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Sample records for acth-dependent cushings syndrome

  1. Difficulties in the diagnosis of ACTH-dependent Cushings syndrome in a patient after left adrenalectomy and treated with glucocorticoids

    International Nuclear Information System (INIS)

    Brzezinska, B.; Junik, R.; Kaminska, A.; Zielinski, G.

    2009-01-01

    Cushings syndrome (CS), that is a consequence of chronic excess of corticosteroides, is most frequently of iatrogenic origin. Corticotropin secreting pituitary adenomas are responsible for most cases of endogenous Cushings syndrome. Difficulties in the diagnosis and treatment of ACTH-dependent Cushings syndrome concern with localization of the source of pathological ACTH secretion, particularly when magnetic resonance imaging is unable to identify the pituitary microadenoma. In this paper we present the case of a patient with symptoms of Cushings syndrome and describe problems with localization of the source of hypercortisolemia. The diagnostic process was additionally complicated by the treatment with corticosteroids, occasionally applied due to concomitant diseases. This delayed the right diagnosis and treatment. (authors)

  2. [Difficulties in the diagnosis of ACTH-dependent Cushing's syndrome in a patient after left adrenalectomy and treated with glucocorticoids].

    Science.gov (United States)

    Brzezińska, Barbara; Junik, Roman; Kamińska, Anna; Zieliński, Grzegorz

    2009-01-01

    Cushing's syndrome (CS), that is a consequence of chronic excess of corticosteroides, is most frequently of iatrogenic origin. Corticotropin secreting pituitary adenomas are responsible for most cases of endogenous Cushing' s syndrome. Difficulties in the diagnosis and treatment of ACTH-dependent Cushing's syndrome concern with localization of the source of pathological ACTH secretion, particularly when magnetic resonance imaging is unable to identify the pituitary microadenoma. In this paper we present the case of a patient with symptoms of Cushing's syndrome and describe problems with localization of the source of hypercortisolemia. The diagnostic process was additionally complicated by the treatment with corticosteroids, occasionally applied due to concomitant diseases. This delayed the right diagnosis and treatment.

  3. Cushing syndrome: update on testing.

    Science.gov (United States)

    Raff, Hershel

    2015-03-01

    Endogenous hypercortisolism (Cushing syndrome) is one of the most enigmatic diseases in clinical medicine. The diagnosis and differential diagnosis of Cushing syndrome depend on proper laboratory evaluation. In this review, an update is provided on selected critical issues in the diagnosis and differential diagnosis of Cushing syndrome: the use of late-night salivary cortisol in initial diagnosis and for postoperative surveillance, and the use of prolactin measurement to improve the performance of inferior petrosal sinus sampling to distinguish Cushing disease from ectopic adrenocorticotropic hormone (ACTH) syndrome during differential diagnosis of ACTH-dependent Cushing syndrome. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Imaging in Cushing's syndrome

    International Nuclear Information System (INIS)

    Sahdev, Anju; Evanson, Jane; Reznek, Rodney H.; Grossman, Ashley B.

    2007-01-01

    Once the diagnosis of Cushing's syndrome (CS) has been established, the main step is to differentiate between ACTH dependent and independent disease. In adults, 80% of CS is due to ACTH-dependent causes and 20% due to adrenal causes. ACTH-secreting neoplasms cause ACTH-dependent CS. These are usually anterior pituitary microadenomas, which result in the classic Cushing's disease. Non-pituitary ectopic sources of ACTH, such as a small-cell lung carcinoma or carcinoid tumours, are the source of the remainder of ACTH-dependent disease. In the majority of patients presenting with clinical and biochemical evidence of CS, modern non-invasive imaging can accurately and efficiently provide the cause and the nature of the underlying pathology. Imaging is essential for determining the source of ACTH in ectopic ACTH production, locating the pituitary tumours and distinguishing adrenal adenomas, carcinomas and hyperplasias. In our chapter we review the adrenal appearances in ACTH-dependent and ACTH-independent CS. We also include a discussion on the use of MRI and CT for the detection and management of pituitary ACTH secreting adenomas. CT of the chest, abdomen and pelvis with intravenous injection of contrast medium is the most sensitive imaging modality for the identification of the ectopic ACTH source and detecting adrenal pathology. MRI is used for characterising adrenal adenomas, problem solving in difficult cases and for detecting ACTH-secreting pituitary adenomas. (author)

  5. Cushing's Syndrome

    Science.gov (United States)

    Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone that ... your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

  6. Cushing syndrome

    Science.gov (United States)

    ... mellitus High blood pressure (hypertension) Increased cholesterol and triglycerides (hyperlipidemia) Women with Cushing syndrome may have: Excess ... Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, ...

  7. Exophthalmos in Cushing's syndrome.

    Science.gov (United States)

    Kelly, W

    1996-08-01

    Exophthalmos was noted in 4 of the 12 patients reported by Harvey Cushing in 1932. Although exophthalmos has often been included in clinical descriptions, no previous study has reported actual measurements in patients with active and treated Cushing's syndrome, and in control patients. The aim of this study was to obtain these measurements. Thirty-one patients with active Cushing's syndrome (19 iatrogenic), 15 with treated Cushing's syndrome, 18 with Graves' ophthalmopathy, 59 control patients, and 3 patients with active Cushing's syndrome plus a family or personal history of thyroid disease. A consecutive series of patients with active and treated Cushing's syndrome were assessed. They were compared with patients with Graves' ophthalmopathy, and with control patients. Exophthalmos was assessed by the author using a Hertel meter. Urinary free cortisol was measured on patients with Cushing's syndrome, and serum thyroxine was estimated for them, and for the patients with Graves' ophthalmopathy. Exophthalmos exceeding 16 mm (> 2 SD above normal mean) was found in 45% of active Cushing's syndrome, 21% of iatrogenic Cushing's syndrome, 20% of treated Cushing's syndrome, 2% of normal controls, and 77% of patients with Graves' ophthalmopathy. No patient with Cushing's syndrome had significant symptoms due to exophthalmos. Patients with active Cushing's syndrome have statistically significant exophthalmos. This rarely causes symptoms, and diminishes when cortisol concentrations become normal. Cushing's syndrome and autoimmune thyroid disease may coexist in patients with exophthalmos.

  8. Treatment of adrenocorticotropin-dependent cushing's syndrome: A consensus statement

    NARCIS (Netherlands)

    B.M.K. Biller; A. Grossman (Ashley Barry); P.M. Stewart; S. Melmed (Shlomo); X. Bertagna; J. Bertherat (Jerome); M. Buchfelder; A. Colao (Annamaria); A.R.M.M. Hermus (Ad); L.J. Hofland (Leo); A. Klibanski; A. Lacroix; J.R. Lindsay; J. Newell-Price; L.K. Nieman; S. Petersenn; N. Sonino; G.K. Stalla (Günter); B. Swearingen; M.L. Vance; J.A.H. Wass (John); M. Boscaro

    2008-01-01

    textabstractObjective: Our objective was to evaluate the published literature and reach a consensus on the treatment of patients with ACTH-dependent Cushing's syndrome, because there is no recent consensus on the management of this rare disorder. Participants: Thirty-two leading endocrinologists,

  9. Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement.

    NARCIS (Netherlands)

    Biller, B.M.; Grossman, A.B.; Stewart, P.M.; Melmed, S.; Bertagna, X.; Bertherat, J.; Buchfelder, M.; Colao, A.; Hermus, A.R.M.M.; Hofland, L.J.; Klibanski, A.; Lacroix, A.; Lindsay, J.R.; Newell-Price, J.; Nieman, L.K.; Petersenn, S.; Sonino, N.; Stalla, G.K.; Swearingen, B.; Vance, M.L.; Wass, J.A.; Boscaro, M.

    2008-01-01

    OBJECTIVE: Our objective was to evaluate the published literature and reach a consensus on the treatment of patients with ACTH-dependent Cushing's syndrome, because there is no recent consensus on the management of this rare disorder. PARTICIPANTS: Thirty-two leading endocrinologists, clinicians,

  10. Imaging in Cushing's syndrome; Imagem em sindrome de Cushing

    Energy Technology Data Exchange (ETDEWEB)

    Sahdev, Anju; Evanson, Jane [St. Bartholomew' s Hospital, London (United Kingdom). Dept. of Diagnostic Imaging; Reznek, Rodney H. [St. Bartholomew' s Hospital, London (United Kingdom). Institute of Cancer. Cancer Imaging; Grossman, Ashley B. [St. Bartholomew' s Hospital, London (United Kingdom). Dept. of Endocrinology]. E-mail: anju.sahdev@bartsandthelondon.nhs.uk

    2007-11-15

    Once the diagnosis of Cushing's syndrome (CS) has been established, the main step is to differentiate between ACTH dependent and independent disease. In adults, 80% of CS is due to ACTH-dependent causes and 20% due to adrenal causes. ACTH-secreting neoplasms cause ACTH-dependent CS. These are usually anterior pituitary microadenomas, which result in the classic Cushing's disease. Non-pituitary ectopic sources of ACTH, such as a small-cell lung carcinoma or carcinoid tumours, are the source of the remainder of ACTH-dependent disease. In the majority of patients presenting with clinical and biochemical evidence of CS, modern non-invasive imaging can accurately and efficiently provide the cause and the nature of the underlying pathology. Imaging is essential for determining the source of ACTH in ectopic ACTH production, locating the pituitary tumours and distinguishing adrenal adenomas, carcinomas and hyperplasias. In our chapter we review the adrenal appearances in ACTH-dependent and ACTH-independent CS. We also include a discussion on the use of MRI and CT for the detection and management of pituitary ACTH secreting adenomas. CT of the chest, abdomen and pelvis with intravenous injection of contrast medium is the most sensitive imaging modality for the identification of the ectopic ACTH source and detecting adrenal pathology. MRI is used for characterising adrenal adenomas, problem solving in difficult cases and for detecting ACTH-secreting pituitary adenomas. (author)

  11. Progestogens and Cushing's syndrome.

    LENUS (Irish Health Repository)

    Harte, C

    2012-02-03

    We report 3 patients where Medroxyprogesterone Acetate (MPA = Provera) and Megestrol Acetate (Megace) in doses used for therapy of breast cancer, caused clinical hypercortisolism and Cushing\\'s syndrome. Studies of the toxicity of Medroxyprogesterone Acetate list the commonest adverse events at 500 mg\\/day as weight gain, water retention, increased blood pressure, tremor, moon face, sweating, muscle cramps, vaginal bleeding and increased appetite. Glucocorticoid-like effects are seen in up to 30% of patients treated for longer than 6 weeks with mostly large doses of the order of 1500 mg\\/day but Cushing\\'s syndrome has been reported in patients taking 400 mg\\/day. Neither the glucocorticoid-like effects or Cushing\\'s syndrome have been previously observed with Megestrol Acetate. In the elderly female population receiving progestogens for neoplastic disease the progestogen itself could be an appreciable cause of morbidity both by causing glucocorticoid-like effects and Cushing\\'s syndrome but also by lack of awareness of the danger of sudden withdrawal of these compounds when the hypothalmic-pituitary-adrenal (HPA) axis is suppressed. The signs and symptoms could be easily overlooked unless appropriate testing for Cushing\\'s syndrome is carried out. While the progestogen may have to be continued indefinitely a dose decrease may be feasible with reduction of morbidity.

  12. Selenium-75-cholesterol imaging and computed tomography of the adrenal glands in differentiating the cause of Cushing's syndrome

    International Nuclear Information System (INIS)

    Miller, J.L.; Smith, J.A.; Mervis, B.; Roman, T.

    1983-01-01

    Measurement of 75 Se-cholesterol (Scintadren) uptake and computed tomography (CT) of the adrenal glands were compared as a means of differentiating the cause of Cushing's syndrome in 11 patients over a 2-year period. Quantitative Scintadren imaging differentiated adrenocorticotrophic hormone (ACTH)-dependent disease from local adrenocortical lesions as the cause of Cushing's syndrome in all the patients studied. CT of the adrenal glands rapidly and accurately detected the adrenal mass lesions in 2 cases and was effective in documenting bilateral hyperplasia due to ectopic ACTH-dependent disease. However, in entopic ACTH (pituitary)-dependent disease the adrenal glands were of normal thickness in all but 2 patients, who had bilateral hyperplasia. Scintadren imaging and CT are useful non-invasive procedures for differentiating local adrenal disease from ACTH-dependent disease as the cause of Cushing's syndrome and should be the initial investigations once a firm clinical and biochemical diagnosis of Cushing's syndrome has been made

  13. [Diagnosis and differential diagnosis of Cushing's syndrome].

    Science.gov (United States)

    Santos, Silvia; Santos, Estefanía; Gaztambide, Sonia; Salvador, Javier

    2009-02-01

    Because of the variability in the clinical expression of Cushing's syndrome, different biochemical tests and imaging techniques must be used for effective diagnosis and treatment. The clinical history is important to assess the general impact of hypercortisolism on organs and systems, as well as to guide suspicion toward more aggressive entities such as overt ectopic ACTH syndrome or to detect an iatrogenic etiology of Cushing's syndrome. The screening phase relies on tests that are sensitive but not specific, such as urinary free cortisol, nocturnal salivary cortisol and 1 mg dexamethasone suppression, which still require established assessment criteria. Confirmation can be obtained by repeating urinary cortisol, 2 mg/day dexamethasone suppression, both alone and combined with corticotropin releasing hormone (CRH), to exclude pseudoCushing states. ACTH dependency can be easily explored by ACTH measurements, but the differential diagnosis between pituitary and ectopic Cushing's disease is difficult and may require invasive procedures such as inferior petrosal sinus sampling. Sophisticated imaging and isotopic techniques play a significant role in locating the source of ACTH in ectopic syndromes but are not always effective. In general, biochemical and imaging tests should be combined in order to assess different mechanisms and perspectives of the syndrome. Rigorous methodology is essential to obtain accurate results, allowing a correct diagnosis and improving therapeutic performance in this devastating disease.

  14. Nuclear imaging for the Cushing's syndrome etiological diagnosis; Les scintigraphies des etiologies du syndrome de Cushing

    Energy Technology Data Exchange (ETDEWEB)

    Nocaudie, M. [Centre Hospitalier Universitaire, 59 - Lille (France). Service central de medecine nucleaire et imagerie fonctionnelle

    2000-11-01

    Etiologic diagnosing of a Cushing's syndrome relies upon the probabilities of the various causes of the pathologies. It takes advantage of the hormonal determinations to establish the mechanism of the hyper-secretion and of the radiological examination to detect morphological abnormalities. The scinti-scans are useful at this time only, to locate hyper-functioning tissue, to guide its resection and to suggest alternative option, either pharmaceutical or radio-metabolic. In the ACTH-independent Cushing's syndrome, noriodocholesterol scintigraphy can indicate that the adrenocortical hyper-functioning is unilateral or that it is bilateral. In the ACTH-dependent Cushing's syndrome, the current somatostatin radio-analogs have not proven their efficiency in pituitary ACTH-producing tumours but they are useful in the diagnosis and the management of bronchial carcinoids as of other neuro-endocrine tumors with the para-neoplastic Cushing's syndrome. (author)

  15. INFANTILE IATROGENIC CUSHING'S SYNDROME

    OpenAIRE

    Katar, Selahattin; Akdeniz, Sedat; ?zbek, M Nuri; Yarami?, Ahmet

    2008-01-01

    High potency or/and extended use of topical corticosteroids, particularly in children, may cause suppression of the hypothalamopituitary-adrenal axis. However, iatrogenic Cushing's syndrome in infantile age group is very rare and only a few patients have been reported to date in the literature. Here, we report a case of iatrogenic Cushing's syndrome in a 6-month-old male child whose parents have admitted to the hospital for overweight and skin fragility.

  16. Infantile iatrogenic Cushing's syndrome.

    Science.gov (United States)

    Katar, Selahattin; Akdeniz, Sedat; Ozbek, M Nuri; Yaramiş, Ahmet

    2008-01-01

    High potency or/and extended use of topical corticosteroids, particularly in children, may cause suppression of the hypothalamopituitary-adrenal axis. However, iatrogenic Cushing's syndrome in infantile age group is very rare and only a few patients have been reported to date in the literature. Here, we report a case of iatrogenic Cushing's syndrome in a 6-month-old male child whose parents have admitted to the hospital for overweight and skin fragility.

  17. [Cushing syndrome: Physiopathology, etiology and principles of therapy].

    Science.gov (United States)

    Chabre, Olivier

    2014-04-01

    The most frequent cause of Cushing's syndrome is iatrogenic, as Cushing's syndrome is the unavoidable consequence of long-term glucocorticoid treatment using more than 7.5 mg prednisone per day. The most frequent cause of endogenous Cushing's syndrome is Cushing's disease (CD), which is an ACTH dependent hypercortisolism linked to a pituitary corticotroph adenoma. This adenoma is often very small, its diagnosis may require bilateral inferior petrosal sinus sampling and the first line treatment of CD is transsphenoidal surgery by an expert neurosurgeon. The second line treatments include drugs that can act either on the pituitary adenoma or on adrenal steroidogenesis, pituitary radiotherapy or bilateral adrenalectomy. Ectopic ACTH dependent Cushing's syndrome is linked either to poorly differentiated endocrine tumors with a very poor prognosis, such as small cell lung cancer, or to well differentiated endocrine tumors, such as bronchial carcinoid tumors, which have a good prognosis when treated by surgery, but may be very difficult to localize. Adrenal Cushing's syndromes, which are independent of pituitary ACTH secretion, include adrenal cortex carcinoma, which requires abdominal surgery with extended adrenalectomy by an expert surgeon, adrenal adenoma which is treated by laparoscopic unilateral adrenalectomy and bilateral macronodular hyperplasia, whose surgical treatment may require unilateral or bilateral adrenalectomy. Treatment of Cushing's syndrome generally leads to spectacular clinical results, which must not hide the fact that the reversibility of some signs is actually incomplete. This underlines the need for a timely multidisciplinary management of the patients by an expert team. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  18. Inferior petrosal sinus sampling in the diagnosis of adrenocorticotropin dependent Cushing syndrome with unknown origin

    International Nuclear Information System (INIS)

    Shen Xuefeng; Yuan Dequan; Yue Ming; Feng Juanjuan

    2011-01-01

    Objective: To evaluate the value of inferior petrosal sinus sampling (IPSS) in the diagnosis of adrenocorticotropic hormone (ACTH) dependent Cushing syndrome (CS) with unknown origin. Methods: IPSS was carried out for the diagnosis of 16 cases with ACTH dependent CS who had not been identified after a series of dexamethasone suppression tests and radiological examinations. The ratio of inferior petrosal sinus/peripheral ACTH was assayed. The sensitivity and specificity of diagnosis of the Cushing disease were estimated. Results: The inferior petrosal sinus/peripheral ACTH ratio was over 2.0 in 13 cases. Twelve cases underwent surgery with pathological diagnosis of pituitary ACTH adenoma, 1 patient relieved after γ knife treatment. The ratio was < 2.0 in 3 cases including 2 pulmonary carcinoid and one pituitary ACTH adenoma. The sensitivity and specify of IPSS for the diagnosis of Cushing disease were 13/14 and 2/2 respectively. Conclusion: IPSS was a safe technique with high sensitivity, specify and infrequent complications in the diagnosis of ACTH dependent Cushing disease. It had great clinical value in the differential diagnosis of ACTH dependent Cushing disease with unknown origin. (authors)

  19. Systemic therapy of Cushing's syndrome.

    Science.gov (United States)

    Eckstein, Niels; Haas, Bodo; Hass, Moritz David Sebastian; Pfeifer, Vladlena

    2014-08-05

    Cushing's disease (CD) in a stricter sense derives from pathologic adrenocorticotropic hormone (ACTH) secretion usually triggered by micro- or macroadenoma of the pituitary gland. It is, thus, a form of secondary hypercortisolism. In contrast, Cushing's syndrome (CS) describes the complexity of clinical consequences triggered by excessive cortisol blood levels over extended periods of time irrespective of their origin. CS is a rare disease according to the European orphan regulation affecting not more than 5/10,000 persons in Europe. CD most commonly affects adults aged 20-50 years with a marked female preponderance (1:5 ratio of male vs. female). Patient presentation and clinical symptoms substantially vary depending on duration and plasma levels of cortisol. In 80% of cases CS is ACTH-dependent and in 20% of cases it is ACTH-independent, respectively. Endogenous CS usually is a result of a pituitary tumor. Clinical manifestation of CS, apart from corticotropin-releasing hormone (CRH-), ACTH-, and cortisol-producing (malign and benign) tumors may also be by exogenous glucocorticoid intake. Diagnosis of hypercortisolism (irrespective of its origin) comprises the following: Complete blood count including serum electrolytes, blood sugar etc., urinary free cortisol (UFC) from 24 h-urine sampling and circadian profile of plasma cortisol, plasma ACTH, dehydroepiandrosterone, testosterone itself, and urine steroid profile, Low-Dose-Dexamethasone-Test, High-Dose-Dexamethasone-Test, after endocrine diagnostic tests: magnetic resonance imaging (MRI), ultra-sound, computer tomography (CT) and other localization diagnostics. First-line therapy is trans-sphenoidal surgery (TSS) of the pituitary adenoma (in case of ACTH-producing tumors). In patients not amenable for surgery radiotherapy remains an option. Pharmacological therapy applies when these two options are not amenable or refused. In cases when pharmacological therapy becomes necessary, Pasireotide should be used in

  20. Is Prolactin Measurement of Value during Inferior Petrosal Sinus Sampling in Patients with ACTH-dependent Cushing’s Syndrome?

    Science.gov (United States)

    Sharma, S. T.; Nieman, L. K.

    2014-01-01

    Inferior petrosal sinus sampling (IPSS) is considered the gold standard test to distinguish between Cushing’s disease (CD) and ectopic ACTH syndrome (EAS). Anomalous venous drainage, abnormal venous anatomy and lack of expertise can lead to false-negative IPSS results and thereby misclassification of patients with ACTH-dependent Cushing’s syndrome. Prolactin measurement during IPSS can improve diagnostic accuracy and decrease false negative results. A baseline prolactin inferior petrosal sinus to peripheral (IPS/P) ratio (ipsilateral to the dominant post-CRH ACTH IPS/P ratio) of 1.8 or more suggests successful catheterization during IPSS. Prolactin-normalized ACTH IPS/P ratios can then be used to differentiate between a pituitary and ectopic source of ACTH. Values ≤ 0.7 are suggestive of EAS and those ≥ 1.3 are indicative of CD but the implication of values between 0.7 and 1.3 remains unclear and needs further investigation. Larger prospective studies are also needed for further evaluation of the role of contralateral prolactin IPS/P ratios, post-CRH prolactin values and prolactin-adjusted ACTH inter-sinus ratios for tumor localization in CD. PMID:23887034

  1. What Causes Cushing's Syndrome?

    Science.gov (United States)

    ... it causes has been given its own name, Cushing disease. Pituitary adenomas account for 70% (in adults) to ... is sometimes called ectopic (pronounced ek-TOP-ik ) Cushing disease and accounts for about 15% of non-medicine- ...

  2. Cushing Syndrome: Diagnostic Workup and Imaging Features, With Clinical and Pathologic Correlation.

    Science.gov (United States)

    Wagner-Bartak, Nicolaus A; Baiomy, Ali; Habra, Mouhammed Amir; Mukhi, Shalini V; Morani, Ajaykumar C; Korivi, Brinda R; Waguespack, Steven G; Elsayes, Khaled M

    2017-07-01

    Cushing syndrome (CS) is a constellation of clinical signs and symptoms resulting from chronic exposure to excess cortisol, either exogenous or endogenous. Exogenous CS is most commonly caused by administration of glucocorticoids. Endogenous CS is subdivided into two types: adrenocorticotropic hormone (ACTH) dependent and ACTH independent. Cushing disease, which is caused by a pituitary adenoma, is the most common cause of ACTH-dependent CS for which pituitary MRI can be diagnostic, with bilateral inferior petrosal sinus sampling useful in equivocal cases. In ectopic ACTH production, which is usually caused by a tumor in the thorax (e.g., small cell lung carcinoma, bronchial and thymic carcinoids, or medullary thyroid carcinoma) or abdomen (e.g., gastroenteropancreatic neuroendocrine tumors or pheochromocytoma), CT, MRI, and nuclear medicine tests are used for localizing the source of ACTH. In ACTH-independent CS, which is caused by various adrenal abnormalities, adrenal protocol CT or MRI is usually diagnostic.

  3. Recent developments in canine Cushing`s syndrome

    NARCIS (Netherlands)

    Galac, S.

    2010-01-01

    Cushing`s syndrome or hypercortisolism is one of the most common endocrinopathies in dogs. Besides the ACTH or pituitary-dependent and adrenal or ACTH-independent hypercortisolism, ectopic ACTH secretion and food-dependent hypercortisolism are described in the dog for the very first time. Ectopic

  4. Limited Diagnostic Utility of Plasma Adrenocorticotropic Hormone for Differentiation between Adrenal Cushing Syndrome and Cushing Disease.

    Science.gov (United States)

    Hong, A Ram; Kim, Jung Hee; Hong, Eun Shil; Kim, I Kyeong; Park, Kyeong Seon; Ahn, Chang Ho; Kim, Sang Wan; Shin, Chan Soo; Kim, Seong Yeon

    2015-09-01

    Measurement of the plasma adrenocorticotropic hormone (ACTH) level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS) and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S) level and results of the high-dose dexamethasone suppression test (HDST). The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing' disease (CD) and compare it with that of the HDST results and serum DHEA-S level. We performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC) after the HDST, and serum DHEA-S were measured. Fifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEA-S, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all Pdisease, especially when the plasma ACTH level alone is not conclusive.

  5. Diagnosis and Management of 253 Cases with Cushing's syndrome in Imam Khomeini Hospital

    Directory of Open Access Journals (Sweden)

    Esteghamati A.R

    2007-09-01

    Full Text Available Background: Definite diagnosis and treatment of Cushing's syndrome is still a dilemma. The aim of this study was to evaluate the accuracy of diagnostic tests and follow-up of patients with Cushing's syndrome."nMethods: Two hundred and fifty three consecutive cases with Cushing's syndrome during 1370-78 were studied. The screening tests were performed in all patients. High dose dexamethasone suppression test (HDDST and ACTH measurement were carried out. MRI/CT Scan were performed and compared with laboratory data and pathologic specimens as a gold standard test."nResults: The age range was 32±11 yrs. The most frequent symptoms were weakness; hypertension, typical striae, and depression .The frequency of hypertension in ACTH-dependent case were 77% vs. 36% in adrenal tumors (P< 0.001. HDDST was positive in 99% of micro and 71% of macroadenomas. Adrenal tumors showed 3.6% suppression but none in ectopic cases. HDDST had a sensitivity of 98%, specificity of 97% and accuracy equals to 98%. The frequency of different etiologies was as following: Cushing's disease in 64.8%, adrenal tumors in 32.8% and ectopic ACTH in 2.4% of patients. Trans-sphenoidal surgery (TSS was performed in 120 patients .The patients were followed for 53±25 months whose remission periods were 46.7±23.8 months (range 4-114 months. Survival analysis showed 93% remission rate in 12mo, 82% in 2yr and only 33% after 5yr.This recurrence didn't have any platue level."nConclusion: In our study, hypertension was more prevalent in ACTH-dependent Cushing's syndrome. HDDST had acceptable sensitivity, specificity and accuracy. Lifelong follow up of pituitary adenomas is inevitable in the case of progressive and gradual nature of recurrence in these tumors.

  6. Paraneoplastic Cushing syndrome, case-series and review of the literature.

    Science.gov (United States)

    Deldycke, Annelies; Haenebalcke, Christel; Taes, Youri

    2017-09-12

    Paraneoplastic Cushing syndrome is a rare condition, representing a small fraction of the adrenocorticotropic hormone (ACTH)-dependent cases of Cushing syndrome Methods: Four case descriptions and literature review, highlighting the diagnostic challenges and treatment options are presented. Different tumor types can be associated with ectopic ACTH secretion. The most common types are bronchial carcinoids and small cell lung carcinoma (SCLC). However, in approximately 10 to 20% of the cases, no overt tumor (occult tumor) can be found. The diagnosis is made in a multistep process. Firstly, hypercortisolemia and adrenocorticotropin hormone dependency have to be confirmed. Distinction between a pituitary or ectopic cause can be cumbersome. MRI of the pituitary gland, a corticotropin releasing hormone stimulation test and a sinus petrosus sampling can be used. Treatment options consist of tumor management, somatostatin analogs, steroidogenesis inhibitors, and bilateral adrenalectomy. Clinicians should consider the diagnosis, and opt for specific treatment, especially in patients with a history of neuroendocrine tumors.

  7. Unusual causes of Cushing's syndrome.

    Science.gov (United States)

    Vassiliadi, Dimitra; Tsagarakis, Stylianos

    2007-11-01

    Although in the majority of the patients with Cushing's syndrome (CS), hypercortisolism is due to ACTH hypersecretion by a pituitary tumour or to ectopic ACTH secretion from an extrapituitary neoplastic lesion or to autonomous cortisol secretion by an adrenal tumour, in occasional patients a much rarer entity may be the cause of the syndrome. Herein, we attempted to summarise and categorise these unusual causes according to their presumed aetiology. To this end, we performed a comprehensive computer-based search for unusual or rare causes of CS. The following unusual forms of CS were identified: (i) ACTH hyperesecretion due to ectopic corticotroph adenomas in the parasellar region or the neurohypophysis, or as part of double adenomas, or gangliocytomas; (ii) ACTH hypersecretion due to ectopic CRH or CRH-like peptide secretion by various neoplasms; (iii) ACTH-independent cortisol hypersecretion from ectopic or bilateral adrenal adenomas; (iv) glucocorticoid hypersensitivity; (v) iatrogenic, due to megestrol administration or to ritonavir and fluticasone co-administration. Such unusual presentations of CS illustrate why Cushing's syndrome represents one of the most puzzling endocrine syndromes.

  8. Exogenous Cushing syndrome

    Science.gov (United States)

    ... syndrome occurs when a person takes man-made (synthetic) glucocorticoid medicines to treat a disease. Glucocorticoids are given for many diseases, such as lung diseases, skin conditions, inflammatory bowel disease, cancer, brain tumors, and ...

  9. Paraneoplastic Cushing Syndrome Due To Wilm's Tumor.

    Science.gov (United States)

    Faizan, Mahwish; Manzoor, Jaida; Saleem, Muhammad; Anwar, Saadia; Mehmood, Qaiser; Hameed, Ambreen; Ali, Agha Shabbir

    2017-05-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.

  10. Cushing's Syndrome From Pituitary Microadenoma and Pulmonary Nodules.

    Science.gov (United States)

    Tating, Dan Louie Renz P; Montevirgen, Natasha Denise S; Cajucom, Loyda

    2016-03-01

    Cushing's syndrome is a state of cortisol excess, possibly from a tumor in the pituitary gland, the adrenal gland, or an ectopic nonpituitary ACTH-secreting source. The first form, pituitary in origin, was originally described by Harvey Cushing, MD, and was labeled as Cushing's disease. Long-term therapy with glucocorticoids also can lead to iatrogenic Cushing's syndrome.

  11. Iatrogenic Cushing's Syndrome Induced by Posaconazole

    OpenAIRE

    Pilmis, Benoit; Coignard-Biehler, Hélène; Jullien, Vincent; Hermine, Olivier; Touraine, Philippe; Lecuit, Marc; Lortholary, Olivier

    2013-01-01

    Iatrogenic Cushing's syndrome is an undesirable outcome of glucocorticoids treatment. It can be increased by pharmacologic interactions. Glucocorticoid therapy, given in association with ritonavir, and some azole treatments are causes of iatrogenic Cushing's syndrome. We present a patient with common-variable immunodeficiency who received 7 years of itraconazole therapy for bronchial colonization with Aspergillus in combination with inhaled fluticasone without any Cushingoid symptoms. After a...

  12. Iatrogenic Cushing's syndrome induced by posaconazole.

    Science.gov (United States)

    Pilmis, Benoit; Coignard-Biehler, Hélène; Jullien, Vincent; Hermine, Olivier; Touraine, Philippe; Lecuit, Marc; Lortholary, Olivier

    2013-11-01

    Iatrogenic Cushing's syndrome is an undesirable outcome of glucocorticoids treatment. It can be increased by pharmacologic interactions. Glucocorticoid therapy, given in association with ritonavir, and some azole treatments are causes of iatrogenic Cushing's syndrome. We present a patient with common-variable immunodeficiency who received 7 years of itraconazole therapy for bronchial colonization with Aspergillus in combination with inhaled fluticasone without any Cushingoid symptoms. After a switch to posaconazole, the patient developed Cushingoid symptoms.

  13. Iatrogenic Cushing's Syndrome in an Infant.

    Science.gov (United States)

    Namburu, Rajendra Prasad; T S, Karthik; Reddy P, Amaresh

    2013-02-01

    A high potency, long acting and/or the extended use of oral corticosteroids, particularly in children, may cause suppression of the hypothalamo-pituitary-adrenal axis. However, the iatrogenic Cushing's syndrome in the infantile age group is rare and only few patients have been reported to date in the literature. Here, we are reporting a case of iatrogenic Cushing's syndrome in a 5-month-old male child, whose parents brought him to the hospital for puffiness of the face and overweight.

  14. Screening for Cushing's syndrome in obese patients

    Directory of Open Access Journals (Sweden)

    Ozay Tiryakioglu

    2010-01-01

    Full Text Available OBJECTIVES: The aim of this study was to examine the frequency of Cushing's syndrome (CS in obese patients devoid of specific clinical symptoms of Cushing's syndrome. METHODS: A total of 150 obese patients (129 female, 21 male; mean age 44.41 ± 13.34 yr; mean BMI 35.76 ± 7.13 were included in the study. As a first screening step, we measured 24-h urinary free cortisol (UFC. An overnight 1-mg dexamethasone suppression test was also performed on all patients. Urinary free cortisol levels above 100 μg/24 h were considered to be abnormal. Suppression of serum cortisol 100 μg/24 h were recorded in 37 patients (24%. Cushing's syndrome was diagnosed in 14 of the 150 patients (9.33%. Etiologic reasons for Cushing's syndrome were pituitary microadenoma (9 patients, adrenocortical adenoma (3 patients, and adrenocortical carcinoma (1 patient. CONCLUSION: A significant proportion (9.33% of patients with simple obesity were found to have Cushing's syndrome. These findings argue that obese patients should be routinely screened for Cushing's syndrome.

  15. Skin manifestations of Cushing's syndrome.

    Science.gov (United States)

    Stratakis, Constantine A

    2016-09-01

    Among the most common diagnostic manifestations of Cushing's syndrome (CS) are those involving the skin; they include violaceous striae, facial acne, hirsutism, acanthosis nigricans (AN), fungal infections, hyperpigmentation (Hp) and easy bruisability. Fortunately, most resolve within a year or two after cure of CS, although light-colored striae can persist for years depending on the age of the patients. AN, Hp, and bruisability usually resolve within months after cure in almost all ages. Facial plethora (along with acne and other facial skin changes) is a typical sign of CS that is due to increased perfusion. It resolves immediately after curative therapy of CS. Typically, the severity of the manifestations does not correlate with the biochemical indices of the disease, pointing to age, gender, genetic and skin-type differences that determine the cutaneous manifestations of CS.

  16. Internal jugular vein adrenocorticotropic hormone estimation for diagnosis of adrenocorticotropic hormone-dependent Cushing's syndrome: Ultrasound-guided direct jugular vein sample collection.

    Science.gov (United States)

    Sahoo, Jaya Prakash; Seith, Ashu; Gupta, Nandita; Dwivedi, Sadanand; Ammini, Ariachery C

    2012-11-01

    To assess the utility of internal jugular vein (IJV) / peripheral adrenocorticotropic hormone (ACTH) gradient in determining the etiology of ACTH- dependent Cushing's syndrome. Patients with ACTH-dependent Cushing's syndrome, (except children less than 12 years), had IJV blood collection under ultrasound guidance using a linear 7 MHZ probe. Blood was collected with a 21 G needle at the level of mandible with the patient in supine position. Six ml of blood was collected sequentially from right and left internal jugular veins for ACTH and prolactin estimation. Peripheral blood for ACTH and prolactin was taken from a previously placed IV cannula in the antecubital vein. Thirty patients (20 F, 10 M, age 14 to 50 yrs) were enrolled for this study. Source of ACTH excess was pituitary in 22, ectopic ACTH in 4, and unknown in 4 cases. Using an IJV: Peripheral ACTH ratio of ≥ 1.6, 15 out of 22 Cushing's disease patients were correctly identified. However, 1 out of 4 ectopic Cushing also had IJV: Peripheral ratio ≥ 1.6. Overall, it had sensitivity of 68% with specificity of 75% while MRI pituitary and HDDST had sensitivity of 86% and 59%, respectively, with specificity of 100% each. IJV: Peripheral ACTH gradient was observed in 68% of patients with Cushing's disease. Simultaneous IJV and peripheral sample collection with CRH stimulation may improve sensitivity and specificity of this test.

  17. Laparoscopic bilateral transperitoneal adrenalectomy for Cushing syndrome: surgical challenges and lessons learnt.

    Science.gov (United States)

    Aggarwal, Sandeep; Yadav, Kunal; Sharma, Aditya P; Sethi, Vrishketan

    2013-06-01

    Laparoscopic adrenalectomy is well established for treatment of adrenal lesions. However, bilateral adrenalectomy for Cushing syndrome is a challenging and time-consuming operation. We report our experience of laparoscopic bilateral adrenalectomy for this disease in 19 patients. From September 2009 to August 2012, we have operated 19 patients with Cushing syndrome and performed bilateral laparoscopic adrenalectomy using the transperitoneal approach; synchronous in 15 patients and staged in 4 patients. In 15 patients, the surgery was carried out sequentially on both the sides in lateral position with intraoperative change in position. Complete adrenalectomy including periadrenal fat was carried out on both the sides. Nineteen patients were referred from Department of Endocrinology for bilateral adrenalectomy for adrenocorticotropin hormone (ACTH)-dependent and ACTH-independent Cushing syndrome. The indications for surgery were Cushing disease in 15 patients, occult/ectopic source of ACTH in 2 patients, and primary adrenal hyperplasia in 2 patients. Fifteen patients underwent bilateral adrenalectomy during the same operation. Four patients underwent staged procedures. All procedures were completed laparoscopically with no conversions. The mean operating time for simultaneous bilateral adrenalectomy was 210 minutes (range, 150 to 240 min). This included the repositioning and reprepping time. There were no major intraoperative complications. The average blood loss was 100 mL (range, 50 to 200 mL). None of the patients required blood transfusions in the postoperative period. The postoperative complications included minor port-site infection in 2 patients. One severely debilitated patient died on the 14th postoperative day because of hospital-acquired pneumonia. The remaining 18 patients have done well in terms of impact on the disease. Laparoscopic bilateral adrenalectomy for Cushing syndrome is feasible and safe. It confers all the advantages of minimally invasive

  18. Inhalational Steroids and Iatrogenic Cushing's Syndrome.

    Science.gov (United States)

    A V, Raveendran

    2014-01-01

    Bronchial asthma (BA) and Allergic rhinitis (AR) are common clinical problems encountered in day to day practice, where inhalational corticosteroids (ICS) or intranasal steroids (INS) are the mainstay of treatment. Iatrogenic Cushing syndrome (CS) is a well known complication of systemic steroid administration. ICS /INS were earlier thought to be safe, but now more and more number of case reports of Iatrogenic Cushing syndrome have been reported, especially in those who are taking cytochrome P450 (CYP 450) inhibitors. Comparing to the classical clinical features of spontaneous Cushing syndrome, iatrogenic Cushing syndrome is more commonly associated with osteoporosis, increase in intra-ocular pressure, benign intracranial hypertension, aseptic necrosis of femoral head and pancreatitis, where as hypertension, hirsuitisum and menstrual irregularities are less common. Endocrine work up shows low serum cortisol level with evidence of HPA (hypothalamo-pituitary-adrenal) axis suppression. In all patients with features of Cushing syndrome with evidence of adrenal suppression always suspect iatrogenic CS. Since concomitant administration of cytochrome P450 inhibitors in patients on ICS/INS can precipitate iatrogenic CS, avoidance of CYP450 inhibitors, its dose reduction or substitution of ICS are the available options. Along with those, measures to prevent the precipitation of adrenal crisis has to be taken. An update on ICS-/INS- associated iatrogenic CS and its management is presented here.

  19. Physiological basis for the etiology, diagnosis, and treatment of adrenal disorders: Cushing's syndrome, adrenal insufficiency, and congenital adrenal hyperplasia.

    Science.gov (United States)

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

    2014-04-01

    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 latter of which is usually due to autonomous adrenal overproduction. The former can be due to a pituitary corticotroph tumor (usually benign) or ectopic ACTH production from tumors outside the pituitary; both of these tumor types overexpress the proopiomelanocortin gene. The converse of Cushing's syndrome is the lack of normal cortisol secretion and is usually due to adrenal destruction (primary adrenal insufficiency) or hypopituitarism (secondary adrenal insufficiency). Secondary adrenal insufficiency can also result from a rapid discontinuation of long-term, pharmacological glucocorticoid therapy because of HPA axis suppression and adrenal atrophy. Finally, mutations in the steroidogenic enzymes of the adrenal cortex can lead to congenital adrenal hyperplasia and an increase in precursor steroids, particularly androgens. When present in utero, this can lead to masculinization of a female fetus. An understanding of the dynamics of the HPA axis is necessary to master the diagnosis and differential diagnosis of pituitary-adrenal diseases. Furthermore, understanding the pathophysiology of the HPA axis gives great insight into its normal control. © 2014 American Physiological Society.

  20. Steven Johnson syndrome in a patient with Cushing's disease.

    Science.gov (United States)

    Mustafa, N; Periyasamy, P; Kamaruddin, N

    2009-09-01

    Cushing's syndrome is a pathological condition associated with excessive cortisol production, the commonest etiology being Cushing's disease. Corticosteroids in high doses have been used in the management of Steven Johnson Syndrome (SJS) with favourable outcome. We describe a patient with Cushing's disease who developed SJS, one week after taking sperulina a product from sea-weed while waiting for transphenoidal surgery.

  1. [Prevalence of arterial hypertension in Cushing's syndrome].

    Science.gov (United States)

    Rodrigues, D; Barros, L; Ruas, L; Gomes, L; Geraldes, E; Ruas, M C

    1997-11-01

    To determine the prevalence of hypertension (HT) in patients with Cushing's syndrome. We studied 23 patients with Cushing's syndrome, 17 women (mean +/- SD age = 42.8 +/- 15.6 years) and 6 men (mean +/- SD age = 34.8 +/- 10.2 years). The etiologies were: 16-Cushing's disease, 3-suprarenal adenoma, 2-suprarenal carcinoma and 2-iatrogenic. Blood pressure (BP) was measured at least three times and we consider hypertension when systolic BP > or = 140 mmHg and/or diastolic BP > or = 90 mmHg. The prevalence of HT, at diagnosis, was 73.9% (64.7% in women and 100% in men) and the mean BP was 163 +/- 25/100 +/- 16 mmHg (158 +/- 26/97 +/- 15 mmHg in women and 182 +/- 5/114 +/- 10 mmHg in men). From the 16 patients with Cushing's disease, 13 (81%) had HT; all the patients (n = 3) with suprarenal adenoma had HT; none with suprarenal carcinoma (n = 2) had HT and only 1 of the 2 patients with iatrogenic Cushing's syndrome had HT. After treatment, 14 patients entered remission, 6 persisted with active disease and 1 was missed during the follow-up. In the group of patients that entered remission, the prevalence of HT at diagnosis was 78.5% (n = 11), 57.1% (n = 8) being after treatment. In this study, we found a high prevalence of HT at diagnosis (73.9%). After treatment and in the patients that entered remission, the prevalence of HT remained high (78.5% vs 57.1%). The high prevalence of HT in Cushing's syndrome, suggests its importance as a morbidity factor and also as an important diagnostic indicator, when present.

  2. A rare cause of Cushing's syndrome

    DEFF Research Database (Denmark)

    Folkestad, Lars; Andersen, Marianne Skovsager; Nielsen, Anne Lerberg

    2014-01-01

    Excess glucocorticoid levels cause Cushing's syndrome (CS) and may be due to pituitary, adrenal or ectopic tumours. Adrenocorticotropic hormone (ACTH) levels are useful in identifying adrenal tumours. In rare cases, ACTH-producing phaeochromocytomas are the cause of CS. We present two cases of ACTH...

  3. Exophthalmos: A Forgotten Clinical Sign of Cushing's Syndrome

    Directory of Open Access Journals (Sweden)

    Aldo Schenone Giugni

    2013-01-01

    Full Text Available Exophthalmos is typically associated with Graves' ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing's syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia.

  4. Clinicopathological correlates of adrenal Cushing's syndrome.

    Science.gov (United States)

    Duan, Kai; Gomez Hernandez, Karen; Mete, Ozgur

    2015-03-01

    Endogenous Cushing's syndrome is a rare endocrine disorder that incurs significant cardiovascular morbidity and mortality, due to glucocorticoid excess. It comprises adrenal (20%) and non-adrenal (80%) aetiologies. While the majority of cases are attributed to pituitary or ectopic corticotropin (ACTH) overproduction, primary cortisol-producing adrenal cortical lesions are increasingly recognised in the pathophysiology of Cushing's syndrome. Our understanding of this disease has progressed substantially over the past decade. Recently, important mechanisms underlying the pathogenesis of adrenal hypercortisolism have been elucidated with the discovery of mutations in cyclic AMP signalling (PRKACA, PRKAR1A, GNAS, PDE11A, PDE8B), armadillo repeat containing 5 gene (ARMC5) a putative tumour suppressor gene, aberrant G-protein-coupled receptors, and intra-adrenal secretion of ACTH. Accurate subtyping of Cushing's syndrome is crucial for treatment decision-making and requires a complete integration of clinical, biochemical, imaging and pathology findings. Pathological correlates in the adrenal glands include hyperplasia, adenoma and carcinoma. While the most common presentation is diffuse adrenocortical hyperplasia secondary to excess ACTH production, this entity is usually treated with pituitary or ectopic tumour resection. Therefore, when confronted with adrenalectomy specimens in the setting of Cushing's syndrome, surgical pathologists are most commonly exposed to adrenocortical adenomas, carcinomas and primary macronodular or micronodular hyperplasia. This review provides an update on the rapidly evolving knowledge of adrenal Cushing's syndrome and discusses the clinicopathological correlations of this important disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  5. [Cushing's syndrome: clinical study of fifteen cases].

    Science.gov (United States)

    Calvo Romero, J M; Morales Pérez, F; Alvarez Barreiro, J A; Diaz Pérez de Madrid, J

    1998-05-01

    To study the epidemiological and clinical features and diagnostic tests of Cushing's syndrome (CS) of non-iatrogenic etiology, because of there are few similar studies in the last ten years. Fifteen cases of CS were diagnosed from 1992 to 1997 at our hospital. We describe the epidemiological, clinical, biochemical, radiologic, therapeutic and evolutive characteristics. Both diabetes mellitus and hypertension were observed in 40% of patients. The frequency of etiologies was: Cushing's disease, 66.6%; ectopic ACTH syndrome, 13.3%; adrenal adenoma, 6.6%; adrenal carcinoma, 6.6%; and undiagnosed, 6.6%. The 24-hour urine free cortisol (UFC) and the overnight 1 mg oral dexamethasone suppression test yielded 93.3 and 100% diagnostic sensitivity for CS, respectively. The overnight 8 mg oral dexamethasone suppression test, the metyrapone test and the 7 mg intravenous dexmethasone test had 75, 50 and 60% diagnostic sensitivity for Cushing's disease, respectively. Ketoconazole treatment had success in to normalize the 24-hour UFC in all patients, except for the case of adrenal carcinoma. The Cushing's disease was the most common form of CS. The 24-hour UFC and overnight 1 mg oral dexamethasone suppression test were good screening studies. Ketoconazole was successful in normalizing the adrenal cortex function.

  6. Cushing's syndrome: hidden risk in usage of topical corticosteroids.

    Science.gov (United States)

    Buluş, Ayşe Derya; Andıran, Nesibe; Koçak, Mesut

    2014-09-01

    Iatrogenic Cushing's syndrome in children may occur as a result of the application of exogenous steroids. Prolonged use of powerful corticosteroids suppresses adrenal functions and iatrogenic Cushing's syndrome may develop particularly in infants who are given topical corticosteroids. We report here a case on three infants having Cushing's syndrome with similar clinical presentations due to overuse of topical steroids for diaper dermatitis. The importance of exercising caution during the use of topical steroids is underlined in this study.

  7. Exogenous Cushing's syndrome and glucocorticoid withdrawal.

    Science.gov (United States)

    Hopkins, Rachel L; Leinung, Matthew C

    2005-06-01

    Glucocorticoid therapy in various forms is extremely common for a wide range of inflammatory, autoimmune, and neoplastic disorders. It is therefore important for the physician to be aware of the possibility of both iatrogenic and factitious Cushing's syndrome. Although most common with oral therapy, it is also important to be alert to the fact that all forms of glucocorticoid delivery have the potential to cause Cushing's syndrome. Withdrawal from chronic glucocorticoid therapy presents significant challenges. These include the possibility of adrenal insufficiency after discontinuation of steroid therapy, recurrence of underlying disease as the glucocorticoid is being withdrawn, and the possibility of steroid withdrawal symptoms. Nonetheless, with patience and persistence, a reasonable approach to withdrawal of glucocorticoid therapy can be achieved.

  8. Two Cases of. Cushing's Syndrome tumour and bilateral hyperplasia

    African Journals Online (AJOL)

    Two patients, one with Cushing's syndrome and one with Cushing's disease, are presented. In the first case the syndrome was caused by a tumour of the right suprarenal gland which was treated by unilateral adrenalectomy, and the second case was diagnosed as hyperplasia of the left suprarenal gland, eventually leading ...

  9. Severe Hypertension Secondary to Renal Artery Stenosis and Cushing's Syndrome

    International Nuclear Information System (INIS)

    Al-Zahrani, Ali S.; Al-Hajjaj, Alya; Al-Watban, Jehad; Kanaan, Imaduddin

    2005-01-01

    We present an unusual patient who simultaneously had severe renal artery stenosis RAS and Cushings syndrome. The case highlights the difficulty of reaching a specific diagnosis of Cushings syndrome and the possible interaction between Cushings syndrome and some other concurrent illnesses that this patient had. A 37-year old man presented with severe hypertension HTN and uncontrolled diabetes mellitus DM without clear physical signs of Cushings syndrome. He was found to have severe osteoporosis, proximal myopathy, several cutaneous warts, tinea versicolor, and chronic viral hepatitis. Captopril-stimulated renal scan and renal artery angiogram revealed severe RAS. Partial balloon dilatation of RAS led to improvement in HTN. Unexpectedly, urine free cortisol 24 hour was found extremely high. Serum adrenocorticotropic hormone ACTH was also elevated and high dose dexamethasone suppression tests were inconclusive. Several imaging studies failed to localize the source of ACTH. Despite normal MRI of the pituitary gland, bilateral inferior petrosal sinus sampling IPSS localized the source of ACTH secretion to the right side of the pituitary gland and right anterior hemihypophysectomy resulted in cure of Cushings disease, HTN, DM, and tinea versicolor with significant improvement in cutaneous warts, osteoporosis, and chronic hepatitis. In conclusion, RAS and Cushings syndrome may occur together. Significant hypercortisolemia can occur without clear signs of Cushings syndrome. Controlling hypercortisolemia is of paramount importance when treating chronic infections in patients with Cushing's syndrome. (author)

  10. Prognosis of patients treated for Cushing syndrome.

    Science.gov (United States)

    Aulinas, Anna; Valassi, Elena; Webb, Susan M

    2014-01-01

    Cushing syndrome (CS), due to an ACTH-secreting pituitary adenoma, adrenal tumors, or ectopic ACTH secretion, causes hypercortisolism. CS is associated with major morbidity, especially metabolic and cardiovascular complications, osteoporosis, psychiatric changes, and cognitive impairment. Despite biochemical "cure" of hypercortisolism and clinical improvement after effective treatment, these complications are only partially reversible. Exacerbation of prior autoimmune diseases is also seen. All of these lead to quality of life impairment and increased mortality. This review addresses the main comorbidities and long-term consequences of CS despite clinical and biochemical "cure". Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.

  11. Cushing's syndrome in children and adolescents

    DEFF Research Database (Denmark)

    Holst, Johanne Marie; Horváth-Puhó, Erzsébet; Jensen, Rikke Beck

    2017-01-01

    OBJECTIVE: Cushing's syndrome (CS) affects all age groups, but epidemiologic data in young patients are very limited. We therefore examined the incidence, prevalence and hospital morbidity of CS in children and adolescents. DESIGN: In a nationwide cohort study, we included all Danish citizens aged...... 0-20 years from 1977 to 2012. Data were obtained from the Danish National Patient Registry using the International Classification of Diseases (ICD) codes and the Danish Civil Registration System. The diagnosis and treatment were validated by means of individual patient charts. Incidence rate of CS...

  12. Paraneoplastic cushing syndrome due to wilm's tumor

    International Nuclear Information System (INIS)

    Faizan, M.; Anwar, S.; Hameed, A.; Manzoor, J.; Saleem, M.; Mehmood, Q.; Ali, A. S.

    2017-01-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, alongwith histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed. (author)

  13. Iatrogenic Cushing's syndrome caused by intranasal steroid use.

    Science.gov (United States)

    Dursun, Fatma; Kirmizibekmez, Heves

    2017-01-01

    Cushing's syndrome (CS) is common after oral steroid use and has also been reported following topical or inhaled use, but it is extremely uncommon after intranasal administration. This is the case of a 6-year-old child who developed Cushing's syndrome after intranasal application of dexamethasone sodium phosphate for a period of 6 months. Pediatricians and other clinical practitioners should be aware that high-dose and long-term nasal steroid administration may cause iatrogenic Cushing's syndrome characterized by complications of glucocorticoid excess as well as serious and even life-threatening complications of adrenal insufficiency.

  14. [Comparison of the severity index in Cushing's syndrome between patients with endogenous and iatrogenic disease].

    Science.gov (United States)

    Pérez, Edgar Gerardo Durán; Hernández, Everardo Alvarez; Zuñiga, Aurora Elizabeth Serralde; Nicolás, Leslye Asela Lujano; Loza, Oscar Moreno; Palomo, Antonio Segovia; Pedraza, Valentín Sánchez; Montaño, Sara Arellano

    2010-11-01

    Cushing's syndrome (CS) is a rare condition that is most often secondary to glucocorticoid (GC) intake. Definitive data on the distinction between endogenous CS (ECS) and yatrogenic (YCS) have not been published. To compare the severity index in Cushing's syndrome (SICS) score in the two etiologies, endogenous and yatrogenic, and identify which clinical parameters of the SICS are the most frequent and representative of CS. We performed an observational, descriptive and cross-sectional study. The ECS group included men and women with a diagnosis of endogenous hypercortisolism, with measurement of adrenocorticotropin hormone (ACTH) and absence of causes of pseudo-Cushing's syndrome. The YCS group included patients with a history of use of high-dose systemic GC, cushingoid facies, absence of causes of pseudo-Cushing's syndrome and no diagnosis of hypertension or diabetes mellitus before the start of GC intake. We investigated the diagnosis of the disease entity causing ECS. In patients with ICS, the characteristics of the GC employed and how these drugs were used were recorded. We calculated daily and cumulative doses in milligrams of prednisone. In all patients, the SICS was applied and the clinical characteristics of the two groups were compared. We included 20 patients, 10 with YCS and 10 with ECS. There were eight women in the ECS group (80%) and five in the YCS group (50%). The diagnoses in patients with ECS were ACTH-dependent CS in nine and ACTH-independent CS in one. The mean daily dose of prednisone equivalent used by patients with YCS was 13.5mg, and the mean total cumulative dose for all patients with ICS was 9962.5mg. The mean total SICS score for the ECS group was 8.6 and was 3.8 points for the YCS group (p <0.005, 95% CI 3.36-6.23). All patients with ECS had a mean score of 6 or higher whereas none of the patients with ICS scored more than 6 points. Fat distribution, hypertension, diabetes mellitus and gender-related changes were more representative of

  15. Cushing's syndrome with an apparently normal CT scan

    International Nuclear Information System (INIS)

    Vlahos, L.; Strigaris, K.; Aliferopoulos, D.; Pontifex, G.

    1981-01-01

    A case of Cushing's syndrome is described in which the CT scan was considered as normal. The diagnosis was established with selective venography and blood sampling and was verified surgically. (orig.)

  16. Iatrogenic Cushing's syndrome in children following nasal steroid.

    Science.gov (United States)

    Oluwayemi, Isaac Oludare; Oduwole, Abiola Olufunmilayo; Oyenusi, Elizabeth; Onyiriuka, Alphonsus Ndidi; Abdullahi, Muhammad; Fakeye-Udeogu, Olubunmi Benedicta; Achonwa, Chidozie Jude; Kouyate, Moustapha

    2014-01-01

    Cushing syndrome is a hormonal disorder caused by prolonged exposure of body tissue to cortisol. We report two cases of iatrogenic Cushing's syndrome in two Nigerian children following intranasal administration of aristobed-N (Betamethasone + Neomycin) given at a private hospital where the children presented with feature of adenoidal hypertrophy. Two months into treatment children were noticed to have developed clinical and laboratory features of iatrogenic Cushing's syndrome with critical adrenal suppression. Serum cortisol (at presentation): 1(st) patient: 12nmol/L (reference range 240-618), 2(nd) 1.69nmol/L. Serum cortisol (3 months after weaning off steroid): 343.27 nmol/L (within normal range for the first patient; second patient newly presented and has just begun steroid weaning off process. The serum cortisol level one month into weaninig off process was 128 nmol/L). Unsupervised topical steroid administration in children can cause adrenal suppression with clinical features of Cushing's syndrome.

  17. Case Of Iatrogenic Cushing's Syndrome By Topical Triamcinolone.

    Science.gov (United States)

    Zil-E-Ali, Ahsan; Janjua, Omer Hanif; Latif, Aiza; Aadil, Muhammad

    2018-01-01

    Cushing's syndrome is a collection of signs and symptoms due to hypercortisolism. Prolong use of topical steroid may cause this syndrome and suppression of hypothalamic and pituitary function, however such events are more common with oral and parenteral route. There are very few cases of Cushing's syndrome with a topical application amongst which triamcinolone is the rarest drug. We report a case of 11-year-old boy is presented who developed Cushing's disease by topical application. The child had body rashes for which the caregiver consulted a local quack, a topical cream of triamcinolone was prescribed. After application for three months, the patient became obese and developed a moon-like face. A thorough biochemical workup and diagnostic test for Cushing's disease was done to confirm. The following case report a dramatic example of development of the syndrome from chronic topical application of the least potent corticosteroid.

  18. Cushing's syndrome in pregnancy and neonatal hypertrophic obstructive cardiomyopathy.

    Science.gov (United States)

    Fayol, L; Masson, P; Millet, V; Simeoni, U

    2004-10-01

    Cushing's syndrome is rare in pregnancy but can cause spontaneous abortion, stillbirth or premature birth. We report a case of transient hypertrophic obstructive cardiomyopathy in a newborn whose mother had hypercortisolism due to a primary adrenal lesion. There was no family history of hypertrophic obstructive cardiomyopathy. Follow-up revealed complete resolution of the cardiac abnormalities in the infant. Cushing's syndrome in the mother resolved after delivery. Although maternal hypercortisolism seldom results in symptomatic hypercortisolism in the newborn, hypertrophic obstructive cardiomyopathy can occur.

  19. Factors predicting the duration of adrenal insufficiency in patients successfully treated for Cushing disease and nonmalignant primary adrenal Cushing syndrome.

    Science.gov (United States)

    Prete, Alessandro; Paragliola, Rosa Maria; Bottiglieri, Filomena; Rota, Carlo Antonio; Pontecorvi, Alfredo; Salvatori, Roberto; Corsello, Salvatore Maria

    2017-03-01

    Successful treatment of Cushing syndrome causes transient or permanent adrenal insufficiency deriving from endogenous hypercortisolism-induced hypothalamus-pituitary-adrenal-axis suppression. We analyzed pre-treatment factors potentially affecting the duration of adrenal insufficiency. We conducted a retrospective analysis on patients successfully treated for Cushing disease (15 patients) who underwent transsphenoidal surgery, and nonmalignant primary adrenal Cushing syndrome (31 patients) who underwent unilateral adrenalectomy, divided into patients with overt primary adrenal Cushing syndrome (14 patients) and subclinical primary adrenal Cushing syndrome (17 patients). Epidemiological data, medical history, and hormonal parameters depending on the etiology of hypercortisolism were collected and compared to the duration of adrenal insufficiency. The median duration of follow-up after surgery for Cushing disease and primary adrenal Cushing syndrome was 70 and 48 months, respectively. In the Cushing disease group, the median duration of adrenal insufficiency after transsphenoidal surgery was 15 months: younger age at diagnosis and longer duration of signs and symptoms of hypercortisolism before diagnosis and surgery were associated with longer duration of adrenal insufficiency. The median duration of adrenal insufficiency was 6 months for subclinical primary adrenal Cushing syndrome and 18.5 months for overt primary adrenal Cushing syndrome. The biochemical severity of hypercortisolism, the grade of hypothalamus-pituitary-adrenal-axis suppression, and treatment with ketoconazole before surgery accounted for longer duration of adrenal insufficiency. In patients with Cushing disease, younger age and delayed diagnosis and treatment predict longer need for glucocorticoid replacement therapy after successful transsphenoidal surgery. In patients with primary adrenal Cushing syndrome, the severity of hypercortisolism plays a primary role in influencing the duration of

  20. An update on Cushing syndrome in pediatrics.

    Science.gov (United States)

    Stratakis, Constantine A

    2018-04-09

    Cushing syndrome (CS) in childhood results mostly from the exogenous administration of glucocorticoids; endogenous CS is a rare disease. The latter is the main reason pediatric patients with CS escape diagnosis for too long. Other barriers to optimal care of a pediatric patient with CS include improper following of the proper sequence of testing for diagnosing CS, which stems from lack of understanding of pathophysiology of the hypothalamic-pituitary-adrenal axis; lack of access to proper (i.e., experienced, state-of-the-art) surgical treatment; and unavailability of well-tolerated and effective medications to control hypercortisolemia. This report reviews the state-of-the-art in diagnosing CS and provides an update on the most recent discoveries in its genetics and treatment. Copyright © 2018. Published by Elsevier Masson SAS.

  1. Pitfalls in the diagnosis of Cushing's syndrome.

    Science.gov (United States)

    Vilar, Lucio; Freitas, Maria da Conceição; Faria, Manuel; Montenegro, Renan; Casulari, Luiz Augusto; Naves, Luciana; Bruno, Oscar D

    2007-11-01

    Among endocrine disorders, Cushing's syndrome (CS) is certainly one of the most challenging to endocrinologists due to the difficulties that often appear during investigation. The diagnosis of CS involves two steps: confirmation of hypercortisolism and determination of its etiology. Biochemical confirmation of the hypercortisolaemic state must be established before any attempt at differential diagnosis. Failure to do so will result in misdiagnosis, inappropriate treatment, and poor management. It should also be kept in mind that hypercortisolism may occur in some patients with depression, alcoholism, anorexia nervosa, generalized resistance to glucocorticoids, and in late pregnancy. Moreover, exogenous or iatrogenic hypercortisolism should always be excluded. The three most useful tests to confirm hypercortisolism are the measurement of 24-h urinary free cortisol levels, low-dose dexamethasone-suppression tests, and determination of midnight serum cortisol or late-night salivary cortisol. However, none of these tests is perfect, each one has different sensitivities and specificities, and several are usually needed to provide a better diagnostic accuracy. The greatest challenge in the investigation of CS involves the differentiation between Cushing's disease and ectopic ACTH syndrome. This task requires the measurement of plasma ACTH levels, non-invasive dynamic tests (high-dose dexamethasone suppression test and stimulation tests with CRH or desmopressin), and imaging studies. None of these tests had 100% specificity and their use in combination is usually necessary. Bilateral inferior petrosal sinus sampling is mainly indicated when non-invasive tests do not allow a diagnostic definition. In the present paper, the most important pitfalls in the investigation of CS are reviewed.

  2. Survey of 26 suprarenal scintigraphs on Cushing's syndrome patients

    International Nuclear Information System (INIS)

    Venot, A.; Luton, J.P.; Roucayrol, J.C.; Bricaire, H.

    On the basis of the results of 26 Cushing's syndrome examinations some new scintigraphic aspects are described, suprarenal scintigraphy is compared with retroperitoneal insufflation, its value in the observation of a medical treatment of Cushing's disease is demonstrated and finally some preliminary results are given on the application in the same pathological context, of 75 Se-6-selenocholesterol which was compared with 131 I-19-iodocholesterol [fr

  3. Hair cortisol in the evaluation of Cushing syndrome.

    Science.gov (United States)

    Hodes, Aaron; Lodish, Maya B; Tirosh, Amit; Meyer, Jerrold; Belyavskaya, Elena; Lyssikatos, Charalampos; Rosenberg, Kendra; Demidowich, Andrew; Swan, Jeremy; Jonas, Nichole; Stratakis, Constantine A; Zilbermint, Mihail

    2017-04-01

    Hair cortisol evaluation has been used to help detect patients with suspected Cushing syndrome. Our goal was to correlate segmental hair cortisol with biochemical testing in patients with Cushing syndrome and controls. This study was a prospective analysis of hair cortisol in confirmed Cushing syndrome cases over 16 months. Thirty-six subjects (26.5 ± 18.9 years, 75% female, and 75% Caucasian) were analyzed by diurnal serum cortisol, 24 h urinary free cortisol corrected for body surface area (UFC/BSA), and 24 h urinary 17-hydroxysteroids corrected for creatinine (17OHS/Cr). Thirty patients were diagnosed with Cushing syndrome, and six were defined as controls. 3-cm hair samples nearest to the scalp, cut into 1-cm segments (proximal, medial, and distal), were analyzed for cortisol by enzyme immunoassay and measured as pmol cortisol/g dry hair. Hair cortisol levels were compared with laboratory testing done within previous 2 months of the evaluation. Proximal hair cortisol was higher in Cushing syndrome patients (266.6 ± 738.4 pmol/g) than control patients (38.9 ± 25.3 pmol/g) (p = 0.003). Proximal hair cortisol was highest of all segments in 25/36 (69%) patients. Among all subjects, proximal hair cortisol was strongly correlated with UFC/BSA (r = 0.5, p = 0.005), midnight serum cortisol (r = 0.4, p = 0.03), and 17OHS/Cr, which trended towards significance (r = 0.3, p = 0.06). Among the three examined hair segments, proximal hair contained the highest cortisol levels and correlated the most with the initial biochemical tests for Cushing syndrome in our study. Further studies are needed to validate proximal hair cortisol in the diagnostic workup for Cushing syndrome.

  4. Iatrogenic Cushing's syndrome and steroid hepatopathy in a cat.

    Science.gov (United States)

    Schaer, M; Ginn, P E

    1999-01-01

    The distinguishing clinical features of Cushing's syndrome in the cat include very friable skin, a high incidence of diabetes mellitus, and the general absence of steroid hepatopathy. This case report describes a nine-year-old, spayed female domestic shorthair with triamcinolone-induced Cushing's syndrome. Unique to this cat were markedly elevated liver enzymes which prompted an expanded clinical evaluation. An ultrasonographic-guided liver biopsy demonstrated diffuse hepatocellular vacuolation that stained periodic acid-Schiff (PAS) positive and was removed subsequently with diastase application, indicating glycogen accumulation. These findings are compatible with the rarely seen syndrome of steroid hepatopathy in the cat.

  5. Iatrogenic Cushing's Syndrome After Topical Steroid Therapy for Psoriasis.

    Science.gov (United States)

    Sahıp, Birsen; Celık, Mehmet; Ayturk, Semra; Kucukarda, Ahmet; Mert, Onur; Dıncer, Nejla; Guldıken, Sıbel; Tugrul, Armagan

    2016-01-01

    Glucocorticoids are used for the treatment of many diseases, such as inflammatory, allergic, autoimmune, and neoplastic diseases. They can be used in the form of topical, oral, inhalable, rectal, and intra-articular agents. Many topical steroid-related iatrogenic Cushing's syndrome cases affecting especially children have been reported in the literature. Topical steroid-related Cushing's syndrome is rarely seen in adults. In this report, we present the case of a 32-year-old male patient with iatrogenic Cushing's syndrome related to long-term clobetasol propionate treatment for psoriasis. In the context of such treatment, the glucocorticoid withdrawal problem has to be overcome. At present there is no consensus on steroid withdrawal. Patients on long-term glucocorticoid treatment must be evaluated for potential adverse effects and withdrawal symptoms by their physician and their endocrinologist.

  6. Is There a Cure for Cushing's Syndrome?

    Science.gov (United States)

    ... also is possible that a person cured of Cushing disease might not recover their previous mental strength, including ... impairments in patients with long-term cure of Cushing’s disease. Journal of Clinical Endocrinology and Metabolism, 95 (6), ...

  7. 131I-19-iodocholesterol adrenal scanning in Cushing's syndrome

    International Nuclear Information System (INIS)

    Charbonnel, B.; Chatal, J.F.; Chupin, M.; Guillon, J.

