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Sample records for acromegaly cushing disease

  1. Prolactinomas, Cushing's disease and acromegaly: debating the role of medical therapy for secretory pituitary adenomas

    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

  2. Genetics Home Reference: Cushing disease

    Skip to main content Your Guide to Understanding Genetic Conditions Enable Javascript for addthis links to activate. ... Conditions Genes Chromosomes & mtDNA Resources Help Me Understand Genetics Home Health Conditions Cushing disease Cushing disease Enable ...

  3. Acromegaly

    Chanson Philippe

    2008-06-01

    Full Text Available Abstract Acromegaly is an acquired disorder related to excessive production of growth hormone (GH and characterized by progressive somatic disfigurement (mainly involving the face and extremities and systemic manifestations. The prevalence is estimated at 1:140,000–250,000. It is most often diagnosed in middle-aged adults (average age 40 years, men and women equally affected. Due to insidious onset and slow progression, acromegaly is often diagnosed four to more than ten years after its onset. The main clinical features are broadened extremities (hands and feet, widened thickened and stubby fingers, and thickened soft tissue. The facial aspect is characteristic and includes a widened and thickened nose, prominent cheekbones, forehead bulges, thick lips and marked facial lines. The forehead and overlying skin is thickened, sometimes leading to frontal bossing. There is a tendency towards mandibular overgrowth with prognathism, maxillary widening, tooth separation and jaw malocclusion. The disease also has rheumatologic, cardiovascular, respiratory and metabolic consequences which determine its prognosis. In the majority of cases, acromegaly is related to a pituitary adenoma, either purely GH-secreting (60% or mixed. In very rare cases, acromegaly is due to ectopic secretion of growth-hormone-releasing hormone (GHRH responsible for pituitary hyperplasia. The clinical diagnosis is confirmed biochemically by an increased serum GH concentration following an oral glucose tolerance test (OGTT and by detection of increased levels of insulin-like growth factor-I (IGF-I. Assessment of tumor volume and extension is based on imaging studies. Echocardiography and sleep apnea testing are used to determine the clinical impact of acromegaly. Treatment is aimed at correcting (or preventing tumor compression by excising the disease-causing lesion, and at reducing GH and IGF-I levels to normal values. Transsphenoidal surgery is often the first-line treatment

  4. Medical treatment of Cushing's Disease.

    Cuevas-Ramos, Daniel; Fleseriu, Maria

    2016-09-01

    Cushing's Syndrome (CS) is a serious endocrine disease that results from the adverse clinical consequences of chronic exposure to high levels of glucocorticoids. Most patients with endogenous CS have an adrenocorticotropin (ACTH)-secreting pituitary corticotroph adenoma, i.e. Cushing's Disease (CD). The first-line therapy for CD is transsphenoidal pituitary surgery. If tumor removal is incomplete or unsuccessful, persistent hypercortisolism will require further treatment. Repeat surgery, medical therapy, radiation and bilateral adrenalectomy are all second line therapy options; however, medical therapy can be also used as first line therapy in patients who cannot undergo surgery, or to decrease cortisol values and/or improve co-morbidities. Medications used in the treatment of CD, classified into three groups: pituitary directed drugs, adrenal steroidogenesis inhibitors and glucocorticoid receptor blockers, are reviewed. Future 'on the horizon' treatment options are also discussed. PMID:26977887

  5. Unmasking sarcoidosis following surgery for Cushing disease

    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 pr...... successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease....

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

    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.

  7. Current status of adrenalectomy for Cushing's disease

    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

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

    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.)

  9. Acromegaly

    ... Research Training & Career Development Grant programs for students, postdocs, and faculty Research at NIDDK Labs, faculty, and ... diabetes, digestive and liver diseases, kidney diseases, weight control and nutrition, urologic diseases, endocrine and metabolic diseases, ...

  10. [Psychological profile with Cushing disease (author's transl)].

    Bastin, C

    Fifty-six patients with clinically and biologically unequivocal Cushing disease underwent psychological evaluation including at least one interview, a graphological test, a tree test, and a Szondi test. Patients were found to be hyperadapted, with a rigid repressive system, concealing major anxiety. These patients are closely dependent on their familial and professional affective relationships. This probably establishes the intensity of their repressive system. They need approval, appreciation, and recognition. Obsessional features are found (scrupulous conscience, compulsive activity) as well as manic-depressive features (fluctuations between depression and euphoric activity acting as a defense against depression). During the interviews it was established that onset of Cushing disease occurred a few months after prolonged stress; this stress dealt electively with the patients affective relationships. PMID:6274029

  11. Unmasking sarcoidosis following surgery for Cushing disease.

    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. PMID:27375832

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

    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)

  13. Adrenal myelolipoma associated with Cushing's disease.

    Bennett, B D; McKenna, T J; Hough, A J; Dean, R; Page, D L

    1980-03-01

    An 18-year-old man with a history of Cushing's disease was treated with a total right and a near total left adrenalectomy in 1956. Pathologic examination of the operative specimen revealed bilateral adrenal hyperplasia. After 13 years, recurrence of symptoms of cortisol excess necessitated cobalt irradiation to the pituitary, which was without clinical effect. After an initial response to the adrenolytic agent, o,p'-DDD, partial relapse occurred. At this time, the recognition of an abdominal mass prompted abdominal exploration revealing a huge adrenal myelolipoma containing adrenal cortical cells distributed diffusely throughout the tumor. Symptoms of adrenal insufficiency developed, and adrenal steroid secretion did not respond to exogenous adrenocorticotropic hormone postoperatively. The case illustrates that adrenal myelolipomas may become very large with continued stimulation by adrenocorticotropic hormone, may contain significant amounts of adrenal cortical tissue, and may be associated with clinical hypercortisolism. PMID:7361728

  14. MANAGEMENT OF ENDOCRINE DISEASE: Acromegaly and cancer: an old debate revisited.

    Boguszewski, Cesar Luiz; Ayuk, John

    2016-10-01

    Based on experimental and animal models, epidemiological data from non-acromegaly populations, and longitudinal and cross-sectional cohorts of patients with acromegaly, a potential association between acromegaly and cancer has long been hypothesized, in particular colorectal cancer, and, to a lesser extent, breast, thyroid and prostate cancers. The exact mechanisms underlying this potential association have not been fully elucidated. Results from studies examining cancer incidence and mortality in acromegaly have been inconsistent, with some demonstrating increased risk, whereas others show no increase. This article reviews the existing data relating to cancer risk and mortality in acromegaly, exploring the limitations of study designs and the impact of changes in disease control and patient outcomes over time. PMID:27089890

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

    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)

  16. Cushing's disease; inferior petrosal sinus venography and samplings

    Hypersecretion of ACTH in patients with Cushing's syndrome originates from either a pituitary tumor (Cushing's disease) or an ectopic ACTH-secreting tumor. These 2 entities may be clinically indistinguishable, and additional difficulty arise because pituitary microadenomas may be radiologically occult. Recently, bilateral selective venous sampling from the inferior petrosal sinuses became the procedure of choice for confirming a false negative study of a combined hormonal test and pituitary ACTH hypersecretion. We performed selective venous catheterization and sampling for ACTH. The central location of the lesion was detected in 1 case (intersinus gradient = 1.1 : 1), and the remaining 5 cases revealed lateralization of the lesions (intersinus gradient = 3.7 - 20.1 : 1), which correlated well with transsphenoidal microadenomectomies in all the cases. We concluded that selective venous ACTH sampling from the inferior petroal sinus is a reliable and useful aid in the diagnosis of Cushing's disease when standard clinical and biochemical studies are inconclusive

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

    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: 3.201, year: 2014

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

    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.

  19. Medical treatment of Cushing's disease: Overview and recent findings

    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

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

    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

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

    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

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

    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.

  3. Treatment of Cushing disease: overview and recent findings

    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

  4. Outcome of Cushing's disease following transsphenoidal surgery in a single center over 20 years.

    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.

  5. Medical therapy in acromegaly.

    Sherlock, Mark

    2011-05-01

    Acromegaly is a rare disease characterized by excess secretion of growth hormone (GH) and increased circulating insulin-like growth factor 1 (IGF-1) concentrations. The disease is associated with increased morbidity and premature mortality, but these effects can be reduced if GH levels are decreased to <2.5 μg\\/l and IGF-1 levels are normalized. Therapy for acromegaly is targeted at decreasing GH and IGF-1 levels, ameliorating patients\\' symptoms and decreasing any local compressive effects of the pituitary adenoma. The therapeutic options for acromegaly include surgery, radiotherapy and medical therapies, such as dopamine agonists, somatostatin receptor ligands and the GH receptor antagonist pegvisomant. Medical therapy is currently most widely used as secondary treatment for persistent or recurrent acromegaly following noncurative surgery, although it is increasingly used as primary therapy. This Review provides an overview of current and future pharmacological therapies for patients with acromegaly.

  6. A 12-month phase 3 study of pasireotide in Cushing's disease

    Colao, Annamaria; Petersenn, Stephan; Newell-Price, John; Findling, James W; Gu, Feng; Maldonado, Mario; Schoenherr, Ulrike; Mills, David; Salgado, Luiz Roberto; Biller, Beverly M K; Jørgensen, Jens Otto Lunde

    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. Familial Cushing's disease with severe weight loss occurring in late childhood.

    Cameron, F J; Warne, G L

    1997-02-01

    We describe a rare case of familial Cushing's disease occurring in a 7-year-old boy, and 19 years of follow up. Our patient first presented soon after his maternal aunt had been treated for Cushing's disease. The clinical presentation was made complicated by the development of an intercurrent eating disorder resembling anorexia nervosa. This resulted in marked weight loss, and even though serum and urinary cortisol levels were elevated, many of the clinical stigmata of Cushing's disease were absent. Eating disorders are relatively uncommon in boys, and in this case there was an organic cause for the abnormal behaviour. This case shows, furthermore, that even the obesity of Cushing's disease can be overcome by the combination of diet and exercise. PMID:9069050

  8. Gêmeas idênticas discordantes para a doença de Cushing: relato de caso Identical twins discordant for Cushing's disease: case report

    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

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

    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. PMID:27104179

  10. Neuropsychiatric disorders and cognitive dysfunction in patients with Cushing's disease

    CHEN Yu-fan; LI Yun-feng; CHEN Xiao; SUN Qing-fang

    2013-01-01

    Objective To review the main neuropsychiatric disorders and cognitive deficits in patients with Cushing's disease (CD) and the associated pathophysiological mechanisms underlying CD.These mechanistic details may provide recommendations for preventing or treating the cognitive impairments and mood disorders in patients with CD.Data sources Data were obtained from papers on psychiatric and cognitive complications in CD published in English within the last 20 years.To perform the PubMed literature search,the following keywords were input:cushing's disease,cognitive,hippocampal,or glucocorticoids.Study selection Studies were selected if they contained data relevant to the topic addressed in the particular section.Because of the limited length of this article,we have frequently referenced recent reviews that contain a comprehensive amalgamation of literature rather than the actual source papers.Results Patients with active CD not only suffer from many characteristic clinical features,but also show some neuropsychiatric disorders and cognitive impairments.Among the psychiatric manifestations,the common ones are emotional instability,depressive disorder,anxious symptoms,impulsivity,and cognitive impairment.Irreversible effects of previous glucocorticoid (GC) excess on the central nervous system,such as hippocampal and the basal ganglia,is the most reasonable reason.Excess secretion of cortisol brings much structural and functional changes in hippocampal,such as changes in neurogenesis and morphology,signaling pathway,gene expression,and glutamate accumulation.Hippocampal volume loss can be found in most patients with CD,and decreased glucose utilization caused by GCs may lead to brain atrophy,neurogenesis impairment,inhibition of long-term potentiation,and decreased neurotrophic factors; these may also explain the mechanisms of GC-induced brain atrophy and hippocampal changes.Conclusions Brain atrophy and hippocampal changes caused by excess secretion of cortisol are

  11. Lethal acute demyelinization with encephalo-myelitis as a complication of cured Cushing's disease.

    Chevalier, N; Hieronimus, S; Vandenbos, F; Delmont, E; Cua, E; Cherick, F; Paquis, P; Michiels, J-F; Fenichel, P; Brucker-Davis, F

    2010-12-01

    Cushing's disease is usually associated with higher mortality rate, especially from cardiovascular causes. Development or exacerbation of autoimmune or inflammatory diseases is known to occur in patients with hypercortisolism after cure. We report for the first time a 34-year old woman with a psychiatric background, who developed four months after the surgical cure of Cushing's disease an acute disseminated encephalomyelitis (ADEM) presenting initially as a psychiatric illness. We hypothesize that the recent correction of hypercortisolism triggered ADEM and that the atypical presentation, responsible for diagnosis delay, led to the death of this patient. PMID:20850107

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

    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.)

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

    Fatemeh Esfahanian; Rozana Kazemi

    2010-01-01

    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 ...

  14. The burden of Cushing's disease: Clinical and health-related quality of life aspects

    R.A. Feelders (Richard); S.J. Pulgar (S.); A. Kempel (A.); A.M. Pereira (Alberto)

    2012-01-01

    textabstractObjective: Cushing's disease (CD) is a rare endocrine disorder characterized by excess secretion of ACTH due to a pituitary adenoma. Current treatment options are limited and may pose additional risks. A literature review was conducted to assess the holistic burden of CD. Design: Studies

  15. Progestogens and Cushing's syndrome.

    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.

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

    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. Patología cardiovascular en la acromegalia Cardiovascular Disease in Acromegaly

    L Fiszlejder

    2012-09-01

    acromegaly, this disease is associated with a two to three-fold increase in cardiovascular risk in relation to the normal adult population. This results from a long term exposure of cardiomyocytes to GH excess, which causes histological changes in the geometric configuration of myofibrils, interstitial fibrosis and biventricular hypertrophy. The natural history of acromegalic heart disease includes several stages. In the early phase, there is a slow development of myocardial hipertrophy, subsequently associated with heart rhythm abnormalities. These arrhythmias, which represent a major risk factor for cardiovascular events, are secondary to the above mentioned structural changes in the myocardium, and make up the so-called "hyperkinetic syndrome of acromegaly". According to various epidemiological studies, a variable rate of patients with acromegaly (25 % to 50 % has hypertension. This complication is secondary to sodium retention and the consequent plasma volume expansion, which implies cardiac overload and constitutes a worsening factor for cardiovascular disease. In the second stage, there are echocardiographic signs of reduced ventricular diastolic filling. The third stage is characterized by alteration of one or more heart valves and impaired systolic and diastolic function at rest, as well as signs of dilated cardiomyopathy, leading to congestive heart failure. This last stage is irreversible even with adequate therapy. Thus, early diagnosis of disease and a close monitoring of serum CH-IGF-I levels are mandatory. No financial conflicts of interest exist.

  18. Cushing's disease to a giant pituitary adenoma in early infancy: CT and MRI features

    We report the case of a 12-month-old girl presenting with diabetes insipidus and Cushing's disease. Brain magnetic resonance imaging (MRI) demonstrated a large tumour arising from the sella turcica, extending up to the foramen of Monro and invading the cavernous sinuses. Surgery was performed to remove the suprasellar part of the tumour, and histology revealed an adrenocorticotrophin (ACTH) secreting pituitary adenoma. This entity is very rare in this age group and the MRI features have not previously been described. (orig.)

  19. Gamma Knife Radiosurgery for Acromegaly

    John D. Rolston

    2012-01-01

    Full Text Available Acromegaly is debilitating disease occasionally refractory to surgical and medical treatment. Stereotactic radiosurgery, and in particular Gamma Knife surgery (GKS, has proven to be an effective noninvasive adjunct to traditional treatments, leading to disease remission in a substantial proportion of patients. Such remission holds the promise of eliminating the need for expensive medications, along with side effects, as well as sparing patients the damaging sequelae of uncontrolled acromegaly. Numerous studies of radiosurgical treatments for acromegaly have been carried out. These illustrate an overall remission rate over 40%. Morbidity from radiosurgery is infrequent but can include cranial nerve palsies and hypopituitarism. Overall, stereotactic radiosurgery is a promising therapy for patients with acromegaly and deserves further study to refine its role in the treatment of affected patients.

  20. Cushing's disease; inferior petrosal sinus venography and samplings

    Chung, Tae Sub; Suh, Jung Ho; Kim, Dong Ik; Lee, Hyun Chul; Huh, Kap Bum; Kim, Young Soo; Chung, Sang Sup [College of Medicine, Yonsei University, Seoul (Korea, Republic of)

    1991-07-15

    Hypersecretion of ACTH in patients with Cushing's syndrome originates from either a pituitary tumor (Cushing's disease) or an ectopic ACTH-secreting tumor. These 2 entities may be clinically indistinguishable, and additional difficulty arise because pituitary microadenomas may be radiologically occult. Recently, bilateral selective venous sampling from the inferior petrosal sinuses became the procedure of choice for confirming a false negative study of a combined hormonal test and pituitary ACTH hypersecretion. We performed selective venous catheterization and sampling for ACTH. The central location of the lesion was detected in 1 case (intersinus gradient = 1.1 : 1), and the remaining 5 cases revealed lateralization of the lesions (intersinus gradient = 3.7 - 20.1 : 1), which correlated well with transsphenoidal microadenomectomies in all the cases. We concluded that selective venous ACTH sampling from the inferior petroal sinus is a reliable and useful aid in the diagnosis of Cushing's disease when standard clinical and biochemical studies are inconclusive.

  1. Dilated Cardiomyopathy Revealing Cushing Disease: A Case Report and Literature Review.

    Marchand, Lucien; Segrestin, Bérénice; Lapoirie, Marion; Favrel, Véronique; Dementhon, Julie; Jouanneau, Emmanuel; Raverot, Gérald

    2015-11-01

    Cardiovascular impairments are frequent in Cushing's syndrome and the hypercortisolism can result in cardiac structural and functional changes that lead in rare cases to dilated cardiomyopathy (DCM). Such cardiac impairment may be reversible in response to a eucortisolaemic state.A 43-year-old man with a medical past of hypertension and history of smoking presented to the emergency department with global heart failure. Coronary angiography showed a significant stenosis of a marginal branch and cardiac MRI revealed a nonischemic DCM. The left ventricular ejection fraction (LVEF) was estimated as 28% to 30%. Clinicobiological features and pituitary imaging pointed toward Cushing's disease and administration of adrenolytic drugs (metyrapone and ketoconazole) was initiated. Despite the normalization of cortisol which had been achieved 2 months later, the patient presented an acute heart failure. A massive mitral regurgitation secondary to posterior papillary muscle rupture was diagnosed as a complication of the occlusion of the marginal branch. After 6 months of optimal pharmacological treatment for systolic heart failure, as well as treatment with inhibitors of steroidogenesis, there was no improvement of LVEF. The percutaneous mitral valve was therefore repaired and a defibrillator implanted. The severity of heart failure contraindicated pituitary surgery and the patient was instead treated by stereotaxic radiotherapy.This is the first case reporting a Cushing's syndrome DCM without improvement of LVEF despite normalization of serum cortisol levels. PMID:26579807

  2. Cushing's Syndrome

    ... Cushing's syndrome, also called hypercortisolism , is a rare endocrine disorder caused by chronic exposure of the body's tissues ... removing the tumor while minimizing the chance of endocrine deficiency or long-term ... for Cushing's Syndrome Clinical Trials ...

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

    Komiya, Ichiro; Ohara, Noriko; Nagasawa, Yoshitaka; Asawa, Takayuki; Hashizume, Kiyoshi (Shinshu Univ., Matsumoto, Nagano (Japan). Faculty of Medicine); Takasu, Nobuyuki; Yamada, Takashi

    1994-04-01

    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).

  4. Acceleration of pubertal development following pituitary radiotherapy for Cushing's disease

    Nicholl, R.M.; Kirk, J.M.W.; Grossman, A.B.; Plowman, P.N.; Besser, G.M.; Savage, M.O. (Saint Bartholomew' s Hospital, London (United Kingdom))

    1993-01-01

    A 7-year-old boy with pituitary dependent Cushing's disease was treated with pituitary irradiation following unsuccessful microadenomectomy. This led to normalization of the hypercortisolaemia, but was followed by GH deficiency. Two years after radiotherapy he had the onset of pubertal development with testicular enlargement to 8 ml bilaterally. Pubertal regression was induced using the long-acting GnRH analogue goserelin. Acceleration of skeletal maturation was also arrested, resulting in improvement of final height prediction. Irradiation directly to the hypothalamo-pituitary region, as well as whole brain irradiation, may thus be associated with accelerated pubertal development. (author).

  5. Monitoring Patient Improvement Parameters following Pasireotide Treatment in Cushing's Disease

    Maria Fleseriu; Chris Yedinak; Jessica Brzana

    2013-01-01

    Cushing’s disease (CD) is a disorder in which chronic excess adrenocorticotropic hormone production is associated with multiple comorbidities and diminished quality of life. Postsurgical monitoring is important, and newer therapies are available for the management of surgical failure or disease recurrence. In this clinical case, we illustrate the importance of the nursing role in long-term management of CD, particularly as nurses may be the first point of contact for patients with CD. Alertne...

  6. A retrospective study of secondary diabetes prevalence in Pheochromocytoma, Cushing and Acromegal patients

    Bastan Hagh M

    1997-07-01

    Full Text Available Some of the endocrinologic diseases, especially Acromegaly, Cushing and Pheochromocytoma have multiple effects on blood glucose metabolism and regulation in non-diabetic patients. In this retrospective survey, records of patients of Tehran Medical Sciences University hospitals have been reviewd. Of 124 Acromegals, GTT was performed for 51 patients, being impaired in 18%. To evaluate diabetes, FBS and BS of 90 patients were checked, overt diabetes was detected in 27%. Among 90 Cushing patients, blood glucose was checked in 60 cases, 47% of these patients had levels above the normal range, and 39% had glucosuria. Among 80 Pheochromocytoma patients, 16 cases (26.5% had overt diabetes. In comparison with other studied, we have obtained a little different results concerning diabetes and impaired GTT prevalence

  7. Clinical features of GH deficiency and effects of 3 years of GH replacement in adults with controlled Cushing's disease

    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. Cushing Disease After Treatment of Nonfunctional Pituitary Adenoma: A Case Report and Literature Review.

    Fang, Hongjuan; Tian, Rui; Wu, Huanwen; Xu, Jian; Fan, Hong; Zhou, Jian; Zhong, Liyong

    2015-12-01

    We describe a very rare case of nonfunctional pituitary adenoma (NFPA) that exhibited corticotrophic activity after resection and radiotherapy. The possible mechanisms of the transformation from NFPA to Cushing disease (CD) are discussed.A 43-year-old man presented with impaired vision, bilateral frontal headaches, and hyposexuality. He had no symptoms suggestive of hypercortisolism, and 8 am plasma cortisol concentration was 67.88 ng/mL. Brain imaging revealed a 15 × 15 × 21-mm sellar mass suggestive of a macroadenoma. The tumor was resected by transsphenoidal surgery and identified by immunohistochemical analysis as a chromophobic adenoma that did not stain for pituitary hormones. The patient was treated with prednisone and levothyroxine replacement therapy. After a third recurrence, the patient presented with clinical features and physical signs of Cushing syndrome. Plasma adrenocorticotropic hormone (ACTH) and cortisol concentrations were elevated, and there was a loss of circadian rhythms. Inferior petrosal sinus sampling after desmopressin showed the central-peripheral ACTH ratio was greater than 3:1. A repeat transsphenoidal resection was undertaken. Immunohistochemistry revealed ACTH positivity. Three months following surgery, imaging showed little residual tumor, but plasma ACTH remained elevated. He was referred for postoperative Gamma Knife radiotherapy.The immunological activity and biological features of the hormones secreted from a pituitary adenoma vary with time. Because long-term outcomes are unpredictable, postoperative follow-up is essential to detect postoperative transformation from NFPA to CD. PMID:26705201

  9. [Mechanism of the development of neuromuscular disorders in Itsenko-Cushing disease].

    Agafonov, B V; Lahutina, T S; Deianova, A F

    1982-01-01

    The results of electromyographic studies indicate that the affection of the neuromuscular system is seen in all the patients with Icenko-Cushing's disease. Changes in different indices of the electromyogram (EMG), i.e. denervation activity, a character of the summary EMG alteration, mean duration of the motor unit action potentials (MUAP), the number of polyphase action potentials, their amplitude and the MUAP synchronization in different spheres indicate a neurogenic character of this affection. It was shown that both denervation and reinnervation processes are more pronounced in the foot muscles than in the hand ones. The denervation process is seemed to be primarily developed in the foot muscles, followed by the hand musculature injury. Therefore, the changes in several EMG parameters may be seen during several stages of the disease in some muscles and not to be observed in the others. PMID:7156070

  10. Cushing disease

    ... the base of the brain. A type of pituitary tumor called an adenoma is the most common cause. ... Treatment involves surgery to remove the pituitary tumor, if ... again and return to normal. During the recovery process from ...

