two receptors: AdipoR1 and AdipoR2. Our aim was to investigate expression of adiponectin system genes in the rat adrenal gland as well as its ontogenetic and physiological control. Furthermore, we examined the effects of acute and prolonged activation of HPA axis on ADN system in adipose tissue. By means of QPCR, ADN and AdipoR1 expression was demonstrated in rat adrenal cortex both at mRNA and protein levels, while AdipoR2 could only be detected at mRNA levels. ADN expression level was significantly upregulated in a developing and regenerating adrenal cortex. Globular domain of adiponectin at 10(-9) M stimulated corticosterone output and BrdU incorporation by cultured rat adrenocortical cells. Moreover, both acute (ACTH and ether stress) and prolonged (ACTH) adrenal stimulation resulted in lowered ADN levels, while expression of AdipoR1 and AdipoR2 was upregulated by the acute treatment. Depending on its site of origin, visceral (VAT) or subcutaneous (SAT) adipose tissue responded differently to alterations in HPA axis. VAT expression of ADN and its receptors remained almost unchanged by experimental manipulations. In SAT, on the other hand, expression of ADN and AdipoR2 was markedly increased by ACTH treatment and stress, while dexamethasone suppressed ADN and AdipoR1 mRNA levels. The results of this study provide new evidence for direct and indirect interactions between adipokines and HPA axis.

  1. The Role of gsp Mutations on the Development of Adrenal Cortical Tumors and Adrenal Hyperplasias

    Directory of Open Access Journals (Sweden)

    Maria Candida Barisson Villares Fragoso

    2016-07-01

    Full Text Available Somatic GNAS point mutations, commonly known as gsp mutations, are involved in the pathogenesis of McCune Albright syndrome and have also been described in autonomous hormone-producing tumors, such as somatotropinoma, corticotrophoma, thyroid cancer, ovarian and testicular Leydig cell tumors and primary macronodular adrenocortical hyperplasia (PMAH. [1-3]The involvement of gsp mutations in adrenal tumors was first described by Lyons et al. in 1990. Since then, several studies have detected the presence of gsp mutations in adrenal tumors, but none of them could explain its presence along or the mechanism that leads to tumor formation and hormone hypersecretion. As a result, the molecular pathogenesis of the majority of sporadic adrenocortical tumors remains unclear. [3] PMAH has also been reported with gsp somatic mutations in a few cases. Fragoso et al. in 2003 identified two distinct gsp somatic mutations affecting arginine residues on codon 201 of GNAS in a few patients with PMAH who lacked any features or manifestations of McCune Albright syndrome. Followed by this discovery, other studies have continued looking for gsp mutations based on strong prior evidence demonstrating that increased cAMP signaling is sufficient for cell proliferation and cortisol production. [2, 4] With consideration for the previously reported findings, we conjecture that although somatic activating mutations in GNAS are a rare molecular event, these mutations could probably be sufficient to induce the development of macronodule hyperplasia and variable cortisol secretion.In this manuscript, we revised the presence of gsp mutations associated with adrenal cortical tumors and hyperplasia.

  2. Prednisolone is associated with a worse lipid profile than hydrocortisone in patients with adrenal insufficiency

    Directory of Open Access Journals (Sweden)

    Marcus Quinkler

    2016-12-01

    Full Text Available Objective: Prednisolone is used as glucocorticoid replacement therapy for adrenal insufficiency (AI. Recent data indicate that its use in AI is associated with low bone mineral density. Data on risk factors for cardiovascular disease in patients with AI treated with prednisolone are scarce, despite this condition being the predominant cause of excess mortality. We aimed to address this question using real-world data from the European Adrenal Insufficiency Registry (EU-AIR. Design/methods: EU-AIR, comprising of 19 centres across Germany, the Netherlands, Sweden and the UK, commenced enrolling patients with AI in August 2012. Patients receiving prednisolone (3–6 mg/day, n = 50 or hydrocortisone (15–30 mg/day, n = 909 were identified and grouped at a ratio of 1:3 (prednisolone:hydrocortisone by matching for gender, age, duration and type of disease. Data from baseline and follow-up visits were analysed. Data from patients with congenital adrenal hyperplasia were excluded. Results: Significantly higher mean ± s.d. total (6.3 ± 1.6 vs 5.4 ± 1.1 mmol/L; P = 0.003 and low-density lipoprotein (LDL cholesterol levels (3.9 ± 1.4 vs 3.2 ± 1.0 mmol/L; P = 0.013 were identified in 47 patients on prednisolone vs 141 receiving hydrocortisone at baseline and at follow-up (P = 0.005 and P = 0.006, respectively. HbA1c, high-density lipoprotein and triglyceride levels, body mass index, systolic and diastolic blood pressure and waist circumference were not significantly different. Conclusions: This is the first matched analysis of its kind. Significantly higher LDL levels in patients receiving prednisolone relative to hydrocortisone could predict a higher relative risk of cardiovascular disease in the former group.

  3. Regulating prefrontal cortex activation

    DEFF Research Database (Denmark)

    Aznar, Susana; Klein, Anders Bue

    2013-01-01

    of emotion-based actions, such as addiction and other impulse-related behaviors. In this review, we give an overview of the 5-HT2A receptor distribution (neuronal, intracellular, and anatomical) along with its functional and physiological effect on PFC activation, and how that relates to more recent findings......The prefrontal cortex (PFC) is involved in mediating important higher-order cognitive processes such as decision making, prompting thereby our actions. At the same time, PFC activation is strongly influenced by emotional reactions through its functional interaction with the amygdala...... is highly expressed in the prefrontal cortex areas, playing an important role in modulating cortical activity and neural oscillations (brain waves). This makes it an interesting potential pharmacological target for the treatment of neuropsychiatric modes characterized by lack of inhibitory control...

  4. Biophysical alterations in lipid rafts from human cerebral cortex associate with increased BACE1/AβPP interaction in early stages of Alzheimer's disease.

    Science.gov (United States)

    Díaz, Mario; Fabelo, Noemí; Martín, Virginia; Ferrer, Isidre; Gómez, Tomás; Marín, Raquel

    2015-01-01

    In the present study, we have assessed the biophysical properties of lipid rafts from different brain areas in subjects exhibiting early neuropathological stages of Alzheimer's disease (AD). By means of steady-state fluorescence polarization analyses using two environment-sensitive fluorescent probes, we demonstrate that lipid rafts from cerebellum, and frontal and entorhinal cortices, exhibit different biophysical behaviors depending on the stage of the disease. Thus, while membrane anisotropies were similar in the cerebellum along stages, lipid rafts from frontal and entorhinal cortices at AD stages I/II and AD III were significantly more liquid-ordered than in control subjects, both at the aqueous interface and hydrophobic core of the raft membrane. Thermotropic analyses demonstrated the presence of Arrhenius breakpoints between 28.3-32.0 °C, which were not influenced by the disease stage. However, analyses of membrane microviscosity (ηapp) demonstrate that frontal and entorhinal lipid rafts are notably more viscous and liquid-ordered all across the membrane from early stages of the disease. These physicochemical alterations in lipid rafts do not correlate with changes in cholesterol or sphingomyelin levels, but to reduced unsaturation index and increased saturate/polyunsaturated ratios in phospholipid acyl chains. Moreover, we demonstrate that β-secretase/AβPP (amyloid-β protein precursor) interaction and lipid raft microviscosity are strongly, and positively, correlated in AD frontal and entorhinal cortices. These observations strengthens the hypothesis that physical properties of these microdomains modulate the convergence of amyloidogenic machinery toward lipid rafts, and also points to a critical role of polyunsaturated fatty acids in amyloidogenic processing of AβPP.

  5. Watery diarrhea, hypokalemia and achlorhydria syndrome due to an adrenal pheochromocytoma

    Institute of Scientific and Technical Information of China (English)

    Shin-ichi Ikuta; Chiaki Yasui; Masahiro Kawanaka; Tsukasa Aihara; Hidenori Yoshie; Hidenori Yanagi; Masao Mitsunobu; Ayako Sugihara; Naoki Yamanaka

    2007-01-01

    Watery diarrhea, hypokalemia and achlorhydria (WDHA)syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma,which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.