    1976-01-01

    7 patients were investigated: 2 bilateral hyperplasia due to pituitary ACTH excess showed bilateral adrenal activity. 1 post-surgical remnant with recurrent Cushing's syndrome was detected. 1 adenoma showed unilateral intense activity and absent activity in the controlateral gland, even after ACTH treatment. 2 carcinomas were weakly imaged. In 1 case, hepatic metastasis showed 131 I-19-iodocholesterol uptake. Adrenal imaging with 131 I-19-iodocholesterol is not a good procedure for assessing hormonal function. It is a valuable and safe tool in the localization and diagnosis of adrenal lesions causing Cushing's syndrome, perhaps better than radiologic procedures. Radiation dosimetry is acceptable [fr

  8. Endoscopic bilateral adrenalectomy in patients with ectopic Cushing's syndrome

    NARCIS (Netherlands)

    W.J. Alberda (Wijnand); C.H.J. van Eijck (Casper); R.A. Feelders (Richard); G. Kazemier (Geert); W.W. de Herder (Wouter); J.W.A. Burger (Jacobus)

    2012-01-01

    textabstractBackground: Bilateral adrenalectomy (BLA) is a treatment option to alleviate symptoms in patients with ectopic Cushing's syndrome (ECS) for whom surgical treatment of the responsible nonpituitary tumor is not possible. ECS patients have an increased risk for complications, because of

  9. Gamma Knife Radiosurgery for Cushing's Disease and Nelson's Syndrome

    Czech Academy of Sciences Publication Activity Database

    Marek, J.; Ježková, J.; Hána, V.; Kršek, M.; Liščák, R.; Vladyka, V.; Pecen, Ladislav

    2015-01-01

    Roč. 18, č. 3 (2015), s. 376-384 ISSN 1386-341X Institutional support: RVO:67985807 Keywords : Cushing's disease * Nelson's syndrome * Gamma knife surgery * Pituitary adenoma Subject RIV: FB - Endocrinology, Diabetology, Metabolism, Nutrition Impact factor: 3.407, year: 2015

  10. Malignant bilateral exophthalmos and secondary glaucoma in iatrogenic Cushing's syndrome.

    Science.gov (United States)

    Boschi, A; Detry, M; Duprez, T; Rolland, F; Plas, B; De Plaen, J; Eloy, P

    1997-04-01

    The authors describe a case of iatrogenic Cushing's syndrome in which an emergency orbital decompression was performed. This procedure was necessary because major ocular hypertension and severe bilateral exophthalmos had caused a decrease in visual function and recurrent painful episodes of eyeball luxation.

  11. Cushing's syndrome: from physiological principles to diagnosis and clinical care

    Science.gov (United States)

    Raff, Hershel; Carroll, Ty

    2015-01-01

    The physiological control of cortisol synthesis in the adrenal cortex involves stimulation of adrenocorticotrophic hormone (ACTH) by hypothalamic corticotrophin-releasing hormone (CRH) and then stimulation of the adrenal by ACTH. The control loop of the hypothalamic–pituitary–adrenal (HPA) axis is closed by negative feedback of cortisol on the hypothalamus and pituitary. Understanding this system is required to master the diagnosis, differential diagnosis and treatment of endogenous hypercortisolism – Cushing's syndrome. Endogenous Cushing's syndrome is caused either by excess ACTH secretion or by autonomous cortisol release from the adrenal cortex. Diagnosis of cortisol excess exploits three physiological principles: failure to achieve the normal nadir in the cortisol diurnal rhythm, loss of sensitivity of ACTH-secreting tumours to cortisol negative feedback, and increased excretion of free cortisol in the urine. Differentiating a pituitary source of excess ACTH (Cushing's disease) from an ectopic source is accomplished by imaging the pituitary and sampling for ACTH in the venous drainage of the pituitary. With surgical removal of ACTH or cortisol-secreting tumours, secondary adrenal insufficiency ensues because of the prior suppression of the HPA axis by glucocorticoid negative feedback. Medical therapy is targeted to the anatomical location of the dysregulated component of the HPA axis. Future research will focus on new diagnostics and treatments of Cushing's syndrome. These are elegant examples of translational research: understanding basic physiology informs the development of new approaches to diagnosis and treatment. Appreciating pathophysiology generates new areas for inquiry of basic physiological and biochemical mechanisms. PMID:25480800

  12. Neonatal Cushing Syndrome: A Rare but Potentially Devastating Disease.

    Science.gov (United States)

    Tatsi, Christina; Stratakis, Constantine A

    2018-03-01

    Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome. More rarely, neonatal CS presents as part of other syndromes such as McCune-Albright syndrome or Beckwith-Wiedemann syndrome. Management usually includes resection of the primary tumor with or without additional medical treatment, but manifestations may persist after resolution of hypercortisolemia. Published by Elsevier Inc.

  13. A black adrenocortical adenoma causing Cushing's syndrome not imaged by radiocholesterol scintigraphy

    International Nuclear Information System (INIS)

    Reschini, E.; Baldini, M.; Cantalamessa, L.

    1990-01-01

    In a 33-year-old female patient with left adrenal tumour and Cushing's syndrome, adrenocortical scintigraphy with radiocholesterol did not image the tumour nor the suppressed contralateral gland. Histology showed a black adrenocortical adenoma composed only of compact cells; there was no evidence of malignancy. This demonstrates that non-visualization of the adrenal glands in a patient with Cushing's syndrome is not invariably due to adrenal carcinoma. The literature on black adrenal adenomas causing Cushing's syndrome is reviewed. (orig.)

  14. Cushing's syndrome and chronic venous ulceration--a clinical challenge.

    Science.gov (United States)

    Biswas, Moushmi; Gibby, Owain; Ivanova-Stoilova, Tzvetanka; Harding, Keith

    2011-02-01

    Cushing's syndrome is a condition caused by high levels of glucocorticoids, or most commonly as a result of prolonged exposure to exogenous steroids. Clinical features include diabetes, hypertension, obesity, skin atrophy, immune suppression and delayed wound healing. We report a patient with iatrogenic Cushing's syndrome, in whom long-term topical steroid therapy was used to treat varicose eczema, which contributed to the development of type 2 diabetes, morbid obesity, sleep apnoea and chronic wound sepsis. In this case, repeated hospital admissions and systemic antibiotics were associated with considerable comorbidity. Aggressive local treatment, consisting of potassium permanganate soaks and irrigating gels, was highly effective in reducing the amount of exudate, pain and preventing from further deterioration of the patient's legs. © 2010 The Authors. © 2010 Blackwell Publishing Ltd and Medicalhelplines.com Inc.

  15. Does the corticoadrenal adenoma with ''pre-Cushing's syndrome'' exist

    International Nuclear Information System (INIS)

    Charbonnel, B.; Chatal, J.F.; Ozanne, P.

    1981-01-01

    An adrenal tumor was discovered fortuitously in a patient with no clinical features of Cushing's syndrome. On adrenal imaging, there was good uptake in the nodule but no visualization of the contralateral adrenal. The latter was seen, however, in a second scan performed under ACTH treatment. In the hormone assessment, basal cortisol and 17-hydroxycorticoids were normal and cortisol diurnal variation was near normal, but a dexamethasone suppression test and ACTH responses to metyrapone and insulin hypoglycemia were abnormal. Eight months after excision of a spongiocytic-type adenoma, the remaining adrenal was visible on scintigram and the hormonal tests were normal. This pattern suggests that the clinical Cushing's syndrome was enough to partially suppress ACTH and, consequently, visualization of the contralateral gland

  16. Medical treatment in Cushing's syndrome: dopamine agonists and cabergoline.

    Science.gov (United States)

    Petrossians, Patrick; Thonnard, Anne-Sophie; Beckers, Albert

    2010-01-01

    Dopamine (DA) is a catecholamine with a wide range of functions and whose five subtype receptors are found in different organs where they exert a mainly inhibitory action. Since this action may also appear in a number of secretory tumors in various locations, DA agonists have elicited some interest as a medical treatment for hypercorticism. Non-iatrogenic Cushing's syndromes are due in 70% of the cases to a pituitary adrenocorticotropic hormone (ACTH)-producing adenoma, and, less frequently, to an adrenal adenoma or an ectopic ACTH secretion by a neuroendocrine tumor. First-line treatment in Cushing's syndrome consists of the surgical removal of the secreting tumor. However, surgery may not achieve a complete cure in a number of cases, hence emphasizing the potential benefit of a medical complementary treatment, which could also benefit patients as an alternative approach, either when waiting for, or when the patient is not eligible for surgery. Studies of corticotropic adenomas have shown that 80% of these tumors express D2 receptors. Clinical trials of DA agonists in Cushing's disease have shown an inhibitory effect of these drugs with an inhibition of ACTH secretion and/or a decrease of tumor size. There are only a few cases of documented use of DA agonists in ectopic ACTH secretion, but when the tumor expresses DA receptors, DA agonists may represent a useful complementary treatment. DA receptors are also expressed in normal and tumoral adrenals, suggesting a potential use of DA agonists in Cushing's syndrome secondary to adrenal tumors. However, clinical data regarding this specific situation are very scarce, maybe due to the relatively high rate of surgical cure of adrenal adenomas. In conclusion, DA agonists represent a potential preparatory or complementary treatment for endogenous Cushing's syndrome, especially in Cushing's disease. These compounds may be underused as suggested by the scarce number of publication and case reports in the literature. In the

  17. ACTH-secreting pancreatic neuroendocrine carcinoma with ovarian and pelvic metastases causing Cushing's syndrome: a case report.

    Science.gov (United States)

    Yao, Wen-Qing; Wu, Xia; Li, Gan-Di; Wu, Wei-Lu; Wang, Wei-Ya

    2015-01-01

    Adrenocorticotropin hormone (ACTH)-secreting pancreatic neuroendocrine carcinoma (NEC) with ovarian and pelvic metastases causing Cushing's syndrome is very rare and might be misdiagnosed. We describe a case of ACTH-secreting pancreatic poorly differentiated NEC developing bilateral ovarian and pelvic metastases. A 27-year-old woman presented with thirst, polydipsia, fatigue and poorly controlled hyperglycemia. Laboratory and imaging investigations revealed hypokalemia, hyperglycaemia, ACTH-dependent hypercortisolemia and a 12-cm mass at the junction of body and tail of the pancreas with ovarian and pelvic nodules. The patient underwent partial pancreatectomy and splenectomy, uterectomy, bilateral oophorectomy, and excision of peritoneal nodules. Tumors in pancreas, ovaries and pelvis were diagnosed as poor-differentiated NEC. After 19-month chemotherapy, she developed pelvic metastasis. The tumor in our case is a large, poorly differentiated NEC secreting ACTH and causing CS, with ovarian metastases. To our knowledge, this new additional case of ACTH-secreting pancreatic NEC with ovarian metastases would add to the better understanding of this tumor.

  18. Outbreak of exogenous Cushing's syndrome due to unlicensed medications.

    Science.gov (United States)

    Azizi, Fereidoun; Jahed, Adel; Hedayati, Mehdi; Lankarani, Mahnaz; Bejestani, Hadi Shahrad; Esfahanian, Fatemeh; Beyraghi, Nargues; Noroozi, Alireza; Kobarfard, Farzad

    2008-12-01

    Despite the widespread medical use of glucocorticoids, reports of factitious administration of these hormones have been uncommon. We herein report an outbreak of Cushing's syndrome in Tehran among the addicts using Tamgesic (a brand of Buprenorphine) to help them through the narcotic withdrawal stage, without knowledge of the glucocorticoid content of the black-market drug. Case histories of 19 patients with a final diagnosis of iatrogenic Cushing's syndrome were reviewed. Liquid chromatography/mass spectrometry (LC-Mass) method was used to evaluate glucocorticoid existence in the brand. High performance liquid chromatography was used to determine plasma dexamethasone level. No buprenorphine was present in the vials. Each Tamgesic vial contained 0.4 mg of Dexamethasone disodium phosphate; Heroin was also found in them. The duration of injection abuse and the total dexamethasone intake was 4.5 (1-18) months and 2.6 (0.8-8) mg/day, respectively. Median plasma dexamethasone concentration was 5.8 nmol/l, with a range of 5-8.7. Physical findings of the cases were not different from those of the classic endogenous Cushing's syndrome but their serum cortisol and urinary free cortisol were suppressed. Severe life-threatening complications were demonstrated in five cases. Surreptitious use of steroids resulting in Cushing's syndrome may be more common in opium addicts; a high degree of suspicion is needed to uncover this disorder. Whenever facing a cushingoid appearance in addicts, the possibility of using black market drugs with corticosteroid contents should be kept in mind.

  19. Lesson of the month 2: Cauda equina in Cushing's syndrome.

    Science.gov (United States)

    Maslin, Douglas; Gounaris, Ioannis; Ng, Kenrick; Corrie, Pippa

    2016-02-01

    We present the case of a 34-year old woman who initially presented with obesity and back pain. She was eventually diagnosed with Cushing's syndrome secondary to an adrenocortical carcinoma that had metastasised to her spine, causing cauda equina compression. The delays in reaching the correct diagnosis caused significant morbidity and exemplify the pitfalls of premature closing, a common cognitive error in diagnostic reasoning. © Royal College of Physicians 2016. All rights reserved.

  20. Selective inferior petrosal sinus sampling without venous outflow diversion in the detection of a pituitary adenoma in Cushing's syndrome

    International Nuclear Information System (INIS)

    Andereggen, Lukas; Schroth, Gerhard; Gralla, Jan; Ozdoba, Christoph; Seiler, Rolf; Mariani, Luigi; Beck, Juergen; Widmer, Hans-Rudolf; Andres, Robert H.; Christ, Emanuel

    2012-01-01

    Conventional MRI may still be an inaccurate method for the non-invasive detection of a microadenoma in adrenocorticotropin (ACTH)-dependent Cushing's syndrome (CS). Bilateral inferior petrosal sinus sampling (BIPSS) with ovine corticotropin-releasing hormone (oCRH) stimulation is an invasive, but accurate, intervention in the diagnostic armamentarium surrounding CS. Until now, there is a continuous controversial debate regarding lateralization data in detecting a microadenoma. Using BIPSS, we evaluated whether a highly selective placement of microcatheters without diversion of venous outflow might improve detection of pituitary microadenoma. We performed BIPSS in 23 patients that met clinical and biochemical criteria of CS and with equivocal MRI findings. For BIPSS, the femoral veins were catheterized bilaterally with a 6-F catheter and the inferior petrosal sinus bilaterally with a 2.7-F microcatheter. A third catheter was placed in the right femoral vein. Blood samples were collected from each catheter to determine ACTH blood concentration before and after oCRH stimulation. In 21 patients, a central-to-peripheral ACTH gradient was found and the affected side determined. In 18 of 20 patients where transsphenoidal partial hypophysectomy was performed based on BIPSS findings, microadenoma was histologically confirmed. BIPSS had a sensitivity of 94% and a specificity of 67% after oCRH stimulation in detecting a microadenoma. Correct localization of the adenoma was achieved in all Cushing's disease patients. BIPSS remains the gold standard in the detection of a microadenoma in CS. Our findings show that the selective placement of microcatheters without venous outflow diversion might further enhance better recognition to localize the pituitary tumor. (orig.)

  1. Neuropsychiatric findings in Cushing syndrome and exogenous glucocorticoid administration.

    Science.gov (United States)

    Starkman, Monica N

    2013-09-01

    This article reviews the neuropsychiatric presentations elicited by spontaneous hypercortisolism and exogenous supraphysiologic glucocorticoids. Patients with Cushing disease and syndrome develop a depressive syndrome: irritable and depressed mood, decreased libido, disrupted sleep and cognitive decrements. Exogenous short-term glucocorticoid administration may elicit a hypomanic syndrome with mood, sleep and cognitive disruptions. Treatment options are discussed. Brain imaging and neuropsychological studies indicate elevated cortisol and other glucocorticoids are especially deleterious to hippocampus and frontal lobe. The research findings also shed light on neuropsychiatric abnormalities in conditions that have substantial subgroups exhibiting elevated and dysregulated cortisol: aging, major depressive disorder and Alzheimer's disease. Copyright © 2013 Elsevier Inc. All rights reserved.

  2. AN INDIVIDUALIZED APPROACH TO THE EVALUATION OF CUSHING SYNDROME.

    Science.gov (United States)

    Sharma, Susmeeta T

    2017-06-01

    Cushing syndrome (CS) is caused by chronic exposure to excess glucocorticoids. Early recognition and treatment of hypercortisolemia can lead to decreased morbidity and mortality. The diagnosis of CS and thereafter, establishing the cause can often be difficult, especially in patients with mild and cyclic hypercortisolism. Surgical excision of the cause of excess glucocorticoids is the optimal treatment for CS. Medical therapy (steroidogenesis inhibitors, medications that decrease adrenocorticotropic hormone [ACTH] levels or glucocorticoid antagonists) and pituitary radiotherapy may be needed as adjunctive treatment modalities in patients with residual, recurrent or metastatic disease, in preparation for surgery, or when surgery is contraindicated. A multidisciplinary team approach, individualized treatment plan and long-term follow-up are important for optimal management of hypercortisolemia and the comorbidities associated with CS. ACTH = adrenocorticotropic hormone; BIPSS = bilateral inferior petrosal sinus sampling; CBG = corticosteroid-binding globulin; CD = Cushing disease; CRH = corticotropin-releasing hormone; CS = Cushing syndrome; Dex = dexamethasone; DST = dexamethasone suppression test; EAS = ectopic ACTH syndrome; FDA = U.S. Food & Drug Administration; HDDST = high-dose DST; IPS/P = inferior petrosal sinus to peripheral; MRI = magnetic resonance imaging; NET = neuroendocrine tumor; PET = positron emission tomography; UFC = urinary free cortisol.

  3. Cushing's syndrome due to interaction between inhaled corticosteroids and itraconazole.

    Science.gov (United States)

    Bolland, Mark J; Bagg, Warwick; Thomas, Mark G; Lucas, Jennifer A; Ticehurst, Rob; Black, Peter N

    2004-01-01

    To report a case of an interaction between inhaled corticosteroids and itraconazole causing iatrogenic Cushing's syndrome and provide a review of the relevant literature. A 70-year-old white woman on long-term treatment with high-dose inhaled corticosteroids for asthma was diagnosed as having Scedosporium apiospermum infection of the skin and subcutaneous tissues. As a result, she was treated with itraconazole for 2 months. She subsequently developed Cushing's syndrome due to a probable cytochrome P450-mediated interaction between itraconazole and budesonide. She also had secondary adrenal insufficiency requiring prolonged treatment with replacement hydrocortisone. Budesonide is a potent glucocorticoid that is metabolized in the liver by the CYP3A4 isoenzyme to inactive metabolites. Itraconazole is a potent cytochrome P450 inhibitor. It can inhibit the metabolism of oral or inhaled corticosteroids, producing cortisol excess leading to Cushing's syndrome and adrenal insufficiency. An assessment of causality indicated a possible adverse interaction between itraconazole and budesonide. The combination of itraconazole and inhaled corticosteroids is increasingly being used to treat conditions such as allergic bronchopulmonary aspergillosis. Clinicians need to be aware of the potential for an interaction between such a combination.

  4. Cushing's syndrome: from physiological principles to diagnosis and clinical care.

    Science.gov (United States)

    Raff, Hershel; Carroll, Ty

    2015-02-01

    The physiological control of cortisol synthesis in the adrenal cortex involves stimulation of adrenocorticotrophic hormone (ACTH) by hypothalamic corticotrophin-releasing hormone (CRH) and then stimulation of the adrenal by ACTH. The control loop of the hypothalamic-pituitary-adrenal (HPA) axis is closed by negative feedback of cortisol on the hypothalamus and pituitary. Understanding this system is required to master the diagnosis, differential diagnosis and treatment of endogenous hypercortisolism--Cushing's syndrome. Endogenous Cushing's syndrome is caused either by excess ACTH secretion or by autonomous cortisol release from the adrenal cortex. Diagnosis of cortisol excess exploits three physiological principles: failure to achieve the normal nadir in the cortisol diurnal rhythm, loss of sensitivity of ACTH-secreting tumours to cortisol negative feedback, and increased excretion of free cortisol in the urine. Differentiating a pituitary source of excess ACTH (Cushing's disease) from an ectopic source is accomplished by imaging the pituitary and sampling for ACTH in the venous drainage of the pituitary. With surgical removal of ACTH or cortisol-secreting tumours, secondary adrenal insufficiency ensues because of the prior suppression of the HPA axis by glucocorticoid negative feedback. Medical therapy is targeted to the anatomical location of the dysregulated component of the HPA axis. Future research will focus on new diagnostics and treatments of Cushing's syndrome. These are elegant examples of translational research: understanding basic physiology informs the development of new approaches to diagnosis and treatment. Appreciating pathophysiology generates new areas for inquiry of basic physiological and biochemical mechanisms. © 2014 The Authors. The Journal of Physiology © 2014 The Physiological Society.

  5. Cushing disease revealed by bilateral atypical central serous chorioretinopathy: case report.

    Science.gov (United States)

    Giovansili, Iama; Belange, Georeges; Affortit, Aude

    2013-01-01

    We report the case of a patient with Cushing disease revealed by bilateral central serous chorioretinopathy (CSCR). We present the clinical history, physical findings, laboratory results, and imaging studies of a 53-year-old Chinese woman with a Cushing disease revealed by bilateral CSCR. The association with CSCR and the pertinent literature are reviewed. A 53-year-old patient initially presented to the Department of Ophthalmology with a 4-week history of decreased vision in the left eye. Standard ophthalmologic examination and fluorescein angiography established the diagnosis of bilateral CSCR. Systemic clinical signs and biochemical analysis indicated hypercortisolism. Magnetic resonance imaging (MRI) of the pituitary gland showed a left-side lesion compatible with a microadenoma. The diagnosis of Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome secondary to a pituitary microadenoma was selected. Endoscopic endonasal transsphenoidal surgery was performed and the pituitary adenoma was successfully removed. The histology confirmed the presence of ACTH-immunopositive pituitary adenoma. Early postoperative morning cortisol levels indicated early remission. At 6 weeks postoperatively, the patient's morning cortisol remains undetectable, and serous retinal detachments had regressed. CSCR is an uncommon manifestation of endogenous Cushing syndrome. It can be the first presentation of hypercortisolism caused by Cushing disease. CSCR should be considered when assessing patients with Cushing syndrome complaining of visual disorders. On the other hand, it is useful in patients with an atypical form of CSCR to exclude Cushing's syndrome.

  6. Ectopic ACTH syndrome

    OpenAIRE

    Isidori, Andrea M.; Lenzi, Andrea

    2007-01-01

    Ectopic adrenocorticotropic secretion (EAS) is responsible for 12-17% of cases of Cushing's syndrome (CS) and covers a range of tumours, from undetectable benign lesions to widespread metastases. The syndrome is often associated with severe hypercortisolaemia, which aggravates the underlying condition. EAS requires a complete workup that includes the establishment of endogenous CS, diagnosis of adrenocorticotropic hormone (ACTH) dependency, localization of the source of ACTH secretion and rap...

  7. Diagnosis and clinical genetics of Cushing syndrome in pediatrics

    Science.gov (United States)

    Stratakis, Constantine A.

    2016-01-01

    SYNOPSIS Endogenous Cushing syndrome (CS) in pediatrics is rare; it may be caused by tumors that produce corticotropin (ACTH) in the pituitary gland (this form of CS is called Cushing disease) or elsewhere (ectopic CS), tumors that produce corticotropin-releasing hormone (CRH) anywhere (mostly neuroendocrine tissues), and finally adrenocortical masses that produce cortisol, such as adrenocortical cancer (ACC) or adenomas, and bilateral adrenocortical hypeprlasia (BAHs). ACC is a very rare cause of CS in children but should be excluded first, especially among younger patients. CS in children is often caused by germline or somatic mutations in an expanding list of genes with implications for the prognosis of the patients and for their families. CS should be early recognized in children; otherwise, it can lead to significant morbidity and mortality. All patients with suspected CS should be referred to specialized clinical centers for work-up; these centers should have access to experienced endocrine and neurological surgeons. PMID:27241967

  8. Increased Adipocyte Size, Macrophage Infiltration, and Adverse Local Adipokine Profile in Perirenal Fat in Cushing's Syndrome

    NARCIS (Netherlands)

    Roerink, S.H.P.P.; Wagenmakers, M.A.E.M.; Langenhuijsen, J.F.; Ballak, D.B.; Rooijackers, H.M.M.; D'Ancona, F.C.H.; Dielen, F.M. van; Smit, J.W.A.; Plantinga, T.S.; Netea-Maier, R.T.; Hermus, A.R.M.M.

    2017-01-01

    OBJECTIVE: To analyze changes in fat cell size, macrophage infiltration, and local adipose tissue adipokine profiles in different fat depots in patients with active Cushing's syndrome. METHODS: Subcutaneous (SC) and perirenal (PR) adipose tissue of 10 patients with Cushing's syndrome was compared to

  9. Cushing's syndrome caused by use of synthetic ocular steroid.

    Science.gov (United States)

    Üstyol, A; Kökali, F; Duru, N; Duman, M A; Elevli, M

    2017-12-01

    Cases of Cushing's syndrome (CS) following ocular steroid use have been reported in recent years, albeit rarely. We report a case of iatrogenic CS in a child induced by fluorometholone-containing eyedrops. Our patient was referred to our endocrinology clinic due to rapid weight gain. His history revealed that 1.5 months previously he had been started on fluorometholone eyedrops. To the best of our knowledge, no cases of CS have been reported following ocular fluorometholone use. Although eyedrops containing potent glucocorticoids may lead to CS, fluorometholone, a relatively less potent steroid, may also cause the syndrome, as in our case. © 2017 John Wiley & Sons Ltd.

  10. Topical-steroid-induced iatrogenic Cushing syndrome in the pediatric age group: A rare case report.

    Science.gov (United States)

    Tiwari, Ashish; Goel, Manjusha; Pal, Pankaj; Gohiya, Poorva

    2013-10-01

    Cushing syndrome, a systemic disorder, is the result of abnormally high blood level of cortisol or other glucocorticoids. The most common cause of Cushing syndrome is prolonged exogenous administration of glucocorticoid hormones. Prolonged use of topical corticosteroids, particularly in children, may cause Cushing syndrome and suppression of the hypothalamopituitory-adrenal axis, which is less common than that of oral or parenteral route. However, iatrogenic Cushing syndrome in the infantile age group due to topical steroid is very rare and only a few patients have been reported to date in the literature. Here we report a case of iatrogenic Cushing syndrome due to topical steroid application in a 5-month-old female child admitted to the hospital for repeated episodes of fever and cough.

  11. A Case of Paraneoplastic Cushing Syndrome Presenting as Hyperglycemic Hyperosmolar Nonketotic Syndrome

    Directory of Open Access Journals (Sweden)

    Christina E. Brzezniak

    2017-04-01

    Full Text Available Carcinoid tumors are neuroendocrine tumors that mainly arise in the gastrointestinal tract, lungs, and bronchi. Bronchopulmonary carcinoids have been associated with Cushing syndrome, which results from ectopic adrenocorticotrophic hormone (ACTH secretion. We report the case of a 65-year-old man, a colonel in the US Air Force, with metastatic bronchopulmonary carcinoid tumors treated on a clinical trial who was hospitalized for complaints of increasing thirst, polydipsia, polyuria, weakness, and visual changes. Decompensated hyperglycemia suggested a diagnosis of hyperglycemic hyperosmolar nonketotic syndrome (HHNS. Additional findings, which included hypokalemia, hypernatremia, hypertension, metabolic alkalosis, moon facies, and striae, raised a red flag for an ectopic ACTH syndrome. Elevated ACTH levels confirmed Cushing syndrome. Treatment with a fluid replacement and insulin drip resulted in immediate symptomatic improvement. Cushing syndrome should be considered in carcinoid patients with physical stigmata such as moon facies and striae. HHNS may be the presenting clinical feature in patients with impaired glucose metabolism.

  12. Preventing Cushing: Iatrogenic Cushing Syndrome due to Ritonavir-Fluticasone Interaction.

    Science.gov (United States)

    Tiruneh, Fasil; Awan, Ahmad; Didana, Abiot; Doshi, Saumil

    2017-07-17

    Ritonavir is commonly used in low doses to boost plasma levels of protease inhibitors in patients with human immunodeficiency virus (HIV) infections. It is also a potent inhibitor of cytochrome P450. We present a 50-year-old African American male with past medical history of HIV on highly active antiretroviral therapy (HAART), which also included ritonavir and long standing asthma that has been treated with inhaled fluticasone, who presented with back pain. He had central obesity, prominent abdominal striae and wasted extremities on physical examination. Laboratory tests showed low morning serum cortisol and suboptimal cosyntropin test consistent with adrenal insufficiency. Computed tomography (CT) of the spine showed a fracture of inferior endplate of the lumbar (L3) vertebra. The cause of osteoporosis is believed to be iatrogenic Cushing syndrome caused by enhanced levels of inhaled fluticasone effects secondary to inhibition of cytochrome P450. The patient was managed surgically and fluticasone was discontinued.

  13. Adrenal incidentalomas showing unilateral concordant visualization by adrenocortical scintigraphy. Comparison with adenomas in Cushing's syndrome

    International Nuclear Information System (INIS)

    Tani, Atsushi; Nakajo, Masayuki; Tsuchimochi, Shinsaku; Nakabeppu, Yoshiaki; Umanodan, Tomokazu

    2000-01-01

    An adrenocortical adenoma causing Cushing's syndrome (Cushing's adenoma) produces a unilateral concordant visualization (UCV) imaging pattern in which the adenoma is only visualized on radioiodocholesterol adrenocortical scintigraphy. But because this imaging pattern is also noted in some patients with adrenal incidentalomas, we examined whether the UCV-incidentaloma was essentially identical with Cushing's adenoma and would develop Cushing's syndrome. The subjects were 9 patients with UCV-incidentalomas (mean size, 30 mm; range, 20-45 mm) and 6 patients with Cushing's adenomas (mean size, 28 mm; range, 25-35 mm). Endocrinological evaluations showed several abnormalities including blunted diurnal rhythm of plasma cortisol within the normal range, low plasma ACTH and/or high 24-hr urinary 17-OHCS levels in 8 of 9 patients with UCV-incidentalomas, but these abnormalities did not meet the diagnostic criteria of Cushing's syndrome. Adrenal uptake of the tracer in the patients with UCV-incidentalomas was not statistically different from that in the patients with Cushing's adenomas and had no relationship with hormonal values in either patient group. Tumor size on CT correlated with the levels of 24-hr urinary 17-OHCS (r=0.75, p=0.02) and plasma cortisol at 7:00 (r=0.82, p=0.007) in the patients with UCV-incidentalomas, but not in the patients with Cushing's adenomas. Although 3 UCV-incidentalomas increased slightly in size, none of 9 patients with UCV-incidentalomas has developed Cushing's syndrome for 4 to 52 months. These results suggest that the UCV-incidentaloma may be essentially different from the Cushing's adenoma and unlikely to develop Cushing's syndrome. (author)

  14. Hypercalcemia in a dog with resolution of iatrogenic Cushing's syndrome.

    Science.gov (United States)

    Nakamura, Momoko; Kawamura, Yuko; Minegishi, Michiyo; Momoi, Yasuyuki; Iwasaki, Toshiroh

    2004-03-01

    A six-year-old spayed Pug was presented with crust formation and ulcer on the skin. The patient had received long-term glucocorticoid therapy for treatment of tentatively diagnosed panniculitis. Severe calcification and pyoderma was observed and the patient was diagnosed with iatrogenic Cushing's syndrome and predonisolone was gradually withdrawn. After the withdrawal, the patient developed marked hypercalcemia (15.3 mg/dl) and finally died from renal failure. It is postulated that the eluted calcium from the calcified lesions may have contributed to the high serum calcium level as the underlying disease was not identified on necropsy.

  15. Budesonide-Related Iatrogenic Cushing's Syndrome in Microscopic Colitis.

    Science.gov (United States)

    Tripathi, Kartikeya; Dunzendorfer, Thomas

    2017-01-01

    Budesonide is the treatment of choice for microscopic colitis because of its excellent risk to benefit ratio. It is a potent, well-absorbed corticosteroid, but because of a high rate of first-pass metabolism in the liver, its systemic bioavailability is low. It has fewer corticosteroid-related adverse effects than prednisone, and adrenal suppression is considered to be rare. We present a middle-aged woman with lymphocytic colitis whose symptoms responded to budesonide but developed budesonide-related iatrogenic Cushing's syndrome. Withdrawal of budesonide led to restoration of normal pituitary-adrenal responsiveness but at the price of recurrent diarrhea due to re-emergence of lymphocytic colitis.

  16. Iatrogenic Cushing's syndrome after triamcinolone plus ritonavir-boosted atazanavir.

    Science.gov (United States)

    Jakeman, Bernadette; Conklin, Jessica; Bouchonville, Matthew; Thornton, Karla

    2015-01-01

    To describe a case of iatrogenic Cushing's syndrome (ICS) following a triamcinolone injection for subscapular bursitis in an HIV-positive patient receiving an antiretroviral regimen that included ritonavir boosted-atazanavir. University outpatient HIV clinic. A 60-year-old HIV-positive man on a ritonavir-boosted, atazanavir-containing antiretroviral regimen was diagnosed with subscapular bursitis. The patient received two intrabursal injections with 1% lidocaine plus triamcinolone 20 mg. Four weeks after the injections, the patient experienced symptoms of Cushing's syndrome with a pronounced drop in his CD4+ T-cell count, requiring treatment of oral candidiasis and prophylaxis for opportunistic infections. The interaction between ritonavir and oral corticosteroids, resulting in ICS, has been established. This case adds to the literature as one of the few cases illustrating that interaction can also occur between ritonavir and intrabursal administration of corticosteroids. This case further supports concerns regarding use of corticosteroids in HIV-infected patients who are treated with ritonavir-containing antiretroviral regimens.

  17. Bilateral inferior petrosal sinus sampling in the diagnosis of Cushing disease

    Directory of Open Access Journals (Sweden)

    Deipolyi AR

    2015-04-01

    Full Text Available Amy R Deipolyi,1 Rahmi Oklu21Vascular and Interventional Radiology, NYU Langone Medical Center, New York, NY, USA; 2Division of Vascular and Interventional Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USAAbstract: Bilateral inferior petrosal sinus sampling (BIPSS is a minimally invasive procedure performed in the workup of adrenocorticotropic hormone (ACTH-dependent Cushing syndrome (CS. Because noninvasive tests in the evaluation of CS patients lack sensitivity, BIPSS is the gold standard in diagnosing Cushing disease (CD, which is a pituitary source of excess ACTH. Here, the pathophysiology of CD and procedural details of BIPSS are reviewed.Keywords: pituitary adenoma, Cushing disease, inferior petrosal sinus, venous sampling

  18. Cushing syndrome: maybe not so uncommon of an endocrine disease.

    Science.gov (United States)

    Guaraldi, Federica; Salvatori, Roberto

    2012-01-01

    Cushing syndrome (CS) is the result of extended exposure to excessive glucocorticoids from endogenous or exogenous sources. Traditionally, the most common cause of endogenous CS is a pituitary adenoma (Cushing disease). Less common causes are adrenocortical tumors and extrapituitary adrenocorticotropin-producing neoplasias. This review provides updated information regarding the potential for increased prevalence of CS in specific patient populations. Here the authors provide to family physicians clinical guidance for recognition of CS by presenting a case, discussing the advantages/disadvantages of the diagnostic tests, and discussing information about the treatment options. CS is expected to have an incidence of 10 to 15 people per million; however, studies of patients with diabetes, obesity, hypertension, and osteoporosis found a high prevalence of CS among these populations. The clinical manifestations of CS range from the distinctive clinical features (purple striae, facial plethora, proximal myopathy) to common conditions such as hypertension, obesity, and diabetes. Clinical practice guidelines recommend biochemical tests to screen patients for CS; however, the sensitivity and specificity of these tests vary, so a careful analysis must be performed to avoid misdiagnosis. CS is challenging to diagnose. Nevertheless, with a systematic approach to testing patients and an increased awareness of the high-risk patient populations, the disease can be identified in a timely manner.

  19. Massive Thoracoabdominal Aortic Thrombosis in a Patient with Iatrogenic Cushing Syndrome

    International Nuclear Information System (INIS)

    Kim, Dong Hun; Choi, Dong Hyun; Lee, Young Min; Kim, Bo Bae; Ki, Young Jae; Kim, Jin Hwa; Chung, Joong Wha; Koh, Young Youp; Kang, Joon Tae; Chae, Seung Seok

    2014-01-01

    Massive thoracoabdominal aortic thrombosis is a rare finding in patients with iatrogenic Cushing syndrome in the absence of any coagulation abnormality. It frequently represents an urgent surgical situation. We report the case of an 82-year-old woman with massive aortic thrombosis secondary to iatrogenic Cushing syndrome. A follow-up computed tomography scan showed a decreased amount of thrombus in the aorta after anticoagulation therapy alone.

  20. [Exogenous Cushing's syndrome as a serious side-effect of therapy with ritonavir an inhaled fluticasone].

    Science.gov (United States)

    Machala, Ladislav; Kréze, Alexander; Jilich, David; Rozsypal, Hanuš

    2011-06-01

    Inhalation of fluticasone is usually devoid of systemic side-effects. The authors describe a case of a young HIV positive woman treated concomitantly with fluticasone and inhibitors of HIV protease ritonavir and lopinavir in which developed a serious endocrine side-effect - an iatrogenic Cushing's syndrome. Plasma concentration of cortisol Cushing's syndrome, and the substitution therapy with hydrocortisone was terminated. Two years later became the patient pregnant and gave birth to a healthy child.

  1. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    OpenAIRE

    Leary, Joyce; Swislocki, Arthur

    2013-01-01

    Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a...

  2. Two cases of Cushing's syndrome due to overuse of topical steroid in the diaper area.

    Science.gov (United States)

    Semiz, Serap; Balci, Yasemin Işik; Ergin, Seniz; Candemir, Maşallah; Polat, Aziz

    2008-01-01

    Topical use of corticosteroids may cause immunosuppression and iatrogenic Cushing's syndrome via hypothalamic-pituitary-adrenal axis. We report two cases with iatrogenic Cushing's syndrome with different clinical outcomes due to abuse of same potent topical steroid clobetasol propionate. One of them died because of fatal disseminated cytomegalovirus infection. The other patient recovered completely. Physicians and parents should be informed about the adverse effects of such potent topical corticosteroids and physicians should prescribe less potent agents, especially during infancy.

  3. Brain Atrophy and Hypomyelination Associated with Iatrogenic Cushing Syndrome in an Infant.

    Science.gov (United States)

    Dogan, Sumeyra; S Dogan, Mehmet; Tutunculer, Filiz; Yapiciugurlar, Ozge; Genchellac, Hakan

    2018-01-01

    Prolonged use of topical corticosteroids, particularly in infants, albeit rare, may lead to Cushing syndrome. Central nervous system abnormalities including brain atrophy and delayed myelination on cranial magnetic resonance imaging has been reported in patients with corticosteroid treatment. We herein report a 5-month-old female infant referred to Department of Pediatric Endocrinology, Edirne, Turkey with brain atrophy and myelination delay that might be due to iatrogenic Cushing syndrome caused by topical corticosteroid use.

  4. Iatrogenic Cushing's syndrome with inhaled steroid plus antidepressant drugs.

    Science.gov (United States)

    Celik, Ozlem; Niyazoglu, Mutlu; Soylu, Hikmet; Kadioglu, Pinar

    2012-08-29

    Current guidelines recommend the use of inhaled corticosteroids (ICS) for suppression of airway inflammation in patients with asthma. Although it is well known that ICS cause dose-related adrenocortical suppression, it is less known that they can lead to iatrogenic Cushing's syndrome (CS). Fluticasone propionate (FP) is an ICS more potent than beclomethasone and budesonide. FP is metabolized as mediated by cytochrome P450 3A4 in the liver and the gut. Systemic bioactivity of FP can increase with the use of drugs that affect the cytochrome P450. Herein, we report the rapid development of iatrogenic CS in a patient receiving paroxetine and mirtazepine for 12 weeks in addition to inhaled FP.

  5. Iatrogenic Cushing's syndrome following short-term intranasal steroid use.

    Science.gov (United States)

    Dutta, Deep; Shivaprasad, K S; Ghosh, Sujoy; Mukhopadhyay, Satinath; Chowdhury, Subhankar

    2012-09-01

    Cushing's syndrome (CS) is common after oral steroid use and has also been reported following topical or inhaled use, but it is extremely uncommon after intranasal administration. In this paper, we present the case of a child who developed CS after intranasal application of combined moxifloxacin-dexamethasone eye drops for epistaxis for a period of 3 months. CS caused by ocular preparations of steroids has not been reported previously. This case report highlights the fact that even eye drops can contain high doses of steroids and can lead to CS especially in children and especially if used intranasally. Ocular steroid drops should not be used intranasally. To minimize gastrointestinal absorption and therefore the risk of CS, nasal sprays should be preferred over nasal drops for intranasal steroid application.

  6. Metabolomic Biomarkers in Urine of Cushing's Syndrome Patients.

    Science.gov (United States)

    Kotłowska, Alicja; Puzyn, Tomasz; Sworczak, Krzysztof; Stepnowski, Piotr; Szefer, Piotr

    2017-01-29

    Cushing's syndrome (CS) is a disease which results from excessive levels of cortisol in the human body. The disorder is associated with various signs and symptoms which are also common for the general population not suffering from compound hypersecretion. Thus, more sensitive and selective methods are required for the diagnosis of CS. This follow-up study was conducted to determine which steroid metabolites could serve as potential indicators of CS and possible subclinical hypercortisolism in patients diagnosed with so called non-functioning adrenal incidentalomas (AIs). Urine samples from negative controls ( n = 37), patients with CS characterized by hypercortisolism and excluding iatrogenic CS ( n = 16), and patients with non-functioning AIs with possible subclinical Cushing's syndrome ( n = 25) were analyzed using gas chromatography-mass spectrometry (GC/MS) and gas chromatograph equipped with flame ionization detector (GC/FID). Statistical and multivariate methods were applied to investigate the profile differences between examined individuals. The analyses revealed hormonal differences between patients with CS and the rest of examined individuals. The concentrations of selected metabolites of cortisol, androgens, and pregnenetriol were elevated whereas the levels of tetrahydrocortisone were decreased for CS when opposed to the rest of the study population. Moreover, after analysis of potential confounding factors, it was also possible to distinguish six steroid hormones which discriminated CS patients from other study subjects. The obtained discriminant functions enabled classification of CS patients and AI group characterized by mild hypersecretion of cortisol metabolites. It can be concluded that steroid hormones selected by applying urinary profiling may serve the role of potential biomarkers of CS and can aid in its early diagnosis.

  7. Risk of cardiovascular events in people prescribed glucocorticoids with iatrogenic Cushing's syndrome: cohort study.

    Science.gov (United States)

    Fardet, Laurence; Petersen, Irene; Nazareth, Irwin

    2012-07-30

    To investigate whether there is an increased risk of cardiovascular events in people who exhibit iatrogenic Cushing's syndrome during treatment with glucocorticoids. Cohort study. 424 UK general practices contributing to The Health Improvement Network database. People prescribed systemic glucocorticoids and with a diagnosis of iatrogenic Cushing's syndrome (n = 547) and two comparison groups: those prescribed glucocorticoids and with no diagnosis of iatrogenic Cushing's syndrome (n = 3231) and those not prescribed systemic glucocorticoids (n = 3282). Incidence of cardiovascular events within a year after diagnosis of iatrogenic Cushing's syndrome or after a randomly selected date, and association between iatrogenic Cushing's syndrome and risk of cardiovascular events. 417 cardiovascular events occurred in 341 patients. Taking into account only the first event by patient (coronary heart disease n = 177, heart failure n = 101, ischaemic stroke n = 63), the incidence rates of cardiovascular events per 100 person years at risk were 15.1 (95% confidence interval 11.8 to 18.4) in those prescribed glucocorticoids and with a diagnosis of iatrogenic Cushing's syndrome, 6.4 (5.5 to 7.3) in those prescribed glucocorticoids without a diagnosis of iatrogenic Cushing's syndrome, and 4.1 (3.4 to 4.8) in those not prescribed glucocorticoids. In multivariate analyses adjusted for sex, age, intensity of glucocorticoid use, underlying disease, smoking status, and use of aspirin, diabetes drugs, antihypertensive drugs, lipid lowering drugs, or oral anticoagulant drugs, the relation between iatrogenic Cushing's syndrome and cardiovascular events was strong (adjusted hazard ratios 2.27 (95% confidence interval 1.48 to 3.47) for coronary heart disease, 3.77 (2.41 to 5.90) for heart failure, and 2.23 (0.96 to 5.17) for ischaemic cerebrovascular events). The adjusted hazard ratio for any cardiovascular event was 4.16 (2.98 to 5.82) when the group prescribed glucocorticoids and with

  8. Avascular necrosis of femoral heads post-adrenal surgery for Cushing's syndrome: a rare presentation.

    LENUS (Irish Health Repository)

    2012-01-31

    Avascular necrosis (AVN) is a well-recognized complication of patients on high-dose steroids for a long time. Exogenous hypercortisolism is a well known cause of AVN and a number of cases have been reported. Cushing\\'s syndrome describes hypercortisolism of any cause endogenous or exogenous. A variety of traumatic and non-traumatic factors contribute to the aetiology of AVN although exogenous glucocorticoids administration and alcoholism are among the most common non-traumatic causes. AVN secondary to endogenous hypercortisolism is rare and very few case reports are available describing this complication. No literature is available on AVN presenting post-adrenal surgery. Here we present a young woman who presented with avascular necrosis of both hips 1 year after adrenalectomy for Cushing\\'s syndrome.

  9. Selective inferior petrosal sinus sampling without venous outflow diversion in the detection of a pituitary adenoma in Cushing's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Andereggen, Lukas [Bern University Hospital, University Institute of Diagnostic and Interventional Neuroradiology, Bern (Switzerland); Bern University Hospital, Department of Neurosurgery, Bern (Switzerland); Schroth, Gerhard; Gralla, Jan; Ozdoba, Christoph [Bern University Hospital, University Institute of Diagnostic and Interventional Neuroradiology, Bern (Switzerland); Seiler, Rolf; Mariani, Luigi; Beck, Juergen; Widmer, Hans-Rudolf; Andres, Robert H. [Bern University Hospital, Department of Neurosurgery, Bern (Switzerland); Christ, Emanuel [Bern University Hospital, Division of Endocrinology, Diabetology and Clinical Nutrition, Bern (Switzerland)

    2012-05-15

    Conventional MRI may still be an inaccurate method for the non-invasive detection of a microadenoma in adrenocorticotropin (ACTH)-dependent Cushing's syndrome (CS). Bilateral inferior petrosal sinus sampling (BIPSS) with ovine corticotropin-releasing hormone (oCRH) stimulation is an invasive, but accurate, intervention in the diagnostic armamentarium surrounding CS. Until now, there is a continuous controversial debate regarding lateralization data in detecting a microadenoma. Using BIPSS, we evaluated whether a highly selective placement of microcatheters without diversion of venous outflow might improve detection of pituitary microadenoma. We performed BIPSS in 23 patients that met clinical and biochemical criteria of CS and with equivocal MRI findings. For BIPSS, the femoral veins were catheterized bilaterally with a 6-F catheter and the inferior petrosal sinus bilaterally with a 2.7-F microcatheter. A third catheter was placed in the right femoral vein. Blood samples were collected from each catheter to determine ACTH blood concentration before and after oCRH stimulation. In 21 patients, a central-to-peripheral ACTH gradient was found and the affected side determined. In 18 of 20 patients where transsphenoidal partial hypophysectomy was performed based on BIPSS findings, microadenoma was histologically confirmed. BIPSS had a sensitivity of 94% and a specificity of 67% after oCRH stimulation in detecting a microadenoma. Correct localization of the adenoma was achieved in all Cushing's disease patients. BIPSS remains the gold standard in the detection of a microadenoma in CS. Our findings show that the selective placement of microcatheters without venous outflow diversion might further enhance better recognition to localize the pituitary tumor. (orig.)

  10. Plasma Steroid Metabolome Profiling for Diagnosis and Subtyping Patients with Cushing Syndrome.

    Science.gov (United States)

    Eisenhofer, Graeme; Masjkur, Jimmy; Peitzsch, Mirko; Di Dalmazi, Guido; Bidlingmaier, Martin; Grüber, Matthias; Fazel, Julia; Osswald, Andrea; Beuschlein, Felix; Reincke, Martin

    2018-03-01

    Diagnosis of Cushing syndrome requires a multistep process that includes verification of hypercortisolism followed by identification of the cause of adrenocortical hyperfunction. This study assessed whether pituitary, ectopic, and adrenal subtypes of Cushing syndrome were characterized by distinct plasma steroid profiles that might assist diagnosis. In this retrospective cross-sectional study, mass spectrometric measurements of a panel of 15 plasma steroids were applied to 222 patient samples tested for Cushing syndrome. Disease was excluded in 138 and confirmed in 51 patients with pituitary Cushing syndrome, 12 with ectopic adrenocorticotropin secretion, and 21 with adrenal disease. Another 277 age- and sex-matched hypertensive and normotensive volunteers were included for comparison. Compared with patients without disease, the largest increases in plasma steroids among patients with Cushing syndrome were observed for 11-deoxycortisol (289%), 21-deoxycortisol (150%), 11-deoxycorticosterone (133%), corticosterone (124%), and cortisol (122%). Patients with ectopic disease showed the most prominent increases, but there was considerable variation for other steroids according to subtype. Patients with adrenal disease had the lowest concentrations of androgens, whereas those with ectopic and pituitary disease showed the lowest concentrations of aldosterone. Plasma 18-oxocortisol was particularly low in ectopic disease. With the use of 10 selected steroids, subjects with and without different Cushing syndrome subtypes could be discriminated nearly as closely as with the use of salivary and urinary free cortisol, dexamethasone-suppressed cortisol, and plasma adrenocorticotropin (9.5% vs 5.8% misclassification). Patients with different subtypes of Cushing syndrome show distinctive plasma steroid profiles that may offer a supplementary single-test alternative for screening purposes. © 2017 American Association for Clinical Chemistry.

  11. Cushing's syndrome, growth impairment, and occult adrenal suppression associated with intranasal steroids.

    Science.gov (United States)

    Perry, R J; Findlay, C A; Donaldson, M D C

    2002-07-01

    We have previously described iatrogenic Cushing's syndrome secondary to intranasal steroids. This report further highlights the potential deleterious effects of intranasal steroids. Nine cases (including the original two cases) are reviewed to show the varied clinical manifestations of adrenal suppression caused by intranasal steroids. Four presented with Cushing's syndrome, three with growth failure, while two asymptomatic patients were discovered in the course of pituitary function testing. Four children had dysmorphic syndromes--Down's, Treacher-Collins, CHARGE association, and campomelic dysplasia--reflecting the vulnerability of such children to ENT problems, together with the difficulty of interpreting steroid induced growth failure in this context. Adrenal suppression was seen not only with betamethasone but also with budesonide, beclomethasone and flunisolide nasal preparations. A careful enquiry as to the use of intranasal steroids should be routine in children presenting with unexplained growth failure or Cushing's syndrome. Particular vigilance/awareness is required in children with dysmorphic syndromes.

  12. [Treatment of iatrogenic Cushing syndrome: questions of glucocorticoid withdrawal].

    Science.gov (United States)

    Igaz, Péter; Rácz, Károly; Tóth, Miklós; Gláz, Edit; Tulassay, Zsolt

    2007-02-04

    Iatrogenic Cushing's syndrome is the most common form of hypercortisolism. Glucocorticoids are widely used for the treatment of various diseases, often in high doses that may lead to the development of severe hypercortisolism. Iatrogenic hypercortisolism is unique, as the application of exogenous glucocorticoids leads to the simultaneous presence of symptoms specific for hypercortisolism and the suppression of the endogenous hypothalamic-pituitary-adrenal axis. The principal question of its therapy is related to the problem of glucocorticoid withdrawal. There is considerable interindividual variability in the suppression and recovery of the hypothalamic-pituitary-adrenal axis, therefore, glucocorticoid withdrawal and substitution can only be conducted in a stepwise manner with careful clinical follow-up and regular laboratory examinations regarding endogenous hypothalamic-pituitary-adrenal axis activity. Three major complications which can be associated with glucocorticoid withdrawal are: i. reactivation of the underlying disease, ii. secondary adrenal insufficiency, iii. steroid withdrawal syndrome. Here, the authors summarize the most important aspects of this area based on their clinical experience and the available literature data.

  13. The Eosinophil Count Tends to Be Negatively Associated with Levels of Serum Glucose in Patients with Adrenal Cushing Syndrome.

    Science.gov (United States)

    Lee, Younghak; Yi, Hyon Seung; Kim, Hae Ri; Joung, Kyong Hye; Kang, Yea Eun; Lee, Ju Hee; Kim, Koon Soon; Kim, Hyun Jin; Ku, Bon Jeong; Shong, Minho

    2017-09-01

    Cushing syndrome is characterized by glucose intolerance, cardiovascular disease, and an enhanced systemic inflammatory response caused by chronic exposure to excess cortisol. Eosinopenia is frequently observed in patients with adrenal Cushing syndrome, but the relationship between the eosinophil count in peripheral blood and indicators of glucose level in patients with adrenal Cushing syndrome has not been determined. A retrospective study was undertaken of the clinical and laboratory findings of 40 patients diagnosed with adrenal Cushing syndrome at Chungnam National University Hospital from January 2006 to December 2016. Clinical characteristics, complete blood cell counts with white blood cell differential, measures of their endocrine function, description of imaging studies, and pathologic findings were obtained from their medical records. Eosinophil composition and count were restored by surgical treatment of all of the patients with adrenal Cushing disease. The eosinophil count was inversely correlated with serum and urine cortisol, glycated hemoglobin, and inflammatory markers in the patients with adrenal Cushing syndrome. Smaller eosinophil populations in patients with adrenal Cushing syndrome tend to be correlated with higher levels of blood sugar and glycated hemoglobin. This study suggests that peripheral blood eosinophil composition or count may be associated with serum glucose levels in patients with adrenal Cushing syndrome. Copyright © 2017 Korean Endocrine Society

  14. The Eosinophil Count Tends to Be Negatively Associated with Levels of Serum Glucose in Patients with Adrenal Cushing Syndrome

    Directory of Open Access Journals (Sweden)

    Younghak Lee

    2017-09-01

    Full Text Available BackgroundCushing syndrome is characterized by glucose intolerance, cardiovascular disease, and an enhanced systemic inflammatory response caused by chronic exposure to excess cortisol. Eosinopenia is frequently observed in patients with adrenal Cushing syndrome, but the relationship between the eosinophil count in peripheral blood and indicators of glucose level in patients with adrenal Cushing syndrome has not been determined.MethodsA retrospective study was undertaken of the clinical and laboratory findings of 40 patients diagnosed with adrenal Cushing syndrome at Chungnam National University Hospital from January 2006 to December 2016. Clinical characteristics, complete blood cell counts with white blood cell differential, measures of their endocrine function, description of imaging studies, and pathologic findings were obtained from their medical records.ResultsEosinophil composition and count were restored by surgical treatment of all of the patients with adrenal Cushing disease. The eosinophil count was inversely correlated with serum and urine cortisol, glycated hemoglobin, and inflammatory markers in the patients with adrenal Cushing syndrome.ConclusionSmaller eosinophil populations in patients with adrenal Cushing syndrome tend to be correlated with higher levels of blood sugar and glycated hemoglobin. This study suggests that peripheral blood eosinophil composition or count may be associated with serum glucose levels in patients with adrenal Cushing syndrome.

  15. Glucocorticoid Regulation of Food-Choice Behavior in Humans: Evidence from Cushing's Syndrome.

    Science.gov (United States)

    Moeller, Scott J; Couto, Lizette; Cohen, Vanessa; Lalazar, Yelena; Makotkine, Iouri; Williams, Nia; Yehuda, Rachel; Goldstein, Rita Z; Geer, Eliza B

    2016-01-01

    The mechanisms by which glucocorticoids regulate food intake and resulting body mass in humans are not well-understood. One potential mechanism could involve modulation of reward processing, but human stress models examining effects of glucocorticoids on behavior contain important confounds. Here, we studied individuals with Cushing's syndrome, a rare endocrine disorder characterized by chronic excess endogenous glucocorticoids. Twenty-three patients with Cushing's syndrome (13 with active disease; 10 with disease in remission) and 15 controls with a comparably high body mass index (BMI) completed two simulated food-choice tasks (one with "explicit" task contingencies and one with "probabilistic" task contingencies), during which they indicated their objective preference for viewing high calorie food images vs. standardized pleasant, unpleasant, and neutral images. All participants also completed measures of food craving, and approximately half of the participants provided 24-h urine samples for assessment of cortisol and cortisone concentrations. Results showed that on the explicit task (but not the probabilistic task), participants with active Cushing's syndrome made fewer food-related choices than participants with Cushing's syndrome in remission, who in turn made fewer food-related choices than overweight controls. Corroborating this group effect, higher urine cortisone was negatively correlated with food-related choice in the subsample of all participants for whom these data were available. On the probabilistic task, despite a lack of group differences, higher food-related choice correlated with higher state and trait food craving in active Cushing's patients. Taken together, relative to overweight controls, Cushing's patients, particularly those with active disease, displayed a reduced vigor of responding for food rewards that was presumably attributable to glucocorticoid abnormalities. Beyond Cushing's, these results may have relevance for elucidating

  16. Cushing Syndrome in a 6-Month-Old Infant due to Adrenocortical Tumor

    Directory of Open Access Journals (Sweden)

    Volmar KeithE

    2009-09-01

    Full Text Available Cushing syndrome is rare in infancy and usually due to an adrenocortical tumor (ACT. We report an infant with Cushing syndrome due to adrenocortical carcinoma. The patient presented at six months of age with a three-month history of growth failure, rapid weight gain, acne, and irritability. Physical examination showed obesity, hypertension, and Cushingoid features. Biochemical evaluation showed very high serum cortisol, mildly elevated testosterone, and suppressed ACTH. Abdominal MRI revealed a heterogeneous right adrenal mass extending into the inferior vena cava. Evaluation for metastases was negative. The tumor was removed surgically en bloc. Pathologic examination demonstrated low mitotic rate, but capsular and vascular invasion. She received no adjuvant therapy. Her linear growth has improved and Cushingoid features resolved. Hormonal markers and quarterly PET scans have been negative for recurrence 24 months postoperatively. In conclusion, adrenocortical neoplasms in children are rare, but should be considered in the differential diagnosis of Cushing syndrome.

  17. Cardiac structure and function in Cushing's syndrome: a cardiac magnetic resonance imaging study.

    Science.gov (United States)

    Kamenický, Peter; Redheuil, Alban; Roux, Charles; Salenave, Sylvie; Kachenoura, Nadjia; Raissouni, Zainab; Macron, Laurent; Guignat, Laurence; Jublanc, Christel; Azarine, Arshid; Brailly, Sylvie; Young, Jacques; Mousseaux, Elie; Chanson, Philippe

    2014-11-01

    Patients with Cushing's syndrome have left ventricular (LV) hypertrophy and dysfunction on echocardiography, but echo-based measurements may have limited accuracy in obese patients. No data are available on right ventricular (RV) and left atrial (LA) size and function in these patients. The objective of the study was to evaluate LV, RV, and LA structure and function in patients with Cushing's syndrome by means of cardiac magnetic resonance, currently the reference modality in assessment of cardiac geometry and function. Eighteen patients with active Cushing's syndrome and 18 volunteers matched for age, sex, and body mass index were studied by cardiac magnetic resonance. The imaging was repeated in the patients 6 months (range 2-12 mo) after the treatment of hypercortisolism. Compared with controls, patients with Cushing's syndrome had lower LV, RV, and LA ejection fractions (P < .001 for all) and increased end-diastolic LV segmental thickness (P < .001). Treatment of hypercortisolism was associated with an improvement in ventricular and atrial systolic performance, as reflected by a 15% increase in the LV ejection fraction (P = .029), a 45% increase in the LA ejection fraction (P < .001), and an 11% increase in the RV ejection fraction (P = NS). After treatment, the LV mass index and end-diastolic LV mass to volume ratio decreased by 17% (P < .001) and 10% (P = .002), respectively. None of the patients had late gadolinium myocardial enhancement. Cushing's syndrome is associated with subclinical biventricular and LA systolic dysfunctions that are reversible after treatment. Despite skeletal muscle atrophy, Cushing's syndrome patients have an increased LV mass, reversible upon correction of hypercortisolism.

  18. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    Directory of Open Access Journals (Sweden)

    Joyce Leary

    2013-01-01

    Full Text Available Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.

  19. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections.

    Science.gov (United States)

    Leary, Joyce; Swislocki, Arthur

    2013-01-01

    Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues.

  20. Concomitant Cushing's syndrome due to adrenal adenoma in a patient with systemic lupus erythematosus.

    Science.gov (United States)

    Shimizu, Masatoshi; Kawata, Masahito; Okada, Toshio; Yuu, Housai; Kurahashi, Toshifumi; Yamanaka, Kunito; Umezu, Keiichi

    2002-11-01

    A 51-year-old woman had been administered 5 mg/day of prednisolone due to systemic lupus erythematosus (SLE). She developed hypertension, hypokalemia and a pathologic pubic fracture during two years before admission. Although iatrogenic Cushing's syndrome was initially suspected, we diagnosed her as concomitant Cushing's syndrome due to a left adrenal tumor. The elevated endogeneous glucocorticoids were evaluated from urinary excretions of 17-hydroxycorticosteroids, which was 2-fold higher than normal and equivalent to 10 mg of prednisolone. After laparoscopic left adrenalectomy, SLE was favorably controlled with 15 mg of prednisolone, the dosage of which was equivalent to the estimated amount of preoperative glucocorticoids.

  1. Ectopic ACTH syndrome complicated by multiple opportunistic infections treated with percutaneous ablation of the adrenal glands.

    Science.gov (United States)

    Chan, Chrystal; Roberts, James Mark

    2017-11-14

    Ectopic adrenocorticotropic hormone (ACTH)-related Cushing's syndrome can lead to multiple complications including severe immunosuppression. If the ACTH-secreting tumour cannot be found, definitive treatment is surgical adrenalectomy, typically followed by glucocorticoid replacement. Here, we present a case of fulminant respiratory failure secondary to coinfection with Pneumocystis jirovecii and cytomegalovirus in a patient with ectopic ACTH-dependent Cushing's syndrome with occult primary. Due to significant deconditioning, she was unable to undergo definitive adrenalectomy and instead underwent percutaneous microwave ablation of the adrenal glands. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  2. Reassessing the reliability of the salivary cortisol assay for the diagnosis of Cushing syndrome.

    Science.gov (United States)

    Zhang, Qian; Dou, Jingtao; Gu, Weijun; Yang, Guoqing; Lu, Juming

    2013-10-01

    The cortisol concentration in saliva is 10-fold lower than total serum cortisol and accurately reflects the serum concentration, both levels being lowest around midnight. The salivary cortisol assay measures free cortisol and is unaffected by confounding factors. This study analysed published data on the sensitivity and specificity of salivary cortisol levels in the diagnosis of Cushing syndrome. Data from studies on the use of different salivary cortisol assay techniques in the diagnosis of Cushing syndrome, published between 1998 and 2012 and retrieved using Ovid MEDLINE®, were analysed for variance and correlation. For the 11 studies analysed, mean sensitivity and specificity of the salivary cortisol assay were both >90%. Repeated measurements were easily made with this assay, enabling improved diagnostic accuracy in comparison with total serum cortisol measurements. This analysis confirms the reliability of the saliva cortisol assay as pragmatic tool for the accurate diagnosis of Cushing syndrome. With many countries reporting a rising prevalence of metabolic syndrome, diabetes and obesity--in which there is often a high circulating cortisol level--salivary cortisol measurement will help distinguish these states from Cushing syndrome.

  3. Zinc alpha-2 glycoprotein is overproduced in Cushing's syndrome.

    Science.gov (United States)

    Escoté, Xavier; Aranda, Gloria B; Mora, Mireia; Casals, Gregori; Enseñat, Joaquim; Vidal, Oscar; Esteban, Yaiza; Halperin, Irene; Hanzu, Felicia A

    2017-01-01

    Cushing syndrome (CS), an endogenous hypercortisolemic condition with increased cardiometabolic morbidity, leads to development of abdominal obesity, insulin resistance, diabetes and proatherogenic dyslipidemia. Zinc alpha-2 glycoprotein (ZAG) is a recently characterized lipolytic adipokine implicated in regulation of adipose tissue metabolism and fat distribution. In vitro and animal studies suggest that glucocorticoids interact with ZAG secretion and action. To assess the relationship between ZAG and glucocorticoids in a human model of hypercortisolism, circulating ZAG levels were tested in patients with CS and its counterpart controls. An observational, cross-sectional study on 39 women, 13 with active CS and 26 controls matched by age and body mass index. Plasma ZAG levels (μg/ml) were measured by ELISA and correlated with hypercortisolism, metabolic, and phenotypic parameters. Plasma ZAG levels were significantly higher in patients with CS compared to controls (64.3±16.6 vs. 44.0±16.1, p=0.002). In a univariate analysis, ZAG levels positively correlated to 24-h urinary free cortisol (p=0.001), body mass index (p=0.02), non-esterified fatty acids (p=0.05), glucose (p=0.003), LDL-C (p=0.028), and type 2 diabetes mellitus (p=0.016), and were inversely related to total adiponectin levels (p=0.035). In a multivariate analysis, after adjusting for CS, ZAG levels only correlated with body mass index (p=0.012), type 2 diabetes mellitus (p=0.004), and glucose (p<0.001). This study provides initial evidence that plasma ZAG levels are higher in patients with CS as compared to controls. The close relationship of ZAG with metabolic and phenotypic changes in CS suggests that ZAG may play a significant role in adipose tissue changes in hypercortisolism. Copyright © 2017 SEEN. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. Iatrogenic Cushing's Syndrome Due to Topical Ocular Glucocorticoid Treatment.

    Science.gov (United States)

    Fukuhara, Daisuke; Takiura, Toshihiko; Keino, Hiroshi; Okada, Annabelle A; Yan, Kunimasa

    2017-02-01

    Iatrogenic Cushing's syndrome (CS) is a severe adverse effect of systemic glucocorticoid (GC) therapy in children, but is extremely rare in the setting of topical ocular GC therapy. In this article, we report the case of a 9-year-old girl suffering from idiopathic uveitis who developed CS due to topical ocular GC treatment. She was referred to the ophthalmology department with a complaint of painful eyes, at which time she was diagnosed with bilateral iridocyclitis and started on a treatment of betamethasone sodium phosphate eye drops. Six months after the initiation of topical ocular GC treatment, she was referred to our pediatric department with stunted growth, truncal obesity, purple skin striate, buffalo hump, and moon face. Because her serum cortisol and plasma adrenocorticotropic hormone levels were undetectable, she was diagnosed with iatrogenic CS. After the doses of topical ocular GC were reduced, the clinical symptoms of CS were improved. The fact that the amount of topical ocular GC with our patient was apparently less than that of similar previous cases tempted us to perform genetic analysis of her NR3C1 gene. We found that our patient had a single heterozygous nucleotide substitution in the 3' untranslated region of the NR3C1 gene, which may explain why she developed CS. However, additional investigations are required to determine if our findings can be extrapolated to other patients. In conclusion, clinicians should be aware that even extremely low doses of topical ocular steroid therapy can cause iatrogenic CS. Copyright © 2017 by the American Academy of Pediatrics.

  5. White matter alterations in the brains of patients with active, remitted, and cured cushing syndrome: a DTI study.

    Science.gov (United States)

    Pires, P; Santos, A; Vives-Gilabert, Y; Webb, S M; Sainz-Ruiz, A; Resmini, E; Crespo, I; de Juan-Delago, M; Gómez-Anson, B

    2015-06-01

    Cushing syndrome appears after chronic exposure to elevated glucocorticoid levels. Cortisol excess may alter white matter microstructure. Our purpose was to study WM changes in patients with Cushing syndrome compared with controls by using DTI and the influence of hypercortisolism. Thirty-five patients with Cushing syndrome and 35 healthy controls, matched for age, education, and sex, were analyzed through DTI (tract-based spatial statistics) for fractional anisotropy, mean diffusivity, axial diffusivity, and radial diffusivity (general linear model, family-wise error, and threshold-free cluster enhancement corrections, P Cushing syndrome with active hypercortisolism, 7 with Cushing syndrome with medication-remitted cortisol, 20 surgically cured, and 35 controls. Cardiovascular risk factors were used as covariates. In addition, correlations were analyzed among DTI values, concomitant 24-hour urinary free cortisol levels, and disease duration. There were widespread alterations (reduced fractional anisotropy, and increased mean diffusivity, axial diffusivity, and radial diffusivity values; P Cushing syndrome compared with controls, independent of the cardiovascular risk factors present. Both active and cured Cushing syndrome subgroups showed similar changes compared with controls. Patients with medically remitted Cushing syndrome also had reduced fractional anisotropy and increased mean diffusivity and radial diffusivity values, compared with controls. No correlations were found between DTI maps and 24-hour urinary free cortisol levels or with disease duration. Diffuse WM alterations in patients with Cushing syndrome suggest underlying loss of WM integrity and demyelination. Once present, they seem to be independent of concomitant hypercortisolism, persisting after remission/cure. © 2015 by American Journal of Neuroradiology.

  6. Diurnal Blood Pressure Variation in Pheochromocytoma, Primary Aldosteronism and Cushing's Syndrome

    Czech Academy of Sciences Publication Activity Database

    Zelinka, T.; Štrauch, B.; Pecen, Ladislav; Widimský jr., J.

    Roc. 18, c. 1 (2004), s. 107-111 ISSN 0950-9240 R&D Projects: GA MŠk LN00B107 Institutional research plan: CEZ:AV0Z1030915 Keywords : primary aldosteronism * pheochromocytoma * Cushing's syndrome * cirardian blood pressure rhythm Subject RIV: BB - Applied Statistics, Operational Research Impact factor: 1.930, year: 2004

  7. Life-threatening Pneumocystis jiroveci pneumonia following treatment of severe Cushing's syndrome

    NARCIS (Netherlands)

    Oosterhuis, J. K.; van den Berg, G.; Monteban-Kooistra, W. E.; Ligtenberg, J. J. M.; Tulleken, J. E.; Zijlstra, J. G.; Meertens, John

    We describe two patients with a severe Cushing's syndrome due to ectopic production of ACTH. Both patients developed a life-threatening Pneumocystis jiroveci pneumonia (PCP) shortly after treatment of the hypercortisolism was started by means of inhibition of production of glucocorticoids and

  8. Fluticasone furoate induced iatrogenic Cushing syndrome in a pediatric patient receiving anti-retroviral therapy.

    Science.gov (United States)

    van den Berg, S A A; van 't Veer, N E; Emmen, J M A; van Beek, R H T

    2017-01-01

    We present a case of iatrogenic Cushing's syndrome, induced by treatment with fluticasone furoate (1-2 dd, 27.5 µg in each nostril) in a pediatric patient treated for congenital HIV. The pediatric patient described in this case report is a young girl of African descent, treated for congenital HIV with a combination therapy of Lopinavir/Ritonavir (1 dd 320/80 mg), Lamivudine (1 dd 160 mg) and Abacavir (1 dd 320 mg). Our pediatric patient presented with typical Cushingoid features (i.e. striae of the upper legs, full moon face, increased body and facial hair) within weeks after starting fluticasone furoate therapy, which was exacerbated after increasing the dose to 2 dd because of complaints of unresolved rhinitis. Biochemical analysis fitted iatrogenic Cushing's syndrome, with a repeatedly low cortisol (iatrogenic Cushing's syndrome in patients treated for HIV due to the strong inhibition of CYP3 enzymes by Ritonavir. Upon discontinuation of fluticasone treatment, the pediatric patient improved both clinically and biochemically with normalisation of cortisol and ACTH within a couple of weeks. Fluticasone therapy may induce iatrogenic Cushing's syndrome in a patient treated with anti-retroviral therapy.Pharmacogenetic analysis, in particular CYP3A genotyping, provides useful information in patients treated for HIV with respect to possible future steroid treatment.Fluticasone furoate is not detected in the Siemens Immulite cortisol binding assay.