  11. Concomitant Cushing's Disease and Marked Hyperprolactinemia: Response to a Dopamine Receptor Agonist.

    Shiraishi, Jun; Koyama, Hidenori; Shirakawa, Manabu; Ishikura, Reiichi; Okazaki, Hirokazu; Kurajoh, Masafumi; Shoji, Takuhito; Moriwaki, Yuji; Yamamoto, Tetsuya; Namba, Mitsuyoshi

    2016-01-01

    A 38-year-old woman was admitted to our hospital because of amenorrhea, multiple bone fractures, and a Cushingoid appearance. Endocrinological investigations revealed that she had co-existing Cushing's disease and prolactinoma, with a serum level of prolactin (PRL) at 1,480 ng/mL, corticotropin (ACTH) at 81.3 pg/mL, and cortisol at 16.6 μg/dL. Due to the lack of indication for transsphenoidal surgery, cabergoline monotherapy was initiated. A 6-month course of treatment resulted in only subtle amelioration of hypercortisolism, while hyperprolactinemia was dramatically improved. In 5 cases of bihormonal (ACTH/PRL) pituitary macroadenoma reported in the English literature, 2 were initially treated with dopaminergic agonists with substantial effectiveness for both PRL and ACTH. We herein report an extremely rare case of bihormonal macroadenoma in which only PRL was responsive to treatment. PMID:27086808

  12. Von Hippel Lindau disease with metastatic pancreatic neuroendocrine tumor causing ectopic Cushing's syndrome.

    Hatipoglu, Esra; Kepicoglu, Hasan; Rusen, Elif; Kabasakal, Levent; Gundogdu, Sadi; Kadioglu, Pinar

    2013-01-01

    We present a 39-year-old woman who was previously diagnosed with Von Hippel Lindau Disease (VHLD). She had surgery and radiotherapy for cranial hemangioblastoma (HA) 11 years ago and had unilateral adrenalectomy for pheochromocytoma in another hospital 6 month prior to her admission to our center. Moon face, buffalo hump, central obesity, progressive weight gain and menstrual irregularities persisted after adrenalectomy. Her laboratory results were consistent with ectopic Cushing's syndrome (ECS). A pancreatic solid mass with a nodule on the left lung were revealed upon computed tomography. In addition, Gallium-68 Somatostatin Receptor PET confirmed the pancreatic involvement and demonstrated additional lesions on the left lung and in the aortocaval lymphatic system on the right side, suggesting metastatic pancreatic neuroendocrine tumor (PNET). Peptide receptor radionuclide therapy (PRRT) with [177Lutetium-DOTA0,Tyr3] octreotate was performed on the patient, with no side effects observed. She was discharged from the hospital 10 days after the first cycle. PMID:23524618

  13. Long-term follow-up of low-dose external pituitary irradiation for Cushing's disease

    Twenty-four patients (three male) with Cushing's disease, aged between 11 and 67 years, were treated with low-dose external pituitary irradiation (20 Gy in eight fractions over 10-12 days) and followed for between 13 and 171 months (median 93 months). Eleven patients (46%) went into remission 4-36 months after irradiation, but five subsequently relapsed. In this series, the low incidence of radiation-induced hypopituitarism and absence of other complications attributable to radiotherapy suggest that low-dose pituitary irradiation may be a useful treatment option in selected patients. However, long-term follow-up has demonstrated a high relapse rate and failure to prevent Nelson's syndrome in adrenalectomized patients, indicating that it should not be used as primary treatment in preference to selective adenomectomy. (author)

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

    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. PMID:26906124

  15. Megavoltage pituitary irradiation in the management of Cushing's disease and Nelson's syndrome: long-term follow-up

    The authors report the long-term follow-up (up to 17.3 years) of the clinical and biochemical effects of megavoltage pituitary irradiation (radiotherapy;RT), administered as primary or secondary therapy, for pituitary Cushing's disease and Nelson's syndrome in 52 patients. Irradiation was administered, from a 4-15 MeV linear accelerator, via a three-field technique, to a total dose of 4500 cGy (rad) in 25 fractions over 35 days. (author)

  16. Modulatory effect of BclI GR gene polymorphisms on the obesity phenotype in Brazilian patients with Cushing's disease

    Ricardo P.P. Moreira

    2013-05-01

    Full Text Available OBJECTIVES: Patients with Cushing's disease exhibit wide phenotypic variability in the severity of obesity, diabetes and hypertension. In the general population, several glucocorticoid receptor genes (NR3C1 and HSD11B1 polymorphisms are associated with altered glucocorticoid sensitivity and/or metabolism, resulting in an increased or reduced risk of an adverse metabolic profile. Our aim was to analyze the association of NR3C1 and HSD11B1 gene variants with the severity of some clinical and hormonal features of Cushing's disease. METHODS: Sixty-four patients presenting with Cushing's disease were diagnosed based on adrenocorticotrophic hormone levels, high-dose dexamethasone suppression tests and/or inferior petrosal sinus sampling and magnetic resonance imaging. The A3669G, ER22/23EK, N363S BclI-NR3C1 and HSD11B1-rs12086634 variants were screened. RESULTS: The BclI, HSD11B1-rs12086634 and A3669G variants were found in 36%, 19.5% and 14% of alleles, respectively. The N363S and ER22/23EK polymorphisms were identified in heterozygosis once in only two patients (1.5% of alleles. There were no differences in the weight gain or prevalence of diabetes and hypertension in the patients carrying the abovementioned alleles compared to the wild-type carriers. Interestingly, the mean body mass index (BMI of the BclI carriers was significantly higher than the non-carriers (34.4±7 kg/m2 vs. 29.6±4.7 kg/m2, respectively. None of the polymorphisms were associated with the basal adrenocorticotrophic hormone, FU levels or F level after dexamethasone suppression testing. CONCLUSION: Although Cushing's disease results from increased glucocorticoid secretion, we observed that interindividual variability in the peripheral glucocorticoid sensitivity, mediated by the glucocorticoid receptor, could modulate the obesity phenotype.

  17. 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

    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.

  18. Evaluation of circulating levels and renal clearance of natural amino acids in patients with Cushing's disease.

    Faggiano, A; Pivonello, R; Melis, D; Alfieri, R; Filippella, M; Spagnuolo, G; Salvatore, F; Lombardi, G; Colao, A

    2002-02-01

    Although the hypercortisolism-induced impairment of protein homeostasis is object of several studies, a detailed evaluation of the complete amino acid profile of patients with Cushing's syndrome (CS) has never been performed. The aim of the current open transversal controlled study was to evaluate serum and urinary concentrations as well as renal clearance of the complete series of natural amino acids and their relationship with glucose tolerance in patients with Cushing's disease (CD). Twenty patients with CD (10 active and 10 cured) and 20 sex- and age-matched healthy controls entered the study. Measurement of serum and urinary levels of the complete series of natural amino acids was performed in all patients analyzed by cationic exchange high performance liquid cromatography (HPLC) after 2 weeks of a standardized protein intake regimen. The renal clearance (renal excretion rate) of each amino acid was calculated on the basis of the serum and urinary concentrations of creatinine and the specific amino acid. Fasting glucose and insulin levels, glucose and insulin response to standard glucose load, insulinogenic and homeostasis model insulin resistance (Homa-R) indexes were also evaluated and correlated to the circulating levels and renal clearances of each amino acid. Significantly higher serum (p<0.01) and urinary (p<0.05) levels of alanine and cystine, lower serum and higher urinary levels of leucine, isoleucine and valine (p<0.05) and higher renal excretion rates of leucine, isoleucine and valine (p<0.01) were found in patients with active CD than in patients cured from the disease and in controls. No difference was found between cured patients and controls. Creatinine clearance was similar in active and cured patients and in controls. In patients with active CD, urinary cortisol levels were significantly correlated to urinary cystine levels (r=0.85; p<0.01) and renal excretion rate of leucine (r=-0.76; p<0.05), isoleucine (r=-0.76; p<0.05) and valine (r=-0

  19. Surgical management of acromegaly: Long term functional outcome analysis and assessment of recurrent/residual disease

    Banerji, Deepu; Das, Nitu K.; Sharma, Siddhiraj; Jindal, Yogesh; Jain, Vijendra K.; Behari, Sanjay

    2016-01-01

    Context: Functional growth hormone producing adenomas have long-term deleterious effects on the visual apparatus, the cardiovascular and musculoskeletal systems, and often predispose to malignancies. Since persistence of acromegaly affects outcome and quality of life, therapeutic interventions become mandatory. Aim: This study represents an analysis of long-term clinical and endocrinal outcome of 115 patients of acromegaly after surgical management. Setting and Design: Tertiary care retrospective study. Materials and Methods: One hundred and fifteen patients (male:female ratio: 1:1.09) with acromegalic features were studied. Apart from acromegalic features, their main clinical presentation also included headache, diminution of vision, field defects, ptosis, irregular menstruation, diabetes insipidus, diabetes mellitus and hypertension. Six of them presented with apoplexy. Their preoperative endocrinal evaluation included basal and suppressed growth hormone (GH), prolactin and thyroid levels. On the basis of axial and coronal CT scan or multiplanar MR imaging or both, the tumors were classified according to their suprasellar and parasellar extension (Hardy's grade). Transnasal trans-sphenoidal surgery (TSS) (n = 37) and sublabial, rhinoseptal TSS (n = 72) were the preferred approaches. Six patients with significant parasellar extensions underwent trans-cranial explorations. The patients were followed up at 6 and 12 weeks and then at 6 monthly intervals. Hormonal and CT/MR evaluation were also done. Attainment of random GH value less than 2.5 µg/L, and the nadir GH value after oral glucose tolerance test (OGTT) less than 1 µg/L were used as the criteria of cure. Findings: The patients were preoperatively in Hardy's tumor grade 0 (29), A (21), A+E (3), B (21), B+E (5), C (9), C+E (10), D (1) D+E (11), E (5), respectively. One hundred and one patients were available for follow-up (FU; median FU duration: 84 months; range: 6 to 132 months). Surgical cure was achieved

  20. A Single-Center 10-Year Experience with Pasireotide in Cushing's Disease: Patients' Characteristics and Outcome.

    Trementino, L; Michetti, G; Angeletti, A; Marcelli, G; Concettoni, C; Cardinaletti, C; Polenta, B; Boscaro, M; Arnaldi, G

    2016-05-01

    Pasireotide is the first pituitary-directed drug approved for treating patients with Cushing's disease (CD). Our 10-year experience with pasireotide in CD is reported here. Twenty patients with de novo, persistent, or recurrent CD after pituitary surgery were treated with pasireotide from December 2003 to December 2014. Twelve patients were treated with pasireotide in randomized trials and 8 patients with pasireotide sc (Signifor(®); Novartis AG, Basel, Switzerland) in clinical practice. The mean treatment duration was 20.5 months (median 9 months; range, 3-72 months). Urinary free cortisol (UFC) levels mean percentage change (± SD) at last follow-up was-40.4% (± 35.1; range, 2-92%; median reduction 33.3%) with a normalization rate of 50% (10/20). Ten patients achieved sustained normalized late night salivary cortisol (LNSC) levels during treatment. LNSC normalization was associated with UFC normalization in 7/10 patients. Serum cortisol and plasma ACTH significantly decreased from baseline to last follow-up. Body weight decrease and blood pressure improvement during pasireotide treatment were independent from UFC response. Glucose profile worsening was observed in all patients except one. The frequency of diabetes mellitus increased from 40% (8/20) at baseline to 85% (17/20) at last follow-up requiring initiation of medical treatment only in 44% of patients. Pasireotide treatment was associated with sustained biochemical and clinical benefit in about 60% of CD patients. Glucose profile alteration is a frequent complication of pasireotide treatment; however, it seems to be easy to manage with diet and lifestyle intervention in almost half of the patients. PMID:27127913

  1. Medical Therapy of Acromegaly

    U. Plöckinger

    2012-01-01

    Full Text Available This paper outlines the present status of medical therapy of acromegaly. Indications for permanent postoperative treatment, postirradiation treamtent to bridge the interval until remission as well as primary medical therapy are elaborated. Therapeutic efficacy of the different available drugs—somatostatin receptor ligands (SRLs, dopamine agonists, and the GH antagonist Pegvisomant—is discussed, as are the indications for and efficacy of their respective combinations. Information on their mechanism of action, and some pharmakokinetic data are included. Special emphasis is given to the difficulties to define remission criteria of acromegaly due to technical assay problems. An algorithm for medical therapy in acromegaly is provided.

  2. Somatotroph Pituitary Adenoma with Acromegaly and Autosomal Dominant Polycystic Kidney Disease – SSTR5 polymorphism and PKD1 mutation

    Syro, Luis V.; Sundsbak, Jamie L.; Scheithauer, Bernd W.; Toledo, Rodrigo A.; Camargo, Mauricio; Heyer, Christina M.; Sekiya, Tomoko; Uribe, Humberto; Escobar, Jorge I.; Vasquez, Martin; Rotondo, Fabio; Toledo, Sergio P. A.; Kovacs, Kalman; Horvath, Eva; Babovic-Vuksanovic, Dusica; Harris, Peter C.

    2014-01-01

    A 39-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) presented with acromegaly and a pituitary macroadenoma. There was a family history of this renal disorder. She had undergone surgery for pituitary adenoma 6 years prior. Physical examination disclosed bitemporal hemianopsia and elevation of both basal growth hormone (GH) 106 ng/mL (normal 0–5) and insulin-like growth factor (IGF-1) 811 ng/mL (normal 48–255) blood levels. A magnetic resonance imaging scan disclosed a 3.0 cm sellar and suprasellar mass with both optic chiasm compression and left cavernous sinus invasion. Histologic, immunohistochemical and ultrastructural studies of the lesion disclosed a sparsely granulated somatotroph adenoma. Standard chromosome analysis on the blood sample showed no abnormality. Sequence analysis of the coding regions of PKD1 and PKD2 employing DNA from both peripheral leukocytes and the tumor revealed the most common PKD1 mutation, 5014_5015delAG. Analysis of the entire SSTR5 gene disclosed the variant c.143C>A (p.L48M, rs4988483) change in the heterozygous state in both blood and tumor, while no pathogenic mutations were noted in the MEN1, AIP, p27Kip1 and SSTR2 genes. To our knowledge, this is the fourth reported case of a GH-producing pituitary adenoma associated with ADPKD, but the first subject to extensive morphological, ultrastructural, cytogenetic and molecular studies. The question arises whether the physical proximity of the PKD1 and SSTR5 genes on chromosome 16 indicates a causal relationship between ADPKD and the somatotroph adenoma. PMID:21744088

  3. High resolution pituitary gland MRI at 7.0 tesla: a clinical evaluation in Cushing's disease

    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.)

  4. High resolution pituitary gland MRI at 7.0 tesla: a clinical evaluation in Cushing's disease

    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.)

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

    Fatemeh Esfahanian; Rozana Kazemi

    2010-01-01

    "nRealizing 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 hospit...

  6. Homocysteine levels in acromegaly patients.

    Hekimsoy, Zeliha; Ozmen, Bilgin; Ulusoy, Sadik

    2005-12-01

    Acromegaly is associated with a two to three-fold increase in mortality related predominantly to cardiovascular disease. The excess mortality is associated most closely with higher levels of growth hormone (GH). Survival in acromegaly may be normalized to a control age-matched rate by controlling GH levels; in particular, GH levels less than 2.5 ng/mL are associated with survival rates equal to those of the general population. Hyperhomocysteinemia has also been recognized as a risk factor for cardiovascular disease, yet there are limited data on the prevalence of hyperhomocysteinemia in patients with acromegaly. Eighteen acromegaly patients (7 male, 11 female, mean age 42.8 +/- 11.0 years) in our endocrine clinic consented to having the following tests performed: complete blood count (CBC), thyroid hormones, folic acid, vitamin B12, plasma homocysteine levels, uric acid, fibrinogen, CRP, fasting glucose, insulin, C-peptide, total serum cholesterol, HDL cholesterol, LDL cholesterol, triglycerides, GH, insulin-like growth factor-1 (IGF-1) and GH levels after an oral glucose tolerance test (OGTT). By history, fourteen had macroadenomas and four had microadenomas; eight had hypertension; two had glucose intolerance, and four had diabetes. Fifteen had had transsphenoidal or transfrontal surgery: two had been cured, but 13 others were taking long-acting octreotide. Five patients had undergone radiotherapy and the acromegaly in two was treated primarily with long-acting octreotide. CBC, thyroid hormone, folic acid, and vit B12 levels were normal in all patients. We divided the patients into two groups according to mean GH levels after an OGTT: Group 1 (GH<2.5 ng/mL, n=10), and Group 2 (GH<2.5 ng/mL, n=8). Comparison of the two groups using Mann-Whitney U testing revealed statistically significant lower levels in Group 1 of the following parameters: GH (1.91 +/- 0.90 vs. 8.58 +/- 5.55 ng/mL, p=0.002), IGF-1 (338.30 +/- 217.90 vs. 509.60 +/- 293.58 ng/dL, p=0.06), GH after

  7. Urinary growth hormone excretion in acromegaly

    Main, K M; Lindholm, J; Vandeweghe, M;

    1993-01-01

    The biochemical assessment of disease activity in acromegaly still presents a problem, especially in treated patients with mild clinical symptoms. We therefore examined the diagnostic value of the measurement of urinary growth hormone (GH) excretion in seventy unselected patients with acromegaly of...... different activity by comparing it to serum GH, serum insulin-like growth factor I (IGF-I) and clinical activity. There were highly significant, positive correlations between urinary GH and serum GH, serum IGF-I as well as clinical activity score (p < 0.00005), although some overlap between the groups was...

  8. Long-term follow-up of low-dose external pituitary irradiation for Cushing's disease

    Littley, M.D.; Shalet, S.M.; Beardwell, C.G.; Ahmed, S.R.; Sutton, M.L. (Christie Hospital, Manchester (UK))

    1990-10-01

    Twenty-four patients (three male) with Cushing's disease, aged between 11 and 67 years, were treated with low-dose external pituitary irradiation (20 Gy in eight fractions over 10-12 days) and followed for between 13 and 171 months (median 93 months). Eleven patients (46%) went into remission 4-36 months after irradiation, but five subsequently relapsed. In this series, the low incidence of radiation-induced hypopituitarism and absence of other complications attributable to radiotherapy suggest that low-dose pituitary irradiation may be a useful treatment option in selected patients. However, long-term follow-up has demonstrated a high relapse rate and failure to prevent Nelson's syndrome in adrenalectomized patients, indicating that it should not be used as primary treatment in preference to selective adenomectomy. (author).

  9. Acromegaly associated with gangliocytoma.

    Crowley, R K

    2009-09-30

    BACKGROUND: Acromegaly secondary to growth hormone-releasing hormone (GHRH) excess is rare. AIMS\\/CASE DESCRIPTION: We report two patients with acromegaly who were diagnosed with sellar gangliocytomas that were immunopositive for GHRH. Tumour tissue persisted after debulking surgery and in the second case this was associated with persistent growth hormone hypersecretion, successfully suppressed by a somatostatin analogue. CONCLUSIONS: The development of functional pituitary adenomas in association with sellar gangliocytomas is poorly understood. We present a brief discussion of the possible aetiology of these unusual pituitary tumours.

  10. An update on the treatment of acromegaly

    Edling KL

    2013-02-01

    Full Text Available Kari L Edling,1 Anthony P Heaney1,21Department of Medicine, 2Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USAAbstract: Acromegaly is caused by pituitary somatotroph hypersecretion of growth hormone leading to elevated hepatic-derived and local levels of insulin-like growth factor-1. It is associated with increased morbidity and mortality due primarily to cardiovascular disease and diabetes mellitus. Normalization of growth hormone and insulin-like growth factor-1 levels has been associated with decreased morbidity from metabolic and cardiovascular effects, as well as reduced overall mortality in epidemiologic studies. Many patients experience a delay in obtaining a diagnosis, have pituitary macroadenomas at presentation, and accordingly, a significant number will not be cured by tumor surgical resection alone. Adjunctive radiation therapy cannot always offer biochemical and clinical disease control and carries a 40% risk of partial or total pituitary failure in the medium term. Several monotherapies or combination medical therapies are currently available for both primary and adjuvant acromegaly treatment, and include long-acting somatostatin analogs, the growth hormone receptor antagonist pegvisomant, and dopamine agonists. Next generation somatostatin analogs and new drug delivery methods of existing agents are in ongoing clinical studies. This paper will review current and novel therapies under development for acromegaly.Keywords: acromegaly, growth hormone, pituitary tumors, somatostatin analog, pasireotide, pegvisomant

  11. Voice characteristics of acromegaly.

    Aydin, Kadriye; Turkyilmaz, Didem; Ozturk, Burak; Dagdelen, Selcuk; Ozgen, Burce; Unal, Faruk; Erbas, Tomris

    2013-03-01

    Acromegaly's effect on voice is still indefinite. We aimed to define acoustic characteristics of patients with acromegaly. Cross-sectional case-control study was designed. Thirty-seven patients with acromegaly and 30 age- and sex-matched healthy controls were included. Fundamental frequency (F0) and measurements related to frequency, amplitude, noise and tremor of the obtained voice sample were analyzed using Multi-Dimensional Voice Program. Absolute jitter (Jita) and jitter percent (Jitt), shimmer in decibel and shimmer percent, noise to harmonic ratio and soft phonation index, fundamental frequency tremor frequency and frequency tremor intensity index represented the parameters related to frequency, amplitude, noise and tremor of the voice sample, respectively. Patients with acromegaly, especially the uncontrolled patients, exhibited significant differences in frequency perturbation measurements. Jitt of all patients and Jita of uncontrolled patients were significantly higher than that of control group (p = 0.044 and p = 0.043, respectively). Jitter which is a measure of frequency perturbation can be assumed as an indicator of hoarse and deepened voice. Jita of all patients and Jitt of uncontrolled patients were elevated, but not reaching a statistical significance. Controlled and active patients had similar analysis of acoustic parameters. In the correlation analysis, shimmer and IGF-1 (insulin like growth factor 1) was found to be positively correlated in all patients with acromegaly and in female patients. When the p value is adjusted according to Bonferroni correction regarding the use of ten parameters for acoustic analysis (so adjusted p is <0.005), all the statistically significant findings become insignificant. Considering the parameters test different properties of voice, it is reasonable to pay attention to the findings. Patients with acromegaly have increased frequency perturbations measures, but this increase is non-significant according to Bonferroni

  12. Imaging in Cushing's syndrome

    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)

  13. A male patient with acromegaly and breast cancer: treating acromegaly to control tumor progression

    Acromegaly is a rare disease associated with an increased risk of developing cancer. We report the case of a 72-year-old man who was diagnosed with acromegaly (IGF-1 770 ng/ml) and breast cancer. Four years before he suffered from a colon-rectal cancer. Pituitary surgery and octreotide-LAR treatment failed to control acromegaly. Normalization of IGF-1 (97 ng/ml) was obtained with pegvisomant therapy. Four years after breast cancer surgery, 2 pulmonary metastases were detected at chest CT. The patient was started on anastrozole, but, contrary to medical advice, he stopped pegvisomant treatment (IGF-I 453 ng/ml). Four months later, chest CT revealed an increase in size of the metastatic lesion of the left lung. The patient was shifted from anastrozole to tamoxifen and was restarted on pegvisomant, with normalization of serum IGF-1 levels (90 ng/ml). Four months later, a reduction in size of the metastatic lesion of the left lung was detected by CT. Subsequent CT scans throughout a 24-month follow-up showed a further reduction in size and then a stabilization of the metastasis. This is the first report of a male patient with acromegaly and breast cancer. The clinical course of breast cancer was closely related to the metabolic control of acromegaly. The rapid progression of metastatic lesion was temporally related to stopping pegvisomant treatment and paralleled a rise in serum IGF-1 levels. Normalization of IGF-1 after re-starting pegvisomant impressively reduced the progression of metastatic breast lesions. Control of acromegaly is mandatory in acromegalic patients with cancer. The online version of this article (doi:10.1186/s12885-015-1400-0) contains supplementary material, which is available to authorized users

  14. Radiation techniques for acromegaly

    Minniti Giuseppe

    2011-12-01

    Full Text Available Abstract Radiotherapy (RT remains an effective treatment in patients with acromegaly refractory to medical and/or surgical interventions, with durable tumor control and biochemical remission; however, there are still concerns about delayed biochemical effect and potential late toxicity of radiation treatment, especially high rates of hypopituitarism. Stereotactic radiotherapy has been developed as a more accurate technique of irradiation with more precise tumour localization and consequently a reduction in the volume of normal tissue, particularly the brain, irradiated to high radiation doses. Radiation can be delivered in a single fraction by stereotactic radiosurgery (SRS or as fractionated stereotactic radiotherapy (FSRT in which smaller doses are delivered over 5-6 weeks in 25-30 treatments. A review of the recent literature suggests that pituitary irradiation is an effective treatment for acromegaly. Stereotactic techniques for GH-secreting pituitary tumors are discussed with the aim to define the efficacy and potential adverse effects of each of these techniques.

  15. Sleep apnoea in acromegaly.

    Perks, W H; Horrocks, P M; Cooper, R A; Bradbury, S; Allen, A; Baldock, N; Prowse, K.; Van't Hoff, W

    1980-01-01

    Day time somnolence or excessive snoring, or both, occurred in five out of 11 patients with acromegaly. All five had episodes of sleep apnoea, and three had the sleep apnoea syndrome. Growth hormone concentrations were higher (p less than 0.025) in these patients than in the six patients without these symptoms. One patient with daytime somnolence and one asymptomatic patient had flow loop evidence of upper airways obstruction. Two of the patients with the sleep apnoea syndrome had cardiomegal...

  16. Diabetic retinopathy in acromegaly

    Said Azzoug

    2014-01-01

    Full Text Available Introduction: Although growth hormone (GH has been implicated in the pathogenesis of diabetic retinopathy (DR, DR is deemed to be rare in patients with GH excess. Our aim was to study its prevalence in subjects with acromegaly suffering from diabetes mellitus (DM, to analyze its characteristics, and to look for predictive factors such as age at diagnosis, GH concentration and duration, DM duration, DM control, and family background. Materials and Methods: Forty patients with acromegaly and DM (21 males, 19 females, median age = 50 years, underwent a systematic ophthalmological examination with dilated funduscopy to seek diabetic retinopathy. Results: Among this population, 05 (12.5% had DR. It was at an early stage or background retinopathy in 3 cases and at a more advanced stage or proliferative retinopathy in 2 cases. We did not find any correlation with age at diagnosis, GH levels and duration, DM duration and family history of DM, but poor glycemic control seems to play a role although statistical analysis showed borderline significance. Conclusion: From this study, we conclude that prevalence of DR in patients with acromegaly is 12.5%, and it is slight or moderate. Among studied factors, only poor glycemic control seems to be implicated in its development.

  17. Cushing's Syndrome

    ... Research Training & Career Development Grant programs for students, postdocs, and faculty Research at NIDDK Labs, faculty, and ... diabetes, digestive and liver diseases, kidney diseases, weight control and nutrition, urologic diseases, endocrine and metabolic diseases, ...

  18. 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

    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

  19. Neurocognitive function in acromegaly after surgical resection of GH-secreting adenoma versus naive acromegaly.

    Juan Francisco Martín-Rodríguez

    Full Text Available Patients with active untreated acromegaly show mild to moderate neurocognitive disorders that are associated to chronic exposure to growth hormone (GH and insulin-like growth factor (IGF-I hypersecretion. However, it is unknown whether these disorders improve after controlling GH/IGF-I hypersecretion. The aim of this study was to compare neurocognitive functions of patients who successfully underwent GH-secreting adenoma transsphenoidal surgery (cured patients with patients with naive acromegaly. In addition, we wanted to determine the impact of different clinical and biochemical variables on neurocognitive status in patients with active disease and after long-term cure. A battery of six standardized neuropsychological tests assessed attention, memory and executive functioning. In addition, a quantitative electroencephalography with Low-Resolution Electromagnetic Tomography (LORETA solution was performed to obtain information about the neurophysiological state of the patients. Neurocognitive data was compared to that of a healthy control group. Multiple linear regression analysis was also conducted using clinical and hormonal parameters to obtain a set of independent predictors of neurocognitive state before and after cure. Both groups of patients scored significantly poorer than the healthy controls on memory tests, especially those assessing visual and verbal recall. Patients with cured acromegaly did not obtain better cognitive measures than naïve patients. Furthermore memory deficits were associated with decreased beta activity in left medial temporal cortex in both groups of patients. Regression analysis showed longer duration of untreated acromegaly was associated with more severe neurocognitive complications, regardless of the diagnostic group, whereas GH levels at the time of assessment was related to neurocognitive outcome only in naïve patients. Longer duration of post-operative biochemical remission of acromegaly was associated with

  20. Lack of diurnal rhythm of low molecular weight insulin-like growth factor binding protein in patients with Cushing's disease

    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 ..mu..g/l) were found between 13.00 and 24.00 h with a rise after midnight to maximum levels (23-71 ..mu..g/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.