  6. High {sup 18}F-fluorodeoxyglocose uptake in adrenal histoplasmosis; a case report

    Energy Technology Data Exchange (ETDEWEB)

    Umeoka, Shigeaki; Saga, Tsuneo; Togashi, Kaori [Kyoto University (Japan). Department of Nuclear Medicine and Diagnostic Imaging; Koyama, Takashi; Higashi, Tatsuya [Kyoto University Hospital (Japan). Department of Radiology; Ito, Noriyuki; Kamoto, Toshiyuki; Ogawa, Osamu [Kyoto University (Japan). Department of Urology; Kotani, Hirokazu [Kyoto University (Japan). Department of Pathology

    2005-12-01

    Adrenal histoplasmosis is one of the most common adrenal granulomatous infections in endemic areas. Although CT or MRI findings of adrenal histoplasmosis have been documented, there are no reports regarding {sup 18}F-fluorodeoxyglocose (FDG) positron emission tomography (PET) findings. We report a case of bilateral adrenal histoplasmosis showing a significantly high uptake of {sup 18}F-fluorodeoxyglocose on PET study. Adrenal histoplasmosis should be considered as one of the differential diagnoses in cases of adrenal tumors with intense FDG uptake, even in non-endemic areas. (orig.)

  7. (18)F-FDG PET/CT in bilateral primary adrenal T-cell lymphoma.

    Science.gov (United States)

    Santhosh, Sampath; Mittal, Bhagwant Rai; Shankar, Praveen; Kashyap, Raghava; Bhattacharya, Anish; Singh, Baljinder; Das, Ashim; Bhansali, Anil

    2011-01-01

    Primary adrenal lymphoma is extremely rare. We report a young patient who presented with non- specific symptoms of fever and abdominal pain. Conventional imaging modalities demonstrated bilateral bulky adrenal masses, and whole-body fluorine-18-fluorodesoxyglucose ((18)F-FDG) positron emission tomography/computed tomography showed intense (18)F-FDG-avid bilateral adrenal masses with no evidence of extra-adrenal spread. A pathological diagnosis of non-Hodgkin lymphoma of peripheral T-cell type was made. The present case indicates that primary adrenal lymphoma should be included in the differential diagnosis of bilateral adrenal masses.

  8. Testicular adrenal rest tumor in infertile man with congenital adrenal hyperplasia: case report and literature review

    Directory of Open Access Journals (Sweden)

    Giovanni Scala Marchini

    Full Text Available CONTEXT: Synthesis of cortisol and aldosterone is impaired in patients with congenital adrenal hyperplasia (CAH because of 21-hydroxylase deficiency. Men with CAH have low fertility rates compared with the normal population, and this is related to testicular adrenal rest tumors. Findings of azoospermia in combination with a testicular tumor on ultrasound are likely to have a mechanical cause, especially when in the testicular mediastinum. The preferred treatment method consists of intensive corticoid therapy. However, when the tumor is unresponsive to steroid therapy, surgical treatment should be considered. CASE REPORT: We present the case of a male patient with CAH due to 21-hydroxylase deficiency who presented a testicular tumor and azoospermia. Treatment with low daily corticoid doses had previously been started by an endocrinologist, but after 12 months, no significant change in sperm count was found. Although the adrenocorticotrophic hormone and 17-hydroxyprogesterone levels returned to normal values, the follicle-stimulating hormone (FSH, luteinizing hormone and testosterone levels remained unchanged. Ultrasound examination confirmed that the testicles were small and heterogenous bilaterally, and revealed a mosaic area at the projection of the testis network bilaterally. Magnetic resonance imaging confirmed the finding. Testicular biopsy revealed the presence of preserved spermatogenesis and spermiogenesis in 20% of the seminiferous tubules in the right testicle. The patient underwent testis-sparing tumor resection. After 12 months of follow-up, there was no tumor recurrence but the patient still presented azoospermia and joined an intracytoplasmic sperm injection program.

  9. Diagnosis and management of congenital adrenal hyperplasia: clinical, molecular and prenatal aspects.

    Science.gov (United States)

    Mathur, R; Kabra, M; Menon, P S

    2001-01-01

    Congenital adrenal hyperplasia (CAH) is the most common cause of female pseudohermaphroditism in Indian children. It is caused by enzymatic defects in the steroidogenic pathway of the adrenal glands and is characterized by impaired cortisol and aldosterone synthesis and overproduction of androgens. The disease usually presents with life-threatening problems and virilization, with long term physical and psychological effects. The clinical and laboratory diagnoses play an important role in deciding the course of treatment, which continues lifelong. To ensure proper growth and development of the patient, optimized disease management and treatment with steroids is required. Often the patient also requires surgical correction. Recent developments in molecular genetics have greatly helped in understanding the pathogenesis of the disease. The gene encoding for steroid 21-hydroxylase, CYP21, is located on the short arm of chromosome 6 in the HLA region and is amplified for genetic diagnosis. Rapid characterization of point mutations is possible using the allele-specific polymerase chain reaction technique in affected children. Counselling, prenatal diagnosis and treatment are recommended in all pregnant women with a positive family history to reduce or eliminate the effects in affected foetuses. This spares the female newborn the consequences of genital ambiguity and problems of gender identity.

  10. Localization of aldosterone-producing adenoma on computed tomography. A comparative study with adrenal scintigraphy and plasma aldosterone concentration in the adrenal or renal vein

    Energy Technology Data Exchange (ETDEWEB)

    Haruyama, K.; Shigetomi, S.; Yamazaki, M.; Toki, T.; Yaginuma, K.; Fukuchi, S. (Fukushima Medical Coll. (Japan))

    1982-09-01

    An abdominal CT scan was performed on six patients with primary aldosteronism, one with idiopathic hyperaldosteronism and one with glucocorticoid responsive hyperaldosteronism; in an attempt to evaluate the utility of this noninvasive procedure. Diagnosis of hyperaldosteronism was made by demonstrating the elevated plasma aldosterone concentration and aldosterone secretion rate, normal excretion rate of urinary 17-OHCS and 17-KS, and low plasma renin activity. The CT scan correctly predicted unilateral adrenal adenoma in all the patients with primary aldosteronism of which the findings were identical to those demonstrated by surgery. The diameter of these tumors ranged from 10 x 7 x 6 to 19 x 17 x 14 mm. Also the CT scan in idiopathic hyperaldosteronism and glucocorticoid responsive hyperaldosteronism showed bilateral adrenal hyperplasia and bilateral normal adrenal glands, respectively. The pathological findings in these two cases disclosed the adrenal hyperplasia of zona glomerulosa and adrenal hyperplasia of zona subglomerulosa accompanied by a normal thickness of the adrenal gland, respectively. The precision of the CT scan, adrenal scintigraphy and determination of plasma aldosterone in the adrenal or renal veins were almost equal to the diagnosis of the localization of adrenal adenoma. It is concluded that the CT scan is a noninvasive and most useful method for the localization of aldosterone-producing adenoma and helpful in distinguishing adrenal adenoma from adrenal hyperplasia.

  11. Occurrence of new neurons in the piriform cortex

    OpenAIRE

    Ti-Fei eYuan; YU-XIANG eLIANG; Kwok-Fai eSo

    2015-01-01

    Adult neurogenesis has been well studied in hippocampus and subventricular zone; while this is much less appreciated in other brain regions, including amygdala, hypothalamus and piriform cortex. The present review aims at summarizing recent advances on the occurrence of new neurons in the piriform cortex, their potential origin and migration route from the subventricular zone. We further discuss the relevant implications in olfactory dysfunction accompanying the neuro-degenerative diseases.

  12. Occurrence of new neurons in the piriform cortex

    Directory of Open Access Journals (Sweden)

    Ti-Fei eYuan

    2015-01-01

    Full Text Available Adult neurogenesis has been well studied in hippocampus and subventricular zone; while this is much less appreciated in other brain regions, including amygdala, hypothalamus and piriform cortex. The present review aims at summarizing recent advances on the occurrence of new neurons in the piriform cortex, their potential origin and migration route from the subventricular zone. We further discuss the relevant implications in olfactory dysfunction accompanying the neuro-degenerative diseases.

  13. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency - management in adults.