  9. A Florid Case of Iatrogenic Cushing's Syndrome Induced by Topical Steroid with Osteoporosis and Hypogonadism.

    Science.gov (United States)

    Sahana, Pranab Kumar; Sarma, Nilendu; Sengupta, Nilanjan; Somani, Prashant Subhash

    2015-01-01

    Here we report a case of a young male who developed full blown iatrogenic Cushing's syndrome after use of superpotent clobetasol propionate cream 0.05% for long duration to suppress psoriatic skin lesions. He also developed osteoporosis and hypogonadism. This case demonstrates that injudicious use of topical steroids can have disastrous consequences.

  10. Le syndrome de cushing chez l'adolescent: à propos de 18 patients ...

    African Journals Online (AJOL)

    adolescent. Elle diffère de la pathologie adulte par le mode de présentation et la prise en charge. Il s'agit d'une étude rétrospective des dossiers de patients suivis pour syndrome de Cushing au service d'endocrinologie de Casablanca entre ...

  11. Reversible brain atrophy and cognitive impairment in an adolescent Japanese patient with primary adrenal Cushing's syndrome.

    Science.gov (United States)

    Ohara, Nobumasa; Suzuki, Hiroshi; Suzuki, Akiko; Kaneko, Masanori; Ishizawa, Masahiro; Furukawa, Kazuo; Abe, Takahiro; Matsubayashi, Yasuhiro; Yamada, Takaho; Hanyu, Osamu; Shimohata, Takayoshi; Sone, Hirohito

    2014-01-01

    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 short duration of Cushing's syndrome probably contributed to the rapid recovery of both cognitive dysfunction and brain atrophy in our patient. Cushing's syndrome should be considered as a possible etiological factor in patients with cognitive impairment and brain atrophy that is atypical for their age.

  12. ECTOPIC CUSHING SYNDROME: A 10-YEAR EXPERIENCE FROM A TERTIARY CARE CENTER IN SOUTHERN INDIA.

    Science.gov (United States)

    Sathyakumar, Samantha; Paul, Thomas Vizhalil; Asha, Hesargatta Shyamsunder; Gnanamuthu, Birla Roy; Paul, M J; Abraham, Deepak Thomas; Rajaratnam, Simon; Thomas, Nihal

    2017-08-01

    Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care center in Southern India. The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features, including radiologic findings and positron emission tomography scans, management, details of follow-up, and outcomes, were documented. We compared the biochemical findings in these patients with 20 consecutive patients with Cushing disease (Cushing syndrome of pituitary origin). A total of 21 patients were studied. The median age at presentation was 34 years (range, 19 to 55 years). Seven patients had thymic carcinoid, 7 had bronchial carcinoid, 3 had lung malignancies, 2 had medullary carcinoma thyroid, 1 patient had a pancreatic neuroendocrine tumor, and 1 patient had an occult source of ACTH. The most common clinical features at presentation were muscle weakness (95%), hyperpigmentation (90%), facial puffiness (76%), easy bruising (61%), edema (57%), and striae (52%). Extensive acne was seen in a large number of patients (43%). Only 3 patients (14%) had central obesity. The median 8 am cortisol was 55.5 μg/dL (range, 3.8 to 131 μg/dL), median 8 am ACTH was 207 pg/mL (range, 31.1 to 703 pg/mL), and the median 24-hour urinary free cortisol was 2,484 μg (range, 248 to 25,438 μg). Basal cortisol and ACTH, as well as midnight cortisol and ACTH level, were markedly higher in patients with ectopic Cushing syndrome as compared to patients with Cushing disease. Twelve of 21 patients had developed life-threatening infections by follow-up. Nine patients had undergone surgical intervention to address the primary tumor. However, only 1 patient exhibited a complete cure on

  13. Gestación y psicosis esteroidea en el curso del síndrome de Cushing Pregnancy and steroid psychosis in the course of Cushing's syndrome

    Directory of Open Access Journals (Sweden)

    Marelys Yanes Quesada

    2011-12-01

    Full Text Available La gestación en el curso del síndrome de Cushing es infrecuente, así como el caso inverso; o sea, que se realice el diagnóstico de síndrome de Cushing durante la gestación. Sin embargo, es posible. Se presenta el caso clínico de una paciente femenina, de 28 años, con un adenoma cortical de suprarrenal derecha productor de cortisol, que en el curso de su enfermedad, resultó embarazada. Se decidió interrupción del embarazo debido al riesgo materno fetal. Durante la evolución del síndrome de Cushing presentó una psicosis esteroidea que requirió tratamiento medicamentoso. Después de realizada la adrenalectomía, se produjo remisión del cuadro clínico y bioquímico. Por tal razón, alertamos a la comunidad médica a insistir en el control preconcepcional de estas pacientes.Pregnancy in the course of Cushing syndrome is not frequent, as well as the contrary case, in other words, that diagnosis of Cushing's syndrome be made during pregnancy. However, it is possible. This is a clinical case of a female patient aged 28 with a cortical adenoma of the right suprarenal gland producing cortisol, which in the course of her disease became pregnant. We decide the termination of pregnancy due to mother-fetus risk. Over the course of Cushing's syndrome there was steroid psychosis requiring drug therapy. After the adrenalectomy threw was a remission of clinical and biochemical picture. Thus, it is necessary to alert the medical community to insist in preconception control of these patients.

  14. Changes in adenosine 5'-monophosphate-activated protein kinase as a mechanism of visceral obesity in Cushing's syndrome.

    Science.gov (United States)

    Kola, Blerina; Christ-Crain, Mirjam; Lolli, Francesca; Arnaldi, Giorgio; Giacchetti, Gilberta; Boscaro, Marco; Grossman, Ashley B; Korbonits, Márta

    2008-12-01

    Features of the metabolic syndrome such as central obesity with insulin resistance and dyslipidemia are typical signs of Cushing's syndrome and common side effects of prolonged glucocorticoid treatment. AMP-activated protein kinase (AMPK), a key regulatory enzyme of lipid and carbohydrate metabolism as well as appetite, is involved in the development of the deleterious metabolic effects of excess glucocorticoids, but no data are available in humans. In the current study, we demonstrate the effect of high glucocorticoid levels on AMPK activity of human adipose tissue samples from patients with Cushing's syndrome. AMPK activity and mRNA expression of genes involved in lipid metabolism were assessed in visceral adipose tissue removed at abdominal surgery of 11 patients with Cushing's syndrome, nine sex-, age-, and weight-matched patients with adrenal incidentalomas, and in visceral adipose tissue from four patients with non-endocrine-related abdominal surgery. The patients with Cushing's syndrome exhibited a 70% lower AMPK activity in visceral adipose tissue as compared with both incidentalomas and control patients (P = 0.007 and P Cushing's syndrome. AMPK activity was inversely correlated with 0900 h serum cortisol and with urinary free cortisol. Our data suggest that glucocorticoids inhibit AMPK activity in adipose tissue, suggesting a novel mechanism to explain the deposition of visceral adipose tissue and the consequent central obesity observed in patients with iatrogenic or endogenous Cushing's syndrome.

  15. Performance of the 4-mg intravenous dexamethasone suppression test in differentiating Cushing disease from pseudo-Cushing syndrome.

    Science.gov (United States)

    Nouvel, Migueline; Rabilloud, Muriel; Raverot, Véronique; Subtil, Fabien; Vouillarmet, Julien; Thivolet, Charles; Jouanneau, Emmanuel; Borson-Chazot, Françoise; Pugeat, Michel; Raverot, Gérald

    2016-02-01

    Discriminating Cushing disease (CD) from pseudo-Cushing syndrome (PCS) is a challenging task that may be overcome with the 4-mg intravenous (IV) dexamethasone suppression test (DST). Assess the performance of the 4-mg IV DST in the differential diagnosis between CD and PCS in well-characterized patients. Retrospective comparative study of subjects seen in a tertiary care unit (November 2008 to July 2011). Thirty-six patients with PCS and 32 patients with CD underwent 4-mg IV dexamethasone infusions from 11 am to 3 pm. Areas Under ROC Curves (AUCs) were estimated and compared for ACTH and cortisol measured at 4 pm the same day (day 1) and 8 am the next day (day 2). The ROC curve of the marker with the highest AUC was used to determine the threshold with the highest specificity for 100% sensitivity. The AUC of ACTH at 8 am on day 2 was estimated at 98.4% (95% CI: [92.1-100]), which is significantly greater than that of ACTH at 4 pm on day 1 (P=0.04) and that of cortisol at 8 am on day 2 (P=0.05). For ACTH at 8 am on day 2, the threshold with the highest specificity for 100% sensitivity was estimated at 14.8 ng/L. At this threshold, the sensitivity was estimated at 100% [89-100] and the specificity at 83.3% [67-94]. The 4-mg IV DST is an easy and accurate tool in distinguishing CD from PCS. It deserves thus a better place in establishing the diagnosis of CD. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  16. Intraocular pressure and its correlation with midnight plasma cortisol level in Cushing's disease and other endogenous Cushing's syndrome

    Directory of Open Access Journals (Sweden)

    Priyadarshini Mishra

    2017-01-01

    Full Text Available Purpose: The purpose of this study is to measure intraocular pressure (IOP and evaluate the correlation between IOP and midnight plasma cortisol (MPC level in patients with Cushing's disease (CD and other endogenous Cushing's syndrome (ECS. Methods: This is a cross-sectional study from a single center including newly diagnosed patients with CD or ECS. All patients underwent detailed ophthalmological evaluation. IOP was measured by Goldmann applanation tonometry in the morning and evening on two consecutive days. MPC value was obtained for each patient. The data were compared using paired and unpaired t-test, Mann–Whitney U-test, and Spearman's rank correlation coefficient. Results: Among 32 patients, 22 were CD (68.75% and 10 patients were other ECS (31.25%. A total of 25 patients (78.12% in our study group had normal IOP (<22 mmHg, and seven patients (21.88% had increased IOP (≥22 mmHg. The percentage of patients with normal IOP was found to be significantly higher compared to percentage of patients with high IOP (P = 0.001 using one-sample Chi-square test. Mean MPC value was 468.6 ± 388.3 nmol/L in patients having IOP ≥22 mmHg and 658.5 ± 584 nmol/L in those with IOP <22 mmHg from both CD and ECS groups, but the difference was not statistically significant. No correlation was found between IOP and MPC (Spearman's rank correlation rho = −0.16 [P = 0.38]. Conclusion: In CD and ECS patients, IOP elevation is an uncommon feature, and high IOP in either group does not correlate with MPC level.

  17. A Case Report of Multiple Endocrine Neoplasia Type IIa Associated with Cushing Syndrome

    Directory of Open Access Journals (Sweden)

    Sh. Borzouei

    2013-10-01

    Full Text Available Introduction: Multiple endocrine neoplasia type IIa (MEN IIa is an autosomal dominant syn-drome characterized bypheochromocytoma ,medullary thyroid carcinoma and hyperparathy-roidism. Pheochromocytoma approximately occurs in 50% of patients with MEN IIa. This tumor has the capacity to produce ACTH ectopically and becomes manifest like Cushing syndrome,although it is very rare. Case Report: We report a 26-year-old woman patient with severe muscle weakness, skin le-sions in extremity, hypertension, new onset diabetes and in the laboratory data hypokalemia, metabolic alkalosis, high serum level of cortisol, metanephrine, normetanephrine, calcitonin and bilateral adrenal mass in computed tomography as the first clinical manifestations of an ACTH-secreting pheochromocytoma. Conclusion: In the patients with hypertension, new onset diabetes and hypokalemia Cushing syndrome and pheochromocytoma should always be ruled out. (Sci J Hamadan Univ Med Sci 2013; 20 (3:260-265

  18. Coexistence of Cushing syndrome from functional adrenal adenoma and Addison disease from immune-mediated adrenalitis.

    Science.gov (United States)

    Colucci, Randall; Jimenez, Rafael E; Farrar, William; Malgor, Ramiro; Kohn, Leonard; Schwartz, Frank L

    2012-06-01

    A 56-year-old woman presented with an incidental adrenal adenoma and physical examination findings that included moderate obesity, a slight cervicothoracic fat pad ("buffalo hump"), increased supraclavicular fat pads, and white abdominal striae. Biochemical workup revealed elevated levels of 24-hour urinary free cortisol but normal serum morning cortisol and suppressed levels of corticotropin, suggestive of adrenal-dependent Cushing syndrome. The resected adrenal gland revealed macronodular cortical hyperplasia with a dominant nodule. Other findings included an absent cortisol response to corticotropin stimulation, presence of serum anti-21-hydroxylase antibodies, and mononuclear cell infiltration--consistent with adrenalitis. The findings represent, to the authors' knowledge, the first known case of a patient with coexistent functional cortisol-secreting macronodular adrenal tumor resulting in Cushing syndrome and immune-mediated adrenalitis resulting in Addison disease.

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

    Directory of Open Access Journals (Sweden)

    Hyeoh Won Yu

    2016-03-01

    Full Text Available 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.

  20. Effects of Glucocorticoids on Bone: What we can Learn from Pediatric Endogenous Cushing's Syndrome.

    Science.gov (United States)

    Tack, L J W; Tatsi, C; Stratakis, C A; Lodish, M B

    2016-11-01

    Chronic exposure to supraphysiologic levels of glucocorticoids (GCs) is associated with impaired bone mineral density, an increase in fracture rates, and, in growing children, compromised linear growth. GCs inhibit bone formation in part by decreasing the number of osteoblasts and by increasing bone resorption by stimulating osteoclasts. While GCs are used to treat many chronic diseases, it is difficult to isolate the effects of the steroids on the bone from the effects of the underlying disease itself. Investigation into the effects of GC exposure on the bone in endogenous Cushing syndrome have contributed to our understanding of bone microarchitecture, growth, healing, and regeneration. We now know that GCs negatively impact bone marrow derived-mesenchymal stromal cells. In children with Cushing syndrome, the potential reversibility of deleterious effects of chronic GC exposure on bone provides insight into the pathophysiology behind pure GC excess. © Georg Thieme Verlag KG Stuttgart · New York.

  1. Case report of a bilateral adrenal myelolipoma associated with Cushing disease.

    Science.gov (United States)

    Park, Se Yoon; Kwak, Mi Kyung; Kim, Hye Jeong; Park, Hyeong Kyu; Suh, Kyo-Il; Yoo, Myung Hi; Jin, So Young; Yun, Sumi; Byun, Dong Won

    2017-12-01

    Adrenal myelolipomas are rare benign tumors, composed of a variable mixture of mature adipose tissue and hematopoietic tissue. These tumors are frequently detected incidentally and are usually asymptomatic, and hormonally inactive. During a routine health checkup, a 52-year-old man was found to have a tumor on the bilateral adrenal glands. Abdominal computed tomography revealed a well-defined, heterogeneously enhanced bilateral adrenal mass, suggesting a myelolipoma. The hormonal evaluation revealed adrenocorticotropic hormone (ACTH) dependent Cushing syndrome. The patient underwent left adrenalectomy, and transsphenoidal resection of a pituitary mass. The final diagnosis was adrenal myelolipoma associated with Cushing disease. Growth of right adrenal myelolipoma was detected during the 7-year follow-up. There were enhancing pituitary lesions in repeat magnetic resonance imaging of the sellar region, which implies persistent or recurrent pituitary adenoma. This case reinforces relationship between Cushing disease and adrenal myelolipoma. To the best of our knowledge, this is the first reported pathologically confirmed bilateral adrenal myelolipoma associated with Cushing disease. This report supports the idea that ACTH is associated with the development of adrenal myelolipoma. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  2. Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient

    DEFF Research Database (Denmark)

    Main, K M; Skov, M; Sillesen, Ida Blok

    2002-01-01

    and 1,600 microg budesonide. The patient experienced symptoms of striae, moon-face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total...... and budesonide inhalation should be monitored regularly for adrenal insufficiency. This may be the first indicator of increased systemic exogenous steroid concentration, before clinical signs of Cushing's syndrome emerge....

  3. Iatrogenic Cushing's syndrome in children presenting at Children's Hospital Lahore using nappy rash ointments.

    Science.gov (United States)

    Sattar, Hina; Manzoor, Jaida; Mirza, Liaqat; Sheikh, Abdul Malik; Butt, Taeed Ahmad

    2015-05-01

    To study the characteristics of infants and children presenting with iatrogenic Cushing's Syndrome due to nappy rash ointments. The descriptive study was conducted at the Children's Hospital, Lahore, from April to September 2013, and comprised patients presenting with cushingoid features and history of using nappy rash ointments. Patients having Cushing's Syndrome due to causes other than iatrogenic were excluded and so were those taking oral or parenteral steroids due to skin allergy, renal or respiratory disease. Demographic data, history and examination of all patients were recorded on a proforma and results were analysed using SPSS 16. Of the total 18 patients, 13(72%) were girls and 5(27%) were boys. Eight (44.4%) patients were younger than 6 months, 6(33.3%) were between 6 months to 1 year, while 4(22.2%) were between 12 and 18 months of age. Clobetasol alone was the most frequently used agent responsible in 13(72%) cases. Duration of use of steroid ointment was as short as 3 weeks to as much as 1 year. All the patients were using disposable diapers. Ointment was prescribed by a doctor in 5(27%) cases and self-prescribed (relative or neighbour) in 13(72%). Self-medication and prolonged use of potent steroid ointments are major contributors in development of iatrogenic Cushing's Syndrome in infants and children. Younger age, female gender and use of disposable diapers were other important predisposing factors.

  4. Cushing syndrome in a young woman due to primary pigmented nodular adrenal disease.

    Science.gov (United States)

    Hackman, Kathryn L; Davis, Anna L; Curnow, Paul A; Serpell, Jonathan W; McLean, Catriona A; Topliss, Duncan J

    2010-01-01

    To report a case of Cushing syndrome due to apparently sporadic primary pigmented nodular adrenal disease in a young woman. We describe the clinical, biochemical, radiologic, and histologic findings of Cushing syndrome due to the rare condition of primary pigmented nodular adrenal disease. A 30-year-old woman presented with a 2-year history of worsening itch without rash over her shoulders and arms and weight gain, particularly around the abdomen and face. Careful questioning did not elicit any history of exogenous glucocorticoid use (systemic or topical), including hydrocortisone. On examination, the patient had a slightly rounded and plethoric face, a small buffalo hump, central adiposity, and thin skin with a few small striae on her inner thighs. No features of the Carney complex were observed. Investigations showed hypercortisolism with suppressed corticotropin and normal adrenal imaging despite documentation of enlarged adrenal glands at removal. High-dose dexamethasone administration was followed by a decrease in urinary free cortisol excretion rather than a paradoxical rise as previously reported in primary pigmented nodular adrenal disease. No mutations were detected in the PRKAR1A gene. Primary pigmented nodular adrenal disease should be suspected in patients with corticotropin-independent Cushing syndrome who have normal adrenal imaging. The role of genetic testing in apparently sporadic cases is not established, but cumulative experience may be helpful in defining the frequency of PRKAR1A mutations.

  5. Residual manifestations of hypercortisolemia following surgical treatment in a patient with Cushing syndrome.

    Science.gov (United States)

    Bartz, Sara K; Karaviti, Lefkothea P; Brandt, Mary L; Lopez, Monica E; Masand, Prakash; Devaraj, Sridevi; Hicks, John; Anderson, Lauren; Lodish, Maya; Keil, Meg; Stratakis, Constantine A

    2015-01-01

    Cushing Syndrome is difficult to diagnose, and the comorbidities and persistent late effects of hypercortisolemia after treatment of the primary disease are challenging for the patient and the endocrinologist. To report the case of a girl with obesity and hypertension, ultimately diagnosed with Cushing syndrome due to primary pigmented nodular adrenocortical disease. In this case, the complications of hypercortisolism persisted short term despite surgical intervention. A 4 year old morbidly obese African-American girl with developmental delay presented with hypertensive emergency in the ER and 18-month history of progressive weight gain. Her previous history included premature adrenarche, hypertension, seizures and a random high cortisol with suppressed ACTH. She was subsequently stabilized, and a diagnostic work-up persistently demonstrated elevated cortisol and suppressed ACTH. An abdominal MRI showed bilateral adrenal multinodular disease, consistent with multinodular hyperplasia of the adrenal glands. Based on these findings the patient underwent a bilateral adrenalectomy, which confirmed primary pigmented nodular adrenocortical disease. The patient had a complicated, protracted post-operative course requiring adjustment of therapy for persistent hypertension. Two months after surgery, she was readmitted to the Emergency Department with hyperpyrexia and hypertension and succumbed to the complications of sepsis. This case highlights the significant diagnostic and therapeutic challenges in treating children with Cushing syndrome. Resolution of the source of hypercortisolemia does not imply regression of hypertension or recovery of the immune system. Although the child underwent bilateral adrenalectomy, persistent consequences of prolonged severe hypercortisolism contributed to her death two months later.

  6. Exogenous Cushing's syndrome due to topical corticosteroid application: case report and review literature.

    Science.gov (United States)

    Tempark, Therdpong; Phatarakijnirund, Voraluk; Chatproedprai, Susheera; Watcharasindhu, Suttipong; Supornsilchai, Vichit; Wananukul, Siriwan

    2010-12-01

    Prolonged use of topical corticosteroids causes systemic adverse effects including Cushing's syndrome and hypothalamic-pituitary-adrenal (HPA) axis suppression, which is less common than that of the oral or parenteral route. At least 43 cases with iatrogenic Cushing syndrome from very potent topical steroid usage (Clobetasol) in children and adult have been published over the last 35 years particularly in developing countries. In children group (n = 22), most are infants with diaper dermatitis and two cases who had started topical application at a very early age and died from severe disseminated CMV infection. For the adult group (n = 21), the most common purpose of steroid use was for treatment of Psoriasis. The recovery period of HPA axis suppression was 3.49 ± 2.92 and 3.84 ± 2.51 months in children and adult, respectively. We report on an 8-month-old female infant who developed Cushing's syndrome and adrenal insufficiency after diaper dermatitis treatment through the misuse of Clobetasol without doctor's prescription. Physiologic dose of hydrocortisone was prescribed to prevent an adrenal crisis for 3 months and discontinued when HPA axis recovery was confirmed by normal morning cortisol and ACTH levels.

  7. Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient.

    Science.gov (United States)

    Main, K M; Skov, M; Sillesen, I B; Dige-Petersen, H; Müller, J; Koch, C; Lanng, S

    2002-01-01

    Treatment of allergic bronchopulmonary aspergillosis with itraconazole is becoming more widespread in chronic lung diseases. A considerable number of patients is concomitantly treated with topical or systemic glucocorticoids for anti-inflammatory effect. As azole compounds inhibit cytochrome P450 enzymes such as CYP3A isoforms, they may compromise the metabolic clearance of glucocorticoids, thereby causing serious adverse effects. A patient with cystic fibrosis is reported who developed iatrogenic Cushing's syndrome after long-term treatment with daily doses of 800 mg itraconazole and 1,600 microg budesonide. The patient experienced symptoms of striae, moon-face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total suppression of spontaneous and stimulated plasma cortisol and adrenocorticotropin. Discontinuation of both drugs led to an improvement in clinical symptoms and recovery of the pituitary-adrenal axis after 3 mo. This observation suggests that the metabolic clearance of buDesonide was compromised by itraconazole's inhibition of cytochrome P450 enzymes, especially the CYP3A isoforms, causing an elevation in systemic budesonide concentration. This provoked a complete suppression of the endogenous adrenal function, as well as iatrogenic Cushing's syndrome. Patients on combination therapy of itraconazole and budesonide inhalation should be monitored regularly for adrenal insufficiency. This may be the first indicator of increased systemic exogenous steroid concentration, before clinical signs of Cushing's syndrome emerge.

  8. Increased Adipocyte Size, Macrophage Infiltration, and Adverse Local Adipokine Profile in Perirenal Fat in Cushing's Syndrome.

    Science.gov (United States)

    Roerink, Sean H P P; Wagenmakers, Margreet A E M; Langenhuijsen, Johan F; Ballak, Dov B; Rooijackers, Hanne M M; d'Ancona, Frank C; van Dielen, François M; Smit, Jan W A; Plantinga, Theo S; Netea-Maier, Romana T; Hermus, Ad R M M

    2017-08-01

    To analyze changes in fat cell size, macrophage infiltration, and local adipose tissue adipokine profiles in different fat depots in patients with active Cushing's syndrome. Subcutaneous (SC) and perirenal (PR) adipose tissue of 10 patients with Cushing's syndrome was compared to adipose tissue of 10 gender-, age-, and BMI-matched controls with regard to adipocyte size determined by digital image analysis on hematoxylin and eosin stainings, macrophage infiltration determined by digital image analysis on CD68 stainings, and adipose tissue leptin and adiponectin levels using fluorescent bead immunoassays and ELISA techniques. Compared to the controls, mean adipocyte size was larger in PR adipose tissue in patients. The percentage of macrophage infiltration of the PR adipose tissue and PR adipose tissue lysate leptin levels were higher and adiponectin levels were lower in SC and PR adipose tissue lysates in patients. The adiponectin levels were also lower in the SC adipose tissue supernatants of patients. Associations were found between the severity of hypercortisolism and PR adipocyte size. Cushing's syndrome is associated with hypertrophy of PR adipocytes and a higher percentage of macrophage infiltration in PR adipose tissue. These changes are associated with an adverse local adipokine profile. © 2017 The Obesity Society.

  9. The diagnostic value of CT scan and selective venous sampling in Cushing's syndrome

    International Nuclear Information System (INIS)

    Negoro, Makoto; Kuwayama, Akio; Yamamoto, Naoto; Nakane, Toshichi; Yokoe, Toshio; Kageyama, Naoki; Ichihara, Kaoru; Ishiguchi, Tsuneo; Sakuma, Sadayuki

    1986-01-01

    We studied 24 patients with Cushing's syndrome in order to find the best way to confirm the pituitary adenoma preoperatively. At first, the sellar content was studied by means of a high-resolution CT scan in each patient. Second, by selective catheterization in the bilateral internal jugular vein and the inferior petrosal sinus, venous samples (c) were obtained for ACTH assay. Simultaneously, peripheral blood sampling (P) was made at the anterior cubital vein for the same purpose, and the C/P ratio was carefully calculated in each patient. If the C/P ratio exceeded 2, it was highly suggestive of the presence of pituitary adenoma. Even by an advanced high-resolution CT scan with a thickness of 2 mm, pituitary adenomas were detected in only 32 % of the patients studied. The result of image diagnosis in Cushing disease was discouraging. As for the chemical diagnosis, the results were as follows. At the early stage of this study, the catheterization was terminated in the jugular veins of nine patients. Among these, in five patients the presence of pituitary adenoma was predicted correctly in the preoperative stage. Later, by means of inferior petrosal sinus samplings, pituitary microadenomas were detected in ten patients among the twelve. Selective venous sampling for ACTH in the inferior petrosal sinus or jugular vein proved to be useful for the differential diagnosis of Cushing's syndrome when other diagnostic measures such as CT scan were inconclusive. (author)

  10. Iatrogenic Cushing syndrome in patients receiving inhaled budesonide and itraconazole or ritonavir: two cases and literature review.

    Science.gov (United States)

    Blondin, Marie-Christine; Beauregard, Hugues; Serri, Omar

    2013-01-01

    To present two cases of iatrogenic Cushing syndrome caused by the interaction of budesonide, an inhaled glucocorticoid, with ritonavir and itraconazole. We present the clinical and biochemical data of two patients in whom diagnosis of Cushing syndrome was caused by this interaction. We also reviewed the pertinent literature and management options. A 71-year-old man was treated with inhaled budesonide for a chronic obstructive pulmonary disease and itraconazole for a pulmonary aspergillosis. The patient rapidly developed a typical Cushing syndrome complicated by bilateral avascular necrosis of the femoral heads. Serum 8:00 AM cortisol concentrations were suppressed at 0.76 and 0.83 μg/dL on two occasions. The patient died 4 days later of a massive myocardial infarction. The second case is a 46-year-old woman who was treated for several years with inhaled budesonide for asthma. She was put on ritonavir, a retroviral protease inhibitor, for the treatment of human immunodeficiency virus (HIV). In the following months, she developed typical signs of Cushing syndrome. Her morning serum cortisol concentration was 1.92 μg/dL. A cosyntropin stimulation test showed values of serum cortisol of Cushing syndrome and secondary adrenal insufficiency due to the association of inhaled corticosteroids with itraconazole or ritonavir.

  11. Combined Ovarian and Adrenal Venous Sampling in the Localization of Adrenocorticotropic Hormone-Independent Ectopic Cushing Syndrome.

    Science.gov (United States)

    Chen, Shi; Li, Ran; Zhang, Xiaobo; Lu, Lin; Li, Ji; Pan, Hui; Zhu, Huijuan

    2018-03-01

    Cushing syndrome is rarely caused by the secretion of cortisol from ovarian tumors. In clinical decision-making, it is important to determine whether the ovarian tumor is capable of secreting cortisol. Selective ovarian and adrenal venous sampling is scarcely reported in the localization of ACTH-independent ectopic Cushing syndrome. We present a case of 40-year-old Chinese woman who had weight gain, hirsutism, hypertension, and menstrual disorder over 6 months. Her physical examination and biochemical assessment revealed adrenocorticotropic hormone-independent Cushing syndrome. Adrenal computed tomography scan indicated no abnormality. A mass of 5.7 cm × 4.2 cm × 3.4 cm was discovered by pelvic ultrasonography. Somatostatin receptor scintigraphy revealed no abnormal radioactivity intake. Combined ovarian and adrenal venous sampling together with a cortisol assay were conducted. Results revealed cortisol concentration of the right-side ovarian vein, left-side ovarian vein, and peripheral vein of 268.60, 29.00, and 35.18 μg/dL, respectively, suggesting a right-side ovarian origin. A right-side salpingo-oophorectomy was performed and the pathological diagnosis revealed ovarian steroid cell tumor, not otherwise specified. The cortisol level was substantially lower after the patient underwent surgery and symptoms of Cushing syndrome disappeared. At 3-year follow-up, the patient remained disease free, and no tumor was observed on pelvic ultrasonogram. Combined ovarian and adrenal venous sampling is valuable in the localization of adrenocorticotropic hormone-independent ectopic Cushing syndrome.

  12. PRKAR1A mutation causing pituitary-dependent Cushing disease in a patient with Carney complex.

    Science.gov (United States)

    Kiefer, Florian W; Winhofer, Yvonne; Iacovazzo, Donato; Korbonits, Márta; Wolfsberger, Stefan; Knosp, Engelbert; Trautinger, Franz; Höftberger, Romana; Krebs, Michael; Luger, Anton; Gessl, Alois

    2017-08-01

    Carney complex (CNC) is an autosomal dominant condition caused, in most cases, by an inactivating mutation of the PRKAR1A gene, which encodes for the type 1 alpha regulatory subunit of protein kinase A. CNC is characterized by the occurrence of endocrine overactivity, myxomas and typical skin manifestations. Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD) is the most frequent endocrine disease observed in CNC. Here, we describe the first case of a patient with CNC and adrenocorticotropic hormone (ACTH)-dependent Cushing disease due to a pituitary corticotroph adenoma. Loss-of-heterozygosity analysis of the pituitary tumour revealed loss of the wild-type copy of PRKAR1A , suggesting a role of this gene in the pituitary adenoma development. PRKAR1A loss-of-function mutations can rarely lead to ACTH-secreting pituitary adenomas in CNC patients. Pituitary-dependent disease should be considered in the differential diagnosis of Cushing syndrome in CNC patients. © 2017 European Society of Endocrinology.

  13. Too little or too much corticosteroid? Coexisting adrenal insufficiency and Cushing's syndrome from chronic, intermittent use of intranasal betamethasone.

    Science.gov (United States)

    Dow, Adrienne; Yu, Run; Carmichael, John

    2013-01-01

    To report the puzzling, rare occurrence of coexisting adrenal insufficiency and Cushing's syndrome from chronic, intermittent use of intranasal betamethasone spray. A 62-year-old male was referred to our endocrinology clinic for management of adrenal insufficiency. This previously healthy individual began to experience chronic sinus symptoms in 2007, was treated with multiple ensuing sinus surgeries, and received oral glucocorticoid for 6 months. In the following 5 years, he suffered severe fatigue and was diagnosed with secondary adrenal insufficiency. He could not be weaned from corticosteroid and developed clear cushingoid features. In our clinic, careful inquiry on medications revealed chronic, intermittent use of high-dose intranasal betamethasone since 2008, which was not apparent to his other treating physicians. His cushingoid features significantly improved after holding intranasal betamethasone. Chronic, intermittent intranasal betamethasone can cause secondary adrenal insufficiency and iatrogenic Cushing's syndrome when used in excess.Topical corticosteroid use should be considered in the differential diagnosis of adrenal insufficiency or Cushing's syndrome.

  14. The Many Faces of Primary Aldosteronism and Cushing Syndrome: A Reflection of Adrenocortical Tumor Heterogeneity.

    Science.gov (United States)

    Mete, Ozgur; Duan, Kai

    2018-01-01

    Adrenal cortical tumors constitute a heterogeneous group of neoplasms with distinct clinical, morphological, and molecular features. Recent discoveries of specific genotype-phenotype correlations in adrenal cortical adenomas have transformed our understanding of their respective endocrine syndromes. Indeed, a proportion of patients with primary aldosteronism are now known to harbor adrenal cortical adenomas with heterogeneous molecular alterations ( KCNJ5, ATP1A1, ATP2B3 , and CACNA1D ) involving the calcium/calmodulin kinase signaling pathway. Several lines of evidence suggest that KCNJ5 -mutant aldosterone-producing adenomas have distinct clinicopathological phenotype compared to those harboring ATP1A1, ATP2B3 , and CACNA1D mutations. Benign adrenal cortical tumors presenting with Cushing syndrome often have diverse mutations ( PRKACA, PRKAR1A, GNAS, PDE11A , and PDE8B ) involving the cyclic AMP signaling pathway. In addition to cortisol-producing adenomas, bilateral micronodular adrenocortical disease and primary bilateral macronodular adrenal hyperplasia (PBMAH) have also expanded the spectrum of benign neoplasms causing adrenal Cushing disease. The recent discovery of inactivating ARMC5 germline mutations in PBMAH has challenged the old belief that this disorder is mainly a sporadic disease. Emerging evidence suggests that PBMAH harbors multiple distinct clonal proliferations, reflecting the heterogeneous genomic landscape of this disease. Although most solitary adrenal cortical tumors are sporadic, there is an increasing recognition that inherited susceptibility syndromes may also play a role in their pathogenesis. This review highlights the molecular and morphological heterogeneity of benign adrenal cortical neoplasms, reflected in the diverse presentations of primary aldosteronism and adrenal Cushing syndrome.

  15. The Many Faces of Primary Aldosteronism and Cushing Syndrome: A Reflection of Adrenocortical Tumor Heterogeneity

    Directory of Open Access Journals (Sweden)

    Ozgur Mete

    2018-03-01

    Full Text Available Adrenal cortical tumors constitute a heterogeneous group of neoplasms with distinct clinical, morphological, and molecular features. Recent discoveries of specific genotype–phenotype correlations in adrenal cortical adenomas have transformed our understanding of their respective endocrine syndromes. Indeed, a proportion of patients with primary aldosteronism are now known to harbor adrenal cortical adenomas with heterogeneous molecular alterations (KCNJ5, ATP1A1, ATP2B3, and CACNA1D involving the calcium/calmodulin kinase signaling pathway. Several lines of evidence suggest that KCNJ5-mutant aldosterone-producing adenomas have distinct clinicopathological phenotype compared to those harboring ATP1A1, ATP2B3, and CACNA1D mutations. Benign adrenal cortical tumors presenting with Cushing syndrome often have diverse mutations (PRKACA, PRKAR1A, GNAS, PDE11A, and PDE8B involving the cyclic AMP signaling pathway. In addition to cortisol-producing adenomas, bilateral micronodular adrenocortical disease and primary bilateral macronodular adrenal hyperplasia (PBMAH have also expanded the spectrum of benign neoplasms causing adrenal Cushing disease. The recent discovery of inactivating ARMC5 germline mutations in PBMAH has challenged the old belief that this disorder is mainly a sporadic disease. Emerging evidence suggests that PBMAH harbors multiple distinct clonal proliferations, reflecting the heterogeneous genomic landscape of this disease. Although most solitary adrenal cortical tumors are sporadic, there is an increasing recognition that inherited susceptibility syndromes may also play a role in their pathogenesis. This review highlights the molecular and morphological heterogeneity of benign adrenal cortical neoplasms, reflected in the diverse presentations of primary aldosteronism and adrenal Cushing syndrome.

  16. Analysis of adrenocortical hyperplasia by computed tomography in patients with Cushing's disease, idiopathic hyperaldosteronism and adrenogenital syndrome

    International Nuclear Information System (INIS)

    Komiya, Ichiro; Ohara, Noriko; Nagasawa, Yoshitaka; Asawa, Takayuki; Hashizume, Kiyoshi; Takasu, Nobuyuki; Yamada, Takashi.

    1994-01-01

    We assessed the usefulness and reliability of computed tomography (CT scan) in evaluating adrenal hyperplasia in 38 patients, including 14 with Cushing's disease, 17 with idiopathic hyperaldosteronism (IHA), and 7 with the adrenogenital syndrome (AGS). Eighty-two normal subjects were also examined. We analyzed the shape of the adrenal gland and quantitated its thickness, width and length. Visual inspection revealed V-shaped right adrenal glands in 100% of patients with Cushing's disease, 94% of patients with IHA, 100% of patients with AGS and in 41% of the normal subjects. Triangular left adrenal glands were observed in 100% of patients with Cushing's disease, 82% of patients with IHA, 67% of patients with AGS and in 12% of the normal subjects. Quantitative analysis showed that the right adrenal gland was significantly thicker and longer in patients with Cushing's disease, IHA and in those with AGS than in normal subjects. The right adrenal gland was significantly wider in the patients with Cushing's disease and AGS than in control subjects. The left adrenal gland was significantly wider and longer in patients with Cushing's disease and AGS than in the normal controls. Analysis of individual data indicated that the upper limit of normal for thickness of the right adrenal was 7 mm. Therefore, adrenal hyperplasia was strongly suggested when the right adrenal gland was more than 7 mm thick. Our findings suggest that the CT scan is useful and reliable in diagnosing adrenal hyperplasia. (author)

  17. Inhaled fluticasone causes iatrogenic cushing's syndrome in patients treated with Ritonavir.

    Science.gov (United States)

    Mahlab-Guri, Keren; Asher, Ilan; Gradstein, Serge; Zung, Amnon; Radian-Sade, Sara; Elbirt, Daniel; Sthoeger, Zev

    2011-10-01

    Ritonavir, a protease inhibitor (PI), is commonly used in the treatment of HIV-1 infection. It is a potent inhibitor of the hepatic cytochrome P450 superfamily. Therefore, its usage with other PI medications leads to significant increases in the levels of the latter PI, which allows a reduction in pill burden. Intranasal and inhaled corticosteroids are widely used for the treatment of allergic rhinitis and asthma. Inhaled steroids do not usually lead to systemic adverse events, since their plasma concentrations are quite low due to extensive first-pass metabolism and clearance by CYP3A4. However, the coadministration of Ritonavir with inhaled (or intranasal) corticosteroids may result in an increase in the plasma corticosteroid levels due to the potent CYP3A4 inhibition by Ritonavir. This may cause Cushing's syndrome (laboratory and clinical) with adrenal suppression. Plasma cortisol and urinary-free cortisol levels were determined using immunoassays. In the Synacthen test, plasma cortisol levels were measured at time 0 as well as at times 60, 120, and 150 minutes following an intramuscular injection of 0.25 mg Synacthen. We present here three HIV-1 female patients aged 12, 55 and 65 years who developed iatrogenic Cushing's syndrome with adrenal suppression following the coadministration of Ritonavir and inhaled Fluticasone, both at the standard recommended doses. The coadministration of Ritonavir and Fluticasone at the recommended doses caused, in our three patients, iatrogenic Cushing's syndrome with adrenal suppression. We suggest that this adverse event is underdiagnosed and high clinical suspicion is needed for early diagnosis and prenention of Addisonian crises. Thus, Fluticasone treatment should be avoided in patients who are treated with Ritonavir. Alternative therapeutic options for asthma control such as oral Montelukast or bronchodilators alone should be considered.

  18. Multiple endocrine adenomatosis with Cushing's disease and the amenorrhea-galactorrhea syndrome responsive to proton beam irradiation

    International Nuclear Information System (INIS)

    Veseley, D.L.; Fass, F.H.

    1981-01-01

    Multiple endocrine adenomatosis (MEA) or neoplasia is a hereditary disorder consisting of tumors of hyperplasia of several endocrine glands. In MEA-1 the pituitary, parathyroids, and pancreatic islets are most frequently involved, while in MEA-2 the thyroid (medullary carcinoma of the thyroid), parathyroids,and adrenals (pheochromocytomas) are the endocrine glands most likely to be involved. Cushings's syndrome may occur in MEA-1 and has also been found in patients with MEA-2, where the cause of Cushing's syndrome is usually ectopic ACTH production from medullary carcinoma of the thyroid. Recently, there have been reports of amenorrhea-galactorrhea syndrome in patients with MEA-1, and confirmation that hyperprolactinemia is associated with this syndrom has been found in patients with MEA-1. The present report details a patient who has been followed up for 20 years since she first presented with amenorrhea and galactorrhea. Ten years after first being seen she was noted to have Cushing's syndrom and hyperparathyroidism due to parathyroid hyperplasia. Both the amenorrhea-galactorrhea syndrome and Cushing's sydrome disappeared with proton beam irradiation to the pituitary

  19. Multiple endocrine adenomatosis with Cushing's disease and the amenorrhea-galactorrhea syndrome responsive to proton beam irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Veseley, D.L.; Fass, F.H.

    1981-09-01

    Multiple endocrine adenomatosis (MEA) or neoplasia is a hereditary disorder consisting of tumors of hyperplasia of several endocrine glands. In MEA-1 the pituitary, parathyroids, and pancreatic islets are most frequently involved, while in MEA-2 the thyroid (medullary carcinoma of the thyroid), parathyroids,and adrenals (pheochromocytomas) are the endocrine glands most likely to be involved. Cushings's syndrome may occur in MEA-1 and has also been found in patients with MEA-2, where the cause of Cushing's syndrome is usually ectopic ACTH production from medullary carcinoma of the thyroid. Recently, there have been reports of amenorrhea-galactorrhea syndrome in patients with MEA-1, and confirmation that hyperprolactinemia is associated with this syndrom has been found in patients with MEA-1. The present report details a patient who has been followed up for 20 years since she first presented with amenorrhea and galactorrhea. Ten years after first being seen she was noted to have Cushing's syndrom and hyperparathyroidism due to parathyroid hyperplasia. Both the amenorrhea-galactorrhea syndrome and Cushing's sydrome disappeared with proton beam irradiation to the pituitary.

  20. Whole body computed tomographic findings of each one case with primary aldosteronism and Cushing syndrome

    International Nuclear Information System (INIS)

    Kamata, Shuji; Kawamura, Koro; Nakamura, Motoyuki

    1980-01-01

    We here report each one case with primary aldosteronism (male, 28 years old) and Cushing syndrome (female, 37 years old). Both of the cases showed characteristic clinical signs of hypertension and typical laboratory findings of adreno-hormonal assays. In performance of whole body computed tomography, clear pictures of tumorous adenomas in both cases were taken and the sizes of adenomas in picture were completely same as the masses obtained by the lateral adrenectomies. As a result, the whole body computed tomography is very useful to diagnose the diseases of adrenal adenoma and hyperplasia. (author)

  1. Iatrogenic Cushing's syndrome related to the interaction between oral budesonide with fluvoxamine: a case report.

    Science.gov (United States)

    Martin, C S; Blaga, C; Lambrescu, I M; Fierbințeanu-Braticevici, C; Fica, S

    2015-08-06

    Budesonide, an oral glucocorticoid indicated for the treatment of Crohn's disease, rarely interferes with the hypothalamic-pituitary-adrenal axis because more than 80% of it is metabolized by cytochrome P450 enzymes. A 33-year-old female patient diagnosed with Crohn's disease, treated with oral budesonide, was admitted for Cushingoid symptoms and signs. The onset coincided with the use of fluvoxamine, a serotonin reuptake inhibitor and also a potent inhibitor of cytochrome P450 enzymes that presumably led to budesonide accumulation. Practitioners should take into consideration the possibility of iatrogenic Cushing's syndrome caused by the association of oral budesonide with a P450 cytochrome inhibitor. © 2015 John Wiley & Sons Ltd.

  2. [Review of online prescriptions of topical steroids for an obese boy with iatrogenic Cushing's syndrome].

    Science.gov (United States)

    Dalby, Sina; Østergaard, Inge

    2017-08-28

    A 14-year-old boy with obesity developed iatrogenic Cushing's syndrome after having received topical steroid therapy for psoriasis. The diagnosis was suspected when he developed striae, moon face and stunted growth. A review of the Danish online registration of prescriptions: Shared Medicine Card, revealed that an amount of 5.1 kg topical steroids had been prescribed during a period of twelve months. Blood tests showed stunted cortisol release. Primary obesity in children is associated with increased growth. Decreased growth warrants further investigations for an underlying condition. This case illustrates the usefulness of Shared Medicine Card, especially when multiple physicians are involved.

  3. Iatrogenic Cushing's Syndrome After Topical Steroid Therapy for Psoriasis

    OpenAIRE

    Sah?p, Birsen; Cel?k, Mehmet; Ayturk, Semra; Kucukarda, Ahmet; Mert, Onur; D?ncer, Nejla; Guld?ken, S?bel; Tugrul, Armagan

    2016-01-01

    Glucocorticoids are used for the treatment of many diseases, such as inflammatory, allergic, autoimmune, and neoplastic diseases. They can be used in the form of topical, oral, inhalable, rectal, and intra-articular agents. Many topical steroid-related iatrogenic Cushing′s syndrome cases affecting especially children have been reported in the literature. Topical steroid-related Cushing′s syndrome is rarely seen in adults. In this report, we present the case of a 32-year-old male patient with ...

  4. Adrenal scintigraphy with 131I-19 iodocholesterol. Interest in Cushing syndrome investigation

    International Nuclear Information System (INIS)

    Vuidard, Claudine.

    1977-01-01

    A review of the physico-chemical properties of 131 I-19-iodocholesterol is followed by a study of its metabolism in both animals and man, giving for the latter the doses delivered to different organs and especially to the gonads. The exploration technique is then described. Scintigraphic examinations are performed on the 3rd day, the 8th day and sometimes the 15th day after injection. However the uptake rate at the adrenal gland depends essentially on the causes of the illness affecting the suprarenal. In Cushing's syndrome the uptake rate is relatively fast and the 1st scintigraphic examination must therefore be carried out earlier (48 hours after injection). The value of isotopic exploration in the etiological evaluation of hypercortisolism is shown to be chiefly morphological, the scintigraphic image serving as a guide to etiological diagnosis. If too adrenal glands are visible this is a sign of bilateral suprerenal hyperplasia. Only one gland clearly visible indicates an adenoma, with functional hypoplasia of the contralateral gland. On the other hand an absence of fixation in the presence of a certain Cushing syndrome would point to malignant cortical carcinoma [fr

  5. Hypertensive crisis in pregnancy due to a metamorphosing pheochromocytoma with postdelivery Cushing's syndrome.

    Science.gov (United States)

    Langton, Katharina; Gruber, Matthias; Masjkur, Jimmy; Steenblock, Charlotte; Peitzsch, Mirko; Meinel, Jörn; Lenders, Jacques; Bornstein, Stefan; Eisenhofer, Graeme

    2018-01-01

    Pheochromocytomas in pregnancy are rare but potentially lethal. Even rarer is the combination of pheochromocytoma in pregnancy with subsequent development of ectopic Cushing's syndrome. We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes. A cesarean section was performed at 32 weeks following a hypertensive crisis after routine administration of betamethasone. Postnatal persistence of signs and symptoms of catecholamine excess led to the diagnosis of a left adrenal pheochromocytoma. Between diagnosis and planned tumor removal, the patient developed signs and symptoms of Cushing's syndrome (facial edema and hirsutism, myopathy and fatigue). Biochemical testing confirmed hypercortisolism with extremely elevated levels of plasma adrenocorticotropin, urinary cortisol and multiple steroids of a plasma panel that were all normal at previous testing. The previously noradrenergic tumor also started producing epinephrine. Histopathological examination confirmed the pheochromocytoma, which was also immunohistochemically positive for adrenocorticotropin. Full post-surgical recovery was sustained with normal blood pressure and biochemical findings after one year. This report not only underlines the chameleon behavior of pheochromocytoma but also illustrates its potential for a metamorphosing presentation. Corticosteroid administration in pregnancy requires a cautious approach in patients with hypertension.

  6. Iatrogenic Cushing's syndrome and osteoporosis due to an interaction between fluticasone and ritonavir.

    Science.gov (United States)

    Azevedo, Luísa; Pêgo, Hugo; Souto Moura, Teresa; Germano, Isabel

    2015-10-29

    The advent of highly active antiretroviral therapy for HIV infection dramatically changed the landscape of the disease. Ritonavir, a protease inhibitor (PI) frequently used in low doses to 'boost' the concentrations of other PIs, inhibits the cytochrome P450 3A4 isoenzyme, a common metabolic pathway to multiple drugs, so the potential for drug interactions is not negligible. A 39-year-old man with HIV-1 infection, treated with a ritonavir-boosted PI, was started on fluticasone/salmeterol inhaler and intranasal fluticasone, in 2009, in the setting of asthma and allergic rhinitis. In 2013, he presented with 1-year evolution of symptoms suggesting Cushing's syndrome, and was experiencing recurrent falls. A spine CT showed a vertical L3 fracture and thoracolumbar erosions; a bone density scan revealed severe osteoporosis. Hormonal assays were compatible with hypothalamic-pituitary-adrenal axis suppression, and iatrogenic Cushing's syndrome due to ritonavir-fluticasone interaction was considered. Fluticasone was suspended and oral corticosteroid replacement initiated, with a favourable outcome. 2015 BMJ Publishing Group Ltd.

  7. Acute and persistent iatrogenic Cushing's syndrome after a single dose of triamcinolone acetonide.

    Science.gov (United States)

    Iglesias, P; González, J; Díez, J J

    2005-12-01

    Iatrogenic Cushing's syndrome is a well-known adverse effect of glucocorticoids. It usually develops after prolonged exposure to excessive amounts of synthetic glucocorticolds. The development of iatrogenic Cushing's syndrome (ICS) after a single and low dose of synthetic glucocorticoid is an exceptional event. Up to now, only a few number of cases have been associated with triamcinolone acetonide and they have always been related to local administration. We report, for the first time, a patient who developed ICS after a single low dose of parenterally (im) administered triamcinolone acetonide. She was a 45-yr-old woman who referred to us because of cushingoid appearance, whose hormonal determinations were suggestive of secondary adrenal insufficiency. Clinical features were developed one month after the administration of a 40 mg single-dose of im triamcinolone acetonide because of acute laryngitis. Endocrinological evaluation confirmed the hypothalamic-pituitary-adrenal (HPA) axis suppression. Eight months later, cushingoid phenotype had completely disappeared and HPA function had spontaneously recovered. We review clinical features and comment on the possible pathogenic mechanisms of this particular and new form of ICS.

  8. Markers of atherosclerosis in patients with Cushing's syndrome: a meta-analysis of literature studies.

    Science.gov (United States)

    Lupoli, Roberta; Ambrosino, Pasquale; Tortora, Anna; Barba, Livia; Lupoli, Gelsy Arianna; Di Minno, Matteo Nicola Dario

    2017-05-01

    Several studies reported an increased cardiovascular (CV) risk in Cushing's syndrome (CS). We performed a meta-analysis on the impact of CS on major markers of atherosclerosis. Studies on intima-media thickness (IMT), carotid plaques prevalence, and flow-mediated dilation (FMD) in CS patients and controls were searched in the PubMed, Web of Science, Scopus, and EMBASE. Differences between cases and controls were expressed as mean difference (MD) with 95% confidence intervals (95%CI) for continuous variables, and as Odds Ratio (OR) with 95%CI for dichotomous variables. Fourteen studies (332 CS, 462 controls) were included. Compared with controls, CS patients showed higher IMT (MD: 0.20 mm; 95% CI: 0.12, 0.28; p atherosclerosis and CV risk. These findings could help establish more specific CV prevention strategies in this clinical setting. Key messages A series of studies reported an increased cardiovascular risk in patients with Cushing's syndrome (CS). In the present meta-analysis we demonstrated that CS is associated with an increased intima-media thickness, higher prevalence of carotid plaques, and lower flow-mediated dilation as compared with controls. These data consistently suggest the need for a strict monitoring of early signs of subclinical atherosclerosis in CS patients.

  9. Evaluation of steroid laboratory tests and adrenal gland imaging with radiocholesterol in the aetiological diagnosis of Cushing's syndrome

    International Nuclear Information System (INIS)

    Barbarino, A.; De Marinis, L.; Liberale, I.; Menini, E.

    1979-01-01

    Basal values of the urinary excretion of 17-oxogenic steroids and serum levels of cortisol were not satisfactory in the differentiation of 'suspected' subjects from patients with true Cushing's syndrome. With a RIA method for serum cortisol determination, the overnight dexamethasone suppression test provided the most reliable single test in establishing adrenocortical hyperfunction. Thirty-five normal subjects, fifty-nine obese patients, thirteen 'suspected' patients and thirteen patients with disease states other than Cushing's syndrome had suppressed values below 4.0 μg/100 ml. None of the ten patients with Cushing's syndrome had a cortisol concentration less than 16.3 μg/100ml. Adrenal gland scintigraphy after radiocholesterol injection is a more valuable tool than the metyrapone test and the high-dose dexamethasone suppression test in the localization and differential diagnosis of adrenocortical lesions causing Cushing's syndrome. It obviates the need for angiographic procedure in the localization of adenomas. It is a reliable technique for identifying functioning adrenal remnants. Therefore a schedule is proposed for studying patients with suspected adrenocortical hyperfunction. (author)

  10. Cushing's syndrome: epidemiology and developments in disease management

    Directory of Open Access Journals (Sweden)

    Sharma ST

    2015-04-01

    Full Text Available Susmeeta T Sharma,1 Lynnette K Nieman,1 Richard A Feelders2 1Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA; 2Division of Endocrinology, Department of Internal Medicine, Erasmus Medical Center, Rotterdam, the Netherlands Abstract: Cushing’s syndrome is a rare disorder resulting from prolonged exposure to excess glucocorticoids. Early diagnosis and treatment of Cushing’s syndrome is associated with a decrease in morbidity and mortality. Clinical presentation can be highly variable, and establishing the diagnosis can often be difficult. Surgery (resection of the pituitary or ectopic source of adrenocorticotropic hormone, or unilateral or bilateral adrenalectomy remains the optimal treatment in all forms of Cushing’s syndrome, but may not always lead to remission. Medical therapy (steroidogenesis inhibitors, agents that decrease adrenocorticotropic hormone levels or glucocorticoid receptor antagonists and pituitary radiotherapy may be needed as an adjunct. A multidisciplinary approach, long-term follow-up, and treatment modalities customized to each individual are essential for optimal control of hypercortisolemia and management of comorbidities. Keywords: Cushing’s syndrome, hypercortisolemia, treatment, epidemiology

  11. VENOUS SAMPLING FOR CUSHING DISEASE: COMPARISON OF INTERNAL JUGULAR VEIN AND INFERIOR PETROSAL SINUS SAMPLING.

    Science.gov (United States)

    Radvany, Martin G; Quinones-Hinojosa, Alfredo; Gallia, Gary L; Wand, Gary S; Salvatori, Roberto

    2016-09-01

    Because magnetic resonance imaging (MRI) fails to detect many adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas, inferior petrosal sinus sampling (IPSS) is considered the gold standard to differentiate Cushing disease (CD) from ectopic ACTH secretion syndrome (EAS). Some authors have suggested internal jugular vein sampling (IJVS) as an alternative to IPSS. We simultaneously compared IJVS to IPSS in 30 consecutive patients referred for ACTH-dependent Cushing syndrome and equivocal MRI exams. Five sites were simultaneously sampled in each patient (right and left IPS, right and left IJV, and femoral vein) before and after the administration of corticotrophin-releasing hormone or desmopressin. The test was considered consistent with CD when the IPS to peripheral ratio was >2 at baseline or >3 after stimulus and the IJV to peripheral ratio was >1.7 at baseline or >2 after stimulus. In 27 of 30 patients, IPSS results were consistent with a central source of ACTH. Two of the other 3 patients had EAS (one lung carcinoid and one occult), and 1 patient had pathology-proven CD. The sensitivity of IPSS was 96.4%. Only 64.2% of these patients had results meeting criteria for a central source of ACTH by IJVS criteria. Twenty patients with centralizing IPPS have undergone pituitary surgery. Of these, the central origin of excessive ACTH was confirmed with certainty in 16 patients. Among these 16 patients, the IPSS sensitivity was 93.8%, whereas 5 patients had false-negative IJVS (68.7% sensitivity). These results do not support the routine use of IJVS in establishing if the pituitary is the source of excessive ACTH. ACTH = adrenocorticotropic hormone CD = Cushing disease CRH = corticotrophin-releasing hormone CS = Cushing syndrome DDAVP = desmopressin EAS = ectopic ACTH secretion IJVS = internal jugular vein sampling IPSS = inferior petrosal sinus sampling JVS = jugular venous sampling MRI = magnetic resonance imaging.

  12. A Challenging Case of an Ectopic Cushing Syndrome

    Directory of Open Access Journals (Sweden)

    Joana Menezes Nunes

    2014-01-01

    Full Text Available Bronchopulmonary carcinoids are rare pulmonary neoplasms although they account for most cases of ectopic ACTH syndromes. When feasible, the mainstay treatment is surgical resection of the tumor. We report the case of a 52-year-old woman with signs and symptoms suggestive of hypercortisolism for 12 months, admitted to our department because of community acquired pneumonia. Blood hormone analysis showed increased levels of ACTH and urinary free cortisol and nonsuppressibility to high- and low-dose dexamethasone tests. Pituitary MRI showed no lesion and no central-to-peripheral ACTH gradient was present in bilateral inferior petrosal sinus sampling. CRH stimulation test suggested an ectopic ACTH source. Thoracic CT scan revealed a nodular region measuring 12 mm located in the inferior lingular lobule of the left superior lung with negative uptake by 18-FDG-PET scan and negative SRS. The patient was successfully treated with an atypical lung resection and histology revealed an atypical bronchial carcinoid tumor with positive ACTH immunoreactivity. This was an interesting case because the patient was admitted due to pneumonia that may have been associated with her untreated and chronic hypercortisolism and a challenging case of ectopic ACTH syndrome due to conflicting results on the diagnostic exams.

  13. Cushing syndrome and the anesthesiologist, two case reports

    Directory of Open Access Journals (Sweden)

    Rudin Domi

    2011-01-01

    Full Text Available Cushing′s syndrome (CS is associated with reduced life quality and increased mortality, mostly due to cardiovascular disease. The features of this syndrome are central obesity, moon facies, facial plethora, supraclavicular fat pads, buffalo hump, and purple striae. Other complications include hyperglycemia, hypertension, proximal muscle weakness, skin thinning, menstrual irregularities, amenorrhea and osteopenia. These make perioperative and anesthetic management difficult and present a challenge to the operating team, especially the anaesthesiologist. In this paper, we present two such cases of CS, which were treated with adrenalectomy. We aim to highlight the special care and precautions that need to be taken while administering anesthesia, and in the post operatory period. Anaesthesia induction in the two cases of CS was done prior to the adrenalectomy procedure and special pre and post operative care was taken. Continuous intra operative monitoring of vitals and checking for the stability of the haemodynamics was performed. With adequate care and using advanced anesthetic techniques, the patients showed uneventful post operative recovery. Though the anesthetic management of patients with CS is difficult, desired results can be achieved with continuous monitoring and special precautions.

  14. Profound amplification of secretory-burst mass and anomalous regularity of ACTH secretory process in patients with Nelson's syndrome compared with Cushing's disease

    NARCIS (Netherlands)

    van Aken, Maarten O.; Pereira, Alberto M.; van den Berg, Gerrit; Romijn, Johannes A.; Veldhuis, Johannes D.; Roelfsema, Ferdinand

    2004-01-01

    As described originally, Nelson's syndrome is characterized by grossly elevated ACTH concentrations, a sellar mass and skin hyperpigmentation emerging in the course of Cushing's disease after bilateral adrenalectomy. No detailed studies have defined whether the mechanisms directing ACTH secretion

  15. PATIENTS' PERCEPTION ON CLINICAL OUTCOME AND QUALITY OF LIFE AFTER A DIAGNOSIS OF CUSHING SYNDROME.

    Science.gov (United States)

    Papoian, Vardan; Biller, Beverly M K; Webb, Susan M; Campbell, Karen K; Hodin, Richard A; Phitayakorn, Roy

    2016-01-01

    Excess cortisol production (Cushing syndrome, CS) is a chronic disease affecting many organ systems and impacting quality of life (QoL). This study analyzed factors associated with self-reported QoL, including aspects related to the diagnosis and treatment modalities of CS. In collaboration with the Cushing's Support and Research Foundation (CSRF), surveys using a validated QoL instrument were sent to CSRF members. Data were analyzed for associations between QoL and demographic, treatment, and disease factors. A total of 269 patients completed the survey. Respondents were 89.9% female, and the mean age was 48 years (SD 12, range 16-76). Respondents visited a median of 4 physicians (range 1-40) prior to the diagnosis of CS, with a median of 5 years (mean 7, SD 5, range 1-30) to obtain a diagnosis, showing a statistically significant negative correlation (Pdisease, highlighting the need for awareness about the disorder. Patients in remission had better QoL, emphasizing the importance of disease control.

  16. Syndroom van Cushing. II. Nieuwe behandelingen

    NARCIS (Netherlands)

    van Aken, M. O.; Feelders, R. A.; van der Lely, A. J.; Romijn, J. A.; Lamberts, S. W. J.; de Herder, W. W.

    2006-01-01

    Several new therapeutic options both medicinal and surgical, have emerged for the treatment of Cushing's syndrome. In Cushing's disease caused by an adrenocorticotropin (ACTH) secreting pituitary adenoma, the introduction ofendoscopic pituitary surgery offers better visualization of the sella than

  17. Cushing's Syndrome in a Morbidly Obese Patient Undergoing Evaluation before Bariatric Surgery

    Directory of Open Access Journals (Sweden)

    Livia Borsoi

    2014-06-01

    Full Text Available Objective: Cushing's syndrome (CS is extremely rare in morbidly obese patients. To date, no occurrences in obese patients with BMI above 60 kg/m2 have been reported in the literature. Case Report: This case report describes a patient who was admitted to the ward of the Clinical Division of Endocrinology and Metabolism of the Medical University of Vienna in preparation for bariatric surgery. The patient was a 49-year-old female who showed morbid obesity (BMI 61.6 kg/m2, hypertension, and substituted hypothyroidism. Preoperative work-up revealed CS due to an adrenal adenoma. Therefore, the patient underwent unilateral adrenalectomy followed by bariatric surgery 6 months later. Conclusion: Since undiagnosed CS might result in severe perioperative complications in a population already at increased risk, this case report underlines the importance of careful endocrine evaluation of morbidly obese patients. After all, even rare endocrine causes should be excluded.

  18. Laparoscopic Bilateral Adrenalectomy in a patient of Cushing syndrome: A Challenge for the Anaesthesiologist

    Directory of Open Access Journals (Sweden)

    Uma K Dahanukar

    2007-01-01

    Full Text Available We present a case of Cushing syndrome who underwent laparoscopic bilateral adrenalectomy and discuss her intraoperative management and postoperative course in ICU, especially pulmonary oedema, that occurred within 3 hours after resection (half life of cortisol is 80-110 minutes. [1] She was diagnosed to have bilateral adrenal hyperplasia with no pituitary involvement on CT scan. Preoperative workup revealed hypokalemia, anaemia, hypertension and hyperglycemia. She was posted for laparoscopic bilateral adrenalectomy. She received general anaesthesia; we did not give epidural analgesia as the patient had fracture of body of L1 vertebrae. Her intra-operative course was uneventful. Post-operative concerns included acute adrenal insufficiency, hypoglycaemia, hypotension and hyperkalemia, which were successfully managed in ICU. Patient was then given oral corticosteroids. One month later she was reassessed and was in better health.

  19. Local Injection of Triamcinolone Acetonide: A Forgotten Aetiology of Cushing's Syndrome.

    Science.gov (United States)

    Sukhumthammarat, Weera; Putthapiban, Prapaipan; Sriphrapradang, Chutintorn

    2017-06-01

    Many different non systemic corticosteroid administrations can cause iatrogenic Cushing's Syndrome (CS). We herein report a case series of iatrogenic CS from keloid scars treatment and aesthetic regimen called mesotherapy. Our first patient developed CS after having exceeded recommended dose of intralesional injection of Triamcinolone Acetonide (TAC). Second case presented with CS followed by unidentified mesotherapy treatment for local fat reduction. Subcutaneous injections of dexamethasone were found to be the part of mesotherapy regimen in one case. Physicians should be insightful in prescribing TAC especially in those patients who have high predisposing factors for developing CS. In the same way, off-label mesotherapy combine with corticosteroid can lead to iatrogenic CS and Hypothalamic-Pituitary-Adrenal (HPA) axis suppression. Currently, there are no standard guidelines for mesotherapy treatment. Therefore, further clinical trials on dosage, duration and effective combination of mesotherapy regimens are needed to increase safety uses.

  20. Iatrogenic Cushing's syndrome and topical steroid therapy: case series and review of the literature.

    Science.gov (United States)

    Decani, Sem; Federighi, Veronica; Baruzzi, Elisa; Sardella, Andrea; Lodi, Giovanni

    2014-12-01

    Topical corticosteroids are considered first-line therapy in patients with chronic inflammatory oral mucosal diseases; among them, clobetasol propionate is one of the most widely used in oral medicine. Under physiological conditions, the transmucosal application is characterized by a significantly greater absorption than the skin application. Contrary to many publications about the side effects of topical corticosteroids in dermatology, few studies have investigated the systemic effects due to local application of these drugs on oral mucosa. Although topical steroid therapy for the management of oral diseases is generally associated with local adverse effects (candidiasis, stomatopyrosis, and hypogeusia), these drugs can also lead to systemic side effects, such as suppression of the hypothalamic-pituitary-adrenal axis and Cushing's syndrome. This review reports five cases of systemic adverse effects caused by clobetasol propionate topical treatment.

  1. Iatrogenic Cushing's Syndrome with Subsequent Adrenal Insufficiency in a Patient with Psoriasis Vulgaris Using Topical Steroids.

    Science.gov (United States)

    Pektas, Suzan Demir; Dogan, Gursoy; Cinar, Nese

    2017-01-01

    Iatrogenic Cushing's syndrome (ICS) is usually related to prolonged and/or high-dose oral or parenteral steroid use. Psoriasis vulgaris (PV) is chronic inflammatory disease and characterized by periods of attack and remission. Topical steroid (TS) is the first choice of treatment for localized and mild PV. The development of systemic side effects of the steroids is usually not observed after TS application. But the risk of developing ICS still exists. In the literature, there are a few adult cases who developed ICS and subsequent adrenal insufficiency associated with TS. In this article, a male patient with PV developing ICS and secondary adrenal insufficiency after treatment of TS for 12 years is presented.

  2. Ritonavir and epidural triamcinolone as a cause of iatrogenic Cushing's syndrome.

    Science.gov (United States)

    Albert, Nicole E; Kazi, Saifullah; Santoro, Jerome; Dougherty, Rebecca

    2012-07-01

    Ritonavir is a protease inhibitor (PI) frequently prescribed with highly active antiretroviral therapy. It functions to boost the effectiveness of other PIs as a result of blocking their breakdown by the cytochrome P450 (3A4) pathway. Through this same mechanism, ritonavir has been shown to cause iatrogenic Cushing's syndrome (ICS) in patients using inhaled fluticasone. In addition, a small number of recent cases suggest that ritonavir may also cause this disorder by prolonging the duration of injected corticosteroids, such as triamcinolone. This case report presents a human immunodeficiency virus (HIV) patient taking ritonavir with ICS and secondary adrenal insufficiency, presumably due to systemic absorption and decreased metabolism of an epidural triamcinolone injection. To the authors knowledge, there have only been 4 previously reported cases describing ritonavir-potentiating ICS after receiving a corticosteroid epidural. This provides further proof that caution should be taken with nonparenteral use of triamcinolone in HIV patients on PIs.

  3. Do patients with type 2 diabetes mellitus have an increased prevalence of Cushing's syndrome?

    DEFF Research Database (Denmark)

    Krarup, Therese; Krarup, Thure; Hagen, Claus

    2012-01-01

    Many clinical features are common for patients with type 2 diabetes mellitus (T2DM) and Cushing's syndrome (CS) such as central obesity, hypertension and dyslipidaemia. Patients with CS often have T2DM. Because T2DM is much more frequent than CS, it is possible that some patients with T2DM have...... increased production of cortisol and thus represent patients with CS. The aim of this review was to evaluate the prevalence of CS in patients with T2DM. A search was performed in PubMed and Medline. We found seven prospective studies, two case-control studies and two cross-sectional studies...... with T2DM varies widely between the different studies, ranging from 0-9.4%. This may be due to patient selection, differences in test methodology (including choice of test), cutoff values and different cortisol assays. The true prevalence of CS in T2DM has not been determined. We need more studies...