  1. Long term follow-up of Cushing's disease treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy

    Subtotal adrenalectomy was given to 10 adult patients with Cushing's disease, concurrently with or following therapeutic regimen by long term reserpine administration and pituitary irradiation. In the present study, we describe long term follow-up results. Two patients died after the operation due to acute adrenal crisis and pneumonia, respectively. The other 8 patients achieved clinical and biochemical remissions and were followed for long term. Three patients relapsed 9, 14 or 17 years after achieving remission, two patients developed hypopituitarism 12 or 20 years after and one died of cerebral vascular accident at 64 years, 5 years after the remission. The remaining 2 patients maintained remission for 10 or 18 years, respectively. During the remission periods of 0.5 to 20 years with a mean of 10.1±6.7 years, 6 of 7 patients examined by 1 mg overnight dexamethasone test showed normal suppressibility of plasma cortisol. Provocative tests of plasma GH by 1-arginine infusion and/or insulin-induced hypoglycemia were performed in 6 patients in the early remission period. All of 5 patients in the arginine infusion test and 3 of 5 in the insulin-induced hypoglycemia test showed normal responses. Furthermore, to facilitate prediction of long term response or failure to our therapeutic regimen, long term reserpine administration and pituitary irradiation, pretreatment clinical and biochemical characteristics were analyzed retrospectively in 3 divided groups; the present 10 patients treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy, 11 patients achieving long term remission treated by our regimen alone, and 7 patients failed with our regimen alone. There were no significant factors predictive of response to our regimen. (author)

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

    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.

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

    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

  4. Megavoltage pituitary irradiation in the management of Cushing's disease and Nelson's syndrome: long-term follow-up

    Howlett, T.A.; Plowman, P.N.; Wass, J.A.H.; Rees, L.H.; Jones, A.E.; Besser, G.M. (Saint Bartholomew' s Hospital, London (UK))

    1989-09-01

    The authors report the long-term follow-up (up to 17.3 years) of the clinical and biochemical effects of megavoltage pituitary irradiation (radiotherapy;RT), administered as primary or secondary therapy, for pituitary Cushing's disease and Nelson's syndrome in 52 patients. Irradiation was administered, from a 4-15 MeV linear accelerator, via a three-field technique, to a total dose of 4500 cGy (rad) in 25 fractions over 35 days. (author).

  5. 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

    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

  6. Iatrogenic Cushing syndrome secondary to topical steroids Case Report

    Çakır, Murat; Ağırman, İlknur; Mungan, İlke; Orhan, Fazıl; Ökten, Ayşenur

    2005-01-01

    Although glucocorticoids are frequently used for a wide variety of skin diseases sometimes they may cause reversible hypothalamic pituitaryadrenal axis suppression in some patients Here we report a 6 month old girl with Cushing syndrome that developed secondary to inappropriate topical corticosteroid use Key words: cushing syndrome topical corticosteroids

  7. Restrictive extraocular myopathy: A presenting feature of acromegaly

    Steven Heireman

    2011-01-01

    Full Text Available A 45-year-old man presented with binocular diplopia in primary gaze for 1 year. Orthoptic evaluation showed 10-prism diopter right eye hypotropia and 6-prism diopter right eye esotropia. The elevation and abduction of the right eye were mechanically restricted. This was associated with systemic features suggestive of acromegaly. Magnetic resonance imaging (MRI of the brain demonstrated a pituitary macroadenoma. An elevated serum insulin-like growth factor I level and the failure of growth hormone suppression after an oral glucose load biochemically confirmed the diagnosis of acromegaly. Computed tomography (CT of the orbit demonstrated bilateral symmetrical enlargement of the medial rectus and inferior rectus muscle bellies. All tests regarding Graves-Basedow disease were negative. Although rare, diplopia due to a restrictive extraocular myopathy could be the presenting symptom of acromegaly.

  8. Mandibular prognathism caused by acromegaly – a surgical orthodontic case

    Proff Peter

    2009-08-01

    Full Text Available Abstract A 22-year-old man presented for orthodontic surgery because of mandibular prognathism. Clinical symptoms suggested acromegaly, and diagnosis was verified by an endocrinologist as well as by radiograph. Bilateral mandibular prognathism often represents the first and most striking physical characteristic of acromegaly; usually, it is also the main reason why patients seek help from orthodontists or maxillo-facial surgeons. This case report recapitulates the clinical and histopathological findings in pituitary growth hormone (GH adenomas and emphasises their importance in surgical orthodontic planning. Mandibular prognatism, macroglossia and abnormal growth of hands and feet represent strong indicators for the diagnosis of acromegaly. This disease and its complications not only affect the entire body but increase mortality if the pituitary gland tumour remains untreated.

  9. Echocardiographic Findings in 11 Cats with Acromegaly

    Myers, J A; Lunn, K.F.; Bright, J.M.

    2014-01-01

    Background Information regarding cardiac changes in domestic cats with acromegaly is limited. Hypothesis/Objectives The objective of this study was to describe the echocardiographic findings in cats with acromegaly. Animals Eighteen cats diagnosed with acromegaly at Colorado State University between 2008 and 2012. Of these 18 cats, 11 had echocardiography performed. Methods A retrospective review of medical records was made to identify cats with acromegaly that also had echocardiography perfo...

  10. Pasireotide: a novel treatment for patients with acromegaly.

    Cuevas-Ramos, Daniel; Fleseriu, Maria

    2016-01-01

    Morbidity and mortality rates in patients with active acromegaly are higher than the general population. Adequate biochemical control restores mortality to normal rates. Now, medical therapy has an increasingly important role in the treatment of patients with acromegaly. Somatostatin receptor ligands (SRLs) are considered the standard medical therapy, either after surgery or as a first-line therapy when surgery is deemed ineffective or is contraindicated. Overall, octreotide and lanreotide are first-generation SRLs and are effective in ~20%-70% of patients. Pegvisomant, a growth hormone receptor antagonist, controls insulin-like growth factor 1 in 65%-90% of cases. Consequently, a subset of patients (nonresponders) requires other treatment options. Drug combination therapy offers the potential for more efficacious disease control. However, the development of new medical therapies remains essential. Here, emphasis is placed on new medical therapies to control acromegaly. There is a focus on pasireotide long-acting release (LAR) (Signifor LAR®), which was approved in 2014 by the US Food and Drug Administration and the European Medicine Agency for the treatment of acromegaly. Pasireotide LAR is a long-acting somatostatin multireceptor ligand. In a Phase III clinical trial in patients with acromegaly (naïve to medical therapy or uncontrolled on a maximum dose of first-generation SRLs), 40 and 60 mg of intramuscular pasireotide LAR achieved better biochemical disease control than octreotide LAR, and tumor shrinkage was noted in both pasireotide groups. Pasireotide LAR tolerability was similar to other SRLs, except for a greater frequency and degree of hyperglycemia and diabetes mellitus. Baseline glucose may predict hyperglycemia occurrence after treatment, and careful monitoring of glycemic status and appropriate treatment is required. A precise definition of patients with acromegaly who will derive the greatest therapeutic benefit from pasireotide LAR remains to be

  11. Clinical use of pasireotide for Cushing's disease in adults

    Ceccato F

    2015-03-01

    Full Text Available Filippo Ceccato, Carla Scaroni, Marco Boscaro Endocrinology Unit, Department of Medicine, Padova University Hospital, Padova, Italy Cushing’s disease: Excessive corticotroph hormone levels sustained by an adrenocorticotropic hormone-secreting pituitary adenoma lead to a severe clinical condition caused by excess cortisol secretion, called Cushing’s disease (CD. Neurosurgery and radiotherapy are used to treat the pituitary adenoma directly, but new medical treatments targeting the corticotroph cells have recently become available. Pasireotide: This is a novel multireceptor ligand somatostatin (SST analog with a high binding affinity for SST receptor 5, the predominant receptor in human corticotroph adenomas that is not downregulated by high cortisol levels (as SST receptor 2 is. Pasireotide has been recently approved by the European Medical Agency and the US Food and Drug Administration for treating adults with CD with recurrent hypercortisolism after surgery, or for whom surgery is not an option. A dose of 600–1,200 µg twice a day can normalize urinary free cortisol levels after 3 months of treatment in up to 28% of patients, reducing their blood pressure and improving their weight, lipid profile, and quality of life. Combining pasireotide with cabergoline to achieve a greater hormone response can normalize cortisol secretion in 50% of patients, and adding ketoconazole induces biochemical control in most patients with CD. Safety and hyperglycemia: The adverse effects of pasireotide are similar to those of other SST analogs, including diarrhea, nausea, and biliary sludge or gallstones. Hyperglycemia is common during pasireotide treatment, which affects the secretion of pancreatic insulin and intestinal glucagon-like peptide 1. Self-monitoring is essential to achieve good metabolic control, and endocrinologists should first administer metformin if insulin resistance is evident and then add dipeptidyl peptidase 4 inhibitors

  12. Mortality in patients with pituitary disease.

    Sherlock, Mark

    2010-06-01

    Pituitary disease is associated with increased mortality predominantly due to vascular disease. Control of cortisol secretion and GH hypersecretion (and cardiovascular risk factor reduction) is key in the reduction of mortality in patients with Cushing\\'s disease and acromegaly, retrospectively. For patients with acromegaly, the role of IGF-I is less clear-cut. Confounding pituitary hormone deficiencies such as gonadotropins and particularly ACTH deficiency (with higher doses of hydrocortisone replacement) may have a detrimental effect on outcome in patients with pituitary disease. Pituitary radiotherapy is a further factor that has been associated with increased mortality (particularly cerebrovascular). Although standardized mortality ratios in pituitary disease are falling due to improved treatment, mortality for many conditions are still elevated above that of the general population, and therefore further measures are needed. Craniopharyngioma patients have a particularly increased risk of mortality as a result of the tumor itself and treatment to control tumor growth; this is a key area for future research in order to optimize the outcome for these patients.

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

    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)

  14. Cushing's Support and Research Foundation

    ... and find other resources. READ MORE > Cushing's Support & Research Foundation Meet CSRF FORUM Meet and share your experiences ... Forum Privacy Policy Disclaimer Copyright © CSRF • Cushing's Support & Research Foundation 60 Robbins Rd, #12 • Plymouth, MA 02360

  15. A Novel Mutation in the type Iα Regulatory Subunit of Protein Kinase A (PRKAR1A) in a Cushing's Syndrome Patient with Primary Pigmented Nodular Adrenocortical Disease.

    Mineo, Ryohei; Tamba, Sachiko; Yamada, Yuya; Okita, Tomonori; Kawachi, Yusuke; Mori, Reiko; Kyo, Mitsuaki; Saisho, Kenji; Kuroda, Yohei; Yamamoto, Koji; Furuya, Akiko; Mukai, Tokuo; Maekawa, Takashi; Nakamura, Yasuhiro; Sasano, Hironobu; Matsuzawa, Yuji

    2016-01-01

    A 40-year-old man presented with Cushing's syndrome due to bilateral adrenal hyperplasia with multiple nodules. Computed tomography scan results were atypical demonstrating an enlargement of the bilateral adrenal glands harboring multiple small nodules, but the lesion was clinically diagnosed to be primary pigmented nodular adrenocortical disease (PPNAD) based on both endocrinological test results and his family history. We performed bilateral adrenalectomy and confirmed the diagnosis histologically. An analysis of the patient and his mother's genomic DNA identified a novel mutation in the type Iα regulatory subunit of protein kinase A (PRKAR1A) gene; p.E17X (c.49G>T). This confirmed the diagnosis of PPNAD which is associated with Carney Complex. PMID:27580546

  16. Stress ulcers - Cushing ulcers: diagnosis, treatment, prevention

    R. Şcerbina

    2012-11-01

    Full Text Available BACKGROUND: Cushing ulcers appear at the 3rd to 5th day after stress situations accompanied with hemorrhage in 5-10%, perforations in 4%. METHODS: Between 2007-2011 in the National Scientific and Practical Center for Emergency Medicine Kishinev, 1917 patients with superior gastric hemorrhages were treated, from which 139 in ICU. RESULTS: In 45 cases were diagnosed Cushing ulcers: men – 30, women – 15 with age between 21 years and 87 years. The disease causes were: cerebral vascular diseases 13(28.8%; fractures with hip replacement – 16 (35.5%; chronic renal insufficiency and septic states - 3 (12.32%; severe head trauma – 10 (22.2%. The mortality rate was 22.2% (10 patients. Gastroscopy revealed hemorrhages followed by endoscopic hemostasis. For the prophylaxis of hemorrhage relapse all patients underwent repeated endoscopic hemostasis 2 to 3 times. One patient was operated for a perforated bulbar Cushing ulcer and another one due to Forrest IA hemorrhage that didn’t respond to endoscopic hemostasis – Bilroth I antrum resection. Both patients died due to multiple organ dysfunction syndrome (MODS. Patients with Cushing ulcers received anti-ulcer treatment: PPI, H2 blockers in maximum dosages, hemostatic and repeated blood plasma transfusions. CONCLUSIONS: Gastroscopy is obligatory in all patients with severe head trauma, septic states, chronic renal insufficiency and severe cerebral vascular diseases. Anti-ulcer and hemostatic treatment insures ulcer healing and scarring in most of the cases.

  17. Cognitive function in acromegaly: description and brain volumetric correlates.

    Sievers, Caroline; Sämann, P G; Pfister, H; Dimopoulou, C; Czisch, M; Roemmler, J; Schopohl, J; Stalla, G K; Zihl, J

    2012-09-01

    In acromegaly, we reported on increased rates of affective disorders such as dysthymia and depression, as well as structural brain changes. Objective of this study was to determine if cognitive impairments in patients with acromegaly exist and whether such impairments are associated with structural brain alterations defined by magnetic resonance imaging (MRI). In this cross-sectional study, 55 patients with biochemically confirmed acromegaly were enrolled. MRI data were compared with 87 control subjects. Main outcome measures were performance levels in 13 cognitive tests covering the domains of attention, memory and executive function, with performance below the cut-off level of the 16th percentile rated as impaired. In addition, individual global and hippocampal volume changes were defined for each patient in reference to a normative sample. We found that up to 33.3% of the patients were impaired in the attention, up to 24.1% in the memory, and up to 16.7% in the executive function domain. 67.3% of the patients failed to reach the cut-off level in at least one subtest. MRI demonstrated increased global, left and right hippocampal grey matter and white matter, particularly early in the disease course. Rather few positive than expected negative correlations could be established between the hippocampal grey matter gain and cognitive performance. Cognitive dysfunction, particularly attentional deficits, are common in acromegaly, rendering neuropsychological testing essential in the diagnostic work-up. PMID:21735089

  18. Estados de pseudo-Cushing Pseudo-Cushing states

    Daniella J.P.C. Romanholi

    2007-11-01

    Full Text Available Síndromes de pseudo-Cushing são um grupo heterogêneo de doenças, incluindo alcoolismo, anorexia nervosa, obesidade visceral e depressão, que compartilham muitas das características clínicas e bioquímicas da síndrome de Cushing. Os mecanismos responsáveis para a gênese da síndrome de pseudo-Cushing são fracamente compreendidos. Tem sido sugerido que o hipercortisolismo da síndrome de pseudo-Cushing pode ser resultante do aumento da secreção do hormônio liberador de corticotrofina (CRH hipotalâmico no contexto de um eixo hipotálamo-hipofisário-adrenal que, de outra maneira, está normalmente constituído. A sobreposição substancial entre as características clínicas e laboratoriais entre muitos pacientes com síndrome de Cushing e aqueles com síndrome de pseudo-Cushing pode tornar o diagnóstico diferencial difícil. Distinguir entre síndrome de pseudo-Cushing e síndrome de Cushing verdadeira é crítico para se prevenir o tratamento desnecessário e potencialmente prejudicial de tais pacientes. Esta breve revisão sumariza os principais eventos patofisiológicos das síndromes de pseudo-Cushing e fornece uma estratégia útil para o seu diagnóstico diferencial.Pseudo-Cushing syndromes are a heterogeneous group of disorders, including alcoholism, anorexia nervosa, visceral obesity, and depression, which share many of the clinical and biochemical features of Cushing's syndrome. The mechanisms responsible for the genesis of pseudo-Cushing's syndrome are poorly understood. It has been suggested that hypercortisolism of pseudo-Cushing syndrome may be the result of increased hypothalamic corticotrophin-releasing hormone (CRH secretion in the context of a hypothalamic-pituitary-adrenal axis that is otherwise normally constituted. The substantial overlap in clinical and biochemical features among several patients with Cushing syndrome and those with pseudo-Cushing syndromes can make the differential diagnosis difficult

  19. Evaluation of left ventricular diastolic function according to new criteria and determinants in acromegaly.

    Akdeniz, Bahri; Gedik, Arzu; Turan, Onur; Ozpelit, Ebru; Ikiz, Ahmet Omer; Itil, Oya; Badak, Ozer; Baris, Nezihi; Cömlekçi, Abdurrahman

    2012-01-01

    Left ventricular diastolic dysfunction (LVDD) develops in the early stages of acromegaly. The purpose of this study was to identify LVDD analyzing by new echocardiograpic criteria as well as to evaluate determinants of the LVDD in acromegaly. This cross-sectional study examined 42 patients with acromegaly; 16 in active disease (AA) and 26 cured/ well controlled (CA), and compared them with 30 healthy controls (CG). Ventricular systolic and diastolic functions were studied by conventional and tissue Doppler imaging based on the E/Em ratio and myocardial performance index (MPI). Other clinical parameters possibly contributing to LVDD in acromegaly were also investigated. The prevalence of LV hypertrophy (33%) and LVDD (35.7%) were increased in acromegaly, however, there were no differences between the AA and CA groups. Acromegalic patients had higher LV volumes and LV mass, and septal E/Em ratio compared to CG, whereas LV ejection fraction and MPI were not different. The presence of acromegaly (r = 0.29, P = 0.013), diabetes mellitus (DM) (r = 0.41, P < 0.001), hypertension (r = 0.35, P = 0.002), and sleep apnea (r = 0.56, P = 0.003) were found to be correlated with LVDD, whereas duration and activity of acromegaly were not. In regression analysis, advanced age (OR: 8.53, P = 0.006) and DM (OR: 25.9, P = 0.007) were found to be independent risk factors for LVDD. The risk of LVDD according to new criteria increases in acromegaly. However, it seems to be related to the presence of DM and advanced age and is independent of disease duration and activity. PMID:23038091

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

    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 131I-19-iodocholesterol

  1. Pasireotide in Acromegaly: A Review.

    McKeage, Kate

    2015-06-01

    Pasireotide (Signifor(®), Signifor(®) LAR) is a somatostatin analogue recently approved for the treatment of acromegaly. Unlike the first-generation agents, octreotide and lanreotide, which bind preferentially to somatostatin receptor (SSTR)-2, pasireotide binds to multiple SSTRs. This article reviews the clinical use and summarizes the pharmacological properties of intramuscular pasireotide in the treatment of acromegaly. The efficacy of pasireotide 40 mg every 28 days was superior to that of intramuscular octreotide 20 mg every 28 days with regard to biochemical control in a 12-month, phase III trial in medically naive patients. Similarly, in a 6-month, phase III trial in patients with acromegaly inadequately controlled with somatostatin analogues for at least 6 months, the efficacy of pasireotide 40 or 60 mg was superior to that of continued octreotide 30 mg or lanreotide autogel 120 mg (each drug was administered once every 28 days) with regard to biochemical control. The tolerability profile of intramuscular pasireotide is generally similar to that of first-generation agents, except for a higher incidence of hyperglycaemia-related adverse events with pasireotide. In clinical trials, the risk of developing pasireotide-associated hyperglycaemia was numerically greater in patients categorized as diabetic or prediabetic at baseline than in those with normal glucose tolerance. Careful monitoring of glycaemic status is required prior to and during pasireotide treatment and antidiabetic therapy should be commenced as indicated. Thus, in the treatment of acromegaly, pasireotide may be a more effective somatostatin analogue than other approved agents of the same class; however, the increased risk of hyperglycaemia needs to be considered and proactively managed. PMID:26017304

  2. Acromegaly Quality of Life Questionnaire (AcroQoL

    Lara Nuria

    2004-02-01

    Full Text Available Abstract Acromegaly is a chronic disease with an important impact on patients, Health Related Quality of Life (HRQoL. The ability to effectively measure Health Related Quality of Life is central to describing the impacts of disease or treatment upon the patient, therefore the importance of having a disease specific questionnaire for acromegaly. For the development of the AcroQoL questionnaire different sources of information were used: first a literature search was performed to identify relevant papers describing the impact of acromegaly in HRQoL, second the main domains of impact on HRQoL were identified by 10 experts endocrinologists, and third ten in-depth semi-structured interviews were conducted in acromegalic patients to identify domains and items related to the self-perceived impact of acromegaly in patients' life. After a proper qualitative analysis a preliminary 38 item questionnaire was obtained. Rasch analysis concluded with a final 22 item questionnaire. The measurement properties (validity and reliability of the resulting final questionnaire were tested and compared using standard procedures (Cronbach's Alpha and item-total correlation. The evaluation of the item parameters confirmed the construct validity of the new instrument. Responsiveness to change was assessed in a small sample of 32 acromegalic patients with active disease in Spain who were administered the AcroQoL and the generic questionnaire EuroQoL 5-D. The results showed a statistically significant relationship between all the dimensions of AcroQoL and the VAS (visual analogic scale of EQ-5D. An improvement in the global score of AcroQoL was related to a global improvement in the VAS of the EQ-5D. Following the current recommended standard methodology the Spanish questionnaire was translated into eleven other languages.

  3. Pasireotide: a novel treatment for patients with acromegaly

    Cuevas-Ramos D

    2016-01-01

    Full Text Available Daniel Cuevas-Ramos,1 Maria Fleseriu2,3 1Department of Endocrinology and Metabolism, Neuroendocrinology Clinic, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico; 2Department of Medicine (Endocrinology, 3Department of Neurological Surgery, Northwest Pituitary Center, Oregon Health & Science University, Portland, OR, USA Abstract: Morbidity and mortality rates in patients with active acromegaly are higher than the general population. Adequate biochemical control restores mortality to normal rates. Now, medical therapy has an increasingly important role in the treatment of patients with acromegaly. Somatostatin receptor ligands (SRLs are considered the standard medical therapy, either after surgery or as a first-line therapy when surgery is deemed ineffective or is contraindicated. Overall, octreotide and lanreotide are first-generation SRLs and are effective in ~20%–70% of patients. Pegvisomant, a growth hormone receptor antagonist, controls insulin-like growth factor 1 in 65%–90% of cases. Consequently, a subset of patients (nonresponders requires other treatment options. Drug combination therapy offers the potential for more efficacious disease control. However, the development of new medical therapies remains essential. Here, emphasis is placed on new medical therapies to control acromegaly. There is a focus on pasireotide long-acting release (LAR (Signifor LAR®, which was approved in 2014 by the US Food and Drug Administration and the European Medicine Agency for the treatment of acromegaly. Pasireotide LAR is a long-acting somatostatin multireceptor ligand. In a Phase III clinical trial in patients with acromegaly (naïve to medical therapy or uncontrolled on a maximum dose of first-generation SRLs, 40 and 60 mg of intramuscular pasireotide LAR achieved better biochemical disease control than octreotide LAR, and tumor shrinkage was noted in both pasireotide groups. Pasireotide LAR tolerability

  4. Cushing's syndrome: update on signs, symptoms and biochemical screening.

    Nieman, Lynnette K

    2015-10-01

    Endogenous pathologic hypercortisolism, or Cushing's syndrome, is associated with poor quality of life, morbidity, and increased mortality. Early diagnosis may mitigate against this natural history of the disorder. The clinical presentation of Cushing's syndrome varies, in part related to the extent and duration of cortisol excess. When hypercortisolism is severe, its signs and symptoms are unmistakable. However, most of the signs and symptoms of Cushing's syndrome are common in the general population (e.g., hypertension and weight gain) and not all are present in every patient. In addition to classical features of glucocorticoid excess, such as proximal muscle weakness and wide purple striae, patients may present with the associated comorbidities that are caused by hypercortisolism. These include cardiovascular disease, thromboembolic disease, psychiatric and cognitive deficits, and infections. As a result, internists and generalists must consider Cushing's syndrome as a cause, and endocrinologists should search for and treat these comorbidities. Recommended tests to screen for Cushing's syndrome include 1  mg dexamethasone suppression, urine free cortisol, and late night salivary cortisol. These may be slightly elevated in patients with physiologic hypercortisolism, which should be excluded, along with exogenous glucocorticoid use. Each screening test has caveats and the choice of tests should be individualized based on each patient's characteristics and lifestyle. The objective of this review is to update the readership on the clinical and biochemical features of Cushing's syndrome that are useful when evaluating patients for this diagnosis. PMID:26156970

  5. Body composition in acromegaly - before and after therapy

    Acromegaly is a disease caused by a benign pituitary tumor producing excess amount of growth hormone. A changed body composition can be a functional parameter of the disorder. The aim of this study is to describe body composition in acromegaly before and after therapy. Total body potassium (TBK) was measured as total exchangeable potassium using 42K by dilution technique or by counting gamma radiation from the naturally present 40K in a high sensitivity 3π whole body counter. Total body water (TBW) was determined with an isotope dilution technique using tritiated water as a tracer. The specific activity was measured in urine or plasma. The predicted values for TBK, TBW, and body fat (BF) were calculated by equations using body weight (BW), body height, age and sex as independent variables. The normal values for BW were calculated by using body height and sex as independent variables

  6. Clinical management of critically ill patients with Cushing's disease due to ACTH-secreting pituitary macroadenomas: effectiveness of presurgical treatment with pasireotide.

    Cannavo, S; Messina, E; Albani, A; Ferrau, F; Barresi, V; Priola, S; Esposito, F; Angileri, F

    2016-06-01

    The management of critically ill Cushing's disease (CD) patients is extremely challenging. Pasireotide is indicated for the treatment of CD patients when pituitary surgery is unfeasible or has not been curative, but no data are available about the use of this drug as pre-operative treatment in critically ill patients. We report the effects of presurgical pasireotide therapy in CD patients in whom hypercortisolism caused life-threatening hypokalemia, alkalosis, and cardio-respiratory complications precluding surgical approach. Clinical, biochemical, and radiological data of two critically ill patients with ACTH-secreting pituitary macroadenoma, before and during first-line presurgical pasireotide treatment (600 μg s.c. bid). During the first 21 days of treatment, pasireotide therapy induced a rapid, partial decrease of plasma ACTH, serum cortisol, and urinary free cortisol levels, with the consequent normalization of serum potassium concentration and arterial blood gases parameters, in both the patients. They did not experience unmanageable side effects and underwent endoscopic transsphenoidal surgery after 4 weeks of effective treatment. Pre-operative MRI evaluation did not show pituitary tumor shrinkage. Surgical cure of CD was obtained in the first patient, while debulking allowed the pharmacological control of hypercortisolism in the second case. We suggest that pasireotide can induce a rapid improvement of clinical and metabolic conditions in critically ill CD patients in whom surgical approach is considered hazardous and need to be delayed. PMID:25877016

  7. Guías para el diagnóstico y tratamiento de acromegalia, prolactinomas y enfermedad de Cushing

    Chen-Ku Chih Hao

    2004-10-01

    (acromegaly and ACTH (Cushing’s disease. There are different opinions about the diagnosis and treatment of these diseases. We present here the guidelines for diagnosis and treatment of these tumors made by the Costa Rican Association of Endocrinology, Diabetes and Nutrition. The main cause of acromegaly is a pituitary tumor that produces growth hormone. The diagnosis is usually made several years after the disease has started because the physical changes occur very slowly. The initial treatment should be surgery and when it fails, medical therapy with octreotide is the main choice. Radiotherapy can be used although it is limited by the long time it takes to start its effect. If there is hyperprolactinemia, other causes of prolactin elevation should be ruled out. If a prolactinoma is identified, the treatment almost always will be with dopamine agonists. Surgery should be performed only in macroprolactinomas with visual field changes that do not shrink with medical treatment. The first choice for medical treatment will be bromocriptine although there are other agents like cabergoline and quinagolide. In Cushing´s disease, there are different tests for screening for hypercortisolism, after which a confirmatory test should be performed. Initial treatment should be surgery followed by radiotherapy. In these cases, medical treatment is less effective than in other pituitary tumors.