    Science.gov (United States)

    Ambroziak, Urszula; Bednarczuk, Tomasz; Ginalska-Malinowska, Maria; Małunowicz, Ewa Maria; Grzechocińska, Barbara; Kamiński, Paweł; Bablok, Leszek; Przedlacki, Jerzy; Bar-Andziak, Ewa

    2010-01-01

    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is one of the most common autosomal recessive hereditary diseases. The impairment of cortisol synthesis leads to excessive stimulation of the adrenal glands by adrenocorticotropic hormone (ACTH), adrenal hyperplasia, and excessive androgen synthesis. The syndrome is characterised by a considerable correlation between the genotype and the phenotype with the type of CYP21A2 gene mutation affecting the severity of 21-hydroxylase deficiency. The clinical manifestations of CAH in adults result from adrenocortical and adrenomedullary insufficiency, hyperandrogenism, and the adverse effects of glucocorticosteroids used for the treatment of the condition. Non-classic CAH may sometimes be asymptomatic. In patients with classic CAH obesity, hyperinsulinaemia, insulin resistance, and hyperleptinaemia are more often seen than in the general population. These abnormalities promote the development of metabolic syndrome and its sequelae, including endothelial dysfunction, and cardiovascular disease. Long-term glucocorticosteroid treatment is also a known risk factor for osteoporosis. Patients with CAH require constant monitoring of biochemical parameters (17a-hydroxyprogesterone [17-OHP] and androstenedione), clinical parameters (body mass, waist circumference, blood pressure, glucose, and lipids), and bone mineral density by densitometry. The principal goal of treatment in adults with CAH is to improve quality of life, ensure that they remain fertile, reduce the manifestations of hyperandrogenisation in females, and minimise the adverse effects of glucocorticosteroid treatment. Patients with classic CAH require treatment with glucocorticosteroids and, in cases of salt wasting, also with a mineralocorticosteroid. Radical measures, such as bilateral adrenalectomy, are very rarely needed. Asymptomatic patients with non-classic CAH require monitoring: treatment is not always necessary. Medical care for patients

  14. [Congenital adrenal hyperplasia due to 21-hydroxylase deficiency--management in adults].

    Science.gov (United States)

    Ambroziak, Urszula; Bednarczuk, Tomasz; Ginalska-Malinowska, Maria; Małunowicz, Ewa Maria; Grzechocińska, Barbara; Kamiński, Paweł; Bablok, Leszek; Przedlacki, Jerzy; Bar-Andziak, Ewa

    2010-01-01

    Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is one of the most common autosomal recessive hereditary diseases. The impairment of cortisol synthesis leads to excessive stimulation of the adrenal glands by adrenocorticotropic hormone (ACTH), adrenal hyperplasia, and excessive androgen synthesis. The syndrome is characterised by a considerable correlation between the genotype and the phenotype with the type of CYP21A2 gene mutation affecting the severity of 21-hydroxylase deficiency. The clinical manifestations of CAH in adults result from adrenocortical and adrenomedullary insufficiency, hyperandrogenism, and the adverse effects of glucocorticosteroids used for the treatment of the condition. Non-classic CAH may sometimes be asymptomatic. In patients with classic CAH obesity, hyperinsulinaemia, insulin resistance, and hyperleptinaemia are more often seen than in the general population. These abnormalities promote the development of metabolic syndrome and its sequelae, including endothelial dysfunction, and cardiovascular disease. Long-term glucocorticosteroid treatment is also a known risk factor for osteoporosis. Patients with CAH require constant monitoring of biochemical parameters (17a-hydroxyprogesterone and androstenedione), clinical parameters (body mass, waist circumference, blood pressure, glucose, and lipids), and bone mineral density by densitometry. The principal goal of treatment in adults with CAH is to improve quality of life, ensure that they remain fertile, reduce the manifestations of hyperandrogenisation in females, and minimise the adverse effects of glucocorticosteroid treatment. Patients with classic CAH require treatment with glucocorticosteroids and, in cases of salt wasting, also with a mineralocorticosteroid. Radical measures, such as bilateral adrenalectomy, are very rarely needed. Asymptomatic patients with non-classic CAH require monitoring: treatment is not always necessary. Medical care for patients with CAH

  15. Lipoid congenital adrenal hyperplasia due to STAR mutations in a Caucasian patient

    Science.gov (United States)

    Kaur, Jasmeet; Casas, Luis

    2016-01-01

    Summary Lipoid congenital adrenal hyperplasia (lipoid CAH), the most severe form of CAH, is most commonly caused by mutations in steroidogenic acute regulatory protein (STAR), which is required for the movement of cholesterol from the outer to the inner mitochondrial membranes to synthesize pregnenolone. This study was performed to evaluate whether the salt-losing crisis and the adrenal inactivity experienced by a Scandinavian infant is due to a de novo STAR mutation. The study was conducted at the University of North Dakota, the Mercer University School of Medicine and the Memorial University Medical Center to identify the cause of this disease. The patient was admitted to a pediatric endocrinologist at the Sanford Health Center for salt-losing crisis and possible adrenal failure. Lipoid CAH is an autosomal recessive disease, we identified two de novo heterozygous mutations (STAR c.444C>A (STAR p.N148K) and STAR c.557C>T (STAR p.R193X)) in the STAR gene, causing lipoid CAH. New onset lipoid CAH can occur through de novo mutations and is not restricted to any specific region of the world. This Scandinavian family was of Norwegian descent and had lipoid CAH due to a mutation in S TAR exons 4 and 5. Overexpression of the STAR p.N148K mutant in nonsteroidogenic COS-1 cells supplemented with an electron transport system showed activity similar to the background level, which was ∼10% of that observed with wild-type (WT) STAR. Protein-folding analysis showed that the finger printing of the STAR p.N148K mutant is also different from the WT protein. Inherited STAR mutations may be more prevalent in some geographical areas but not necessarily restricted to those regions. Learning points STAR mutations cause lipoid CAH.This is a pure population from a caucasian family.Mutation ablated STAR activity.The mutation resulted in loosely folded conformation of STAR. PMID:27047663

  16. Clinical insights into the safety and utility of the insulin tolerance test (ITT) in the assessment of the hypothalamo-pituitary-adrenal axis.

    LENUS (Irish Health Repository)

    Finucane, Francis M

    2008-10-01

    The insulin tolerance test (ITT) is the gold standard for assessing GH and cortisol production in pituitary disease. However, areas of uncertainty remain regarding its safety in older people, the optimal duration of the test and its performance in insulin resistant states. Whether basal cortisol concentration can reliably predict an adequate adrenal response to hypoglycaemia remains to be determined.

  17. Mifepristone Accelerates HPA Axis Recovery in Secondary Adrenal Insufficiency.

    Science.gov (United States)

    Cohan, Pejman

    2016-01-01

    Context. Transient secondary adrenal insufficiency (SAI) is an expected complication following successful adenomectomy of ACTH-secreting pituitary adenomas or unilateral adrenalectomy for cortisol-secreting adrenal adenomas. To date, no pharmacological therapy has been shown to hasten recovery of the hypothalamic-pituitary-adrenal (HPA) axis in this clinical scenario. Case Description. A 33-year-old woman underwent uncomplicated unilateral adrenalectomy for a 3.7 cm cortisol-secreting adrenal adenoma. Postoperatively, she developed SAI and was placed on hydrocortisone 15 mg/day, given in divided doses. In the ensuing six years, the patient's HPA axis failed to recover and she remained corticosteroid-dependent. Quarterly biochemical testing (after withholding hydrocortisone for 18 hours) consistently yielded undetectable serum cortisol and subnormal plasma ACTH levels. While she was on hydrocortisone 15 mg/day, mifepristone was initiated and gradually titrated to a maintenance dose of 600 mg/day after 5 months. Rapid recovery of the HPA axis was subsequently noted with ACTH rising into the supranormal range at 4 months followed by a subsequent rise in cortisol levels into the normal range. After 6 months, the dose of hydrocortisone and mifepristone was lowered and both were ultimately stopped after 8 months. The HPA axis remains normal after an additional 16 months of follow-up. Conclusion. Mifepristone successfully restored the HPA axis in a woman with prolonged secondary adrenal insufficiency (SAI) after adrenalectomy for Cushing's syndrome (CS).