  4. Paediatric cyclical Cushing's disease due to corticotroph cell hyperplasia.

    LENUS (Irish Health Repository)

    Noctor, E

    2015-06-01

    Cushing\\'s disease is very rare in the paediatric population. Although uncommon, corticotroph hyperplasia causing Cushing\\'s syndrome has been described in the adult population, but appears to be extremely rare in children. Likewise, cyclical cortisol hypersecretion, while accounting for 15 % of adult cases of Cushing\\'s disease, has only rarely been described in the paediatric population. Here, we describe a very rare case of a 13-year old boy with cyclical cortisol hypersecretion secondary to corticotroph cell hyperplasia.

  5. Relationship of serum lipids to adrenal-gland uptake of 6β-[131I] iodomethyl-19-norcholesterol in Cushing's syndrome

    International Nuclear Information System (INIS)

    Valk, T.W.; Gross, M.D.; Freitas, J.E.; Swanson, D.P.; Schteingart, D.E.; Beierwaltes, W.H.

    1980-01-01

    An alteration in serum cholesterol levels has been suggested as a possible modifier of adrenal uptake of the cholesterol analog, 6β-[ 131 I]iodomethyl-19-norcholesterol (NP-59). To assess the effect of hypercholesterolemia upon NP-59 adrenal uptake, patients with Cushing's syndrome (eight with pituitary-dependent, four with ACTH-independent, and two with ectopic-ACTH syndrome) were selected for retrospective analysis based on the availability of serum cholesterol (n = 14) and triglyceride (n = 10) concentrations obtained at the time of adrenal scintigraphy. A negative correlation (r = -0.78, p < 0.01) was found between NP-59 uptake and serum cholesterol levels in patients with pituitary-dependent Cushing's disease. Compared with pituitary-dependent disease, the ectopic-ACTH syndrome and ACTH-independent states demonstrated equal or greater adrenal uptake of NP-59 at similar serum cholesterol concentrations. Serum triglyceride concentrations did not correlate with total adrenal uptake of NP-59 in any of the patient groups studied. Increased serum cholesterol concentrations are associated with diminished adrenal uptake of NP-59, and in some cases may limit the diagnoic efficacy of adrenal scintigraphy in Cushing's syndrome

  6. In what clinical settings should Cushing´s syndrome be suspected? ¿En qué situaciones clínicas debe sospecharse un síndrome de Cushing?

    Directory of Open Access Journals (Sweden)

    Oscar D. Bruno

    2009-12-01

    Full Text Available Despite its low frequency, endogenous Cushing's syndrome is not an exceptional clinical entity. A growing number of cases are currently derived to specialized centers suggesting an increasing knowledge of the clinical features of hypercortisolism by specialists of diverse branches of clinical medicine. Clinical signs derive from an exaggeration of the physiological actions of cortisol inducing protein breakdown, hyperglycemia, fat mobilization, dyslipidemia, hydrosaline retention, immunosuppression and increased susceptibility to infection. Despite its low specificity, symptoms such as unexplained development of central obesity, mood changes, fatigue, weakness, myopathy, easy bruisability, red striae, arterial hypertension, diabetes and hyperlipidemia, are suggestive of the diagnosis. From an epidemiological point of view, Cushing's syndrome is to be suspected and consequently searched for among patients with uncontrolled high blood pressure or diabetes mellitus, metabolic syndrome, polycystic ovarian syndrome, osteoporosis, depression or adrenal incidentaloma. True Cushing's syndrome has to be differentiated from pseudo syndromes. Most sensitive physical signs for discriminating Cushing's syndrome from pseudo-Cushing states are the presence of supraclavicular fat pads, myopathy, thin skin and easy bruising. The recognition of the clinical manifestations of Cushing's syndrome and of the sub-populations at risk of contracting the disease should be improved through medical education at the medical school and at postgraduate levels. Clinical detection of Cushing's syndrome must be performed mainly by non-endocrinologists, yet its etiological diagnosis and therapeutic management is to be carried out in highly experienced and specialized centers, to ensure the best results in the treatment of this really challenging endocrine disturbance.El síndrome de Cushing no es una entidad clínica excepcional, pese a su baja frecuencia. El creciente número de

  7. Cushing's Syndrome

    Science.gov (United States)

    ... there may be increased growth of facial and body hair, and menstrual periods may become irregular or stop ... there may be increased growth of facial and body hair, and menstrual periods may become irregular or stop ...

  8. Cushing's Syndrome

    Science.gov (United States)

    ... the shoulders) thinner skin that is easily bruised cuts, scratches, and insect bites that take a long time to heal pink or purple stretch marks on your skin (especially the arms, breasts, abdomen ...

  9. Cushing's Syndrome

    Science.gov (United States)

    ... high blood pressure, abnormal levels of cholesterol and triglycerides in the blood, and insulin resistance-also mimics ... which breaks down glucose for energy regulating the metabolism of proteins, carbohydrates, and fats The hypothalamus sends ...

  10. Small cell carcinoma of the prostate presenting with Cushing Syndrome. A narrative review of an uncommon condition.

    Science.gov (United States)

    Rueda-Camino, José Antonio; Losada-Vila, Beatriz; De Ancos-Aracil, Cristina Lucía; Rodríguez-Lajusticia, Laura; Tardío, Juan Carlos; Zapatero-Gaviria, Antonio

    2016-01-01

    Small cell carcinoma (SCC) of the prostate is an uncommon condition; there are very few cases in which presenting symptoms are consistent with Cushing Syndrome (CS). We report a new case in which CS triggers the suspicion of an SCC of the prostate and a review of the published cases of SCC of the prostate presenting with CS. The origin of these neoplasms is still unclear. It may be suspected when laboratory features appear in patients diagnosed with prostatic adenocarcinoma which becomes resistant to specific therapy. SCC usually occurs after the 6th decade. Patients suffering SCC of the prostate presenting with CS usually present symptoms such as hypertension, hyperglycemia, alkalosis or hypokalemia; cushingoid phenotype is less frequent. Cortisol and ACTH levels are often high. Prostatic-specific antigen levels are usually normal. CT scan is the preferred imaging test to localize the lesion, but its performance may be improved by adding other tests, such as FDG-PET scan. All patients have metastatic disease at the time of diagnosis. Lymph nodes, liver and bone are the most frequent metastases sites. Surgery and Ketokonazole are the preferred treatments for CS. The prognosis is very poor: 2- and 5-year survival rates are 27.5 and 14.3%, respectively. Key messages When a patient presents with ectopic Cushing Syndrome but lungs are normal, an atypical localization should be suspected. We should suspect a prostatic origin if Cushing Syndrome is accompanied by obstructive inferior urinary tract symptoms or in the setting of a prostatic adenocarcinoma with rapid clinical and radiological progression with relatively low PSA levels. Although no imaging test is preferred to localize these tumors, FDG-PET-TC can be very useful. Hormone marker scintigraphy (e.g. somatostatin) could be used too. As Cushing Syndrome is a paraneoplastic phenomenon, treatment of the underlying disease may help control hypercortisolism manifestations. These tumors are usually metastatic by the

  11. Coagulation Profile in Patients with Different Etiologies for Cushing Syndrome: A Prospective Observational Study.

    Science.gov (United States)

    Tirosh, Amit; Lodish, Maya; Lyssikatos, Charalampos; Belyavskaya, Elena; Feelders, Richard A; Stratakis, Constantine A

    2017-05-01

    Previous studies reported a higher prevalence of venous-thromboembolic events among patients with Cushing disease (CD) compared to those with ACTH-independent Cushing syndrome (CS) from adrenal sources. The objective of the current study was to evaluate the coagulation profile of patients with CS from different etiologies. A prospective observational study was conducted at a clinical research center. The study included adult patients admitted for evaluation of suspected CS (n=85), that were divided into 3 groups: CD (n=22), ACTH-independent CS from an adrenal tumor/hyperplasia (adrenal CS, n=21), and a control group consisting of subjects with negative screening for CS (rule-out CS, n=42). Coagulation profiles were drawn before and 8.5±4.3 months after surgery (trans-sphenoidal or adrenalectomy, n=18), and included fibrinogen, Factor VIII (FVIII), von Willebrand factor antigen (vWF:Ag), plasminogen activator inhibitor-1 (PAI-1), antithrombin III (ATIII), Protein C (PC), Protein S (PS), α2-antiplasmin (α2AP), and aPTT measurements. Patients with CD had higher baseline mean cortisol levels, ATIII activity and vWF:Ag levels compared with adrenal CS. Differences in ATIII activity and vWF:Ag levels remained even after controlling for BMI, and ATIII after also controlling for 24-h urinary free cortisol collections. Our study showed for the first time the differences in coagulation profiles between various etiologies of CS. We assume that the higher cortisol burden among CD patients may explain the differences found in the coagulation profile as well as the higher risk for VTE compared with primary adrenal CS patients. © Georg Thieme Verlag KG Stuttgart · New York.

  12. A perforated diverticulum in Cushing's disease

    OpenAIRE

    de Havenon, Adam; Ehrenkranz, Joel

    2011-01-01

    We report a case of perforated colonic diverticulum in Cushing's disease. Although perforated diverticuli have been described in patients with Cushing's syndrome secondary to exogenous glucocorticoids, this complication has not been described in patients with Cushing's disease. Patients with hypercortisolism, from either exogenous or endogenous sources, should be monitored for diverticular perforation.

  13. Impacto de la acromegalia y el síndrome de Cushing sobre la salud, una perspectiva vivencial Impact of acromegaly and the Cushing's syndrome, a prospect experience

    Directory of Open Access Journals (Sweden)

    Loraine Ledón Llanes

    2011-08-01

    Full Text Available Objetivo: describir el impacto de la acromegalia y el síndrome de Cushing sobre la salud, desde la perspectiva de personas con estas enfermedades. Métodos: se utilizó un diseño descriptivo, transversal y metodología cualitativa. Participaron 12 mujeres y 8 varones con cambios corporales resultantes de estas enfermedades, atendidos en el Instituto Nacional de Endocrinología, en La Habana, Cuba. Se realizaron entrevistas en profundidad y técnicas psicológicas proyectivas. El material recogido fue transcrito íntegramente, sometido a codificación, interpretación y triangulación. Los aspectos éticos fueron considerados. Resultados: emergieron 5 categorías interconectadas que fundamentaron el impacto de dichas enfermedades sobre la salud: la construcción de sus manifestaciones en términos de síntoma, las trayectorias para acceder a los servicios de salud, la comunicación del diagnóstico, el origen de la enfermedad y su valoración como experiencia de vida. Conclusiones: desde la perspectiva de los sujetos, la acromegalia y el síndrome de Cushing provocaron un impacto significativo de salud como resultado de: sus manifestaciones psíquicas y corporales, las características del proceso de atención y el diagnóstico. El género, las particularidades de la enfermedad y la escasa difusión de información sobre estas enfermedades influyeron sobre las decisiones de salud. El cuerpo se reconstruyó como desvalorizado, y la enfermedad se estructuró como proceso que provocó modificaciones globales en sus vidas.Objective: to describe the impact of acromegaly and the Cushing's syndrome on health, from the prospective of the persons presenting these diseases. Methods: a descriptive, cross-sectional design and qualitative methodology were used. Twelve women and 8 men with body changes caused by these diseases, seen in the National Institute of Endocrinology in La Habana, Cuba, participated in this study. Interviews in depth were applied as

  14. Hypertension and other morbidities with Cushing's syndrome associated with corticosteroids: a review.

    Science.gov (United States)

    Peppa, Melpomeni; Krania, Maria; Raptis, Sotirios A

    2011-01-01

    Corticosteroids constitute an ideal treatment for various inflammatory and autoimmune disorders due to their anti-inflammatory and immunomodulatory actions. However, corticosteroids have a considerable number of side effects, including hypertension, diabetes, lipid disorders, sleep apnea, osteoporosis, myopathy, and disorders of coagulation and fibrinolysis, which are components of Cushing's syndrome (CS). Corticosteroid-induced side effects are dependent on the formulation, route, dose, and time of exposure. However, the underlying pathogenetic mechanisms have not been clearly defined. A large body of evidence supports the role of an imbalance between vasoconstriction and vasodilation with possible links to nitric oxide, prostanoids, angiotensin II, arginine vasopressin, endothelins, catecholamines, neuropeptide Y, and atrial natriuretic peptide. Increased oxidative stress, renin-angiotensin system activation, increased pressor response, metabolic syndrome, and sleep apnea appear to be pathogenetically involved as well. The ideal treatment is the withdrawal of corticosteroids, which is most often impossible due to the exacerbation of the underlying disease. Alternatively, a careful plan, including the proper selection of the formulation, time, and route, should be made, and each side effect should be treated properly. The focus of the research should be to develop synthetic corticosteroids with anti-inflammatory effects but fewer metabolic effects, which so far has been unsuccessful.

  15. Otolaryngic manifestations of Cushing disease.

    Science.gov (United States)

    Kuan, Edward C; Peng, Kevin A; Suh, Jeffrey D; Bergsneider, Marvin; Wang, Marilene B

    2017-08-01

    Cushing disease is a relatively rare cause of Cushing syndrome secondary to a hyperfunctioning pituitary adenoma. In addition to signs and symptoms of hypercortisolism, Cushing disease may present with diverse otolaryngic manifestations, which may guide diagnosis and management. We performed a retrospective chart review of patients who were found to have Cushing disease and who underwent transnasal transsphenoidal surgery for pituitary adenomas between January 1, 2007, and July 1, 2014, at a tertiary academic medical center. There were 37 consecutive patients in this series with Cushing disease caused by a pituitary adenoma. Fifteen (41%) patients complained of visual changes. Five (14%) patients suffered from obstructive sleep apnea. Four (11%) patients had thyroid disease. Other symptoms included hearing loss, vertigo, tinnitus, epistaxis, dysphagia, and salivary gland swelling. Although Cushing disease traditionally presents with classic "Cushingoid" systemic features, it also may present with various otolaryngic manifestations. A thorough workup by otolaryngologists is critical in the comprehensive management of these patients.

  16. Iatrogenic cushing syndrome secondary to a probable interaction between voriconazole and budesonide.

    Science.gov (United States)

    Jones, Whitney; Chastain, Cody A; Wright, Patty W

    2014-07-01

    Oral budesonide is commonly used for the management of Crohn's disease given its high affinity for glucocorticoid receptors and low systemic activity due to extensive first-pass metabolism through hepatic cytochrome P450 (CYP) 3A4. Voriconazole, a second-generation triazole antifungal agent, is both a substrate and potent inhibitor of CYP isoenzymes, specifically CYP2C19, CYP2C9, and CYP3A4; thus, the potential for drug-drug interactions with voriconazole is high. To our knowledge, drug-drug interactions between voriconazole and corticosteroids have not been specifically reported in the literature. We describe a 48-year-old woman who was receiving oral budesonide 9 mg/day for the management of Crohn's disease and was diagnosed with fluconazole-resistant Candida albicans esophagitis; oral voriconazole 200 mg every 12 hours for 3 weeks was prescribed for treatment. Because the patient experienced recurrent symptoms of dysphagia, a second 3-week course of voriconazole therapy was taken. Seven weeks after originally being prescribed voriconazole, she came to her primary care clinic with elevated blood pressure, lower extremity edema, and weight gain; she was prescribed a diuretic and evaluated for renal dysfunction. At a follow-up visit 6 weeks later with her specialty clinic, the patient's blood pressure was elevated, and her physical examination was notable for moon facies, posterior cervical fat pad prominence, and lower extremity pitting edema. Iatrogenic Cushing syndrome due to a drug-drug interaction between voriconazole and budesonide was suspected, and voriconazole was discontinued. Budesonide was continued as previously prescribed for her Crohn's disease. On reevaluation 2 months later, the patient's Cushingoid features had markedly regressed. To our knowledge, this is the first published case report of iatrogenic Cushing syndrome due to a probable interaction between voriconazole and oral budesonide. In patients presenting with Cushingoid features who

  17. Iatrogenic Cushing's syndrome in HIV-infected patients receiving ritonavir and inhaled fluticasone: description of 4 new cases and review of the literature.

    Science.gov (United States)

    Valin, Nadia; De Castro, Nathalie; Garrait, Valerie; Bergeron, Anne; Bouche, Clara; Molina, Jean Michel

    2009-01-01

    Protease inhibitors boosted with ritonavir can lead to drug-drug interactions, particularly with inhaled corticosteroids such as fluticasone, because of the potent inhibition of cytochrome P450-3A4 activity. We report 4 cases of iatrogenic Cushing's syndrome after concomitant administration of inhaled fluticasone and antiretroviral therapy including a protease inhibitor boosted with ritonavir. Although typical manifestations were present, diagnosis of Cushing's syndrome was delayed because the patients were suspected to have antiretroviral therapy-associated lipodystrophy, which shares common clinical features with Cushing's syndrome. Biochemical tests confirmed iatrogenic Cushing's syndrome and clinical symptoms resolved after stopping ritonavir or fluticasone. The differences between the clinical symptoms of Cushing's syndrome and lipodystrophy are discussed as well as their frequency in the cases reported in the literature. The recommendation that concomitant administration of inhaled or intranasal fluticasone and ritonavir be prohibited must be implemented among practitioners who treat HIV-infected patients, and if long-term inhaled steroids are required, other drugs should be preferred.

  18. Decreased prefrontal functional brain response during memory testing in women with Cushing's syndrome in remission.

    Science.gov (United States)

    Ragnarsson, Oskar; Stomby, Andreas; Dahlqvist, Per; Evang, Johan A; Ryberg, Mats; Olsson, Tommy; Bollerslev, Jens; Nyberg, Lars; Johannsson, Gudmundur

    2017-08-01

    Neurocognitive dysfunction is an important feature of Cushing's syndrome (CS). Our hypothesis was that patients with CS in remission have decreased functional brain responses in the prefrontal cortex and hippocampus during memory testing. In this cross-sectional study we included 19 women previously treated for CS and 19 controls matched for age, gender, and education. The median remission time was 7 (IQR 6-10) years. Brain activity was studied with functional magnetic resonance imaging during episodic- and working-memory tasks. The primary regions of interest were the prefrontal cortex and the hippocampus. A voxel-wise comparison of functional brain responses in patients and controls was performed. During episodic-memory encoding, patients displayed lower functional brain responses in the left and right prefrontal gyrus (pmemory retrieval, the patients displayed lower functional brain responses in several brain areas with the most predominant difference in the right prefrontal cortex (pmemory task, patients had lower response in the prefrontal cortices bilaterally (pmemory task compared with a simpler one. In conclusion, women with CS in long-term remission have reduced functional brain responses during episodic and working memory testing. This observation extends previous findings showing long-term adverse effects of severe hypercortisolaemia on brain function. Copyright © 2017 Elsevier Ltd. All rights reserved.

  19. Fluconazole and acetazolamide in the treatment of ectopic Cushing's syndrome with severe metabolic alkalosis.

    Science.gov (United States)

    Schwetz, Verena; Aberer, Felix; Stiegler, Claudia; R Pieber, Thomas; Obermayer-Pietsch, Barbara; Pilz, Stefan

    2015-01-01

    Cushing's syndrome (CS) due to ectopic ACTH production accounts for about 10% of all types of CS and is frequently associated with metabolic alkalosis. Treatment of CS involves surgical resection and/or medical therapy to control hypercortisolism. We present the case of an 80-year-old woman affected by CS due to an unknown cause. The patient had severe metabolic alkalosis with refractory hypokalemia. To treat the underlying CS, fluconazole was initiated due to unavailability of ketoconazole. In spite of markedly decreasing cortisol levels, metabolic alkalosis persisted. Treatment of metabolic alkalosis with acetazolamide was thus initiated and pH levels successfully lowered. This case report shows that hypercortisolism can be effectively treated with fluconazole in cases where ketoconazole is unavailable or not tolerated and that persistent severe metabolic alkalosis caused by glucocorticoid excess can be safely and successfully treated with acetazolamide. Hypercortisolism can be effectively treated with fluconazole where ketoconazole is unavailable or not tolerated.Glucocorticoid excess can cause severe metabolic alkalosis.Persistent severe metabolic alkalosis can be safely and successfully treated with acetazolamide.

  20. Discovery of Cushing's Syndrome After Bariatric Surgery: Multicenter Series of 16 Patients.

    Science.gov (United States)

    Javorsky, Bradley R; Carroll, Ty B; Tritos, Nicholas A; Salvatori, Roberto; Heaney, Anthony P; Fleseriu, Maria; Biller, Beverly M K; Findling, James W

    2015-12-01

    The aim of this study is to demonstrate the importance of considering Cushing's syndrome (CS) as a potential etiology for weight gain and metabolic complications in patients undergoing bariatric surgery (BS). This is a retrospective chart review case series of patients (n = 16) with CS from five tertiary care centers in the USA who had BS. Median age at BS surgery was 35.5 years (median 2.5 years between BS and CS surgery). CS was not identified in 12 patients prior to BS. Four patients had CS surgery prior to BS, without recognition of recurrent or persistent CS until after BS. Median body mass index (BMI) values before BS, nadir after BS, prior to surgery for CS, and after surgery for CS were 47, 31, 38, and 35 kg/m(2), respectively. Prior to BS, 55 % of patients had hypertension and 55 % had diabetes mellitus. Only 17 % had resolution of hypertension or diabetes mellitus after BS. CS may be under-recognized in patients undergoing BS. Testing for CS should be performed prior to BS in patients with features of CS and in post-operative BS patients with persistent hypertension, diabetes mellitus, or excessive weight regain. Studies should be conducted to determine the role of prospective testing for CS in subjects considering BS.

  1. [Primary Neuroendocrine Carcinoma of Thymus Caused Cushing Syndrome: Surgical Treatment and Prognosis Analysis].

    Science.gov (United States)

    Li, Li; Chen, Yeye; Li, Shanqing; Liu, Hongsheng; Huang, Cheng; Qin, Yingzhi

    2015-07-01

    Primary neuroendocrine carcinoma of thymus (pNECT) is a rare thymic neoplasm. Some pNECTs could produce an adrenocorticotropic hormone and cause Cushing syndrome (CS). The aim os this study is to discuss the diagnostic technique and surgical management of pNECT-caused CS and analyze prognosis factors to improve the clinical experience of the disease. The outcome of surgery and follow-up of 14 cases (eight males and six females) of pNECT-caused CS were retrospectively analyzed from November 1987 to June 2013. The median age of the patients was 29, and the median duration of the disease was four months (1 month-44 months). All cases exhibited clinical evidence for the diagnosis of CS, and thoracic computed tomography (CT) was used to detect thymic tumors. Surgical treatment significantly decreased the concentration of both serum cortisol and adrenocorticotropic hormone (Pdisease with aggressive characteristics and unclear prognosis. Early diagnosis and therapy is a challenge for clinicians. Thoracic CT is important for disease location and preoperative evaluation and should be routinely applied to all CS patients to allow early surgery and improved prognosis.

  2. Primary Neuroendocrine Carcinoma of Thymus Caused Cushing Syndrome: 
Surgical Treatment and Prognosis Analysis

    Directory of Open Access Journals (Sweden)

    Li LI

    2015-07-01

    Full Text Available Background and objective Primary neuroendocrine carcinoma of thymus (pNECT is a rare thymic neoplasm. Some pNECTs could produce an adrenocorticotropic hormone and cause Cushing syndrome (CS. The aim os this study is to discuss the diagnostic technique and surgical management of pNECT-caused CS and analyze prognosis factors to improve the clinical experience of the disease. Methods The outcome of surgery and follow-up of 14 cases (eight males and six females of pNECT-caused CS were retrospectively analyzed from November 1987 to June 2013. Result The median age of the patients was 29, and the median duration of the disease was four months (1 month-44 months. All cases exhibited clinical evidence for the diagnosis of CS, and thoracic computed tomography (CT was used to detect thymic tumors. Surgical treatment significantly decreased the concentration of both serum cortisol and adrenocorticotropic hormone (P<0.01 but caused one death in the perioperative period. With multidisciplinary therapy, the median survival was 38 months. Conclusion pNECT-caused CS is a rare disease with aggressive characteristics and unclear prognosis. Early diagnosis and therapy is a challenge for clinicians. Thoracic CT is important for disease location and preoperative evaluation and should be routinely applied to all CS patients to allow early surgery and improved prognosis.

  3. Germline PRKACA amplification leads to Cushing syndrome caused by 3 adrenocortical pathologic phenotypes.

    Science.gov (United States)

    Carney, J Aidan; Lyssikatos, Charalampos; Lodish, Maya B; Stratakis, Constantine A

    2015-01-01

    We describe the pathology of 5 patients with germline PRKACA copy number gain and Cushing syndrome: 4 males and 1 female, aged 2 to 43 years, including a mother and son. Imaging showed normal or slightly enlarged adrenal glands in 4 patients and a unilateral mass in the fifth. Biochemically, the patients had corticotropin-independent hypercortisolism. Four underwent bilateral adrenalectomy; unilateral adrenalectomy was performed in the patient with the adrenal mass. Pathologically, 3 patients, including the 1 with the tumor (adenoma), had primary pigmented nodular adrenocortical disease with extranodular cortical atrophy and mild intracapsular and extracapsular extension of cortical cells. The other 2 patients had cortical hyperplasia and prominent capsular and extracapsular micronodular cortical hyperplasia. Immunoperoxidase staining revealed differences for synaptophysin, inhibin-A, and Ki-67 (nuclei) in the atrophic cortices (patients 1, 2, and 3) and hyperplastic cortices (patients 4 and 5) and for Ki-67 (nuclei) and vimentin in the extracortical nodules in the 2 groups of patients. β-Catenin stained the cell membrane, cytoplasm, and nuclei of the adenoma. The patients were well at follow-up (1-23 years); 24-hour urinary cortisol excretion was elevated in the patient who had unilateral adrenalectomy. Copyright © 2014 Elsevier Inc. All rights reserved.

  4. A rare presentation of subclinical Cushing's syndrome as a pubic fracture.

    Science.gov (United States)

    Arduc, Ayse; Dogan, Bercem Aycicek; Akbaba, Gulhan; Dagdelen, Iffet; Kucukler, Kerim; Isik, Serhat; Ozuguz, Ufuk; Berker, Dilek; Guler, Serdar

    2014-01-01

    Osteoporosis and bone fractures are commonly seen in patients with Cushing's syndrome (CS). Fractures usually occur in the vertebrae and ribs whereas pubic fractures are less common. Similar to obvious hypercortisolemia, subclinical hypercortisolemia can increase the risk of fractures. However, in subclinical cases, bone fractures are very rarely seen as the presenting symptom. We herein report the case of a 62-year-old postmenopausal woman who was presented with a pubic fracture. During the evaluation of the fracture, thoracoabdominal magnetic resonance imaging of the patient demonstrated an adrenal mass. Although the patient did not show any signs of overt hypercortisolism, an endocrinologic evaluation revealed hypercortisolism due to an adrenal tumor. Adrenalectomy was performed, which resulted in a cure of the disease. During the orthopedic follow-up, the patient's pubic area pain gradually improved, and the pubic fracture healed without any accompanying new bone fractures. One year after the surgery, a remarkable improvement was detected in the patient's bone density in spite of the lack of administration of any medications for osteoporosis. Subclinical CS can present as a pubic fracture, and awareness of this relationship can help physicians to diagnose the disease.

  5. Circadian profile of serum melatonin in patients with Cushing's syndrome or acromegaly.

    Science.gov (United States)

    Terzolo, M; Piovesan, A; Alì, A; Codegone, A; Pia, A; Reimondo, G; Torta, M; Paccotti, P; Borretta, G; Angeli, A

    1995-01-01

    We evaluated the 24-h profile of serum melatonin (MT) in 16 patients with Cushing's syndrome (11 pituitary- and 5 adrenal-dependent) aged 38.1 +/- 13.8 years and in 19 patients with acromegaly due to GH-secreting pituitary adenoma aged 52 +/- 11.9 years. Thirty-three healthy subjects aged 30 +/- 7 years and 26 healthy subjects aged 59 +/- 12 years served as age-matched controls for the two groups of patients, respectively. All the patients had active disease and were off treatment; none had overt hypopituitarism. Blood drawings were collected at 08:00, 12:00, 16:00, 20:00, 22:00, 24:00, 02:00, 04:00 and 06:00 h in controls and patients. Serum MT, cortisol, GH and IGF-I were measured by specific RIA kits and ACTH by IRMA. Cosinor rhythmometry was employed for chronobiological analysis. The acromegalic patients displayed, as a whole group, higher 24-h mean MT levels with respect to age-matched controls (M +/- SD: 52.9 +/- 14.6 vs 32.9 +/- 8.0 pg/ml, p vs 50.0 +/- 16.0 pg/ml, NS) without any difference between pituitary- and adrenal-dependent forms. No correlation was found between MT and either ACTH or cortisol levels.(ABSTRACT TRUNCATED AT 250 WORDS)

  6. New diagnostic criteria of adrenal subclinical Cushing's syndrome: opinion from the Japan Endocrine Society.

    Science.gov (United States)

    Yanase, Toshihiko; Oki, Yutaka; Katabami, Takuyuki; Otsuki, Michio; Kageyama, Kazunori; Tanaka, Tomoaki; Kawate, Hisaya; Tanabe, Makito; Doi, Masaru; Akehi, Yuko; Ichijo, Takamasa

    2018-03-23

    New diagnostic criteria and the treatment policy for adrenal subclinical Cushing's syndrome (SCS) are proposed on behalf of the Japan Endocrine Society. The Japanese version has been published, and the essential contents are presented in this English-language version. The current diagnostic criteria for SCS have elicited two main problems: (i) the relatively low reliability of a low range of serum cortisol essential for the diagnosis by an overnight 1-mg dexamethasone suppression test (DST); (ii) different cutoff values for serum cortisol after a 1-mg DST compared with those of other countries. Thus, new criteria are needed. In the new criteria, three hierarchical cortisol cutoff values, 5.0, 3.0 and 1.8 μg/dL, after a 1-mg DST are presented. Serum cortisol ≥5 μg/dL after a 1-mg DST alone is considered sufficient to judge autonomous cortisol secretion for the diagnosis of SCS, and the current criterion based on serum cortisol ≥3 μg/dL after a 1-mg DST can continue to be used. Clinical evidence suggests that serum cortisol ≥1.8-2.9 μg/dL after a 1-mg DST is not always normal, so cases who meet the cutoff value as well as a basal adrenocorticotropic hormone (ACTH) level DST (or are disheartened by treatment-resistant problems) or suspicious cases of adrenal cancer according to tumor imaging.

  7. Iatrogenic Cushing's Syndrome Due to Intranasal Usage of Ophthalmic Dexamethasone: A Case Report.

    Science.gov (United States)

    Orton, Sarah; Censani, Marisa

    2016-05-01

    Iatrogenic Cushing's syndrome (ICS) is caused by exogenous corticosteroid administration with suppression of the hypothalamic-pituitary-adrenal axis. It has been commonly described with oral and topical steroid use, but scarce reports have documented intranasal steroid usage as the etiology in infancy. In this article, we describe a case of a 4-month-old infant who developed ICS after 6 weeks of intranasal dexamethasone ophthalmic solution administration for nasal obstruction. To our knowledge, this is the youngest patient reported with ICS due to intranasal use of a prescribed dose of an ophthalmic steroid. His hypothalamic-pituitary-adrenal axis recovered fully 4.5 months after steroid discontinuation. Because of the small body surface area and supine position during administration, infants are particularly susceptible to ICS. Given that intranasal steroids are commonly prescribed to infants and children for a variety of diagnoses, this case highlights the risks inherent in the use of intranasal steroid drops, particularly in young infants, for both adrenal suppression and linear growth deceleration, even with short-term use. Close monitoring of these patients' height and weight should occur while on steroid treatment, with every effort made to decrease or discontinue steroid use when possible. Copyright © 2016 by the American Academy of Pediatrics.

  8. Cushing's syndrome with adrenal suppression induced by inhaled budesonide due to a ritonavir drug interaction in a woman with HIV infection.

    Science.gov (United States)

    Yoganathan, K; David, L; Williams, C; Jones, K

    2012-07-01

    A 48-year-old woman with HIV infection developed Cushingoid features while she was taking ritonavir-boosted darunavir. Cushing's syndrome was confirmed due to the drug interaction between ritonavir and budesonide. Diagnosis of iatrogenic Cushing's syndrome in HIV-positive patients who are on ritonavir-boosted protease inhibitors (PIs) presents a clinical challenge due to similar clinical features of lipohypertrophy related to ritonavir-boosted PIs. Although this complication has been widely described with the use of inhaled fluticasone, the interaction with inhaled budesonide at therapeutic dose is not widely recognized.

  9. Biosynthesis of Various Steroids in vitro by Isolated Adrenal Cells in Primary Aldosteronism, Cushing's Syndrome, and Adrenogenital Syndrome due to Adrenocortical Adenoma

    OpenAIRE

    MIZUNO, SHIGERU; FUNAHASHI, HIROOMI

    1981-01-01

    To a further understanding of the role of steroid hormones in adrenal disorders, we have prepared free cell system of adrenal cells, using adrenal tissues that had been removed by operation from (i) cases of Cushing's syndrome due to adrenocortical adenoma or adrenocortical hyperplasia, (ii) a case of primary aldosteronism, and (iii) a patient with virilizing adrenal tumor. Twelve important steroid hormones were measured, such as pregnenolone, cortisol and aldosterone, which were produced by ...

  10. Acute development of Cushing syndrome in an HIV-infected child on atazanavir/ritonavir based antiretroviral therapy.

    Science.gov (United States)

    Dubrocq, Gueorgui; Estrada, Andrea; Kelly, Shannon; Rakhmanina, Natella

    2017-01-01

    An 11-year-old male with perinatally acquired human immune deficiency virus (HIV) infection on antiretroviral regimen, which included abacavir plus lamivudine (Epzicom), didanosine, ritonavir and atazanavir presented with bilateral axillary striae, increased appetite, fatigue, facial swelling and acute weight gain. Two months prior to presentation, the patient had received a diagnostic and therapeutic intra-articular triamcinolone injection in the knee for pain relief and subsequently became progressively swollen in the face, developed striae bilaterally at the axillae, experienced increased appetite, fatigue and an 8 pound weight gain. During the endocrine workup, suspicion for adrenal insufficiency prompted 24-h urine collection for free cortisol, which was found to be undetectable (below LLQ of 1.0 µg/L). This prompted further evaluation of the hypothalamic-pituitary axis (HPA) by standard dose adrenocorticotropic hormone (ACTH) stimulation test. A 250 µg cosyntropin stimulation test was performed and confirmed HPA axis suppression. Baseline cortisol level was iatrogenic Cushing syndrome and suppression of HPA axis secondary to the drug interaction between ritonavir (RTV) and intra-articular triamcinolone injection. Following endocrine evaluation and workup, the patient was admitted for planned orthopaedic procedure including elective left hamstring lengthening, distal femoral osteotomy and patellar tendon advancement. Taking into consideration the diagnosis of iatrogenic Cushing syndrome, at the start of the surgical procedure, 100 mg IV stress dose of hydrocortisone followed by 50 mg hydrocortisone every 8 h for 24 h was administered. Stress dosing was discontinued 24 h after the procedure. Throughout the hospitalization and upon discharge, the patient continued his ART. From initial presentation, patient has remained clinically stable throughout surgery and postoperative period. Drug-drug interaction between ritonavir and triamcinolone can

  11. Cushing's Support and Research Foundation

    Science.gov (United States)

    ... high blood pressure, type II diabetes, polycystic ovarian syndrome, endometriosis, obesity, adult acne, metabolic disease, and panic attacks, but not Cushing's. After diagnosis and treatment, my weight is back to normal, and I ...

  12. Adrenal suppression and Cushing's syndrome secondary to an interaction between ritonavir and fluticasone: a review of the literature.

    Science.gov (United States)

    Foisy, M M; Yakiwchuk, E M K; Chiu, I; Singh, A E

    2008-07-01

    The purpose of this article is to provide a systematic overview of the literature on adrenal suppression and Cushing's syndrome secondary to an interaction between inhaled/intranasal fluticasone and ritonavir. The clinical presentation, diagnosis and management will be discussed. A literature search using Medline and EMBASE and a search of abstracts of the three previous years of major HIV-related conferences were carried out. There were 25 cases (15 adult and 10 paediatric) of significant adrenal suppression secondary to an interaction between ritonavir and inhaled fluticasone, and three cases involving ritonavir and intranasal fluticasone. Cases with other steroids were not reported; however, there were cases of adrenal suppression with itraconazole [also a potent cytochrome p (CYP) 3A4 inhibitor] and inhaled budesonide. Clinicians need to differentiate between antiretroviral-induced lipodystrophy syndrome and iatrogenic Cushing's syndrome secondary to glucocorticoid use. Long-term fluticasone and ritonavir should be avoided. If ritonavir is required, another inhaled steroid such as low-dose budesonide or beclomethasone can be used cautiously. Upon discontinuation of inhaled corticosteroids, close monitoring for symptoms of adrenal insufficiency is warranted. The need for steroid replacement therapy at physiological doses should be assessed. The combination of ritonavir and fluticasone should be avoided. Budesonide, beclomethasone, triamcinolone and flunisolide appear to be safer options.

  13. Clinical study on postoperative steroid hormon replacement for preclinical Cushing's syndrome

    International Nuclear Information System (INIS)

    Furuta, Nozomu; Koide, Haruhisa; Sasaki, Hiroshi; Miki, Jun; Kimura, Takahiro; Egawa, Shin

    2009-01-01

    Diagnostic criteria for preclinical Cushing's syndrome (PCS) were reported in 1996. However, requirement of postoperative steroid hormone replacement is still controversial issue. In this study, we observed recent surgical cases retrospectively and evaluate the use of postoperative steroid hormone replacement. Eighteen patients with PCS underwent surgery from 1997 to 2007 in Jikei University Hospital. Thirteen of them received postoperative steroid hormone replacement. We investigated preoperative hormone activity by 131 I-adosterol scintigraphy and suppression of adrenocorticotropic hormone (ACTH) and evaluated the requirement of postoperative steroid hormone replacement. Preoperative serum cortisol was normal range in all patients. Serum ACTH was suppressed in 10 of them (56%). In 131 I-adosterol scintigraphy, accumulation in ipsilateral side was observed in all patients. Accumulation in contralateral side was observed in 13 patients whose serum ACTH had tendency to be suppressed. Mean period of steroid hormone replacement was 19.8 weeks. Patients with lower preoperative ACTH tended to require longer period until withdrawal of steroid hormone replacement. In addition, patients received steroid hormone replacement with higher starting dose significantly required longer period. Three of them had complications during tapering of steroid hormone. Postoperative adrenal insufficiency is important issue as postoperative management of PCS patients whose function of contralateral adrenal or pituitary gland is suppressed. 131 I-adosterol scintigraphy and preoperative serum ACTH were important factors to evaluate the requirement of postoperative steroid hormone replacement. Especially, patients with low preoperative serum ACTH tended to require long duration of postoperative steroid hormone replacement. On the other hand, patients with accumulation of contralateral side in 131 I-adosterol scintigraphy and without suppression of serum ACTH may not require steroid hormone

  14. Ketoconazole therapy: an efficacious alternative to achieve eucortisolism in patients with Cushing&'s syndrome Tratamiento con ketoconazol: Una alternativa eficaz para lograr el eucortisolismo en pacientes con síndrome de Cushing

    Directory of Open Access Journals (Sweden)

    Daniel Moncet

    2007-02-01

    Full Text Available Cushing&'s syndrome (CS is a serious condition requiring drug management in diverse clinical settings. Fifty four patients (44 females, 10 males with CS, aged 14-63, received ketoconazole (KTZ prior to surgery (n= 27, as complementary therapy after surgery and/or radiotherapy (n= 16, or as primary treatment (n= 11. It was given at a 600 (500 - 600 mg/day (median - CI95 maintenance dose for periods ranging from 15 days to 13 years. Clinical signs, hepatic enzymes and urinary free cortisol (UFC were evaluated before and during KTZ treatment. UFC normalised or decreased to subnormal values in 85% of the patients, in 5 to 150 days after starting treatment; although failing to normalise, UFC decreased to 12-48% of pre-treatment values in the remaining patients. Clinical signs improved throughout. Side effects were adrenal insufficiency (18.5%, reversible hepatic toxicity (11%, allergic skin rash (5.5% and gastric intolerance (3.7%; in 11% of patients, an "escape phenomenon" was observed. Twenty-four out of the total (44.4% were treated for prolonged periods, from one up to 13 years. In conclusion, this study confirms that KTZ is an effective and generally well tolerated treatment for CS particularly: a shortly before surgery, b because of persistent hypercortisolism after surgery or awaiting the results of radiotherapy, c as a reasonable option in patients with CS of unknown aetiology and, d as long-term therapy in any case of unsolved hypercortisolism after failure of current treatments.El síndrome de Cushing (SC es un trastorno grave que requiere frecuentemente tratamiento medicamentoso. Cincuenta y cuatro pacientes (44 mujeres, 10 varones de 14-63 años de edad con SC, recibieron ketoconazol (KTZ previo a cirugía (n=27, como complemento luego de cirugía y/o radioterapia (n=16, o como tratamiento primario (n=11. La dosis de mantenimiento fue de 600 (500 - 600 mg/día (mediana-IC95 durante 15 días a 13 años. Los signos clínicos, hepatograma y

  15. Resposta do ACTH plasmático ao estímulo com CRH ou CRH + desmopressina em pacientes com síndrome de Cushing ACTH- dependente submetidos a cateterismo bilateral simultâneo dos seios petrosos inferiores Response of plasmatic ACTH to CRH or combined CRH/desmopressin stimulation in patients with ACTHdependent Cushing's syndrome submitted to simultaneous bilateral petrosal sinuses sampling

    Directory of Open Access Journals (Sweden)

    Daniella Maria Carneiro do Rêgo

    2007-12-01

    Full Text Available OBJETIVO: Comparar a resposta do ACTH plasmático após estímulo com CRH ou CRH + desmopressina em pacientes com síndrome de Cushing ACTH-dependente que realizaram cateterismo bilateral simultâneo dos seios petrosos inferiores. MATERIAIS E MÉTODOS: O procedimento foi realizado em 21 pacientes - 14 mulheres e 7 homens - com síndrome de Cushing ACTH-dependente no período de janeiro de 1998 a dezembro de 2003. Após a cateterização de ambos os seios petrosos, amostras de sangue para ACTH foram colhidas, simultaneamente, nos seios petrosos e veia periférica, tanto no estado basal como após três e cinco minutos da administração de CRH humano (100 mg (6 pacientes ou CRH + desmopressina (100 mg + 10 mg (15 pacientes. RESULTADOS: Aos três minutos, houve aumento percentual do ACTH tanto no grupo CRH (257,77 ± 240,36 no seio petroso direito e 718,78 ± 1.358,82 no seio petroso esquerdo [média ± desvio-padrão] como no grupo CRH + desmopressina (1.263,35 ± 1.842,91 no seio petroso direito [p = 0,06] e 583,93 ± 1.020,03 no seio petroso esquerdo [p = 0,83]. Aos cinco minutos houve declínio percentual do ACTH no grupo do CRH (181,07 ± 217,39 no seio petroso direito e 188,25 ± 270,15 no seio petroso esquerdo e aumento progressivo no grupo do CRH + desmopressina (1.365,29 ± 1.832,31 no seio petroso direito [p = 0,03] e 866,43 ± 1.431,72 no seio petroso esquerdo [p = 0,11]. Nos três pacientes com secreção ectópica não houve gradiente. CONCLUSÃO: A estimulação combinada CRH + desmopressina induziu maior produção de ACTH em adenomas corticotróficos em comparação ao CRH, o que pode melhorar a sensibilidade diagnóstica deste procedimento.OBJECTIVE: To compare the responses of plasmatic ACTH to CRH or combined CRH/desmopressin stimulation in patients with ACTH-dependent Cushing's syndrome submitted to simultaneous, bilateral inferior petrosal sinuses sampling. MATERIALS AND METHODS: The procedure was performed in 21 patients - 14

  16. Aberrant expression of glucagon receptors in adrenal glands of a patient with Cushing's syndrome and ACTH-independent macronodular adrenal hyperplasia

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    Valeria de Miguel

    2010-06-01

    Full Text Available Adrenocorticotropin (ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH is a rare cause of Cushing´s syndrome, characterized by bilateral adrenal lesions and excess cortisol production despite ACTH suppression. Cortisol synthesis is produced in response to abnormal activation of G-protein- coupled receptors, such as gastric inhibitory peptide, vasopressin, beta adrenergic agonists, LH/hCG and serotonin receptors. The aim of this study was to analyze the expression of glucagon receptors in adrenal glands from an AIMAH patient. A patient with ACTH-independent Cushing´s syndrome and bilateral macronodular adrenal hyperplasia was screened for altered activation of adrenal receptors by physiological (mixed meal and pharmacological (gonadotrophin releasing hormone, ACTH and glucagon tests. The results showed abnormally high levels of serum cortisol after stimulation with glucagon. Hypercortisolism was successfully managed with ketoconazole treatment. Interestingly, a 4-month treatment with a somatostatin analogue (octreotide was also able to reduce cortisol secretion. Finally, Cushing's syndrome was cured after bilateral adrenalectomy. Abnormal mRNA expression for glucagon receptor in the patient´s adrenal glands was observed by Real-Time PCR procedure. These results strongly suggest that the mechanism of AIMAH causing Cushing´s syndrome in this case involves the illicit activation of adrenal glucagon receptors. This is the first case reported of AIMAH associated with ectopic glucagon receptors.

  17. Romiplostim therapy as a second-line treatment before splenectomy for refractory immune thrombocytopenia in a cirrhotic patient with iatrogenic Cushing syndrome secondary to corticosteroids.

    Science.gov (United States)

    Casanovas Taltavull, Teresa; Peña-Cala, Maria Carmen

    2017-02-01

    Our case report discusses the usefulness of administering romiplostim as a second-line treatment before splenectomy in a cirrhotic patient with immune thrombocytopenia who developed corticosteroid-induced Cushing's syndrome. Corticosteroids were tapered and consequently withdrawn. The patient made a full recovery postsplenectomy.

  18. Management of Cushing disease.

    Science.gov (United States)

    Tritos, Nicholas A; Biller, Beverly M K; Swearingen, Brooke

    2011-05-01

    Cushing disease is caused by a corticotroph tumor of the pituitary gland. Patients with Cushing disease are usually treated with transsphenoidal surgery, as this approach leads to remission in 70-90% of cases and is associated with low morbidity when performed by experienced pituitary gland surgeons. Nonetheless, among patients in postoperative remission, the risk of recurrence of Cushing disease could reach 20-25% at 10 years after surgery. Patients with persistent or recurrent Cushing disease might, therefore, benefit from a second pituitary operation (which leads to remission in 50-70% of cases), radiation therapy to the pituitary gland or bilateral adrenalectomy. Remission after radiation therapy occurs in ∼85% of patients with Cushing disease after a considerable latency period. Interim medical therapy is generally advisable after patients receive radiation therapy because of the long latency period. Bilateral adrenalectomy might be considered in patients who do not improve following transsphenoidal surgery, particularly patients who are very ill and require rapid control of hypercortisolism, or those wishing to avoid the risk of hypopituitarism associated with radiation therapy. Adrenalectomized patients require lifelong adrenal hormone replacement and are at risk of Nelson syndrome. The development of medical therapies with improved efficacy might influence the management of this challenging condition.

  19. Forkhead box O3 plays a role in skeletal muscle atrophy through expression of E3 ubiquitin ligases MuRF-1 and atrogin-1 in Cushing's syndrome.

    Science.gov (United States)

    Kang, Seol-Hee; Lee, Hae-Ahm; Kim, Mina; Lee, Eunjo; Sohn, Uy Dong; Kim, Inkyeom

    2017-06-01

    Cushing's syndrome is caused by overproduction of the adrenocorticotropic hormone (ACTH), which stimulates the adrenal grand to make cortisol. Skeletal muscle wasting occurs in pathophysiological response to Cushing's syndrome. The forkhead box (FOX) protein family has been implicated as a key regulator of muscle loss under conditions such as diabetes and sepsis. However, the mechanistic role of the FOXO family in ACTH-induced muscle atrophy is not understood. We hypothesized that FOXO3a plays a role in muscle atrophy through expression of the E3 ubiquitin ligases, muscle RING finger protein-1 (MuRF-1), and atrogin-1 in Cushing's syndrome. For establishment of a Cushing's syndrome animal model, Sprague-Dawley rats were implanted with osmotic minipumps containing ACTH (40 ng·kg -1 ·day -1 ). ACTH infusion significantly reduced muscle weight. In ACTH-infused rats, MuRF-1, atrogin-1, and FOXO3a were upregulated and the FOXO3a promoter was targeted by the glucocorticoid receptor (GR). Transcriptional activity and expression of FOXO3a were significantly decreased by the GR antagonist RU486. Treatment with RU486 reduced MuRF-1 and atrogin-1 expression in accordance with reduced enrichment of FOXO3a and Pol II on the promoters. Knockdown of FOXO3a prevented dexamethasone-induced MuRF-1 and atrogin-1 expression. These results indicate that FOXO3a plays a role in muscle atrophy through expression of MuRF-1 and atrogin-1 in Cushing's syndrome. Copyright © 2017 the American Physiological Society.

  20. Dyslipidemia and chronic inflammation markers are correlated with telomere length shortening in Cushing's syndrome.

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    Anna Aulinas

    Full Text Available Cushing's syndrome (CS increases cardiovascular risk (CVR and adipocytokine imbalance, associated with an increased inflammatory state. Telomere length (TL shortening is a novel CVR marker, associated with inflammation biomarkers. We hypothesized that inflammatory state and higher CVR in CS might be related to TL shortening, as observed in premature aging.To evaluate relationships between TL, CVR and inflammation markers in CS.In a cross-sectional study, 77 patients with CS (14 males, 59 pituitary-, 17 adrenal- and 1 ectopic-origin; 21 active disease and 77 age-, gender-, smoking-matched controls were included. Total white blood cell TL was measured by TRF-Southern technique. Clinical data and blood samples were collected (lipids, adrenal function, glucose. Adiponectin, interleukin-6 (IL6 and C-reactive protein (CRP were available in a subgroup of patients (n=32. Correlations between TL and clinical features were examined and multiple linear regression analysis was performed to investigate potential predictors of TL.Dyslipidemic CS had shorter TL than non-dyslipidemic subjects (7328±1274 vs 7957±1137 bp, p<0.05. After adjustment for age and body mass index, cured and active CS dyslipidemic patients had shorter TL than non-dyslipidemic CS (cured: 7187±1309 vs 7868±1104; active: 7203±1262 vs 8615±1056, respectively, p<0.05. Total cholesterol and triglycerides negatively correlated with TL (r-0.279 and -0.259, respectively, p<0.05, as well as CRP and IL6 (r-0.412 and -0.441, respectively, p<0.05. No difference in TL according the presence of other individual CVR factors (hypertension, diabetes mellitus, obesity were observed in CS or in the control group. Additional TL shortening was observed in dyslipidemic obese patients who were also hypertensive, compared to those with two or less CVR factors (6956±1280 vs 7860±1180, respectively, p<0.001. Age and dyslipidemia were independent negative predictors of TL.TL is shortened in dyslipidemic CS

  1. Cushing’s Syndrome with Concurrent Diabetes Mellitus in a Rhesus Monkey

    Science.gov (United States)

    1999-05-01

    adverse effects ( 10). Feldman et al. evaluated ketoconazole in over 50 dogs with spontaneous hyperadrenocorticism due to Cushing’s disease and...reports from humans and dogs . Moreover, there are no reports of experimentally induced animals models of both diseases con- currently. Recently, a...most useful as a monitoring aid to assess antecedent glycemic control (4, 5). Other rule-outs on the differential diagnosis list were

  2. Iatrogenic Cushing's syndrome due to drug interaction between glucocorticoids and the ritonavir or cobicistat containing HIV therapies.

    Science.gov (United States)

    Elliot, Emilie R; Theodoraki, Aikaterini; Jain, Lakshmi R; Marshall, Neal J; Boffito, Marta; Baldeweg, Stephanie E; Waters, Laura J

    2016-10-01

    Ritonavir and cobicistat, used as pharmacokinetic enhancers in combination with some antiretrovirals (ARVs) for the treatment of HIV, are potent inhibitors of the CYP3A4 isoenzyme. Most glucocorticoids are metabolised via the CYP3A4 pathway and iatrogenic Cushing's syndrome (ICS), with possible secondary adrenal insufficiency (SAI), is a recognised complication following co-administration with ritonavir or cobicistat. A structured approach for identifying and managing potentially affected individuals has not been established.We systematically identified patients with ICS/SAI and found substantial heterogeneity in clinical practice across three large London HIV centres. While this significant drug interaction and its complications are now well-recognised, it is apparent that there is no standardised approach to management or guidance for the general physician. Here we describe the management of ICS/SAI in our current practice, review the available evidence and suggest practice recommendations. © Royal College of Physicians 2016. All rights reserved.

  3. Clinical features, diagnosis, treatment and molecular studies in paediatric Cushing's syndrome due to primary nodular adrenocortical hyperplasia

    DEFF Research Database (Denmark)

    Storr, Helen L; Mitchell, J H; Swords, F M

    2004-01-01

    BACKGROUND: Primary nodular adrenocortical hyperplasia (PNAH) is a well recognized, but infrequently studied cause of paediatric Cushing's syndrome (CS). OBJECTIVE: To assess presentation, diagnosis, radiological imaging, treatment and molecular analysis of patients with childhood-onset CS due...... to PNAH. PATIENTS: Four males and two females (median age 12.9 years, range 10.9-16.9 years) were studied. RESULTS: All had growth failure (mean height SDS -1.2; range -2.5-0.0), weight gain [mean body mass index (BMI) SDS 3.5; range 2.5-4.6] and clinical virilization, while five had hypertension [mean...... systolic blood pressure (SBP) 130 mmHg, diastolic blood pressure (DBP) 83 mmHg]. One patient had generalized lentigines, one had a tibial chondromyxomatous cyst and two had facial freckling. One patient had a family history of primary nodular adrenocortical disease. The diagnosis of CS was based...

  4. Acute development of Cushing syndrome in an HIV-infected child on atazanavir/ritonavir based antiretroviral therapy

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    Gueorgui Dubrocq

    2017-10-01

    Full Text Available An 11-year-old male with perinatally acquired human immune deficiency virus (HIV infection on antiretroviral regimen, which included abacavir plus lamivudine (Epzicom, didanosine, ritonavir and atazanavir presented with bilateral axillary striae, increased appetite, fatigue, facial swelling and acute weight gain. Two months prior to presentation, the patient had received a diagnostic and therapeutic intra-articular triamcinolone injection in the knee for pain relief and subsequently became progressively swollen in the face, developed striae bilaterally at the axillae, experienced increased appetite, fatigue and an 8 pound weight gain. During the endocrine workup, suspicion for adrenal insufficiency prompted 24-h urine collection for free cortisol, which was found to be undetectable (below LLQ of 1.0 μg/L. This prompted further evaluation of the hypothalamic–pituitary axis (HPA by standard dose adrenocorticotropic hormone (ACTH stimulation test. A 250 μg cosyntropin stimulation test was performed and confirmed HPA axis suppression. Baseline cortisol level was <1 μg/dL and stimulated cortisol level at 30 min was 3.8 μg/dL. The patient was diagnosed with iatrogenic Cushing syndrome and suppression of HPA axis secondary to the drug interaction between ritonavir (RTV and intra-articular triamcinolone injection. Following endocrine evaluation and workup, the patient was admitted for planned orthopaedic procedure including elective left hamstring lengthening, distal femoral osteotomy and patellar tendon advancement. Taking into consideration the diagnosis of iatrogenic Cushing syndrome, at the start of the surgical procedure, 100 mg IV stress dose of hydrocortisone followed by 50 mg hydrocortisone every 8 h for 24 h was administered. Stress dosing was discontinued 24 h after the procedure. Throughout the hospitalization and upon discharge, the patient continued his ART. From initial presentation, patient has remained clinically stable throughout

  5. Iatrogenic Cushing's syndrome due to coadministration of ritonavir and inhaled budesonide in an asthmatic human immunodeficiency virus infected patient.

    Science.gov (United States)

    Kedem, Eynat; Shahar, Eduardo; Hassoun, Gamal; Pollack, Shimon

    2010-09-01

    Iatrogenic Cushing's syndrome (CS) is caused by exposure to glucocorticoids and may be promoted by interaction with additional drugs. It is well known in asthmatic human immunodeficiency virus (HIV)-infected patients treated with inhaled fluticasone with ritonavir-containing antiretroviral regimen (cART). The authors present an asthmatic HIV-infected Ethiopian woman, treated with fluticasone/salmeterol, commencing cART with tenofovir, emtricitabine, and lopinavir/ritonavir. During 7 months she gained 9 kg and hyperpigmentation, mild edema, marked abdominal striae, and increase in blood pressure were noted. Plasma am and urine free cortisol levels confirmed CS diagnosis and fluticasone was discontinued. Complete resolution of CS occurred within 2 months. However, frequent asthma symptoms required resumption of inhaled corticosteroid (ICS) treatment, and budesonide/formeterol was prescribed. Soon reemergence of symptomatic CS was noted. Ritonavir dose was halved, but CS symptoms continued to develop. Budesonide was stopped and montelukast initiated. Resolution of cushingoid symptoms was observed within weeks. Corticosteroids are metabolized by cytochrome P450 3A4 (CYP3A4). Fluticasone has the longest glucocorticoid receptor-binding half-life and is 300 times more lipophilic than budesonide. Inhaled fluticasone possesses a high suppression rate of hypothalamic-pituitary-adrenal axis. Ritonavir, a potent CYP3A4 inhibitor, may inhibit corticosteroid degradation and increase its accumulation. Inhaled budesonide is less likely to cause adrenal suppression. Diagnosing Cushing's syndrome presents a clinical challenge due to similarities with clinical manifestations and side effects related to cART. In patients treated with inhaled or intranasal corticosteroids together with cART there may be a higher incidence of iatrogenic CS. CS should be looked for, and management considered carefully.

  6. Massive neonatal adrenal enlargement due to cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex: findings in Cushing syndrome associated with hemihypertrophy.

    Science.gov (United States)

    Carney, J Aidan; Ho, Josephine; Kitsuda, Kazuteru; Young, William F; Stratakis, Constantine A

    2012-10-01

    Described in this article is the massive enlargement of both adrenal glands in 3 newborns-2 girls and 1 boy. Two had hemihypertrophy and other congenital abnormalities but no identified genetic mutation; the third had genetically proven Beckwith-Wiedemann syndrome. Two had severe Cushing syndrome, the third had hypercortisolemia but no clinical Cushing syndrome. Bilateral adrenalectomy cured Cushing syndrome in the 2 with severe symptoms; total adrenal weight in these patients was 44 and 53 g, respectively. Unilateral adrenalectomy was performed in the third patient: the gland weighed 52 g; postoperatively, the patient's hypercortisolemia normalized, and, concomitantly, the enlarged contralateral adrenal gland had a 5-fold decrease in size with slight enlargement 6 years postoperatively. Microscopically, the 3 patients had similar pathology: massive adrenal enlargement due to a combination of cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex. The pathologic findings were most likely the result of the genetic mutation identified in 1 patient and of an unknown mutation in the remaining 2 patients.

  7. Effect of hypercortisolism control on high blood pressure in Cushing's syndrome Efecto del control del hipercortisolismo sobre la hipertensión arterial en el síndrome de Cushing

    Directory of Open Access Journals (Sweden)

    Reynaldo M. Gómez

    2007-10-01

    Full Text Available Many hypertensive patients affected by endogenous Cushing's syndrome (CS persist with high blood pressure (HBP despite good control of cortisol excess. We assessed the effect of preoperative ketoconazole administration and of definitive treatment of CS on arterial hypertension and analysed the factors involved in the persistence of hypertension. We assessed retrospectively 71 patients with CS and HBP (60 women, 11 men; 50 pituitary, 21 adrenal successfully treated by surgery and/or radiotherapy; 19 of them received ketoconazole (KNZ before surgery. After treatment, patients were divided into those with persistent high blood pressure (PHBP and those with normal blood pressure (NBP. As possible predictive factors for PHBP we analysed age, duration and family history of HBP, pre-treatment 24 hour urinary free cortisol (24h-UFC and body mass index (BMI. HBP normalized in 53 out of 71 patients (74.6%, regardless of the origin of Cushing's syndrome. PHBP patients were older (p=0.003, had longer duration (p=0.007 and higher systolic blood pressure before treatment (p=0.046 than NBP patients. Thirteen out of 19 patients (68.4% treated with ketoconazole, normalized their hypertension and remained normotensive after successful surgery. Five patients became normotensive only after surgery. In conclusion: a blood pressure levels normalized in most patients after remission of CS; b ketoconazole was effective for the control of HBP, and seems to be a good indicator of post-surgical outcome, and c higher age at presentation, longer duration of hypertension and higher systolic blood pressure figures before treatment negatively influence normalization of blood pressure after resolution of Cushing's syndrome.Muchos pacientes con síndrome de Cushing (SC permanecen hipertensos a pesar del control del exceso glucocorticoideo. Investigamos el efecto de la administración de ketoconazol (KNZ y del tratamiento definitivo del SC sobre la hipertensión arterial (HTA

  8. Not all glucocorticoid-induced obesity is the same: differences in adiposity among various diagnostic groups of Cushing syndrome.

    Science.gov (United States)

    London, E; Lodish, M; Keil, M; Lyssikatos, C; de la Luz Sierra, M; Nesterova, M; Stratakis, C A

    2014-11-01

    The cAMP signaling pathway is implicated in bilateral adrenocortical hyperplasias (BAHs), which are often associated with ACTH-independent Cushing syndrome (CS). Although CS is invariably associated with obesity and is frequently associated with PKA signaling defects, we recently reported that its different forms appear to also present with variable weight gain and adiposity. The present study was aimed at characterizing further the phenotypic and molecular differences in periadrenal adipose tissue (PAT) among patients with subtypes of CS, by anthropometric/biochemical analyses and quantification of PKA expression and activity in BAHs in comparison to a non-CS group with aldosterone producing adenomas (APAs). Glucocorticoid levels, serum parameters, and BMI were analyzed among a larger patient cohort including those with different forms of CS, APAs, and Cushing disease. Abdominal CT scans were available for a small subset of patients examined for fat distribution. PAT collected during adrenalectomy was assayed for PKA activity, cAMP, and PKA expression. BMI and BMI z-score were lower in adults with PPNAD with PRKAR1A mutations and in pediatric patients with PPNAD with and without PRKAR1A mutations, respectively. Patients with PPNAD had higher cAMP levels in PAT and different fat distribution. Thus, PKA activity in PAT differed between CS diagnostic groups. Increased cAMP and PKA activity may have contributed to phenotypic differences among subtypes of CS. In agreement with the known roles of cAMP signaling in the regulation of adiposity, patients with PPNAD were less obese than other patients with CS. © Georg Thieme Verlag KG Stuttgart · New York.

  9. Experience with 99mTc-tektrotyd in the diagnosis of ectopic localization of acth-secreting tumors in patients with cushings syndrome

    International Nuclear Information System (INIS)

    Novikova, T.G.; Makeev, S.S.; Koval', S.S.; Guk, N.A.

    2015-01-01

    The differential diagnosis of Cushings disease is often carried out with Cushings syndrome, caused by the presence of tumors producing bioactive ACTH or ACTH-like substance. The aim of the study was to determine the effectiveness of the use of scintigraphy with 99m Tc-Tektrotyd in the diagnosis of ectopic localization of ACTH-secreting tumors in patients with Cushings syndrome. The survey of 25 patients with elevated levels of ACTH in the peripheral blood, allowed in 10 (40 %) patients identify foci of increased uptake radiopharmaceutical analog of somatostatin. It was found that the scintigraphy 99m Tc-Tektrotyd is sensitive and specific method in determining the foci of ectopic ACTH production. The use of scintigraphy with 99m Tc-Tektrotyd may be a good alternative to studies with octreotide labeled with 1U In or 123 I in the diagnosis of ectopic ACTH syndrome due to lower radiation dose to the patient, the higher picture quality and greater availability of this radiopharmaceutical

  10. HIV infection and Cushing's disease

    Directory of Open Access Journals (Sweden)

    Lalić Tijana

    2014-01-01

    Full Text Available Introduction: People with AIDS can have a dysfunction of the hypothalamic - pituitary-adrenal axis. With regard to HIV infection, most often mentioned is iatrogenic Cushing's syndrome or Pseudo-Cushing's Syndrome. So far there are described only two cases of Cushing disease in HIV -infected persons. Case report: A 48-year-old patient, after eleven years of HIV infection and a year since the introduction of HAART, was diagnosed with Cushing's disease based on cushingoid habitus, lack of suppression of cortisol in screening, elevated ACTH and pituitary tumor. She had transfenoidal surgery and histopathologic findings corresponded to basophilic adenoma. After the operation, short time on hydrocortisone substitution, she generally felt well with regular ART. Four years later, again easily bruising, facial redness, oily skin with acne, weight gain, uneven distribution of stomach adipose tissue, sweating, oligomenorrhea and high blood pressure. There was no rest/relapse of tumor on control pituitary MRI. Initially, elevated ACTH, valid cortisol in daily profiles, later the absence of the suppression of cortisol after 4 mg (LDST and 8 mg (HDST of dexamethasone along with maintenance of higher ACTH, indicate recurrence of clinical and laboratory relapse wherefore ketoconazole was introduced. Despite increasing doses of ketoconazole, she held slightly higher morning cortisol, ACTH and with persisting Cushing's syndrome. Conclusion: The coexistence of the two entities could lead to overlapping metabolic and phenotypic characteristics and the interaction between and/or synergism.

  11. Rapid response of hypercortisolism to vandetanib treatment in a patient with advanced medullary thyroid cancer and ectopic Cushing syndrome

    International Nuclear Information System (INIS)

    Pitoia Fabian; Bueno, Fernanda; Schmidt, Angelica; Lucas, Sabrina; Cross, Graciela

    2015-01-01

    Medullary thyroid carcinoma (MTC) may rarely present with paraneoplastic syndromes. Among the most frequent ones are the appearance of diarrhea and ectopic Cushing syndrome (ECS). The ECS in the context of MTC is usually present in patients with distant metastatic disease. The use of drugs such as ketoconazole, metyrapone, somatostatin analogs and etomidate have been ineffective alternatives to control hypercortisolism in these patients. Bilateral adrenalectomy is often required to manage this situation. Recently, the use of tyrosine kinase inhibitors has been shown to be a useful tool to achieve eucortisolism in patients with metastatic MTC and ECS. We present a patient with sporadic advanced persistent and progressive MTC with lymph node and liver metastases, which after 16 years of followup developed an ECS. After one month of 300 mg/day vandetanib treatment, a biochemical and clinical response of the ECS was achieved but it did not result in significant reduction of tumor burden. However the patient reached criteria for stable disease according to response evaluation criteria in solid tumors (RECIST 1.1) after 8 months of follow-up. (author)

  12. Rapid response of hypercortisolism to vandetanib treatment in a patient with advanced medullary thyroid cancer and ectopic Cushing syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Pitoia Fabian; Bueno, Fernanda; Schmidt, Angelica; Lucas, Sabrina; Cross, Graciela, E-mail: fpitoia@intramed.net [Division de Endocrinologia, Hospital de Clinicas, Universidad de Buenos Aires Buenos Aires (Argentina)

    2015-08-15

    Medullary thyroid carcinoma (MTC) may rarely present with paraneoplastic syndromes. Among the most frequent ones are the appearance of diarrhea and ectopic Cushing syndrome (ECS). The ECS in the context of MTC is usually present in patients with distant metastatic disease. The use of drugs such as ketoconazole, metyrapone, somatostatin analogs and etomidate have been ineffective alternatives to control hypercortisolism in these patients. Bilateral adrenalectomy is often required to manage this situation. Recently, the use of tyrosine kinase inhibitors has been shown to be a useful tool to achieve eucortisolism in patients with metastatic MTC and ECS. We present a patient with sporadic advanced persistent and progressive MTC with lymph node and liver metastases, which after 16 years of followup developed an ECS. After one month of 300 mg/day vandetanib treatment, a biochemical and clinical response of the ECS was achieved but it did not result in significant reduction of tumor burden. However the patient reached criteria for stable disease according to response evaluation criteria in solid tumors (RECIST 1.1) after 8 months of follow-up. (author)

  13. Peptide Receptor Radionuclide Therapy with177Lu-DOTATATE for Metastatic Neuroendocrine Tumor Occurring in Association with Multiple Endocrine Neoplasia Type 1 and Cushing's Syndrome.

    Science.gov (United States)

    Naik, Chinna; Basu, Sandip

    2017-01-01

    Neuroendocrine tumor (NET) occurring in association with other endocrine syndromes forms a distinct entity. The aim was to assess the therapy response profile of the routine peptide receptor radionuclide therapy (PRRT) in this relatively uncommon but clinically challenging subgroup of patients. A retrospective analysis was undertaken from the case records from those who were treated with 177 Lu-DOTATATE for metastatic NET. In addition to assessing the therapeutic efficacy, emphasis was also given to study lesional sites and scan pattern. A total of 5 cases were found: In this series of five cases, four belonged to multiple endocrine neoplasia type 1 (MEN1) syndrome; in these four MEN1 syndrome patients, the primary site of NET was thymic region ( n = 1), duodenum ( n = 1), and pancreas ( n = 2). The fifth case was of Cushing's syndrome with the primary site of NET in the thymus. A good symptomatic response was observed in all MEN1 syndrome cases (100%) and progression of symptoms in the patient with Cushing's syndrome. The biochemical response (assessed by measurement of tumor marker serum chromogranin A) demonstrated very good partial response (defined by more than 75% reduction of tumor marker) in 2 MEN1 cases and Cushing's syndrome, good partial response (25-75% reduction of tumor marker) in the remaining 2 MEN1 cases. Scan wise (assessed by technetium [ 99m Tc]-hydrazinonicotinamide [HYNIC]-tektrotyd [TOC]/ 68 Ga-DOTA-NOC/TATE positron emission tomography-computed tomography [PET-CT] and fluorodeoxyglucose [FDG] PET-CT) partial response was observed in 3 MEN1 cases, stable disease was noted in one MEN1 case and disease progression was noted in the patient with Cushing's syndrome. The change in FDG uptake was found to be an important sensitive scan parameter in the treatment evaluation of NETs compared to somatostatin receptor-based imaging in the cases with low MiB1 index. In our series, good palliative response to 177 Lu-DOTA-octreotate (DOTATATE) PRRT was

  14. Iatrogenic Cushing's syndrome with osteoporosis and secondary adrenal failure in human immunodeficiency virus-infected patients receiving inhaled corticosteroids and ritonavir-boosted protease inhibitors: six cases.