  8. Development of acromegaly in patients with prolactinomas

    Andersen, Marianne; Hagen, Casper; Frystyk, Jan;

    2003-01-01

    OBJECTIVES: Patients with prolactinomas and patients with acromegaly often have heterogenous adenomas. In this study we have focused on patients with prolactinomas who developed acromegaly and acromegalic patients with hyperprolactinaemia. Our hypothesis is that some patients with...... hyperprolactinaemia may develop clinical acromegaly. METHODS: We have included patients examined at department M, Odense University Hospital between 1996 and 2001. Seventy-eight patients with prolactinomas, 65 females and 13 males, with a median age (range) of 30 Years (14-74) and 47 Years (20-66), respectively, were...

  9. 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

    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

  10. Clinical importance of achieving biochemical control with medical therapy in adult patients with acromegaly

    Christofides, Elena A

    2016-01-01

    In acromegaly, achieving biochemical control (growth hormone [GH] level GH and IGF-1 and delay in patients receiving appropriate treatment, which consequently increases mortality risk. In this review, the clinical impact of elevated GH and IGF-1 levels, the effectiveness of current therapies, and the potential role of novel treatments for acromegaly will be discussed. Clinical burden of acromegaly and benefits associated with management of GH and IGF-1 levels will be reviewed. Major treatment paradigms in acromegaly include surgery, medical therapy, and radiotherapy. With medical therapies, such as somatostatin analogs, dopamine agonists, and GH receptor antagonists, a substantial proportion of patients achieve reduced GH and normalized IGF-1 levels. In addition, signs and symptoms, quality of life, and comorbidities have also been reported to improve to varying degrees in patients who achieve biochemical control. Currently, there are several innovative therapies in development to improve patient outcomes, patient use, and access. Timely biochemical control of acromegaly ensures that the patient can ultimately improve morbidity and mortality from this disease and its extensive consequences. PMID:27471378

  11. Long term follow-up of Cushing's disease treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy

    Murayama, Masanori; Yasuda, Keigo; Minamori, Yoshiaki; Mercado-Asis, L.B.; Morita, Hiroyuki; Miura, Kiyoshi (Gifu Univ. (Japan). Faculty of Medicine); Yamakita, Noriyoshi

    1994-02-01

    Subtotal adrenalectomy was given to 10 adult patients with Cushing's disease, concurrently with or following therapeutic regimen by long term reserpine administration and pituitary irradiation. In the present study, we describe long term follow-up results. Two patients died after the operation due to acute adrenal crisis and pneumonia, respectively. The other 8 patients achieved clinical and biochemical remissions and were followed for long term. Three patients relapsed 9, 14 or 17 years after achieving remission, two patients developed hypopituitarism 12 or 20 years after and one died of cerebral vascular accident at 64 years, 5 years after the remission. The remaining 2 patients maintained remission for 10 or 18 years, respectively. During the remission periods of 0.5 to 20 years with a mean of 10.1[+-]6.7 years, 6 of 7 patients examined by 1 mg overnight dexamethasone test showed normal suppressibility of plasma cortisol. Provocative tests of plasma GH by 1-arginine infusion and/or insulin-induced hypoglycemia were performed in 6 patients in the early remission period. All of 5 patients in the arginine infusion test and 3 of 5 in the insulin-induced hypoglycemia test showed normal responses. Furthermore, to facilitate prediction of long term response or failure to our therapeutic regimen, long term reserpine administration and pituitary irradiation, pretreatment clinical and biochemical characteristics were analyzed retrospectively in 3 divided groups; the present 10 patients treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy, 11 patients achieving long term remission treated by our regimen alone, and 7 patients failed with our regimen alone. There were no significant factors predictive of response to our regimen. (author).

  12. Síntomas psicóticos, paranoides y catatónicos como debut de enfermedad de Cushing en una paciente con síndrome de Turner: Reporte de caso y revisión de la litera Psicotic, paranoid and catatonic symptoms as Cushing disease onset in a Turner syndrome patient: Case report and literature revision

    Ray Ticse A

    2012-03-01

    Full Text Available Se conoce que el hipercortisolismo y la sobreactividad del eje hipotalamo pituitario adrenal están asociados a la presentación de enfermedades psiquiátricas. En pacientes con síndrome de Cushing son frecuentes los trastornos del ánimo aunque también se presentan casos de psicosis durante el curso de la enfermedad. Sin embargo es inusual la presencia y más aún el debut con un cuadro de psicosis catatónica. El exceso de cortisol produce daño estructural en el sistema nervioso central tanto reversible como irreversible, especialmente en el hipocampo. Presentamos el caso de una paciente mujer joven con diagnóstico previo de síndrome de Turner, cariotipo 45 X0, quien presentó un cuadro psiquiátrico caracterizado por síntomas depresivos psicóticos y posteriormente catatonia como forma inusual de debut de enfermedad de Cushing y presentó mejoría posterior a la resección de la pituitaria. No se tiene evidencia que el síndrome de Turner influya sobre esta rara forma de presentación.It is known that hipercortisolism and overactivity of the Hypotalamus pituitary adrenal axis are associated to psiquiatric diseases presentation. In patients with Cushingsyndrome, mood disorders are common, also psicosis cases also are present during the disease. But, the presence and mostly the onset with a episode of catatonic psicosis is unusual. The cortisol excess produce structural damage in the Central Nervous System, reversible and irreversible, specially in the hypocampus. We show the case of a young woman with previous diagnostic of Turner syndrome, karyotype 45 X0, who presented a psychiatric episode caracterized for depresive psicotic symptoms and posteriorly a catatonic state as an unusual form of Cushing disease onset. This patient presented health improvement after pituitary resection. There is not evidencia that Turner syndrome influes over this unusual form of presentation.

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

    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. PMID:26955131

  14. Cushing syndrome due to adrenal tumor

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

  15. Acromegaly

    ... women and erectile dysfunction in men. If the tumor affects the part of the pituitary that controls the thyroid—another hormone-producing gland—then thyroid hormones may decrease. Too little thyroid ...

  16. Acromegaly

    ... skin (sebaceous glands), thickening of the skin, skin tags (growths) Sleep apnea Widened fingers or toes, with ... to the principles of the Health on the Net Foundation (www.hon.ch). The information provided herein ...

  17. Cushing's syndrome: Stepwise approach to diagnosis

    Lila, Anurag R.; Sarathi, Vijaya; Jagtap, Varsha S.; Bandgar, Tushar; Menon, Padmavathy; Shah, Nalini S.

    2011-01-01

    The projected prevalence of Cushing's syndrome (CS) inclusive of subclinical cases in the adult population ranges from 0.2–2% and it may no longer be considered as an orphan disease (2–3 cases/million/year). The recognition of CS by physicians is important for early diagnosis and treatment. Late-night salivary cortisol, dexamethasone suppressiontesti, or 24-h urine free cortisol are good screening tests. Positively screened cases need stepwise evaluation by an endocrinologist. This paper discusses the importance of screening for CS and suggests a stepwise diagnostic approach to a case of suspected hypercortisolism. PMID:22145134

  18. The Modern Criteria for Medical Management of Acromegaly.

    Frara, Stefano; Maffezzoni, Filippo; Mazziotti, Gherardo; Giustina, Andrea

    2016-01-01

    Acromegaly is an insidious disorder characterized by excess secretion of growth hormone (GH) and elevated circulating levels of insulin-like growth factor-I (IGF-I), generally caused by a pituitary adenoma. It is a rare disease associated with an average 10-year reduction in life expectancy due to metabolic, cardiovascular, and cerebrovascular comorbidities and reduced quality of life caused by paresthesias, fatigue, osteoarthralgia, or bone fractures. In 2000, Cortina Consensus Conference established general criteria for diagnosis and biochemical control of acromegaly, which have been revised in recent years, adapting them to emerging clinical evidences as well as the evolving assay techniques. Authors have proposed a binary definition of cure for acromegaly, where both GH and IGF-I are important determinants: the former is more linked to the presence of residual adenomatous tissue, while the latter to the peripheral activity of the disease. Control of tumor growth and complications is also an essential goal of treatment. Surgical, medical, and radiotherapy approaches are all valid alternatives. The surgical option is, however, unsuccessful in about 50% of patients. Somatostatin analogs (SRLs), octreotide LAR, and lanreotide ATG can inhibit cell growth, besides their beneficial effects on GH hypersecretion and on most comorbidities. Pasireotide is a new multireceptor-targeted SRL with reported superior biochemical efficacy to octreotide, due to higher affinity for SSTR-5, but potentially causing detrimental effects on glucose homeostasis. Pegvisomant could be a valid choice in all patients resistant to SRLs. It is a competitive GH antagonist, which efficaciously blocks IGF-I production, inhibiting the dimerization of GH receptor. Normal IGF-I levels represent, therefore, its only relevant efficacy endpoint, while only few cases of tumor growth on pegvisomant have been reported, so far. PMID:26940387

  19. Study to Allow Access to Pasireotide for Patients Benefiting From Pasireotide Treatment in a Novartis-sponsored Study.

    2016-02-28

    Cushing's Disease,; Acromegaly,; Neuroendocrine Tumors,; Pituitary Tumors; Ectopic ACTH Secreting (EAS) Tumors,; Dumping Syndrome,; Prostate Cancer,; Melanoma Negative for bRAF,; Melanoma Negative for nRAS

  20. Genetic Predictors of Response to Different Medical Therapies in Acromegaly.

    Ramos-Leví, Ana M; Bernabeu, Ignacio; Sampedro-Núñez, Miguel; Marazuela, Mónica

    2016-01-01

    In the era of predictive medicine, management of diseases is evolving into a more personal and individualized approach, as more data are available regarding clinical, biochemical, radiological, molecular, histopathological, and genetic aspects. In the particular setting of acromegaly, which is a rare, chronic, debilitating, and disfiguring disease, an optimized approach deems even more necessary, especially because of an associated increased morbidity and mortality, the impact on patients' quality of life, and the increased cost of frequently necessary life-long treatments. In this paper, we review the available studies that address potential genetic influences on acromegaly, their role in the outcome, and response to treatments, as well as their contribution to the risk of developing side effects. We focus mainly on pharmacogenetic factors involved during treatment with dopamine agonists, somatostatin analogs, and pegvisomant. Specifically, mutations in dopamine receptors, somatostatin receptors, growth hormone receptors, and metabolic pathways involved in growth hormone action; polymorphisms in the insulin-like growth factor and the insulin-like growth factor binding proteins; and polymorphisms in other genes that may determine differences in the frequency of developing adverse events. PMID:26940388

  1. Sequential growth hormone deficiency and acromegaly.

    Heffernan, A.

    1988-01-01

    This is the case of a patient with a pituitary tumour presenting initially with growth hormone deficiency and requiring treatment with human growth hormone. Eight years later he represented with acromegaly. This sequence of events has not to my knowledge been reported previously.

  2. Growth Hormone and Insulin Signaling in Acromegaly

    Dal, Jakob; Lundby Høyer, Katrine; Pedersen, Steen Bønløkke;

    2016-01-01

    CONTEXT: Somatostatin analogues (SA) used in acromegaly to suppress GH secretion and tumor growth also suppress insulin secretion and may impact GH signaling. OBJECTIVE: To compare GH and insulin signaling after intravenous GH exposure in acromegalic patients controlled by surgery (n=9) or SA (n=...

  3. Treating Cushing's Disease in Dogs

    ... Emiten Radiación Fraude en la Salud Medicamentos Nutrición Productos Veterinarios Productos de Tabaco Salud Infantil Salud de la Mujer Suplementos Dietéticos Vacunas, Sangre y Productos Biológicos Resources for You Sign up for Consumer ...

  4. Insulin action in human adipose tissue in acromegaly.

    Bolinder, J.; Ostman, J; Werner, S.; Arner, P.

    1986-01-01

    The mechanisms underlying insulin resistance in acromegaly were investigated. Adipose tissue was obtained from nine patients with acromegaly who had in vivo insulin resistance and from 14 matched healthy control subjects. Receptor binding and the antilipolytic effect of insulin were determined in isolated fat cells. Insulin-induced glucose oxidation at a physiological hexose concentration was investigated in fat segments. In fat cells obtained from acromegaly patients after an overnight fast,...

  5. [Cushing's ulcer in a child (apropos of a case)].

    Vergos, M; Ribault, L; Coullet, Y

    1988-01-01

    Cushing's ulcus is an uncommon disease occurring during the regression of acute neurologic diseases. It counts for 30 p.c. of causes of acute digestive hemorrhages in children. Prognosis is very bad and any surgical treatment has to be as preservative as possible, classically achieving a hemostatic ligation associated with a pyloroplasty and a truncal vagotomy. Mortality rate is 20 p.c. PMID:3367768

  6. Pseudo-Cushing - A Clinical Challenge?

    Pecori Giraldi, Francesca; Ambrogio, Alberto G

    2016-01-01

    The distinction between Cushing's syndrome and pseudo-Cushing is a major clinical challenge. Indeed, any endocrinologist used to dealing with Cushing's syndrome has certainly faced this dilemma more than once and is aware that there are no clear-cut solutions. Several factors contribute to this ongoing quandary, such as unbalanced epidemiology, overlap in clinical features and inherent variability in test responses. Thus, extreme care has to be taken in both excluding and confirming Cushing's syndrome in patients with mild clinical features and borderline laboratory alterations. PMID:27211794

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

    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)

  8. Acromegaly and diabetes mellitus associated with hyperthyroidism.

    Deng, Datong; Luo, Li; Chen, Mingwei; Xu, Min; Wang, Youmin

    2014-01-01

    A 66-year-old woman with acromegaly and diabetes mellitus as well as primary hyperthyroidism is described. Serum GH Levels were inappprpriately high.MRI revealed an enlarged sella turcica with intrasellar mass. Her HbAlc was 12.2% and fasting blood glucose 8.89 mmol/l. Thyroid hormone levels in serum and thyroidal radioiodine uptake values were elevated, while TSH measurements in serum were low. Anti TPO antibodies were negative, TSH receptor antibodies were normal. Thyrotoxicosis as the first presenting illness in acromegaly was particulary uncommon. An ultrasound thyroid scan showed a multinodular goiter. Histology of the pituitary lesion showed a typical eosinophilic adenoma which only secreted GH when tested with specific immunostain. Post-operatively, the patient's clinical conditions improved, however, secondary hypoadrenalism appeared. PMID:24977962

  9. MRI of pituitary adenomas in acromegaly

    Adenomas causing acromegaly represent at least a quarter of pituitary adenomas. We studied 12 patients presenting with active acromegaly due to a pituitary adenoma with a 1.5 T superconductive MRI unit. All had T1-weighted sagittal and coronal sections before and after Gd-DTPA; six had coronal T2-weighted images. Surgical correlation was obtained in seven patients. Histologically, there were eight growth hormone (GH)-secreting and three mixed [GH and prolactin (PRL) secreting[ adenomas, and one secreting GH, PRL and follicle-stimulating hormone. Macroadenomas (10) were more frequent than microadenomas (2). No correlation was found between serum GH and tumour size. There were nine adenomas in the lateral part of the pituitary gland; seven showed lateral or infrasellar invasion. Homogeneous, isointense signal on T1-and T2-weighted images was observed in six cases. Heterogeneous adenomas had cystic or necrotic components. (orig.). With 5 figs., 3 tabs

  10. AIP mutations in young patients with acromegaly and the Tampico Giant: the Mexican experience.

    Ramírez-Rentería, Claudia; Hernández-Ramírez, Laura C; Portocarrero-Ortiz, Lesly; Vargas, Guadalupe; Melgar, Virgilio; Espinosa, Etual; Espinosa-de-Los-Monteros, Ana Laura; Sosa, Ernesto; González, Baldomero; Zúñiga, Sergio; Unterländer, Martina; Burger, Joachim; Stals, Karen; Bussell, Anne-Marie; Ellard, Sian; Dang, Mary; Iacovazzo, Donato; Kapur, Sonal; Gabrovska, Plamena; Radian, Serban; Roncaroli, Federico; Korbonits, Márta; Mercado, Moisés

    2016-08-01

    Although aryl hydrocarbon receptor-interacting protein (AIP) mutations are rare in sporadic acromegaly, their prevalence among young patients is nonnegligible. The objectives of this study were to evaluate the frequency of AIP mutations in a cohort of Mexican patients with acromegaly with disease onset before the age of 30 and to search for molecular abnormalities in the AIP gene in teeth obtained from the "Tampico Giant". Peripheral blood DNA from 71 patients with acromegaly (51 females) with disease onset age of disease onset of 23 years) and correlated with clinical, biochemical and imaging characteristics. Sequencing was also carried out in DNA extracted from teeth of the Tampico Giant. Five patients (7 %) harboured heterozygous, germline mutations of the AIP gene. In two of them (a 9-year-old girl with gigantism and a young man with symptoms of GH excess since age 14) the c.910C>T (p.Arg304Ter), well-known truncating mutation was identified; in one of these two cases and her identical twin sister, the mutation proved to be a de novo event, since neither of their parents were found to be carriers. In the remaining three patients, new mutations were identified: a frameshift mutation (c.976_977insC, p.Gly326AfsTer), an in-frame deletion (c.872_877del, p.Val291_Leu292del) and a nonsense mutation (c.868A > T, p.Lys290Ter), which are predicted to be pathogenic based on in silico analysis. Patients with AIP mutations tended to have an earlier onset of acromegaly and harboured larger and more invasive tumours. A previously described genetic variant of unknown significance (c.869C > T, p.Ala299Val) was identified in DNA from the Tampico Giant. The prevalence of AIP mutations in young Mexican patients with acromegaly is similar to that of European cohorts. Our results support the need for genetic evaluation of patients with early onset acromegaly. PMID:27033541

  11. Complications of Cushing's syndrome: state of the art.

    Pivonello, Rosario; Isidori, Andrea M; De Martino, Maria Cristina; Newell-Price, John; Biller, Beverly M K; Colao, Annamaria

    2016-07-01

    Cushing's syndrome is a serious endocrine disease caused by chronic, autonomous, and excessive secretion of cortisol. The syndrome is associated with increased mortality and impaired quality of life because of the occurrence of comorbidities. These clinical complications include metabolic syndrome, consisting of systemic arterial hypertension, visceral obesity, impairment of glucose metabolism, and dyslipidaemia; musculoskeletal disorders, such as myopathy, osteoporosis, and skeletal fractures; neuropsychiatric disorders, such as impairment of cognitive function, depression, or mania; impairment of reproductive and sexual function; and dermatological manifestations, mainly represented by acne, hirsutism, and alopecia. Hypertension in patients with Cushing's syndrome has a multifactorial pathogenesis and contributes to the increased risk for myocardial infarction, cardiac failure, or stroke, which are the most common causes of death; risks of these outcomes are exacerbated by a prothrombotic diathesis and hypokalaemia. Neuropsychiatric disorders can be responsible for suicide. Immune disorders are common; immunosuppression during active disease causes susceptibility to infections, possibly complicated by sepsis, an important cause of death, whereas immune rebound after disease remission can exacerbate underlying autoimmune diseases. Prompt treatment of cortisol excess and specific treatments of comorbidities are crucial to prevent serious clinical complications and reduce the mortality associated with Cushing's syndrome. PMID:27177728

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

    Measurement of 75Se-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. The Role of Radiotherapy in Acromegaly.

    Hannon, Mark J; Barkan, Ariel L; Drake, William M

    2016-01-01

    Radiotherapy has, historically, played a central role in the management of acromegaly, and the last 30 years have seen substantial improvements in the technology used in the delivery of radiation therapy. More recently, the introduction of highly targeted radiotherapy, or 'radiosurgery', has further increased the therapeutic options available in the management of secretory pituitary tumors. Despite these developments, improvements in primary surgical outcomes, an increase in the range and effectiveness of medical therapy options, and long-term safety concerns have combined to dictate that, although still deployed in selected cases, the use of radiotherapy in the management of acromegaly has declined steadily over the past 2 decades. In this article, we review some of the main studies that have documented the efficacy of pituitary radiotherapy on growth hormone hypersecretion and summarize the data around its potential deleterious effects, including hypopituitarism, cranial nerve damage, and the development of radiation-related intracerebral tumors. We also give practical recommendations to guide its future use in patients with acromegaly, generally, as a third-line intervention after neurosurgical intervention in combination with various medical therapy options. PMID:26088716

  14. Depression in acromegaly treated with escitalopram and cognitive therapy

    De Sousa Avinash

    2009-01-01

    Full Text Available Depression is one of the commonest disorders encountered in general hospital psychiatry. Acromegaly is a condition with excessive growth hormone secretion that may at times present with oversychopathology. We present the case of a 33-year-old lady with depression and acromegaly that successfully resolved after treatment with escitalopram and cognitive therapy.

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

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

    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)

  17. Prevalence of colonic polyp and its predictors in patients with acromegaly

    Purnima Agarwal

    2016-01-01

    Full Text Available Background: There are conflicting data regarding the prevalence of colorectal polyp in patients with acromegaly. Subjects and Methods: Consecutive forty-seven acromegalic patients (21 men, 26 women, with a mean age of (40 ± 12 years attending endocrinology outpatient department underwent full colonoscopy. All the patients underwent clinical and biochemical evaluation (glucose suppressed growth hormone (GH, Insulin-like growth factor-1 [IGF-1], fasting insulin, and glucose. The control group (n = 120 for colonoscopy was adult subjects undergoing evaluation for symptoms of irritable bowel syndrome. Clinical and biochemical parameters in acromegalic patients with colonic polyp were compared to those without a polyp. Results: Patients with acromegaly had significantly higher prevalence of colonic polyp as compared to control subjects (10.6% vs. 0.8%. None of the patients with polyp had skin tags. There was no significant difference between subjects with and without colonic polyp in duration of illness, basal, and glucose-suppressed GH and most recent IGF-1. Fasting blood sugar was significantly higher (P < 0.05 in adenoma group after adjusting for age, body mass index (BMI, and insulin levels. Patients in adenoma group showed a trend toward male gender and younger age as compared to those without adenoma. Conclusions: Subjects with acromegaly as compared to control have a higher prevalence of colonic polyps. There was no association of polyps seen with age, BMI, skin tags, homeostasis model assessment of insulin resistance index, duration of disease, and basal and glucose-suppressed GH and IGF-1 levels. There were no specific predictive factors detected. Screening full colonoscopy is recommended in all cases with acromegaly.

  18. ontaneous Remission of Acromegaly Due to Apoplexy

    Cevdet Duran

    2008-01-01

    Full Text Available Pituitary apoplexy is a rare clinical syndrome characterized by sudden onset of headache and vomiting, accompanied by visual disturbance or ocular motility impairment and pituitary insufficiency. Pituitary apoplexy presenting with these symptoms is seen in approximately 3% of patients with surgically treated pituitary adenomas. In acromegalic patients, apoplexy can be related to some provocating factors and rarely may be spontaneous. We present the case of an acromegalic patient with spontaneous remission after apoplexy. A 39-year-old male patient had transsphenoidal microsurgery for acromegaly in 1994 but remission was not achieved. After a 10-year period without follow-up, he experienced severe headache, nausea, vomiting, photophobia, and visual disturbance in July 2005 and scheduled an appointment with an ophthalmologist. His complaints ceased spontaneously within weeks. In October 2005, sellar MRI examination revealed a 3x2.5 cm mass of adenomatous tissue with necrosis. On admission to our clinic in November 2005, visual acuity, visual fields, and ocular motility, were normal; but results of laboratory tests revealed panhypopituitarism. He was thought to have apoplexy of a growth hormone secreting tumor, and hormone replacement therapy was initiated for panhypopituitarism. Because his symptoms had regressed, surgery was not considered. On rare occasions, acromegaly spontaneously remits after apoplexy, resulting in improved control of the functional adenoma without surgical intervention. Turk Jem 2008; 12: 80-2

  19. Acute exacerbation of upper airway obstruction in acromegaly

    Rees, P J; Hay, J G; Webb, J R

    1982-01-01

    A 71-year-old man with acromegaly is described. Investigations for dyspnoea and daytime somnolence showed upper airway obstruction but not sleep apnoea. An upper respiratory tract infection precipitated stridor which required urgent tracheostomy.

  20. Arthropathie destructrice des epaules au cours d�une acromegalie

    Nessrine Akasbi

    2011-10-01

    Full Text Available L�acromegalie est une maladie endocrinienne rare, en rapport avec une hypersecretion d�hormone de croissance. Elle a des consequences rhumatologiques: l�arthropathie peripherique, l�atteinte rachidienne et les syndromes canalaires. L�atteinte articulaire accompagne une acromegalie active, sa survenue apres un traitement radical et une remission complete est rare. Nous presentons le cas d�une patiente de 70 ans ayant un antecedent d�acromegalie sur adenome hypophysaire il y a 25 ans, traitee chirurgicalement et declaree en remission complete, a developpe une arthropathie destructrice des deux epaules. Le but de notre observation est de mettre le point sur la possibilite d�une atteinte articulaire au cours de l�acromegalie et de son retentissement fonctionnelle.

  1. Cushing's ulcer in traumatic brain injury

    Biteghe-bi-Nzeng Alain; WANG Yun-jie

    2008-01-01

    Traumatic brain injury(TBI)remains a complicated and urgent disease in our modernized cities. It becomes now a public health disease. We have got more and more patients in Neurosurgery Intensive Care Unit following motor vehicle accidents and others causes. TBI brings multiple disorders,from the primary injury to secondary injury. The body received the disturbances in the brain,in the hypothalamo-pituitary-adrenocortical(HPA)axis,in the gastric mucosa,in the immune and neuroendocrine systems.The mortality of TBI is more than 50 000 deaths/year, the third of the mortality of all iniuries. Cushing ulcer is one of the severe complications of TBI and its mortality rate is more than 50%. Many studies have improved the management of TBI and the associated complications to give patients a better outcome. Furthers studies need to be done based on the similar methodology to clarify the different steps of the HPA axis and the neuroendocrine change associated. The aim of the present review is to assess the clinical and endocrinal features of hypopituitarism and stress ulcer following TBI.

  2. Gene Expression Signature in Adipose Tissue of Acromegaly Patients

    Hochberg, Irit; Tran, Quynh T; Barkan, Ariel L.; Saltiel, Alan R.; Chandler, William F.; Bridges, Dave

    2015-01-01

    To study the effect of chronic excess growth hormone on adipose tissue, we performed RNA sequencing in adipose tissue biopsies from patients with acromegaly (n = 7) or non-functioning pituitary adenomas (n = 11). The patients underwent clinical and metabolic profiling including assessment of HOMA-IR. Explants of adipose tissue were assayed ex vivo for lipolysis and ceramide levels. Patients with acromegaly had higher glucose, higher insulin levels and higher HOMA-IR score. We observed several...