  18. Development of automated detection of radiology reports citing adrenal findings

    Science.gov (United States)

    Zopf, Jason; Langer, Jessica; Boonn, William; Kim, Woojin; Zafar, Hanna

    2011-03-01

    Indeterminate incidental findings pose a challenge to both the radiologist and the ordering physician as their imaging appearance is potentially harmful but their clinical significance and optimal management is unknown. We seek to determine if it is possible to automate detection of adrenal nodules, an indeterminate incidental finding, on imaging examinations at our institution. Using PRESTO (Pathology-Radiology Enterprise Search tool), a newly developed search engine at our institution that mines dictated radiology reports, we searched for phrases used by attendings to describe incidental adrenal findings. Using these phrases as a guide, we designed a query that can be used with the PRESTO index. The results were refined using a modified version of NegEx to eliminate query terms that have been negated within the report text. In order to validate these findings we used an online random date generator to select two random weeks. We queried our RIS database for all reports created on those dates and manually reviewed each report to check for adrenal incidental findings. This survey produced a ground- truth dataset of reports citing adrenal incidental findings against which to compare query performance. We further reviewed the false positives and negatives identified by our validation study, in an attempt to improve the performance query. This algorithm is an important step towards automating the detection of incidental adrenal nodules on cross sectional imaging at our institution. Subsequently, this query can be combined with electronic medical record data searches to determine the clinical significance of these findings through resultant follow-up.

  19. Pituitary-Adrenal Axis in Prader Willi Syndrome

    Directory of Open Access Journals (Sweden)

    Olivia S. Edgar

    2016-01-01

    Full Text Available Purpose: Prader Willi syndrome (PWS is a rare genetic condition that has concurrent endocrinological insufficiencies. The presence of growth hormone deficiency has been well documented, but adrenal insufficiency (AI is not widely reported. A review was conducted to investigate its prevalence and relevance in PWS in both adults and children. Methodology: A literature review was performed with the search terms “Prader-Willi syndrome” and “adrenal insufficiency”. Results: The review found studies disagree on the prevalence and method of investigation of AI in PWS. Case studies demonstrate that patients with PWS are at risk of premature death, often secondary to respiratory infections. The possibility that this may be the result of the inability to mount an effective cortisol response has been studied, with some evidence confirming AI in PWS patients. Most reports agreed AI is present in PWS, however, Farholt et al. showed no HPA axis dysfunction in adults, suggesting that perhaps it is rare in adults, and children should be the focus of further studies. Conclusion: AI is present in some patients with PWS. Further research is required to ensure optimal treatment can be implemented and to prevent premature deaths related to adrenal insufficiency. Clinicians should have a low threshold for testing the adrenal axis and considering treatment for adrenal insufficiency in PWS patients.

  20. The prevalence of adrenal incidentaloma in routine clinical practice.

    LENUS (Irish Health Repository)

    Davenport, Colin

    2011-03-10

    The prevalence of adrenal incidentaloma (AI) on computed tomography (CT) in the general population has been reported to be as high as 4.2%. However, many of the previous studies in this field utilised a prospective approach with analysis of CT scans performed by one or more radiologists with a specialist interest in adrenal tumours and a specific focus on identifying the presence of an adrenal mass. A typical radiology department, with a focus on the patient\\'s presenting complaint as opposed to the adrenal gland, may not be expected to diagnose as many adrenal incidentalomas as would be identified in a dedicated research protocol. We hypothesised that the number of AI reported in routine clinical practice is significantly lower than the published figures would suggest. We retrospectively reviewed the reports of all CT thorax and abdomen scans performed in our hospital over a 2 year period. 3,099 patients underwent imaging, with 3,705 scans performed. The median age was 63 years (range 18-98). Thirty-seven true AI were diagnosed during the time period studied. Twenty-two were diagnosed by CT abdomen (22\\/2,227) and 12 by CT thorax (12\\/1,478), a prevalence of 0.98 and 0.81% with CT abdomen and thorax, respectively, for AI in routine clinical practice.

  1. The prevalence of adrenal incidentaloma in routine clinical practice.

    LENUS (Irish Health Repository)

    Davenport, Colin

    2012-02-01

    The prevalence of adrenal incidentaloma (AI) on computed tomography (CT) in the general population has been reported to be as high as 4.2%. However, many of the previous studies in this field utilised a prospective approach with analysis of CT scans performed by one or more radiologists with a specialist interest in adrenal tumours and a specific focus on identifying the presence of an adrenal mass. A typical radiology department, with a focus on the patient\\'s presenting complaint as opposed to the adrenal gland, may not be expected to diagnose as many adrenal incidentalomas as would be identified in a dedicated research protocol. We hypothesised that the number of AI reported in routine clinical practice is significantly lower than the published figures would suggest. We retrospectively reviewed the reports of all CT thorax and abdomen scans performed in our hospital over a 2 year period. 3,099 patients underwent imaging, with 3,705 scans performed. The median age was 63 years (range 18-98). Thirty-seven true AI were diagnosed during the time period studied. Twenty-two were diagnosed by CT abdomen (22\\/2,227) and 12 by CT thorax (12\\/1,478), a prevalence of 0.98 and 0.81% with CT abdomen and thorax, respectively, for AI in routine clinical practice.

  2. Addison disease in patients treated with glucocorticoid therapy.

    LENUS (Irish Health Repository)

    Cronin, C C

    2012-02-03

    Acute adrenal crisis in patients with unrecognized chronic adrenocortical failure is difficult to diagnose and potentially fatal. We describe 2 patients with acute adrenal crisis whose diagnoses were hindered because of concomitant glucocorticoid treatment. Acute adrenal insufficiency is primarily a state of mineralocorticoid deficiency. Prednisolone and prednisone, the most frequently prescribed anti-inflammatory corticosteroid agents, have minimal mineralocorticoid activity. Several conditions that may be treated with pharmacological glucocorticoids are associated with an increased risk of Addison disease. An acute adrenal crisis, against which concurrent glucocorticoid therapy does not confer adequate protection, may develop in such patients.

  3. [The effect of loading tests on the activity and reactivity of the sympathetic-adrenal system in subjects with arterial hypertension and overweight].

    Science.gov (United States)

    Kartvelishvili, H Iu

    1997-01-01

    Regulatory potentialities were studied of the sympathetic-adrenal system that reveal themselves in response to psychoemotional testing and acute peroral salt loading. In the group of over-weight (OW) patients presenting with hypertensive disease (HD), 1-h excretion of catecholamines following the psychoemotional testing was not different from that during the testing itself, which fact suggests more prolonged reaction of the sympathetic-adrenal system to stress in these examinees. An acute peroral salt loading causes increase in activity of the hormonal and mediator links of the sympathetic-adrenal system in HD patients both overweight and in the normal range body weight (BW). It was only those persons presenting with normal BW that were retaining sodium after the test.

  4. Role of the adrenal medulla in control of blood pressure and renal function during furosemide-induced volume depletion

    DEFF Research Database (Denmark)

    Hasbak, Philip; Petersen, Jørgen Søberg; Shalmi, Michael

    1995-01-01

    Farmakologi, furosemide, adrenaline, renal function, adrenal medullectomy, arterial blood pressure......Farmakologi, furosemide, adrenaline, renal function, adrenal medullectomy, arterial blood pressure...