    Science.gov (United States)

    Samaras, Katherine; Pett, Sarah; Gowers, Andrew; McMurchie, Marilyn; Cooper, David A

    2005-07-01

    Ritonavir, a protease inhibitor (PI), is a potent inhibitor of cytochrome P450 3A4. This pharmacological effect, even at low doses (Cushing's syndrome when treated with inhaled fluticasone at varying doses for asthma while concurrently treated with low-dose ritonavir-boosted PI antiretroviral therapy (ART) regimens for HIV infection. There was evidence of adrenal suppression in all patients on stimulation studies. After the withdrawal of inhaled fluticasone, four patients became symptomatic of hypocortisolism, and three required oral corticosteroid support for several months. Other complications included evidence of osteoporosis (n = 3), crush fractures (n = 1), and exacerbation of preexisting type 2 diabetes mellitus (n = 1). In part, the diagnosis of fluticasone-induced Cushing's syndrome was delayed because all patients had preexisting body composition changes of ART-associated lipodystrophy, masking the Cushing's features. Practitioners should be aware of the impact on the adrenal axis of coadministration of PI-based ART regimens with inhaled corticosteroids and the potential for exacerbating or even inducing other metabolic conditions, such as osteoporosis or diabetes.

  15. Diagnosis of Cushing`s disease in children: a challenge for the radiologist

    Energy Technology Data Exchange (ETDEWEB)

    Kalifa, G. [Service de Radiologie, Hopital St. Vincent de Paul, 75 Paris (France); Adamsbaum, C. [Service de Radiologie, Hopital St. Vincent de Paul, 75 Paris (France); Carel, J.C. [Service d`Endocrinologie, Hopital St. Vincent de Paul, 75 Paris (France); Andre, C. [Service de Radiologie, Hopital St. Vincent de Paul, 75 Paris (France); Bougneres, P.F. [Service d`Endocrinologie, Hopital St. Vincent de Paul, 75 Paris (France); Chaussain, J.L. [Service d`Endocrinologie, Hopital St. Vincent de Paul, 75 Paris (France)

    1994-12-01

    Cushing`s disease is the most common cause of Cushing`s syndrome in children and is almost always related to over secretion of ACTH by the pituitary gland. It is important to identify a secreting adenoma prior to surgery. Seven cases studied with MRI are reviewed. In five cases the MRI findings suggested adenoma. Three secreting adenomas were confirmed at surgery. One case was in fact a cyst of the pars intermedia, and nothing could be found in the last case. Two patients presented with apparently normal findings on MRI, which was confirmed. There is a close correlation between identifying an adenoma and the success of surgery. (orig.)

  16. Plasma adiponectin levels are increased despite insulin resistance in corticotropin-releasing hormone transgenic mice, an animal model of Cushing syndrome.

    Science.gov (United States)

    Shinahara, Masayuki; Nishiyama, Mitsuru; Iwasaki, Yasumasa; Nakayama, Shuichi; Noguchi, Toru; Kambayashi, Machiko; Okada, Yasushi; Tsuda, Masayuki; Stenzel-Poore, Mary P; Hashimoto, Kozo; Terada, Yoshio

    2009-01-01

    Adiponectin (AdN), an adipokine derived from the adipose tissue, has an insulin-sensitizing effect, and plasma AdN is shown to be decreased in obesity and/or insulin resistant state. To clarify whether changes in AdN are also responsible for the development of glucocorticoid-induced insulin resistance, we examined AdN concentration in plasma and AdN expression in the adipose tissue, using corticotropin-releasing hormone (CRH) transgenic mouse (CRH-Tg), an animal model of Cushing syndrome. We found, unexpectedly, that plasma AdN levels in CRHTg were significantly higher than those in wild-type littermates (wild-type: 19.7+/-2.5, CRH-Tg: 32.4+/-3.1 microg/mL, pAdN mRNA and protein levels were significantly decreased in the adipose tissue of CRH-Tg. Bilateral adrenalectomy in CRH-Tg eliminated both their Cushing's phenotype and their increase in plasma AdN levels (wild-type/sham: 9.4+/-0.5, CRH-Tg/sham: 15.7+/-2.0, CRH-Tg/ADX: 8.5+/-0.4 microg/mL). These results strongly suggest that AdN is not a major factor responsible for the development of insulin resistance in Cushing syndrome. Our data also suggest that glucocorticoid increases plasma AdN levels but decreases AdN expression in adipocytes, the latter being explained possibly by the decrease in AdN metabolism in the Cushing state.

  17. [Cushing's disease caused by pituitary macroadenoma].

    Science.gov (United States)

    Rubatino, Antônio C; Pereira, Rodrigo Ferreira; Benchimol, Isaac; Laun, Ingeborg Christa

    2004-12-01

    Cushing's syndrome comprises the symptoms and signs associated with prolonged exposure to inappropriately elevated levels of free plasma glucocorticoids. When iatrogenic causes are excluded, the commonest cause of Cushing's syndrome is Cushing's disease, accounting for approximately 70% of cases. We present the case of a 20-year-old male patient with central obesity, moon face and purple-red striae, whose diagnostic investigation shows a pituitary macroadenoma. The patient was submitted to transsphenoidal hypophysectomy, but developed early recurrence. He was submitted to a second transsphenoidal intervention followed by pituitary radiation. Presently, the patient is in clinical and laboratory remission.

  18. Cushing disease

    International Nuclear Information System (INIS)

    Torres Esteche, V.; Menafra Prieto, M.; Ormaechea Gorricho, R.; Vignolo Scalone, G.; Larre Borges, A.

    1998-01-01

    A review of the Cushings disease in its various aspects. It highlights the importance of early diagnosis to avoid repercussions hypercortisolism secondary to parenchymal. We describe the findings in the Nuclear Magnetic Resonance (NMR), noting that the pituitary adenoma is often of small size and sometimes not visible on MRI. The treatment of choice remains surgical treatment other contingencies exist for particular cases (Author) [es

  19. A Cushing's syndrome patient's severe insomnia and morning blood pressure surge both improved after her left adrenal tumor resection.

    Science.gov (United States)

    Imaizumi, Yuki; Ibaraki, Ai; Asada, Satoshi; Tominaga, Mitsuhiro; Hayashi, Hiroyuki; Tsuchihashi, Takuya; Eguchi, Kazuo; Kario, Kazuomi; Taketomi, Akira

    2016-12-01

    Underlying mechanisms of the elevated risks of hypertension and cardiovascular disease (CVD) in Cushing's syndrome (CS) are unclear. We treated an adult woman with CS because of a cortisol-secreting adrenal tumor. After tumor resection, the 24-h blood pressure (BP) level improved from 156/91 to 131/84 mmHg; the morning BP surprisingly improved from 174/98 to 127/93 mmHg, although we reduced her antihypertensive medication. Her sleep quality (by the Pittsburgh Sleep Quality Index) improved from 7 to 2 points. Disturbed circadian BP rhythm is often observed in CS, but was reported only as altered nocturnal BP fall. This is the first report showing the disappearance of the morning BP surge evaluated by ambulatory BP monitoring with postsurgery sleep quality improvement. Poor-quality sleep, followed by exaggerated morning BP surge may thus be a cause of CS-related cardiovascular events. Sleep quality and BP circadian rhythm evaluations may clarify hypertension and high CVD risk in CS.

  20. Pediatric Cushing's disease: Management Issues.

    Science.gov (United States)

    Savage, Martin O; Storr, Helen L

    2012-12-01

    Cushing's disease (CD), caused by an ACTH-secreting pituitary corticotroph adenoma, is the commonest cause of Cushing syndrome in children over 5 years of age. It is rare in the pediatric age range and presents difficult diagnostic and therapeutic challenges. Key presenting features include weight gain, growth failure and change in facial appearance. Most pediatric endocrinologists have limited experience managing children or adolescents with CD and thus benefit from close consultation with adult colleagues. We describe a diagnostic protocol which broadly follows the model for adult patients. Treatment strategies are examined and appraised. The management of pediatric CD patients after cure is also discussed.

  1. Cushing disease with pregnancy.

    Science.gov (United States)

    Gopal, Raju A; Acharya, Shrikrishna V; Bandgar, Tushar R; Menon, Padma S; Shah, Nalini S

    2012-07-01

    Pregnancy occurs rarely in patients with Cushing syndrome (CS) due to hypercortisolism. So far, about 150 cases of CS in pregnancy have been reported in the literature. We describe a 22-year-old female who presented in pregnancy with clinical features of CS. She delivered at 34 weeks of gestation and baby had transient adrenal insufficiency in the neonatal period. Mother underwent transsphenoidal surgery 1 year postpartum and on follow up she is under remission. Neonatal hypoadrenalism should be anticipated in maternal CS.

  2. Iatrogenic Cushing's syndrome in an HIV-infected patient treated with inhaled corticosteroids (fluticasone propionate) and low dose ritonavir enhanced PI containing regimen.

    Science.gov (United States)

    Clevenbergh, P; Corcostegui, M; Gérard, D; Hieronimus, S; Mondain, V; Chichmanian, R M; Sadoul, J L; Dellamonica, P

    2002-04-01

    In HIV-infected patients, ritonavir, a potent cytochrome P450 inhibitor, is increasingly used to improve the pharmacokinetic profile of the associated protease inhibitor. HIV physicians are often faced with potential drug-drug interaction while treating associated diseases. We report the case of an HIV-infected patient with clinical features of Cushing's syndrome due to the interaction of low dose ritonavir with inhaled fluticasone propionate (FP). Safety of life-long CYP450 inhibition has still to be demonstrated. Copyright 2002 The British Infection Society.

  3. Unmasking sarcoidosis following surgery for Cushing disease

    DEFF Research Database (Denmark)

    Fraes Diernaes, Jon Erik; Bygum, Anette; Poulsen, Per L

    2016-01-01

    We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman...... for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed...... successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease....

  4. Diagnosis of Icenko-Cushing's disease and syndrome by angiography and the determination of the content of hormones in blood samples from the adrenal vein and vena cava inferior

    International Nuclear Information System (INIS)

    Yugrinov, O.G.; Slavnov, V.N.; Rybakov, S.I.; Yakovlev, A.A.

    1983-01-01

    The paper is concerned with the procedure of a comprehensive examination of patients with hypercorticoidism including angiography of the adrenals and the determination of the hydrocortisone level in the blood. The results of the studies on 32 patients with Icenko-Cushing's syndrome and 95 patients with Icenko-Cushing's disease are presented. X-ray appearance of the focal and diffuse adrenal disorders is described. The authors provide some data on the concentration of hydrocortisone in blood samples and on the content of corticotropin The importance of these data for diagnosis and differential diagnosis of Icenko-Cushing's disease and syndrome is shown. In the authors' opinion, the chief method in this diagnostic complex should be selective adrenal venography

  5. Verbal and visual memory performance and hippocampal volumes, measured by 3-Tesla magnetic resonance imaging, in patients with Cushing's syndrome.

    Science.gov (United States)

    Resmini, Eugenia; Santos, Alicia; Gómez-Anson, Beatriz; Vives, Yolanda; Pires, Patricia; Crespo, Iris; Portella, Maria J; de Juan-Delago, Manel; Barahona, Maria-José; Webb, Susan M

    2012-02-01

    Cushing's syndrome (CS) affects cognition and memory. Our objective was to evaluate memory and hippocampal volumes (HV) on 3-tesla magnetic resonance imaging (3T MRI) in CS patients and controls. Thirty-three CS patients (11 active, 22 cured) and 34 controls matched for age, sex, and education underwent Rey Auditory Verbal Learning Test and Rey-Osterrieth Complex Figure memory tests. Gray matter and HV were calculated on 3T MRI, using FreeSurfer image analyses software. No differences in HV were observed between active and cured CS or controls. Memory performance was worse in CS patients than controls (P visual memory (P = 0.04) than controls. In 12 CS patients, memory was below normative cutoff values for verbal (n = 6, cured), visual memory (n = 10, six cured) or both (n = 4); these patients with severe memory impairments showed smaller HV compared with their matched controls (P = 0.02 with verbal impairment; P = 0.03 with visual impairment). They were older (P = 0.04), had shorter education (P = 0.02), and showed a trend toward longer duration of hypercortisolism (P = 0.07) than the remaining CS patients. Total (P = 0.004) and cortical (P = 0.03) brain gray matter volumes were decreased in CS compared with controls, indicating brain atrophy, whereas subcortical gray matter (which includes HV) was reduced only in the 12 patients with severe memory impairment. Verbal and visual memory is worse in CS patients than controls, even after biochemical cure. HV was decreased only in those whose memory scores were below normative cutoff values.

  6. Neuroendocrine axis of stress, metabolic syndrome and psychiatric disorders in cushing's sindrome Ejes neuroendocrinos del estrés, síndrome metabólico y alteraciones psiquiátricas del síndrome de Cushing

    Directory of Open Access Journals (Sweden)

    Leidy Alexandra Lezcano Tobón

    2005-04-01

    Full Text Available The role of abnormalities of the hypophysishypothalamus- adrenal gland axis (HHA in the ongoing stress and depression is analyzed. Besides, it is evidenced that some problems, until recently considered merely endocrine-metabolic such as Cushing's syndrome (either clinical or subclinical and obesity (mainly when it is part of the metabolic syndrome may have a common etiological basis, be it a primary event, a comorbidity or a trigger in individuals with genetic susceptibility to states of maladaptative chronic stress. Some harmful effects of severe and/or ongoing hypercortisolism on some brain areas and the possibility of permanent alterations in some neuronal circuits are described. Finally, and according to some clinical evidences, the potential therapeutic role of antiglucocorticoids in the management of refractory depression is explored, as well as the role that early psychiatric intervention and antidepressant pharmacological treatment may play in some patients with the metabolic syndrome or increased cardiovascular risk. Se analiza el papel de las anormalidades del eje hipotálamo-hipófisis-adrenales (HHA en el estrés sostenido y la depresión. Además se pone en evidencia que algunos problemas hasta hace poco considerados puramente endocrino-metabólicos como el síndrome de Cushing (SC (clínico o subclínico y la obesidad, principalmente cuando hace parte del síndrome metabólico (SM, pueden tener una base etiológica común, como evento primario, comorbilidad o disparador en individuos con susceptibilidad genética a los estados de estrés crónico maladaptativo. Se describen algunas acciones lesivas del hipercortisolismo severo y/o sostenido en algunas áreas cerebrales y la posibilidad de producir alteraciones permanentes en algunos circuitos neuronales. Por último se explora, de acuerdo con algunas evidencias clínicas, el papel terapéutico potencial de fármacos antiglucocorticoides en el manejo de la depresión refractaria y

  7. [Iatrogenic Cushing's syndrome, diabetes mellitus and secondary adrenal failure in a human immunodeficiency virus patient treated with ritonavir boosted atazanavir and fluticasone].

    Science.gov (United States)

    Collet-Gaudillat, C; Roussin-Bretagne, S; Desforges-Bullet, V; Petit-Aubert, G; Doll, J; Beressi, J -P

    2009-09-01

    Ritonavir and atazanavir (ATZ) are protease inhibitors (PI) that inhibit the P450 3A4 cytochrome. They are used together to boost ATZ levels and reduce pill burden in human immunodeficiency virus infection, but association with medications metabolized by this cytochrome can cause serious adverse effects. Several cases of Cushing's syndrome have been reported when patients received inhaled therapy with fluticasone for asthma, sometimes complicated by secondary adrenal failure after stopping fluticasone. We report a case of Cushing's syndrome associated with onset of diabetes mellitus in a patient treated with boosted PI (ATZ and ritonavir) for HIV 2 (CD4360/ml). Asthma was treated with inhaled fluticasone 1500mug/day for several months that was stopped at admission. A few days later, typical secondary adrenal failure developed and was confirmed by dosage of cortisol and ACTH, both low. Hydrocortisone replacement treatment resulted in rapid improvement of symptoms. Diabetes was initially treated with insulin then sulfonyluraes, but repeated hypoglycemias lead to diet alone. Physicians should be aware of the potential danger of the association of "boosted" IP and some kind of inhaled corticotherapy.

  8. Internal jugular vein adrenocorticotropic hormone estimation for diagnosis of adrenocorticotropic hormone-dependent Cushing′s syndrome: Ultrasound-guided direct jugular vein sample collection

    Directory of Open Access Journals (Sweden)

    Jaya Prakash Sahoo

    2012-01-01

    Full Text Available Aim of Study: To assess the utility of internal jugular vein (IJV / peripheral adrenocorticotropic hormone (ACTH gradient in determining the etiology of ACTH- dependent Cushing′s syndrome. Materials and Methods: Patients with ACTH-dependent Cushing′s syndrome, (except children less than 12 years, had IJV blood collection under ultrasound guidance using a linear 7 MHZ probe. Blood was collected with a 21 G needle at the level of mandible with the patient in supine position. Six ml of blood was collected sequentially from right and left internal jugular veins for ACTH and prolactin estimation. Peripheral blood for ACTH and prolactin was taken from a previously placed IV cannula in the antecubital vein. Results: Thirty patients (20 F, 10 M, age 14 to 50 yrs were enrolled for this study. Source of ACTH excess was pituitary in 22, ectopic ACTH in 4, and unknown in 4 cases. Using an IJV: Peripheral ACTH ratio of ≥ 1.6, 15 out of 22 Cushing′s disease patients were correctly identified. However, 1 out of 4 ectopic Cushing also had IJV: Peripheral ratio ≥ 1.6. Overall, it had sensitivity of 68% with specificity of 75% while MRI pituitary and HDDST had sensitivity of 86% and 59%, respectively, with specificity of 100% each. Conclusion: IJV: Peripheral ACTH gradient was observed in 68% of patients with Cushing′s disease. Simultaneous IJV and peripheral sample collection with CRH stimulation may improve sensitivity and specificity of this test.

  9. Impaired microvascular reactivity and endothelial function in patients with Cushing's syndrome: Influence of arterial hypertension

    Czech Academy of Sciences Publication Activity Database

    Prázný, M.; Ježková, J.; Horová, E.; Lazárová, V.; Hána, V.; Kvasnička, J.; Pecen, Ladislav; Marek, J.; Škrha, J.; Kršek, M.

    2008-01-01

    Roč. 57, č. 1 (2008), s. 13-22 ISSN 0862-8408 Institutional research plan: CEZ:AV0Z10300504 Keywords : Cushing’s syndrome * vascular reactivity * endothelial function * oxidative stress * laser Doppler flowmetry Subject RIV: FB - Endocrinology, Diabetology, Metabolism, Nutrition Impact factor: 1.653, year: 2008

  10. Síndrome de Cushing por uso abusivo de descongestionante nasal contendo dexametasona: Relato de caso Cushing syndrome after abusive use of a nasal preparation containing dexamethasone: A case report

    Directory of Open Access Journals (Sweden)

    Dayse Caldas

    2001-01-01

    glycocorticoids were used topically. We relate the case of a sixteen-year-old boy with allergic rhinitis who developed exogenous Cushing syndrome after abusive use of a nasal spray containing dexamethasone in a daily dose varying from 0.5-0.7mg during 4 years. Once the diagnosis of exogenous Cushing syndrome was established, the spray containing dexamethasone was substituted by another one without steroids associated with the prescription of dexamethasone tablets in progressively lower doses. About 3 month later, when complete restoration of the hypothalamic-pituitary-adrenal axis was observed, the tablets were discontinued. We would like to emphasize the importance of a strict follow-up of all patients with chronic use of any drug containing glycocorticoids; it is recommended in some cases to determine urinary free or plasmatic cortisol after the new low-dose (1mcg ACTH stimulation test, in order to come to the diagnosis of incipient adrenal insufficiency, which could offer some danger in the presence of mild to moderate stress. Since the possibility of severe systemic side effects remains with the use of nasal steroids, we should whenever feasible prefer the use of glycocorticoids with lower bioavailability and emphasize the importance of respecting the dose and the time prescribed.

  11. Incidence and late prognosis of cushing's syndrome: a population based study

    DEFF Research Database (Denmark)

    Lindholm, J; Juul, Svend; Jørgensen, Jens Otto Lunde

    2001-01-01

    The main purpose was to assess the incidence and late outcome of Cushing’s syndrome, particularly in Cushing’s disease. Information for all patients diagnosed with Cushing’s syndrome during an 11-yr period in Denmark was retrieved. The incidence was 1.2–1.7/million·yr (Cushing’s disease), 0.......6/million·yr (adrenal adenoma) and 0.2/million·yr (adrenal carcinoma). Other types of Cushing’s syndrome were rare. In 139 patients with nonmalignant disease, 11.1% had died during follow-up (median, 8.1 yr; range, 3.1–14.0), yielding a standard mortality ratio (SMR) of 3.68 [95% confidence interval (CI), 2...... separately, including a questionnaire on their perceived quality of health. In 45 patients with Cushing’s disease who had been cured through transsphenoidal neurosurgery, only 1 had died (SMR, 0.31; CI, 0.01–1.72) compared with 6 of 20 patients with persistent hypercortisolism after initial neurosurgery (SMR...

  12. Treating Cushing's Disease in Dogs

    Science.gov (United States)

    ... For Consumers Home For Consumers Consumer Updates Treating Cushing's Disease in Dogs Share Tweet Linkedin Pin it More ... a tumor on an adrenal gland. Symptoms of Cushing's Disease Cushing's disease typically occurs in middle-aged to ...

  13. Genetics Home Reference: Cushing disease

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Cushing disease Cushing disease Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Cushing disease is caused by elevated levels of a hormone ...

  14. Syndrome de Cushing pendant la grossesse: à propos d'un cas d'adénome surrénalien

    Science.gov (United States)

    Touiti, Amal; El Mghari, Ghizlane; El Ansari, Nawal

    2015-01-01

    Nous rapportons le cas d'une patiente de 28 ans qui a présenté durant le deuxième trimestre de la grossesse une prise excessive du poids avec bouffissure du visage et apparition de vergetures pourpres au niveau de l'abdomen. Le diagnostic positif de syndrome de Cushing a été posé après l'accouchement devant deux CLU élevés et un test de freinage minute négatif. L’étiologie retenue était un adénome cortisolique. La prise en charge a consisté en une surrénalectomie gauche. PMID:26491524

  15. Iatrogenic Cushing's syndrome and secondary adrenal insufficiency after a single intra-articular administration of triamcinolone acetonide in HIV-infected patients treated with ritonavir.

    Science.gov (United States)

    Yombi, J C; Maiter, D; Belkhir, L; Nzeusseu, A; Vandercam, B

    2008-12-01

    The development of an iatrogenic Cushing's syndrome (ICS) followed by secondary adrenal failure remains an exceptional event after a single dose administration of a synthetic glucocorticoid. Medical attention has been drawn recently on the possible impact of ritonavir-based antiretroviral regimens on the systemic deleterious effects of a chronic administration of corticosteroids in HIV-infected patients. Three HIV-infected patients treated by a ritonavir-boosted protease inhibitor (PI) regimen received a single intra-articular injection of 40 mg triamcinolone acetonide in our university hospital. The three patients rapidly developed signs and symptoms of ICS followed by secondary adrenal insufficiency. Special attention must be paid when a single administration of corticosteroids has to be given in HIV-positive patients under ritonavir-boosted antiretroviral treatment, as these patients are at risk of developing early cushingoid features and a prolonged suppression of their hypothalamic-pituitary-adrenal axis.

  16. Current status of adrenalectomy for Cushing's disease

    International Nuclear Information System (INIS)

    Brunicardi, F.C.; Rosman, P.M.; Lesser, K.L.; Andersen, D.K.

    1985-01-01

    To evaluate the current use of adrenalectomy in the treatment of Cushing's disease, we reviewed seven consecutive patients who have undergone adrenalectomy for Cushing's disease at this medical center during 1983 to 1984. Seventy-one percent (5/7) had pituitary, or type I, Cushing's disease, while 29% (2/7) had adrenal, or type II, Cushing's disease from either an adenoma or an adrenocortical carcinoma. Presenting signs and symptoms, either initially or at the time of recurrence, were typical of Cushing's syndrome. Four of five patients with type I disease had recurrent disease after transphenoidal hypophysectomy, bilateral adrenalectomy, or unilateral adrenalectomy. In three of five patients, medical therapy of hypercortisolism was abandoned because of adverse side effects. Preoperative evaluation in all patients included cortisol and ACTH levels, dexamethasone suppression tests, and computerized tomography (both abdominal and head). In patients with a prior history of adrenalectomy, radiocholesterol scans were also performed and were useful. Angiographic procedures were not required in these patients. In patients with type I disease, posterior operative approaches were used. In patients with type II disease, an anterolateral approach was used. Posterolateral incisions are preferred over Hugh-Young incisions and provide better exposure with a reduced risk of poor wound healing. Morbidity and mortality included one death and three nonhealing wounds. In the six surviving patients, symptoms resolved with variable frequency. Findings suggestive of Nelson's syndrome (hyperpigmentation) have occurred in two patients; serial computerized tomographic scans fail to reveal evidence of pituitary tumors

  17. Cushing Syndrome: Other FAQs

    Science.gov (United States)

    ... NICHD Research Information Find a Study More Information Amenorrhea About NICHD Research Information Find a Study More ... treatments? Related A-Z Topics Adrenal Gland Disorders Amenorrhea Pituitary Tumors NICHD News Spotlights Spotlight: Selected NICHD ...

  18. Ectopic Cushing syndrome

    Science.gov (United States)

    ... diabetes mellitus High blood pressure High cholesterol and triglycerides Women may have: Excess hair growth on the ... Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, ...

  19. Case report of severe Cushing's syndrome in medullary thyroid cancer complicated by functional diabetes insipidus, aortic dissection, jejunal intussusception, and paraneoplastic dysautonomia: remission with sorafenib without reduction in cortisol concentration.

    Science.gov (United States)

    Hammami, Muhammad M; Duaiji, Najla; Mutairi, Ghazi; Aklabi, Sabah; Qattan, Nasser; Abouzied, Mohei El-Din M; Sous, Mohamed W

    2015-09-09

    Normalization of cortisol concentration by multikinase inhibitors have been reported in three patients with medullary thyroid cancer-related Cushing's syndrome. Aortic dissection has been reported in three patients with Cushing's syndrome. Diabetes insipidus without intrasellar metastasis, intestinal intussusception, and paraneoplastic dysautonomia have not been reported in medullary thyroid cancer. An adult male with metastatic medullary thyroid cancer presented with hyperglycemia, hypernatremia, hypokalemia, hypertension, acne-like rash, and diabetes insipidus (urine volume >8 L/d, osmolality 190 mOsm/kg). Serum cortisol, adrenocorticoitropic hormone, dehydroepiandrostenedione sulfate, and urinary free cortisol were elevated 8, 20, 4.4, and 340 folds, respectively. Pituitary imaging was normal. Computed tomography scan revealed jejunal intussusception and incidental abdominal aortic dissection. Sorafenib treatment was associated with Cushing's syndrome remission, elevated progesterone (>10 fold), normalization of dehydroepiandrostenedione sulfate, but persistently elevated cortisol concentration. Newly-developed proximal lower limb weakness and decreased salivation were associated with elevated ganglionic neuronal acetylcholine receptor (alpha-3) and borderline P/Q type calcium channel antibodies. Extreme cortisol concentration may have contributed to aortic dissection and suppressed antidiuretic hormone secretion; which combined with hypokalemia due cortisol activation of mineralocorticoid receptors, manifested as diabetes insipidus. This is the first report of paraneoplastic dysautonomia and jejunal intussusception in medullary thyroid cancer, they may be related to medullary thyroid cancer's neuroendocrine origin and metastasis, respectively. Remission of Cushing's syndrome without measurable reduction in cortisol concentration suggests a novel cortisol-independent mechanism of action or assay cross-reactivity. Normalization of dehydroepiandrostenedione

  20. Iatrogenic Cushing's syndrome and adrenal insufficiency in infants on intranasal dexamethasone drops for nasal obstruction - Case series and literature review.

    Science.gov (United States)

    Joshi, Rohan R; Maresh, Alison

    2018-02-01

    The use of intranasal steroid drops for nasal obstruction in infants is common practice and can prevent more invasive surgical procedures; however, it is not without complication. We describe 2 cases of iatrogenic Cushing's secondary to nasal steroids in infants with nasal obstruction, discuss the etiology of this unusual complication, and review previous literature reports. While reporting in the literature is sparse, these cases highlight the risk of development of adrenal insufficiency with usage of nasal steroid drops in infants as well as the need for close monitoring of administration and tapering of the drops. Additionally, we suggest an approach to the infant with symptomatic nasal obstruction that addresses the usage of intranasal steroid drops and emphasizes the need for quick tapering and possible endocrine consultation when appropriate. Copyright © 2017 Elsevier B.V. All rights reserved.

  1. Aberrant expression of glucagon receptors in adrenal glands of a patient with Cushing's syndrome and ACTH-independent macronodular adrenal hyperplasia Expresion aberrante de receptores de glucagón en tejido adrenal de un paciente con síndrome de Cushing e hiperplasia adrenal macronodular indedependiente de ACTH

    Directory of Open Access Journals (Sweden)

    Valeria de Miguel

    2010-06-01

    Full Text Available Adrenocorticotropin (ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH is a rare cause of Cushing´s syndrome, characterized by bilateral adrenal lesions and excess cortisol production despite ACTH suppression. Cortisol synthesis is produced in response to abnormal activation of G-protein- coupled receptors, such as gastric inhibitory peptide, vasopressin, beta adrenergic agonists, LH/hCG and serotonin receptors. The aim of this study was to analyze the expression of glucagon receptors in adrenal glands from an AIMAH patient. A patient with ACTH-independent Cushing´s syndrome and bilateral macronodular adrenal hyperplasia was screened for altered activation of adrenal receptors by physiological (mixed meal and pharmacological (gonadotrophin releasing hormone, ACTH and glucagon tests. The results showed abnormally high levels of serum cortisol after stimulation with glucagon. Hypercortisolism was successfully managed with ketoconazole treatment. Interestingly, a 4-month treatment with a somatostatin analogue (octreotide was also able to reduce cortisol secretion. Finally, Cushing's syndrome was cured after bilateral adrenalectomy. Abnormal mRNA expression for glucagon receptor in the patient´s adrenal glands was observed by Real-Time PCR procedure. These results strongly suggest that the mechanism of AIMAH causing Cushing´s syndrome in this case involves the illicit activation of adrenal glucagon receptors. This is the first case reported of AIMAH associated with ectopic glucagon receptors.La hiperplasia adrenal macronodular bilateral independiente de ACTH (HAMIA es una causa infrecuente de Síndrome de Cushing, caracterizada por lesiones adrenales bilaterales, hipercortisolismo y ACTH plasmática suprimida. La síntesis de cortisol estaría regulada a través de ligandos de receptores asociados a proteína G que se expresan en forma aberrante en la corteza de las glándulas adrenales. El objetivo de este trabajo es analizar

  2. Clinical outcomes of surgical treatment and longitudinal non-surgical observation of patients with subclinical Cushing's syndrome and nonfunctioning adrenocortical adenoma.

    Science.gov (United States)

    Maehana, Takeshi; Tanaka, Toshiaki; Itoh, Naoki; Masumori, Naoya; Tsukamoto, Taiji

    2012-04-01

    To investigate the outcomes of the surgical management and longitudinal assessment of patients with subclinical Cushing's syndrome (SCS) and nonfunctioning adrenocortical adenoma (NFA). Between the years 1995 and 2008, 73 patients with asymptomatic adrenocortical adenoma were enrolled. They were informed of the risks and benefits of adrenalectomy and conservative management, and then chose the treatment. SCS was observed in 13 patients (17.8%) and NFA in 60 patients (82.2%). Tumor size in SCS was significantly larger than that in NFA (34.6 ± 9.7 mm vs. 24.5 ± 8.0 mm in diameter, P=0.001). Of the SCS patients, 7 also suffered from hypertension (HT), 2 from diabetes mellitus (DM) and 3 from hyperlipidemia (HL). After adrenalectomy, the insulin dose could be reduced in 2 (100%) patients with DM, in 5 (71.4%) of those with HT and in 2 (66.7%) of those with HL. In the NFA surveillance group, 1 (2.6%) case developed into SCS 3 years after the initial presentation and an increase in size of the tumor was observed in 1 (2.6%), with a mean follow-up of 51.2 months. Surgical resection may be beneficial for the control of SCS and is likely to provide improvement of concomitant HT, DM and HL. Although NFA can be managed conservatively, its size and hormonal activities may change longitudinally. Thus, long-term follow-up is necessary for NFA.

  3. Use of radioguided surgery with [111In]-pentetreotide in the management of an ACTH-secreting bronchial carcinoid causing ectopic Cushing's syndrome.

    Science.gov (United States)

    Grossrubatscher, E; Vignati, F; Dalino, P; Possa, M; Belloni, P A; Vanzulli, A; Bramerio, M; Marocchi, A; Rossetti, O; Zurleni, F; Loli, P

    2005-01-01

    Intraoperative [111In]-pentetreotide scintigraphy with a hand-held gamma detector probe has recently been proposed to increase the intraoperative detection rate of small neuroendocrine tumors and their metastases. We report a case of a 28-yr-old woman with ectopic Cushing's syndrome due to an ACTH-secreting bronchial carcinoid, in whom the use of radioguided surgery improved disease management. At presentation, radiolabeled pentetreotide scintigraphy was the only procedure able to detect the ectopic source of ACTH. After radiologic confirmation, the patient underwent removal of a bronchial carcinoid, with disease persistence. After surgery, pentetreotide scintigraphy showed pathologic uptake in the mediastinum not previously detected at surgery and only subsequently confirmed by radiologic studies. Despite a second thoracic exploration, hormonal, scintigraphic, and radiological evidence of residual disease persisted. Radioguided surgery was then performed using a hand-held gamma probe 48 h after iv administration of a tracer dose of radiolabeled [111In-DTPA-D-Phe1]-pentetreotide, which permitted detection and removal of multiple residual mediastinal lymph node metastases. Clinical and radiologic cure, with no evidence of tracer uptake at pentetreotide scintigraphy, was subsequently observed. The use of an intraoperative gamma counter appears a promising procedure in the management of metastatic ACTH-secreting bronchial carcinoids.

  4. Ectopic corticotroph syndrome

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    Penezić Zorana

    2004-01-01

    Full Text Available INTRODUCTION Endogenous Cushing's syndrome is a clinical state resulting from prolonged, inappropriate exposure to excessive endogenous secretion of Cortisol and hence excess circulating free cortisol, characterized by loss of the normal feedback mechanisms of the hypothalamo-pituitary-adrenal axis and the normal circadian rhythm of cortisol secretion [2]. The etiology of Cushing's syndrome may be excessive ACTH secretion from the pituitary gland, ectopic ACTH secretion by nonpituitary tumor, or excessive autonomous secretion of cortisol from a hyperfunctioning adrenal adenoma or carcinoma. Other than this broad ACTH-dependent and ACTH-independent categories, the syndrome may be caused by ectopic CRH secretion, PPNAD, MAH, ectopic action of GIP or catecholamines, and other adrenel-dependent processes associated with adrenocortical hyperfunction. CASE REPORT A 31 year-old men with b-month history of hyperpigmentation, weight gain and proximal myopathy was refereed to Institute of Endocrinology for evaluation of hypercortisolism. At admission, patient had classic cushingoid habit with plethoric face, dermal and muscle atrophy, abdominal strie rubrae and centripetal obesity. The standard laboratory data showed hyperglycaemia and hypokaliemia with high potassium excretion level. The circadian rhythm of cortisol secretion was blunted, with moderately elevated ACTH level, and without cortisol suppression after low-dose and high-dose dexamethason suppression test. Urinary 5HIAA was elevated. Abdominal and sellar region magnetic resonance imaging was negative. CRH stimulation resulted in ACTH increase of 87% of basal, but without significant increase of cortisol level, only 7%. Thoracal CT scan revealed 14 mm mass in right apical pulmonary segment. A wedge resection of anterior segment of right upper lobe was performed. Microscopic evaluation showed tumor tissue consisting of solid areas of uniform, oval cells with eosinophilic cytoplasm and centrally

  5. A patient with Cushing disease lateralizing a pituitary adenoma by inferior petrosal sinus sampling using desmopressin: a case report.

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    Lim, Joo Hee; Kim, Soo Jung; Jung, Mo Kyung; Kim, Ki Eun; Kwon, Ah Reum; Chae, Hyun Wook; Kim, Duk Hee; Kim, Ho-Seong

    2016-03-01

    A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain. Random serum cortisol level, 24-hour urinary free cortisol excretion, and overnight and low-dose dexamethasone suppression tests suggested Cushing syndrome. Midnight adrenocorticotropic hormone (ACTH) level and high-dose dexamethasone suppression test confirmed Cushing disease. Pituitary magnetic resonance imaging was suspicious for microadenoma. To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS) was performed by desmopressin use to stimulate ACTH. Finally, the patient was diagnosed with Cushing disease due to ACTH-secreting pituitary microadenoma, lateralized to the left side; subsequently underwent transsphenoidal surgery. Here we report a case of a 14-year-old girl diagnosed with Cushing disease with a pituitary tumor lateralized by IPSS using desmopressin, which is very rare in pediatric Cushing disease.

  6. A patient with Cushing disease lateralizing a pituitary adenoma by inferior petrosal sinus sampling using desmopressin: a case report

    Directory of Open Access Journals (Sweden)

    Joo Hee Lim

    2016-03-01

    Full Text Available A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain. Random serum cortisol level, 24-hour urinary free cortisol excretion, and overnight and low-dose dexamethasone suppression tests suggested Cushing syndrome. Midnight adrenocorticotropic hormone (ACTH level and high-dose dexamethasone suppression test confirmed Cushing disease. Pituitary magnetic resonance imaging was suspicious for microadenoma. To eliminate ectopic ACTH syndrome, and lateralize the pituitary tumor, inferior petrosal sinus sampling (IPSS was performed by desmopressin use to stimulate ACTH. Finally, the patient was diagnosed with Cushing disease due to ACTH-secreting pituitary microadenoma, lateralized to the left side; subsequently underwent transsphenoidal surgery. Here we report a case of a 14-year-old girl diagnosed with Cushing disease with a pituitary tumor lateralized by IPSS using desmopressin, which is very rare in pediatric Cushing disease.

  7. Bilateral Testicular Tumors Resulting in Recurrent Cushing Disease After Bilateral Adrenalectomy.

    Science.gov (United States)

    Puar, Troy; Engels, Manon; van Herwaarden, Antonius E; Sweep, Fred C G J; Hulsbergen-van de Kaa, Christina; Kamphuis-van Ulzen, Karin; Chortis, Vasileios; Arlt, Wiebke; Stikkelbroeck, Nike; Claahsen-van der Grinten, Hedi L; Hermus, Ad R M M

    2017-02-01

    Recurrence of hypercortisolism in patients after bilateral adrenalectomy for Cushing disease is extremely rare. We present a 27-year-old man who previously underwent bilateral adrenalectomy for Cushing disease with complete clinical resolution. Cushingoid features recurred 12 years later, with bilateral testicular enlargement. Hormonal tests confirmed adrenocorticotropic hormone (ACTH)-dependent Cushing disease. Surgical resection of the testicular tumors led to clinical and biochemical remission. Gene expression analysis of the tumor tissue by quantitative polymerase chain reaction showed high expression of all key steroidogenic enzymes. Adrenocortical-specific genes were 5.1 × 105 (CYP11B1), 1.8 × 102 (CYP11B2), and 6.3 × 104 (MC2R) times higher than nonsteroidogenic fibroblast control. This correlated with urine steroid metabolome profiling showing 2 fivefold increases in the excretion of the metabolites of 11-deoxycortisol, 21-deoxycortisol, and total glucocorticoids. Leydig-specific genes were 4.3 × 101 (LHCGR) and 9.3 × 100 (HSD17B3) times higher than control, and urinary steroid profiling showed twofold increased excretion of the major androgen metabolites androsterone and etiocholanolone. These distinctly increased steroid metabolites were suppressed by dexamethasone but unresponsive to human chorionic gonadotropin stimulation, supporting the role of ACTH, but not luteinizing hormone, in regulating tumor-specific steroid excess. We report bilateral testicular tumors occurring in a patient with recurrent Cushing disease 12 years after bilateral adrenalectomy. Using mRNA expression analysis and steroid metabolome profiling, the tumors demonstrated both adrenocortical and gonadal steroidogenic properties, similar to testicular adrenal rest tumors found in patients with congenital adrenal hyperplasia, suggesting the presence of pluripotent cells even in patients without congenital adrenal hyperplasia. Copyright © 2017 by the Endocrine Society

  8. Efficacy of dexamethasone suppression test during the diagnosis of primary pigmented nodular adrenocortical disease in Chinese adrenocorticotropic hormone-independent Cushing syndrome.

    Science.gov (United States)

    Chen, Shi; Li, Ran; Lu, Lin; Duan, Lian; Zhang, Xuebin; Tong, Anli; Pan, Hui; Zhu, Huijuan; Lu, Zhaolin

    2018-01-01

    To evaluate the cut-off value of the ratio of 24 h urinary free cortisol (24 h UFC) levels post-dexamethasone to prior-dexamethasone in dexamethasone suppression test (DST) during the diagnosis of primary pigmented nodular adrenocortical disease in Chinese adrenocorticotropic hormone-independent Cushing syndrome. Retrospective study. The patients diagnosed with primary pigmented nodular adrenocortical disease (PPNAD, n = 25), bilateral macronodular adrenal hyperplasia (BMAH, n = 27), and adrenocortical adenoma (ADA, n = 84) were admitted to the Peking Union Medical College Hospital from 2001 to 2016. Serum cortisol, adrenocorticotropic hormone (ACTH), and 24 h UFC were measured before and after low-dose dexamethasone suppression test (LDDST) and high-dose dexamethasone suppression test (HDDST). After LDDST and HDDST, 24 h UFC elevated in patients with PPNAD (paired t-test, P = 0.007 and P = 0.001), while it remained unchanged in the BMAH group (paired t-test, P = 0.471 and P = 0.414) and decreased in the ADA group (paired t-test, P = 0.002 and P = 0.004). The 24 h UFC level after LDDST was higher in PPNAD and BMAH as compared to ADA (P < 0.017), while no significant difference was observed between PPNAD and BMAH. After HDDST, 24 h UFC was higher in patients with PPNAD as compared to that of ADA and BMAH (P < 0.017). The cut-off value of 24 h UFC (Post-L-Dex)/(Pre-L-Dex) was 1.16 with 64.0% sensitivity and 77.9% specificity, and the cut-off value of 24 h UFC (Post-H-Dex)/(Pre-H-Dex) was 1.08 with 84.0% sensitivity and 75.6% specificity. The ratio of post-dexamethasone to prior-dexamethasone had a unique advantage in distinguishing PPNAD from BMAH and ADA.

  9. Osteoporosis in Сhildren with Itsenko — Cushing Disease: Etiology, Pathogenesis, Diagnosis

    Directory of Open Access Journals (Sweden)

    M.O. Ryznychuk

    2016-03-01

    Full Text Available The review describes the etiology, pathogenetic mechanisms of osteoporosis in children with Itsenko — Cushing syndrome. The new methods for the diagnosis of osteoporosis in children were also analyzed.

  10. Strongyloides stercoralis Hyperinfection Syndrome Presenting as Severe, Recurrent Gastrointestinal Bleeding, Leading to a Diagnosis of Cushing Disease

    Science.gov (United States)

    Yee, Brittany; Chi, Nai-Wen; Hansen, Lawrence A.; Lee, Roland R.; U, Hoi-Sang; Savides, Thomas J.; Vinetz, Joseph M.

    2015-01-01

    A 50-year-old male immigrant from Ethiopia presented for consultation after 3 years of hematochezia/melena requiring > 25 units of blood transfusions. Physical examination revealed severe proximal muscle wasting and weakness, central obesity, proptosis, and abdominal striae, accompanied by eosinophilia, elevated hemoglobin A1c, elevated 24-hour urinary cortisol, lack of suppression of 8 am cortisol levels by 1 mg dexamethasone, and inappropriately elevated random adrenocorticotropic hormone (ACTH) level. Histopathological examination of gastrointestinal biopsies showed large numbers of Strongyloides stercoralis, indicating Strongyloides hyperinfection. Treatment with 2 days of ivermectin led to resolution of gastrointestinal bleeding. This syndrome was due to chronic immunosuppression from a pituitary ACTH (corticotroph) microadenoma, of which resection led to gradual normalization of urine cortisol, improved glycemic control, resolution of eosinophilia, and no recurrence of infection. PMID:26195463

  11. Unmasking sarcoidosis following surgery for Cushing disease.

    Science.gov (United States)

    Diernaes, Jon E F; Bygum, Anette; Poulsen, Per L

    2016-01-01

    We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman presented with symptoms of hypercortisolism such as progressive weight gain, Cushingoid appearance, proximal myopathy, easy bruising, and amenorrhea. Blood testing including inferior petrosal sinus sampling uncovered an ACTH-producing microadenoma in the right aspect of the anterior pituitary gland for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed clinically and a suspicion of underlying sarcoidosis was substantiated by lung imaging and elevated plasma interleukin (IL)-2 receptor. One month later, the lesions spontaneously resolved without therapy other than maintenance glucocorticoid replacement. Physicians should be aware that patients undergoing successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease.

  12. Harvey Cushing Treated the First Known Patient With Carney Complex.

    Science.gov (United States)

    Tsay, Cynthia J; Stratakis, Constantine A; Faucz, Fabio Rueda; London, Edra; Stathopoulou, Chaido; Allgauer, Michael; Quezado, Martha; Dagradi, Terry; Spencer, Dennis D; Lodish, Maya

    2017-10-01

    Carney complex (CNC) is a syndrome characterized by hyperplasia of endocrine organs and may present with clinical features of Cushing syndrome and acromegaly due to functional adrenal and pituitary gland tumors. CNC has been linked to mutations in the regulatory subunit of protein kinase A type I-alpha ( PRKAR1A ) gene. Tissue samples were taken from the hypothalamus or thalamus or tumors of patients with pituitary adenomas seen and operated on by neurosurgeon Harvey Cushing between 1913 and 1932. Following DNA extraction, sequencing for genes of interest was attempted, including PRKAR1A , AIP , USP8 , GNAS1 , and GPR101 , to explore the possibility that these mutations associated with acromegaly, CNC, and Cushing syndrome have been conserved over time. We report a patient described by Dr. Cushing in 1914 with a clinical presentation and postmortem findings suggestive of CNC. Genetic sequencing of the hypothalamus and pituitary adenoma revealed a germline heterozygous p.Arg74His mutation in the PRKAR1A gene, a codon previously described as mutated in CNC, but with a novel amino acid change. This patient is, to our knowledge, the first molecularly confirmed individual with CNC. This case demonstrates the power of modern genetics in studying archived tissues and the importance of recording detailed clinical notes in the diagnosis of disease.

  13. Pituitary radiotherapy for Cushing's disease.

    Science.gov (United States)

    Losa, Marco; Picozzi, Piero; Redaelli, Maria Grazia; Laurenzi, Andrea; Mortini, Pietro

    2010-01-01

    The treatment of choice for Cushing's disease is pituitary surgery. Second-line treatments include repeat pituitary surgery, radiation therapy, medical therapy, and bilateral adrenalectomy. The most used modalities to irradiate patients with Cushing's disease include fractionated radiotherapy and single-dose Gamma Knife. We aim to review the efficacy and safety of radiotherapy in patients with persistent or recurring Cushing's disease. Remission of Cushing's disease after radiotherapy ranges from 42 to 83%. There seems to be no clear difference according to the technique of radiation used. Most patients experience remission of disease within 3 years from treatment, with only few cases reaching normal cortisol secretion after a longer follow-up. Control of tumor growth varies from 93 to 100%. Severe side effects of radiotherapy, such as optic neuropathy and radionecrosis, are uncommon. New-onset hypopituitarism is the most frequent side effect of radiation, occurring in 30-50% of patients treated by fractionated radiotherapy while it has been reported in 11-22% of patients after Gamma Knife. Radiotherapy is an effective second-line treatment in patients with Cushing's disease not cured by surgery. Consideration of the advantages and disadvantages of radiotherapy in comparison with other therapeutic options should always be carried out in the single patient before deciding the second-line therapeutic strategy for persisting or recurring Cushing's disease. Copyright © 2010 S. Karger AG, Basel.

  14. Macroadenomas Causing Cushing’s Disease

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    Aydin K

    2014-01-01

    Full Text Available Kortikotrope Hypophysen-Makroadenome vs. Mikroadenome als Ursache des Morbus Cushing. Ziel: Kortikotrope Hypophysen- Makroadenome werden für eine geringe Zahl von Fällen des Cushing-Syndroms verantwortlich gemacht, man geht jedoch davon aus, dass ihre genaue Prävalenz unterschätzt wird. Des Weiteren fallen die klinischen und biochemischen Manifestationen kortikotroper Makro- und Mikroadenome sehr unterschiedlich aus und in bisherigen Studien wurde nur eine geringe Anzahl von Patienten eingeschlossen. In dieser Arbeit analysieren und vergleichen wir die klinischen Charakteristika und biochemischen Eigenschaften von Patienten mit kortikotropen Makro- und Mikroadenomen, die Morbus Cushing verursachen. Methoden: Wir schlossen 107 Patienten mit M. Cushing ein (67 Mikroadenome, 40 Makroadenome. Klinische Charakteristika, biochemische Analysen des Hyperkortisolismus, Ergebnisse des Hypophysen-Imaging, Behandlungsmodalitäten sowie Raten für Remission und Wiederauftreten wurden erfasst. Ergebnisse: Das Durchschnittsalter der Patienten lag bei 46,5 ± 11,4 Jahren für Makroadenome und 37,3 ± 12,1 Jahre für Mikroadenome (p 0,001. Die Geschlechterverteilung war bei den Makroadenompatienten gleich, während bei den Mikroadenompatienten ein Vorherrschen der Frauen offensichtlich war (p = 0,001. Verglichen mit den Makro- war die klinische Evidenz des Hyperkortisolismus bei den Mikroadenomen stärker ausgeprägt. Obwohl die Präsentation der klinischen Manifestationen unterschiedlich war, waren die biochemische Analyse des Hyperkortisolismus (inklusive Basalkortisol, ACTH, 24-Stunden-Harnkortisol-Levels und Ansprechen auf eine niedrig dosierte Dexamethason-Suppression zwischen den Gruppen ähnlich. Die Unterdrückbarkeit des Serumkortisols nach Gabe von hochdosiertem Dexamethason war für Makroadenome niedriger, erreichte aber keine Signifikanz (p = 0,074. Die Remissionsraten nach dem ersten hypophysenchirurgischen Eingriff (67,5 vs. 82,8 % sowie die

  15. Overnight Dexamethasone Suppression Test in the Diagnosis of Cushing's Disease

    Directory of Open Access Journals (Sweden)

    Fatemeh Esfahanian

    2010-08-01

    Full Text Available Realizing the cause of Cushing's Syndrome (CS is one of the most challenging processes in clinical endocrinology. The long high dose dexamethasone suppression test (standard test is costly and need an extended inpatient stay. In this study we want to show the clinical utility of the overnight 8 mg dexamethasone suppression test (DST for differential diagnosis of CS in a referral center. Retrospectively from 2002-2005 we selected the patients of endocrinology ward in Imam hospital who were admitted with the diagnosis of Cushing syndrome and had 8 mg DST (modified test along with classic DST. In modified test a decrease in an 8 AM serum cortisol level of 50% or more is thought to indicate suppression and we compared the results of modified test with standard test. This test had been done on 42 patients: 10 male (23% and 32 female (76%. The mean age of patients was 31.39 (15-63, 32 with proven pituitary Cushing's disease, 7 with primary adrnal tumors and 3 with ectopic ACTH syndrome. The standard test according to 50% suppression of UFC had 90.62% sensitivity, and according to 90% suppression had 43.75% sensitivity. The sensitivity of this test was 71.85% for serum cortisol suppression. The modified test (8 mg overnight DST had 78% sensitivity. All of these tests had 100% specificity for the diagnosis of Cushing's disease. The positive predictive vale (PPV of all of these tests was 100%. The negative predictive value (NPV of modified test for the diagnosis of Cushing's disease was 58.82%. In standard test the NPV of serum cortisol was 52.6%, UFC 50% had 76.9% NPV and UFC 90% had 35.7% NPV. The results of serum cortisol suppression in modified test is better than standard test. Although 50% suppression of UFC in standard test had greater sensitivity than modified test, collecting of urine is difficult, time consuming and needing hospitalization, so we advice modified test that is much simpler and more convenient instead of standard test in the first

  16. Diabetes in Cushing Disease.

    Science.gov (United States)

    Mazziotti, G; Formenti, A M; Frara, S; Maffezzoni, F; Doga, M; Giustina, A

    2017-05-01

    This review focuses on the pathophysiological and clinical aspects of diabetes mellitus occurring in patients with Cushing disease (CD). Insulin resistance and impairment in insulin secretion are both involved in the pathogenesis of glucocorticoid-induced diabetes. Correction of glucocorticoid excess does not always resolve abnormalities of glucose homeostasis, and correction of hyperglycaemia is specifically required. In fact, insulin resistance may persist even after correction of glucocorticoid excess and diabetes needs to be treated for long term. On the other hand, emerging drugs used in the treatment of CD, such as the novel somatostatin analog pasireotide, may have direct effects on glucose homeostasis regardless of control of cortisol excess. Diabetes mellitus is a frequent and early complication of CD with important diagnostic, prognostic and therapeutic implications. Specifically, diagnosis of CD in patients with diabetes may be difficult due to potential misinterpretation of markers of cortisol hypersecretion. Moreover, diabetes mellitus is often difficult to be controlled in CD requiring a careful and dedicated therapeutic approach. Finally, the coexistence of diabetes may influence the therapeutic decision making in CD, since drugs used in this setting may variably influence glucose homeostasis regardless of control of hypercortisolism.

  17. In the overnight dexamethasone suppression test, 1.0 mg loading is superior to 0.5 mg loading for diagnosing subclinical adrenal Cushing's syndrome based on plasma dexamethasone levels determined using liquid chromatography-tandem mass spectrometry.

    Science.gov (United States)

    Sasaki, Yosuke; Katabami, Takuyuki; Asai, Shiko; Fukuda, Hisashi; Tanaka, Yasushi

    2017-09-30

    The low-dose dexamethasone suppression test (DST) is one of the commonly used initial tests for endogenous Cushing's syndrome (CS). However, there are two loading dose regimens (0.5-mg and 1-mg), which may cause some confusion in daily practice in Japan; furthermore, there are no reports regarding whether 0.5-mg DST is a better loading dose for detecting adrenal subclinical CS (SCS) based on the plasma dexamethasone (DEX) levels. Therefore, the aims of this study were (a) to develop a novel assay to measure DEX by using liquid chromatography tandem-mass spectrometry (LC-MS/MS) and (b) to compare between the 0.5-mg and 1-mg DST for SCS diagnosis based on the DEX levels. The study retrospectively analyzed 52 consecutive subjects hospitalized for diagnosis of adrenal incidentaloma but who did not exhibit an overt CS phenotype; eight (15.4%) patients were affected with adrenal SCS. Inter-individual variability of DEX levels after the DST was high, but intra-individual variability was low. DEX levels after 1-mg loading in each patient was around two times higher than those after 0.5-mg loading (ρ = 0.853 and p DST, respectively (p DST, respectively, did not exhibit DEX levels >2.2 ng/mL. These results clearly indicate that the 1-mg DST is superior to 0.5-mg loading for the diagnosis of adrenal SCS.

  18. Diagnostic usefulness of 3 tesla MRI of the brain for cushing disease in a child.

    Science.gov (United States)

    Ono, Erina; Ozawa, Ayako; Matoba, Kaori; Motoki, Takanori; Tajima, Asako; Miyata, Ichiro; Ito, Junko; Inoshita, Naoko; Yamada, Syozo; Ida, Hiroyuki

    2011-10-01

    It is sometimes difficult to confirm the location of a microadenoma in Cushing disease. Recently, we experienced an 11-yr-old female case of Cushing disease with hyperprolactinemia. She was referred to our hospital because of decrease of height velocity with body weight gain. On admission, she had typical symptoms of Cushing syndrome. Although no pituitary microadenomas were detected on 1.5 Tesla MRI of the brain, endocrinological examinations including IPS and CS sampling were consistent with Cushing disease with hyperprolactinemia. Oral administration of methyrapone instead of neurosurgery was started after discharge, but subsequent 3 Tesla MRI of the brain clearly demonstrated a 3-mm less-enhanced lesion in the left side of the pituitary gland. Finally, transsphenoidal surgery was performed, and a 3.5-mm left-sided microadenoma was resected. Compared with 1.5 Tesla MRI, 3 Tesla MRI offers the advantage of a higher signal to noise ratio (SNR), which provides higher resolution and proper image quality. Therefore, 3 Tesla MRI is a very useful tool to localize microadenomas in Cushing disease in children as well as in adults. It will be the first choice of radiological examinations in suspected cases of Cushing disease.

  19. Health care resource use and costs among patients with cushing disease.

    Science.gov (United States)

    Swearingen, Brooke; Wu, Ning; Chen, Shih-Yin; Pulgar, Sonia; Biller, Beverly M K

    2011-01-01

    To assess health care costs associated with Cushing disease and to determine changes in overall and comorbidity-related costs after surgical treatment. In this retrospective cohort study, patients with Cushing disease were identified from insurance claims databases by International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) codes for Cushing syndrome (255.0) and either benign pituitary adenomas (227.3) or hypophysectomy (07.6×) between 2004 and 2008. Each patient with Cushing disease was age- and sex-matched with 4 patients with nonfunctioning pituitary adenomas and 10 population control subjects. Comorbid conditions and annual direct health care costs were assessed within each calendar year. Postoperative changes in health care costs and comorbidity-related costs were compared between patients presumed to be in remission and those with presumed persistent disease. Of 877 identified patients with Cushing disease, 79% were female and the average age was 43.4 years. Hypertension, diabetes mellitus, and hyperlipidemia were more common among patients with Cushing disease than in patients with nonfunctioning pituitary adenomas or in control patients (PCushing disease had significantly higher total health care costs (2008: $26 440 [Cushing disease] vs $13 708 [nonfunctioning pituitary adenomas] vs $5954 [population control], Pdisease-related costs with remission. A significant increase in postoperative health care costs was observed in those patients not in remission. Patients with Cushing disease had more comorbidities than patients with nonfunctioning pituitary adenomas or control patients and incurred significantly higher annual health care costs; these costs decreased after successful surgery and increased after unsuccessful surgery.

  20. Usefulness of composite methionine-positron emission tomography/3.0-tesla magnetic resonance imaging to detect the localization and extent of early-stage Cushing adenoma.

    Science.gov (United States)

    Ikeda, Hidetoshi; Abe, Takehiko; Watanabe, Kazuo

    2010-04-01

    Fifty to eighty percent of Cushing disease is diagnosed by typical endocrine responses. Recently, the number of diagnoses of Cushing disease without typical Cushing syndrome has been increasing; therefore, improving ways to determine the localization of the adenoma and making an early diagnosis is important. This study was undertaken to determine the present diagnostic accuracy for Cushing microadenoma and to compare the differences in diagnostic accuracy between MR imaging and PET/MR imaging. During the past 3 years the authors analyzed the diagnostic accuracy in a series of 35 patients with Cushing adenoma that was verified by surgical pituitary exploration. All 35 cases of Cushing disease, including 20 cases of "overt" and 15 cases of "preclinical" Cushing disease, were studied. Superconductive MR images (1.5 or 3.0 T) and composite images from FDG-PET or methionine (MET)-PET and 3.0-T MR imaging were compared with the localization of adenomas verified by surgery. The diagnostic accuracy of superconductive MR imaging for detecting the localization of Cushing microadenoma was only 40%. The causes of unsatisfactory results for superconductive MR imaging were false-negative results (10 cases), false-positive results (6 cases), and instances of double pituitary adenomas (3 cases). In contrast, the accuracy of microadenoma localization using MET-PET/3.0-T MR imaging was 100% and that of FDG-PET/3.0-T MR imaging was 73%. Moreover, the adenoma location was better delineated on MET-PET/MR images than on FDG-PET/MR images. There was no significant difference in maximum standard uptake value of adenomas evaluated by MET-PET between preclinical Cushing disease and overt Cushing disease. Composite MET-PET/3.0-T MR imaging is useful for the improvement of the delineation of Cushing microadenoma and offers high-quality detectability for early-stage Cushing adenoma.

  1. Major Harvey Cushing's difficulties with the British and American armies during World War I.

    Science.gov (United States)

    Carey, Michael E

    2014-08-01

    This historical review explores Harvey Cushing's difficulties with both the British and American armies during his World War I service to definitively examine the rumor of his possible court martial. It also provides a further understanding of Cushing the man. While in France during World War I, Cushing was initially assigned to British hospital units. This service began in May 1917 and ended abruptly in May 1918 when the British cashiered him for repeated censorship violations. Returning to American command, he feared court martial. The army file on this matter (retrieved from the United States National Archives) indicates that US Army authorities recommended that Cushing be reprimanded and returned to the US for his violations. The army carried out neither recommendation, and no evidence exists that a court martial was considered. Cushing's army career and possible future academic life were protected by the actions of his surgical peers and Merritte Ireland, Chief Surgeon of the US Army in France. After this censorship episode, Cushing was made a neurosurgical consultant but was also sternly warned that further rule violations would not be tolerated by the US Army. Thereafter, despite the onset of a severe peripheral neuropathy, probably Guillian Barré's syndrome, Cushing was indefatigable in ministering to neurosurgical needs in the US sector in France. Cushing's repeated defying of censorship regulations reveals poor judgment plus an initial inability to be a "team player." The explanations he offered for his censorship violations showed an ability to bend the truth. Cushing's war journal is unclear as to exactly what transpired between him and the British and US armies. It also shows no recognition of the help he received from others who were instrumental in preventing his ignominious removal from service in France. Had that happened, his academic future and ability to train future neurosurgical leaders may have been seriously threatened. Cushing's foibles

  2. Sustained remission of Cushing's disease with mitotane and pituitary irradiation

    International Nuclear Information System (INIS)

    Schteingart, D.E.; Tsao, H.S.; Taylor, C.I.; McKenzie, A.; Victoria, R.; Therrien, B.A.

    1980-01-01

    Low doses of mitotane were given orally to 36 patients with Cushing's disease, concurrently with or after pituitary cobalt irradiation. Clinical and biochemical remission occurred in 29. The response to treatment occurred early in 17 patients and late in 12. The different pattern of response to mitotane was not related to the dose given or to its serum level. Early biochemical indicators of adrenal suppression with mitotane were a sharp decrease in adrenal response to the infusion of ACTH and in plasma levels of dehydroepiandrosterone sulfate. Although mitotane was given together with pituitary irradiation, initial remission was due mainly to the adrenal effect of mitotane. Plasma ACTH levels were still elevated when cortisol had returned to normal. In seventeen of the 29 patients who responded to treatment drug therapy has been discontinued, and they remain in remission of Cushing's syndrome. Side-effects have been dose dependent, with anorexia, nausea, decreased memory, and gynecomastia in men being the commonest

  3. The treatment of Cushing's disease

    International Nuclear Information System (INIS)

    Lamberts, S.W.J.; Lange, S.A. de; Singh, R.; Fermin, H.; Klijn, J.G.M.; Jong, F.H. de; Birkenhaeger, J.C.

    1980-01-01

    This study compares the results of transsphenoidal operation in 11 consecutive patients with Cushing's disease with those obtained in 29 patients by unilateral adrenalectomy followed by external pituitary irradiation (4500 rad). It is concluded that transsphenoidal surgery is the treatment of choice but if no transient adrenal insufficiency develops after removal of the pituitary (micro)adenoma, then additional external pituitary irradiation seems suitable treatment. (Auth.)

  4. Hypoadrenia following adrenal venography in Cushing's disease

    International Nuclear Information System (INIS)

    Goth, M.; Szilagy, G.; Irsy, G.; Szabolcs, I.; Berentey, E.; Molnar, F.; Magyar, E.

    1984-01-01

    Three patients with Cushing's disease are reported. In two patients the hyperfunction of the adrenal glands creased following selective adrenal venography, moreover hypoadrenia has developed. In the third case necrosis following venography was proved by surgery and confirmed by histology. Regarding the therapeutic possibilities of Cushing's diesease the use of this diagnostic procedure is taken into consideration but not recommended. (orig.)

  5. Pituitary gland imaging in Cushing's disease

    International Nuclear Information System (INIS)

    Bonneville, J.F.; Cattin, F.; Bonneville, F.; Schillo, F.; Jacquet, G.

    2003-01-01

    Specific MR techniques are r-required for optimal detection of adrenocorticotropic hormone secreting adenomas responsible for Cushing's disease. Adequate MR sequences, high resolution coronal T1 and T2 - weighted images, dynamic MR imaging, post-gadolinium delayed images, dose of gadolinium adjusted for each sequence can routinely demonstrate pituitary adenomas less than 3 mm in Cushing's disease. (authors)

  6. Diagnosis of Cushing's disease in children: a challenge for the radiologist

    International Nuclear Information System (INIS)

    Kalifa, G.; Adamsbaum, C.; Carel, J.C.; Andre, C.; Bougneres, P.F.; Chaussain, J.L.

    1994-01-01

    Cushing's disease is the most common cause of Cushing's syndrome in children and is almost always related to over secretion of ACTH by the pituitary gland. It is important to identify a secreting adenoma prior to surgery. Seven cases studied with MRI are reviewed. In five cases the MRI findings suggested adenoma. Three secreting adenomas were confirmed at surgery. One case was in fact a cyst of the pars intermedia, and nothing could be found in the last case. Two patients presented with apparently normal findings on MRI, which was confirmed. There is a close correlation between identifying an adenoma and the success of surgery. (orig.)

  7. Macro-adénome surrénalien masquant une hyperplasie micronodulaire des surrénales au cours d’un syndrome de Cushing ACTH indépendant et hypokaliémie réfractaire

    Science.gov (United States)

    Alaya, Wafa; Bouchahda, Haifa; Fradi, Asma; Zantour, Baha; Sfar, Mohamed Habib

    2017-01-01

    L’association d’une hyperplasie micronodulaire des surrénales à un macro-adénome surrénalien au cours d’un syndrome de Cushing (SC) ACTH-indépendant est rare et suscite beaucoup de questions. Nous en rapportons un cas. Patiente âgée de 35 ans nous a été adressée pour suspicion d’un SC devant une obésité facio-tronculaire avec hypokaliémie d’origine rénale. Les explorations hormonales ont objectivé un SC ACTH-indépendant et le scanner abdominal a montré macro-adénome surrénalien gauche de 2cm avec une surrénale droite normale. La patiente a eu une surrénalectomie gauche. Cependant, l’hypercortisolisme et l’hypokaliémie ont persisté. L’examen anatomopathologique a permis de conclure à un adénome corticosurrénalien de 2,5cm, avec une hyperplasie micronodulaire non pigmentée des surrénales (i-MAD). La patiente a eu une surrénalectomie droite, suivie d’une insuffisance surrénalienne. Paradoxalement, l’hypokaliémie a persisté sans autres anomalies ni explication évidente (magnésémie, pH sanguin et urinaire, bilan phosphocalcique et échographie rénale normaux) nécessitant une supplémentation parentérale puis orale par du KCl. The association between micronodular adrenal hyperplasia and macro-adrenal adenoma in patients with ACTH-independent Cushing’s syndrome (CS) is rare and raises a lot of questions. We here report the case of a 35-year old female patient referred to us for suspected CS due to central obesity associated with renal hypokalaemia. Hormonal explorations objectified ACTH-independent CS and abdominal CT scan showed left macro-adrenal adenoma measuring 2cm in diameter associated with normal right adrenal gland. The patient underwent left adrenalectomy. However, hypercortisolism and hypokalaemia persisted. Anatomo-pathological examination allowed the diagnisis of adrenocortical adenoma measuring 2,5cm in diameter associated with unpigmented micronodular adrenal hyperplasia (i-Mad). The patient

  8. Peptide Receptor Radionuclide Therapy with 177Lu-DOTATATE for Metastatic Neuroendocrine Tumor Occurring in Association with Multiple Endocrine Neoplasia Type 1 and Cushing's Syndrome

    OpenAIRE

    Naik, Chinna; Basu, Sandip

    2017-01-01

    Neuroendocrine tumor (NET) occurring in association with other endocrine syndromes forms a distinct entity. The aim was to assess the therapy response profile of the routine peptide receptor radionuclide therapy (PRRT) in this relatively uncommon but clinically challenging subgroup of patients. A retrospective analysis was undertaken from the case records from those who were treated with 177Lu-DOTATATE for metastatic NET. In addition to assessing the therapeutic efficacy, emphasis was also gi...

  9. Cushing's disease in the elderly patient

    Directory of Open Access Journals (Sweden)

    Maritza Cardosa Samón

    2012-05-01

    Full Text Available Introduction: Cushing's disease arises from increased ACTH secretion from a pituitary tumor that stimulates the area fasciculata of the adrenal cortex and produces hypersecretion of glucocorticoids. Objectives: To explain the clinical and humoral manifestations of Cushing's disease in elderly adults. Methods: The article is a descriptive and retrospective study of a case report on a 62 year old patient that is admitted to our Center with manifestations of facial plethora and centripetal obesity. Onset was characterized by hypertension and diabetes mellitus. Definitive diagnosis was Cushing's disease from a macroadenoma with increased ACTH secretion.