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

    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)

  4. Bilateral adrenalectomy for Cushing's syndrome: Pros and cons

    O P Prajapati

    2015-01-01

    Full Text Available Aim: To assess the outcome of patients undergoing bilateral adrenalectomy for Cushing's syndrome (CS. Methods: All patients who underwent bilateral adrenalectomy for CS at the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences hospital between 1991 and 2013 were included. Medical records were reviewed to obtain patient characteristics and follow-up data. Results: Twenty-seven patients were studied. Mean age was 28.74 ± 12.95 years (range 9–60, male:female ratio was 1.7:1. About half that is, 48.19% were of Cushing's disease (failed trans-sphenoidal surgery [TSS], 37.04% were of ectopic CS (ECS, and 14.81% were of CS due to bilateral adrenal pathology. Median follow-up period was 80.5 months. Before surgery, 74.1% patients had body mass index > which after surgery declined to <25 in 75% of them. Hypertension was present in 85.2% and after surgery resolved in 40%. Diabetes mellitus was present in 44.4% and after surgery resolved in 33% of them. Hirsutism and proximal muscle weakness were present in 55.6% and 70.4% patients, respectively, and after surgery improved markedly in all patients. Adrenal crisis developed in 36.3% and Nelson's syndrome in 41.7% patients during follow-up. Three patients died in perioperative period while three succumbed to the disease during follow-up. Two patients developed recurrence of endogenous cortisol production during the follow-up period. Conclusions: Bilateral adrenalectomy is a valid treatment option for palliating severe symptoms in Pituitary Cushing's with failed TSS and unlocalized ECS but the procedure is curative for CS due to bilateral adrenal disease. Overall morbidity and mortality is higher than other endocrine operations. Co-morbidities tend to be more severe and are a risk factor for mortality during the time patient survives.

  5. Synchronous Acromegaly and Gastrointestinal Stromal Tumor: A Case Report

    Hüsniye Başer

    2014-06-01

    Full Text Available Acromegaly is a rare endocrine disorder characterized by the manifestations of sustained hypersecretion of growth hormone and concomitant elevations in circulating concentrations of insulin-like growth factor-1. It has been reported that patients with acromegaly are at the increased risk of developing malignant tumors, particularly colorectal cancer. Gastrointestinal stromal tumors are mesenchymal tumors of the digestive tract. An association between gastrointestinal stromal tumors and insulin-like growth factor system has been reported. Here, we report a patient diagnosed with synchronous acromegaly and gastrointestinal stromal tumor. A 59-year-old man with iron deficiency anemia presented with enlarged hands, coarse facial feature and several skin tags. Thyroid function tests were within normal range. Growth hormone was 5.14 ng/mL, insulin-like growth factor-1 was 820 ng/mL, and no growth hormone suppression was observed on 75g oral glucose tolerance test. Pituitary magnetic resonance imaging revealed microadenoma, and the patient was diagnosed with acromegaly. Upper gastrointestinal tract endoscopy revealed an ulcerovegetan mass in the duodenum and the results of the histopathologcal analysis was consistent with gastrointestinal stromal tumor. The association of synchronous and asynchronous gastrointestinal stromal tumors with other malignancies have been reported. The most common accompanying neoplasms are colorectal and gastric adenocarcinomas, as well as pancreatic tumors. However, in the literature, the number of reported cases of synchronous acromegaly and gastrointestinal stromal tumor are limited, and there are no sufficient data on this association. Turk Jem 2014; 2: 52-55

  6. A specific nursing educational program in patients with Cushing's syndrome.

    Martínez-Momblán, M Antonia; Gómez, Carmen; Santos, Alicia; Porta, Nuria; Esteve, Julia; Úbeda, Inmaculada; Halperin, Irene; Campillo, Beatriz; Guillaumet, Montserrat; Webb, Susan M; Resmini, Eugenia

    2016-07-01

    Cushing's syndrome (CS) is a rare endocrine disease, due to cortisol hypersecretion. CS patients have comorbidities, often still present after biochemical cure. Specific nursing healthcare programs to address this disease and achieve improved health related quality of life (HRQoL) are lacking. Thus, an educational nursing intervention, through the development and promotion of specific educational tools, appears to be justified. The objective of this study is to assess the effectiveness of an educational nursing program in CS patients on HRQoL, clinical parameters, level of pain and physical activity, patterns of rest, and use of health resources. A prospective, randomized study was conducted in two reference hospitals for CS. Sixty-one patients (mean age 47 ± 12.7 years, 83.6 % females) were enrolled and divided into 2 groups: an "intervention" group where educational sessions were performed over 9 months and a "control" group, without these sessions. Specific questionnaires were used at the beginning and end of the study. After educational sessions, the intervention group had a better score in the CushingQoL questionnaire (p physical activity (p physical activity (r = 0.89, p physical activity, healthy lifestyle, better sleep patterns, and reduced pain in CS patients, influencing HRQoL and reducing consumption of health resources. Moreover, the brief nature of the program suggests it as a good candidate to be used in CS patients. PMID:26400845

  7. Cardiac effects of 3 months treatment of acromegaly evaluated by magnetic resonance imaging and B-type natriuretic peptides

    Andreassen, Mikkel; Faber, Jens Oscar; Kjær, Andreas;

    2010-01-01

    acromegaly is initiated. This was a three months prospective study investigating short-term cardiac effects of treatment in acromegalic patients. Cardiac function was evaluated by the gold standard method cardiac magnetic resonance imaging (CMRI) and circulating levels of B-type natriuretic peptides (BNP and......Long-term treatment of acromegaly prevents aggravation and reverses associated heart disease. A previous study has shown a temporary increase in serum levels of the N-terminal fraction of pro B-type natriuretic peptide (NT-proBNP) suggesting an initial decline in cardiac function when treatment of...... increase in EDVI, and increased levels of BNP and NT-proBNP suggesting an initial decrease in cardiac function....

  8. Results of Gamma Knife Radiosurgery in Acromegaly

    Alberto Franzin

    2012-01-01

    Full Text Available Objective. Single-session radiosurgery with Gamma Knife (GK may be a potential adjuvant treatment in acromegaly. We analyzed the safety and efficacy of GK in patients who had previously received maximal surgical debulking at our hospital. Methods. The study was a retrospective analysis of hormonal, radiological, and ophthalmologic data collected in a predefined protocol from 1994 to 2009. The mean age at treatment was 42.3 years (range 22–67 yy. 103 acromegalic patients participated in the study. The median follow-up was 71 months (IQ range 43–107. All patients were treated with GK for residual or recurrent GH-secreting adenoma. Results. Sixty-three patients (61.2% reached the main outcome of the study. The rate of remission was 58.3% at 5 years (95% CI 47.6–69.0%. Other 15 patients (14.6% were in remission after GK while on treatment with somatostatin analogues. No serious side effects occurred after GK. Eight patients (7.8% experienced a new deficit of pituitary function. New cases of hypogonadism, hypothyroidism, and hypoadrenalism occurred in 4 of 77 patients (5.2%, 3 of 95 patients (3.2%, and 6 of 100 patients at risk (6.0%, respectively. Conclusion. In a highly selected group of acromegalic patients, GK treatment had good efficacy and safety.

  9. A rare cause of Cushing's syndrome

    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...

  10. Cardiovascular autonomic function in Cushing's syndrome.

    Fallo, F; Maffei, P; Dalla Pozza, A; Carli, M; Della Mea, P; Lupia, M; Rabbia, F; Sonino, N

    2009-01-01

    Cardiac autonomic dysfunction is associated with increased cardiovascular mortality. No data on sympathovagal balance are available in patients with Cushing's syndrome, in whom cardiovascular risk is high. We studied 10 patients with newly diagnosed Cushing's syndrome (1 male/9 females; age mean+/-SD, 47+/-10 yr) and 10 control subjects matched for age, sex, body mass index, and cardiovascular risk factors. In both groups there were 7 patients with arterial hypertension, 3 with diabetes mellitus, and 2 with obesity. Cardiac autonomic function was evaluated by analysis of short time heart rate variability (HRV) measures in frequency domain over 24-h, daytime, and nighttime. The 24-h ambulatory blood pressure monitoring and echocardiography were also performed. In comparison with controls, patients with Cushing's syndrome had lower 24-h (1.3+/-0.6 vs 3.7+/-1.5, mean+/-SD, p<0.01), daytime (2.0+/-1.4 vs 4.5+/-1.6, p<0.01), and night-time (1.0+/-0.4 vs 3.5+/-2.3, p<0.01) low-frequency/ high frequency (LF/HF) power ratio. In the presence of similar LF power, the difference was due to elevation in HF power in Cushing's syndrome compared to controls: 24-h, 12.7+/-6.7 vs 5.8+/-2.8, p<0.01; daytime, 10.2+/-7.3 vs 4.5+/-2.1, p<0.05; nighttime, 14.2+/-7.0 vs 7.8+/-4.7, p<0.05. Eight Cushing patients vs 4 controls had a non-dipping blood pressure profile. At echocardiography, Cushing patients had a greater left ventricular mass index and/or relative wall thickness, and impaired diastolic function, compared with controls. Compared to controls, patients with Cushing's syndrome showed a sympathovagal imbalance, characterized by a relatively increased parasympathetic activity. Whether this autonomic alteration is meant to counterbalance cortisol-induced effects on blood pressure and cardiac structure/function or has a different pathophysiological significance is still unknown. PMID:19337014

  11. Evaluation of proopiomelanocortin mRNA in the peripheral blood from patients with Cushing's syndrome of different origin.

    Bondioni, S; Mantovani, G; Polentarutti, N; Ambrosi, B; Loli, P; Peverelli, E; Lania, A G; Beck-Peccoz, P; Spada, A

    2007-11-01

    ACTH-dependent Cushing's syndrome is due to ACTH overproduction originating from a pituitary corticotroph adenoma (Cushing's disease) or from ectopic tumors (ectopic ACTH syndrome). Due to difficulties in the differential diagnosis between these two forms of hypercortisolism it would be important to have molecular tools able to discriminate the two conditions. It is known that proopiomelanocortin (POMC) gene transcription can originate messengers of different length. ACTHomas show the normal 1072 nucleotides (nt) transcript, whereas ectopic tumors seem to be associated with a longer mRNA form (1450 nt). In order to analyse the presence of different POMC transcripts, we extracted total RNA from peripheral lymphocytes of 10 patients with Cushing's disease, 10 with ectopic Cushing syndrome, and 20 controls as well as from pituitary tissues (2 ACTH-omas and a normal pituitary polyA+ sample). Northern blot analysis correctly revealed a 1072 nt mRNA molecule in pituitary ACTH-oma and in the normal pituitary polyA+ RNA samples, whereas neither this molecule nor other alternative transcripts were detected in blood samples from patients and controls. These data were confirmed by the more sensitive RT-PCR technique. This study further underlines the need for alternative approaches in the diagnosis of ACTH-dependent Cushing's syndrome. PMID:18075284

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

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

    2016-03-01

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

  13. Pregnancy in acromegaly patients treated with pegvisomant.

    van der Lely, A J; Gomez, Roy; Heissler, Joseph F; Åkerblad, Ann-Charlotte; Jönsson, Peter; Camacho-Hübner, Cecilia; Kołtowska-Häggström, Maria

    2015-08-01

    To summarize all available data on pregnancy outcome of acromegaly patients exposed to the growth hormone receptor antagonist pegvisomant (PEGV) during pregnancy as present in the Pfizer's Global Safety Database. Pfizer's Global Safety Database contains adverse event data obtained from the following sources: spontaneous reports, health authorities, Pfizer-sponsored post-marketing surveillance program (ACROSTUDY), customer engagement programs, and clinical studies, reported regardless of outcome. The safety database was searched up to 10th March 2014. From the 35 pregnancy cases, 27 involved maternal [mean age (range) 33.3 years (23-41) and 8 paternal (33.7 years (32-38)] PEGV exposure. Two female patients were reported with two pregnancy cases each. Fetal outcome was normal in 14 (4 paternal) of the 18 reported as live birth, while 4 cases (1 paternal) did not specify the birth outcome. At conception, PEGV mean dose (range) was 15.3 mg/d (4.3-30). In 3 cases of maternal exposure of the 18 cases reporting live birth, PEGV was continued throughout the pregnancy in a dose of 12.1 mg/d (10-15). In 5 cases (all maternal) an elective termination of the pregnancy was performed with no reported fetal abnormalities, 2 cases (maternal) reported a non-PEGV-related spontaneous abortion and in 1 maternal case an ectopic pregnancy occurred. In 9 cases (3 paternal), the fetal outcome was not reported. Three women reported gestational diabetes; one woman continued PEGV treatment during pregnancy. Although the number of reported pregnancies with exposure to PEGV is very small, the presented data reflect the largest series of data available to date and do not suggest adverse consequences of PEGV on pregnancy outcome. Nevertheless, it should be stressed that PEGV should not be used during pregnancy unless absolutely necessary. PMID:25542184

  14. Post-operative Streptococcus pneumoniae meningoencephalitis complicating surgery for acromegaly in an identical twin.

    Cote, David J; Iuliano, Sherry L; Smith, Timothy R; Laws, Edward R

    2015-06-01

    This case report provides provocative and useful data regarding two aspects of acromegaly and its management. The patient, who is one of a pair of identical twins, has no known hereditary, genetic or otherwise potentially etiologic factors as compared to her unaffected sister. Secondly, transsphenoidal surgery, which was ultimately successful, was complicated by pneumococcal meningitis, an unusual event with only four previously reported patients, three of whom ended in death or major neurologic deficits. In this case, a 57-year-old woman gradually developed classical signs and symptoms of acromegaly while her identical twin sister remained normal with no evidence of endocrine disease. Endoscopic transsphenoidal surgery was complicated by the development of meningitis 25 days after surgery. This was controlled following a difficult hospital course. Streptococcus pneumoniae meningoencephalitis is a rare but life-threatening complication of transsphenoidal surgery. A high index of suspicion for incipient meningitis should be maintained when patients present with severe headache and increased intracranial pressure, even if they initially lack the typical symptoms and signs. Immediate and aggressive treatment is necessary to avoid significant neurologic deficit. PMID:25861890

  15. Síndrome de Cushing e Gravidez

    Agapito, A; Afonso, A.; Fonseca, F; Neves, A.; Revez, A; Leite da Silva; Charneco da Costa, J

    1986-01-01

    Descreve-se um caso de gravidez em doente com Síndrome de Cushing por adenoma da supra-renal, tratado cirurgicamente após o parto e com recuperação total do eixo hipotálamo-hipófise-supra-renal. A gravidez teve evolução favorável, com parto pré-termo e recém-nascido normal.

  16. Computed tomography of adrenal Cushing's adenoma

    CT findings of 22 patients with surgically confirmed adrenal Cushing's adenomas were compared with pathologic findings. The cut surfaces of the adenomas showed mixture of yellow and brown areas in various proportions and were classified into three patterns; speckled brown areas in yellow background, geometrically brown areas in yellow background, totally brown or black surface. The maximum diameters of the cut surfaces were measured. The CT appearances of Cushing's adenomas after intravenous contrast administration have various patterns of enhancement and classified into three patterns; speckled, geometrical, and homoenous. The maximum diameters of the adenomas in CT images were also measured. Correlation between the CT and gross appearances of the specimens showed that while brown areas in adenomas were strongly enhanced, yellow areas were poorly enhanced. Histologically, brown areas in adenomas consist of compact-like cells with rich intercellular space and yellow areas consist of clear-like cells with poor intercellular space. The patterns of contrast enhancement some to depend on the cell types of adenomas. Difference in the intercellular space between compact and clear-like cells may have altered the patterns of contrast enhancement. The study also revealed that predominantly brownish adenomas were smaller in size than predominantly yellowish ones. As compact-like cells which make up the brown areas in Cushing's adenoma are thought to be more active in producing and secreting steroid hormones than clear-like cells, this result suggests that clinical symptoms may appear earlier in predominantly brownish adenomas than in predominantly yellowish ones. (author)

  17. Pituitary tumors containing cholecystokinin

    Rehfeld, J F; Lindholm, J; Andersen, B N;

    1987-01-01

    We found small amounts of cholecystokinin in the normal human adenohypophysis and therefore examined pituitary tumors from 87 patients with acromegaly, Cushing's disease, Nelson's syndrome, prolactinoma, or inactive pituitary adenomas. Five adenomas associated with Nelson's syndrome contained...

  18. Surgical management of acromegaly in a resource-challenged environment

    Idowu Olufemi Emmanuel

    2015-01-01

    Full Text Available The management of acromegaly caused by an uncommon growth hormone-secreting pituitary adenoma can be challenging in low-resource African subregion. We conducted a study over a 2-year period to describe the results and challenges following surgical treatment of this rare condition in our centre. The clinical outcome was defined as successful based on the surgeon′s intraoperative observation, postoperative neuroimaging findings and neuroendocrinological results. A total of three patients (two males and one female aged 19-32 years were included. Visual impairment was the main presenting symptom in all the three patients. The postoperative period was uneventful. Acromegaly is an uncommon disorder in our region. Surgery is the treatment of choice in low-resource practice.

  19. Combined treatment of somatostatin analogues with pegvisomant in acromegaly.

    Franck, S E; Muhammad, A; van der Lely, A J; Neggers, S J C M M

    2016-05-01

    Treatment of acromegaly with monotherapy long-acting somatostatin analogues (LA-SSA) as primary treatment or after neurosurgery can only achieve complete normalization of insulin-like growth factor I (IGF-I) in roughly 40 % of patients. Recently, one of the acromegaly consensus groups has recommended switching to combined treatment of LA-SSA and pegvisomant (PEGV) in patients with partial response to LA-SSAs. This combination of LA-SSA and PEGV, a growth hormone receptor antagonist, can normalize IGF-I levels in virtually all patients, requiring that the adequate dose of PEGV is used. The required PEGV dose varies significantly between individual acromegaly patients. One of the advantages of the combination therapy is that tumor size control or even tumor shrinkage can be observed in a vast majority of patients. The main side effects of the combination treatment are gastrointestinal symptoms, lipohypertrophy and transient elevated liver transaminases. In this review we provide an overview of the efficacy and safety of the combined treatment of LA-SSAs with PEGV. PMID:26661938

  20. Cushing's syndrome masquerading as treatment resistant depression

    B N Anil Kumar

    2016-01-01

    Full Text Available Treatment resistant depression (TRD is a common clinical occurrence among patients treated for major depressive disorder. A significant proportion of patients remain significantly depressed in spite of aggressive pharmacological and psychotherapeutic approaches. Management of patient with treatment resistant depression requires thorough evaluation for physical causes. We report a case of recurrent depressive disorder, who presented with severe depressive episode without psychotic symptoms, not responding to multiple adequate trials of antidepressants, who on investigation was found to have Cushing's syndrome and responded well to Ketoconazole.

  1. Cushing's Syndrome Masquerading as Treatment Resistant Depression.

    Anil Kumar, B N; Grover, Sandeep

    2016-01-01

    Treatment resistant depression (TRD) is a common clinical occurrence among patients treated for major depressive disorder. A significant proportion of patients remain significantly depressed in spite of aggressive pharmacological and psychotherapeutic approaches. Management of patient with treatment resistant depression requires thorough evaluation for physical causes. We report a case of recurrent depressive disorder, who presented with severe depressive episode without psychotic symptoms, not responding to multiple adequate trials of antidepressants, who on investigation was found to have Cushing's syndrome and responded well to Ketoconazole. PMID:27335521

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

    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

  3. Diagnosis in the cushing's syndrome revisited

    The diagnostic procedure for the differential diagnosis of Cushing's syndrome is reported in this paper based on the experience of 23 cases. Inappropiate cortisol secretion was established by an absent cortisol circadian rhythm and absent cortisol suppression after overnight dexamethasone suppression test. The ACTH serum levels were then determined in basal conditions and after insulin-induced hypoglycemia (0.15 U/kg b.w. insulin i.v.). ACTH was low or undetectable in 9 patients, and high or normal-high in 14 patients. In the first group of patients an adrenal trasmission computed tomography (CT) was performed and showd an adrenal adenima in 6 patients, adrenal carcinoma in 2 patients and hyperplasia of residual adrenal gland in 1 patient, who had previously undergone monolateral adrenalectomy. These patients underwent surgical treatment, except the patient with adrenal hyperplasia. In the second group of patients, negative in 4 patients, doubtful in 1 patient. Surgical exploration by transsphenoidal route was performed, and an ACTH-producing adenima removed in all cases. Radicalization with hypophysectomy was necessary in 2 patients, while other 2 patients are under observation for the suspicion of a recurrent pituitary tumor. In all patients adrenal scintiscan was also performed, and confirmed the suspicion pointed out by CT scan. A relatively simple protocol with a functional test (ACTH determination) and a morphological one (computed tomography), can be reliably applied in the differential diagnosis of Cushing syndrome

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

    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)

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

    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)

  6. Pulmonary mineralization in four dogs with Cushing's syndrome

    The clinical and imaging features of four dogs with Cushing's syndrome and pulmonary mineralization are reviewed. Three dogs presented with a primary complaint of respiratory distress/dyspnea. Three dogs had pituitary dependent Cushing's syndrome, while the remaining one dog had iatrogenic Cushing's syndrome. Each dog had clinical features typical for Cushing's syndrome. Two of the dogs were euthanized due to progressive hypoxemia. In each dog, the serum calcium, phosphorous, blood urea nitrogen and creatinine were normal. A generalized increase in unstructured interstitial pulmonary opacity with diffuse mineralization was noted on thoracic radiographs of all dogs. In one dog, an ill-defined nodular interstitial pattern of mineralization was present. Delayed bone phase scintigraphy using 99mTechnetium methylene diphosphonate documented generalized pulmonary uptake in two dogs. 99mTechnetium labeled microaggregated albumin lung perfusion scans were normal in these two dogs. 99mTc-MDP scintigraphy can provide useful information in diagnosing pulmonary mineralization in Cushingoid dogs

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

    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.)

  8. Nuclear imaging for the Cushing's syndrome etiological diagnosis

    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)

  9. PET imaging in ectopic Cushing syndrome: a systematic review.

    Santhanam, Prasanna; Taieb, David; Giovanella, Luca; Treglia, Giorgio

    2015-11-01

    Cushing syndrome due to endogenous hypercortisolism may cause significant morbidity and mortality. The source of excess cortisol may be adrenal, pituitary, or ectopic. Ectopic Cushing syndrome is sometimes difficult to localize on conventional imaging like CT and MRI. After performing a multilevel thoracoabdominal imaging with CT, the evidence regarding the use of radiotracers for PET imaging is unclear due to significant molecular and etiological heterogeneity of potential causes of ectopic Cushing's syndrome. In our systematic review of literature, it appears that GalLium-based (Ga68) somatostatin receptor analogs have better sensitivity in diagnosis of bronchial carcinoids causing Cushing syndrome and FDG PET appears superior for small-cell lung cancers and other aggressive tumors. Further large-scale studies are needed to identify the best PET tracer for this condition. PMID:26206753

  10. Combination of Klinefelter Syndrome and Acromegaly: A Rare Case Report.

    Fang, Hongjuan; Xu, Jian; Wu, Huanwen; Fan, Hong; Zhong, Liyong

    2016-04-01

    Klinefelter syndrome (KS) is the most common chromosomal aneuploidy in male population, which demonstrates an unusual association with acromegaly. We herein present a rare case involving the confirmation of KS 2 years after surgical treatment for acromegaly.A 27-year-old man presented with an acromegalic appearance. Endocrinological examination revealed a high growth hormone (GH) concentration, low testosterone concentration, and high follicle-stimulating hormone and luteinizing hormone concentration. Brain imaging revealed a 9 × 6 × 7- mm sellar low-density nodule suggestive of a microadenoma. Trans-sphenoidal surgery was undertaken, and immunohistochemistry revealed GH positivity. Two years after surgery, the patient underwent examination for infertility. He presented with diminished pubic hair, and small and firm testes. Hormonal assay revealed hypergonadotrophic hypogonadism on the basis of decreased serum total testosterone (testosterone undecanoate was begun. Brain imaging showed no delayed enhancement in the saddle region of the pituitary gland, but the concentration of plasma insulin-like growth factor maintained a high level. The patient's GH concentration was not significantly suppressed by the GH glucose suppression test. In this consideration, he was referred for postoperative somatostatin analogue treatment to control GH hypersecretion.The misdiagnosis or delayed diagnosis of KS is mainly because of substantial variations in clinical presentation and insufficient professional awareness of the syndrome itself. As the simultaneous occurrence of KS and acromegaly is rare, and the association between them remains unclear, we suggest that complete pituitary hormonal screening and conventional pituitary MRI should be essential for patients with KS to screen for pituitary tumor. PMID:27124035

  11. Nonsuppressible Oral Dexamethasone Suppression Tests but Not Cushing Syndrome

    Abilash Nair; Atul Dhingra; Anjana Gopi; Jyotsna, Viveka P.

    2016-01-01

    In spite of the presence of definitive diagnostic criteria to diagnose Cushing syndrome diagnosis may become challenging. We report a young female with mild clinical features of Cushing syndrome, who had nonsuppressible oral dexamethasone suppression tests; also she had a suspicious pituitary lesion. She underwent pituitary surgery and a pituitary microadenoma (non-ACTH staining) was removed. Now she had come to us with similar complaints to those before. Again she had nonsuppressible oral de...

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

    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)

  13. New Insights into Medical Treatment of Acromegaly

    Neggers, Bas

    2011-01-01

    textabstractGiants and Acromegalics fascinated people, since ancient times. Historical artifacts, paintings, illustrations, photographs or articles have documented many. The earliest medical reports date back to 1516. In 1864 Verga was the first to describe an acromegalic in medical literature and called it “prosopectasia”. However the article did not really characterize the disease. Pierre Marie was the first to do so and describe the disease and gave it the final name “acromegalie”, in 1886...