  5. Clinical and pathological analysis of 116 cases of adult adrenal cortical adenoma and literature review

    Directory of Open Access Journals (Sweden)

    Jia AH

    2015-05-01

    Full Text Available Ai-Hua Jia,1 Hong-Quan Du,2 Min-Hua Fan,2 Yu-Hong Li,1 Jun-Long Xu,1 Gui-Fen Niu,2 Jie Bai,2 Guang-Zhen Zhang,2 Yu-Bo Ren1 1Department of Pathology, Liaocheng People’s Hospital, Liaocheng, People’s Republic of China; 2Department of Endocrinology, Liaocheng People’s Hospital, Liaocheng, People’s Republic of China Background: The aim of this study is to investigate origin, gross features, microscopic features, immunohistochemical properties, and differential diagnosis of adrenal cortical adenoma (ACA in patients ≥20 years old.Methods: The clinicopathological features of 116 cases of ACA and the immunohistochemical features of 50 cases of ACA were evaluated, and the relevant literature was reviewed.Results: In our cohort, 76.72% (89/116 of the cases were functional, and 27 cases had non-functional, benign adrenal adenomas. ACA presented as an island tumor with an envelope, and the mean tumor size was 3.6 cm (range 1–5 cm, with a mean tumor weight of 9.28 g (range 5–113 g. The shape of the tumor cells was consistent, and mitosis was rarely observed. Forty of the 46 patients with cortisol-secreting ACA had tumors containing granule cells. Primary aldosteronism was observed in 43 cases. Thirty-eight cases had endoscopically visible tumors, with clear cells and lipid-rich cytoplasm arranged in irregular patches or strips. Cortisol-producing ACAs were associated with atrophy of the non-tumorous cortex. Adrenocortical adenomas displayed positive immunohistochemical staining for MELAN-A, Syn (46 of 50 cases of ACA, NSE (44 of 50 cases of ACA, Vim (42 of 50 cases of ACA and Ki-67 <5% (24 of 50 cases of ACA; the remaining 26 cases were negative for Ki-67.Conclusion: Prediction of endocrine syndrome in functional ACA was possible based on its structure and morphologic features, which could prevent an unanticipated postoperative crisis. However, a clinical study is needed to validate these findings. Keywords: adrenal cortical adenoma

  6. [Raman spectra of monkey cerebral cortex tissue].

    Science.gov (United States)

    Zhu, Ji-chun; Guo, Jian-yu; Cai, Wei-ying; Wang, Zu-geng; Sun, Zhen-rong

    2010-01-01

    Monkey cerebral cortex, an important part in the brain to control action and thought activities, is mainly composed of grey matter and nerve cell. In the present paper, the in situ Raman spectra of the cerebral cortex of the birth, teenage and aged monkeys were achieved for the first time. The results show that the Raman spectra for the different age monkey cerebral cortex exhibit most obvious changes in the regions of 1000-1400 and 2800-3000 cm(-1). With monkey growing up, the relative intensities of the Raman bands at 1313 and 2885 cm(-1) mainly assigned to CH2 chain vibrational mode of lipid become stronger and stronger whereas the relative intensities of the Raman bands at 1338 and 2932 cm(-1) mainly assigned to CH3 chain vibrational mode of protein become weaker and weaker. In addition, the two new Raman bands at 1296 and 2850 cm(-1) are only observed in the aged monkey cerebral cortex, therefore, the two bands can be considered as a character or "marker" to differentiate the caducity degree with monkey growth In order to further explore the changes, the relative intensity ratios of the Raman band at 1313 cm(-1) to that at 1338 cm(-1) and the Raman band at 2885 cm(-1) to that at 2 932 cm(-1), I1313/I1338 and I2885/I2932, which are the lipid-to-protein ratios, are introduced to denote the degree of the lipid content. The results show that the relative intensity ratios increase significantly with monkey growth, namely, the lipid content in the cerebral cortex increases greatly with monkey growth. So, the authors can deduce that the overmuch lipid is an important cause to induce the caducity. Therefore, the results will be a powerful assistance and valuable parameter to study the order of life growth and diagnose diseases.

  7. Genetics Home Reference: intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and ...

    Science.gov (United States)

    ... IMAGe syndrome intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies Enable Javascript to view ... combination of intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies is commonly known by ...

  8. Genetics Home Reference: congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency

    Science.gov (United States)

    ... due to 11-beta-hydroxylase deficiency congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency Enable Javascript ... PDF Open All Close All Description Congenital adrenal hyperplasia (CAH) due to 11-beta-hydroxylase deficiency is ...

  9. Muscle strength in girls with congenital adrenal hyperplasia.

    Science.gov (United States)

    Rodda, C; Jones, D A; Round, J; Grant, D B

    1987-05-01

    To assess whether exposure to excessive adrenal androgens increases muscle strength in girls with congenital adrenal hyperplasia, eighteen girls with 21 hydroxylase type congenital adrenal hyperplasia (CAH), aged 4.3-12.1 years were studied and compared with 78 healthy control girls aged 4-16 years. Maximum voluntary isometric strength was measured using a muscle testing chair, and the highest value from at least three attempts with each leg was recorded. Height and weight were also measured in the patients and controls. When compared with normal girls of similar age, some of the girls with CAH seemed unusually strong, but this difference was less marked when the CAH girls were compared with controls of similar weights. However, our data suggest that girls diagnosed later in childhood may have unusual muscle strength.

  10. Feminizing adrenal tumors: Our experience about three cases

    Directory of Open Access Journals (Sweden)

    Chentli Farida

    2013-01-01

    Full Text Available Feminizing adrenal tumors (FATs are very rare as they account for less than 2% of all the adrenal neoplasms. Their prognosis is deemed to be very poor. We aimed to present a mono centre (adult and pediatric experience over a long period of time (January 1980 to Jun 2012. During the study period, we observed only three cases in men aged 22 (2 cases and 45 (1 case. They all consulted for a painful gynecomastia, decreased libido and impotency. Estradiol was high in two cases at presentation, and after a relapsing tumor in the third one. All had big adrenal tumors (5.9, 6, and 17 cm, and a mixed secretion composed by high estradiol and cortisol. The pathological study argued for malignancy in two cases. But, only one had diffuse metastasis and died 4 years after diagnosis; the others diagnosed one and three years ago are still alive without any metastasis or relapsing.

  11. Pure Androgen-Secreting Adrenal Adenoma Associated with Resistant Hypertension

    Directory of Open Access Journals (Sweden)

    René Rodríguez-Gutiérrez

    2013-01-01

    Full Text Available Pure androgen-secreting adrenal adenoma is very rare, and its diagnosis remains a clinical challenge. Its association with resistant hypertension is uncommon and not well understood. We present an 18-year-old female with a 10-year history of hirsutism that was accidentally diagnosed with an adrenal mass during the evaluation of a hypertensive crisis. She had a long-standing history of hirsutism, clitorimegaly, deepening of the voice, and primary amenorrhea. She was phenotypically and socially a male. FSH, LH, prolactin, estradiol, 17-hydroxyprogesterone, and progesterone were normal. Total testosterone and DHEA-S were elevated. Cushing syndrome, primary aldosteronism, pheochromocytoma, and nonclassic congenital adrenal hyperplasia were ruled out. She underwent adrenalectomy and pathology reported an adenoma. At 2-month followup, hirsutism and virilizing symptoms clearly improved and blood pressure normalized without antihypertensive medications, current literature of this unusual illness and it association with hypertension is presented and discussed.

  12. Ganglioneuroma of adrenal gland in a patient with Turner syndrome.

    Science.gov (United States)

    Kamoun, Mahdi; Mnif, Mouna Feki; Rekik, Nabila; Belguith, Neila; Charfi, Nadia; Mnif, Lilia; Elleuch, Mouna; Mnif, Fatma; Kamoun, Thouraya; Mnif, Zeinab; Kamoun, Hassen; Sellami-Boudawara, Tahia; Hachicha, Mongia; Abid, Mohamed

    2010-04-01

    A 15-year-old girl with Turner syndrome was unexpectedly found to have a left suprarenal mass. Extensive investigations showed a clinically and biochemically inapparent mass. Computed tomography disclosed a well-defined solid lesion in the left adrenal measuring 6.5 x 5 cm with minimal contrast enhancement. Laparoscopic adrenalectomy was done. Histologic examination revealed an encapsulated mass originated from the left adrenal medulla. Tumor tissue comprised abundant collagen fibers and spindloid cells admixed with mature ganglion cells. The tumor was diagnosed as left adrenal ganglioneuroma. According to literature, we report the eighth case of ganglioneuroma complicating Turner syndrome. Patients with this syndrome are predisposed to the development of neuroblastoma and related tumors. Reasons for this predisposition might relate to genetic and hormonal factors. Given that these tumors are often limited stage and of good prognosis, we recommend their screening in all patients with Turner syndrome.

  13. Tumor necrosis factor-α regulates glucocorticoid synthesis in the adrenal glands of Trypanosoma cruzi acutely-infected mice. the role of TNF-R1.