  10. Cyclin E-Mediated Human Proopiomelanocortin Regulation as a Therapeutic Target for Cushing Disease.

    Science.gov (United States)

    Liu, Ning-Ai; Araki, Takako; Cuevas-Ramos, Daniel; Hong, Jiang; Ben-Shlomo, Anat; Tone, Yukiko; Tone, Masahide; Melmed, Shlomo

    2015-07-01

    /E2F1 signaling is a previously unappreciated molecular mechanism underlying neuroendocrine regulation of the hypothalamic-pituitary-adrenal axis, providing a subcellular therapeutic target for small molecule cyclin-dependent kinase 2 inhibitors of pituitary ACTH-dependent hypercortisolism, ie, Cushing disease.

  11. Treatment of Cushing disease: overview and recent findings

    Directory of Open Access Journals (Sweden)

    Tatiana Mancini

    2010-10-01

    Full Text Available Tatiana Mancini1, Teresa Porcelli2, Andrea Giustina21Department of Internal Medicine and Medical Specialties, San Marino Hospital, San Marino, Republic of San Marino, 2Department of Medical and Surgical Sciences, University of Brescia, Brescia, ItalyAbstract: Endogenous Cushing syndrome is an endocrine disease caused by excessive secretion of adrenocorticotropin hormone in approximately 80% of cases, usually by a pituitary corticotroph adenoma (Cushing disease [CD]. It is a heterogeneous disorder requiring a multidisciplinary and individualized approach to patient management. The goals of treatment of CD include the reversal of clinical features, the normalization of biochemical changes with minimal morbidity, and long-term control without recurrence. Generally, the treatment of choice is the surgical removal of the pituitary tumor by transsphenoidal approach, performed by an experienced surgeon. Considering the high recurrence rate, other treatments should be considered. Second-line treatments include more radical surgery, radiation therapy, medical therapy, and bilateral adrenalectomy. Drug treatment has been targeted at the hypothalamic or pituitary level, at the adrenal gland, and also at the glucocorticoid receptor level. Frequently, medical therapy is performed before surgery to reduce the complications of the procedure, reducing the effects of severe hypercortisolism. Commonly, in patients in whom surgery has failed, medical management is often essential to reduce or normalize the hypercortisolemia, and should be attempted before bilateral adrenalectomy is considered. Medical therapy can be also useful in patients with CD while waiting for pituitary radiotherapy to take effect, which can take up to 10 years or more. So far, results of medical treatment of CD have not been particularly relevant; however, newer tools promise to change this scenario. The aim of this review is to analyze the results and experiences with old and new medical

  12. What Are the Treatments for Cushing's Syndrome?

    Science.gov (United States)

    ... NICHD Research Information Find a Study More Information Amenorrhea About NICHD Research Information Find a Study More ... FAQs » Related A-Z Topics Adrenal Gland Disorders Amenorrhea Pituitary Tumors NICHD News Spotlights Spotlight: Selected NICHD ...

  13. What Are the Symptoms of Cushing's Syndrome?

    Science.gov (United States)

    ... NICHD Research Information Find a Study More Information Amenorrhea About NICHD Research Information Find a Study More ... diagnosed? » Related A-Z Topics Adrenal Gland Disorders Amenorrhea Pituitary Tumors NICHD News Spotlights Spotlight: Selected NICHD ...

  14. Cushing syndrome due to adrenal tumor

    Science.gov (United States)

    ... diabetes mellitus High blood pressure Increased cholesterol and triglycerides Women often have: Excess hair growth on the ... Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, ...

  15. Cabergoline therapy for Cushing disease throughout pregnancy.

    Science.gov (United States)

    Woo, Irene; Ehsanipoor, Robert M

    2013-08-01

    Cushing disease during pregnancy is rare and is associated with significant maternal and fetal morbidity and mortality. Transsphenoidal pituitary surgery is the first-line therapy; however, in cases of failed surgery or in patients who are not surgical candidates, medical therapy has been used to control symptoms. A 29-year-old woman with Cushing disease and a noncurative transsphenoidal pituitary surgery was successfully treated with cabergoline, a dopamine agonist. After approximately 1 year of therapy, she became pregnant. She was maintained on high-dose cabergoline throughout her pregnancy and had an uncomplicated antenatal course. She went into spontaneous labor at 38 weeks of gestation and delivered a healthy female neonate. Cabergoline can be used to manage Cushing disease successfully during pregnancy with an opportunity for a favorable outcome.

  16. Subtle cognitive impairments in patients with long-term cure of Cushing's disease

    NARCIS (Netherlands)

    Tiemensma, Jitske; Kokshoorn, Nieke E.; Biermasz, Nienke R.; Keijser, Bart-Jan S. A.; Wassenaar, Moniek J. E.; Middelkoop, Huub A. M.; Pereira, Alberto M.; Romijn, Johannes A.

    2010-01-01

    Active Cushing's disease is associated with cognitive impairments. We hypothesized that previous hypercortisolism in patients with Cushing's disease results in irreversible impairments in cognitive functioning. Therefore, our aim was to assess cognitive functioning after long-term cure of Cushing's

  17. Cushing's disease: diagnostic evaluation, therapeutics and prognostic

    International Nuclear Information System (INIS)

    Pereira, M.A.A.; Jugue, S.M.; Moura, O.M.D.; Gross, K.; Halpern, A.; Nicolau, W.; Liberman, B.; Bloise, W.; Mendonca, B.; Cabral, N.D.; Marino Junior, R.; Wajchenberg, B.L.

    1992-01-01

    Aspects as clinical, diagnosis, laboratory tests and radiological evaluations concerning Cushing's disease are analysed in 56 patients. Several options of non medicamental therapy are presented. The relationship between preoperative data (hormonal and radiological information), postoperative findings and hypophyseal adenomectomy is discussed. (M.A.C.)

  18. Cushing Disease in a patient with Multiple Endocrine Neoplasia type 2B.

    Science.gov (United States)

    Kasturi, Kannan; Fernandes, Lucas; Quezado, Martha; Eid, Mary; Marcus, Leigh; Chittiboina, Prashant; Rappaport, Mark; Stratakis, Constantine A; Widemann, Brigitte; Lodish, Maya

    2017-06-01

    Multiple endocrine neoplasia type 2B (MEN2B) is a rare autosomal-dominant cancer syndrome characterized in part by metastatic medullary thyroid cancer (MTC) and pheochromocytoma. Cushing disease is a rare cause of endogenous hypercortisolism in children. We describe a 21-year-old African-American male who was diagnosed at age 10 with an ACTH-secreting pituitary microadenoma. At age 16 he developed medullary thyroid cancer and was found to have multiple endocrine neoplasia type 2B with the characteristic M918T mutation of the RET proto-oncogene. Following thyroidectomy, he was initiated on Vandetanib, a tyrosine kinase inhibitor, and has since had stable disease over the last 5 years. Our patient is the first individual with MEN2B to be described with Cushing disease. The RET oncogene may play a role in pituitary tumorigenesis; alternatively, the coexistence of these two entities may represent an extremely rare coincidence.

  19. Medical treatment of Cushing disease: new targets, new hope.

    Science.gov (United States)

    Fleseriu, Maria

    2015-03-01

    This article provides an update on current medical therapies for the treatment of Cushing disease. This information will be of value in determining patients' suitability for certain medical treatments. An approach of combining drugs from the same or different classes could potentially increase the number of patients in whom Cushing can be controlled while minimizing adverse effects, although larger studies are needed. Successful clinical management of patients with Cushing disease remains a challenge. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. Dermatofitose por Tricophyton rubrum como infecção oportunista em pacientes com doença de Cushing Dermatophytosis caused by Tricophyton rubrum as an opportunistic infection in patients with Cushing disease

    Directory of Open Access Journals (Sweden)

    Isy Peixoto

    2010-12-01

    Full Text Available O dermatófito Trichophyton rubrum é um agente comum nas micoses superficiais, podendo apresentar lesões extensas pauci-inflamatórias de evolução crônica, especialmente em imunocomprometidos. O hipercortisolismo, na síndrome de Cushing, aumenta o risco de infecções, resultado do efeito imunossupressor dos glicocorticóides. Os casos relatados apresentam duas formas distintas de dermatofitose, em pacientes com doença de Cushing, causadas por Tricophyton rubrum e posterior remissão após normalização da cortisolemia.Trichophyton rubrum is a common agent found in superficial mycoses, which present ample nonin?ammatory lesions, with chronic evolution, especially in immunocompromised patients. The hypercortisolism in Cushing's syndrome increases the risk of infections as a result of the immunosuppressive effect of glucocorticoids. The reported cases here refer to two different types of dermatophytosis caused by Trichophyton rubrum in patients with Cushing's disease, resistant to antifungal treatment. The disease remitted after the levels of cortisol went back to normal.

  1. Pituitary Apoplexy due to the Diagnostic Test in a Cushing"s Disease Patient.

    Science.gov (United States)

    Kuzu, Fatih; Unal, Mustafa; Gul, Sanser; Bayraktaroglu, Taner

    2018-01-01

    Pituitary apoplexy is a medical condition that needs urgent diagnosis and treatment. It may occur spontaneously or may be precipitated by a variety of reasons including dynamic endocrine tests. Although pituitary apoplexy is usually seen in nonfunctional pituitary adenoma, it can also be seen in ACTH secreting macroadenomas. ACTH secreting adenomas present usually as microadenomas and in these patients apoplexy is rarely seen. In this paper we present a 30-year-old male patient with a history of Cushing"s disease who suffered from pituitary apoplexy after the 1 mg dexamethasone suppression test. He underwent endoscopic endonasal transsphenoidal surgery and his symptoms and signs were significantly improved.

  2. Computerized tomography of adrenal glands in the investigation of Cushing's syn

    International Nuclear Information System (INIS)

    Abucham Filho, J.; Albertotti, C.; Kater, C.E.; Vieira, J.G.H.; Chacra, A.R.

    1983-01-01

    Computerized tomography of the adrenal glands was performed in 10 patients with Cushing's syndrome using a G.E. 8800 CT/T Body Scanner. The tomographic findings of unilateral adrenal masses in 4 patients were confirmed by surgery. In the remaining 6 patients, computerized tomography did not identify any masses, and both glands were well visualized and showed normal shape. In these patients, measurement of the adrenal glands revealed both normal (n=1) and enlarged glands (n=5). The findings of adrenalectomy (n=4) or transphenoidal surgery and follow-up (n=2) established the diagnosis of adrenal phyperplasia in all patients. (Author) [pt

  3. Pituitary gland imaging in Cushing's disease; IRM - Maladie de Cushing. L'imagerie hypophysaire dans la maladie de Cushing

    Energy Technology Data Exchange (ETDEWEB)

    Bonneville, J.F.; Cattin, F.; Bonneville, F.; Schillo, F.; Jacquet, G. [Centre Hospitalier Universitaire, 25 - Besancon (France)

    2003-02-01

    Specific MR techniques are r-required for optimal detection of adrenocorticotropic hormone secreting adenomas responsible for Cushing's disease. Adequate MR sequences, high resolution coronal T1 and T2 - weighted images, dynamic MR imaging, post-gadolinium delayed images, dose of gadolinium adjusted for each sequence can routinely demonstrate pituitary adenomas less than 3 mm in Cushing's disease. (authors)

  4. Syndromes that Link the Endocrine System and Genitourinary Tract.

    Science.gov (United States)

    Özlük, Yasemin; Kılıçaslan, Işın

    2015-01-01

    The endocrine system and genitourinary tract unite in various syndromes. Genitourinary malignancies may cause paraneoplastic endocrine syndromes by secreting hormonal substances. These entities include Cushing`s syndrome, hypercalcemia, hyperglycemia, polycythemia, hypertension, and inappropriate ADH or HCG production. The most important syndromic scenarios that links these two systems are hereditary renal cancer syndromes with specific genotype/phenotype correlation. There are also some very rare entities in which endocrine and genitourinary systems are involved such as Carney complex, congenital adrenal hyperplasia and Beckwith-Wiedemann syndrome. We will review all the syndromes regarding manifestations present in endocrine and genitourinary organs.

  5. A 7-month-old infant with cushing's disease

    International Nuclear Information System (INIS)

    Abbas, R.; Hamid, M.H.; Sarwar, M.; Butt, T.A.; Qureshi, A.; Malik, N.

    2013-01-01

    Cushings disease in children is not rare but in infants it is quite rare and an important medical condition needing proper line of investigations and management options. Craniopharyngioma as a cause of Cushings disease is well reported and practical inference of the condition is of clinical importance. Craniopharyngioma generally affects children at 5 - 10 years of age and is rarely seen in infancy. It usually manifests as endocrinological deficits such as short stature, delayed puberty, and obesity. We report the case of a 7 months old infant who presented with obesity and Cushing disease associated with craniopharyngioma. (author)

  6. Inpatient Complications After Transsphenoidal Surgery in Cushing's Versus Non-Cushing's Disease Patients.

    Science.gov (United States)

    Svider, Peter F; Raikundalia, Milap D; Pines, Morgan J; Baredes, Soly; Folbe, Adam J; Liu, James K; Eloy, Jean Anderson

    2016-01-01

    Transsphenoidal surgery (TSS) harbors a potential for hypopituitarism, cerebrospinal fluid (CSF) leaks, and other complications. We utilized the Nationwide Inpatient Sample Database (NIS) to compare inpatient complication rates between Cushing's disease (CD) and non-Cushing's disease (NCD) patients undergoing TSS. Inpatient hospitalization data for 960 CD and 12 110 NCD patients who underwent TSS between 2002 and 2010 were accessed. Demographic information, outcomes, and complication rates were evaluated. Patients with CD had a female predilection (81.7%) and were younger (40.5 ± 14.4 years) than NCD patients (47.8% female; 52.1 ± 16.3 years) (P iatrogenic pituitary disorders overall. No differences were noted in the rate of early CSF leaks between postoperative TSS CD and NCD patients. Postoperative diabetes insipidus did not significantly differ between groups after adjusting for confounders. Only odds of urinary/renal complications in CD patients was significant after adjustment. © The Author(s) 2015.

  7. Iatrogenic Cushing’s syndrome and Secondary Adrenal Insufficiency in an HIV Patientreceiving Fluticasone and Ritonavir

    Directory of Open Access Journals (Sweden)

    Imro Vlasveld

    2014-04-01

    Full Text Available Objectives: To illustrate that the protease inhibitor (PI ritonavir, widely used as part of the treatment for HIV, might cause drug–drug interactions with inhaled corticosteroids. Material and methods: A case report is presented. Results: An HIV-positive patient presented with gradually changing body composition that was ascribed to lipodystrophy. Finally, iatrogenic Cushing's syndrome with secondary adrenal insufficiency was diagnosed due to a drug–drug interaction of ritonavir and fluticasone. Conclusion: Lipodystrophy might mimic Cushing's syndrome. The combination of ritonavir and inhaled fluticasone may lead to systemic steroid excess causing Cushing's syndrome and secondary adrenal insufficiency.

  8. POSTSURGICAL RECURRENT CUSHING DISEASE: CLINICAL BENEFIT OF EARLY INTERVENTION IN PATIENTS WITH NORMAL URINARY FREE CORTISOL.

    Science.gov (United States)

    Carroll, Ty B; Javorsky, Bradley R; Findling, James W

    2016-10-01

    To assess the performance of biochemical markers in the detection of recurrent Cushing disease (CD), as well as the potential benefit of early intervention in recurrent CD patients with elevated late-night salivary cortisol (LNSC) and normal urinary free cortisol (UFC). The design was a single-center, retrospective chart review. Patients treated by the authors from 2008-2013 were included. Recurrence was defined by postsurgical remission of CD with subsequent abnormal LNSC, UFC, or dexamethasone suppression test (DST). We identified 15 patients with postsurgical recurrent CD after initial remission; all but one underwent testing with LNSC, DST, and UFC. Although 12 of 15 patients had normal UFC at time of recurrence, DST was abnormal in 11 of 15, and all 14 patients with LNSC results had ≥1 elevated measurement. Nine patients (7 with normal UFC) showed radiologic evidence of a pituitary tumor at time of recurrence. Among the 14 patients with available follow-up data, 12 have demonstrated significant improvement since receiving treatment. Five patients underwent repeat pituitary surgery and 4 achieved clinical and biochemical remission. Eight patients received mifepristone or cabergoline, and 6 showed clinical and/or biochemical improvement. Three patients (2 with prior mifepristone) underwent bilateral adrenalectomy and 2 demonstrated significant clinical improvements. LNSC is more sensitive than UFC or DST for detection of CD recurrence. Prompt intervention when LNSC is elevated, despite normal UFC, may yield significant clinical benefit for many patients with CD. Early treatment for patients with recurrent CD should be prospectively evaluated, utilizing LNSC elevation as an early biochemical marker. ACTH = adrenocorticotropic hormone CD = Cushing disease CS = Cushing syndrome CV = coefficient of variation DST = dexamethasone suppression test IPSS = inferior petrosal sinus sampling LNSC = late-night salivary cortisol QoL = quality of life TSS = transsphenoidal

  9. Sustained remission of Cushing's disease with mitotane and pituitary irradiation

    Energy Technology Data Exchange (ETDEWEB)

    Schteingart, D.E.; Tsao, H.S.; Taylor, C.I.; McKenzie, A.; Victoria, R.; Therrien, B.A.

    1980-05-01

    Low doses of mitotane were given orally to 36 patients with Cushing's disease, concurrently with or after pituitary cobalt irradiation. Clinical and biochemical remission occurred in 29. The response to treatment occurred early in 17 patients and late in 12. The different pattern of response to mitotane was not related to the dose given or to its serum level. Early biochemical indicators of adrenal suppression with mitotane were a sharp decrease in adrenal response to the infusion of ACTH and in plasma levels of dehydroepiandrosterone sulfate. Although mitotane was given together with pituitary irradiation, initial remission was due mainly to the adrenal effect of mitotane. Plasma ACTH levels were still elevated when cortisol had returned to normal. In seventeen of the 29 patients who responded to treatment drug therapy has been discontinued, and they remain in remission of Cushing's syndrome. Side-effects have been dose dependent, with anorexia, nausea, decreased memory, and gynecomastia in men being the commonest.

  10. Single-Session CT-Guided Percutaneous Microwave Ablation of Bilateral Adrenal Gland Hyperplasia Due to Ectopic ACTH Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Sarma, Asha, E-mail: ashasarma@gmail.com; Shyn, Paul B., E-mail: pshyn@partners.org [Brigham and Women’s Hospital, Department of Radiology (United States); Vivian, Mark A. [University of Manitoba, Department of Radiology (Canada); Ng, Ju-Mei [Brigham and Women’s Hospital, Department of Anesthesiology (United States); Tuncali, Kemal [Brigham and Women’s Hospital, Department of Radiology (United States); Lorch, Jorchen H. [Dana Farber Cancer Institute, Department of Medicine (United States); Zaheer, Sarah N.; Gordon, Michael S. [Brigham and Women’s Hospital, Department of Endocrinology (United States); Silverman, Stuart G. [Brigham and Women’s Hospital, Department of Radiology (United States)

    2015-10-15

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: One was not a surgical candidate, and the other had undergone unsuccessful surgery. Following the procedure, both patients achieved substantial decreases in serum cortisol, symptomatic improvement, and decreased anti-hypertensive medication requirements.

  11. ACTH-Secreting tumor of cecum. Case report

    Directory of Open Access Journals (Sweden)

    N S Kuznetsov

    2013-06-01

    Full Text Available Ectopic hormonesecreting of cecum are rare. We report the case of a 52yearold woman with a typical clin ical picture of Cushing disease. Diagnostic tests confirmed ACTH dependent Cushing’s syndrome, which is caused by neuroendocrine tumor of the cecum. Due to the rarity of the disease, we believe it appropriate to share our experience of this group of patients.

  12. BIOCHEMICAL CONTROL DURING LONG-TERM FOLLOW-UP OF 230 ADULT PATIENTS WITH CUSHING DISEASE: A MULTICENTER RETROSPECTIVE STUDY.

    Science.gov (United States)

    Geer, Eliza B; Shafiq, Ismat; Gordon, Murray B; Bonert, Vivien; Ayala, Alejandro; Swerdloff, Ronald S; Katznelson, Laurence; Lalazar, Yelena; Manuylova, Ekaterina; Pulaski-Liebert, Karen J; Carmichael, John D; Hannoush, Zeina; Surampudi, Vijaya; Broder, Michael S; Cherepanov, Dasha; Eagan, Marianne; Lee, Jackie; Said, Qayyim; Neary, Maureen P; Biller, Beverly M K

    2017-08-01

    Cushing disease (CD) results from excessive exposure to glucocorticoids caused by an adrenocorticotropic hormone-secreting pituitary tumor. Inadequately treated CD is associated with significant morbidity and elevated mortality. Multicenter data on CD patients treated in routine clinical practice are needed to assess treatment outcomes in this rare disorder. The study purpose was to describe the burden of illness and treatment outcomes for CD patients. Eight pituitary centers in four U.S. regions participated in this multicenter retrospective chart review study. Subjects were CD patients diagnosed at ≥18 years of age within the past 20 years. Descriptive statistical analyses were conducted to examine presenting signs, symptoms, comorbidities, and treatment outcomes. Of 230 patients, 79% were female (median age at diagnosis, 39 years; range, 18 to 78 years). Length of follow-up was 0 to 27.5 years (median, 1.9 years). Pituitary adenomas were 0 to 51 mm. The most common presenting comorbidities included hypertension (67.3%), polycystic ovary syndrome (43.5%), and hyperlipidemia (41.5%). Biochemical control was achieved with initial pituitary surgery in 41.4% patients (91 of 220), not achieved in 50.0% of patients (110 of 220), and undetermined in 8.6% of patients (19 of 220). At the end of follow-up, control had been achieved with a variety of treatment methods in 49.1% of patients (110 of 224), not achieved in 29.9% of patients (67 of 224), and undetermined in 21.0% of patients (47 of 224). Despite multiple treatments, at the end of follow-up, biochemical control was still not achieved in up to 30% of patients. These multicenter data demonstrate that in routine clinical practice, initial and long-term control is not achieved in a substantial number of patients with CD. BLA = bilateral adrenalectomy CD = Cushing disease CS = Cushing syndrome eCRF = electronic case report form MRI = magnetic resonance imaging PCOS = polycystic ovary syndrome.

  13. Thin-section CT of Cushing's disease

    International Nuclear Information System (INIS)

    Takahashi, Tatsuo; Kuwayama, Akio; Katoh, Tetsuo; Ichihara, Kaoru; Kageyama, Naoki; Nakamura, Koji.

    1983-01-01

    Using 1.5 mm contiguous sections with a GE CT/T 8800 scanner, we investigated the sellar regions of 22 cases of Cushing's diseases which had been diagnosed endocrinologically. Each sellar turcica was normal in size, and in only 5 cases were there significant findings on 2 mm-thick sellar-floor polytomography. Nine tumors appeared as regions of a hypodense area, and three tumors were diagnosed by indirect signs, for example, stalk deviation and diaphragmatic plane asymmetry. The other 10 cases, especially those previously operated on or irradiated, were diagnosed as falsely positive or negative. Because it is best of the microadenomas appear hypodense within the strongly contrast-enhanced anterior pituitary glands, it is better for scans to be obtained immediately after rapid intravenous contrast infusion. Hypodense areas of microadenomas are best demonstrated on direct coronal scans or reversed scans of 1.5 mm-thickness thin-slice sections. By these methods, microadenomas, if they are over 5-6 mm in diameter, can appear as hypodense. Sellar floor findings by means of thin-section CT were more sensitive than those of polytomography and had more advantages in local diagnosis. If the tumor were over 4 mm in diameter, local changes in the sellar floor could be demonstrated by thin-section CT, but by polytomography no changes in the sellar floor could be demonstrated until the tumor size reached 6 mm. (author)

  14. Candida albicans endophthalmitis in a patient with a non-functioning pituitary adenoma evolving into Cushing׳s disease: A case report

    Directory of Open Access Journals (Sweden)

    Eun Kyoung Lee

    2014-10-01

    Full Text Available A 53-year-old woman presented with complaints of blurred vision in the left eye. She had been treated for recurrent non-functioning pituitary adenoma (NFPA. A vitreous biopsy followed by histopathologic examination showed the presence of Candida albicans. Meanwhile, Cushing׳s disease was diagnosed and gamma knife surgery was performed. Vitrectomy and antifungal treatment improved ocular infection and inflammation. Herein, we describe the first case of C. albicans endophthalmitis in a patient with NFPA evolving into Cushing׳s disease.

  15. Sulfur amino acids in Cushing's disease: insight in homocysteine and taurine levels in patients with active and cured disease.

    Science.gov (United States)

    Faggiano, Antongiulio; Melis, Daniela; Alfieri, Raffaele; De Martino, MariaCristina; Filippella, Mariagiovanna; Milone, Francesco; Lombardi, Gaetano; Colao, Annamaria; Pivonello, Rosario

    2005-12-01

    Cushing's syndrome is associated with an increased cardiovascular risk. Although a series of cardiovascular risk factors have been identified, sulfur amino acids (SAAs), recently indicated as independent cardiovascular risk factors, have been poorly investigated in patients with Cushing's syndrome. The aim of this cross-sectional controlled study was to evaluate serum and urinary levels and urinary excretion rate (ER) of SAAs in patients with Cushing's disease (CD) during the active disease and after long-term disease remission. Forty patients with CD (20 with active disease and 20 with cured disease for at least 5 yr) and 40 controls entered the study. Serum and urinary concentrations and urinary ER of SAAs, namely methionine, cystine, homocysteine, and taurine, were measured by means of cationic exchange HPLC. Serum folic acid and vitamin B12 levels were also evaluated in patients and controls and correlated to SAA levels. CD patients with active disease had higher serum and urinary concentrations of cystine and homocysteine, and lower serum and higher urinary concentrations and ER of taurine than cured patients and controls. Vitamin B12 levels were significantly decreased in patients with active disease compared with cured patients and controls, whereas folic acid levels were slightly decreased in patients than in controls. In patients with active CD, urinary cortisol concentrations were significantly and inversely correlated to serum taurine and directly correlated to taurine urinary ER, and fasting serum glucose levels were significantly correlated to taurine urinary ER. At the multiple regression analysis, urinary cortisol concentrations were the best predictors of taurine ER. CD is associated with hyperhomocysteinemia and hypotaurinemia. Glucocorticoid excess, acting directly or indirectly, seems to be the most responsible for this imbalance in SAA levels. The long-term disease remission is accompanied by normalization of SAA levels. Hyperhomocysteinemia and

  16. Kinderen met obesitas en afbuigende lengtegroeicurve: ziekte van Cushing?

    NARCIS (Netherlands)

    van Wezel, Esther M.; van Santen, Hanneke M.; van Furth, Wouter R.; Reneman, Liesbeth; Bisschop, Peter H.; van Trotsenburg, A. S. Paul

    2012-01-01

    We describe two children who presented with extreme weight gain and failure to grow in height, and who were both diagnosed with Cushing's disease. Following preoperative metyrapone treatment, both children successfully underwent curative transsphenoidal surgery. While obesity is a common and

  17. Insights on accelerated skeletal repair in Cushing's disease

    Directory of Open Access Journals (Sweden)

    So-Young Kim

    2015-06-01

    In this patient, spontaneous recovery of trabecular bone architecture was reflected by the early correction in TBS. Subsequent TPTD treatment was associated with marked improvement in BMD, presumably due to enhanced mineralization. Complete skeletal repair was achieved by this two-step mechanism in a very short time following successful surgical treatment for Cushing's disease.

  18. Cortisol, obesity and the metabolic syndrome: A cross-sectional study of obese subjects and review of the literature

    OpenAIRE

    Abraham, SB; Rubino, D; Sinaii, N; Ramsey, S; Nieman, LK

    2013-01-01

    Circulating cortisol and psychosocial stress may contribute to the pathogenesis of obesity and metabolic syndrome. To evaluate these relationships, we performed a cross-sectional study of 369 overweight and obese subjects and 60 healthy volunteers and reviewed the previous literature. Overweight and obese subjects had at least two other features of Cushing?s syndrome. They underwent measurements representing cortisol dynamics (24h urine cortisol excretion (UFC), bedtime salivary cortisol, 1 m...

  19. Cushing Disease Presenting as Primary Psychiatric Illness: A Case Report and Literature Review.

    Science.gov (United States)

    Rasmussen, Sean A; Rosebush, Patricia I; Smyth, Harley S; Mazurek, Michael F

    2015-11-01

    We report the case of a woman with long-standing refractory depression and psychotic features who was eventually diagnosed with Cushing disease. After surgical treatment of a pituitary adenoma, she experienced gradual psychiatric recovery and was eventually able to discontinue all psychotropic medication. We review the psychiatric components of Cushing disease, implications of psychiatric illnesses for the treatment and prognosis of Cushing disease, and potential pathophysiological mechanisms linking glucocorticoid excess to psychiatric illness.

  20. Inhalational Steroids and Iatrogenic Cushing’s Syndrome

    Science.gov (United States)

    A.V, Raveendran

    2014-01-01

    Bronchial asthma (BA) and Allergic rhinitis (AR) are common clinical problems encountered in day to day practice, where inhalational corticosteroids (ICS) or intranasal steroids (INS) are the mainstay of treatment. Iatrogenic Cushing syndrome (CS) is a well known complication of systemic steroid administration. ICS /INS were earlier thought to be safe, but now more and more number of case reports of Iatrogenic Cushing syndrome have been reported, especially in those who are taking cytochrome P450 (CYP 450) inhibitors. Comparing to the classical clinical features of spontaneous Cushing syndrome, iatrogenic Cushing syndrome is more commonly associated with osteoporosis, increase in intra-ocular pressure, benign intracranial hypertension, aseptic necrosis of femoral head and pancreatitis, where as hypertension, hirsuitisum and menstrual irregularities are less common. Endocrine work up shows low serum cortisol level with evidence of HPA (hypothalamo-pituitary-adrenal) axis suppression. In all patients with features of Cushing syndrome with evidence of adrenal suppression always suspect iatrogenic CS. Since concomitant administration of cytochrome P450 inhibitors in patients on ICS/INS can precipitate iatrogenic CS, avoidance of CYP450 inhibitors, its dose reduction or substitution of ICS are the available options. Along with those, measures to prevent the precipitation of adrenal crisis has to be taken. An update on ICS-/INS- associated iatrogenic CS and its management is presented here. PMID:25674177

  1. AACE/ACE Disease State Clinical Review: Medical Management of Cushing Disease.

    Science.gov (United States)

    Hamrahian, Amir H; Yuen, Kevin C J; Hoffman, Andrew R

    2014-07-01

    To review available medical therapies for patients with Cushing disease and to provide a roadmap for their use in clinical practice. PubMed searches were performed to identify all of the available published data on medical management of Cushing disease. Medical therapy is usually not the first-line treatment for patients with Cushing disease but may be used to improve clinical manifestations of Cushing disease in patients who are not suitable candidates for surgery, following unsuccessful surgery or recurrence, or as a "bridge therapy" in those who have undergone radiotherapy. Medical therapy may also be used in preoperative preparation of patients with severe disease. Current available medical options for patients with Cushing disease include centrally acting agents, steroidogenesis inhibitors, and a glucocorticoid receptor antagonists. At present, there are no head-to-head studies comparing the efficacy, tolerability, and safety of different U.S. Food and Drug Administration (FDA)- and non-FDA-approved drugs in patients with Cushing disease. With the initiation of new studies and the completion of ongoing clinical trials, the number of FDA-approved drugs for medical treatment of Cushing disease is expected to increase. Medical therapy has an important adjunctive role in the management of patients with Cushing disease. The decision to initiate medical treatment depends on many factors, including patient characteristics and preference. Long-term studies are needed to better define the clinical efficacy, safety, and tolerability of medical treatment of Cushing disease, including the role of combination therapies.

  2. Hypophysectomy for a dog with coexisting Cushing's disease and diabetes mellitus.

    Science.gov (United States)

    Ishino, Hirokazu; Hara, Yasushi; Teshima, Takahiro; Tanaka, Shigeki; Takekoshi, Susumu; Nezu, Yoshinori; Harada, Yasuji; Yogo, Takuya; Sako, Toshinori; Koyama, Hidekazu; Teramoto, Akira; Osamura, Robert Y; Tagawa, Masahiro

    2010-03-01

    An 11-year-old male mixed breed dog diagnosed with Cushing's disease and diabetes mellitus was treated by hypophysectomy. After surgery, the hypercortisolemia disappeared and the diabetes status improved. The insulin requirement to control hyperglycemia gradually decreased. At 12 weeks after surgery, there was no requirement for insulin and we suspected the diabetes was completely resolved. In the present case, diabetes mellitus seems to be secondary to Cushing's disease. In conclusion, this mixed breed dog with coexisting Cushing's disease and diabetes mellitus is the first case showing the effectiveness of hypophysectomy to treat diabetes mellitus secondary to Cushing's disease in dogs.

  3. NIH Researchers Find Potential Genetic Cause of Cushing Syndrome

    Science.gov (United States)

    ... the CABLES1 gene are a novel cause of Cushing’s disease. Endocrine-Related Cancer 2017; doi: 10.1530/ERC-17-0131 ### This page last reviewed on June 20, 2017 Institute/Center Eunice Kennedy ... 20892 U.S. Department of Health and Human Services Back to Top

  4. Urinary free cortisol in the diagnosis of Cushing's syndrome: How ...

    African Journals Online (AJOL)

    These biochemical tests include the measurement of excess total endogenous cortisol secretion assessed by 24‑hour urinary free cortisol (UFC), loss of the normal feedback of the hypothalamo‑pituitary‑adrenal axis assessed by suppressibility after dexamethasone testing, and disturbance of the normal circadian rhythm of ...

  5. How Do Health Care Providers Diagnose Cushing's Syndrome?

    Science.gov (United States)

    ... NICHD Research Information Find a Study More Information Amenorrhea About NICHD Research Information Find a Study More ... cure? » Related A-Z Topics Adrenal Gland Disorders Amenorrhea Pituitary Tumors NICHD News Spotlights Spotlight: Selected NICHD ...

  6. LONG-TERM OUTCOME OF THE DIFFERENT TREATMENT ALTERNATIVES FOR RECURRENT AND PERSISTENT CUSHING DISEASE.

    Science.gov (United States)

    Espinosa-de-Los-Monteros, Ana Laura; Sosa-Eroza, Ernesto; Espinosa, Etual; Mendoza, Victoria; Arreola, Rocio; Mercado, Moises

    2017-07-01

    Treatment alternatives for persistent and recurrent Cushing disease (CD) include pituitary surgical re-intervention, radiation therapy (RT), pharmacotherapy, and bilateral adrenalectomy (BA). The decision of which of these alternatives is better suited for the individual patient rests on clinical judgment and the availability of resources. This retrospective cohort study was performed at a referral center to evaluate the long-term efficacy of different secondary interventions for persistent and recurrent CD. We evaluated the hospital charts of 84 patients (77 female, median age 34 years, median follow up 6.3 years) with CD diagnosed, treated, and followed at our multidisciplinary clinic according to a pre-established protocol. Of the 81 patients who were initially treated with transsphenoidal surgery (TSS), 61.7% had a long-lasting remission, 16% had persistent disease, and 22% achieved remission but relapsed during follow-up. The most frequently used secondary treatment was pituitary re-intervention, followed by ketoconazole, RT, and BA. Early remissions were observed in 66.6% of the re-operated and in 58.3% of the radiated patients; long-lasting remission was achieved in 33.3% and 41.6% of these patients, respectively. Nelson syndrome developed in 41.6% of the patients who underwent BA. Upon last follow-up, 88% of all the patients are in remission, and 9.5% are biochemically controlled with ketoconazole. The efficacy of treatment alternatives for recurrent or persistent CD varies considerably among patients and multiple interventions are often required to achieve long-lasting remission. ACTH = adrenocorticotrophic hormone; BA = bilateral adrenalectomy; CBG = cabergoline; CD = Cushing disease; CV = coefficient of variation; DXM = dexamethasone; IQR = interquartile range; RT = radiation therapy; SRS = stereotactic radiosurgery; TSS = transsphenoidal surgery; UFC = urinary free cortisol; ULN = upper limit of normal.

  7. Endoscopic Endonasal Surgery for Remission of Cushing Disease Caused by Ectopic Intracavernous Macroadenoma: Case Report and Literature Review.

    Science.gov (United States)

    Koutourousiou, Maria; Winstead, Welby I

    2017-02-01

    Complete surgical resection of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the gold standard of treatment of Cushing disease. Ectopic location of these adenomas is an extremely rare condition that may compromise the diagnosis and surgical success. We present the first case of an ectopic intracavernous ACTH-secreting macroadenoma totally resected with endoscopic endonasal surgery (EES). A 36-year-old woman presented with Cushing syndrome. Increased ACTH, serum cortisol, and free urine cortisol levels were identified; however, pituitary magnetic resonance imaging failed to show a pituitary tumor; instead, a parasellar lesion in the left cavernous sinus (CS) was noticed. Inferior petrosal sinus sampling showed a significant central to peripheral and lateralized left-sided ACTH gradient. The patient underwent EES. No tumor was found in the sella; however, the left CS was widely explored and a tumor was found lateral to the paraclival segment of the carotid artery. There were no complications after EES. Pathology confirmed the diagnosis of an ACTH-secreting adenoma. During the immediate postoperative course, serum cortisol levels decreased lower than 5 μg/dL. Postoperative magnetic resonance imaging showed complete tumor resection. At 20 months follow-up, the patient remained in clinical and biochemical remission of Cushing disease. Only 12 cases of ectopic intracavernous ACTH-secreting adenomas have been reported and all were microadenomas. The presence of an ectopic ACTH-secreting macroadenoma in the CS represents a surgical challenge. EES is the ideal approach for complete resection of ectopic intracavernous adenomas, allowing for a wide exploration of the CS with no surgical complications. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Successful treatment of Cushing's disease using yttrium-90 rods

    International Nuclear Information System (INIS)

    White, M.C.; Doyle, F.H.; Mashiter, K.; Joplin, G.F.

    1982-01-01

    Interstitial irradiation using yttrium-90 ( 90 Y) rods implanted by needle into the pituitary gland was used as primary treatment in 16 patients with pituitary dependent Cushing's disease. Clinical and biochemical remission was observed within three to six months in 13 and in the remaining three after a supplementary implant. There was no perioperative morbidity. Follow-up from the time of definitive operation ranged from six to 123 months (mean 39). No recurrence has been observed. The return of a normal diurnal cortisol rhythm has been observed in 10/12 patients studied after remission. Some form of long-term pituitary hormone replacement therapy was required in only the six patients who had received the largest irradiation dose. Implantation of 90 Y is safe and effective treatment for patients with Cushing's disease, comparing favourably with selective trans-sphenoidal pituitary surgery. (author)

  9. Harvey Cushing and the evolution of a polymath.

    Science.gov (United States)

    Tilney, N L

    1986-03-01

    The remarkable productivity of the later career of Cushing is well known. Energies and drive undiminished throughout his life, he contributed consistently and prolifically to the field of surgical procedures, history, biography and education at the very least. Consorting with the famous, honored over the world, his shadow is still seen. Early factors which influenced his personality and career have been analyzed herein. Many of these, individually or in concert, affected the extraordinary life of this complex man.

  10. Seasonal changes in circadian peripheral plasma concentrations of melatonin, serotonin, dopamine and cortisol in aged horses with Cushing's disease under natural photoperiod.

    Science.gov (United States)

    Haritou, S J A; Zylstra, R; Ralli, C; Turner, S; Tortonese, D J

    2008-08-01

    Equine pituitary pars intermedia dysfunction (PPID) is a common and serious condition that gives rise to Cushing's disease. In the older horse, it results in hyperadrenocorticism and disrupted energy metabolism, the severity of which varies with the time of year. To gain insight into the mechanism of its pathogenesis, 24-h profiles for peripheral plasma melatonin, serotonin, dopamine and cortisol concentrations were determined at the winter and summer solstices, and the autumn and spring equinoxes in six horses diagnosed with Cushing's disease and six matched controls. The nocturnal rises in plasma melatonin concentrations, although different across seasons, were broadly of the same duration and similar amplitude in both groups of animals (P > 0.05). The plasma concentrations of cortisol did not show seasonal variation and were different in diseased horses only in the summer when they were higher across the entire 24-h period (P melatonin are similar in Cushingoid and control horses reveals that the inability to read time of year by animals suffering from Cushing's syndrome is an unlikely reason for the disease. In addition, the results provide evidence that alterations in the dopaminergic and serotoninergic systems may participate in the pathogenesis of PPID.

  11. Medical treatment of Cushing's disease: Overview and recent findings

    Directory of Open Access Journals (Sweden)

    Stephanie Smooke Praw

    2009-10-01

    Full Text Available Stephanie Smooke Praw1, Anthony P Heaney1,21Department of Medicine, 2Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USAAbstract: Cushing's disease, due to pituitary adrenocorticotropic hormone (ACTH hypersecretion, is the most common etiology of spontaneous excess cortisol production. The majority of pituitary tumors causing Cushing's disease measure <1 cm and the excess morbidity associated with these tumors is mostly due to the effects of elevated, nonsuppressible, ACTH levels leading to adrenal steroid hypersecretion. Elevated circulating cortisol levels lead to abnormal fat deposition, hypertension, diabetes, coronary artery disease, osteoporosis, muscle weakness and psychological disturbances. At experienced centers, initial surgical remission rate via transnasal, transphenoidal resection approaches 80% for tumors less than 1 cm, but may be as low as 30% for larger lesions and long-term recurrence in all groups approaches 25%. Residual disease may be managed with more radical surgery, pituitary-directed radiation, bilateral adrenalectomy, or medical therapy. This paper addresses current and novel therapies in various stages of development for Cushing’s disease.Keywords: Cushing's disease, treatment, pasireotide, PPAR-γ, 11 β-hydroxysteroid dehydrogenase inhibitors, dopamine agonists

  12. Predictors of resource utilization in transsphenoidal surgery for Cushing disease.

    Science.gov (United States)

    Little, Andrew S; Chapple, Kristina

    2013-08-01

    The short-term cost associated with subspecialized surgical care is an increasingly important metric and economic concern. This study sought to determine factors associated with hospital charges in patients undergoing transsphenoidal surgery for Cushing disease in an effort to identify the drivers of resource utilization. The authors analyzed the Nationwide Inpatient Sample (NIS) hospital discharge database from 2007 to 2009 to determine factors that influenced hospital charges in patients who had undergone transsphenoidal surgery for Cushing disease. The NIS discharge database approximates a 20% sample of all inpatient admissions to nonfederal US hospitals. A multistep regression model was developed that adjusted for patient demographics, acuity measures, comorbidities, hospital characteristics, and complications. In 116 hospitals, 454 transsphenoidal operations were performed. The mean hospital charge was $48,272 ± $32,060. A multivariate regression model suggested that the primary driver of resource utilization was length of stay (LOS), followed by surgeon volume, hospital characteristics, and postoperative complications. A 1% increase in LOS increased hospital charges by 0.60%. Patient charges were 13% lower when performed by high-volume surgeons compared with low-volume surgeons and 22% lower in large hospitals compared with small hospitals. Hospital charges were 12% lower in cases with no postoperative neurological complications. The proposed model accounted for 46% of hospital charge variance. This analysis of hospital charges in transsphenoidal surgery for Cushing disease suggested that LOS, hospital characteristics, surgeon volume, and postoperative complications are important predictors of resource utilization. These findings may suggest opportunities for improvement.

  13. Treatment of Cushing's disease in juveniles with intestinal pituitary irradiation

    International Nuclear Information System (INIS)

    Cassar, J.; Doyle, F.H.; Mashiter, K.; Joplin, G.F.

    1979-01-01

    Nine juvenile patients (five boys and four girls aged 10-18) with Cushing's disease were treated with pituitary implantation of 198 Au and/or 90 Y. No patient had any surgical complication from this procedure. At the latest assessment. 3 months to 17 years after operation, Cushing's disease was in remission in all the patients; the response time following operation was a few days to 3 months. Radiology of the pituitary fossa at time of pituitary implantation was normal in all patients and remains so. The final height in six patients is 149-172 cm (59-67.5 inches) and three patients who continue to grow have increased by 13.6 and 3 cm since implantation. Only one patient required full pituitary hormone replacement therapy, and he had been previously treated by external irradiation, and one other patient failed to complete puberty. In all the other seven sexual maturation is normal and one has fathered two children. It is concluded that pituitary implantation with interstitial irradiation is a satisfactory form of treatment for Cushing's disease in juveniles. (author)

  14. Bilateral Testicular Tumors Resulting in Recurrent Cushing Disease After Bilateral Adrenalectomy

    NARCIS (Netherlands)

    Puar, T.; Engels, M.; Herwaarden, A.E. van; Sweep, F.C.; Hulsbergen-van de Kaa, C.A.; Kamphuis-van Ulzen, K.; Chortis, V.; Arlt, W.; Stikkelbroeck, N.; Claahsen-van der Grinten, H.L.; Hermus, A.R.M.M.

    2017-01-01

    Context: Recurrence of hypercortisolism in patients after bilateral adrenalectomy for Cushing disease is extremely rare. Patient: We present a 27-year-old man who previously underwent bilateral adrenalectomy for Cushing disease with complete clinical resolution. Cushingoid features recurred 12 years

  15. Quality of life in patients after long-term biochemical cure of Cushing's disease

    NARCIS (Netherlands)

    van Aken, M. O.; Pereira, A. M.; Biermasz, N. R.; van Thiel, S. W.; Hoftijzer, H. C.; Smit, J. W. A.; Roelfsema, F.; Lamberts, S. W. J.; Romijn, J. A.

    2005-01-01

    To evaluate the long-term impact of cured Cushing's disease on subjective well-being, we assessed quality of life by validated health-related questionnaires in 58 patients cured from Cushing's disease by transsphenoidal surgery (n = 58), some of whom received additional radiotherapy (n = 11) and/or

  16. Quality of life in patients after long-term biochemical cure of cushing's disease

    NARCIS (Netherlands)

    M.O. van Aken (Maarten); A.M. Pereira (Alberto); N.R. Biermasz; S.W. van Thiel (Sjoerd); H. Hoftijzer (Hendrieke); J.W.A. Smit (Jan); F. Roelfsema (Ferdinand); S.W.J. Lamberts (Steven); J.A. Romijn (Johannes)

    2005-01-01

    textabstractTo evaluate the long-term impact of cured Cushing's disease on subjective well-being, we assessed quality of life by validated health-related questionnaires in 58 patients cured from Cushing's disease by transsphenoidal surgery (n = 58), some of whom received additional radiotherapy (n =

  17. Outcome of Cushing's disease following transsphenoidal surgery in a single center over 20 years.

    LENUS (Irish Health Repository)

    Hassan-Smith, Zaki K

    2012-04-01

    Historically, Cushing\\'s disease (CD) was associated with a 5-yr survival of just 50%. Although advances in CD management have seen mortality rates improve, outcome from transsphenoidal surgery (TSS), the current first-line treatment, varies significantly between centers.

  18. Gêmeas idênticas discordantes para a doença de Cushing: relato de caso Identical twins discordant for Cushing's disease: case report

    Directory of Open Access Journals (Sweden)

    MARCELO MAIA PINHEIRO

    1999-09-01

    Full Text Available A doença de Cushing é rara em crianças e sua ocorrência em gêmeos é ainda mais rara. O presente estudo relata a ocorrência de gêmeas idênticas discordantes quanto à doença de Cushing. Uma das gêmeas, desenvolveu síndrome de Cushing aos 10 anos de idade. Sua investigação demonstrou cortisol urinário elevado, ACTH sérico elevado e testes de supressão com dexametasona compatíveis com doença de Cushing. A RMN mostrou macroadenoma hipofisário que foi operado por via transesfenoidal. O estudo imuno-histoquímico confirmou a presença de células produtoras de ACTH. A paciente entrou em remissão clínica e laboratorial desde a cirurgia. Após o desaparecimento dos estigmas do Cushing, a paciente reiniciou ganho estatural, mas permanece menor que sua irmã gêmea. Esta última permanece saudável 4 anos após o início da doença de sua irmã. Este é o terceiro caso do gênero relatado na literatura. Estes achados sugerem que fatores não genéticos estão envolvidos na gênese da doença de Cushing.Cushing's disease is rare in children and its ocurrence in identical twins is extremely rare. This paper reports on identical twins discordant for Cushing's disease. One of them first presented with a cushingoid phenotype by the age of 10. Her evaluation showed an increased urinary free-cortisol and serum ACTH. Her pattern in the dexametazone supression tests was compatible with Cushing's disease. MRI disclosed a pituitary macroadenoma which was removed by the transesphenoidal approach. Immunohistochemical studies of the tumor showed the presence of ACTH-producing cells. The patient went into clinical and laboratorial remission after surgery. She re-started to grow after the disappearance of the Cushing's phenotype but she is still shorter than her healthy sister. The latter remains disease-free 4 years after her sister's diagnosis. This represents the third such case reported in the literature. Our findings suggest that acquired

  19. Clinical and morphological characteristic of ACTH producing tumors of various localization and the ectopic Cushing’s syndrome

    Directory of Open Access Journals (Sweden)

    L. E. Gurevich

    2017-01-01

    Full Text Available Background: Ectopic adrenocorticotropic hormone (ACTH-dependent Cushing's syndrome is a type hypercorticism caused by ectopic production of ACTH and/or its precursors by neuroendocrine tumors (NET of various localization, such as lung and thymus carcinoids, less frequently those of pancreas and gastrointestinal tract, medullary thyroid cancer, pheochromocytoma, small cell lung cancer, as well as some other tumor types. The wide spectrum of tumors associated with the ectopic ACTH syndrome (EAS makes their diagnosis and treatment a complicated issue.Materials and methods: The study was done with surgical and diagnostic biopsy samples from 60 patients who had tumors with EAS, 36 (60% of them being bronchopulmonary carcinoids, 10 (16.7% thymus carcinoids, 5 (8.3% pancreatic NETs, 3 (5% medullary thyroid cancers, 3 (5% NETs of unknown primary localization, and NET of appendix, cecum and pheochromocytoma (one case of each, i.e. 1.7%, 1.7%, 1.7%. There were 38 female and 22 male patients (1.72:1, with their mean age of 39 ± 14 years (range, 16 to 77 years. We analyzed their clinical data, as well as the results of morphological and immunohistochemical examination of the tumors.Results: According to the World Health Organization classification (2015, typical carcinoids (TC of the lung were found in 77.8% (28/36 of the bronchopulmonary tumors. 22.2% (8/36 of the bronchopulmonary tumors and all thymus tumors (100%, n = 10 were classified as atypical carcinoids (ATC. Four pancreatic NETs were classified as Grade 2 (G2, 1 as G3, and NETs of the cecum and appendix as G1 and G2, respectively. At present, 29 (48% patients are living with no relapse (mean age 39 ± 13.5 years, whereas 22 (37% of patients developed a relapse (mean age 35 ± 13 years, and 15 (25% of them died, regardless of the age of the patients The history of the disease was not traced in 4 cases. 5-year relapse-free survival of patients with TC of the

  20. DHEA(S)--a novel marker in Cushing's disease.

    Science.gov (United States)

    Burkhardt, T; Schmidt, N O; Vettorazzi, E; Aberle, J; Mengel, M; Flitsch, J

    2013-03-01

    Dehydroepiandrosterone sulfate (DHEA(S)) is a multi-functional steroid implicated in a broad range of biological effects, including obesity, diabetes, bone metabolism, neuroprotection, and anti-tumorigenesis. It has not yet undergone wider research in the context of Cushing's disease. The objective of this study was to determine if perioperative blood levels of DHEA(S) correlate with levels of ACTH and cortisol, and therefore may be useful as a new, additional marker for the early definition of cure in patients suffering from Cushing's disease. Forty-two consecutive patients undergoing transsphenoidal surgery for Cushing's disease from September 2009 to September 2010 were perioperatively monitored for ACTH, cortisol, and DHEA(S). Pre-operative blood samples revealed ACTH levels of median 65 ng/l (range 11-1,183 ng/l, standard deviation 183.76), cortisol of median 257 μg/l (range 93-803 μg/l, standard deviation 140.88), and DHEA(S) of median 2.22 mg/l (range 0.44-7.79 mg/l, standard deviation 1.82) according to the pathology of Cushing's disease. Postoperative blood samples drawn over a 7-day time span showed a drop in median ACTH to just 14.5 % (median: 9 ng/l, range 2-44, standard deviation 12.75) of its median preoperative figure. Median cortisol levels were reduced to 6.9 % (median: 18 μg/l, range 10-190 μg/l, standard deviation 38.04) of their preoperative values and DHEA(S) levels decreased to 17 % (median: 0.38 mg/l, range 0.05-2.29, standard deviation 0.51). In persistent disease, no patient showed a drop in DHEA(S) below 38 % of its preoperative figures. DHEA(S) shows the potential to become an additional marker in the diagnosis and follow-up of patients. However, it needs to be examined further, including whether DHEA(S) may also be a useful predictor of recovery of the HPA-axis after successful surgery.

  1. A case with post-irradiation cerebral vascular disease accompanied by Cushing's disease

    International Nuclear Information System (INIS)

    Shinoda, Keiichi; Kuriyama, Takanobu; Kimura, Fumiharu; Kawamura, Hiroshi; Takamatsu, Junta; Mozai, Toshiji

    1986-01-01

    A 37-year-old man with Cushing's disease developed cerebral infarction 13 years after Co-60 irradiation of a total dose of 60 Gy for pituitary adenoma. Cerebral angiography showed stenosis or occlusion of the bilateral carotid arteries and basilar artery which were in the radiation fields. A basophilic adenoma was resected, and symptoms of Cushing's disease have resolved. The vascular lesions are likely to be not only radiation-induced, but also stimulated by hyperlipemia and hypertension associated with Cushing's disease. It is recommended to eliminate factors, such as hyperlipemia and hypertension, stimulatig vascular damage in the management of patients treated with radiation therapy. The relevant literature is reviewed. (Namekawa, K.)

  2. Bienestar psicológico de personas con síndrome de Cushing

    Directory of Open Access Journals (Sweden)

    Mónica María Novoa-Gómez

    2013-01-01

    Full Text Available El objetivo de este estudio exploratorio es analizar la relación entre el bienestar psicológico y las condiciones médicas y terapéuticas de personas con diagnóstico de síndrome de Cushing. Los instrumentos utilizados fueron el Índice de Bienestar subjetivo (The Psychological Well-Being Index; Dupuy, 1984 y el Cushing’s Syndrome Severity Index (CSI. La selección de la muestra se realizó por conveniencia; considerando que es un síndrome de muy baja incidencia, se estableció contacto con cinco pacientes. Se utilizaron estadísticos descriptivos que facilitaron la obtención de información sobre la muestra. Los resulatdos permiten concluir que la severidad de los síntomas asociados al síndorme, evaluados a través del CSI, implican malestar moderado en el bienestar psicológico, corroborando lo señalado por la literatura, así como que las alteraciones de orden psicológico influyen en el proceso de enfermedad o recuperación.

  3. Radiation and combined treatment of Itsenko -Cushing's disease

    International Nuclear Information System (INIS)

    Barkanov, A.I.; Morozov, A.I.; Pirogov, A.I.; Postnikov, D.A.; Shadyeva, M.M.; Roshchina, V.S.; Devyatykh, Yu.N.

    1980-01-01

    The authors made observations of 123 patients with the Itsenko - Cushing disease. The mild form of the disease was diagnosed in 27.7 per cent of the patients; moderate in 52 percent, and severe in 20.3 per cent of the patients. A total of 78 patients underwent tele-gamma-therapy in doses of 40-45 Gy, and 45 patients underwent combined treatment consisting in unilateral adrenalectomy and irradiation with the same doses. Protracted remissions with a reverse development of the symptoms of the disease were reached in 69.2 per cent of the patients who had undergone radiation treatment and in 64.4 per cent of the patients who had undergone combined treatment. Radiotherapy was most effective in patients with mild and moderate forms of Itsenko-Cushing's disease, in case of a severe form combined treatment is indicated. Optimal single focal doses are 1.6-1.8 Gy, and cumulative ones 40-45 Gy

  4. Nelson syndrome: definition and management.

    Science.gov (United States)

    Barber, T M; Adams, E; Wass, J A H

    2014-01-01

    Nelson syndrome is an important complication of treatment with total bilateral adrenalectomy (TBA) for patients with refractory Cushing's disease. Although early cases of Nelson syndrome often presented with the clinical features of large sellar masses, the modern face of Nelson syndrome has changed primarily due to earlier detection (with highly resolved magnetic resonance imaging (MRI) and sensitive ACTH assays) and greater awareness of the condition, resulting in reduced morbidity and mortality. Although lack of administration of neoadjuvant pituitary radiotherapy post-TBA surgery may predict future development of Nelson syndrome, other predictive factors remain controversial. Therefore, Nelson syndrome should be screened for closely and long-term in all patients with a history of Cushing's disease and TBA. The diagnosis of Nelson syndrome remains controversial, and the pathogenesis of this condition is incompletely understood. Current hypotheses include the "released negative feedback" mechansism (residual pituitary corticotropinoma cells are "released" from the negative feedback effects of cortisol following TBA), and the "aggressive corticotropinoma" mechanism (Nelson syndrome is most likely to develop in those patients with refractory treatments - including TBA - for an underlying aggressive corticotropinoma). Effective management of Nelson syndrome with pituitary surgery and radiotherapy is often a challenge. Other therapies (such as Gamma Knife surgery and temozolomide) play an important role and merit further research into their efficacy and placement in the management pathway of Nelson syndrome. © 2014 Elsevier B.V. All rights reserved.

  5. Growth hormone and prolactin responses to corticotrophin-releasing-hormone in patients with Cushing's disease: a paracrine action of the adenomatous corticotrophic cells?

    Science.gov (United States)

    Loli, P; Boccardi, E; Branca, V; Bramerio, M; Barberis, M; Losa, M; Terreni, M T; Lodrini, S; Pollo, B; Vignati, F

    1998-10-01

    In patients with Cushing's disease multihormonal responses to ovine corticotrophin releasing hormone (oCRH) have been detected in blood from inferior petrosal sinuses. This finding has been explained by co-secretion of other hormones, in addition to ACTH, by the pituitary adenoma itself or by paracrine effects exerted by the adenoma on normal periadenomatous pituitary cells. To assess these hypotheses we compared the presence of a CRH induced GH and/or PRL response during inferior petrosal sinus sampling to the immunohistochemical detection of PRL and GH in adenomatous tissue removed from patients with Cushing's disease. Twenty-two patients with Cushing's disease and two patients with ectopic ACTH syndrome due to a bronchial carcinoid were studied; each patient had undergone preoperative inferior petrosal sinus sampling for diagnostic purposes with determination of GH and PRL in addition to ACTH, before and after administration of oCRH. Immunohistochemical studies for ACTH, GH and PRL detection were carried out on adenomatous tissue removed at surgery in the patients with pituitary dependent Cushing's disease and on the carcinoid tumours from the two patients with ectopic ACTH syndrome. All pituitary adenomas immunostained for ACTH, and four adenomas immunostained for GH or PRL in addition to ACTH. A PRL increase in the inferior petrosal sinus after oCRH administration was found in 11 of 22 patients, but none of their tumours immunostained for PRL. Immunostaining for PRL was found in the pituitary tumours from two patients but in neither patient was there a PRL response after oCRH. A GH response was found in 13 of 20 patients in whom it was sought; no patient showed immunostaining in their tumour. GH immunostaining was found in two tumours but in neither patient was there a GH response after oCRH. The oCRH-induced increase of GH and PRL was always recorded in the dominant inferior petrosal sinus. The ACTH response to oCRH was significantly higher in patients who

  6. A 12-month phase 3 study of pasireotide in Cushing's disease

    DEFF Research Database (Denmark)

    Colao, Annamaria; Petersenn, Stephan; Newell-Price, John

    2012-01-01

    Cushing's disease is associated with high morbidity and mortality. Pasireotide, a potential therapy, has a unique, broad somatostatin-receptor-binding profile, with high binding affinity for somatostatin-receptor subtype 5....

  7. Preserved GLP-1 effects in a diabetic patient with Cushing's disease

    DEFF Research Database (Denmark)

    Ritzel, R A; Kleine, N; Holst, Jens Juul

    2007-01-01

    CONTEXT: A patient with diabetes mellitus, who participated in a study with intravenous administration of GLP-1, was later found to have Cushing's disease (markedly elevated 24 h urinary cortisol excretion and inadequate suppression of fasting cortisol with 2 mg dexamethasone). His diabetic state...... mellitus due to Cushing's disease with GLP-1 actions in typical type 2 diabetes. DESIGN AND METHODS: GLP-1 (1.2 pmol/kg/min) and placebo had been infused into ten patients with diabetes mellitus over 4 h in the fasting state. The results from the patient with Cushing's disease (C) were compared to the data...... with Cushing's disease compared to those with type 2 diabetes. CONCLUSIONS: The insulinotropic, glucagonostatic and glucose-lowering actions of GLP-1 in a patient with diabetes mellitus due to cortisol excess were similar to actions in typical type 2 diabetes. Therefore incretin mimetics might be a novel...

  8. The case of Cushings disease imaging by SPECT examination without manifestation of adenoma in MRI examination

    International Nuclear Information System (INIS)

    Gierach, M.; Pufal, J.; Pilecki, S.; Junik, R.

    2005-01-01

    The aim of our study was to evaluate the possibility of imaging the pathological accumulation of 99mT c-MIBI in the pituitary gland in patients with Cushings disease when MRI examination does not show microadenomas. Cushings disease was diagnosed in a 27 year old male on the basis of clinical and biochemical findings. The blood cortisol level of the patient was elevated (the average level was 47 microgram/dl) and it showed no changeability of day and night rhythm. In the patient with Cushing's disease, during the SPECT examination, an increased accumulation of 99mT c-MIBI in the pituitary gland was noticed. MRI scanning was negative. Single photon emission computed tomography using 99mT c-MIBI is a useful and sensitive means of pituitary gland microadenoma detection in patients with Cushings disease when microadenoma is not detected during MRI scanning and when the results of dexamethasone suppression test is positive. (author)

  9. Harvey Cushing, the spine surgeon: the surgical treatment of Pott disease.

    Science.gov (United States)

    Bydon, Ali; Dasenbrock, Hormuzdiyar H; Pendleton, Courtney; McGirt, Matthew J; Gokaslan, Ziya L; Quinones-Hinojosa, Alfredo

    2011-08-01

    Review of historical archival records. Describe Harvey Cushing's patients with spinal pathology. Harvey Cushing was a pioneer of modern surgery but his work on spine remains largely unknown. Review of the Chesney Medical Archives of the Johns Hopkins Hospital from 1896 to 1912. This is the first time that Cushing's spinal cases while he was at the Johns Hopkins Hospital, including those with Pott disease, have been described.Cushing treated three young men with psoas abscesses secondary to Pott disease during his residency: he drained the abscesses, debrided any accompanying necrotic vertebral bodies, irrigated the cavity with salt, and left the incision open to close by secondary intention. Although Cushing used Koch's "tuberculin therapy" (of intravenous administration of isolated tubercular bacilli) in one patient, he did not do so in the other two, likely because of the poor response of this first patient. Later in his tenure, Cushing performed a laminectomy on a patient with kyphosis and paraplegia secondary to Pott disease. These cases provide a view of Cushing early in his career, pointing to the extraordinary degree of independence that he had during his residency under William Steward Halsted; these cases may have been important in the surgical upbringing both of Cushing and his coresident, William Stevenson Baer, who became the first professor of Orthopedics at Johns Hopkins Hospital. At the turn of the last century, Pott disease was primarily treated by immobilization with bed rest, braces, and plaster-of-paris jackets; some surgeons also employed gradual correction of the deformity by hyperextension. Patients who failed a trial of conservative therapy (of months to years) were treated with a laminectomy. However, the limitations of these strategies led to the development of techniques that form the basis of contemporary spine surgery-instrumentation and fusion.

  10. Unusual Cushing’s Syndrome and Hypercalcitoninaemia due to a Small Cell Prostate Carcinoma

    Directory of Open Access Journals (Sweden)

    Antonio Balestrieri

    2016-01-01

    Full Text Available A 75-year-old man was hospitalized because of severe hypokalaemia due to ACTH dependent Cushing’s syndrome. Total body computed tomography (TBCT and 68 Gallium DOTATATE PET/CT localized a voluminous prostate tumour. A subsequent transurethral prostate biopsy documented a small cell carcinoma positive for ACTH and calcitonin and negative for prostatic specific antigen (PSA at immunocytochemical study; serum prostatic specific antigen (PSA was normal. Despite medical treatments, Cushing’s syndrome was not controlled and the patient’s clinical condition progressively worsened. Surgical resection was excluded; the patient underwent a cycle of chemotherapy followed by febrile neutropenia and fatal intestinal perforation. This case report describes a rare case of Cushing’s syndrome and hypercalcitoninaemia due to a small cell carcinoma of the prostate, a rare tumour with very few therapeutic options and negative prognosis.

  11. Myopathy in horses with pituitary pars intermedia dysfunction (Cushing's disease).

    Science.gov (United States)

    Aleman, M; Watson, J L; Williams, D C; LeCouteur, R A; Nieto, J E; Shelton, G D

    2006-11-01

    Fifteen horses with pituitary pars intermedia dysfunction were studied. The horses were of various breeds and between 15 and 28 years of age. Control horses matched for breed and age were studied for comparison. Evaluations included complete blood cell count and serum biochemical analysis, electromyography, and gluteus medius muscle biopsies for histochemical, morphometric, and ultrastructural analysis. No differences were found between groups of horses on routine laboratory analysis or electromyography. We demonstrated that muscle wasting in diseased horses was the result of atrophy of types 2A and 2B muscle fibers and loss of type 2B myofibers. Mild non-specific non-inflammatory myopathic alterations such as myofiber size variation, internal nuclei, perimysial, endomysial and sarcoplasmic fat accumulation were observed. At the ultrastructural level, subsarcolemmal mitochondrial accumulation and increased lipid droplets were evident. Similar to other species, this study confirmed atrophy of type 2 fibers as the cause of muscle mass loss in horses with Cushing's disease.

  12. Cushing proximal symphalangism and the NOG and GDF5 genes

    International Nuclear Information System (INIS)

    Plett, Sara K.; Berdon, Walter E.; Oklu, Rahmi; Cowles, Robert A.; Campbell, John B.

    2008-01-01

    Proximal symphalangism (SYM1) is an autosomal-dominant developmental disorder of joint fusion. This disorder is best known from famous historical descriptions of two large kindred: Cushing's description in 1916 of the ''straight-fingered'' Brown family of Virginia and Drinkwater's description in 1917 of the British Talbot family of noble blood, descended from the English war hero John Talbot, the first Earl of Shrewsbury (1388-1453). Recent genetic studies link this phenotype to expression of abnormal genes at future joint sites: too little expression of NOG, a growth antagonist, or overexpression of GDF5, a growth agonist, results in cartilage overgrowth and bony fusion. This review unites in depth the first historical accounts of SYM1 with a clinical description and reviews the current understanding of the molecular mechanism underlying what is likely the oldest dominant trait ever studied. (orig.)

  13. Postoperative plasma cortisol levels predict long-term outcome in patients with Cushing's disease and determine which patients should be treated with pituitary irradiation after surgery

    International Nuclear Information System (INIS)

    Imaki, Toshihiro; Tsushima, Toshio; Hizuka, Naomi; Odagiri, Emi; Murata, Yoji; Takano, Kazue; Suda, Toshihiro

    2001-01-01

    Transsphenoidal surgery is the treatment of choice for ACTH-producing pituitary adenoma (Cushing's disease) and pituitary irradiation is widely considered the most appropriate treatment for patients with Cushing's disease for whom transsphenoidal surgery has been unsuccessful. We studied 49 consecutive patients who underwent transsphenoidal surgery for the treatment of Cushing's disease at Tokyo Women's Medical University from 1977-1997 with a mean follow-up duration of 87.6 months (range, 24-253 months). We examined the relationship between postoperative endocrinological data, assessed between 3 and 8 weeks after surgery, and long-term outcome and efficacy of pituitary irradiation after surgery. Long-term remission was defined as the regression of the symptom and signs of Cushing's syndrome, and restoration of normal levels of plasma ACTH, cortisol and urinary free cortisol, together with adequate suppression of morning plasma cortisol levels following the administration of low dose (1 mg) of dexamethasone. Thirty patients had no additional treatment after pituitary surgery. Only 1 of 25 patients (4%) whose postoperative plasma cortisol level was less than 2 μg/dl developed recurrent disease whereas 3 out of 5 patients with postoperative plasma cortisol levels higher than 2 μg/dl relapsed. Postoperative external pituitary radiation was used to treat the remaining 19 patients. Four patients who received radiation therapy had a low or undetectable postoperative plasma cortisol level (<2 μg/dl, 56 nmol/L) and all of these patients developed hypopituitarism whereas 5 patients with subnormal plasma cortisol levels (2.0-10.0 μg/dl) remained in remission. Among 10 patients with persistent disease after surgery, 6 entered remission 6-47 months after irradiation but one of them subsequently relapsed after 108 months. These results suggest that additional therapy should be avoided in patients with a postoperative plasma cortisol less than 2 μg/dl because relapse is

  14. Long-term predictive value of postsurgical cortisol concentrations for cure and risk of recurrence in Cushing's disease

    NARCIS (Netherlands)

    Pereira, Alberto M.; van Aken, Maarten O.; van Dulken, Hans; Schutte, Pieter J.; Biermasz, Nienke R.; Smit, Jan W. A.; Roelfsema, Ferdinand; Romijn, Johannes A.

    2003-01-01

    We assessed the value of postoperative plasma cortisol concentrations to predict cure and recurrence of Cushing's disease after transsphenoidal surgery (TS). Seventy-eight of 80 consecutive patients treated by TS for Cushing's disease were evaluated. TS cured 72% (n = 56) of the patients. Two weeks

  15. On the shoulders of giants: Harvey Cushing's experience with acromegaly and gigantism at the Johns Hopkins Hospital, 1896-1912.

    NARCIS (Netherlands)

    Pendleton, C.; Adams, H.; Salvatori, R.; Wand, G.; Quinones-Hinojosa, A.