  14. Diagnostic challenges in acromegaly: a case-based review.

    Bonert, Vivien

    2009-12-01

    Acromegaly is a rare, chronic condition caused by sustained and unregulated oversecretion of growth hormone (GH), usually attributed to a pituitary adenoma. Prolonged exposure to excessive amounts of GH and its target hormone, insulin-like growth factor-1 (IGF-1), results in pronounced metabolic changes and tissue enlargement that ultimately lead to increased morbidity and early mortality. As early diagnosis of acromegaly can have substantial beneficial effects on quality of life and overall survival for patients, it is important that the tests used to diagnose the condition are accurate, with highly reproducible results. The first kits used to measure GH and IGF-1 were radioimmunoassay, with many limitations that necessitated the development of more sensitive tools. Newer assays, although better than previous assays, are far from ideal. Simple changes that may improve the testing process include the adoption of mass units for GH interpretation and the use of a single recombinant calibrant. Furthermore, the conversion factors and reference ranges used to describe the normal limits for GH and IGF-1 levels require refinement. Physicians should be aware of the GH and IGF-1 assays used in their reference laboratories, and ensure that they know the appropriate assay cut-off values, to avoid misinterpreting results. PMID:20129191

  15. A Rare Cause of Acromegaly: Short Review of McCune Albright Syndrome

    Yusuf Aydın

    2009-06-01

    Full Text Available McCune-Albright syndrome (MAS is characterized by a triad of poly/monostotic fibrous dysplasia, café-au-lait macules, and hyperfunctioning endocrinopathies, including growth hormone (GH excess. Acromegaly, as a manifestation of endocrine hyperfunction with MAS is uncommon. We report a 34-year-old man with MAS and acromegaly, in whom surgical removal of the pituitary tumour has been technically difficult because of bone deformities. A combination of a long-acting somatostatin analogue (Sandostatin LAR and external irradiation were therefore used as treatment. Acromegaly associated with MAS is very rarely seen, and has been the subject of approximately 70 published reports. We present a case of acromegaly associated with MAS and a brief survey of relevant literature. Turk Jem 2009; 13: 13-5

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

    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)

  17. Diagnosis application of ACTH radioimmunoassay in diseases of hypothalamus, hypophysis and adrenal axis

    The diagnostic value of 900-1,100 am plasma ACTH radioimmunoassay were studied in 10 patients with Cushing's disease before and after treatment, three patients with Cushing's syndrome with adrenal tumours, one Nelson's syndrome patient; 13 patients with Addison's disease and 12 patients with hypo-pituitarism. Twenty-seven normal subjects were controls. The measurement of basal plasma ACTH gave good differentiation between: a. pituitary Cushing's disease from adrenal tumors; b. Addison's disease from hypo-pituitarism. However this assay has a limited value for the differentiation between Cushing's disease from normal subjects and it is often unhelpful in the differential diagnosis of hypo-pituitarism from normal subjects. (author)

  18. Retrospective Analysis of Cushing’s Disease with or without Hyperprolactinemia

    2014-01-01

    Objective. We compared the characteristics of patients with Cushing's disease alone with those of patients with Cushing's disease and hyperprolactinemia. Methods. Eighty-four patients were enrolled between 2002 and 2011, in a hospital in China. Clinical, endocrinological, and histopathological data, MRI scans, and surgical outcomes were reviewed throughout the follow-up period. Results. Patients with Cushing's disease and hyperprolactinemia had a younger age at diagnosis (30.28 ± 14.23 versus...

  19. Nonsuppressible Oral Dexamethasone Suppression Tests but Not Cushing Syndrome.

    Nair, Abilash; Dhingra, Atul; Gopi, Anjana; Jyotsna, Viveka P

    2016-01-01

    In spite of the presence of definitive diagnostic criteria to diagnose Cushing syndrome diagnosis may become challenging. We report a young female with mild clinical features of Cushing syndrome, who had nonsuppressible oral dexamethasone suppression tests; also she had a suspicious pituitary lesion. She underwent pituitary surgery and a pituitary microadenoma (non-ACTH staining) was removed. Now she had come to us with similar complaints to those before. Again she had nonsuppressible oral dexamethasone suppression tests. As the diurnal variation of serum and salivary cortisol was maintained and urinary free cortisol was normal, further evaluation with IV dexamethasone suppression test was performed which clearly ruled out Cushing syndrome. The patient was not on any medicines known to alter dexamethasone metabolism. Fat malabsorption was also ruled out using appropriate tests. The reason for this discrepancy is thought to be altered (increased) metabolism of dexamethasone in this patient as it is widely variable in the general population. PMID:27092281

  20. Tumorigenesis of papillary thyroid cancer is not BRAF-dependent in patients with acromegaly.

    Hee Kyung Kim

    Full Text Available INTRODUCTION: Several studies have reported a high frequency of papillary thyroid cancer (PTC in patients with acromegaly. The aim of this study was to determine the prevalence and predictors of thyroid cancer in patients with acromegaly and to investigate the frequency of the BRAFV600E mutation in PTC patients with and without acromegaly. MATERIALS AND METHODS: We conducted a retrospective study of 60 patients with acromegaly. Thyroid ultrasonography (US and US-guided fine needle aspiration were performed on nodules with sonographic features of malignancy. We selected 16 patients with non-acromegalic PTC as a control group. The BRAFV600E mutation was analyzed in paraffin-embedded surgical specimens of PTC by real-time polymerase chain reaction, and tumor specimens from patients with PTC were stained immunohistochemically with an antibody against insulin-like growth factor-1 receptor β (IGF-1Rβ. RESULTS: Thyroid cancer was found in 15 (25.0% patients. No differences in age, sex, initial growth hormone (GH and IGF-1 percentage of the upper limit of normal values or treatment modalities were observed between patients with and without PTC. Acromegaly was active in 12 of 15 patients at the time of PTC diagnosis; uncontrolled acromegaly had a significantly higher frequency in the PTC group (60% than in the non-PTC group (28.9% (p = 0.030. The BRAFV600E mutation was present in only 9.1% (1/11 of PTC patients with acromegaly, although 62.5% (10/16 of control patients with PTC had the mutation (p = 0.007. IGF-1Rβ immunostaining showed moderate-to-strong staining in all malignant PTC cells in patients with and without acromegaly. Significantly less staining for IGF-1Rβ was observed in normal adjacent thyroid tissues of PTC patients with acromegaly compared with those without (p = 0.014. CONCLUSION: The prevalence of PTC in acromegalic patients was high (25%. An uncontrolled hyperactive GH-IGF-1 axis may play a dominant role in the

  1. 131I-19-iodocholesterol adrenal scanning in Cushing's syndrome

    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 131I-19-iodocholesterol uptake. Adrenal imaging with 131I-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

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

    An alteration in serum cholesterol levels has been suggested as a possible modifier of adrenal uptake of the cholesterol analog, 6β-[131I]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

  3. Genetics of primary bilateral macronodular adrenal hyperplasia: a model for early diagnosis of Cushing's syndrome?

    Drougat, Ludivine; Espiard, Stéphanie; Bertherat, Jerôme

    2015-10-01

    Long-term consequences of cortisol excess are frequent despite appropriate treatment after cure of Cushing's syndrome. This might be due to diagnostic delay, often difficult to reduce in rare diseases. The identification of a genetic predisposing factor might help to improve early diagnosis by familial screening. Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of Cushing's syndrome. Hypercortisolism in PBMAH is most often diagnosed between the fifth and sixth decades of life. The bilateral nature of the adrenocortical tumors and the occurrence of rare clear familial forms suggest a genetic origin. Indeed, a limited subset of PBMAH can be observed as part of multiple tumors syndromes due to alterations of the APC, Menin or Fumarate Hydratase genes. Rare variants of the phosphodiesterases PDE11A have been associated with PBMAH. The recent identification of ARMC5 germline alterations in 25-50% of PBMAH patients without obvious familial history or associated tumors opens new perspectives. ARMC5 alterations follow the model of a tumor suppressor gene: a first germline inactivating mutation of this 16p located gene is followed by a somatic secondary hit on the other allele (inactivating mutation or allelic loss). Functional studies demonstrate that ARMC5 controls apoptosis and steroid synthesis. The phenotype of index cases patients with the mutation seems more severe than the one of WT index cases. However, phenotype variability within a family is often observed. This review summarizes the genetics of PBMAH, focusing on ARMC5, which offer new perspectives for early diagnosis of Cushing's syndrome. PMID:26264719

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

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

    2016-06-01

    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

  5. Gamma-knife radiosurgery in acromegaly: a 4-year follow-up study.

    Attanasio, Roberto; Epaminonda, Paolo; Motti, Enrico; Giugni, Enrico; Ventrella, Laura; Cozzi, Renato; Farabola, Mario; Loli, Paola; Beck-Peccoz, Paolo; Arosio, Maura

    2003-07-01

    , who already had partial hypopituitarism, after 2 and 6 yr, respectively. No patient developed visual deficits. GK is a valid adjunctive tool in the management of acromegaly that controls GH/IGF-I hypersecretion and tumor growth, with shrinkage of adenoma and no recurrence of the disease in the considered observation period and with low acute and chronic toxicity. PMID:12843150

  6. ACTH deficiency, higher doses of hydrocortisone replacement, and radiotherapy are independent predictors of mortality in patients with acromegaly.

    Sherlock, M

    2009-11-01

    A number of retrospective studies report that patients with acromegaly have increased morbidity and premature mortality, with standardized mortality ratios (SMR) of 1.3-3. Many patients with acromegaly develop hypopituitarism as a result of the pituitary adenoma itself or therapies such as surgery and radiotherapy. Pituitary radiotherapy and hypopituitarism have also been associated with an increased SMR.

  7. Cardiac effects of 3 months treatment of acromegaly evaluated by magnetic resonance imaging and B-type natriuretic peptides

    Andreassen, Mikkel; Faber, Jens; Kjær, Andreas;

    2010-01-01

    Long-term treatment of acromegaly prevents aggravation and reverses associated heart disease. A previous study has shown a temporary increase in serum levels of the N-terminal fraction of pro B-type natriuretic peptide (NT-proBNP) suggesting an initial decline in cardiac function when treatment of...... NT-proBNP). CMRI was performed at baseline and after 3 months of treatment. Levels of IGF-I, BNP and NT-proBNP were measured after 0, 1, 2 and 3 months. Eight patients (5 males and 3 females, mean age 53 ± 12 years (range 30-70)) and 8 matched healthy control subjects were included. Median IGF-I Z...

  8. Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome

    Primary aldosteronism (PA), an autonomous aldosterone hypersecretion from adrenal adenoma and/or hyperplasia, and subclinical Cushing syndrome (SCS), a mild but autonomous cortisol hypersecretion from adrenal adenoma without signs or symptoms of Cuhing's syndrome, are now well-recognized clinical entities of adrenal incidentaloma. However, the clinicopathological features of PA associated with SCS (PA/SCS) remain unknown. The present study was undertaken to study the prevalence of PA/SCS among PA patients diagnosed at our institute, and characterize their clinicopathlogical features. The prevalence of PA/SCS was 8 of 38 PA patients (21%) studied. These 8 PA/SCS patients were significantly older and had larger tumor, higher serum potassium levels, lower basal plasma levels of aldosterone, adrenocorticotropic hormone (ACTH) and dehydroepiandrosterone sulfate (DHEA-S) as well as lower response of aldosterone after ACTH stimulation than those in 12 patients with aldosterone-producing adenoma without hypercortisolism. All 8 PA/SCS patients showed unilateral uptake by adrenal scintigraphy at the ipsilateral side, whereas the laterality of aldosterone hypersecretion as determined by adrenal venous sampling varied from ipsilateral (3), contralateral (2), and bilateral side (2). 6 PA/SCS patients who underwent adrenalectomy required hydrocortisone replacement postoperatively. Histopathological analysis of the resected adrenal tumors from 5 PA/SCS patients revealed a single adenoma in 3, and double adenomas in 2, with varying degrees of positive immunoreactivities for steroidgenic enzymes 3β-hydroxysteroid dehydrogenase (HSD), P450C17) by immunohistochemical study as well as CYP11B2 mRNA expression as measured by real-time radiotherapy-polymerase chain reaction (RT-PCR). In conclusion, PA/SCS consists of a variety of adrenal pathologies so that therapeutic approach differs depending on the disease subtype. (author)

  9. New developments in the medical treatment of Cushing's syndrome

    R. van der Pas (Rob); W.W. de Herder (Wouter); L.J. Hofland (Leo); R.A. Feelders (Richard)

    2012-01-01

    textabstractCushing's syndrome (CS) is a severe endocrine disorder characterized by chronic cortisol excess due to an ACTH-secreting pituitary adenoma, ectopic ACTH production, or a cortisol-producing adrenal neoplasia. Regardless of the underlying cause, untreated CS is associated with considerable

  10. Chronic Glucocorticoid Hypersecretion in Cushing's Syndrome Exacerbates Cognitive Aging

    Michaud, Kathy; Forget, Helene; Cohen, Henri

    2009-01-01

    Cumulative exposure to glucocorticoid hormones (GC) over the lifespan has been associated with cognitive impairment and may contribute to physical and cognitive degeneration in aging. The objective of the present study was to examine whether the pattern of cognitive deficits in patients with Cushing's syndrome (CS), a disorder characterized by…

  11. LUPUS ERITEMATOSO SISTÉMICO SÍNDROME DE CUSHING Y PSIQUIATRÍA DE ENLACE Erythematous systemic lupus, Cushing`s syndrome and psychiatric

    Ludwig Pájaro Silva

    2005-04-01

    Full Text Available Se presenta el caso de una paciente con diagnóstico de lupus eritematoso sistémico y síndrome de Cushing quien presentó síntomas afectivos de características depresivas e insomnio de conciliación, quien recibió múltiples tratamientos farmacológicos con pobre respuesta inicial. La psiquiatría de enlace consolida una alternativa de tratamiento multidisciplinario para una aproximación más eficiente a las patologías médicas con síntomas psiquiátricos.We present a case of systemic erythematosus lupus and Cushing's syndrome who has affective symptoms of depressive characteristic and insomnia and multiple pharmacological treatments with poor response. The intervention by psychiatry liaison is an alternative of multidisciplinary treatment for an efficient approximation of medical pathologies with psychiatric symptoms.

  12. The application of bilateral inferior petrosal sinus sampling in diagnosing ACTH-dependent Cushing's syndrome

    Objective: To discuss the clinical values of bilateral inferior petrosal sinus sampling (BIPSS) in diagnosing ACTH-dependent Cushing's syndrome (CS). Methods: BIPSS and pituitary MRI examination were performed in 43 patients with CS, which was followed by pituitary surgery later. The clinical data and imaging findings were retrospectively analyzed. The diagnostic values of the two kinds of examinations were analyzed by receiver operating characteristic (ROC) curve and were compared with the area under the ROC curves (AUC). Results: In making the qualitative diagnosis of CS with BIPSS, the sensitivity, specificity, positive predictive value and negative predictive value was 0.95, 1.00, 1.00 and 0.71 respectively, while the AUC was 0.97, which was larger than that obtained from MRI. In making the diagnosis of the lesion's localization for Cushing's disease, the accuracy of BIPSS was 84.21%, which was much higher than that of pituitary MRI. Conclusion: BIPSS can be safely and successfully performed in most cases. With excellent diagnostic accuracy, both qualitative and localizing, BIPSS should be regarded as a useful and reliable method in diagnosing ACTH-dependent CS. (authors)

  13. Macroscopic brain architecture changes and white matter pathology in acromegaly: a clinicoradiological study

    Sievers, C.; Sämann, P. G.; T. Dose; Dimopoulou, C.; Spieler, D.; Roemmler, J.; Schopohl, J.; Mueller, M.; Schneider, H. J.; Czisch, M.; Pfister, H; Stalla, G K

    2008-01-01

    Although long-term exposure of the brain to increased GH/IGF-1 likely influences cerebral functions, no in vivo studies have been directed towards changes of the brain structure in acromegaly. Here, we used high resolution magnetic resonance images to compare volumes of gray matter (GM), white matter (WM) and cerebrospinal fluid (CSF) of forty-four patients with acromegaly to an age and gender matched, healthy control group (n = 44). In addition, white matter lesions (WMLs) were quantified an...

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

    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.)

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

    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. Acute abdominal pain in a man with Cushing syndrome.

    Rahmanian, M; Nedooshan, J J; Rafat, S; Rafie, R; Rafiei, M; Moghadam, R N

    2015-10-01

    Arterial thrombosis or emboli have rarely been reported in Cushing syndrome (CS). Here we describe the first case of mesenteric ischaemia secondary to ventricular emboli in a patient with CS. Laboratory evaluation showed increased fibrinogen and factor VIII. Previous studies showed that venous thromboembolism (VTE) increases in CS. This case for the first time described arterial system thrombosis and emboli in a patient with adrenocorticotropin (ACTH)-dependent CS. PMID:25943108

  17. Disease: H00260 [KEGG MEDICUS

    Full Text Available cal disease is a form of ACTH-independent adrenal hyperplasia resulting in endogenous Cushing's syndrome. End... OMIM: 610489 610475 603390 PMID:18549891 Powell AC, Stratakis CA, Patronas NJ, Steinberg SM, Batista D, Alexander HR, Pin...rome caused by adrenocortical tumors and hyperplasias (corticotropin- independent Cushing syndrome). End...ocr Dev 13:117-32 (2008) PMID:12119280 Bourdeau I, Stratakis CA Cyclic AMP-dependent signaling aberrations in...gpank JF, Keil M, Bartlett DL, Libutti SK Operative management of Cushing syndrome secondary t

  18. Bilateral catheterization of inferior petrosal sinous: Utility in Cushing syndrome

    The aim of this study is to present our experience on bilateral and simultaneous inferior petrous sinus catheterization, on those patients with ACTH -dependent Cushing's syndrome. We describe the procedure and our results. Material and Method: A retrospective study was held between January 2003 and September 2009, including nine patients (2 men, 7 women) presenting ACTH - dependent Cushing's syndrome. Simultaneous inferior petrosal sinus catheterization was performed in all of them, sampling basal ACTH and after CRH stimulation. ACTH levels gradient in different pituitary locations and peripheral blood levels was recorded. Diagnosis was suggested when inappropriate and maintained hypercortisolemia. High urinary free cortisol levels and no response to dexamethasone suppression were detected. Eight out of nine patients had a prior negative imaging test result. Results: Inferior petrosal sinus bilateral catheterization was successfully performed in all cases, with no evidence of further complications. The results showed definitive diagnosis in all cases. In four patients ACTH levels gradient was lateralized to the left, leading to a specific surgical approach. One patient presented pituitary ACTH - secreting adenoma. Two other patients showed ectopic ACTH production, one showed suprarenal adenoma secreting ACTH and other one showed response to pituitary stimulation without side lateralisation, presenting a histological diagnosis of pituitary hyperplasia. Conclusion: Petrosal sinus catheterization is shown to be an efficient procedure to manage Cushing's syndrome differential diagnosis and to obtain specific anatomical information.

  19. Glucose homeostasis and safety in patients with acromegaly converted from long-acting octreotide to pegvisomant

    A.L. Barkan (Ariel); P. Burman (Pia); D.R. Clemmons (David); M.T. Drake (Marcus); R.F. Gagel (Robert); P.E. Harris (Philip); P. Trainer; A-J. van der Lely (Aart-Jan); M.L. Vance

    2005-01-01

    textabstractContext: In clinical practice, patients with acromegaly may be switched from therapy with long-acting somatostatin analogs to pegvisomant. The effect of changing therapies on glucose homeostasis and safety has not been reported. Objectives: The objectives of this study were to monitor ch

  20. Spontaneous remission of acromegaly or gigantism due to subclinical apoplexy of pituitary growth hormone adenoma

    WANG Xian-ling; DOU Jing-tao; L(U) Zhao-hui; ZHONG Wen-wen; BA Jian-ming; JIN Du; LU Ju-ming; PAN Chang-yu; MU Yi-ming

    2011-01-01

    Background Subclinical apoplexy of pituitary functional adenoma can cause spontaneous remission of hormone hypersecretion.The typical presence of pituitary growth hormone (GH) adenoma is gigantism and/or acromegaly.We investigated the clinical characteristics of patients with spontaneous partial remission of acromegaly or gigantism due to subclinical apoplexy of GH adenoma.Methods Six patients with spontaneous remission of acromegaly or gigantism were enrolled.The clinical characteristics,endocrinological evaluation and imageological characteristics were retrospectively analyzed.Results In these cases,the initial clinical presences were diabetes mellitus or hypogonadism.No abrupt headache,vomiting,visual function impairment,or conscious disturbance had ever been complained of.The base levels of GH and insulin growth factor-1 (IGF-1) were normal or higher,but nadir GH levels were all still >1 μg/L in 75 g oral glucose tolerance test.Magnetic resonance imaging detected enlarged sella,partial empty sella and compressed pituitary.The transsphenoidal surgery was performed in 2 cases,and the other patients were conservatively managed.All the patients were in clinical remission.Conclusions When the clinical presences,endocrine evaluation,biochemical examination and imageology indicate spontaneous remission of GH hypersecretion in patients with gigantism or acromegaly,the diagnosis of subclinical apoplexy of pituitary GH adenoma should be presumed.To these patients,conservative therapy may be appropriate.

  1. Value of petrosal sinus sampling: coexisting acromegaly, empty sella and meningioma

    Yarman, S. [Div. of Endocrinology, Metabolism and Nutrition, Istanbul Univ., Istanbul (Turkey); Minareci, Oe. [Dept. of Radiology, Medical Faculty of Istanbul Univ., Istanbul (Turkey)

    2004-12-01

    Simultaneous occurrence of an intracranial meningioma and a growth hormone (GH)-producing pituitary adenoma is exceedingly rare, as is coexistence of an empty sella and acromegaly. We report all these rare entities in the same patient. We evaluated the role of inferior petrosal sinus sampling for lateralisation of an adenoma in this patient. (orig.)

  2. Value of petrosal sinus sampling: coexisting acromegaly, empty sella and meningioma

    Simultaneous occurrence of an intracranial meningioma and a growth hormone (GH)-producing pituitary adenoma is exceedingly rare, as is coexistence of an empty sella and acromegaly. We report all these rare entities in the same patient. We evaluated the role of inferior petrosal sinus sampling for lateralisation of an adenoma in this patient. (orig.)

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

    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)

  4. Patient-reported outcomes of parenteral somatostatin analogue injections in 195 patients with acromegaly

    Strasburger, Christian J; Karavitaki, Niki; Störmann, Sylvère; Trainer, Peter J; Kreitschmann-Andermahr, Ilonka; Droste, Michael; Korbonits, Márta; Feldmann, Berit; Zopf, Kathrin; Sanderson, Violet Fazal; Schwicker, David; Gelbaum, Dana; Haviv, Asi; Bidlingmaier, Martin; Biermasz, Nienke R

    2016-01-01

    Background Long-acting somatostatin analogues delivered parenterally are the most widely used medical treatment in acromegaly. This patient-reported outcomes survey was designed to assess the impact of chronic injections on subjects with acromegaly. Methods The survey was conducted in nine pituitary centres in Germany, UK and The Netherlands. The questionnaire was developed by endocrinologists and covered aspects of acromegaly symptoms, injection-related manifestations, emotional and daily life impact, treatment satisfaction and unmet medical needs. Results In total, 195 patients participated, of which 112 (57%) were on octreotide (Sandostatin LAR) and 83 (43%) on lanreotide (Somatuline Depot). The majority (>70%) of patients reported acromegaly symptoms despite treatment. A total of 52% of patients reported that their symptoms worsen towards the end of the dosing interval. Administration site pain lasting up to a week following injection was the most frequently reported injection-related symptom (70% of patients). Other injection site reactions included nodules (38%), swelling (28%), bruising (16%), scar tissue (8%) and inflammation (7%). Injection burden was similar between octreotide and lanreotide. Only a minority of patients received injections at home (17%) and 5% were self-injecting. Over a third of patients indicated a feeling of loss of independence due to the injections, and 16% reported repeated work loss days. Despite the physical, emotional and daily life impact of injections, patients were satisfied with their treatment, yet reported that modifications that would offer major improvement over current care would be ‘avoiding injections’ and ‘better symptom control’. Conclusion Lifelong injections of long-acting somatostatin analogues have significant burden on the functioning, well-being and daily lives of patients with acromegaly. PMID:26744896

  5. Ectopic Cushing syndrome secondary to recurrent pancreatoblastoma in a child: Lessons learnt

    Sajid S Qureshi

    2015-01-01

    Full Text Available Although rare, pancreatoblastoma is the most common pancreatic tumor in children. Cushing syndrome secondary to ectopic secretion of adrenocorticotropic hormone (ACTH from a pancreatoblastoma is very rare with only two previously reported cases. We present the management and the lesson learnt in a 3-year-old child with recurrent pancreatoblastoma with Cushing syndrome.

  6. Total and free insulin-like growth factor I, insulin-like growth factor binding protein 3 and acid-labile subunit reflect clinical activity in acromegaly

    Sneppen, S B; Lange, Merete Wolder; Pedersen, L M;

    2001-01-01

    insulin-like growth factor binding protein-3 (IGFBP-3) with PV(pos) of 0.69 and 0.71 and PV(neg) of 0.91 and 0.92 respectively. We conclude that free IGF-I is more closely related than total IGF-I to perceived disease activity and is as such useful when evaluating previously treated acromegaly for disease...... activity. Total IGF-I, IGFBP-3 and ALS possess a higher PV(neg) for the clinical disease activity. None of the parameters can at present be claimed to be superior to the others and thus all the measured parameters are recommended to be part of the evaluation of acromegalic patients....

  7. Pituitary tumors containing cholecystokinin

    Rehfeld, J F; Lindholm, J; Andersen, B N;

    1987-01-01

    We found small amounts of cholecystokinin in the normal human adenohypophysis and therefore examined pituitary tumors from 87 patients with acromegaly, Cushing's disease, Nelson's syndrome, prolactinoma, or inactive pituitary adenomas. Five adenomas associated with Nelson's syndrome contained......'s disease and 7 acromegaly with adenomas containing ACTH. The cholecystokinin peptides from the tumors were smaller and less sulfated than cholecystokinin from normal pituitary glands. We conclude that ACTH-producing pituitary cells may also produce an altered form of cholecystokinin....

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

    Ashish Tiwari

    2013-01-01

    Full Text Available 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.

  9. Endocrine & Metabolic Diseases A-Z

    ... Program (NHPP): Information for People Treated with Pituitary Human Growth Hormone (Summary) Back to Top C Cushing's Syndrome Defines ... Program (NHPP): Information for People Treated with Pituitary Human Growth Hormone (Summary) Back to Top H Hashimoto's Disease Describes ...

  10. [Recovery of Cushing syndrome revealing McCune-Albright syndrome].

    Halioui-Louhaichi, S; Dridi, Y; Azzabi, O; Selmi, I; Fetni, I; Siala, N; Maherzi, A

    2016-01-01

    Cushing syndrome (CS) is a rare feature of McCune-Albright syndrome. Treatments consist of bilateral adrenalectomy followed by lifelong glucocorticoid and mineralocorticoid treatment. However, cases of spontaneous remission of CS have been reported in the literature. We report a case of McCune-Albright syndrome with CS treated with metyrapone for 30 months with prolonged remission after a 12-year follow-up. Adrenalectomy may be avoided in some cases of CS caused by McCune-Albright syndrome. Metyrapone could be a good alternative to surgical treatment. PMID:26552628

  11. Is Iatrogenic Cushing s a New Form of Child Neglect?

    Habip Almis

    2016-01-01

    Child abuse is an increasingly important issue. One of the main types of abuse is child neglect, that is, behavior in which the child is knowingly or unknowingly injured. In this article; we report a case of iatrogenic Cushing%u2019s syndrome due to clobetasol 17-propionate treatment that was used inappropriately, in order to discuss whether or not this is a form of child neglect. It is one of the basic tasks of families to protect the health of the child and learn how to treat that child whe...