    Directory of Open Access Journals (Sweden)

    Silvina R Villar

    Full Text Available Adrenal steroidogenesis is under a complex regulation involving extrinsic and intrinsic adrenal factors. TNF-α is an inflammatory cytokine produced in response to tissue injury and several other stimuli. We have previously demonstrated that TNF-R1 knockout (TNF-R1(-/- mice have a dysregulated synthesis of glucocorticoids (GCs during Trypanosoma cruzi acute infection. Since TNF-α may influence GCs production, not only through the hypothalamus-pituitary axis, but also at the adrenal level, we now investigated the role of this cytokine on the adrenal GCs production. Wild type (WT and TNF-R1(-/- mice undergoing acute infection (Tc-WT and Tc-TNF-R1(-/- groups, displayed adrenal hyperplasia together with increased GCs levels. Notably, systemic ACTH remained unchanged in Tc-WT and Tc-TNF-R1(-/- compared with uninfected mice, suggesting some degree of ACTH-independence of GCs synthesis. TNF-α expression was increased within the adrenal gland from both infected mouse groups, with Tc-WT mice showing an augmented TNF-R1 expression. Tc-WT mice showed increased levels of P-p38 and P-ERK compared to uninfected WT animals, whereas Tc-TNF-R1(-/- mice had increased p38 and JNK phosphorylation respect to Tc-WT mice. Strikingly, adrenal NF-κB and AP-1 activation during infection was blunted in Tc-TNF-R1(-/- mice. The accumulation of mRNAs for steroidogenic acute regulatory protein and cytochrome P450 were significantly increased in both Tc-WT and Tc-TNF-R1(-/- mice; being much more augmented in the latter group, which also had remarkably increased GCs levels. TNF-α emerges as a potent modulator of steroidogenesis in adrenocortical cells during T. cruzi infection in which MAPK pathways, NF-κB and AP-1 seem to play a role in the adrenal synthesis of pro-inflammatory cytokines and enzymes regulating GCs synthesis. These results suggest the existence of an intrinsic immune-adrenal interaction involved in the dysregulated synthesis of GCs during murine Chagas

  14. Adrenal hyperactivity, injuries and hominization Hiperactivación adrenal, noxas y hominización

    Directory of Open Access Journals (Sweden)

    William Alvarez Gaviria

    2001-02-01

    Full Text Available In this revision it is postulated that the hominization is a by-product of the endocrine-metabolic adjustment to that hominid ancestors were subjected about 5 million years ago. A bigger adrenalization index is demonstrated in the hominids that partly could explain our fenotype, our behavior and the biggest propensity to certain pathologies. En esta revisión se postula que la hominización es un subproducto del ajuste endocrino-metabólico a que fueron sometidos los ancestros de los homínidos hace unos 5 millones de años. Se demuestra un mayor índice de adrenalización en los homínidos que pudiera en parte explicar nuestro fenotipo, nuestra conducta y la mayor propensión a determinadas patologías.

  15. Etiology of common childhood acute lymphoblastic leukemia: the adrenal hypothesis

    DEFF Research Database (Denmark)

    Schmiegelow, K.; Vestergaard, T.; Nielsen, S.M.;

    2008-01-01

    The pattern of infections in the first years of life modulates our immune system, and a low incidence of infections has been linked to an increased risk of common childhood acute lymphoblastic leukemia (ALL). We here present a new interpretation of these observations--the adrenal hypothesis......--that proposes that the risk of childhood ALL is reduced when early childhood infections induce qualitative and quantitative changes in the hypothalamus-pituitary-adrenal axis that increase plasma cortisol levels. This may directly eliminate leukemic cells as well as preleukemic cells for the ALL subsets...

  16. Uncommon Cause of Acute Adrenal Failure - Case Report

    Directory of Open Access Journals (Sweden)

    Tariq

    2010-12-01

    Full Text Available Adrenomyeloneuropathy is a rare X-linked inherited disorder of peroxisomes characterized by accumulation of very-long-chain fatty acids (VLCFA in the central and peripheral nervous system, adrenal glands and testes, leading to dysfunction of these organs and systems (1. Here, we report a case of adrenomyeloneuropathy presenting initially as acute adrenal crisis, which progressed rapidly within one year to variant neurological manifestations, dementia, sensory, motor and psycho-intellectual dysfunction, and generalized spasticity. Turk Jem 2010; 14: 103-5

  17. Xanthogranulomatous adrenalitis in a neonate: CT and US findings

    Energy Technology Data Exchange (ETDEWEB)

    Trinavarat, Panruethai; Sasiwimonphan, Kewalee [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Radiology, Faculty of Medicine, Bangkok (Thailand); Sansopha, Lalana [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Pathology, Faculty of Medicine, Bangkok (Thailand); Vejchapipat, Paisarn [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Surgery, Faculty of Medicine, Bangkok (Thailand); Sosothikul, Darintr [Chulalongkorn University, King Chulalongkorn Memorial Hospital, Department of Paediatrics, Faculty of Medicine, Bangkok (Thailand)

    2009-03-15

    We report a 46-day-old female infant with xanthogranulomatous adrenalitis. Ultrasonography showed a complex, solid-cystic right suprarenal mass with poorly defined margins. Colour flow Doppler revealed the solid portion of the mass to be vascular. CT demonstrated a heterogeneous cystic and solid mass with some contrast enhancement in the inferior part of the lesion. There was compression of the adjacent upper pole of the right kidney and the lateral aspect of the inferior vena cava. The mass was completely removed; histological examination revealed xanthogranulomatous adrenalitis. At the time of this report she remained well 3 years following surgery. (orig.)

  18. Arecoline inhibits catecholamine release from perfused rat adrenal gland

    Institute of Scientific and Technical Information of China (English)

    Dong-yoon LIM; Il-sik KIM

    2006-01-01

    Aim: To study the effect of arecoline, an alkaloid isolated from Areca catechu, on the secretion of catecholamines (CA) evoked by cholinergic agonists and the membrane depolarizer from isolated perfused rat adrenal gland. Methods: Adrenal glands were isolated from male Sprague-Dawley rats. The adrenal glands were perfused with Krebs bicarbonate solution by means of a peristaltic pump. The CA content of the perfusate was measured directly using the fluorometric method.Results: Arecoline (0.1-1.0 mmol/L) perfused into an adrenal vein for 60 min produced dose- and time-dependent inhibition in CA secretory responses evoked by acetylcholine (ACh) (5.32 mmol/L), 1.1-dimethyl-4-phenyl piperazinium iodide (DMPP) (100 μmol/L for 2 min) and 3-(m-choloro-phenyl-carbamoyl-oxy)-2-butynyl trimethyl ammonium chloride (McN-A-343) (100 μmol/L for 2 min). However, lower doses of arecoline did not affect CA secretion of high K+ (56 mmol/L); higher doses greatly reduced CA secretion of high K+. Arecoline also failed to affect basal catecholamine output. Furthermore, in adrenal glands loaded with arecoline (0.3 mmol/L), CA secretory response evoked by Bay-K-8644 (10 μmol/L), an activator of L-type Ca2+ channels, was markedly inhibited, whereas CA secretion by cyclopiazonic acid (10 μmol/L), an inhibitor of cytoplasmic Ca2+-ATPase, was not affected. Nicotine (30 μmol/L), which was peffused into the adrenal gland for 60min, however, initially enhanced ACh-evoked CA secretory responses. As time elapsed, these responses became more inhibited, whereas the initially enhanced high K+-evoked CA release diminished. CA secretion evoked by DMPP and McNA-343 was significantly depressed in the presence of nicotine. Conclusion:Arecoline dose-dependently inhibits CA secretion from isolated perfused rat adrenal gland evoked by activation of cholinergic receptors. At lower doses arecoline does not inhibit CA secretion through membrane depolarization, but at larger doses it does. This inhibitory

  19. Observation on local and/or unilateral pathologic changes in renal cortex by CT scan

    Energy Technology Data Exchange (ETDEWEB)

    Ishikawa, Isao; Shinoda, Akira (Kanazawa Medical Univ. (Japan)); Onouchi, Zengoro; Saito, Yasuhito; Matsuura, Hajime

    1984-03-01

    Renal cortex visualization after bolus injection of contrast medium using computed tomography (CT), was obtained in 132 consecutive patients with renal disease. Local pathological changes in the functioning cortex of the kidney were easily recognized in 37 cases and unilateral cortical thinning was found in 17 cases. Unilateral poor enhancement of the cortex with bilateral equal cortex thickness was noted in 4 cases. Several representative cases are reported with CT scans. The cortex at the posterior aspect of the renal graft compressed on psoas muscle was thinner than that at the anterior aspect in renal transplant cases. The macroscopic observation on the renal cortex presented here is far superior to the nephrogram or pyelogram seen through conventional radiographic examination. In vivo cortex visualization will correlate renal biopsy findings with the state of the whole kidney.