    2011-01-01

    A review of Dr. Cushing's surgical cases at Johns Hopkins Hospital revealed new information about his early operative experience with acromegaly. Although in 1912 Cushing published selective case studies regarding this work, a review of all his operations for acromegaly during his early years has

  16. Santiago Ramón y Cajal and Harvey Cushing: two forefathers of neuroscience and neurosurgery.

    Science.gov (United States)

    Zamora-Berridi, Grettel J; Pendleton, Courtney; Ruiz, Gabriel; Cohen-Gadol, Aaron A; Quiñones-Hinojosa, Alfredo

    2011-11-01

    To summarize the extraordinary accomplishments, and the commonalities, between Santiago Ramon y Cajal and Harvey Williams Cushing. Existing literature describing the lives and achievements of Ramón y Cajal and Cushing, as well as personal communication, and the surgical records of the Johns Hopkins Hospital, from 1896 to 1912, were reviewed. Both Ramón y Cajal and Cushing were men of unusually broad interests and talents, and these shared characteristics undoubtedly influenced the career paths and scientific investigations they pursued. Although Santiago Ramón y Cajal and Harvey Williams Cushing never directly interacted, the links between them can be traced through some of their disciples, including Pío del Río Hortega, Wilder Penfield, and Percival Bailey. Ramón y Cajal and Cushing are widely considered the forefathers of neuroscience and neurosurgery, respectively, and their discoveries have made lasting impressions on both the scientific and medical communities. Copyright © 2011 Elsevier Inc. All rights reserved.

  17. Outcome of Microscopic Transsphenoidal Surgery in Cushing Disease: A Case Series of 96 Patients.

    Science.gov (United States)

    Shirvani, Manochehr; Motiei-Langroudi, Rouzbeh; Sadeghian, Homa

    2016-03-01

    To analyze the results of transsphenoidal surgery in patients with Cushing disease and outcome. Retrospective analysis of the records of 96 patients with Cushing disease from 1997 to 2012. There were 73 females and 23 males, with a mean follow-up of 44 months (range, 3-13 years). The sex ratio was significantly different in children and teenagers versus adults. Magnetic resonance imaging showed microadenoma, macroadenoma, and no adenoma in 66, 18, and 12 patients, respectively. There was no surgical mortality. Early remission (normal 24-hour urinary free cortisol and basal serum cortisol Cushing disease. Macroadenomas, cavernous sinus invasion, and harder tumor consistencies, however, are associated with lower remission rates (higher disease persistence) and younger age, higher preoperative cortisol levels, and longer follow-up periods are associated with higher recurrence. Copyright © 2016 Elsevier Inc. All rights reserved.

  18. The second Cushing-Henson conjecture for the Beverton-Holt q-difference equation

    Directory of Open Access Journals (Sweden)

    Martin Bohner

    2017-01-01

    Full Text Available In this paper, we study the second Cushing-Henson conjecture for the Beverton-Holt difference equation with periodic inherent growth rate and periodic carrying capacity in the quantum calculus setting. We give a short summary of recent results regarding the Beverton-Holt difference and \\(q\\-difference equation and introduce the theory of quantum calculus briefly. Next, we analyze the second Cushing-Henson conjecture. We extend recent studies in [The Beverton-Holt q-difference equation with periodic growth rate, Difference Equations, Discrete Dynamical Systems, and Applications, Springer-Verlag, Berlin, Heidelberg, New York, 2015, pp. 3-14] and state a modified formulation of the second Cushing-Henson conjecture for the Beverton-Holt \\(q\\-difference equation as a generalization of existing formulations.

  19. Successful long-term treatment of Cushing disease with mifepristone (RU486).

    Science.gov (United States)

    Basina, Marina; Liu, Hau; Hoffman, Andrew R; Feldman, David

    2012-01-01

    We describe a girl with Cushing disease for whom surgery and radiation treatments failed and the subsequent clinical course with mifepristone therapy. We present the patient's clinical, biochemical, and imaging findings. A 16-year-old girl presented with classic Cushing disease. After transsphenoidal surgery, Cyberknife radiosurgery, ketoconazole, and metyrapone did not control her disease, and she was prescribed mifepristone, which was titrated to a maximal dosage of 1200 mg daily with subsequent symptom improvement. Mifepristone (RU486) is a high-affinity, nonselective antagonist of the glucocorticoid receptor. There is limited literature on its use as an off-label medication to treat refractory Cushing disease. Over her 8-year treatment with mifepristone, her therapy was complicated by hypertension and hypokalemia requiring spironolactone and potassium chloride. She received a 2-month drug holiday every 4 to 6 months to allow for withdrawal menstrual bleeding with medroxyprogesterone acetate. Urinary cortisol, serum cortisol, and corticotropin levels remained elevated during mifepristone drug holidays. While on mifepristone, her signs and symptoms of Cushing disease resolved. Repeated magnetic resonance imaging demonstrated stable appearance of the residual pituitary mass. Bilateral adrenalectomy was performed, and mifepristone was discontinued after 95 months of medical therapy. We describe the longest duration of mifepristone therapy thus reported for the treatment of refractory Cushing disease. Mifepristone effectively controlled all signs and symptoms of hypercortisolism. Menstruating women who take the drug on a long-term basis should receive periodic drug holidays to allow for menses. The lack of reliable serum biomarkers to monitor the success of mifepristone therapy requires careful clinical judgment and may make its use difficult in Cushing disease.

  20. The Cushing Reflex: Oliguria as a Reflection of an Elevated Intracranial Pressure

    Directory of Open Access Journals (Sweden)

    K. Leyssens

    2017-01-01

    Full Text Available Oliguria is one of the clinical hallmarks of renal failure. The broad differential diagnosis is well known, but a rare cause of oliguria is intracranial hypertension (ICH. The actual knowledge to explain this relationship is scarce. Almost all literature is about animals where authors describe the Cushing reflex in response to ICH. We hypothesize that the Cushing reflex is translated towards the sympathetic nervous system and renin-angiotensin-aldosterone system with a subsequent reduction in medullary blood flow and oliguria. Recently, we were confronted with a patient who had complicated pituitary surgery and displayed multiple times an oliguria while he developed ICH.

  1. Endoscopic transsphenoidal surgery for cushing disease: techniques, outcomes, and predictors of remission.

    Science.gov (United States)

    Starke, Robert M; Reames, Davis L; Chen, Ching-Jen; Laws, Edward R; Jane, John A

    2013-02-01

    The efficacy of endoscopic transsphenoidal surgery (ETS) for Cushing disease has not been clearly established. To assess efficacy of a pure endoscopic approach for treatment of Cushing disease and determine predictors of remission. A prospectively acquired database of 61 patients undergoing ETS was reviewed. Remission was defined as postoperative morning serum cortisol of Cushing [100%], macroadenomas [87%]). At 2- to 3-month evaluations, 45 of 49 patients (91.8%) were in remission. Fifty patients were followed for at least 12 months (mean, 28 months; range, 12-72). Forty-two (84%) achieved remission from a single ETS. In these patients, there was no significant difference in remission rates between microadenomas (93%), magnetic resonance imaging-negative (70%), and macroadenomas (77%). Patients with history of previous surgery (n = 14, 23%) were 9 times less likely to achieve follow-up remission (P = .021). In-house cortisol level of Cushing disease provides high rates of remission with low rates of complications regardless of size. Although patients with a history of previous surgery are less likely to achieve remission, the majority can still achieve remission following treatment.

  2. Evidence for orphan nuclear receptor TR4 in the etiology of Cushing disease

    NARCIS (Netherlands)

    Du, Li; Bergsneider, Marvin; Mirsadraei, Leili; Young, Steven H.; Jonker, Johan W.; Downes, Michael; Yong, William H.; Evans, Ronald M.; Heaney, Anthony P.

    2013-01-01

    Cushing disease (CD) is a life-threatening disorder attributed to excess pituitary tumor-derived adrenocorticotrophic hormone (ACTH) and adrenal steroid secretion caused by pituitary tumors. Whereas CD was first described in 1932, the underlying genetic basis driving tumor growth and ACTH secretion

  3. Central diabetes insipidus after transsphenoidal surgery in dogs with Cushing's disease.

    Science.gov (United States)

    Teshima, Takahiro; Hara, Yasushi; Taoda, Takahiro; Teramoto, Akira; Tagawa, Masahiro

    2011-01-01

    Transsphenoidal surgery (TSS) is an effective treatment for canine Cushing's disease, as well as human Cushing's disease. In humans, only the pituitary adenoma tissue is resected by TSS. However, in dogs, the whole pituitary including normal tissue is resected. Hence, central diabetes insipidus (CDI) may complicate the postoperative course in almost all dogs with Cushing's disease treated by TSS. However, it is difficult to assess the duration of the postoperative CDI, and whether it may be transient or permanent. In this study, postoperative CDI in 21 dogs with Cushing's disease and its predicted prognosis by preoperative parameters was investigated. In this study, CDI after TSS was classified as either transient or permanent based on the requirement for desmopressin. Preoperative circulating serum cortisol concentrations and pre- and post-operative plasma AVP concentrations were not significantly different between the transient CDI dogs and permanent CDI dogs. The duration of postoperative CDI was not correlated to the signal intensity ratio (posterior lobe of the pituitary/cerebral cortex), which is obtained from preoperative magnetic resonance imaging (MRI). However, the pituitary height/brain area (P/B) ratio of the permanent CDI dogs was significantly greater than that of the transient CDI dogs. In addition, there was a significant difference between the enlarged-pituitary (P/B ratio > 0.31) transient CDI dogs and the permanent CDI dogs. These results suggest that the incidence of postoperative permanent CDI is strongly influenced by the pituitary size.

  4. Cytoenzymologic activities of some oxidroeductases and alkaline phosphatase of leucocytes in Basedow, Cushing and Addison diseases.

    Science.gov (United States)

    Tasca, L; Onicescu, D

    1975-01-01

    Leukocytes, approached by histoenzymological methods for demonstration of dihydrofolate dehydrogenase, NADH2-diaphorase, lactate dehydrogenase and alcaline phosphatase activities, provided information about the impaired metabolic balance of thyrotoxicosis, Cushing and Addison diseases. The most relevant variations were found in thyrotoxicosis, the most sensitive enzyme was dihydrofolate dehydrogenase and the less sensitive was alcaline phosphatase. The neutrophils and lymphocytes had more evident enzymic variations.

  5. Long-term follow-up on Cushing disease patient after transsphenoidal surgery

    Directory of Open Access Journals (Sweden)

    Insook Jeong

    2014-09-01

    Full Text Available Cushing disease is caused by excessive adrenocorticotropic hormone (ACTH production by the pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed-up on a 16-year-old male Cushing disease patient who presented with rapid weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free cortisol and lack of overnight cortisol suppression by low-dose dexamethasone test. The serum cortisol and 24-hour urine free cortisol, by high-dose dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free cortisol levels were persistently high. Thus the patient underwent a total hypophysectomy, since which time he has been treated with hydrocortisone, levothyroxine, recombinant human growth hormone, and testosterone enanthate. Intravenous bisphosphonate for osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a Cushing disease patient according to growth profile, pubertal status, and responses to hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of Cushing disease patients who have undergone transsphenoidal surgery.

  6. Long-term follow-up on Cushing disease patient after transsphenoidal surgery.

    Science.gov (United States)

    Jeong, Insook; Oh, Moonyeon; Kim, Ja Hye; Cho, Ja Hyang; Choi, Jin-Ho; Yoo, Han-Wook

    2014-09-01

    Cushing disease is caused by excessive adrenocorticotropic hormone (ACTH) production by the pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed-up on a 16-year-old male Cushing disease patient who presented with rapid weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free cortisol and lack of overnight cortisol suppression by low-dose dexamethasone test. The serum cortisol and 24-hour urine free cortisol, by high-dose dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free cortisol levels were persistently high. Thus the patient underwent a total hypophysectomy, since which time he has been treated with hydrocortisone, levothyroxine, recombinant human growth hormone, and testosterone enanthate. Intravenous bisphosphonate for osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a Cushing disease patient according to growth profile, pubertal status, and responses to hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of Cushing disease patients who have undergone transsphenoidal surgery.

  7. Harvey Cushing as a book collector, bibliophile, and archivist: the precedence for the genesis of the Brain Tumor Registry. Historical vignette.

    Science.gov (United States)

    Wahl, Christopher J; Tubbs, R Shane; Spencer, Dennis D; Cohen-Gadol, Aaron A

    2009-11-01

    Influenced by individuals such as his parents, Osler, and Halsted, and by his early medical student experience, Harvey Cushing developed a strong interest in collecting, especially antiquarian medical books. Even today, his collection housed at Yale University is one of the most prestigious in the world. Cushing's interest in archives is further manifested and reinforced by his establishment of the Cushing Brain Tumor Registry. The following is a review of Cushing's background not as an eminent clinician and surgeon but as an individual best described as a bibliophile, archivist, and ardent collector of medical paraphernalia.

  8. Prolactinomas, Cushing's disease and acromegaly: debating the role of medical therapy for secretory pituitary adenomas

    Directory of Open Access Journals (Sweden)

    Bonert Vivien S

    2010-05-01

    Full Text Available Abstract Pituitary adenomas are associated with a variety of clinical manifestations resulting from excessive hormone secretion and tumor mass effects, and require a multidisciplinary management approach. This article discusses the treatment modalities for the management of patients with a prolactinoma, Cushing's disease and acromegaly, and summarizes the options for medical therapy in these patients. First-line treatment of prolactinomas is pharmacotherapy with dopamine agonists; recent reports of cardiac valve abnormalities associated with this class of medication in Parkinson's disease has prompted study in hyperprolactinemic populations. Patients with resistance to dopamine agonists may require other treatment. First-line treatment of Cushing's disease is pituitary surgery by a surgeon with experience in this condition. Current medical options for Cushing's disease block adrenal cortisol production, but do not treat the underlying disease. Pituitary-directed medical therapies are now being explored. In several small studies, the dopamine agonist cabergoline normalized urinary free cortisol in some patients. The multi-receptor targeted somatostatin analogue pasireotide (SOM230 shows promise as a pituitary-directed medical therapy in Cushing's disease; further studies will determine its efficacy and safety. Radiation therapy, with medical adrenal blockade while awaiting the effects of radiation, and bilateral adrenalectomy remain standard treatment options for patients not cured with pituitary surgery. In patients with acromegaly, surgery remains the first-line treatment option when the tumor is likely to be completely resected, or for debulking, especially when the tumor is compressing neurovisual structures. Primary therapy with somatostatin analogues has been used in some patients with large extrasellar tumors not amenable to surgical cure, patients at high surgical risk and patients who decline surgery. Pegvisomant is indicated in patients

  9. Ectopic adrenocorticotropic hormone syndrome presenting as hypokalemic metabolic alkalosis and hypertension

    Directory of Open Access Journals (Sweden)

    Mansoor C Abdulla

    2016-01-01

    Full Text Available The ectopic adrenocorticotropic hormone (ACTH syndrome is an uncommon cause of hypercortisolism, which should be considered in patients with hypokalemic metabolic alkalosis and hypertension in the context of lung neoplasm. We report a 60-year-old male patient with severe hypertension, metabolic alkalosis, and hypokalemia as the initial manifestations of an ACTH-secreting small cell lung carcinoma. Ectopic Cushing's syndrome should always be ruled out in patients with severe hypertension and hypokalemia.

  10. Pituitary function following megavoltage therapy for Cushings' disease; long term follow up

    International Nuclear Information System (INIS)

    Sharpe, G.F.; Kendall-Taylor, P.; Prescott, R.W.G.; Ross, W.M.; Davison, C.; Watson, M.J.; Cook, D.B.

    1985-01-01

    Eight patients who had received megavoltage therapy for Cushings' disease 5-12 years previously have been reviewed. The long term response to this therapy was assessed with respect to efficacy of treatment in inducing continued remission and disturbance of hypothalamic-pituitary function. One patient showed clear evidence of relapse of Cushings' disease. One patient had unequivocal hypopituitarism. Basal levels of growth hormone (GH), TSH, LH, and FSH were not statistically different from controls, but provocative testing revealed significant abnormalities of response of cortisol/ACTH, GH, prolactin and LH. Six out of eight patients had absent diurnal cortisol variation and five patients had elevated serum prolactin levels. Thus, in this group of patients normal pituitary-adrenal function has not been satisfactorily restored. It is clear that significant disturbances of hypothalamic-pituitary function follow megavoltage therapy and these may progress to overt hypopituitarism. (author)

  11. CyberKnife Radiosurgery in the Multimodal Management of Patients with Cushing Disease.

    Science.gov (United States)

    Moore, Justin M; Sala, Elisa; Amorin, Alvaro; Martinez, Hector; Bhowmik, Aprotim C; Chang, Steven D; Soltys, Scott G; Harsh, Griffith R; Katznelson, Laurence

    2018-04-01

    Surgery is the primary treatment for Cushing disease. When surgery is unsuccessful in normalizing hypercortisolism, adjuvant radiation, such as stereotactic radiosurgery, may be useful to improve biochemical control. This retrospective study included a cohort of consecutive patients treated with CyberKnife (CK) radiosurgery for active Cushing disease at Stanford Hospital and Clinics. As first-line treatment, all patients underwent transsphenoidal surgery with histologic demonstration of an adrenocorticotropic hormone-producing pituitary adenoma. CK was performed as adjuvant therapy for persistent or recurrent disease. The median time between surgery and CK was 14 ± 34 months. Before CK, median maximal diameter of tumors was 9 mm (range, 7-32 mm), with cavernous sinus invasion in all patients (100%) and abutment of the optic chiasm in 1 patient (14.2%). With an average follow-up of 55.4 months, normalization of hypercortisolism was achieved in 4 patients (57.1%): 2 patients (28.5%) achieved normalization of the hypothalamic-pituitary-adrenal axis without glucocorticoid replacement, and 2 patients developed hypoadrenalism (28.5%). The median time to biochemical remission was 12.5 months. Hypopituitarism occurred in only 1 patient (14.2%), and no patients had visual complications. Time between surgery and radiotherapy of Cushing disease, we demonstrate that CK is an effective treatment with rare complications. Copyright © 2018 Elsevier Inc. All rights reserved.

  12. Postoperative plasma cortisol levels predict long-term outcome in patients with Cushing's disease and determine which patients should be treated with pituitary irradiation after surgery

    Energy Technology Data Exchange (ETDEWEB)

    Imaki, Toshihiro; Tsushima, Toshio; Hizuka, Naomi; Odagiri, Emi; Murata, Yoji; Takano, Kazue [Tokyo Women' s Medical Univ. (Japan). Inst. of Clinical Endocrinology; Suda, Toshihiro

    2001-02-01

    Transsphenoidal surgery is the treatment of choice for ACTH-producing pituitary adenoma (Cushing's disease) and pituitary irradiation is widely considered the most appropriate treatment for patients with Cushing's disease for whom transsphenoidal surgery has been unsuccessful. We studied 49 consecutive patients who underwent transsphenoidal surgery for the treatment of Cushing's disease at Tokyo Women's Medical University from 1977-1997 with a mean follow-up duration of 87.6 months (range, 24-253 months). We examined the relationship between postoperative endocrinological data, assessed between 3 and 8 weeks after surgery, and long-term outcome and efficacy of pituitary irradiation after surgery. Long-term remission was defined as the regression of the symptom and signs of Cushing's syndrome, and restoration of normal levels of plasma ACTH, cortisol and urinary free cortisol, together with adequate suppression of morning plasma cortisol levels following the administration of low dose (1 mg) of dexamethasone. Thirty patients had no additional treatment after pituitary surgery. Only 1 of 25 patients (4%) whose postoperative plasma cortisol level was less than 2 {mu}g/dl developed recurrent disease whereas 3 out of 5 patients with postoperative plasma cortisol levels higher than 2 {mu}g/dl relapsed. Postoperative external pituitary radiation was used to treat the remaining 19 patients. Four patients who received radiation therapy had a low or undetectable postoperative plasma cortisol level (<2 {mu}g/dl, 56 nmol/L) and all of these patients developed hypopituitarism whereas 5 patients with subnormal plasma cortisol levels (2.0-10.0 {mu}g/dl) remained in remission. Among 10 patients with persistent disease after surgery, 6 entered remission 6-47 months after irradiation but one of them subsequently relapsed after 108 months. These results suggest that additional therapy should be avoided in patients with a postoperative plasma cortisol less

  13. Cushing`s disease: Fibrinogen and D-dimer levels fail to normalize despite early postoperative remission - a prospective, controlled study.

    Science.gov (United States)

    Witek, Przemysław; Zieliński, Grzegorz; Szamotulska, Katarzyna; Witek, Joanna; Kamiński, Grzegorz

    2016-01-01

    Effective transsphenoidal surgery (TSS) for Cushing`s disease (CD) normalizes cortisol levels and reduces complications of hypercortisolism. However, there is evidence of increased cardiovascular morbidity even after successful surgery. A prospective, controlled study on the dynamics of fibrinogen and D-dimer levels with a six-month follow-up after an effective TSS for CD. Forty patients with CD and forty healthy age- and sex-matched subjects were included. We assessed ACTH, urinary and serum cortisol, and fibrinogen and D-dimer levels before TSS and during follow-up. Baseline BMI (P < 0.001), fibrinogen (P = 0.002), and D-dimer (P = 0.001) levels in CD patients were significantly higher than those in healthy controls. High fibrinogen levels in the CD group were independent of BMI, and were positively associated with hsCRP (rS = 0.61, P < 0.001) and arterial hypertension (P = 0.029). After the six-month follow-up we confirmed a sustained difference between the remission group and controls in fibrinogen and D-dimer levels (P = 0.001 and P = 0.017, respectively). Despite early biochemical remission of CD the levels of fibrinogen and D-dimer failed to decrease. This probably contributes to the high risk of thrombotic events and indicates the need for a close follow-up for signs of thromboembolic and cardiovascular complications in patients with early CD remission. (Endokrynol Pol 2016; 67 (3): 283-291).

  14. Outcome of using the histological pseudocapsule as a surgical capsule in Cushing disease.

    Science.gov (United States)

    Jagannathan, Jay; Smith, Rene; DeVroom, Hetty L; Vortmeyer, Alexander O; Stratakis, Constantine A; Nieman, Lynnette K; Oldfield, Edward H

    2009-09-01

    Many patients with Cushing disease still have active or recurrent disease after pituitary surgery. The histological pseudocapsule of a pituitary adenoma is a layer of compressed normal anterior lobe that surrounds the adenoma and can be used during surgery to identify and guide removal of the tumor. In this study the authors examined the results of using the pseudocapsule as a surgical capsule in the resection of adenomas in patients with Cushing disease. The authors reviewed a prospective database of data obtained in patients with Cushing disease who underwent surgery. The analysis included all cases in which a lesion was identified during surgery and in which the lesion was believed to be confined to the pituitary gland in patients with Cushing disease between January 1990 and March 2007. Since the objective was to determine the success of using the pseudocapsule as a surgical capsule, patients with invasive tumors and patients in whom no lesion was identified during surgery-challenging cases for surgical success-were excluded from analysis. In 261 patients an encapsulated adenoma was identified at surgery. Tumor was visible on MR imaging in 135 patients (52%); in 126 patients (48%) MR imaging detected no tumor. The range of tumor size overlapped considerably in the groups with positive and negative MR imaging results, indicating that in addition to size other features of the adenoma influence the results of MR imaging. In 252 patients hypercortisolism resolved after the first operation, whereas in 9 patients (3 with positive MR imaging and 6 with negative MR imaging) early reoperation was required. Hypercortisolism resolved in all 261 patients (256 with hypocortisolism and 5 with eucortisolism) before hospital discharge. Forty-six patients (18%) had postoperative electrolyte abnormalities (30 with hyponatremia and 16 with diabetes insipidus), but only 2 patients required treatment at discharge. The mean clinical follow-up duration was 84 months (range 12

  15. Harvey Cushing's repair of a dural defect after a traumatic brain injury: novel use of a fat graft.

    Science.gov (United States)

    Zaidi, Hasan A; Pendleton, Courtney; Cohen-Gadol, Aaron A; Quinones-Hinojosa, Alfredo

    2011-01-01

    A review of Harvey Cushing's surgical cases at Johns Hopkins Hospital revealed new information about his early work with the use of fat grafts to close dural defects. The Johns Hopkins Hospital surgical records from 1896 to 1912 were reviewed. A single case in which Cushing used an autologous fat graft to repair a dural defect in 1912 after a traumatic brain injury was selected for further study. An 18-year-old white female patient presented with recurring seizures in her sleep approximately 12 years after a traumatic brain injury. A depressed skull fracture as a result of this injury was explored by Cushing, and a dural defect was found. Fat was harvested from the thigh of the patient and was sutured in layers to cover the defect. Cushing noted that this was the first of its kind of operation and "probably of very little use." The patient was discharged within 2 weeks after the operation despite recurrence of her seizure episodes. Despite its questionable functional success, we report herein a previously unpublished operative case by Harvey Cushing's in which an autologous fat graft was used to close a traumatic wound resulting in a dural defect. This report predates currently known published reports of the use of fat to seal dural defects. Cushing recognized that a factor found in fat tissue may aid in wound healing. Contemporary studies indicate the presence of mesenchymal stem cells in fat tissue may be responsible for the accelerated healing and reduced incidence of cerebral spinal fluid leaks after cranial surgery. Copyright © 2011 Elsevier Inc. All rights reserved.

  16. A rare case of multiple pituitary adenomas in an adolescent Cushing disease presenting as a vertebral compression fracture

    Directory of Open Access Journals (Sweden)

    Ji-Yeon Song

    2017-09-01

    Full Text Available Cushing disease in children and adolescents, especially with multiple pituitary adenomas (MPAs, is very rare. We report 17-year-old boy with MPAs. He presented with a vertebral compression fracture, weight gain, short stature, headache, and hypertension. On magnetic resonance imaging (MRI, only a left pituitary microadenoma was found. After surgery, transient clinical improvement was observed but headache and hypertension were observed again after 3 months later. Follow-up MRI showed a newly developed right pituitary microadenoma 6 months after the surgery. The need for careful clinical and radiographic follow-up should be emphasized in the search for potential MPAs in patients with persistent Cushing disease.

  17. A rare case of multiple pituitary adenomas in an adolescent Cushing disease presenting as a vertebral compression fracture.

    Science.gov (United States)

    Song, Ji-Yeon; Mun, Sue-Jean; Sung, Soon-Ki; Hwang, Jae-Yeon; Baik, Seung-Kug; Kim, Jee Yeon; Cheon, Chong-Kun; Kim, Su-Young; Kim, Yoo-Mi

    2017-09-01

    Cushing disease in children and adolescents, especially with multiple pituitary adenomas (MPAs), is very rare. We report 17-year-old boy with MPAs. He presented with a vertebral compression fracture, weight gain, short stature, headache, and hypertension. On magnetic resonance imaging (MRI), only a left pituitary microadenoma was found. After surgery, transient clinical improvement was observed but headache and hypertension were observed again after 3 months later. Follow-up MRI showed a newly developed right pituitary microadenoma 6 months after the surgery. The need for careful clinical and radiographic follow-up should be emphasized in the search for potential MPAs in patients with persistent Cushing disease.

  18. Outcome of Transsphenoidal Surgery for Cushing Disease: A Single-Center Experience Over 32 Years.

    Science.gov (United States)

    Chandler, William F; Barkan, Ariel L; Hollon, Todd; Sakharova, Alla; Sack, Jayson; Brahma, Barunashish; Schteingart, David E

    2016-02-01

    Transsphenoidal surgery is the standard approach for treating Cushing disease. Evidence is needed to document effectiveness. To analyze results of transsphenoidal surgery in 276 consecutive patients, including 19 children. Medical records were reviewed for patients treated initially with surgery for Cushing disease from 1980 to 2012. Radiographic features, pathology, remissions, recurrences, and complications were recorded. Patients were categorized for statistical analysis based on tumor size (microadenomas, macroadenomas, and negative imaging) and remission type (type 1 = morning cortisol ≤3 μg/dL; type 2 = morning cortisol normal). Females comprised 78% of patients and were older than men. Imaging showed 50% microadenomas, 13% macroadenomas, and 37% negative for tumor. Remission rates for microadenomas, macroadenomas, and negative imaging were 89%, 66%, and 71%, respectively. Patients with microadenomas were more likely to have type 1 remission. Pathology showed adrenocorticotropic hormone-secreting adenomas in 82% of microadenomas, in 100% of macroadenomas, and in 43% of negative imaging. The incidence of hyperplasia was 8%. The finding of hyperplasia or no tumor on pathology predicted treatment failure. The recurrence rate was 17%, with an average time to recurrence of 4.0 years. Patients with type 1 remission had a lower rate of recurrence (13% type 1 vs 50% type 2) and a longer time to recurrence. Children had similar imaging findings, remission rates, and pathology. There were no operative deaths. Transsphenoidal surgery provides a safe and effective treatment for Cushing disease. For both adults and children, the best outcomes occurred in patients with microadenomas and/or those with type 1 remission.

  19. Metabolic complications of endogenous Cushing: patient selection for screening

    Directory of Open Access Journals (Sweden)

    Zh E Belaya

    2013-03-01

    Full Text Available Aims: this study evaluates the most common associations of symptoms and complications in patients with Cushing’s syndrome (CS in order to choose a potential population to be screened for CS and estimates the diagnostic accuracy of first line screening tests (cortisol, ACTH to differentiate ACTH-ectopic CS from Cushing’s disease. Materials and Methods: The clinical data of 259 patients with proven CS during 2001–2011 was analyzed. The clinical presentations of 197 patients (159 Cushing’s disease, 28 ACTH-ectopic CS and 10 cases of benign cortisol-secreting adrenal adenoma were compared according to the cause of hypercortisolism. ROC-analysis was performed to estimate the diagnostic accuracy of the first line tests (cortisol, ACTH to suggest ACTH-ectopic CS. A threshold for the test with the highest area under the curves was chosen based on the maximum sum of the sensitivity and specificity. Results: The most frequent complaints were related to fatigue, muscle weakness, weight gain and changes in appearance (facial plethora and fullness, striae. Among the complications of CS the most frequent were being overweight or obese (71%, hypertension (63%, dislipoproteinemia (41%, low traumatic fractures (43% and steroid-induced diabetes (31%. In women, 16% were older than 50, in those who were younger amenorrhea was registered in 43%. The patients with ACTH-ectopic CS had higher rate of low traumatic fractures (p=0.04, increased serum late-night cortisol, 24 hours urinary free cortisol, morning and evening ACTH and lower levels of potassium (p<0.01 for all parameters. Plasma late-night ACTH measurements showed the highest AUC (0,811 (95% CI 0,712–0,909 to differentiate ACTH-ectopic CS from Cushing’s disease. A cut off value of 108.9 pg/ml for late-night ACTH yielded a sensitivity of 60,7% and a specificity of 79%. Conclusions: patients with a coexistence of obesity, muscle weakness, fatigue, some components of metabolic syndrome and especially

  20. Cushing's disease as a cause of severe osteoporosis: a clinical challenge.

    Science.gov (United States)

    Abdel-Kader, Nadia; Cardiel, Mario H; Navarro Compan, Victoria; Piedra Priego, Juan; González, Ana

    2012-01-01

    Secondary osteoporosis is a frequently underestimated bone disorder. It is a secondary cause of bone loss that affects more than half of men and premenopausal and perimenopausal women, and about one-fitfth of postmenopausal women. We herein report an uncommon case of multiple fractures due to secondary osteoporosis caused by Cushing's disease. In this case the appearance of fractures in a 41 years old woman was the sign of alarm that ultimately lead us to the diagnosis. Copyright © 2011 Elsevier España, S.L. All rights reserved.

  1. Cushing's syndrome due to pharmacological interaction in a cystic fibrosis patient

    DEFF Research Database (Denmark)

    Main, K M; Skov, M; Sillesen, Ida Blok

    2002-01-01

    and 1,600 microg budesonide. The patient experienced symptoms of striae, moon-face, increased facial hair growth, mood swings, headaches, weight gain, irregular menstruation despite oral contraceptives and increasing insulin requirement for diabetes mellitus. Endocrine investigations revealed total...

  2. The usefulness of tomodensitometry in the aetiological diagnosis of Cushing's syndrome [hypercorticism

    International Nuclear Information System (INIS)

    Delisle, F.

    1996-01-01

    By using tomodensitometry it is possible to observe, during the same examination, the pituitary region and the adrenal glands. Possible abnormalities in these regions can be interpreted to determine the cause of spontaneous hypercorticism and in this way treatment can be better adapted to the situation

  3. [Atypical Cushing's syndrome in a dog. A case report. Ein Fallbericht].

    Science.gov (United States)

    Hoffrogge, Stefanie; Fels, Lena; Schmicke, Marion; Mischke, Reinhard

    2017-06-20

    In a 12-year-old male Labrador Retriever, presented due to other disease symptoms, clinical signs of hyperadrenocorticism (polyuria, polydipsia, abdominal distention, muscle atrophy) were an incidental finding. Abnormal laboratory results and sonographic findings of the adrenal glands, but negative low-dose dexamethasone suppression tests with low basal cortisol concentrations, a negative andrenocorticotropic hormone (ACTH)-stimulation test and exclusion of iatrogenic hyperadrenocorticism, suggested an atypical hyperadrenocorticism (AHAC). Results of further examinations, particularly stimulation of progesterone production by ACTH (0 h value: 0.21 ng/ml; 1 h value: 4.9 ng/ml) and good response to therapy with trilostane, supported this diagnosis. However, it has to be critically considered, whether and to what extent additionally present diseases (arthroses, testicular tumour) played a role regarding the symptoms and laboratory results in this dog. This case illustrates the difficulties with the diagnosis of AHAC.

  4. Orbital floor triamcinolone causing Cushing's syndrome in a patient treated with Kaletra for HIV 1.

    Science.gov (United States)

    McConkey, Hannah Zelie Ruth; Williams, Helen; Kulasegaram, Ranjababu; Graham, Elizabeth

    2013-02-25

    We report the first known case of iatrogenic cushingoid features following orbital floor triamcinolone, a synthetic corticosteroid, in a patient taking Kaletra (200 mg lopinavir/50 mg ritonavir) twice daily and Truvada (tenofovir/emtricitabine) once daily for HIV 1. Previous reports have included similar findings following epidural triamcinolone injections and with inhaled fluticasone.

  5. Impaired microvascular reactivity and endothelial function in patients with Cushing's syndrome

    Czech Academy of Sciences Publication Activity Database

    Durovcová, V.; Prázný, M.; Ježková, J.; Horová, E.; Hána, V.; Kvasnička, J.; Pecen, Ladislav; Marek, J.; Škrha, J.; Kršek, M.

    2008-01-01

    Roč. 16, - (2008), P400 ISSN 1479-6848. [European Congress of Endocrinology. 03.05.2008-07.05.2008, Berlin] Institutional research plan: CEZ:AV0Z10300504 Subject RIV: FB - Endocrinology, Diabetology, Metabolism, Nutrition

  6. Genetics Home Reference: primary macronodular adrenal hyperplasia

    Science.gov (United States)

    ... Support and Research Foundation: Genetic Changes Found in Cushing's Disease, Adrenal Tumors, and Adrenal Hyperplasia MalaCards: acth-independent ... macronodular adrenal hyperplasia 2 Merck Manual (Home Edition): Cushing ... Adrenal Diseases Foundation: Cushing's Syndrome Orphanet: Cushing syndrome due to ...

  7. Clinical features of GH deficiency and effects of 3 years of GH replacement in adults with controlled Cushing's disease

    DEFF Research Database (Denmark)

    Höybye, Charlotte; Ragnarsson, Oskar; Jönsson, Peter J

    2010-01-01

    Patients in remission from Cushing's disease (CD) have many clinical features that are difficult to distinguish from those of concomitant GH deficiency (GHD). In this study, we evaluated the features of GHD in a large cohort of controlled CD patients, and assessed the effect of GH treatment....

  8. Increased intracranial pressure and cardiac arrest after heart transplantation. What about the Cushing response in a denervated heart? Case report.

    Science.gov (United States)

    Moskopp, D; Kehl, G; Horch, C; Puskás, Z; Wassmann, H; Schuierer, G; Fingerhut, D

    1994-01-01

    A 13-year-old boy suffered cerebrovascular complications after heart transplantation (ischemic mass effect in the posterior cranial fossa). He had to be resuscitated from cardiac arrest with coma. After a modified cerebellar hemispherectomy the course was favorable.--The most conclusive explanation for the acute event is that a Cushing response was preserved even in the presumably denervated heart.

  9. Results of treatment of Icenko-Cushing disease with proton beam irradiation of the pituitary gland

    International Nuclear Information System (INIS)

    Marova, E.I.; Starkova, N.T.; Kirpatovskaya, L.E.; Kolesnikova, G.S.; Bukhman, A.I.; Rozhinskaya, L.Ya.; Bel'chenko, L.V.

    1987-01-01

    Proton beam therapy was given to 98 patients with Icenko-Cushing disease aged 15 to 40. Mild cases were treated by proton beam irradiation only while severe cases were managed using proton beam therapy combined with unilateral adrenalectomy or ortho-para-DDD. Catamnesis duration varied from 3 to 5 years. In most cases the exposure dose was 80-90 Gy (50-110 Gy). The procedure was well tolerated by all the patients. A dynamic multipolar converting method with 15-20 entrance poles in the left temporal area was employed (with the beam energy of 200 MeV). Stabilization of the course of disease and some clinical improvement were observed in most of the patients 3-4 months after proton beam therapy. In 6-36 months after irradiation 90% of the patients showed normal biochemical indices and the absence of any clinical signs of the disease. Thus the results of proton beam therapy of 98 patients with Icenko-Cushing disease after a follow-up of 3-5 years showed a high efficacy of this type of treatment. The method can be used alone or in combination with unilateral adrenalectomy as well as with oral administration of ortho-para-DDD

  10. Currently used and investigational drugs for Cushing´s disease.

    Science.gov (United States)

    Ciato, Denis; Mumbach, Aizhar G; Paez-Pereda, Marcelo; Stalla, Günter K

    2017-01-01

    Cushing's disease (CD) is caused by a corticotroph adenoma of the pituitary gland that secretes excess adrenocorticotropic hormone (ACTH) causing increased morbidity and mortality. Surgery is the treatment of choice, but is not always successful. Alternatives include radiotherapy, adrenal surgery, and pharmaceutical therapy. The latter is increasingly gaining momentum due to the recent development of compounds that reduce hypercortisolaemia or its symptoms, acting through different mechanisms. Areas covered: In this article, the authors provide a complete overview of the treatment options for Cushing´s disease, including adrenal-directed, tumor-targeted, and peripheral therapies that are currently used or in development, and discuss their potential advantages and limitations. Expert opinion: Considering the lack of long-term remission in up to half of the patients after surgery, and the delayed response to radiotherapy along with potential side effects, there is a strong need for an effective pharmaceutical treatment. Pasireotide, mifepristone, ketoconazole and metyrapone have been approved by regulatory authorities but their use remains limited due to considerable costs and side effects. Research in this field has focused recently on the improvement of pre-existing drugs and the development of safe new ones. However, few approaches aim at targeting the source of the disease, the ACTH-secreting adenoma.

  11. Long-term results of stereotactic radiosurgery to the pituitary gland in Cushing's disease

    International Nuclear Information System (INIS)

    Degerblad, M.; Raehn, T.; Bergstrand, G.; Thoren, M.

    1986-01-01

    Gamma radiation from 60 Co delivered with stereotactic technique was given to the pituitary gland in 35 patients, aged 18-65 years, with Cushing's disease. The doses were 70-100 Gy in each single irradiation. The size of the sella turcica was normal in the majority of the patients. The observation time was 3-9 years in 29 patiens. Out of them, 14 (48%) obtained clinical remission and normal urinary cortisol after one irradiation. Eight achieved remission after two to four irradiations. In total, 22 out of 29 patients (76%) obtained remission. In 12 of them remission was obtained in 1 year and in another 10 within 3 years. No recurrences were observed. Improvement was seen in 2 patients after one and three irradiations. Bilateral adrenalectomy was performed in 5 patients owing to unsatisfactory effect of irradiation. Pituitary insufficiency with gonadotropin, thyrotropin or cortocotropin failure was demonstrated in 12 of 22 patients in remission. This occurred 4 months to 7 years after the first irradiation. Another 6 patients were followed less than 3 years after the first irradiation. Two obtained remission after the first treatment, whereas the other 4 improved. Stereotactic pituitary irradiation is suggested as a non-invasive therapeutic alternative in Cushing's disease for example in patients with considerable surgical risk or as a supplement to pituitary microsurgery. (author)

  12. Iatrogenic Cushing’s syndrome and Secondary Adrenal Insufficiency in an HIV Patientreceiving Fluticasone and Ritonavir

    OpenAIRE

    Imro Vlasveld; Maarten O. van Aken; Cees Van Nieuwkoop

    2014-01-01

    Objectives: To illustrate that the protease inhibitor (PI) ritonavir, widely used as part of the treatment for HIV, might cause drug–drug interactions with inhaled corticosteroids. Material and methods: A case report is presented. Results: An HIV-positive patient presented with gradually changing body composition that was ascribed to lipodystrophy. Finally, iatrogenic Cushing's syndrome with secondary adrenal insufficiency was diagnosed due to a drug–drug interaction of ritonavir and ...

  13. Comorbidity and cardiovascular risk factors in adult GH deficiency following treatment for Cushing's disease or non-functioning pituitary adenomas during childhood

    DEFF Research Database (Denmark)

    Ragnarsson, Oskar; Höybye, Charlotte; Jönsson, Peter J

    2012-01-01

    Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood.......Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood....

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

    Science.gov (United States)

    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 latter of which is usually due to autonomous adrenal overproduction. The former can be due to a pituitary corticotroph tumor (usually benign) or ectopic ACTH production from tumors outside the pituitary; both of these tumor types overexpress the proopiomelanocortin gene. The converse of Cushing’s syndrome is the lack of normal cortisol secretion and is usually due to adrenal destruction (primary adrenal insufficiency) or hypopituitarism (secondary adrenal insufficiency). Secondary adrenal insufficiency can also result from a rapid discontinuation of long-term, pharmacological glucocorticoid therapy because of HPA axis suppression and adrenal atrophy. Finally, mutations in the steroidogenic enzymes of the adrenal cortex can lead to congenital adrenal hyperplasia and an increase in precursor steroids, particularly androgens. When present in utero, this can lead to masculinization of a female fetus. An understanding of the dynamics of the HPA axis is necessary to master the diagnosis and differential diagnosis of pituitary-adrenal diseases. Furthermore, understanding the pathophysiology of the HPA axis gives great insight into its normal control. PMID:24715566

  15. Clinical Guidelines for the Diagnosis and Treatment of Cushing's Disease in Korea

    Directory of Open Access Journals (Sweden)

    Kyu Yeon Hur

    2015-03-01

    Full Text Available Cushing's disease (CD is a rare disorder characterized by the overproduction of adrenocorticotropic hormone due to a pituitary adenoma that ultimately stimulates excessive cortisol secretion from the adrenal glands. Prior to the detection of pituitary adenomas, various clinical signs of CD such as central obesity, moon face, hirsutism, and facial plethora are usually already present. Uncontrolled hypercortisolism is associated with metabolic, cardiovascular, and psychological disorders that result in increased mortality. Hence, the early detection and treatment of CD are not only important but mandatory. Because its clinical manifestations vary from patient to patient and are common in other obesity-related conditions, the precise diagnosis of CD can be problematic. Thus, the present set of guidelines was compiled by Korean experts in this field to assist clinicians with the screening, diagnoses, and treatment of patients with CD using currently available tests and treatment modalities.

  16. Rapid Deterioration of Latent HBV Hepatitis during Cushing Disease and Posttraumatic Stress Disorder after Earthquake.

    Science.gov (United States)

    Tashiro, Ryosuke; Ogawa, Yoshikazu; Tominaga, Teiji

    2017-07-01

    Reactivation of the hepatitis B virus (HBV) is a risk in the 350 million HBV carriers worldwide. HBV reactivation may cause hepatocellular carcinoma, cirrhosis, and fulminant hepatitis, and HBV reactivation accompanied with malignant tumor and/or chemotherapy is a critical problem for patients with chronic HBV infection. Multiple risk factors causing an immunosuppressive state can also induce HBV reactivation.We present a case of HBV reactivation during an immunosuppressive state caused by Cushing disease and physical and psychological stress after a disaster. A 47-year-old Japanese woman was an inactive HBV carrier until the Great East Japan Earthquake occurred and follow-up was discontinued. One year after the earthquake she had intractable hypertension, and her visual acuity gradually worsened. Head magnetic resonance imaging showed a sellar tumor compressing the optic chiasm, and hepatic dysfunction with HBV reactivation was identified. Endocrinologic examination established the diagnosis as Cushing disease. After normalization of hepatic dysfunction with antiviral therapy, transsphenoidal tumor removal was performed that resulted in subtotal removal except the right cavernous portion. Steroid hormone supplementation was discontinued after 3 days of administration, and gamma knife therapy was performed for the residual tumor. Eighteen months after the operation, adrenocorticotropic hormone and cortisol values returned to normal. The patient has been free from tumor regrowth and HBV reactivation throughout the postoperative course.Accomplishment of normalization with intrinsic steroid value with minimization of steroid supplementation should be established. Precise operative procedures and careful treatment planning are essential to avoid HBV reactivation in patients with this threatening condition. Georg Thieme Verlag KG Stuttgart · New York.

  17. Symptomatic cycling Cushing disease managed by simultaneous bilateral laparoscopic adrenalectomy in a 11-year-old boy

    Directory of Open Access Journals (Sweden)

    Khalid M Al-Otaibi

    2014-01-01

    Full Text Available We report symptomatic cycling Cushing disease in a 11-year-old boy that was managed with simultaneous bilateral laparoscopic adrenalectomy. Positioning and the surgical technique have been fully described. Excellent results were achieved. Recent application of laparoscopic adrenalectomy for various adrenal pathology is highly effective and offers better results than open surgery. Post-operative recovery after laparoscopic technique is significantly shorter than the open technique.

  18. A C-terminal HSP90 inhibitor restores glucocorticoid sensitivity and relieves a mouse allograft model of Cushing disease.

    Science.gov (United States)

    Riebold, Mathias; Kozany, Christian; Freiburger, Lee; Sattler, Michael; Buchfelder, Michael; Hausch, Felix; Stalla, Günter K; Paez-Pereda, Marcelo

    2015-03-01

    One function of the glucocorticoid receptor (GR) in corticotroph cells is to suppress the transcription of the gene encoding proopiomelanocortin (POMC), the precursor of the stress hormone adrenocorticotropin (ACTH). Cushing disease is a neuroendocrine condition caused by partially glucocorticoid-resistant corticotroph adenomas that excessively secrete ACTH, which leads to hypercortisolism. Mutations that impair GR function explain glucocorticoid resistance only in sporadic cases. However, the proper folding of GR depends on direct interactions with the chaperone heat shock protein 90 (HSP90, refs. 7,8). We show here that corticotroph adenomas overexpress HSP90 compared to the normal pituitary. N- and C-terminal HSP90 inhibitors act at different steps of the HSP90 catalytic cycle to regulate corticotroph cell proliferation and GR transcriptional activity. C-terminal inhibitors cause the release of mature GR from HSP90, which promotes its exit from the chaperone cycle and potentiates its transcriptional activity in a corticotroph cell line and in primary cultures of human corticotroph adenomas. In an allograft mouse model, the C-terminal HSP90 inhibitor silibinin showed anti-tumorigenic effects, partially reverted hormonal alterations, and alleviated symptoms of Cushing disease. These results suggest that the pathogenesis of Cushing disease caused by overexpression of heat shock proteins and consequently misregulated GR sensitivity may be overcome pharmacologically with an appropriate HSP90 inhibitor.

  19. Cushing disease

    Science.gov (United States)

    ... in the popliteal fossa Striae on the leg References Molitch ME. Anterior pituitary. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 224. Stewart PM, Newell-Price JDC. The adrenal cortex. In: Melmed S, Polonsky KS, ...

  20. ENFERMEDAD DE CUSHING: PRESENTACIÓN DE UN CASO EN PACIENTE JOVEN

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    Brenda Anahí López López

    2014-12-01

    Full Text Available Los adenomas de la hipófisis son la causa más frecuente de los síndromes de hipersecreción e hiposecreción de hormonas hipofisarias. La prevalencia es de 200 casos por millón de habitantes. Los adenomas funcionales, secretan un exceso de la hormona correspondiente a las células afectadas y de ahí se manifiesta su síndrome clínico característico. Se describe el caso de un adenoma hipofisiario  en una paciente de 17 años, quien acude a consulta por presentar acné resistente a tratamientos anteriores. No responde nuevamente al tratamiento indicado y presenta nuevas manifestaciones clínicas (aumento de peso sin causa aparente, estrías violáceas, giba cervical, dolor de cabeza y problemas visuales, se toman los niveles de cortisol plasmático y urinarios, encontrándolos elevados. Se le prescribe 1 mg de dexametasona oral seguido de la medición del nivel de cortisol al día siguiente, además se procede a realizar una resonancia magnética (RNM, que mostró dos imágenes sugestivas de adenomas en la adenohipófisis. Dichas masas fueron extraídas mediante un abordaje endonasal endoscópico transesfenoidal, el estudio patológico confirma que corresponden a adenomas hipofisarios productores de ACTH, confirmando el diagnóstico de Enfermedad de Cushing. La  mayoría de los casos reportados de adenomas hipofisiarios corresponden a pacientes adultos mayores. La  paciente forma parte de un raro número de jóvenes que llegan a presentar este tipo de tumor.  El diagnóstico temprano y la extracción oportuna de los adenomas propicia a que los efectos producidos por el exceso de producción hormonal de las células afectadas se reviertan completamente, sin dejar secuelas graves en el paciente. Palabras Clave: Adenoma hipofisario, Enfermedad de Cushing, ACTH (hormona adrenocorticotropa.

  1. Harvey Cushing: first guest at "The Divine Banquet of the Brayne".

    Science.gov (United States)

    Modlin, I M; Shin, J H

    1993-04-01

    Harvey Cushing will remain forever ensconced in surgical memory as the visionary who established contemporary neurosurgery. Raised in Cleveland in a middle-class family with a medical background, he gave little indication early of his later prowess. A college education at Yale University yielded a modest performance, although he showed somewhat better promise as a medical student at Harvard University. During his surgical training at the Johns Hopkins Medical School, he emerged as a bright, industrious young man with a glint of steel to his intellect. From Halsted he learned the academic basis of surgery and the meticulousness necessary for technical success. Osler became his friend and provided him with a foundation in the history of medicine and the recognition of its importance in shaping the future. The decision to spend a year in Europe visiting clinics and renowned surgeons may have laid the foundations for the subsequent configuration of his career. In Britain he saw firsthand the glorious museums and libraries of the Hunters and the legacy that their schools of surgery had endowed to the nation. In Paris and London he mixed with the surgical aristocracy of the times and learned the power of societies and the traditions of their patrons. In Berne he was exposed to the surgical purity and physiologic expertise of Kocher, supported by the estimable scientific practice of Kroneker. As an "arbeid," Kocher posed him the problem of establishing the mechanism of the regulation of cerebral perfusion. This question, which he so rapidly and successfully unraveled, probably played a large part in igniting his enthusiasm toward the study of the brain as a future career. In his travels he encountered diverse intellects and experienced a wide panoply of European medical thought. He conversed widely at both a social and professional level and integrated all information into meticulously kept diaries that served as not only a record but also a template from which later

  2. Treatment of Cushing's disease with adrenal blocking drugs and megavoltage therapy to the pituitary

    International Nuclear Information System (INIS)

    Ross, W.M.; Evered, D.C.; Hunter, P.; Benaim, M.; Cook, D.; Hall, R.

    1979-01-01

    Eighteen patients with Cushing's disease were seen over a 40-month period and considered for treatment by pituitary irradiation and adrenal blocking drugs. Fourteen patients entered the study and each received megavoltage therapy to give a mean dose of 4600 rad to the pituitary over 31 days. Each patient was treated for one (two patients) or two (12 patients) years with one or both of the adrenocortical enzyme inhibitors, metyrapone or aminoglutethimide to suppress cortisol secretion. Doses were adjusted to maintain urinary free cortisol secretion below 300 nmol/24 h. One patient failed to complete the trial. Normal urinary free cortisol excretion and plasma cortisol concentration were maintained after treatment in eight of the remaining 13 patients after therapy. Only one patient required cortisol replacement and normal menstrual function was restored in five of the six women. The remaining five patients relapsed and four were subsequently treated by total adrenalectomy. It was noted that the patients who responded to treatment were substantially younger than the therapeutic failures. It is suggested that this treatment is most useful in the management of younger patients. (author)

  3. Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up.

    Science.gov (United States)

    Valderrábano, Pablo; Aller, Javier; García-Valdecasas, Leopoldo; García-Uría, José; Martín, Laura; Palacios, Nuria; Estrada, Javier

    2014-04-01

    Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). However, the best treatment option when hypercortisolism persists or recurs remains unknown. The aim of this study was to analyze the short and long-term outcome of repeat TSS in this situation and to search for response predictors. Data from 26 patients with persistent (n=11) or recurrent (n=15) hypercortisolism who underwent repeat surgery by a single neurosurgeon between 1982 and 2009 were retrospectively analyzed. Remission was defined as normalization of urinary free cortisol (UFC) levels, and recurrence as presence of elevated UFC levels after having achieved remission. The following potential outcome predictors were analyzed: adrenal status (persistence or recurrence) after initial TSS, tumor identification in imaging tests, degree of hypercortisolism before repeat TSS, same/different surgeon in both TSS, and time to repeat surgery. Immediate postoperative remission was achieved in 12 patients (46.2%). Five of the 10 patients with available follow-up data relapsed after surgery (median time to recurrence, 13 months). New hormone deficiencies were seen in seven patients (37%), and two patients had cerebrospinal fluid leakage. No other major complications occurred. None of the preoperative factors analyzed was predictive of surgical outcome. When compared to initial surgery, repeat TSS for CD is associated to a lower remission rate and a higher risk of recurrence and complications. Further studies are needed to define outcome predictors. Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.

  4. Variability of Late-Night Salivary Cortisol in Cushing Disease: A Prospective Study.

    Science.gov (United States)

    Sandouk, Zahrae; Johnston, Philip; Bunch, Dustin; Wang, Sihe; Bena, James; Hamrahian, Amir; Kennedy, Laurence

    2018-03-01

    The frequency of variable hormonogenesis in patients with Cushing disease (CD) but without cyclical symptoms is unclear. To assess the frequency of variable hormonogenesis in patients presenting with CD. Over a 6-month period, patients with confirmed or suspected CD provided late-night salivary samples for up to 42 consecutive nights. Of 19 patients confirmed to have CD, 16 provided at least 7 consecutive salivary samples, and 13 provided at least 21; these 16 patients are the subjects of this report. Twelve patients had at least three peak and two trough levels of late-night salivary cortisol (LNSC) but in only two patients were strict criteria for cyclical hormonogenesis fulfilled; variation was assessed as random in the others. Eight patients had de novo CD, and eight had recurrent/persistent disease. All patients with recurrent/persistent CD had two or more normal results, and in four of these patients, >50% of LNSC were normal. In six patients with de novo disease with at least one normal LNSC level, the maximum levels ranged from 1.55 to 15.5 times the upper limit of normal. Extreme fluctuations of cortisol production, measured by sequential LNSC, are common in CD. In newly diagnosed disease, this may only occasionally impair diagnostic ability, whereas in most patients with recurrent/persistent disease after pituitary surgery, LNSC is frequently within the reference range, with potential to cause diagnostic problems.

  5. Idiopathic Intracranial Hypertension After Surgical Treatment of Cushing Disease: Case Report and Review of Management Strategies.

    Science.gov (United States)

    Wagner, Jeffrey; Fleseriu, Cara M; Ibrahim, Aly; Cetas, Justin S

    2016-12-01

    Idiopathic intracranial hypertension (IIH) in patients with Cushing disease (CD), after treatment, is rarely described, in adults. The cause is believed to be multifactorial, potentially related to a relative decrease in cortisol after surgical resection or medical treatment of a corticotroph pituitary adenoma. We investigate our center's CD database (140 surgically and 60 medically [primary or adjunct] treated patients) for cases of IIH, describe our center's experience with symptomatic IIH, and review treatment strategies in adults with CD after transsphenoidal resection. We present the case of a 22-year-old woman who presented with worsening headache, nausea, vomiting, blurry vision, diplopia, visual loss, and facial numbness 14 weeks after surgical resection of adrenocorticotropic hormone-positive pituitary adenoma. Her CD had been in remission since surgery, with subsequent adrenal insufficiency (AI), which was initially treated with supraphysiologic glucocorticoid replacement, tapered down to physiologic doses at the time the IIH symptoms developed. Symptomatic IIH is rare in adult patients but can be severe and result in permanent vision loss. A high index of suspicion should be maintained and a fundus examination is necessary to exclude papilledema, whenever there are suggestive symptoms that initially may overlap with AI. It is possible that some cases of mild IIH are misdiagnosed as GC withdrawal or AI; however, further studies are needed. Treatment consists of reinitiation of higher steroid doses together with acetazolamide with or without cerebrospinal fluid diversion and the priority is to preserve vision and reverse any visual loss. Published by Elsevier Inc.

  6. Rapid appearance of transient secondary adrenocortical insufficiency after alpha-particle radiation therapy for Cushing's disease

    International Nuclear Information System (INIS)

    Cook, D.M.; Jordan, R.M.; Kendall, J.W.; Linfoot, J.A.

    1976-01-01

    A 17-year-old woman received 12,000 rads of alpha-particle radiation for the treatment of Cushing's disease. One day after the completion of therapy, the patient developed nausea, vomiting, headache, and postural hypotension. Laboratory evaluation demonstrated a marked fall of the previously elevated urinary 17-hydroxycorticosteroids (17-OHCS) and undetectable plasma cortisols. The urinary 17-OHCS transiently returned to supranormal levels but over a 2 1 / 2 -week period decreased and then remained low. The patient also demonstrated a subnormal urinary aldosterone excretion in relation to plasma renin activity (PRA) during 10 mEq/24 h sodium restriction. The remainder of the endocrine evaluation was normal, suggesting that pituitary function otherwise remained intact. One and one-half years after alpha-particle therapy, the patient's urinary 17-OHCS were normal and responded normally to metyrapone. The relationship between urinary aldosterone excretion and PRA also was normal. It is postulated that there was an infarction of an ACTH secreting pituitary tumor leaving the remainder of the pituitary intact. A chronically elevated circulating level of ACTH with sudden loss of ACTH secretion appeared to have been responsible for the initial low urinary aldosterone as well as the low urinary 17-OHCS. This is the first reported case of a presumed pituitary tumor infarction in association with alpha-particle pituitary radiation

  7. Long term follow-up of patients with Cushing's disease treated by interstitial irradiation

    International Nuclear Information System (INIS)

    Sandler, L.M.; Richards, N.T.; Carr, D.H.; Mashiter, K.; Joplin, G.F.

    1987-01-01

    The first 86 patients with Cushing's disease treated with interstitial irradiation (by needle implantation) as the sole therapy were reviewed. In the 82 patients who were reassessed 1 yr after treatment 63 (77%) achieved remission. This study comprises the outcome and complications in the 54 patients who had a remission and whom we were able to follow. The follow-up period ranged from 3-26 yr (mean, 10.5) from the time of remission. No instance of clinical or radiological relapse has occurred. Of these 54 patients, yttrium-90 alone was used in 32, of whom 12 (37%) required corticosteroid or T4 replacement therapy in a mean time of 3.5 months; in 7 of these 12 we elected to give an ablative dose. Gold-198 alone was used in 15 patients, of whom 7 (47%) developed hypopituitarism in a mean time of 76 months. Both isotopes were used in 7 patients. A diurnal serum cortisol rhythm was found in 28 of the 31 patients who were not receiving corticosteroid therapy. In 5 of the 7 patients with an initially abnormal pituitary fossa, serial radiological studies revealed remodelling in 3. There have been no complications in the last 17 years. Pituitary implantation with yttrium-90 is an effective alternative to transsphenoidal hypophysectomy, with a high remission rate, no recurrence (as yet), no operative complications, and avoidance of hormone replacement in the majority

  8. [Carbohydrate metabolism in patients with acromegaly and Itsenko-Cushing disease].

    Science.gov (United States)

    Matchekhina, L V; Belaya, Zh E; Melnichenko, G A; Shestakova, M V

    2015-01-01

    The relevance of investigating carbohydrate metabolism (CM) in patients with acromegaly and Itsenko-Cushing disease is attributable to frequent glucose metabolic disturbances, on the one hand, and to difficulties in choosing sugar-lowering therapy in these categories of patients, on the other. The efficiency of hyperglycemia treatment in these patients may be reduced due to problems in achieving remission/cure of the underlying disease and to specific therapy favoring hyperglycemia. The top-priority tasks are to search for ways of reducing the frequency of CM abnormalities in patients with neuroendocrine diseases and to elaborate sugar-lowering therapy regimens. There is a growing interest in studies of the role of the incretin system in the pathogenesis of secondary hyperglycemias associated with neuroendocrine diseases. Nevertheless, few works have been published on this subject matter because of its novelty. There is a need for a further closer study of the specific features of incretin system function and the pharmacodynamics of incretin mimetics that are potential candidates as first-line drugs to treat secondary hyperglycemias. This paper attempts to summarize the available data obtained from studies into CM in neuroendocrine diseases.

  9. A prospective study of appetite and food craving in 30 patients with Cushing's disease.

    Science.gov (United States)

    Geer, Eliza B; Lalazar, Yelena; Couto, Lizette M; Cohen, Vanessa; Lipton, Lianna R; Shi, Wei; Bagiella, Emilia; Conwell, Irene; Bederson, Joshua; Kostadinov, Jane; Post, Kalmon D; Freda, Pamela U

    2016-04-01

    Glucocorticoid (GC) exposure increases food intake, but the mechanisms in humans are not known. Investigation of appetite and food craving has not been done in patients with chronic GC exposure due to Cushing's disease (CD), either before or after treatment, and could provide insight into mechanisms of food intake and obesity in these patients. To examine whether surgical remission of CD changes appetite (prospective consumption, hunger, satisfaction, and fullness) and food cravings (sweet, salty, fatty, and savory); and to identify predictors of appetite and craving in CD remission. 30 CD patients, mean age 40.0 years (range 17-70), mean BMI 32.3 ± 6.4, were prospectively studied before and at a mean of 17.4 mo. after remission. At each visit fasting and post-test meal (50% carbohydrate, 35% protein, 15% fat) appetite and craving scores were assessed. Remission decreased prospective consumption, sweet and savory craving (p patients may stimulate the drive to eat by enhancing craving, rather than regulating the sensation of hunger. Continued alterations in appetite regulation due to abdominal fat mass and circulating cortisol could play a role in the cardiovascular and metabolic risk that has been reported in CD patients despite remission.

  10. Kidney Stones as an Underrecognized Clinical Sign in Pediatric Cushing Disease.

    Science.gov (United States)

    Rahman, Sara H; Papadakis, Georgios Z; Keil, Margaret F; Faucz, Fabio R; Lodish, Maya B; Stratakis, Constantine A

    2016-03-01

    To investigate the prevalence of kidney stones in a population of children with Cushing disease (CD) and to compare it with the prevalence of kidney stones in healthy children. Clinical and biochemical data from 139 pediatric patients with CD (68 females, 71 males) were analyzed retrospectively. Computed tomography scans were reviewed for kidney stones. Among 139 patients, 27 with CD (19.4%) had either radiographic evidence and/or a history of kidney stones. Those with kidney stones had higher urine free cortisol (P = .008) and transsphenoidal surgery at an older age (P = .007). The average urinary calcium/creatinine ratio was elevated in patients with CD (0.22 ± 0.11). The prevalence of kidney stones was higher in children with CD than in normal children (19.42% vs 1.0%; P kidney stones are an underestimated complication of pediatric CD, especially when compared with the prevalence of nephrolithiasis in the general pediatric population. Long-term consequences for kidney function are not known and need to be studied. Published by Elsevier Inc.

  11. Voxel-based comparison of brain glucose metabolism between patients with Cushing's disease and healthy subjects

    Directory of Open Access Journals (Sweden)

    Shuai Liu

    2018-01-01

    Full Text Available Cognitive impairment and psychiatric symptoms are common in patients with Cushing's disease (CD owing to elevated levels of glucocorticoids. Molecular neuroimaging methods may help to detect changes in the brain of patients with CD. The aim of this study was to investigate the characteristics of brain metabolism and its association with serum cortisol level in CD. We compared brain metabolism, as measured using [18F]-fluorodeoxyglucose positron emission tomography (FDG PET, between 92 patients with CD and 118 normal subjects on a voxel-wise basis. Pearson correlation was performed to evaluate the association between cerebral FDG uptake and serum cortisol level in patients with CD. We demonstrated that certain brain regions in patients with CD showed significantly increased FDG uptake, including the basal ganglia, anteromedial temporal lobe, thalamus, precentral cortex, and cerebellum. The clusters that demonstrated significantly decreased uptake were mainly located in the medial and lateral frontal cortex, superior and inferior parietal lobule, medial occipital cortex, and insular cortex. The metabolic rate of the majority of these regions was found to be significantly correlated with the serum cortisol level. Our findings may help to explain the underlying mechanisms of cognitive impairment and psychiatric symptoms in patients exposed to excessive glucocorticoids and evaluate the efficacy of treatments during follow-up.

  12. Lack of diurnal rhythm of low molecular weight insulin-like growth factor binding protein in patients with Cushing's disease

    International Nuclear Information System (INIS)

    Degerblad, M.; Povoa, G.; Thoren, M.; Wivall, I.-L.; Hall, K.

    1989-01-01

    A specific radioimmunoassay with antibodies raised against the 25 kD insulin-like growth factor binding protein (25 kD IGFBP) in amniotic fluid was used to measure levels of cross-reacting protein in human serum and plasma. Plasma samples collected continually at 20-min intervals during 24-h in 6 healthy adults revealed a distinct diurnal rhythm in the concentration of 25 kD IGFBP. The lowest levels (9-13 μg/l) were found between 13.00 and 24.00 h with a rise after midnight to maximum levels (23-71 μg/l) between 03.00 and 09.00 h. There was no relation between the patterns of GH and 25 kD IGFBP. In 3 patients with active Cushing's disease, the levels of 25 kD IGFBP in plasma samples collected during 12 h. 19.00-07.00 h, were generally low and without nocturnal variations. One of the patients studied after extirpation of a pituitary adenoma displayed a nocturnal rhythm with maximum levels of 25 kD IGFBP between 03.00 and 07.00 h. Eight patients treated with stereotactic pituitary irradiation owing to Cushing's disease also showed a distinct nocturnal increase of 25 kD IGFBP. The results indicate the existence of a diurnal rhythm of 25 kD IGFBP in adults. Further, low levels and lack of diurnal rhythm of 25 kD IGFBP are demonstrated in Cushing's disease. (author)

  13. Persistent adrenocorticotropin response to desmopressin in the early postoperative period predicts recurrence of Cushing's disease.

    Science.gov (United States)

    Losa, Marco; Bianchi, Roberto; Barzaghi, Raffaella; Giovanelli, Massimo; Mortini, Pietro

    2009-09-01

    Patients with Cushing's disease (CD) often show an ACTH and cortisol response to desmopressin (DDAVP). We tested whether persistence of a positive response to DDAVP after successful surgery identifies patients at risk of CD recurrence. We prospectively included all CD patients who had a positive response to DDAVP before successful surgery from 1995 through 2007. The study was performed at a university hospital. One hundred seventy-four patients with CD, 148 women and 26 men, mean age 36.1 +/- 0.8 yr, were studied. The median follow-up after surgery was 58 months (interquartile range 22-93 months). DDAVP test was performed immediately before and after surgery. An ACTH and cortisol increment of at least 30 and 20% above baseline, respectively, were considered as a positive response to DDAVP. The risk of CD recurrence was analyzed according to the postoperative hormonal response to DDAVP. Recurrence of CD occurred in 19 patients (10.9%). The recurrence-free survival at 5 yr was 89.8% [95% confidence interval (CI) 84.2-95.4]. Patients with a positive ACTH response had a 5-yr recurrence-free survival of 82.6% (95% CI 70.6-94.6%) as compared with 94.0% (95% CI 88.2-99.8%; P < 0.01) in patients without it. Multivariate analysis showed that persistence of a positive ACTH response to DDAVP was significantly associated with CD recurrence. Positive ACTH response to DDAVP after surgery is associated with an increased risk of CD recurrence. However, the specificity and predictive value of this finding are low.

  14. Outcomes of Primary Transsphenoidal Surgery in Cushing Disease: Experience of a Tertiary Center.

    Science.gov (United States)

    Keskin, Fatma Ela; Ozkaya, Hande Mefkure; Bolayirli, Murat; Erden, Secil; Kadioglu, Pınar; Tanriover, Necmettin; Gazioglu, Nurperi

    2017-10-01

    To report the initial and long-term remission rates and related factors, secondary treatments, and outcomes of a series of patients with Cushing disease (CD). We included 147 consecutive adult patients with CD who underwent primary transsphenoidal surgery (TSS) between 1998 and 2014 in this study. Eighty-two were followed up in the Cerrahpasa Medical Faculty Endocrinology and Metabolism outpatient clinic. Patients were requested to attend a long-term remission assessment; 55 could be contacted, and data for the remaining 27 patients' last visit to the outpatient clinics were reviewed for early and late remission. Six patients were excluded from the study. Magnetic resonance imaging (MRI) findings and pathologic results including mitosis, Ki-67 levels, and P53 in immunostaining of all patients were evaluated. Data of 82 patients with CD with an average age of 36 years [interquartile range: 29-47] were analyzed with a mean follow-up of 7.5 years [interquartile range: 5-10]. Overall initial remission rates were 72.3% after TSS. Among the 82 patients, 16 patients had Gamma Knife radiosurgery and 7 patients underwent adrenalectomy. After these additional treatments, the long-term remission rate was found as 69.7%. The highest remission rates were with microadenomas. Recurrence was most frequently seen in patients without tumor evidence on MRI. Patients with high Ki-67 levels had higher recurrence rates in long-term follow-up (P = 0.02). Life-long follow-up for patients with CD seems essential. Undetectable tumors on MRI before TSS and high Ki-67 immunopositivity were found as risk factors for tumor recurrence. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Stereotactic Radiosurgery for Cushing Disease: Results of an International, Multicenter Study.