  12. Morphological and Histopathological Changes in Orofacial Structures of Experimentally Developed Acromegaly-Like Rats: An Overview

    Masahiro Iikubo

    2012-01-01

    Full Text Available Tongue enlargement and mandibular prognathism are clinically recognized in almost all patients with acromegaly. An acromegaly-like rat model recently developed by exogenous administration of insulin-like growth factor I (IGF-I was used to investigate morphological and histopathological changes in orofacial structures and to clarify whether these changes were reversible. Exogenous administration of IGF-I evoked specific enlargement of the tongue with identifiable histopathological changes (increased muscle bundle width, increased space between muscle bundles, and increased epithelial thickness, elongation of the mandibular alveolar bone and ascending ramus, and lateral expansion of the mandibular dental arch. Regarding histopathological changes in the mandibular condyle, the cartilaginous layer width, bone matrix ratio, and number of osteoblasts were all significantly greater in this rat model. After normalization of the circulating IGF-I level, tongue enlargement and histopathological changes in the tongue and mandibular condyle were reversible, whereas morphological skeletal changes in the mandible remained.

  13. Cushing's syndrome: Stepwise approach to diagnosis

    Lila, Anurag R.; Vijaya Sarathi; Jagtap, Varsha S.; Tushar Bandgar; Padmavathy Menon; Nalini S. Shah

    2011-01-01

    The projected prevalence of Cushing′s syndrome (CS) inclusive of subclinical cases in the adult population ranges from 0.2-2% and it may no longer be considered as an orphan disease (2-3 cases/million/year). The recognition of CS by physicians is important for early diagnosis and treatment. Late-night salivary cortisol, dexamethasone suppressiontesti, or 24-h urine free cortisol are good screening tests. Positively screened cases need stepwise evaluation by an endocrinologist. This paper disc...

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

    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

  15. The care continuum in acromegaly: how patients, nurses, and physicians can collaborate for successful treatment experiences

    Plunkett C

    2015-07-01

    Full Text Available Cynthia Plunkett, Ariel L BarkanDivision of Endocrinology, University of Michigan Medical Center, Ann Arbor, MI, USAAbstract: Patients with acromegaly (a condition of chronic growth hormone hypersecretion by a pituitary adenoma often require pharmacological treatment. Somatostatin analogs (SSAs such as pasireotide, lanreotide, and octreotide are frequently used as first-line medical therapy. As SSAs are delivered by regular subcutaneous or intramuscular injections, they can result in injection-related pain or anxiety and can be challenging to fit into patients’ lifestyles. When combined with the prolonged, debilitating psychological complications associated with acromegaly, these administration challenges can negatively impact compliance, adherence, and quality of life. Proactively managing patients’ expectations and providing appropriate, timely guidance are crucial for maximizing adherence, and ultimately, optimizing the treatment experience. As part of ongoing clinical research since 1997, our team at the University of Michigan has used SSAs to treat 30 patients with acromegaly. Based on our clinical experiences with multiple SSA administration regimens (long-acting intramuscular, long-acting deep subcutaneous, and twice-daily subcutaneous, we generated a dialog map that guides health care professionals through the many sensitive and complex patient communication issues surrounding this treatment process. Beginning with diagnosis, the dialog map includes discussion of treatment options, instruction on proper drug administration technique, and ensuring of appropriate follow-up care. At each step, we provide talking points that address the following: the patients’ clinical situation; their geographic, economic, and psychological concerns; and their inclination to communicate with clinicians. We have found that involving patients, nurses, and physicians as equal partners in the treatment process optimizes treatment initiation, adherence

  16. Late onset isotretinoin resistant acne conglobata in a patient with acromegaly

    Jain Kapil; Jain V; Aggarwal Kamal; Bansal Anu

    2008-01-01

    A 55 year-old male presented with multiple pus-discharging abscesses and sinuses and mutilating scarring on the gluteal region and back prevalent for the last ten years with exacerbations and remissions. Physical examination revealed acromegaly with frontal bossing, prognathism, a barrel chest and acral hypertrophy. Dermatological examination revealed cutis verticis gyrata, thick eyelids, a large triangular nose, a thickened lower lip, macroglossia, widely spaced teeth and widened skin pores ...

  17. Iatrogenic Cushing syndrome caused by ocular glucocorticoids in a child.

    Messina, Maria Francesca; Valenzise, Mariella; Aversa, Salvatore; Arrigo, Teresa; De Luca, Filippo

    2009-01-01

    A boy aged 7.6 years presented to our Unit of Paediatric Endocrinology for evaluation of obesity. Progressive weight gain (10 kg) started 6 months earlier after an accidental penetrating orbital injury on the right eye. During this period the child has been treated with oral betamethasone (0.5 mg/day) for 1 month and dexamethasone 2% ocular drops (2 hourly by day) for 6 months. Physical examination showed he was 113.5 cm in height (-1.5 SD), weight 36.0 kg, blood pressure 110/90 mmHg (90th centile), body mass index 28 (+5 SD), truncal obesity, buffalo hump, "moon-face", increased lanugo hair and supraclavicular fullness. Endocrinological work-up revealed undetectable levels of basal adrenocorticotropic hormone (ACTH), basal and ACTH-stimulated cortisol and 24 h urine excretion cortisol, confirming the diagnosis of iatrogenic Cushing syndrome. The abrupt withdrawal of ocular glucocorticoids by the parents evoked two adrenal crises; 4 months later the patient recovered. In conclusion, we would alert doctors that every formulation of glucocorticoids, no ocular drops excluded, can determine severe systemic side effects and iatrogenic Cushing syndrome. PMID:21686405

  18. Mifepristone Treatment of Cushing's Syndrome in a Pediatric Patient.

    Banerjee, Ronadip R; Marina, Neyssa; Katznelson, Laurence; Feldman, Brian J

    2015-11-01

    Cushing's syndrome (CS) in the pediatric population is challenging to diagnose and treat. Although next-generation medical therapies are emerging for adults with CS, none are currently approved or used in children. Here we describe the first use of mifepristone, a glucocorticoid receptor antagonist, to treat CS in a pediatric subject. The patient, a 14-year-old girl with an 18-month history of metastatic neuroendocrine carcinoma, suffered from fatigue, profound myopathy, irritability, and depression. She was found to have hypertension, hypokalemia, and worsening control of her preexisting type 1 diabetes. In this report, we detail our clinical evaluation that confirmed CS caused by an ectopic adrenocorticotropic hormone secreting tumor. Surgical and radiation therapies were not pursued because of her poor functional status and limited life expectancy, and medical treatment of CS was indicated for symptom relief. Mifepristone treatment provided rapid improvement in glycemic control, insulin resistance, and hypertension as well as significant diminishment of her myopathy and fatigue. Hypokalemia was managed with an oral potassium replacement and dose escalation of spironolactone; no other significant adverse effects were observed. Despite successful palliation of Cushing's signs and symptoms, the patient died of progression of her cancer. This case demonstrates the safety and efficacy of mifepristone treatment in a pediatric patient with symptomatic, ectopic CS. We conclude that, in appropriate pediatric patients with CS, glucocorticoid receptor antagonism with mifepristone should be considered to control the effects of hypercortisolism and to improve quality of life. PMID:26459648

  19. Prevalence of colonic polyp and its predictors in patients with acromegaly

    Agarwal, Purnima; Rai, Praveer; Jain, Manoj; Mishra, Shambhavi; Singh, Uttam; Gupta, Sushil Kumar

    2016-01-01

    Background: There are conflicting data regarding the prevalence of colorectal polyp in patients with acromegaly. Subjects and Methods: Consecutive forty-seven acromegalic patients (21 men, 26 women), with a mean age of (40 ± 12 years) attending endocrinology outpatient department underwent full colonoscopy. All the patients underwent clinical and biochemical evaluation (glucose suppressed growth hormone (GH), Insulin-like growth factor-1 [IGF-1], fasting insulin, and glucose). The control group (n = 120) for colonoscopy was adult subjects undergoing evaluation for symptoms of irritable bowel syndrome. Clinical and biochemical parameters in acromegalic patients with colonic polyp were compared to those without a polyp. Results: Patients with acromegaly had significantly higher prevalence of colonic polyp as compared to control subjects (10.6% vs. 0.8%). None of the patients with polyp had skin tags. There was no significant difference between subjects with and without colonic polyp in duration of illness, basal, and glucose-suppressed GH and most recent IGF-1. Fasting blood sugar was significantly higher (P GH and IGF-1 levels. There were no specific predictive factors detected. Screening full colonoscopy is recommended in all cases with acromegaly. PMID:27366708

  20. Pegvisomant-induced serum insulin-like growth factor-I normalization in patients with acromegaly returns elevated markers of bone turnover to normal

    Parkinson, C; Kassem, M; Heickendorff, Lene;

    2003-01-01

    Active acromegaly is associated with increased biochemical markers of bone turnover. Pegvisomant is a GH receptor antagonist that normalizes serum IGF-I in 97% of patients with active acromegaly. We evaluated the effects of pegvisomant-induced serum IGF-I normalization on biochemical markers of b...

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

    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.

  2. A Zuni Artist looks at Frank Hamilton Cushing: Cartoons by Phil Hughte, Pueblo of Zuni Arts and Crafts, Zuni, New Mexico

    Douglas R. Givens

    1996-05-01

    Full Text Available This book is a very unique look into the legacy of Frank Hamilton Cushing at Zuni and his influence on the Zuni people by the Zuni cartoonist Phil Hughte. This volume of Hughte's cartoons depicts Cushing at various periods and important moments of his career at Zuni. Although Hughte's cartoons selected Cushing moments at Zuni require no words to express Cushing's influence on the pueblo, Hughte provides captions to each of his cartoons designed to explain what Cushing was doing to the reader who is not intimately knowledgeable about Cushing's work.

  3. Scintigraphy of the Adrenal Cortex in Cushing's Syndrome with NP-59: A Case Report

    Objectives: To describe the case of a patient with ACTH-dependent Cushing's syndrome that illustrates the usefulness of nuclear medicine in an important study that is rarely performed and, therefore, promote the knowledge of it, which is also scarce. Study Design: Description of case, the application of scintigraphy using radiolabelled noryodocolesterol I-131 and display pictures serial adrenal. Patient: female, 22 years, with a picture of 2 years of evolution studied by Cushing syndrome, ACTH dependent

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

    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.

  5. Nested stromal epithelial tumor of liver presenting with Cushing syndrome: a rare case report.

    Geramizadeh, Bita; Foroutan, Hamidreza; Foroutan, Ali; Bordbar, Mohammadreza

    2012-01-01

    Nested stromal and epithelial tumor of the liver is an extremely rare pediatric hepatic tumor. To the best of our knowledge, about 25 cases have been reported in the English literature so far, few of which accompanied with Cushing syndrome. Herein we report our experience with an 8-year-old boy presented with Cushing's syndrome because of ectopic ACTH production by this tumor. PMID:22771659

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

    Kim, Dong Hun; Choi, Dong Hyun; Lee, Young Min; Kim, Bo Bae; Ki, Young Jae; Kim, Jin Hwa; Chung, Joong Wha; Koh, Young Youp [Chosun University School of Medicine, Gwangju (Korea, Republic of); Kang, Joon Tae; Chae, Seung Seok [Dept. of nternal Medicine, Mokpo Jung-Ang General Hospital, Mokpo (Korea, Republic of)

    2014-10-15

    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.

  7. [CHALLENGES IN THE DIAGNOSIS OF CUSHING'S SYNDROME IN THE MODERN ERA].

    Saiegh, Leonard; Sheikh-Ahmad, Mohammad; Reut, Maria; Jubran, Yousef; Shechner, Carmela

    2015-12-01

    Cushing's syndrome results from prolonged and excessive exposure to medically prescribed corticosteroids, or from excess endogenous cortisol secretion. When endogenous cortisol secretion is suspected, several screening tests are conducted in order to confirm or to rule out the diagnosis. In recent years, as the cut-off point of cortisol concentration on the 1 mg overnight dexamethasone suppression test was lowered, the prevalence of Cushing's syndrome has increased, and more cases of mild syndromes, with negative results on one or more screening tests, have increasingly been reported. In this paper, we will describe the various screening tests used for Cushing's syndrome, and will discuss their degree of sensitivity in the diagnosis of mild cases. We conclude that, in cases of mild syndromes, the sensitivity of some tests appears to be notably lower than has been reported. Until recently, the major challenge has been to distinguish between pseudo-Cushing's states and Cushing's syndrome. Today, however, the challenge has become to avoid misdiagnosis of mild cases, presenting with normal results on some screening tests. The sensitivity of urinary free cortisol seems to be lower than previously reported. Therefore, we recommend not to rely solely on this test in ruling out Cushing's syndrome. PMID:26897783

  8. Cushing proximal symphalangism and the NOG and GDF5 genes

    Plett, Sara K. [Columbia University, College of Physicians and Surgeons, New York, NY (United States); Berdon, Walter E.; Oklu, Rahmi [Columbia Presbyterian Medical Center, Department of Radiology, New York, NY (United States); Cowles, Robert A. [Morgan Stanley Children' s Hospital of New York-Presbyterian, Division of Pediatric Surgery, Department of Surgery, New York, NY (United States); Campbell, John B. [Arnold Palmer Hospital for Children, Department of Radiology, Orlando, FL (United States)

    2008-02-15

    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.)

  9. Is Iatrogenic Cushing s a New Form of Child Neglect?

    Habip Almis

    2016-02-01

    Full Text Available Child abuse is an increasingly important issue. One of the main types of abuse is child neglect, that is, behavior in which the child is knowingly or unknowingly injured. In this article; we report a case of iatrogenic Cushing%u2019s syndrome due to clobetasol 17-propionate treatment that was used inappropriately, in order to discuss whether or not this is a form of child neglect. It is one of the basic tasks of families to protect the health of the child and learn how to treat that child when he or she is ill. We believe that by being adequately informed by the health professionals, families may reduce the risk of child neglect.

  10. Mifepristone Improves Octreotide Efficacy in Resistant Ectopic Cushing's Syndrome

    Moraitis, Andreas G.; Auchus, Richard J.

    2016-01-01

    A 30-year-old Caucasian man presented with severe Cushing's syndrome (CS) resulting from ectopic adrenocorticotropin syndrome (EAS) from a metastatic pancreatic neuroendocrine tumor. The patient remained hypercortisolemic despite treatment with steroidogenesis inhibitors, chemotherapy, and octreotide long-acting release (LAR) and was enrolled in a 24-week, phase 3 clinical trial of mifepristone for inoperable hypercortisolemia. After mifepristone was added to ongoing octreotide LAR treatment, EAS symptoms essentially resolved. Cortisol decreased dramatically, despite mifepristone's competitive glucocorticoid receptor antagonist effects. The clinical and biochemical effects reversed upon mifepristone discontinuation despite the continued use of octreotide LAR therapy. Substantial improvement in octreotide LAR efficacy with mifepristone use was noted in this patient with ectopic CS, consistent with upregulation of somatostatin receptors previously downregulated by hypercortisolemia. PMID:26989527

  11. Cushing proximal symphalangism and the NOG and GDF5 genes

    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.)

  12. A Rare Case of Subclinical Primary Aldosteronism and Subclinical Cushing's Syndrome without Cardiovascular Complications.

    Kitajima, Natsumi; Seki, Toshiro; Yasuda, Atsushi; Oki, Masayuki; Takagi, Atsushi; Hanai, Kazuya; Terachi, Toshiro; Fukagawa, Masafumi

    2016-04-01

    We report a rare case of subclinical primary aldosteronism (PA) and subclinical Cushing's syndrome (CS). A 49-year-old woman was referred to our hospital for the evaluation of an adrenal incidentaloma. The patient had no previous medical history and no family history of notable illness. Her blood pressure was 103/60 mmHg. She had no Cushingoid features. Routine laboratory examinations were within the normal ranges including normokalemia. Based on the endocrinological results and imaging findings, we finally made a diagnosis of subclinical PA caused by both adrenal glands and subclinical CS caused by bilateral adrenal tumors. Interestingly, this patient had no risk factors for cardiovascular disease. In addition, the optimal management of patients with subclinical CS and subclinical PA has not been established. Therefore, we are observing her without medical therapy. Four years after diagnosis, no cardiovascular complications have been detected, including cerebral infarction, chronic kidney disease, cardiomegaly on echocardiography, and atherosclerosis on carotid ultrasonography. One important question is why the excessive hormone secretion did not affect the cardiovascular status of this patient. In this regard, we discuss several possible mechanisms including mineralocorticoid resistance and glucocorticoid sensitivity. PMID:27050894

  13. Long-term results of fractionated stereotactic radiotherapy as third-line treatment in acromegaly.

    Diallo, Alpha M; Colin, Philippe; Litre, Claude F; Diallo, Mamadou M; Decoudier, Bénédicte; Bertoin, Florence; Higel, Brigitte; Patey, Martine; Rousseaux, Pascal; Delemer, Brigitte

    2015-12-01

    The treatment of acromegaly is based on surgery, drugs, and radiotherapy as a third-line option. Fractionated stereotactic radiotherapy (FSRT) is a new technique with a need for long-term evaluation. The purpose of the study was to evaluate long-term results of FSRT in acromegaly. Overall, 34 patients [sex ratio 1.12, age 45 (5-65) years] with a pituitary adenoma of 24.5 (9-76) mm including 20 invasive tumors were treated by radiotherapy in fractionated stereotactic conditions delivering 50 gy in 27 sessions. Baseline growth hormone (GH) and IGF1 levels were 18 (±14.5) and 632.6 (±339) µg/L, respectively. Indications of FSRT were failure of surgery and drug treatments (n = 30) or contraindication/refusal of surgery (n = 4). Hormonal control was defined by normal age- and sex-adjusted IGF1. Remission was defined by hormonal control after withdrawal of drugs for a minimum of three consecutive months. Data were analyzed in SPSS software with a significance level at p < 0.05. After a mean follow-up of 152 months, hormonal control was achieved in 33 patients (97 %) with withdrawal of drugs in 13 patients (38.2 %) without any recurrence. Factors found to be significantly associated to remission in a multivariate Cox regression were lower baseline hormone levels (GH and IGF1) and smaller tumor size. Tumor control was achieved in all patients. Acquired hypopituitarism after radiotherapy was the main side effect reported with a rate of 39 %. FSRT seems to be an effective and well tolerated third-line treatment of acromegaly, particularly adapted to macro adenomas treatment. PMID:25956280

  14. Reduced basal ATP synthetic flux of skeletal muscle in patients with previous acromegaly.

    Julia Szendroedi

    Full Text Available BACKGROUND: Impaired mitochondrial function and ectopic lipid deposition in skeletal muscle and liver have been linked to decreased insulin sensitivity. As growth hormone (GH excess can reduce insulin sensitivity, we examined the impact of previous acromegaly (AM on glucose metabolism, lipid storage and muscular ATP turnover. PARTICIPANTS AND METHODS: Seven AM (4f/3 m, age: 46+/-4 years, BMI: 28+/-1 kg/m(2 and healthy volunteers (CON: 3f/4 m, 43+/-4 years, 26+/-2 kg/m(2 matched for age and body mass underwent oral glucose testing for assessment of insulin sensitivity (OGIS and ss-cell function (adaptation index, ADAP. Whole body oxidative capacity was measured with indirect calorimetry and spiroergometry. Unidirectional ATP synthetic flux (fATP was assessed from (31P magnetic resonance spectroscopy (MRS of calf muscle. Lipid contents of tibialis anterior (IMCLt and soleus muscles (IMCLs and liver (HCL were measured with (1H MRS. RESULTS: Despite comparable GH, insulin-like growth factor-1 (IGF-I and insulin sensitivity, AM had approximately 85% lower ADAP (p<0.01 and approximately 21% reduced VO(2max (p<0.05. fATP was similarly approximately 25% lower in AM (p<0.05 and related positively to ADAP (r = 0.744, p<0.01, but negatively to BMI (r = -0.582, p<0.05. AM had approximately 3 fold higher HCL (p<0.05 while IMCLt and IMCLs did not differ between the groups. CONCLUSIONS: Humans with a history of acromegaly exhibit reduced insulin secretion, muscular ATP synthesis and oxidative capacity but elevated liver fat content. This suggests that alterations in ss-cell function and myocellular ATP production may persist despite normalization of GH secretion after successful treatment of acromegaly.

  15. Gallbladder villous adenoma in a patient with acromegaly: A case report

    Miodrag Krstic; Tamara Alempijevic; Bojan Stimec; Marjan Micev; Miroslav Milicevic; Dragan Micic; Goran Jankovic

    2007-01-01

    Villous adenomas are benign epithelial lesions with malignant potential that can occur in any part of the gastrointestinal tract. We present a case of a middle age woman with acromegaly who was investigated for nonspecific gastrointestinal complaints. Ultrasonography and subsequent endosonography diagnosed a large (4.5 cm), hyperechoic, sessile polyp with numerous pedicles. An open cholecystectomy was performed and revealed a villous adenoma with several foci of carcinoma in situ. Detailed investigations showed no other tumors of the gastrointestinal tract. After five years of follow-up, the patient reports no complaints, and the results of laboratory testing and imaging studies are within the normal range.

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

    Tani, Atsushi; Nakajo, Masayuki; Tsuchimochi, Shinsaku; Nakabeppu, Yoshiaki; Umanodan, Tomokazu [Kagoshima Univ. (Japan). Faculty of Medicine

    2000-06-01

    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)

  17. Change in quality of life in patients with acromegaly after treatment with octreotide LAR: first application of AcroQoL in Korea

    Chin, Sang Ouk; Chung, Choon Hee; Chung, Yoon-Sok; Kim, Byung-Joon; Kim, Hee Young; Kim, In-Ju; Kim, Jung Guk; Kim, Min-Seon; Kim, Seong-Yeon; Lee, Eun Jig; Lee, Ki Young; Kim, Sung-Woon

    2015-01-01

    Objectives This study was designed to investigate changes in health-related quality of life (HRQoL) of patients with acromegaly in Korea after medical treatment with octreotide LAR using the validated Korean version of the acromegaly quality of life questionnaire (AcroQoL). Design A prospective, open-label, single-arm study. Setting 11 tertiary centres in Korea. Participants 58 Korean patients (aged 21–72 years) who were newly diagnosed with acromegaly between 2009 and 2012 were prescribed oc...

  18. Screening for acromegaly by application of a simple questionnaire evaluating the enlargement of extremities in adult patients seen at primary health care units.

    Rosario, Pedro Weslley; Calsolari, Maria Regina

    2012-06-01

    The objective of this study was to screen for acromegaly by application of a simple questionnaire in patients seen at primary health care units. A total of 17,000 patients of both genders >18 and physiognomy (confirmed by the comparison of photographs), in addition to the enlargement of extremities. The present investigation suggests a much higher prevalence of acromegaly in the adult population than that reported traditionally. We propose that screening based on phenotypic alterations is cost-effective since these changes occur early and almost universally in acromegaly and are uncommon in the general population. PMID:21380935

  19. Imaging of bronchial carcinoid tumors associated to Cushing syndrome with 111In-Octreoscan scintigraphy and immunoscintigraphy with anti-chromogranin monoclonal antibodies. Report of two cases.

    Carretta, A; Chiesa, G; Magnani, P; Songini, C; Melloni, G; Zannini, P; Grossi, A

    1997-04-01

    Bronchial carcinoid tumors are neuroendocrine neoplasms capable of expressing somatostatin receptors and of secreting neuromediators such as ACTH and chromogranins. Radiologic appearance is usually non-specific and has to be distinguished from benign pulmonary nodules and other malignant diseases. Standard radiological techniques have limited accuracy in the evaluation of such lesions. Radioisotopic imaging techniques may increase the specificity of diagnostic assessment. The role of immunoscintigraphy with anti-chromogranin A and B monoclonal antibodies (MoAbs) and of 111In-Octreoscan scintigraphy is evaluated in two cases of bronchial carcinoid tumors associated to Cushing syndrome. PMID:9201136

  20. Long-term safety of pegvisomant in patients with acromegaly: Comprehensive review of 1288 subjects in ACROSTUDY

    A-J. van der Lely (Aart-Jan); B.M.K. Biller; T. Brue (Thierry); M. Buchfelder; E. Ghigo (Ezio); R. Gomez (Roy); J. Hey-Hadavi (Judith); F. Lundgren (Frida); N. Rajicic; C.J. Strasburger; S.M. Webb (Susan); M. Kołtowska-Häggström (Maria)

    2012-01-01

    textabstractContext: Pegvisomant is a GH receptor antagonist. The ACROSTUDY is a global safety surveillance study of long-term treatment of acromegaly with pegvisomant. Objective: The objective of the study was to monitor long-term safety and treatment outcomes. Design: ACROSTUDY is open to all pati

  1. Challenges in early operative approaches to intramedullary spinal cord tumors: Harvey Cushing's perspective.

    Pendleton, Courtney; Rincon-Torroella, Jordina; Gokaslan, Ziya L; Jallo, George I; Quinones-Hinojosa, Alfredo

    2015-10-01

    Although Harvey Cushing was mostly known for his contributions to brain tumor surgery, he was also a pioneer in the development of spinal cord surgery. This lesser known facet of Cushing's career can provide a fresh and unique perspective into how the founders of neurosurgery surmounted early challenges in the field. The authors bring to light and examine for the first time Cushing's unpublished writing "Technique of Laminectomy" along with his first 3 documented intramedullary spinal cord tumor (IMSCT) cases at the Johns Hopkins Hospital. The authors draw lessons from the challenges in pathological classification, preoperative diagnosis, tumor localization, and surgical technique of that time. Although Cushing's attempts at exploration and resection of IMSCT as described here were of limited success, his ability to adapt his clinical and surgical technique to the challenges of the time, as well as develop skills to successfully manipulate the spinal cord during these exploratory procedures without the patients incurring neurological damage, postoperative infection, or complications, is a testament to his determination to advance the field and his meticulous operative technique. In spite of the limitations imposed on the pioneer neurosurgeons, Harvey Cushing and his contemporaries persevered through many of the challenges and built an essential part of neurosurgery's common story. PMID:26115026

  2. Macroscopic brain architecture changes and white matter pathology in acromegaly: a clinicoradiological study.

    Sievers, C; Sämann, P G; Dose, T; Dimopoulou, C; Spieler, D; Roemmler, J; Schopohl, J; Mueller, M; Schneider, H J; Czisch, M; Pfister, H; Stalla, G K

    2009-01-01

    Although long-term exposure of the brain to increased GH/IGF-1 likely influences cerebral functions, no in vivo studies have been directed towards changes of the brain structure in acromegaly. Here, we used high resolution magnetic resonance images to compare volumes of gray matter (GM), white matter (WM) and cerebrospinal fluid (CSF) of forty-four patients with acromegaly to an age and gender matched, healthy control group (n = 44). In addition, white matter lesions (WMLs) were quantified and graded. Patients exhibited larger GM (+3.7% compared with controls, P = 0.018) and WM volumes (+5.1%, P = 0.035) at the expense of CSF. Differences of WML counts between patients and controls were subtle, however, showing more patients in the 21-40 lesions category (P = 0.044). In conclusion, this MRI study provides first evidence that acromegalic patients exhibit disturbances of the macroscopic brain tissue architecture. Furthermore, acromegalic patients may have an increased risk of neurovascular pathology, likely due to secondary metabolic and vascular comorbidities. PMID:18836838

  3. Late onset isotretinoin resistant acne conglobata in a patient with acromegaly

    Jain Kapil

    2008-01-01

    Full Text Available A 55 year-old male presented with multiple pus-discharging abscesses and sinuses and mutilating scarring on the gluteal region and back prevalent for the last ten years with exacerbations and remissions. Physical examination revealed acromegaly with frontal bossing, prognathism, a barrel chest and acral hypertrophy. Dermatological examination revealed cutis verticis gyrata, thick eyelids, a large triangular nose, a thickened lower lip, macroglossia, widely spaced teeth and widened skin pores with wet and oily skin. Hair was fine and nails were flat and wide. There were multiple inflammatory papules, tender nodules, draining sinuses, and grouped, polyporous comedones as well as multiple and extensive depressed and keloidal scars localized predominantly over the gluteal region with a few scattered lesions over the back. A computed tomography (CT scan showed widened sella turcica. His basal fasting growth hormone (GH levels were markedly raised (230 ng/mL; normal 1-5 ng/mL while the prolactin levels were moderately raised (87 ng/mL; normal 2-5 ng/mL. These findings were consistent with a diagnosis of acromegaly. The patient was put on antibiotics, nonsteroidal antiinflammatory drugs and isotretinoin at a dose of 1 mg/kg/day, which was increased to 1.5 mg/kg/day. Except for an initial mildly beneficial response, the skin lesions were largely resistant to high doses of isotretinoin at the end of four months.