  20. Adrenal Rest Tumor from the Greater Omentum Mimicking Exophytic Hepatocellular Carcinoma (HCC): A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Hyung Jo; Kim, Seong Hoon; Shin, Hyun Woong [Daegu Fatima Hospital, Daegu (Korea, Republic of); Park, Jae Bok [Catholic University of Daegu, Gyeongsan (Korea, Republic of); Jo, Hyun Chul; Son, Mi Young; Kim, Tae Bong [Daegu Veterans Hospital, Daegu (Korea, Republic of)

    2010-02-15

    Adrenal rest tumors are aberrant adrenocortical tissue which has been most commonly described in abdominal and pelvic sites. To our knowledge, there has been no previous description of an adrenal rest tumor of the greater omentum. We present a case of a pathologically confirmed adrenal rest tumor of the greater omentum in a 76-year- old man

  1. File list: NoD.Oth.50.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available NoD.Oth.50.AllAg.Adrenal_Glands hg19 No description Others Adrenal Glands SRX300959...50,SRX300954,SRX213925,SRX213927,SRX252733,SRX213917 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/NoD.Oth.50.AllAg.Adrenal_Glands.bed ...

  2. File list: DNS.Oth.20.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Oth.20.AllAg.Adrenal_Glands hg19 DNase-seq Others Adrenal Glands SRX040375,SRX2...01798,SRX026916,SRX100961,SRX201797,SRX100963,SRX121284 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/DNS.Oth.20.AllAg.Adrenal_Glands.bed ...

  3. File list: ALL.Oth.10.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available ALL.Oth.10.AllAg.Adrenal_Glands hg19 All antigens Others Adrenal Glands SRX300959,S...17,SRX213925 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/ALL.Oth.10.AllAg.Adrenal_Glands.bed ...

  4. File list: NoD.Oth.10.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available NoD.Oth.10.AllAg.Adrenal_Glands hg19 No description Others Adrenal Glands SRX300959...72,SRX252733,SRX300950,SRX300954,SRX213917,SRX213925 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/NoD.Oth.10.AllAg.Adrenal_Glands.bed ...

  5. File list: NoD.Oth.20.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available NoD.Oth.20.AllAg.Adrenal_Glands hg19 No description Others Adrenal Glands SRX300959...48,SRX300950,SRX252733,SRX300954,SRX213925,SRX213917 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/NoD.Oth.20.AllAg.Adrenal_Glands.bed ...

  6. File list: DNS.Oth.50.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Oth.50.AllAg.Adrenal_Glands hg19 DNase-seq Others Adrenal Glands SRX040375,SRX2...01798,SRX026916,SRX100963,SRX201797,SRX121284,SRX100961 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/DNS.Oth.50.AllAg.Adrenal_Glands.bed ...

  7. File list: DNS.Oth.05.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available DNS.Oth.05.AllAg.Adrenal_Glands hg19 DNase-seq Others Adrenal Glands SRX040375,SRX1...00961,SRX201798,SRX026916,SRX100963,SRX121284,SRX201797 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/DNS.Oth.05.AllAg.Adrenal_Glands.bed ...

  8. File list: ALL.Oth.05.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available ALL.Oth.05.AllAg.Adrenal_Glands hg19 All antigens Others Adrenal Glands SRX300959,S...54,SRX213917 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/ALL.Oth.05.AllAg.Adrenal_Glands.bed ...

  9. File list: ALL.Oth.50.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available ALL.Oth.50.AllAg.Adrenal_Glands hg19 All antigens Others Adrenal Glands SRX300959,S...33,SRX213917 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/ALL.Oth.50.AllAg.Adrenal_Glands.bed ...

  10. File list: ALL.Oth.20.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available ALL.Oth.20.AllAg.Adrenal_Glands hg19 All antigens Others Adrenal Glands SRX300959,S...25,SRX213917 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/ALL.Oth.20.AllAg.Adrenal_Glands.bed ...

  11. File list: NoD.Oth.05.AllAg.Adrenal_Glands [Chip-atlas[Archive

    Lifescience Database Archive (English)

    Full Text Available NoD.Oth.05.AllAg.Adrenal_Glands hg19 No description Others Adrenal Glands SRX300959...50,SRX213925,SRX342272,SRX252733,SRX300954,SRX213917 http://dbarchive.biosciencedbc.jp/kyushu-u/hg19/assembled/NoD.Oth.05.AllAg.Adrenal_Glands.bed ...

  12. Absence of clinically relevant growth acceleration in untreated children with non-classical congenital adrenal hyperplasia.

    NARCIS (Netherlands)

    Pijnenburg-Kleizen, K.J.; Borm, G.F.; Otten, B.J.; Schott, D.A.; Akker, E.L. van den; Stokvis-Brantsma, W.H.; Voorhoeve, P.G.; Bakker, B.; Claahsen-van der Grinten, H.L.

    2012-01-01

    BACKGROUND/AIMS: In classical congenital adrenal hyperplasia (CAH), elevation of adrenal androgens leads to accelerated growth and bone maturation with compromised adult height. In untreated children with non-classical CAH (NC-CAH), in which adrenal androgens are generally only slightly increased, g

  13. [Adrenal hemorrhage in a newborn with factor V Leiden--a clinical case].

    Science.gov (United States)

    Vulkova, A; Kovacheva, K; ionov, M; Rosmanova, R; Atanasova, V; Slavkova, N; Ivanov, P

    2009-01-01

    Neonatal adrenal hemorrhage is frequently associated with birth trauma, perinatal asphyxia, septicemia, coagulation defects and thromboembolism. We report a case of bilateral adrenal hemorrhage in newborn baby with perinatal asphyxia. Ultrasound findings of subacute adrenal hemorrhage draw attention to its presumable antenatal genesis. DNA analysis for thrombophilic mutations identifies factor V Leiden.

  14. Urine Steroid Metabolomics as a Biomarker Tool for Detecting Malignancy in Adrenal Tumors

    NARCIS (Netherlands)

    Arlt, Wiebke; Biehl, Michael; Taylor, Angela E.; Hahner, Stefanie; Libe, Rossella; Hughes, Beverly A.; Schneider, Petra; Smith, David J.; Stiekema, Han; Krone, Nils; Porfiri, Emilio; Opocher, Giuseppe; Bertherat, Jerome; Mantero, Franco; Allolio, Bruno; Terzolo, Massimo; Nightingale, Peter; Shackleton, Cedric H. L.; Bertagna, Xavier; Fassnacht, Martin; Stewart, Paul M.

    2011-01-01

    Context: Adrenal tumors have a prevalence of around 2% in the general population. Adrenocortical carcinoma (ACC) is rare but accounts for 2-11% of incidentally discovered adrenal masses. Differentiating ACC from adrenocortical adenoma (ACA) represents a diagnostic challenge in patients with adrenal

  15. Cortisol-dependent stress effects on cell distribution in healthy individuals and individuals suffering from chronic adrenal insufficiency.