    Science.gov (United States)

    Mehta, Gautam U; Ding, Dale; Patibandla, Mohana Rao; Kano, Hideyuki; Sisterson, Nathaniel; Su, Yan-Hua; Krsek, Michal; Nabeel, Ahmed M; El-Shehaby, Amr; Kareem, Khaled A; Martinez-Moreno, Nuria; Mathieu, David; McShane, Brendan; Blas, Kevin; Kondziolka, Douglas; Grills, Inga; Lee, John Y; Martinez-Alvarez, Roberto; Reda, Wael A; Liscak, Roman; Lee, Cheng-Chia; Lunsford, L Dade; Vance, Mary Lee; Sheehan, Jason P

    2017-11-01

    Cushing disease (CD) due to adrenocorticotropic hormone-secreting pituitary tumors can be a management challenge. To better understand the outcomes of stereotactic radiosurgery (SRS) for CD and define its role in management. International, multicenter, retrospective cohort analysis. Ten medical centers participating in the International Gamma Knife Research Foundation. Patients with CD with >6 months endocrine follow-up. SRS using Gamma Knife radiosurgery. The primary outcome was control of hypercortisolism (defined as normalization of free urinary cortisol). Radiologic response and adverse radiation effects (AREs) were recorded. In total, 278 patients met inclusion criteria, with a mean follow-up of 5.6 years (0.5 to 20.5 years). Twenty-two patients received SRS as a primary treatment of CD. Mean margin dose was 23.7 Gy. Cumulative initial control of hypercortisolism was 80% at 10 years. Mean time to cortisol normalization was 14.5 months. Recurrences occurred in 18% with initial cortisol normalization. Overall, the rate of durable control of hypercortisolism was 64% at 10 years and 68% among patients who received SRS as a primary treatment. AREs included hypopituitarism (25%) and cranial neuropathy (3%). Visual deficits were related to treatment of tumor within the suprasellar cistern (P = 0.01), whereas both visual (P < 0.0001) and nonvisual cranial neuropathy (P = 0.02) were related to prior pituitary irradiation. SRS for CD is well tolerated and frequently results in control of hypercortisolism. However, recurrences can occur. SRS should be considered for patients with persistent hypercortisolism after pituitary surgery and as a primary treatment in those unfit for surgery. Long-term endocrine follow-up is essential after SRS. Copyright © 2017 Endocrine Society

  16. Somatic USP8 Gene Mutations Are a Common Cause of Pediatric Cushing Disease.

    Science.gov (United States)

    Faucz, Fabio R; Tirosh, Amit; Tatsi, Christina; Berthon, Annabel; Hernández-Ramírez, Laura C; Settas, Nikolaos; Angelousi, Anna; Correa, Ricardo; Papadakis, Georgios Z; Chittiboina, Prashant; Quezado, Martha; Pankratz, Nathan; Lane, John; Dimopoulos, Aggeliki; Mills, James L; Lodish, Maya; Stratakis, Constantine A

    2017-08-01

    Somatic mutations in the ubiquitin-specific protease 8 (USP8) gene have been recently identified as the most common genetic alteration in patients with Cushing disease (CD). However, the frequency of these mutations in the pediatric population has not been extensively assessed. We investigated the status of the USP8 gene at the somatic level in a cohort of pediatric patients with corticotroph adenomas. The USP8 gene was fully sequenced in both germline and tumor DNA samples from 42 pediatric patients with CD. Clinical, biochemical, and imaging data were compared between patients with and without somatic USP8 mutations. Five different USP8 mutations (three missense, one frameshift, and one in-frame deletion) were identified in 13 patients (31%), all of them located in exon 14 at the previously described mutational hotspot, affecting the 14-3-3 binding motif of the protein. Patients with somatic mutations were older at disease presentation [mean 5.1 ± 2.1 standard deviation (SD) vs 13.1 ± 3.6 years, P = 0.03]. Levels of urinary free cortisol, midnight serum cortisol, and adrenocorticotropic hormone, as well as tumor size and frequency of invasion of the cavernous sinus, were not significantly different between the two groups. However, patients harboring somatic USP8 mutations had a higher likelihood of recurrence compared with patients without mutations (46.2% vs 10.3%, P = 0.009). Somatic USP8 gene mutations are a common cause of pediatric CD. Patients harboring a somatic mutation had a higher likelihood of tumor recurrence, highlighting the potential importance of this molecular defect for the disease prognosis and the development of targeted therapeutic options. Copyright © 2017 Endocrine Society

  17. Evaluation of depression, quality of life and body image in patients with Cushing's disease.

    Science.gov (United States)

    Alcalar, Nilufer; Ozkan, Sedat; Kadioglu, Pinar; Celik, Ozlem; Cagatay, Penbe; Kucukyuruk, Baris; Gazioglu, Nurperi

    2013-09-01

    The aim of this study was to evaluate patients with Cushing's disease (CD) who had undergone transsphenoidal surgery in terms of depression, quality of life (QoL), and perception of body image in comparison to healthy controls. Forty patients with CD and 40 healthy controls matched for demographic characteristics were included in the study. The subjects were evaluated with the Beck depression inventory (BDI), the health survey-short form (SF-36) and the multidimensional body-self relations questionnaire (MBSRQ). Subgroups of the patients with CD were formed on the basis of remission status and BDI scores. In this study, QoL in the general health category and body image were lower in the patients with CD than in the healthy subjects. However, no differences in depression scores were found between the two groups. When the CD group was evaluated according to remission rate, the mean BDI score was significantly higher in the CD patients without remission than in both the CD patients with remission and the healthy subjects (p = 0.04). However, the physical functioning, bodily pain and general health scores of the CD patients without remission on the SF-36 questionnaire were lower than in the CD patients in remission and the healthy subjects (p = 0.002, p = 0.04, p = 0.002, respectively). Fitness evaluation, health evaluation and body areas satisfaction scores of the MBSRQ were significantly different in the three groups (p = 0.003, p = 0.009 and p = 0.001, respectively). In this study, patients with CD were found to have lower QoL, lower body image perception and higher levels of depression compared to healthy controls, particularly if the disease is persistant despite surgery.

  18. Harvey Cushing's case series of trigeminal neuralgia at the Johns Hopkins Hospital: a surgeon's quest to advance the treatment of the 'suicide disease'.

    NARCIS (Netherlands)

    Adams, H.; Pendleton, C.; Latimer, K.; Cohen-Gadol, A.A.; Carson, B.S.; Quinones-Hinojosa, A.

    2011-01-01

    BACKGROUND: A review of Dr. Harvey Cushing's surgical cases at the Johns Hopkins Hospital provided insight into his early work on trigeminal neuralgia (TN). There was perhaps no other affliction that captured his attention in the way that TN did, and he built a remarkable legacy of successful

  19. The autopsy was conducted "Under most inauspicious circumstances:" John Turner, Harvey Cushing's case XXXII, and his unwitting contributions to the early understanding of acromegaly.

    Science.gov (United States)

    Pendleton, Courtney; Wand, Gary; Quinones-Hinojosa, Alfredo

    2010-12-01

    Harvey Cushing's monograph The Pituitary Body and Its Disorders describes Case XXXII, a 36-year-old man who presented with gigantism in 1910. The detailed post-mortem exam findings are prefaced with a cryptic statement, describing "inauspicious circumstances" surrounding the autopsy. Although contemporary biographies of Cushing have offered insight into these circumstances, the original surgical file for Case XXXII has not been previously reviewed. The original Johns Hopkins Hospital surgical records were reviewed, and the case of John Turner, who Cushing identified by name in his monograph The Pituitary Body and Its Disorders, was selected for further review. A review of the original surgical file revealed a typewritten note by Dr. Crowe, one of the surgeons who performed the post-mortem exam, with a handwritten addendum by Dr. Cushing. This document provides detail regarding the "inauspicious circumstances" surrounding the autopsy. Namely, the autopsy was conducted without permission of the family, during the funeral service, following a payment to the undertaker. The new information regarding the autopsy of John Turner offers insight into the previously incompletely described circumstances surrounding the autopsy. Additionally, the case illuminates the obligations and ethical quandaries that physician-scientists face.

  20. AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS AND AMERICAN COLLEGE OF ENDOCRINOLOGY DISEASE STATE CLINICAL REVIEW: DIAGNOSIS OF RECURRENCE IN CUSHING DISEASE.

    Science.gov (United States)

    Fleseriu, Maria; Hamrahian, Amir H; Hoffman, Andrew R; Kelly, Daniel F; Katznelson, Laurence

    2016-12-01

    Recurrence of hypercortisolemia after initial treatment of Cushing disease (CD) is more common than previously thought, with a third of patients suffering a recurrence over their lifetime. Awareness of this high rate and delayed timeline (sometimes decades) of potential recurrence is critical and patients with CD should be monitored at regular intervals throughout their lives. In this manuscript, we review the complex evaluation needed for defining CD remission versus persistent disease after surgery, and focus on challenges in diagnosing early recurrent hypercortisolemia. Late night salivary cortisol appears to be an earlier predictor of recurrence when compared with urinary free cortisol (UFC) excretion. We also review the criteria suggested to define recurrence of hypercortisolemia in patients treated with medical therapy. Further research is needed to determine the optimal way to evaluate a patient with CD recurrence as well as the riskbenefit ratio of treatment in early, mild recurrent disease. ACTH = adrenocorticotropic hormone AI = adrenal insufficiency CD = Cushing disease CDDT = coupled dexamethasone desmopressin test CR = circadian rhythm CRH = corticotropin-releasing hormone GC = glucocorticoid GCR = global clinical response HPA = hypothalamic-pituitary-adrenal LDDST = low-dose dexamethasone suppression test LNSC = late-night salivary cortisol ODST = overnight dexamethasone suppression test TSS = trans-sphenoidal surgery.

  1. High resolution pituitary gland MRI at 7.0 tesla: a clinical evaluation in Cushing's disease

    Energy Technology Data Exchange (ETDEWEB)

    Rotte, Alexandra A.J. de; Groenewegen, Amy; Rutgers, Dik R.; Witkamp, Theo; Luijten, Peter R.; Hendrikse, Jeroen [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Zelissen, Pierre M.J. [University Medical Center Utrecht, Department of Internal Medicine (Section of Endocrinology), Utrecht (Netherlands); Meijer, F.J.A. [Radboud University Medical Center, Department of Radiology and Nuclear Medicine, Nijmegen (Netherlands); Lindert, Erik J. van [Radboud University Medical Center, Department of Neurosurgery, Nijmegen (Netherlands); Hermus, Ad [Radboud University Medical Center, Department of Internal Medicine (Section of Endocrinology), Nijmegen (Netherlands)

    2016-01-15

    To evaluate the detection of pituitary lesions at 7.0 T compared to 1.5 T MRI in 16 patients with clinically and biochemically proven Cushing's disease. In seven patients, no lesion was detected on the initial 1.5 T MRI, and in nine patients it was uncertain whether there was a lesion. Firstly, two readers assessed both 1.5 T and 7.0 T MRI examinations unpaired in a random order for the presence of lesions. Consensus reading with a third neuroradiologist was used to define final lesions in all MRIs. Secondly, surgical outcome was evaluated. A comparison was made between the lesions visualized with MRI and the lesions found during surgery in 9/16 patients. The interobserver agreement for lesion detection was good at 1.5 T MRI (κ = 0.69) and 7.0 T MRI (κ = 0.62). In five patients, both the 1.5 T and 7.0 T MRI enabled visualization of a lesion on the correct side of the pituitary gland. In three patients, 7.0 T MRI detected a lesion on the correct side of the pituitary gland, while no lesion was visible at 1.5 T MRI. The interobserver agreement of image assessment for 7.0 T MRI in patients with Cushing's disease was good, and lesions were detected more accurately with 7.0 T MRI. (orig.)

  2. High resolution pituitary gland MRI at 7.0 tesla: a clinical evaluation in Cushing's disease

    International Nuclear Information System (INIS)

    Rotte, Alexandra A.J. de; Groenewegen, Amy; Rutgers, Dik R.; Witkamp, Theo; Luijten, Peter R.; Hendrikse, Jeroen; Zelissen, Pierre M.J.; Meijer, F.J.A.; Lindert, Erik J. van; Hermus, Ad

    2016-01-01

    To evaluate the detection of pituitary lesions at 7.0 T compared to 1.5 T MRI in 16 patients with clinically and biochemically proven Cushing's disease. In seven patients, no lesion was detected on the initial 1.5 T MRI, and in nine patients it was uncertain whether there was a lesion. Firstly, two readers assessed both 1.5 T and 7.0 T MRI examinations unpaired in a random order for the presence of lesions. Consensus reading with a third neuroradiologist was used to define final lesions in all MRIs. Secondly, surgical outcome was evaluated. A comparison was made between the lesions visualized with MRI and the lesions found during surgery in 9/16 patients. The interobserver agreement for lesion detection was good at 1.5 T MRI (κ = 0.69) and 7.0 T MRI (κ = 0.62). In five patients, both the 1.5 T and 7.0 T MRI enabled visualization of a lesion on the correct side of the pituitary gland. In three patients, 7.0 T MRI detected a lesion on the correct side of the pituitary gland, while no lesion was visible at 1.5 T MRI. The interobserver agreement of image assessment for 7.0 T MRI in patients with Cushing's disease was good, and lesions were detected more accurately with 7.0 T MRI. (orig.)

  3. Serotonin syndrome

    Science.gov (United States)

    Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

  4. [Study on the reproducibility of ACTH concentrations in plasma of horses with and without equine Cushing syndrome].

    Science.gov (United States)

    Gehlen, Heidrun; Bradaric, Zrinkja

    2013-01-01

    The evaluation of plasma ACTH and the dexamethasone suppression test are considered the methods of choice to evaluate the course of therapy of pituitary pars intermedia dysfunction (PPID). Sampling protocols as well as vacutainers for analysis differ between the laboratories. To evaluate the reproducability of plasma ACTH measurement between four different laboratories (A, B, C, D) in Germany as well as within the laboratories themselves, ten horses with previously diagnosed PPID and four healthy horses were sampled and analyzed. Each laboratory received two differently labeled samples of each horse which had been drawn at the same time (blinded samples). Sampling was performed in the morning at the same time. The sampling vacutainers (with and without addition of coagulation and proteinase inhibitors) and postage of the samples was performed according to laboratory standards. In one laboratory the influence of the time of centrifugation (immediately after taking blood versus after one hour) was determined. The samples were processed and analyzed according to laboratory protocols. Determination of ACTH levels was performed using chemiluminescence immunoassay. In total 132 blood samples were analyzed. The results of doubled blood samples of the same horse showed a standard deviation ranging from +/- 6 to +/- 27 pg/ml within the laboratories (Ø 19,29 pg/ml). The standard deviation of the repeatability of the variation coefficient was 13,48%. Blood samples of the same horse resulted in ACTH levels of 121 pg/ml in the first probe and in < 5 pg/ml in the second probe. Standard deviation of measured ACTH values between the laboratories was +/- 26,4 pg/ml (Ø 27,44 pg/ml). The standard deviation of the reproducibility of the variation coefficient was 18,36%. In a 20 year old gelding the lowest ACTH value was 60.9 pg/ml whereas the highest measured value was 108 pg/ml. Immediate centrifugation of blood samples resulted in significantly higher ACTH values at an average of 11.6 pg/ml. The additional use of proteinase inhibitors (aprotinine) showed no influence on ACTH levels in this study.

  5. Injecting epidural and intra-articular triamcinolone in HIV-positive patients on ritonavir: beware of iatrogenic Cushing's syndrome.

    Science.gov (United States)

    Maviki, M; Cowley, P; Marmery, H

    2013-02-01

    We report two HIV-positive patients on highly active antiretroviral therapy (HAART) who developed clinical features in keeping with secondary adrenal suppression following epidural and subacromial triamcinolone. Both patients were on ritonavir-boosted protease inhibitor containing HAART and both required maintenance hydrocortisone therapy following diagnosis. This highlights the need for radiologists and clinicians practicing these injections to be aware of this complication, to elicit an accurate drug history, and to take adequate measures to minimize these adverse effects.

  6. Beals Syndrome

    Science.gov (United States)

    ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

  7. Comparative assessment of adrenal function in Icenko-Cushing's disease according to the diagnostic pneumoretroperitoneum and morphological findings

    International Nuclear Information System (INIS)

    Tyurin, E.I.; Trofimov, V.M.

    1981-01-01

    A comparative analysis has been carried out, based on the adrenals X-ray trials, preceded by pneumoretroperitoneum, and surgical findings in 47 patients with Icenko-Cushing's disease and 20 to 50 years. Total bilateral adrenalectomy has been applied to surgical intervention. It has been established that the adrenals, either having normal morphology or showing signs of hyperplasia, have appeared unaltered in shape and size at tomograms. The X-ray picture peculiarities have been detected rather by the patients obesity rate, than the weight and histologic structure of the glands. Massive consolidation, found in most patients over the kidney at the plain X-ray film present no true enlargement of the adrenals, but should be considered as the reflection of the paranephral capsule, circumvening them [ru

  8. The recovery of the hypothalamo-pituitary-adrenal axis after transsphenoidal operation in three patients with Cushing's disease

    International Nuclear Information System (INIS)

    Lamberts, S.W.J.; Klijn, J.G.M.; Jong, F.H. de; Birkenhager, J.C.

    1981-01-01

    The recovery of the hypothalamo-pituitary-adrenal axis after selective transsphenoidal adenomectomy was studied in 3 patients with Cushing's disease by measuring basal plasma ACTH and cortisol concentrations, cortisol secretion rate, the diurnal rhythm of cortisol, and the reaction of cortisol to lysine vasopressin (LVP), of compound S to metyrapone and of cortisol and growth hormone to an insulin-induced hypoglycaemia. The third patient had been treated previously by external pituitary irradiation. In 2 patients basal plasma ACTH levels returned within normal values before plasma cortisol, but no supra-physiological plasma concentrations of ACTH were seen as has been observed after withdrawal of exogenous glucocorticoids. With regard to the different stimulation tests: at first the normal reaction of plasma cortisol to LVP returned after 3 months, at the same time as the restoration of growth hormone secretion in response to hypoglycaemia. A normalization of the reaction to metyrapone was seen thereafter while finally the reaction of cortisol to an insulin-induced hypoglycaemia and the diurnal rhythm of plasma cortisol returned 15 to 18 months after operation in the first patient and after 12 months in the second patient. Selective adenomectomy had also been carried out in the third patient, as evidenced by normal TSH, LH and FSH secretion. Hypocortisolism, and a deficient ACTH and growth hormone secretion in response to the stimuli mentioned, however, did not normalize up till 22 months after operation. The restoration of the hypothalamo-pituitary-adrenal axis after selective pituitary adenomectomy in Cushing's disease was prevented in this patient by prior external pituitary irradiation. (author)

  9. Raltegravir-induced Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome - implications for clinical practice and patient safety.

    Science.gov (United States)

    Perry, Melissa E O; Almaani, Noor; Desai, Nemesha; Larbalestier, Nick; Fox, Julie; Chilton, Daniella

    2013-08-01

    This case report describes two severe antiretroviral drug adverse reactions that occurred in the same patient. A 55-year-old HIV-positive African woman received a single epidural triamcinolone injection for pain relief of postherpetic neuralgia. Forty-one days later, she developed severe iatrogenic Cushing's syndrome due to the drug-drug interaction between triamcinolone and her boosted protease inhibitor therapy. The patient's antiretroviral regimen was thus changed to replace her protease inhibitor with the integrase inhibitor raltegravir. Shortly after commencing the drug, the patient developed a severe adverse drug reaction manifesting as Drug Reaction (or Rash) with Eosinophilia and Systemic Symptoms (DRESS) syndrome. First described in 1996, this hypersensitivity syndrome presents with severe skin reaction as well as fever, rash, lymphadenopathy and internal organ involvement with marked eosinophilia. Clinicians should be aware of raltegravir-induced DRESS syndrome as well as the potential for drug-drug interactions due to protease inhibitor-based therapy.

  10. Etiology and clinical profile of patients with Cushing′s syndrome: A single center experience

    Directory of Open Access Journals (Sweden)

    Ariacherry C Ammini

    2014-01-01

    Full Text Available Background: There is little published literature on the profile of patients with Cushing′s syndrome (CS from India. The aim of this study was to compile data of CS patients treated at this hospital. Materials and Methods: Patients referred to the endocrine services of this hospital for diagnosis/treatment of CS from January 1985 to July 2012 were the subjects for this study. All patients had detailed medical history, physical examination and biochemical and hormonal assays (which changed with availability of tests and changing views. Assays for plasma adrenocorticotropic hormone (ACTH (late 90s, salivary cortisol estimation, IJV sampling for ACTH and corticotrophin releasing hormone stimulation tests were added on later. Imaging included computed tomography (CT, magnetic resonance imaging (since the late 80′s and 68 Ga DOTA-TOC/FDG PET-CT (2008. Results: Three hundred sixty-four patients (250 females, 114 males, age 6 months to 65 years, mean 28 years + 12 years were diagnosed to have CS during this period. Two hundred and ninety-three patients (80.5% were ACTH dependent (CD 215, ectopic ACTH syndrome 22, occult ACTH source 56 while 71 (19.5% were ACTH independent (adrenal carcinoma 36, adenoma 30, primary pigmented nodular adrenal disease 4, AIMAH 1. Pituitary macro adenoma was seen in 14% of the CD cases. The most common presenting complaints were hypertension and diabetes mellitus. A total of 63% patients complained of weight gain while 15% had lost weight. Myopathy, infections, skeletal fractures and psychiatric problems were the other common observations in our patients. Conclusion: The clinical spectrum was broad. CD was the most common cause for CS.

  11. Successful treatment of Cushing's disease with o,p - DDD followed by pituitary irradiation in a 19-year-old male patient

    International Nuclear Information System (INIS)

    Dickerman, Z.; Kaufman, H.; Laron, Z.; Tel Aviv Univ.

    1979-01-01

    A 19-year-old male patient with Cushing's disease was treated for 15 months with a gastric-insoluble preparation of o.p'-DDD. 12 months after the start of the o.p'-DDD therapy, the dose was reduced from 6 to 2 g/day and external pituitary irradiation (4,480 rads) was initiated. No disturbance in the secretion of human growth hormone, thyroid-stimulating hormone, luteinizing hormone, follicle-stimulating hormone or prolactin was revealed. The clinical and laboratory signs of Cushing's disease disappeared gradually, and the patient tolerated the drug well, even at a dose of 12 g/day. At present, two years after the discontinuation of o.p'-DDD therapy and pituitary irradiation, the patient is symptom free and receives no medication. (B.G.)

  12. Internal jugular vein: Peripheral vein adrenocorticotropic hormone ratio in patients with adrenocorticotropic hormone-dependent Cushing′s syndrome: Ratio calculated from one adrenocorticotropic hormone sample each from right and left internal jugular vein during corticotrophin releasing hormone stimulation test

    Directory of Open Access Journals (Sweden)

    Sachin Chittawar

    2013-01-01

    Full Text Available Background: Demonstration of central: Peripheral adrenocorticotropic hormone (ACTH gradient is important for diagnosis of Cushing′s disease. Aim: The aim was to assess the utility of internal jugular vein (IJV: Peripheral vein ACTH ratio for diagnosis of Cushing′s disease. Materials and Methods: Patients with ACTH-dependent Cushing′s syndrome (CS patients were the subjects for this study. One blood sample each was collected from right and left IJV following intravenous hCRH at 3 and 5 min, respectively. A simultaneous peripheral vein sample was also collected with each IJV sample for calculation of IJV: Peripheral vein ACTH ratio. IJV sample collection was done under ultrasound guidance. ACTH was assayed using electrochemiluminescence immunoassay (ECLIA. Results: Thirty-two patients participated in this study. The IJV: Peripheral vein ACTH ratio ranged from 1.07 to 6.99 ( n = 32. It was more than 1.6 in 23 patients. Cushing′s disease could be confirmed in 20 of the 23 cases with IJV: Peripheral vein ratio more than 1.6. Four patients with Cushing′s disease and 2 patients with ectopic ACTH syndrome had IJV: Peripheral vein ACTH ratio less than 1.6. Six cases with unknown ACTH source were excluded for calculation of sensitivity and specificity of the test. Conclusion: IJV: Peripheral vein ACTH ratio calculated from a single sample from each IJV obtained after hCRH had 83% sensitivity and 100% specificity for diagnosis of CD.

  13. Fanconi syndrome

    Science.gov (United States)

    De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

  14. Duane Syndrome

    Science.gov (United States)

    ... Frequently Asked Questions Español Condiciones Chinese Conditions Duane Syndrome En Español Read in Chinese What is Duane Syndrome? Duane syndrome, also called Duane retraction syndrome (DRS), ...

  15. Hunter Syndrome

    Science.gov (United States)

    ... in girls. There's no cure for Hunter syndrome. Treatment of Hunter syndrome involves management of symptoms and complications. Symptoms Hunter syndrome is one type of a group of inherited metabolic disorders called mucopolysaccharidoses (MPSs), and Hunter syndrome is ...

  16. Hamartomatous polyposis syndromes

    DEFF Research Database (Denmark)

    Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus

    2014-01-01

    Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as ......Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes...

  17. What Are Some Types of Adrenal Gland Disorders?

    Science.gov (United States)

    ... Tumor of the pituitary gland (this is called Cushing's disease) Tumor of the adrenal gland (as explained above) ... the adrenal glands to produce too much cortisol. Cushing's disease refers to pituitary tumors that cause Cushing's syndrome . ...

  18. MR findings in iliotibial band syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Nishimura, G.; Yamato, M. [Department of Radiology, Dokkyo University School of Medicine, 880 Kitakobayashi, Mibu, Shimotsuga-gun, Tochigi-ken 321-02 (Japan); Tamai, K. [Department of Orthopedic Surgery, Dokkyo University School of Medicine, Tochigi-ken (Japan); Takahashi, J. [Department of Orthopedic Surgery, Shimizu Municipal Hospital, Shimizu (Japan); Uetani, M. [Department of Radiology, Nagasaki University School of Medicine, Nagasaki (Japan)

    1997-09-01

    Objective. To elucidate the MR findings in iliotibial band (ITB) syndrome. Design and patients. The subjects comprised four patients (five knees) with lateral knee pain: two athletes and two non-athletes. One non-athlete was engaged in work requiring repetitive knee movement, and the other suffered from Cushing syndrome and had bilateral abnormalities. All patients were suspected of having a lateral meniscal tear prior to MR examination, but physical examination following provisional MR diagnosis warranted the final diagnosis. MR studies included fast spin echo sagittal imaging, fat-saturated fast spin echo proton density coronal imaging, and T2* radial imaging. Twelve normal volunteers were examined. Results and conclusion. Fat-saturated coronal imaging demonstrated an ill-defined, high-intensity area deep to the ITB. T2* radial imaging showed an identical, but less conspicuous, abnormality. The MR finding suggested soft tissue inflammation and/or edema rather than focal fluid collection in the bursae. The signal alteration predominated in the region beneath the posterior fibers of the ITB, thus supporting the current opinion that the posterior fibers of the ITB are tighter against the lateral femoral epicondyle than the anterior fibers. The ITB itself did not show any signal alteration or increased thickness. (orig.). With 4 figs., 1 tab.

  19. Relationship of time--dose factors to tumor control and complications in the treatment of Cushing's disease by irradiation

    International Nuclear Information System (INIS)

    Aristizabal, S.; Caldwell, W.L.; Avila, J.; Mayer, E.G.

    1977-01-01

    The records of the Radiotherapy Division of the Radiology Department of Vanderbilt University Hospital were reviewed for the period 1952 to 1970. During those 19 years 45 patients with a well-documented diagnosis of Cushing's disease were treated initially by external irradiation of the pituitary. All of the patients were treated with megavoltage equipment using photons. When the results of irradiation are compared against total doses of radiation, it is evident that the control rate is unsatisfactory at doses less than 4000 rad and the maximum benefits of irradiation are evident in the 4500 to 5000 rad dose range. It is also clear that the complication rate increases as the dose exceeds 4800 rad. If the various treatment regimens of irradiation are converted to ''equivalent'' doses by the Nominal Standard Dose (NSD) or Time-Dose-Fractionation (TDF) methods, the relationship between ''dose'' and efficacy of therapy and complications is demonstrated. In order to reduce the possibility of treatment-related morbidity, the use of three or more small (4 x 4 cm) treatment portals or rotational techniques is recommended to a pituitary dose of 4600 to 5000 rad treating 5 days a week for 5 to 6 weeks

  20. Paintings from the Cushing/Whitney Library of Yale University: the Eleventh Kenneth Fitzpatrick Russell Memorial Lecture - 2012.

    Science.gov (United States)

    Low, Gordon

    2013-12-01

    In the early years of the introduction of Christianity into China, one American medical missionary distinguished himself by his kindness and generosity towards the Chinese people, and also by his surgical prowess. Peter Parker (1804-1888) was a graduate from the Yale University Medical School and was also an ordained minister. He came to the south China city of Canton (now called Guangzhou) in 1834, built a hospital, treated the patients without charge and also groomed medical apprentices to help him in his work. He so impressed a Chinese artist, Lam Qua (1801-1860) that Lam offered to paint in oil, without charge, some of the unusual patients of Parker. Parker stayed in China until 1857. Altogether, there are about 109 paintings. More than 80 of them are in the Harvey Cushing/John Hay Whitney Medical Historical Library of the Yale University Medical School, New Haven, and 23 of them are in Guy's Hospital, London. A description of some of the paintings, together with accounts of Parker and Lam are discussed. © 2013 Royal Australasian College of Surgeons.

  1. Valoración de Cortisol y ACTH postoperatorios en enfermedad de Cushing y su correlación con características clínicas a largo plazo

    OpenAIRE

    Abellán Galiana, Pablo

    2017-01-01

    La enfermedad de Cushing (EC) es un raro trastorno con una incidencia de 0,7-2,4 casos por millón de habitantes al año. La EC se caracteriza por un estado de hipercortisolismo, ocasionado por un adenoma hipofisario secretor de ACTH. Los adenomas hipofisarios productores de ACTH son la causa más frecuente de hipercortisolismo endógeno, suponiendo el 65-70% de los casos de síndrome de Cushing endógeno. La EC da lugar a obesidad, diabetes mellitus, hipertensión arterial, debilidad muscular, oste...

  2. Despistaje rutinario de Síndrome de Cushing en pacientes con obesidad mórbida: ¿es realmente necesario?

    OpenAIRE

    María Rosa Alhambra Expósito; Carmen Tenorio Jiménez; María José Molina Puerta; Gregorio Manzano García; Inmaculada Prior Sánchez; Concepción Muñoz Jiménez; María Ángeles Gálvez Moreno

    2014-01-01

    Introducción: El síndrome de Cushing (SC) es más frecuente en pacientes con síndrome metabòlico. Estudios previos han sugerido que es necesario realizar un despistaje de SC en pacientes obesos; sin embargo, estudios más recientes sólo recomiendan el despistaje en pacientes con hipertensión o diabetes mal controlados, a pesar de tratamiento. Objetivo: Evaluar la prevalencia de SC oculto en pacientes con obesidad mórbida. Pacientes y métodos: Estudio observacional retrospectivo en el que se inc...

  3. A comparison of the steady-state facility in the RELAP-UK code with the CUSH code and with collant flow in the Winfrith SGHWR

    International Nuclear Information System (INIS)

    Roberts, H.A.; Smith, C.P.

    1976-02-01

    Provision of capability for performing steady-state calculations in RELAP-UK has led to the possibility of the wider use of this code for steady-state assessments of the behaviour of commercial systems with complicated circuit arrangements. In the studies discussed in this report, the first objective is to demonstrate the performance of the RELAP-UK code in a steady-state role, and to make comparisons with the CUSH code, and with measurements obtained on the Winfrith Steam-Generating Heavy Water Reactor. (U.K.)

  4. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood ... fibrillin. A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

  5. WIEDEMANN SYNDROME

    African Journals Online (AJOL)

    hi-tech

    BILATERAL BENIGN HAEMORRHAGIC ADRENAL CYSTS IN BECKWITH - WIEDEMANN. SYNDROME: CASE REPORT. P. ANOOP and M. A. ANJAY. SUMMARY. Beckwith-Wiedemann syndrome is the most common overgrowth malformation syndrome. The classical features include macrosomia, macroglossia, ...

  6. Tourette syndrome

    Science.gov (United States)

    Gilles de la Tourette syndrome; Tic disorders - Tourette syndrome ... Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. The disorder is likely passed down through families. ...

  7. Williams syndrome

    Science.gov (United States)

    Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. It may be passed down in families. ... history of the condition. However, people with Williams syndrome have a 50% chance of passing the disorder ...

  8. Piriformis Syndrome

    Science.gov (United States)

    ... the National Library of Medicine’s MedlinePlus Piriformis Syndrome Metabolic Syndrome Show More Show Less Search Disorders SEARCH SEARCH Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Piriformis syndrome ...

  9. Preoperative Lateralization Modalities for Cushing Disease: Is Dynamic Magnetic Resonance Imaging or Cavernous Sinus Sampling More Predictive of Intraoperative Findings?

    Science.gov (United States)

    Sun, Hai; Yedinak, Chris; Ozpinar, Alp; Anderson, Jim; Dogan, Aclan; Delashaw, Johnny; Fleseriu, Maria

    2015-06-01

    Objective To analyze whether cavernous sinus sampling (CSS) and dynamic magnetic resonance imaging (dMRI) are consistent with intraoperative findings in Cushing disease (CD) patients. Design Retrospective outcomes study. Setting Oregon Health & Science University; 2006 and 2013. Participants A total of 37 CD patients with preoperative dMRI and CSS to confirm central adrenocorticotropic hormone (ACTH) hypersecretion. Patients were 78% female; mean age was 41 years (at diagnosis), and all had a minimum of 6 months of follow-up. Main Outcome Measures Correlations among patient characteristics, dMRI measurements, CSS results, and intraoperative findings. Results All CSS indicated presence of CD. Eight of 37 patients had no identifiable tumor on dMRI. Three of 37 patients had no tumor at surgery. dMRI tumor size was inversely correlated with age (rs = - 0.4; p = 0.01) and directly correlated to intraoperative lateralization (rs = 0.3; p < 0.05). Preoperative dMRI was directly correlated to intraoperative lateralization (rs = 0.5; p < 0.002). CSS lateralization showed no correlation with intraoperative findings (rs = 0.145; p = 0.40) or lateralization observed on preoperative dMRI (rs = 0.17; p = 0.29). Postoperative remission rate was 68%. Conclusion dMRI localization was most consistent with intraoperative findings; CSS results were less reliable. Results suggest that small ACTH-secreting tumors continue to pose a challenge to reliable preoperative localization.

  10. Pediatric Cushing disease: disparities in disease severity and outcomes in the Hispanic and African-American populations.

    Science.gov (United States)

    Gkourogianni, Alexandra; Sinaii, Ninet; Jackson, Sharon H; Karageorgiadis, Alexander S; Lyssikatos, Charalampos; Belyavskaya, Elena; Keil, Margaret F; Zilbermint, Mihail; Chittiboina, Prashant; Stratakis, Constantine A; Lodish, Maya B

    2017-08-01

    BackgroundLittle is known about the contribution of racial and socioeconomic disparities to severity and outcomes in children with Cushing disease (CD).MethodsA total of 129 children with CD, 45 Hispanic/Latino or African-American (HI/AA) and 84 non-Hispanic White (non-HW), were included in this study. A 10-point index for rating severity (CD severity) incorporated the degree of hypercortisolemia, glucose tolerance, hypertension, anthropomorphic measurements, disease duration, and tumor characteristics. Race, ethnicity, age, gender, local obesity prevalence, estimated median income, and access to care were assessed in regression analyses of CD severity.ResultsThe mean CD severity in the HI/AA group was worse than that in the non-HW group (4.9±2.0 vs. 4.1±1.9, P=0.023); driving factors included higher cortisol levels and larger tumor size. Multiple regression models confirmed that race (P=0.027) and older age (P=0.014) were the most important predictors of worse CD severity. When followed up a median of 2.3 years after surgery, the relative risk for persistent CD combined with recurrence was 2.8 times higher in the HI/AA group compared with that in the non-HW group (95% confidence interval: 1.2-6.5).ConclusionOur data show that the driving forces for the discrepancy in severity of CD are older age and race/ethnicity. Importantly, the risk for persistent and recurrent CD was higher in minority children.

  11. Primary Endoscopic Transnasal Transsphenoidal Surgery for Magnetic Resonance Image-Positive Cushing Disease: Outcomes of a Series over 14 Years.

    Science.gov (United States)

    Kuo, Chao-Hung; Yen, Yu-Shu; Wu, Jau-Ching; Chen, Yu-Chun; Huang, Wen-Cheng; Cheng, Henrich

    2015-09-01

    There are scant data of endoscopic transsphenoidal surgery (ETS) with adjuvant therapies of Cushing disease (CD). To report the remission rate, secondary management, and outcomes of a series of CD patients. Patients with CD with magnetic resonance imaging (MRI)-positive adenoma who underwent ETS as the first and primary treatment were included. The diagnostic criteria were a combination of 24-hour urine-free cortisol, elevated serum cortisol levels, or other tests (e.g., inferior petrosal sinus sampling). All clinical and laboratory evaluations and radiological examinations were reviewed. Forty consecutive CD patients, with an average age of 41.0 years, were analyzed with a mean follow-up of 40.2 ± 29.6 months. These included 22 patients with microadenoma and 18 with macroadenoma, including 9 cavernous invasions. The overall remission rate of CD after ETS was 72.5% throughout the entire follow-up. Patients with microadenoma or noninvasive macroadenoma had a higher remission rate than those who had macroadenoma with cavernous sinus invasion (81.8% or 77.8% vs. 44.4%, P = 0.02). After ETS, the patients who had adrenocorticotropic hormone-positive adenoma had a higher remission rate than those who had not (76.5% vs. 50%, P = 0.03). In the 11 patients who had persistent/recurrent CD after the first ETS, 1 underwent secondary ETS, 8 received gamma-knife radiosurgery (GKRS), and 2 underwent both. At the study end point, two (5%) of these CD patients had persistent CD and were under the medication of ketoconazole. For MRI-positive CD patients, primary (i.e., the first) ETS yielded an overall remission rate of 72.5%. Adjuvant therapies, including secondary ETS, GKRS, or both, yielded an ultimate remission rate of 95%. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. Anestesia general y analgesia para la resección de nódulo hepático en perro con enfermedad de Cushing y diabético: caso clínico

    OpenAIRE

    Costa, Margarida; Alexandre, Nuno; Pinho, Leonor; Martín, Maria; Gracia, Monica; Celdrán, Diego; Lima, Juan; Sánchez, Francisco

    2010-01-01

    O objectivo deste trabalho foi proporcionar uma anestesia inalatória e analgesia multimodal segura e eficaz num caso clínico de excisão cirurgica de um nódulo hepático num cão diabético, com insuficiência cardiaca e sindrome de cushing.

  13. An Atraumatic Symphysiolysis with a Unilateral Injured Sacroiliac Joint in a Patient with Cushing's Disease: A Loss of Pelvic Stability Related to Ligamentous Insufficiency?

    Science.gov (United States)

    Höch, Andreas; Pieroh, Philipp; Dehghani, Faramarz; Josten, Christoph; Böhme, Jörg

    2016-01-01

    Glucocorticoids are well known for altering bone structure and elevating fracture risk. Nevertheless, there are very few reports on pelvic ring fractures, compared to other bones, especially with a predominantly ligamentous insufficiency, resulting in a rotationally unstable pelvic girdle. We report a 39-year-old premenopausal woman suffering from an atraumatic symphysiolysis and disruption of the left sacroiliac joint. She presented with external rotational pelvic instability and immobilization. Prior to the injury, she received high-dose glucocorticoids for a tentative diagnosis of rheumatoid arthritis over two months. This diagnosis was not confirmed. Other causes leading to the unstable pelvic girdle were excluded by several laboratory and radiological examinations. Elevated basal cortisol and adrenocorticotropic hormone levels were measured and subsequent corticotropin-releasing hormone stimulation, dexamethasone suppression test, and petrosal sinus sampling verified the diagnosis of adrenocorticotropic hormone-dependent Cushing's disease. The combination of adrenocorticotropic hormone-dependent Cushing's disease and the additional application of exogenous glucocorticoids is the most probable cause of a rare atraumatic rotational pelvic instability in a premenopausal patient. To the authors' knowledge, this case presents the first description of a rotationally unstable pelvic ring fracture involving a predominantly ligamentous insufficiency in the context of combined exogenous and endogenous glucocorticoid elevation.

  14. A designated centre for people with disabilities operated by The Cheshire Foundation in Ireland, Cork

    LENUS (Irish Health Repository)

    Noctor, E

    2015-06-01

    Cushing\\'s disease is very rare in the paediatric population. Although uncommon, corticotroph hyperplasia causing Cushing\\'s syndrome has been described in the adult population, but appears to be extremely rare in children. Likewise, cyclical cortisol hypersecretion, while accounting for 15 % of adult cases of Cushing\\'s disease, has only rarely been described in the paediatric population. Here, we describe a very rare case of a 13-year old boy with cyclical cortisol hypersecretion secondary to corticotroph cell hyperplasia.

  15. Long-term outcomes of tissue-based ACTH-antibody assay-guided transsphenoidal resection of pituitary adenomas in Cushing disease.

    Science.gov (United States)

    Erfe, J Mark; Perry, Avital; McClaskey, John; Inzucchi, Silvio E; James, Whitney Sheen; Eid, Tore; Bronen, Richard A; Mahajan, Amit; Huttner, Anita; Santos, Florecita; Spencer, Dennis

    2017-10-13

    OBJECTIVE Cushing disease is caused by a pituitary micro- or macroadenoma that hypersecretes adrenocorticotropic hormone (ACTH), resulting in hypercortisolemia. For decades, transsphenoidal resection (TSR) has been an efficacious treatment but with certain limitations, namely precise tumor localization and complete excision. The authors evaluated the novel use of a double-antibody sandwich assay for the real-time quantitation of ACTH in resected pituitary specimens with the goals of augmenting pathological diagnosis and ultimately improving long-term patient outcome. METHODS This study involved a retrospective review of records and an analysis of assay values, pathology slides, and MRI studies of patients with Cushing disease who had undergone TSR in the period from 2009 to 2014 and had at least 1 year of follow-up in coordination with an endocrinologist. In the operating room, biopsy specimens from the patients had been analyzed for tissue ACTH concentration. Additional samples were simultaneously sent for frozen-section pathological analysis. The ACTH assay performance was compared against pathology assessments of surgical tumor samples using receiver operating characteristic (ROC) analysis and against pre- and postoperative MRI studies. RESULTS Fourteen patients underwent TSR with guidance by ACTH-antibody assay and pathological assessment of 127 biopsy samples and were followed up for an average of 3 years. The ACTH threshold for discriminating adenomatous from normal tissue was 290,000 pg/mg of tissue, based on jointly maximized sensitivity (95.0%) and specificity (71.3%). Lateralization discordance between preoperative MRI studies and surgical visualization was noted in 3 patients, confirming the impression that MRI alone may not achieve optimal localization. A majority of the patients (85.7%) attained long-term disease remission based on urinary free cortisol levels, plasma cortisol levels, and long-term corticosteroid therapy. Comparisons of patient

  16. Myelodysplastic Syndromes

    Science.gov (United States)

    ... blood cells, and the cells have a specific mutation in their DNA. Myelodysplastic syndrome with excess blasts — ... Chemicals linked to myelodysplastic syndromes include tobacco smoke, pesticides and industrial chemicals, such as benzene. Exposure to ...

  17. Moebius Syndrome

    Science.gov (United States)

    ... and supports a broad range of research on neurogenetic disorders, including Moebius syndrome. The goals of these ... and supports a broad range of research on neurogenetic disorders, including Moebius syndrome. The goals of these ...

  18. Pendred Syndrome

    Science.gov (United States)

    ... scan) to look for two characteristics of Pendred syndrome. One characteristic might be a cochlea with too few turns. ... Inner Ear Credit: NIH Medical Arts A second characteristic of Pendred syndrome is an enlarged vestibular aqueduct (see figure). The ...

  19. Rett Syndrome

    Science.gov (United States)

    Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and ...

  20. Ohtahara Syndrome

    Science.gov (United States)

    ... but be profoundly handicapped. As they grow, some children will progress into other epileptic disorders such as West syndrome and Lennox-Gestaut syndrome. What research is being done? The NINDS conducts and supports an extensive research program on seizures ...

  1. Gardner's syndrome

    International Nuclear Information System (INIS)

    Sobrado Junior, C.W.; Bresser, A.; Cerri, G.G.; Habr-Gama, A.; Pinotti, H.W.; Magalhaes, A.

    1988-01-01

    A case of familiar poliposis of colon related to a right mandibular osteoma is reported (this association is usually called Gardner's syndrome). Radiologic pictures ae shown and some commentaries about this syndrome concerning the treatment are made. (author) [pt

  2. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete X ... work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  3. Metabolic Syndrome

    Science.gov (United States)

    Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These conditions ... agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

  4. Felty syndrome

    Science.gov (United States)

    Seropositive rheumatoid arthritis (RA); Felty's syndrome ... The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with ...

  5. Usher Syndrome

    Science.gov (United States)

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

  6. Piriformis syndrome

    Science.gov (United States)

    Pseudosciatica; Wallet sciatica; Hip socket neuropathy; Pelvic outlet syndrome; Low back pain - piriformis ... Sciatica is the main symptom of piriformis syndrome. Other symptoms include: Tenderness or a dull ache in ...

  7. Dressler's Syndrome

    Science.gov (United States)

    ... syndrome Overview Dressler's syndrome is a type of pericarditis — inflammation of the sac surrounding the heart (pericardium). ... reducing its ability to pump blood efficiently. Constrictive pericarditis. Recurring or chronic inflammation can cause the pericardium ...

  8. Primary Small-Cell Carcinoma of the Palate with Cushing’s Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Yingqiu Song

    2012-01-01

    Full Text Available We report a 24-year-old woman presenting with a relapsed soy-bean-size tender mass at the junction of the soft and hard palate and a history of palatine tumor of small cell carcinoma. Reexcision surgery was performed and histopathological features were consistent. The patient was treated with six cycles of chemotherapy consisting of etoposide and cisplatin. After one year, the patient developed bone metastases and Cushing's syndrome, and successfully recovered with subsequent chemotherapy with irinotecan and cisplatin plus radiotherapy. There was no evidence of recurrence or metastasis for more than three years. Small cell carcinoma originating in the head and neck region has been reported to be highly aggressive and has a poor prognosis. This is the first case report of a patient with relapsed primary small cell carcinoma of the palate and successfully treated with second-line chemotherapy and local radiotherapy.

  9. McCune-Albright syndrome

    Directory of Open Access Journals (Sweden)

    Collins Michael T

    2008-05-01

    Full Text Available Abstract McCune-Albright syndrome (MAS is classically defined by the clinical triad of fibrous dysplasia of bone (FD, café-au-lait skin spots, and precocious puberty (PP. It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. FD can involve a single or multiple skeletal sites and presents with a limp and/or pain, and, occasionally, a pathologic fracture. Scoliosis is common and may be progressive. In addition to PP (vaginal bleeding or spotting and development of breast tissue in girls, testicular and penile enlargement and precocious sexual behavior in boys, other hyperfunctioning endocrinopathies may be involved including hyperthyroidism, growth hormone excess, Cushing syndrome, and renal phosphate wasting. Café-au-lait spots usually appear in the neonatal period, but it is most often PP or FD that brings the child to medical attention. Renal involvement is seen in approximately 50% of the patients with MAS. The disease results from somatic mutations of the GNAS gene, specifically mutations in the cAMP regulating protein, Gs alpha. The extent of the disease is determined by the proliferation, migration and survival of the cell in which the mutation spontaneously occurs during embryonic development. Diagnosis of MAS is usually established on clinical grounds. Plain radiographs are often sufficient to make the diagnosis of FD and biopsy of FD lesions can confirm the diagnosis. The evaluation of patients with MAS should be guided by knowledge of the spectrum of tissues that may be involved, with specific testing for each. Genetic testing is possible, but is not routinely available. Genetic counseling, however, should be offered. Differential diagnoses include neurofibromatosis, osteofibrous dysplasia, non-ossifying fibromas, idiopathic central precocious puberty, and ovarian neoplasm. Treatment is dictated by the tissues affected, and the extent to which they are affected. Generally, some form of surgical intervention

  10. International Rett Syndrome Foundation

    Science.gov (United States)

    ... Newsletters & Reports About Rett Syndrome What is Rett Syndrome? Rett Syndrome Diagnosis Boys with MECP2 Clinics FAQs Glossary ... Newsletters & Reports About Rett Syndrome What is Rett Syndrome? Rett Syndrome Diagnosis Boys with MECP2 Clinics FAQs Glossary ...

  11. [Capgras syndrome].

    Science.gov (United States)

    Alcoverro Fortuny, O; Sierra Acín, A C

    2001-01-01

    The authors report a case of Capgras' syndrome in a 16-years-old child, who had been hospitalized for psychotic disorder. A review of the literature is performed. Most authors state that Capgras' syndrome would represent a symptom of underlying medical o functional disorders, although the term syndrome is used. The main etiopathogenic hypothesis of this syndrome are put forward (psychodynamic, disconnection, neuropsychological and medical).

  12. Velocardiofacial Syndrome

    Science.gov (United States)

    Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

    2009-01-01

    Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

  13. PREDICTORS OF BIOCHEMICAL REMISSION AND RECURRENCE AFTER SURGICAL AND RADIATION TREATMENTS OF CUSHING DISEASE: A SYSTEMATIC REVIEW AND META-ANALYSIS.

    Science.gov (United States)

    Abu Dabrh, Abd Moain Abu; Singh Ospina, Naykky M; Al Nofal, Alaa; Farah, Wigdan H; Barrionuevo, Patricia; Sarigianni, Maria; Mohabbat, Arya B; Benkhadra, Khalid; Carranza Leon, Barbara G; Gionfriddo, Michael R; Wang, Zhen; Mohammed, Khaled; Ahmed, Ahmed T; Elraiyah, Tarig A; Haydour, Qusay; Alahdab, Fares; Prokop, Larry J; Murad, Mohammad Hassan

    2016-04-01

    We conducted a systematic review and meta-analysis to synthesize the evidence about predictors that may affect biochemical remission and recurrence after transsphenoidal surgery (TSS), radiosurgery (RS), and radiotherapy (RT) in Cushing disease. We searched multiple databases through December 2014 including original controlled and uncontrolled studies that enrolled patients with Cushing disease who received TSS (first-line), RS, or RT. We extracted data independently, in duplicates. Outcomes of interest were biochemical remission and recurrence. A meta-analysis was conducted using the random-effects model to estimate event rates with 95% confidence intervals (CIs). First-line TSS was associated with high remission (76% [95% CI, 72 to 79%]) and low recurrence rates (10% [95% CI, 6 to 16%]). Remission after TSS was higher in patients with microadenomas or positive-adrenocorticotropic hormone tumor histology. RT was associated with a high remission rate (RS, 68% [95% CI, 61 to 77%]; RT, 66% [95% CI, 58 to 75%]) but also with a high recurrence rate (RS, 32% [95% CI, 16 to 60%]; RT, 26% [95% CI, 14 to 48%]). Remission after RS was higher at short-term follow-up (≤2 years) and with high-dose radiation, while recurrence was higher in women and with lower-dose radiation. Remission was after RT in adults who received TSS prior to RT, and with lower radiation doses. There was heterogeneity (nonstandardization) in the criteria and cutoff points used to define biochemical remission and recurrence. First-line TSS is associated with high remission and low recurrence, while RS and RT are associated with reasonable remission rates but important recurrence rates. The current evidence warrants low confidence due to the noncomparative nature of the studies, high heterogeneity, and imprecision.

  14. [Pitfalls in the diagnosis of hypercortisolemia].

    Science.gov (United States)

    Maciejewski, Adam; Stellmach, Angelika; Łącka, Katarzyna

    2015-10-01

    Cushing's syndrome (CS) is relatively rare disorder affecting 2-5 per million per year, although the issue of establishing the diagnosis of CS and differential diagnosis of the disease are a significant clinical problem. CS is usually the result of excessive exogenous glucocorticoids usage (iatrogenic CS), endogenous CS can be divided into ACTH-dependent and ACTH-independent. Regardless of its etiology, the most important steps in establishing the diagnosis of CS are taking careful history and examination. The symptoms with high discriminatory value are myopathy, reddish purple striae, easy bruising and plethora. Knowledge of the pathomechanisms leading to the development of CS symptoms, facilitates establishing the diagnosis and understanding the importance of early diagnosis. Although the sensitivity and specificity of laboratory test have increased and imaging techniques developed, establishing the diagnosis of CS is still a challenging problem in clinical practice. When choosing appropriate diagnostic test we should remember of both advantages and limitations of each of them. The increasing popularity of late night/midnight salivary cortisol measurement as a first line diagnostic test is observed, also urinary free cortisol measurement, 1mg dexamethasone overnight suppression test and midnight serum cortisol measurement are used in the initial testing for hypercortisolemia. Subclinical CS as well as cyclical or episodic CS may be challenging especially. Another diagnostic problem is differentiation between functional hypercortisolism (pseudo-Cushing's syndrome) and pathological hypercortisolism with organic changes (CS). Right and early diagnosis is of vital importance in patients with CS because of large extent of complications resulting from untreated hypercortisolemia. In the course of CS many different organs and systems can be affected, leading to increase in total morbidity and mortality. © 2015 MEDPRESS.

  15. Cortisol, obesity, and the metabolic syndrome: a cross-sectional study of obese subjects and review of the literature.

    Science.gov (United States)

    Abraham, S B; Rubino, D; Sinaii, N; Ramsey, S; Nieman, L K

    2013-01-01

    Circulating cortisol and psychosocial stress may contribute to the pathogenesis of obesity and metabolic syndrome (MS). To evaluate these relationships, a cross-sectional study of 369 overweight and obese subjects and 60 healthy volunteers was performed and reviewed the previous literature. Overweight and obese subjects had at least two other features of Cushing's syndrome. They underwent measurements representing cortisol dynamics (24 h urine cortisol excretion (UFC), bedtime salivary cortisol, 1 mg dexamethasone suppression test) and metabolic parameters (BMI, blood pressure (BP); fasting serum triglycerides, HDL, insulin, and glucose). Subjects also completed the Perceived Stress Scale (PSS). UFC, salivary cortisol, and weight from 60 healthy volunteers were analyzed. No subject had Cushing's syndrome. UFC and dexamethasone responses were not associated with BMI or weight. However, salivary cortisol showed a trend to increase as BMI increased (P cortisol levels were weak to moderately correlated with fasting insulin (rs = -0.31, P = 0.01) and HOMA-IR (rs = -0.31, P = 0.01) in men and systolic (rs = 0.18, P = 0.02) and diastolic BP (rs = 0.20, P = 0.009) in women. PSS results were higher in obese subjects than controls, but were not associated with cortisol or metabolic parameters. As expected, WC correlated with fasting insulin, HOMA-IR, and systolic BP (adjusted for BMI and gender; P cortisol and metabolic parameters. Taken together, these data do not support a strong relationship between systemic cortisol or stress and obesity or MS. Copyright © 2013 The Obesity Society.

  16. Surrénalectomie par abord coelioscopique: Expérience du Centre ...

    African Journals Online (AJOL)

    The adrenal gland diseases encountered were Conn adenoma in 4, Cushing\\'s syndrome in 3 (with one case of Cushing\\'s disease), pheochromocytoma in 3 and incidentaloma in 2 cases. The mean tumor size was 5.5 cm (range 1.8 to 9 cm). The operative time ranged from 70 to 135 minutes for unilateral adrenalectomy ...

  17. Revesz syndrome

    Directory of Open Access Journals (Sweden)

    Dayane Cristine Issaho

    2015-04-01

    Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

  18. Gorlin syndrome

    Directory of Open Access Journals (Sweden)

    Basanti Devi

    2013-01-01

    Full Text Available Gorlin Syndrome, a rare genodermatosis, otherwise known as Nevoid basal cell carcinoma syndrome (NBCCS is a multisystem disease affecting skin, nervous system, eyes, endocrine glands, and bones. It is characterized by multiple basal cell carcinomas, palmoplantar pits, jaw cysts, and bony deformities like kyphoscoliosis and frontal bossing. We would like to report a case of Gorlin syndrome with classical features, as this is a rare genodermatosis.

  19. Urofacial syndrome

    Directory of Open Access Journals (Sweden)

    Kamal F Akl

    2012-01-01

    Full Text Available The urofacial syndrome is characterized by functional obstructive uropathy asso-ciated with an inverted smile. The importance of the subject is that it sheds light, not only on the muscles of facial expression, but also on the inheritance of voiding disorders and lower urinary tract malformations. We report a 10-year-old-male patient who had the urofacial syndrome. Early diagnosis of the urofacial syndrome is important to avoid upper urinary tract damage and renal failure.

  20. Joubert Syndrome

    Science.gov (United States)

    ... syndrome is inherited in an autosomal recessive manner (meaning both parents must have a copy of ... physical, occupational, and speech therapy may benefit some children. Infants with abnormal ...

  1. Reye's Syndrome

    Science.gov (United States)

    ... that contain aspirin. Some hospitals and medical facilities conduct newborn screenings for fatty acid oxidation disorders to determine which children are at greater risk of developing Reye's syndrome. ...

  2. Angelman Syndrome

    Science.gov (United States)

    ... See More About Research The NINDS supports and conducts research on neurogenetic disorders such as Angelman syndrome, to develop techniques to diagnose, treat, ... Publications Definition Angelman ...

  3. Diurnal ACTH and plasma cortisol variations in healthy dogs and in those with pituitary-dependent Cushing's syndrome before and after treatment with retinoic acid.

    Science.gov (United States)

    Castillo, V A; Cabrera Blatter, M F; Gómez, N V; Sinatra, V; Gallelli, M F; Ghersevich, M C

    2009-04-01

    Daytime variations in ACTH and plasma cortisol were studied in healthy dogs and in dogs with pituitary-dependent hypercortisolism (PDH), before and after treatment with retinoic acid. In control dogs ACTH showed a higher concentration at 8.00 AM and between 2.00 and 6.00 PM, with the lowest concentration registered at 10.00 AM (pCortisol did not show significant differences. In dogs with PDH, ACTH was lower at 8.00 AM (ACTH: pcortisol concentration was registered at 8.00 AM and 8.00 PM and the highest at 4.00 PM (p<0.05 vs. 8.00 AM and p<0.01 vs. 8.00 PM). After treatment, the lowest ACTH concentration was registered at 10.00 AM (p<0.01 vs. 2.00 and 4.00 PM). To conclude, the adrenal is desensitized in PDH possibly showing negative in diagnostic tests.

  4. Russell-Silver syndrome

    Science.gov (United States)

    Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome ... One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11. Most of the time, it ...

  5. Peptic ulcer disease in endogenous hypercortisolism: myth or reality?

    Science.gov (United States)

    Hatipoglu, Esra; Caglar, Asli Sezgin; Caglar, Erkan; Ugurlu, Serdal; Tuncer, Murat; Kadioglu, Pinar

    2015-11-01

    Many clinicians believe hypercortisolism is ulcerogenic. However, data from clinical studies show that prophylaxis for peptic ulcer disease is no longer recommended in patients receiving corticosteroid treatment. This has not yet been verified in endogenous hypercortisolism by controlled clinical studies. The purpose of the current study was to evaluate the relationship between endogenous Cushing's syndrome (CS) and peptic ulcer disease and Helicobacter pylori infection. The study group contained 20 cases with CS resulting from ACTH-dependent endogenous hypercortisolism. The control groups consisted of 14 age- and gender-matched cases receiving exogenous corticosteroid therapy and 100 cases of dyspepsia with non-cushingoid features. Upper gastrointestinal endoscopy was performed on all cases. Biopsies were taken from five different points: two samples from the antrum, two samples from the corpus, and one sample from the fundus. A histological diagnosis of Helicobacter pylori infection was also obtained from evaluation of biopsy specimens. The frequency of stomach and duodenal ulcers did not vary between the groups (p = 0.5 and p = 0.7). Antral gastritis was less frequent and pangastritis was more common in cases with CS compared to the healthy controls (p = 0.001 and p peptic ulcer or Helicobacter pylori infection. Prophylactic use of proton pump inhibitors is not compulsory for hypercortisolism of any type.

  6. [Ballantyne syndrome or mirror syndrome].

    Science.gov (United States)

    Torres-Gómez, Luis Guillermo; Silva-González, María Eugenia; González-Hernández, Rigoberto

    2010-11-01

    Ballantyne syndrome or mirror syndrome is a triad consisting of the presence of fetal hydrops, generalized edema placentomegaly mother. May be related to any cause of fetal hydrops. The fetal prognosis is poor in untreated cases, the mother has reference to be the cause or the termination of pregnancy. Present the case of a 26-year-old who developed mirror syndrome secondary to non-immune fetal hydrops of unknown origin, accompanied by preeclampsia.

  7. Hyperthyroidism

    Science.gov (United States)

    ... Navigation Endocrine Diseases Acromegaly Adrenal Insufficiency & Addison's Disease Cushing's Syndrome Graves' Disease Hashimoto's Disease Hyperthyroidism (Overactive Thyroid) Hypothyroidism (Underactive Thyroid) ...

  8. Graves' Disease

    Science.gov (United States)

    ... Navigation Endocrine Diseases Acromegaly Adrenal Insufficiency & Addison's Disease Cushing's Syndrome Graves' Disease Hashimoto's Disease Hyperthyroidism (Overactive Thyroid) Hypothyroidism (Underactive Thyroid) ...

  9. Antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Cervera, Ricard; Piette, Jean-Charles; Font, Josep

    2002-01-01

    To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

  10. Ascher syndrome

    Directory of Open Access Journals (Sweden)

    Zhifang Zhai

    2015-03-01

    Full Text Available Ascher syndrome is a rare, benign skin disorder characterized by a double upper lip, blepharochalasis, and nontoxic enlargement of the thyroid gland. The exact cause is unknown, but it is considered to be a hereditary disease with an autosomal dominant trait. We report here a case of forme fruste Ascher syndrome in a 29-year-old man.

  11. Passwell syndrome

    Directory of Open Access Journals (Sweden)

    Muhammed K

    2003-03-01

    Full Text Available There is an expanding list of syndromes that combine ichthyosis with neuroectodermal and mesodermal defects. We report a syndrome of congenital ichthyosis with atrophy, mental retardation, dwarfism, aminoaciduria, primary amenorrhoea and underdeveloped secondary sexual characters in a 38-year-old woman of non consanguinous parentage.

  12. Proteus syndrome

    Directory of Open Access Journals (Sweden)

    George Renu

    1993-01-01

    Full Text Available A case of proteus syndrome in a 20 year old male is repoted. Hemihypertrophy, asymmetric megalodactyly, linear epidermal naevus, naevus flammeus, angiokeratoma, lymphangioma circumscriptum, thickening of the palms and soles, scoliosis and varicose veins were present. There are only few reports of these cases in adults. The syndrome has not been reported from India.

  13. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine; Bødtger, Uffe; Heltberg, Ole

    2014-01-01

    Lemierre's syndrome is an often un-diagnosed disease seen in previously healthy young subjects, presenting with symptoms of pharyngitis, fever and elevated markers of inflammation. The syndrome is characterised by infectious thrombosis of the jugular vein due to infection with Fusobacteria, causing...

  14. Tourette Syndrome

    Science.gov (United States)

    If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics are throat- ... spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. It ...

  15. Fahr's Syndrome

    Science.gov (United States)

    ... or 50s, although it can occur at any time in childhood or adolescence. × Definition Fahr's Syndrome is a rare, genetically dominant, inherited ... or 50s, although it can occur at any time in childhood or adolescence. View Full Definition Treatment There is no cure for Fahr's Syndrome, ...

  16. TAFRO Syndrome.

    Science.gov (United States)

    Igawa, Takuro; Sato, Yasuharu

    2018-02-01

    TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Clinical and radiological manifestations of paraneoplastic syndrome of bronchogenic carcinoma

    Directory of Open Access Journals (Sweden)

    Goldner Branislav

    2005-01-01

    Full Text Available The objective of this study was to present some clinical and radiological manifestations of PNS in relation to bronchogenic carcinoma (BC and to evaluate the usefulness of imaging findings in the diagnosis of asymptomatic BC. In the study group of 204 patients (146 male and 58 female with proven bronchogenic carcinoma, PNS was present in 18 (8.62% patients. The patients with PNS were divided into two groups. The first one consisted of 13 (72.2% patients with symptoms related to primary tumours while the second one consisted of 5 (27.7% patients with symptoms, at initial appearance, indicative of disorders of other organs and systems. The predominant disorder was Lambert-Eaton Syndrome, associated with small-cell carcinoma. Endocrine manifestations included: inappropriate antidiuretic hormone production syndrome (small-cell carcinoma, a gonadotropin effect with gynaecomastia and testicular atrophy (planocellular carcinoma, small-cell carcinoma, a case of Cushing Syndrome (small-cell carcinoma, and hyper-calcaemia, due to the production of the parathyroid hormone-related peptide, which was associated with planocellular carcinoma. A rare case of bilateral exophthalmos was found as PNS at adenocarcinoma. Digital clubbing and hypertrophic osteoarthropathy (HO were associated with planocellular and adenocarcinoma, while clubbing was much more common than HO, especially among women. The differences between the two groups were related to the time of PNS appearance. In the first group, PNS occurred late on in the illness, while in the second group, PNS preceded the diagnosis of BC. Alternatively, the disappearance of a clinical or a radiological manifestation of PNS after surgery or chemotherapy may be an indicator of an improvement in health or PNS may be the first sign of illness recurrence. Radiological manifestations of PNS in asymptomatic patients may serve as a useful screen for identifying primary BC. In symptomatic patients, it may be an

  18. CHARGE syndrome

    Directory of Open Access Journals (Sweden)

    Prasad Chitra

    2006-09-01

    Full Text Available Abstract CHARGE syndrome was initially defined as a non-random association of anomalies (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness. In 1998, an expert group defined the major (the classical 4C's: Choanal atresia, Coloboma, Characteristic ears and Cranial nerve anomalies and minor criteria of CHARGE syndrome. Individuals with all four major characteristics or three major and three minor characteristics are highly likely to have CHARGE syndrome. However, there have been individuals genetically identified with CHARGE syndrome without the classical choanal atresia and coloboma. The reported incidence of CHARGE syndrome ranges from 0.1–1.2/10,000 and depends on professional recognition. Coloboma mainly affects the retina. Major and minor congenital heart defects (the commonest cyanotic heart defect is tetralogy of Fallot occur in 75–80% of patients. Choanal atresia may be membranous or bony; bilateral or unilateral. Mental retardation is variable with intelligence quotients (IQ ranging from normal to profound retardation. Under-development of the external genitalia is a common finding in males but it is less apparent in females. Ear abnormalities include a classical finding of unusually shaped ears and hearing loss (conductive and/or nerve deafness that ranges from mild to severe deafness. Multiple cranial nerve dysfunctions are common. A behavioral phenotype for CHARGE syndrome is emerging. Mutations in the CHD7 gene (member of the chromodomain helicase DNA protein family are detected in over 75% of patients with CHARGE syndrome. Children with CHARGE syndrome require intensive medical management as well as numerous surgical interventions. They also need multidisciplinary follow up. Some of the hidden issues of CHARGE syndrome are often forgotten, one being the feeding adaptation of these children, which needs an early aggressive approach from a feeding team. As the child

  19. A retrospective study comparing the outcome of horses undergoing small intestinal resection and anastomosis with a single layer (Lembert) or double layer (simple continuous and Cushing) technique.

    Science.gov (United States)

    Close, Kristyn; Epstein, Kira L; Sherlock, Ceri E

    2014-05-01

    To (1) compare postoperative complications and survival in horses after small intestinal resection and anastomosis using 2 anastomosis techniques (single layer Lembert; double layer simple continuous oversewn with Cushing), and (2) to compare outcome by anastomosis type (jejunoileostomy; jejunojejunostomy). Retrospective case series. Horses (n = 53). Medical records (July 2006-July 2010) of all horses that had small intestinal resection and anastomosis. Horses were divided into groups based on technique and type of anastomosis. Comparisons of pre- and intraoperative findings (disease severity), postoperative complications, and survival rates were made between groups. There were no differences in disease severity, postoperative complications, or survival between single layer (n = 23) or double layer (n = 31) anastomoses. There were no differences in disease severity or survival between jejunoileostomy (n = 16) or jejunojejunostomy (n = 38). There was a higher incidence of postoperative colic in hospital after jejunoileostomy (13/16) compared with jejunojejunostomy (18/38) (P = .0127). Postoperative complications and survival are comparable between horses undergoing single layer and double layer small intestinal end-to-end anastomoses. With the exception of increased postoperative colic in the hospital, postoperative complications and survival after jejunoileostomy and jejunojejunostomy are also comparable. © Copyright 2014 by The American College of Veterinary Surgeons.

  20. The Value of Perioperative Levels of ACTH, DHEA, and DHEA-S and Tumor Size in Predicting Recurrence of Cushing Disease.

    Science.gov (United States)

    El Asmar, Nadine; Rajpal, Aman; Selman, Warren R; Arafah, Baha M

    2018-02-01

    Despite the development of hypocortisolemia after corticotroph surgical adenomectomy, 15% to 20% patients have recurrence of Cushing disease (CD). In this study, we investigated the effect of tumor size and the value of perioperative assessment of corticotropin (ACTH) and adrenal steroid levels in predicting recurrence. Perioperatively, no glucocorticoids were administered until the serum cortisol was ≤3 μg/dL. Blood samples were obtained before and repeatedly after adenomectomy in 79 patients with CD. Of these, 66 had a nadir serum cortisol of ≤3.0 μg/dL and clinical and biochemical remissions. During a median follow-up of 131 months, 11 of 66 had disease recurrence (REC), whereas 55 of 66 did not (NO-REC). Preoperative hormone levels in the REC and NO-REC groups were similar. After adenomectomy, a brief and similar increase in ACTH, cortisol, and dehydroepiandrosterone (DHEA) levels was observed in both groups followed by gradual decline in those levels. Although REC and NO-REC patients had similar cortisol levels (3.4 ± 1.7 μg/dL vs 2.9 ± 2.2 μg/dL) at the 36th postoperative hour, their respective ACTH (33 ± 7.1 ng/L vs 12.1 ± 5.4 ng/L; P 20 in all REC patients and disease recurrence, particularly in those with profound hypocortisolemia. Copyright © 2017 Endocrine Society