    Science.gov (United States)

    Geiger, Ashley M; Pitts, Kenneth P; Feldkamp, Joachim; Kirschbaum, Clemens; Wolf, Jutta M

    2015-11-01

    Chronic adrenal insufficiency (CAI) is characterized by a lack of glucocorticoid and mineralocorticoid production due to destroyed adrenal cortex cells. However, elevated cortisol secretion is thought to be a central part in a well-orchestrated immune response to stress. This raises the question to what extent lack of cortisol in CAI affects stress-related changes in immune processes. To address this question, 28 CAI patients (20 females) and 18 healthy individuals (11 females) (age: 44.3 ± 8.4 years) were exposed to a psychosocial stress test (Trier Social Stress Test: TSST). Half the patients received a 0.03 mg/kg body weight injection of hydrocortisone (HC) post-TSST to mimic a healthy cortisol stress response. Catecholamines and immune cell composition were assessed in peripheral blood and free cortisol measured in saliva collected before and repeatedly after TSST. CAI patients showed norepinephrine (NE) stress responses similar to healthy participants, however, epinephrine (E) as well as cortisol levels were significantly lower. HC treatment post-TSST resulted in cortisol increases comparable to those observed in healthy participants (interaction effects--NE: F=1.05, p=.41; E: F=2.56, p=.045; cortisol: F=13.28, pcortisol's central involvement in post-stress lymphocyte migration from blood into immune-relevant body compartments. As such, future studies should investigate whether psychosocial stress exposure may put CAI patients at an increased health risk due to attenuated immune responses to pathogens.

  16. Insulin and IGF1 receptors are essential for XX and XY gonadal differentiation and adrenal development in mice.

    Directory of Open Access Journals (Sweden)

    Jean-Luc Pitetti

    Full Text Available Mouse sex determination provides an attractive model to study how regulatory genetic networks and signaling pathways control cell specification and cell fate decisions. This study characterizes in detail the essential role played by the insulin receptor (INSR and the IGF type I receptor (IGF1R in adrenogenital development and primary sex determination. Constitutive ablation of insulin/IGF signaling pathway led to reduced proliferation rate of somatic progenitor cells in both XX and XY gonads prior to sex determination together with the downregulation of hundreds of genes associated with the adrenal, testicular, and ovarian genetic programs. These findings indicate that prior to sex determination somatic progenitors in Insr;Igf1r mutant gonads are not lineage primed and thus incapable of upregulating/repressing the male and female genetic programs required for cell fate restriction. In consequence, embryos lacking functional insulin/IGF signaling exhibit (i complete agenesis of the adrenal cortex, (ii embryonic XY gonadal sex reversal, with a delay of Sry upregulation and the subsequent failure of the testicular genetic program, and (iii a delay in ovarian differentiation so that Insr;Igf1r mutant gonads, irrespective of genetic sex, remained in an extended undifferentiated state, before the ovarian differentiation program ultimately is initiated at around E16.5.

  17. Insulin and IGF1 receptors are essential for XX and XY gonadal differentiation and adrenal development in mice.

    Science.gov (United States)

    Pitetti, Jean-Luc; Calvel, Pierre; Romero, Yannick; Conne, Béatrice; Truong, Vy; Papaioannou, Marilena D; Schaad, Olivier; Docquier, Mylène; Herrera, Pedro Luis; Wilhelm, Dagmar; Nef, Serge

    2013-01-01

    Mouse sex determination provides an attractive model to study how regulatory genetic networks and signaling pathways control cell specification and cell fate decisions. This study characterizes in detail the essential role played by the insulin receptor (INSR) and the IGF type I receptor (IGF1R) in adrenogenital development and primary sex determination. Constitutive ablation of insulin/IGF signaling pathway led to reduced proliferation rate of somatic progenitor cells in both XX and XY gonads prior to sex determination together with the downregulation of hundreds of genes associated with the adrenal, testicular, and ovarian genetic programs. These findings indicate that prior to sex determination somatic progenitors in Insr;Igf1r mutant gonads are not lineage primed and thus incapable of upregulating/repressing the male and female genetic programs required for cell fate restriction. In consequence, embryos lacking functional insulin/IGF signaling exhibit (i) complete agenesis of the adrenal cortex, (ii) embryonic XY gonadal sex reversal, with a delay of Sry upregulation and the subsequent failure of the testicular genetic program, and (iii) a delay in ovarian differentiation so that Insr;Igf1r mutant gonads, irrespective of genetic sex, remained in an extended undifferentiated state, before the ovarian differentiation program ultimately is initiated at around E16.5.

  18. Word Recognition in Auditory Cortex

    Science.gov (United States)

    DeWitt, Iain D. J.

    2013-01-01

    Although spoken word recognition is more fundamental to human communication than text recognition, knowledge of word-processing in auditory cortex is comparatively impoverished. This dissertation synthesizes current models of auditory cortex, models of cortical pattern recognition, models of single-word reading, results in phonetics and results in…

  19. Atrophy pattern and significance of frontal cortex and cingulate cortex in patients with Alzheimer′s disease%阿尔茨海默病患者额叶、扣带回皮层萎缩模式观察及意义

    Institute of Scientific and Technical Information of China (English)

    赵辉; 武文博; 钱来; 李政; 张冰

    2014-01-01

    Objective To investigate the atrophy pattern and its significance of frontal cortex and cingulate cortex in Alzheimer′s disease ( AD) .Methods According to the scors of MMSE and CDR , 28 AD patients were divided into the mild AD group (n=14) and the moderate AD group (n=14);20 healthy person were as the normol control group (NC group).The cognitive function were assessed by MMSE , CDR, MOCA, ADL, HAMD and NPI.All subjects received MRI inspection.Then analysis of image and data was based on the model using FreeSurfer v 5.1.0 software.Results In NC, the mild and moderate AD groups , the scores of MMSE and MOCA decreased gradually , the scores of CDR , ADL and NPI increased gradually (all P0.05).The left caudal middle frontal and the left lateral or-bito frontal were the risk factors for dementia in AD (all P<0.05).Conclusions The atrophy of frontal lobe and cingu-late cortex existed in mild AD patients and the atrophy of each subregion was inhomogeneity .The left caudal middle frontal and the left lateral orbito frontal could predict cognitive impairment in AD .%目的:观察阿尔茨海默病( AD)患者额叶、扣带回皮层的萎缩模式,并探讨其意义。方法根据简易智能状态检查( MMSE)评分及临床痴呆评定量表( CDR)评分将28例AD患者分为轻度AD组、中度AD组各14例,同期体检健康者20例作为正常对照组。采用MMSE、CDR、蒙特利尔认知评估量表( MOCA )、日常生活活动量表(ADL)、汉密尔顿抑郁量表(HAMD)、神经精神量表(NPI),评估各组的认知功能。各组均行头颅MRI检查,Free-Surfer v5.1.0软件分析图像及数据。结果正常对照组、轻度AD组及中度AD组的MMSE、MOCA均依次降低, CDR、ADL、NPI均依次升高;组间两两比较,P均<0.05。额叶皮层各亚区中,轻度AD组、中度AD组双侧内侧眶回、外侧眶回及眶部皮层厚度均小于正常对照组,中度AD组左侧额中

  20. Physical, social and societal functioning of children with congenital adrenal hyperplasia (CAH and their parents, in a Dutch population

    Directory of Open Access Journals (Sweden)

    Sanches Sarita A

    2012-02-01

    Full Text Available Abstract Background Most research concerning congenital adrenal hyperplasia (CAH and related conditions caused by primary adrenal insufficiency, such as Addison's or Cushing's disease, has focused on medical aspects rather than on patients' quality of life. Therefore, our objective was to investigate the physical, social and societal functioning of children with CAH and their parents in a Dutch population. Methods The study is descriptive and cross-sectional. Self-designed questionnaires, based on questionnaires developed in the Netherlands for different patient groups, were sent to parents of children with CAH between 0 and 18 years old. Participants were recruited through the Dutch patient group for Adrenal Disease (NVACP and six hospitals in the Netherlands. Three different questionnaires were designed for parents: for children aged 0 - 4, aged 4 - 12 and aged 12 - 18. Additionally, a fourth questionnaire was sent to adolescents with CAH aged 12 - 18. Main outcome measures were experienced burden of the condition, self-management and participation in several areas, such as school and leisure time. Results A total of 106 parents returned the questionnaire, 12 regarding pre-school children (0-4 years, 63 regarding primary school children (4-12 years, and 32 regarding secondary school children (12-18 years, combined response rate 69.7%. Also, 24 adolescents returned the questionnaire. Children and adolescents with CAH appear to be capable of self-management at a young age. Experienced burden of the condition is low, although children experience several health related problems on a daily basis. Children participate well in school and leisure time. Few children carry a crisis card or emergency injection with them. Conclusions Overall, our research shows that, according to their parents, children with CAH experience few negative effects of the condition and that they participate well in several areas such as school and leisure time. However