  4. Metastatic pituitary carcinoma in a patient with acromegaly: a case report

    Sreenan Seamus

    2012-09-01

    Full Text Available Abstract Introduction Asymptomatic pituitary abnormalities occur in about 10% of cranial magnetic resonance imaging scans, but metastatic carcinoma of the pituitary gland is rare: 133 cases have been reported. Two thirds secreted either prolactin or adrenocorticotropic hormone, and another 24% were non-secreting. Case presentation A 42-year-old Caucasian man lived for 30 years after the diagnosis of a pituitary tumor whose clinical and biochemical features were those of acromegaly and hypogonadism. Radiotherapy, totaling 7300 rad, was administered to the sella over two courses. Growth hormone levels normalized, but he developed both thyroid and adrenal insufficiency, and replacement therapy was commenced. Fourteen years later, growth hormone levels again became elevated, and bromocriptine was commenced but led to side effects that could not be tolerated. An attempted surgical intervention failed, and octreotide and pergolide were used in succession. Twenty-seven years after the diagnosis, a mass from an excisional biopsy of below the angle of the mandible proved to be metastatic pituitary carcinoma. Immunohistochemical staining was positive for synaptophysin, growth hormone, and prolactin. One year later, an octreotide scan showed uptake at the sella, neck, and spleen. Our patient declined further active oncology treatment. Conclusions Metastatic pituitary carcinoma associated with acromegaly is particularly rare. To the best of our knowledge, this is the eighth such case and is the first report of growth hormone and prolactin present in the metastatic mass.

  5. Efficacy and safety of monotherapy by pegvisomant, a growth hormone receptor antagonist, in Japanese patients with acromegaly.

    Shimatsu, Akira; Nagashima, Masahito; Hashigaki, Satoshi; Ohki, Nobuhiko; Chihara, Kazuo

    2016-04-25

    Pegvisomant is a GH receptor antagonist and strong inhibitor of insulin-like growth factor I (IGF-I) production. The treatment goal for acromegaly is to normalize serum IGF-I levels and attenuate associated symptoms. The efficacy and safety of pegvisomant as treatment for acromegaly have been reported in Caucasians, but not in Japanese. Here we report the clinical experience of using pegvisomant in Japanese patients with acromegaly. The efficacy and safety data for pegvisomant from two open-labeled clinical studies in Japan, conducted from 2004 to 2007, were re-analyzed using the new Japanese age- and sex-matched normative ranges for IGF-I. Eighteen patients with active acromegaly were enrolled in an initial pivotal study, and 16 of them were moved to a long-term (max 168 weeks) extension study. The dose of pegvisomant in the extension study was adjusted to 10-30 mg per day according to IGF-I levels. IGF-I normalization was observed in 81.3% (13/16 patients) during the extension study. The mean percentage decrease from baseline in serum IGF-I level was 64.7% at the time of last observation. The clinical symptoms and overall health status were improved, and the ring size was reduced over time until Week 12 and maintained. For safety, no clinically significant changes were observed both in the pituitary tumor size and the anti-GH antibody level. Three subjects were withdrawn from the studies due to an abnormal elevation of liver enzymes which resolved after discontinuation. Pegvisomant demonstrated excellent clinical efficacy and was well tolerated in Japanese patients with acromegaly. PMID:26796763

  6. Therapeutic Strategies for the Treatment of Severe Cushing's Syndrome.

    Alexandraki, Krystallenia I; Grossman, Ashley B

    2016-03-01

    Severe Cushing's syndrome presents an acute emergency and is defined by massively elevated random serum cortisol [more than 36 μg/dL (1000 nmol/L)] at any time or a 24-h urinary free cortisol more than fourfold the upper limit of normal and/or severe hypokalaemia (acute psychosis, progressive debilitating myopathy, thromboembolism or uncontrolled hyperglycaemia and ketocacidosis. Treatment focuses on the management of the severe metabolic disturbances followed by rapid resolution of the hypercortisolaemia, and subsequent confirmation of the cause. Emergency lowering of the elevated serum cortisol is most rapidly achieved with oral metyrapone and/or ketoconazole; if parenteral therapy is required then intravenous etomidate is rapidly effective in almost all cases, but all measures require careful supervision. The optimal order and combination of drugs to treat severe hypercortisolaemia-mostly in the context of ectopic ACTH-secreting syndrome, adrenocortical carcinoma or an ACTH-secreting pituitary adenoma (mainly macroadenomas)-is not yet established. Combination therapy may be useful not only to rapidly control cortisol excess but also to lower individual drug dosages and consequently the possibility of adverse effects. If medical treatments fail, bilateral adrenalectomy should be performed in the shortest possible time span to prevent the debilitating complications of uncontrolled hypercortisolaemia. PMID:26833215

  7. Total and free insulin-like growth factor I, insulin-like growth factor binding protein 3 and acid-labile subunit reflect clinical activity in acromegaly

    Sneppen, S B; Lange, Merete Wolder; Pedersen, L M; Kristensen L, L Ø; Main, K M; Juul, A; Skakkebaek, N E; Feldt-Rasmussen, U

    2001-01-01

    insulin-like growth factor binding protein-3 (IGFBP-3) with PV(pos) of 0.69 and 0.71 and PV(neg) of 0.91 and 0.92 respectively. We conclude that free IGF-I is more closely related than total IGF-I to perceived disease activity and is as such useful when evaluating previously treated acromegaly for disease...... the inactive and the active groups, we found that positive and negative predictive values (PV(pos), PV(neg)) for clinical disease activity of total and free insulin-like growth factor-I (IGF-I) were 0.59, 0.90 and 1.00, 0.82 respectively. Acid-labile subunit (ALS) showed diagnostic merit similar to...... activity. Total IGF-I, IGFBP-3 and ALS possess a higher PV(neg) for the clinical disease activity. None of the parameters can at present be claimed to be superior to the others and thus all the measured parameters are recommended to be part of the evaluation of acromegalic patients....

  8. A genetic and molecular update on adrenocortical causes of Cushing syndrome.

    Lodish, Maya; Stratakis, Constantine A

    2016-05-01

    Primary adrenal Cushing syndrome is the result of cortisol hypersecretion mainly by adenomas and, rarely, by bilateral micronodular or macronodular adrenocortical hyperplasia. cAMP-dependent protein kinase A (PKA) signalling is the major activator of cortisol secretion in the adrenal cortex. Many adenomas and hyperplasias associated with primary hypercortisolism carry somatic or germline mutations in genes that encode constituents of the cAMP-PKA pathway. In this Review, we discuss Cushing syndrome and its linkage to dysregulated cAMP-PKA signalling, with a focus on genetic findings in the past few years. In addition, we discuss the presence of germline inactivating mutations in ARMC5 in patients with primary bilateral macronodular adrenocortical hyperplasia. This finding has implications for genetic counselling of affected patients; hitherto, most patients with this form of adrenal hyperplasia and Cushing syndrome were thought to have a sporadic and not a familial disorder. PMID:26965378

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

    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.

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

    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.)

  11. Abnormalities of growth hormone release in response to human pancreatic growth hormone releasing factor (GRF (1-44) ) in acromegaly and hypopituitarism.

    Wood, S M; Ch'ng, J L; Adams, E. F.; Webster, J D; Joplin, G F; Mashiter, K; Bloom, S. R.

    1983-01-01

    Human pancreatic growth hormone releasing factor (GRF (1-44)) is the parent molecule of several peptides recently extracted from pancreatic tumours associated with acromegaly. A study was conducted to examine its effects on the release of growth hormone in normal volunteers and in patients with hypopituitarism and acromegaly. GRF (1-44) dose dependently stimulated the release of growth hormone in normal people and produced no appreciable side effect. This response was grossly impaired in pati...

  12. Bronchopulmonary Carcinoids causing Cushing Syndrome: Results from a Multicentric Study Suggesting a More Aggressive Behavior.

    Lococo, Filippo; Margaritora, Stefano; Cardillo, Giuseppe; Filosso, Perluigi; Novellis, Pierluigi; Rapicetta, Cristian; Carleo, Francesco; Bora, Giulia; Cesario, Alfredo; Stefani, Alessandro; Rossi, Giulio; Paci, Massimiliano

    2016-03-01

    Objective Cushing syndrome (CS) caused by bronchopulmonary carcinoids (BCs) is a very rare entity. The aim of this study was to revisit the features of a multicenter clinical series to identify significant prognostic factors. Methods From January 2002 to December 2013, the clinical and pathological data of 23 patients (treated in five different institutions) were retrospectively reviewed. Survival analysis was performed to explore the relative weight of potential prognostic factors. Results Median age and male/female ratio were 48 years and 14/9, respectively. Most (> 80%) of the patients presented with CS-related symptoms at diagnosis. Tumor location was peripheral in 13 patients (57%) and central in 10 (43%). All patients but two (treated with chemotherapy) underwent surgical resection with curative intent. Definitive cyto/histology was indicative of typical carcinoid (TC) in 16 cases (70%) and atypical carcinoid (AC) in 7 cases (30%). A complete remission of CS was obtained in 16 cases (70%). Lymph nodal involvement was detected in 11 cases (48%), with N2 disease occurring in 7 (∼ 30% of all cases). Four patients (22%) experienced a relapse of the disease after radical surgery. Overall 5-year survival (long-term survival, LTS) was 60%, better in TCs when compared with AC (LTS: 66 v s. 48%, p = 0.28). Log-rank analysis identified ECOG performance status, cTNM and cN staging, pTNM and pN staging, persistence of CS and relapses (local p = 0.006; distant p = 0.001) as significant prognostic factors in this cohort of patients. Conclusion BCs causing CS are characterized by a high rate of lymph-nodal involvement, a suboptimal prognosis (5-year survival = 60%, 66% in TCs) and a remarkable risk of relapse even after radical resection. Advanced stage, lymph-nodal involvement and the persisting of the CS after treatment correlate with a poor prognosis. PMID:26220696

  13. Harvey Cushing and pituitary Case Number 3 (Mary D.): the origin of this most baffling problem in neurosurgery.

    Pascual, José María; Prieto, Ruth

    2016-07-01

    From the very beginning of his career, Harvey Williams Cushing (1869-1939) harbored a deep interest in a complex group of neoplasms that usually developed at the infundibulum. These were initially known as "interpeduncular" or "suprasellar" cysts. Cushing introduced the term "craniopharyngioma" for these lesions, which he believed represented one of the most baffling problems faced by neurosurgeons. The patient who most influenced Cushing's thinking was a 16-year-old seamstress named "Mary D.," whom he attended in December 1901, exactly the same month that Alfred Fröhlich published his seminal article describing an adiposogenital syndrome in a young boy with a pituitary cyst. Both Cushing's and Fröhlich's patients showed similar symptoms caused by the same type of tumor. Notably, Cushing and Fröhlich had met one another and became good friends in Liverpool the summer before these events took place. Their fortunate relationship led Cushing to realize that Fröhlich's syndrome represented a state of hypopituitarism and provided a useful method of diagnosing interpeduncular cysts. It is noteworthy that Cushing's very first neurosurgical procedure on a pituitary tumor was performed in the case of Mary D.'s "interpeduncular cyst," on February 21, 1902. Cushing failed to remove this lesion, which was later found during the patient's autopsy. This case was documented as Pituitary Case Number 3 in Cushing's masterpiece, The Pituitary Body and Its Disorders, published in 1912. This tumor was considered "a teratoma"; however, multiple sources of evidence suggest that this lesion actually corresponded to an adamantinomatous craniopharyngioma. Unfortunately, the pathological specimens of this lesion were misplaced, and this prompted Cushing's decision to retain all specimens and documents of the cases he would operate on throughout his career. Accordingly, Mary D.'s case crystallized the genesis of the Cushing Brain Tumor Registry, one of Cushing's major legacies to

  14. Measuring tongue volumes and visualizing the chewing and swallowing process using real-time TrueFISP imaging - initial clinical experience in healthy volunteers and patients with acromegaly

    Ajaj, W.; Goyen, M.; Herrmann, B.; Massing, S.; Goehde, S.; Lauenstein, T.; Ruehm, S.G. [University Hospital, Department of Diagnostic and Interventional Radiology, Essen (Germany)

    2005-05-01

    This study assessed both two-dimensional (2D) TrueFISP imaging for quantifying tongue volume and real-time TrueFISP imaging for evaluating chewing and swallowing in healthy volunteers and patients with acromegaly. In 50 healthy volunteers, tongue volumes were measured using a 2D TrueFISP sequence. Chewing and swallowing were visualized using a real-time TrueFISP sequence. Ten patients with acromegaly were examined twice with the same magnetic resonance imaging protocol: once prior to therapy and a second time 6 months after therapy. Prior to therapy, healthy volunteers had an average tongue volume of 140 ml for men and 90 ml for women, and patients with acromegaly had an average tongue volume of 180 ml for men and 145 ml for women. However, 6 months after therapy the mean tongue volumes in patients with acromegaly had decreased to 154 ml in the men and to 125 ml in the women. The chewing and swallowing process was normal in all volunteers. Prior to therapy, just two patients showed a chewing and swallowing pathology, which disappeared after therapy. Patients with acromegaly had larger tongue volumes than healthy volunteers, and TrueFISP imaging proved feasible for visualizing chewing and swallowing in real time and is capable of detecting possible pathologies. Furthermore, TrueFISP imaging can be used to monitor therapeutic approaches in patients with acromegaly. (orig.)

  15. The possible role of mRNA expression changes of GH/IGF-1/insulin axis components in subcutaneous adipose tissue in metabolic disturbances of patients with acromegaly.

    Touskova, V; Klouckova, J; Durovcova, V; Lacinova, Z; Kavalkova, P; Trachta, P; Kosak, M; Mraz, M; Haluzikova, D; Hana, V; Marek, J; Krsek, M; Haluzik, M

    2016-07-18

    We explored the effect of chronically elevated circulating levels of growth hormone (GH)/insulin-like-growth-factor-1 (IGF-1) on mRNA expression of GH/IGF-1/insulin axis components and p85alpha subunit of phosphoinositide-3-kinase (p85alpha) in subcutaneous adipose tissue (SCAT) of patients with active acromegaly and compared these findings with healthy control subjects in order to find its possible relationships with insulin resistance and body composition changes. Acromegaly group had significantly decreased percentage of truncal and whole body fat and increased homeostasis model assessment-insulin resistance (HOMA-IR). In SCAT, patients with acromegaly had significantly increased IGF-1 and IGF-binding protein-3 (IGFBP-3) expression that both positively correlated with serum GH. P85alpha expression in SCAT did not differ from control group. IGF-1 and IGFBP-3 expression in SCAT were not independently associated with percentage of truncal and whole body fat or with HOMA-IR while IGFBP-3 expression in SCAT was an independent predictor of insulin receptor as well as of p85alpha expression in SCAT. Our data suggest that GH overproduction in acromegaly group increases IGF-1 and IGFBP-3 expression in SCAT while it does not affect SCAT p85alpha expression. Increased IGF-1 or IGFBP-3 in SCAT of acromegaly group do not appear to contribute to systemic differences in insulin sensitivity but may have local regulatory effects in SCAT of patients with acromegaly. PMID:27070751

  16. Clinical effectiveness and cost-effectiveness of pegvisomant for the treatment of acromegaly: a systematic review and economic evaluation

    Connock Martin J

    2009-10-01

    Full Text Available Abstract Background Acromegaly, an orphan disease usually caused by a benign pituitary tumour, is characterised by hyper-secretion of growth hormone (GH and insulin-like growth factor I (IGF-1. It is associated with reduced life expectancy, cardiovascular problems, a variety of insidiously progressing detrimental symptoms and metabolic malfunction. Treatments include surgery, radiotherapy and pharmacotherapy. Pegvisomant (PEG is a genetically engineered GH analogue licensed as a third or fourth line option when other treatments have failed to normalise IGF-1 levels. Methods Evidence about effectiveness and cost-effectiveness of PEG was systematically reviewed. Data were extracted from published studies and used for a narrative synthesis of evidence. A decision analytical economic model was identified and modified to assess the cost-effectiveness of PEG. Results One RCT and 17 non-randomised studies were reviewed for effectiveness. PEG substantially reduced and rapidly normalised IGF-1 levels in the majority of patients, approximately doubled GH levels, and improved some of the signs and symptoms of the disease. Tumour size was unaffected at least in the short term. PEG had a generally safe adverse event profile but a few patients were withdrawn from treatment because of raised liver enzymes. An economic model was identified and adapted to estimate the lower limit for the cost-effectiveness of PEG treatment versus standard care. Over a 20 year time horizon the incremental cost-effectiveness ratio was £81,000/QALY and £212,000/LYG. To reduce this to £30K/QALY would require a reduction in drug cost by about one third. Conclusion PEG is highly effective for improving patients' IGF-1 level. Signs and symptoms of disease improve but evidence is lacking about long term effects on improved signs and symptoms of disease, quality of life, patient compliance and safety. Economic evaluation indicated that if current standards (UK for determining cost

  17. Decreased trabecular bone biomechanical competence, apparent density, IGF-II and IGFBP-5 content in acromegaly

    Ueland, Thor; Ebbesen, Ebbe Nils; Thomsen, Jesper Skovhus;

    2002-01-01

    BACKGROUND: Earlier studies on the effect of excess growth hormone (GH) on trabecular bone have been conflicting. Since insulin-like growth factors (IGFs) and their binding proteins (IGFBPs) in part mediate the effects of GH, the present study aimed to investigate trabecular bone composition of...... found in trabecular bone content of IGF-I, IGFBP-3, or osteocalcin. However, IGF-II and IGFBP-5 content was decreased (P < 0.001 and P < 0.05, respectively). CONCLUSIONS: The present study demonstrates reduced trabecular biomechanical competence and apparent density in acromegaly, supporting previous...... observations of an unfavourable effect of chronic excess GH on the axial skeleton. Furthermore, we demonstrate decreased trabecular bone content of IGF-II and IGFBP-5 in these patients. However, we found no direct causal relationship between trabecular bone density and bone content of IGF-system components....

  18. Investigation of the immunological and receptor activity of human growth hormone in patients with acromegaly

    Human growth hormone (hGH) was measured by means of the radioimmunoassay (RIA) and the radioreceptor assay (RRA). The receptors were liver plasma membranes (LPM) of pregnant rabbits. In the RIA, no cross-reaction was found with hPRL, whereas in the RRA the cross-reaction was 3 p.c. The Scatchard analysis revealed two binding sites for hGH at the receptor. Pre-treatment with hGH and Cortisol brought about an enhanced affinity without change of the specific bonding, whereas pre-treatment with bromocriptin showed no significant effect. Hypophyseal hGH was separated by means of gel chromatography into big-big and big-little hGH and a reduced receptor activity of the higher molecular hGH fraction was shown. The Scatchard analysis indicated a more unspecific bonding characteristic of the big hGH. Stimulation of hGH secretion by insulin hypoglycemia provoked an overproportional increase in big hGH in healthy persons, whereas in patients with acromegaly the secretion of little hGH was enhanced. The suppression of hGH secretion by long-term bromocriptin treatment led to a significant rise of the RIA/RRA quotient in patients with post-operative florid acromegaly. Acute administration of BC was shown to induce a stronger hGH drop in the RRA of responders than in their RIA, as compared to non-responders. By chromatographic separation it was found that in responders the secretion of little hGH is selectively inhibited, but no in non-responders. (orig.)

  19. CUSH - A computer programme for the steady state hydraulic analysis of steam generating reactor systems

    The CUSH programme is written in FORTRAN and versions exist for the IBM 7090, IBM 7030 and EEC KDF-9 Computers. The Programme requires a three dimensional specification of the core power distribution and calculates the distribution of flow and coolant density within the core and primary circuit. (author)

  20. Two diagnoses become one? Rare case report of anorexia nervosa and Cushing's syndrome.

    Sawicka, Nadia; Gryczyńska, Maria; Sowiński, Jerzy; Tamborska-Zedlewska, Monika; Ruchała, Marek

    2013-01-01

    Hypothalamic-pituitary-adrenal axis impairment in anorexia nervosa is marked by hypercortisolemia, and psychiatric disorders occur in the majority of patients with Cushing's syndrome. Here we report a patient diagnosed with anorexia nervosa who also developed Cushing's syndrome. A 26-year-old female had been treated for anorexia nervosa since she was 17 years old, and also developed depression and paranoid schizophrenia. She was admitted to the Department of Endocrinology, Metabolism, and Internal Medicine with a preliminary diagnosis of Cushing's syndrome. Computed tomography revealed a 27 mm left adrenal tumor, and she underwent laparoscopic adrenalectomy. She was admitted to hospital 6 months after this procedure, at which time she did not report any eating or mood disorder. This is a rare case report of a patient with anorexia nervosa in whom Cushing's syndrome was subsequently diagnosed. Diagnostic difficulties were caused by the signs and symptoms presenting in the course of both disorders, ie, hypercortisolemia, osteoporosis, secondary amenorrhea, striae, hypokalemia, muscle weakness, and depression. PMID:23579693

  1. Acromegaly Clinical Trial Methodology Impact on Reported Biochemical Efficacy Rates of Somatostatin Receptor Ligand Treatments: A Meta-Analysis

    CARMICHAEL, JOHN D.; Bonert, Vivien S.; Nuño, Miriam; Ly, Diana; Melmed, Shlomo

    2014-01-01

    Introduction: Biochemical efficacy of somatostatin receptor ligand (SRL) treatment in acromegaly is defined by metrics for GH and IGF-1 control. Since the earliest therapeutic trials, biochemical control criteria, medical formulations, and assay techniques have evolved. Materials and Methods: We searched PubMed for English-language trials published from 1974 to 2012 evaluating 10 or more patients, with a duration of more than 3 months and biochemical control as a key objective. We used a rand...

  2. Mifepristone effects on tumor somatostatin receptor expression in two patients with Cushing's syndrome due to ectopic adrenocorticotropin secretion.

    Bruin, C. de; Hofland, L.J.; Nieman, L.K.; Koetsveld, P.M. van; Waaijers, A.M.; Sprij-Mooij, D.M.; Essen, M. van; Lamberts, S.W.J.; Herder, W.W. de; Feelders, R.A.

    2012-01-01

    CONTEXT: Two patients presented with Cushing's syndrome due to ectopic ACTH secretion. Initial localization studies included computed tomography, magnetic resonance imaging, and octreoscans ((111)In-pentreotide scintigraphy), which were negative in both patients. They were treated with the glucocort

  3. Serum insulin-like growth factor-1 measurement in the diagnosis and follow-up of patients with acromegaly: preliminary data.

    Guitelman, Mirtha; Radczuk, Graciela; Basavilbaso, Natalia García; Oneto, Adriana; Basso, Armando

    2010-01-01

    Measurement of serum insulin-like growth factor-1 (IGF-I) is the current method for diagnosing and monitoring acromegaly. However, the use of commercially available kits needs to be validated. In our study, we have investigated the use of two different IGF-I immunoassays in patients already diagnosed with acromegaly. We compared a two-site immunoradiometric assay with ethanol-acid extraction (IRMA-DSL) and a solid-phase chemiluminescent immunometric assay (ICMA-IMMULITE), correlating the clinical finding with the biochemical results. A total of 102 samples (77 women and 25 men aged 18-79 years) were analyzed with the two different IGF-I assays. Sixty-four of samples had been taken from patients with acromegaly in different stages. Pearson regression showed a high correlation coefficient; otherwise, Bland and Altman analyses showed a mean difference of 177.6 ng/ml, with upper and lower limits of -183.5 and 538.7 ng/ml in the 102 samples studied. Normal serum IGF-I was found in 64 and 41.5% of patients with treated acromegaly when measured by ICMA and IRMA, respectively. In our study, IGF-I-ICMA had a better clinical correlation in patients with treated acromegaly. The reevaluation of current IGF-I immunoassays is necessary to correctly interpret treatment response in acromegalic patients and thus achieve a better correlation between clinical and biochemical results. PMID:20616505

  4. Case Report: A case report of acromegaly associated with primary aldosteronism [v1; ref status: indexed, http://f1000r.es/2ny

    Joanna Matrozova

    2014-02-01

    Full Text Available We describe a patient with a rare combination of acromegaly and primary aldosteronism. A 37 year-old female patient was diagnosed with acromegaly on the basis of typical clinical, hormonal and image characteristics. She presented also with one of the most common co-morbidities – arterial hypertension. The patient has been regularly followed-up and after three surgical interventions, irradiation and adjuvant treatment with a dopamine agonist, acromegaly was finally controlled in 2008 (20 years after diagnosis. Arterial hypertension however, remained a therapeutic problem even after prescription of four antihypertensive drugs. She had normal biochemical parameters, except for low potassium levels 3.2 (3.5-5.6 mmol/l. This raised the suspicion of primary hyperaldosteronism, confirmed by a high aldosterone to plasma rennin activity ratio, high aldosterone level after a Captopril challenge test and visualization of a 35 mm left adrenal nodule on a CT scan. After an operation, the patient recovered from hypokalemia and antihypertensive therapy was reduced to a small dose of a Ca blocker. Co-morbid arterial hypertension is common in acromegaly, though it is rare for this to be caused by Conn’s adenoma. The association of Conn’s adenoma with acromegaly has been interpreted in two lines: as a component of multiple endocrine neoplasia type (MEN1 syndrome or as a direct mitogenic effect of hyperactivated GH-IGF1 axis.

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

    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)

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

    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)

  7. Streptomyces infection in Cushing syndrome: A case report and literature review

    Masoud Ataiekhorasgani

    2014-01-01

    Full Text Available Streptomyces are saprophytic soil organisms rarely known to cause invasive infections. Streptomyces is the largest genus, producing antibacterial, antifungal and antiparasitic drugs. The case was a 24-year-old man, admitted for sudden dyspnea, fever and sputum and decreased sound in the left lung. The chest X-ray showed hydropneumothorax. After chest tube insertion, lung expansion did not happen. Pleural effusion was exudative with gram-positive bacillus and Streptomyces in culture. Owing to symptoms of Cushing in history, examination and laboratory work-up for Cushing was done and finally he underwent bilateral adrenalectomy. The patient was on antibiotic broad spectrum antibiotic and then was changed to antibiotic as organism was sensitive to and discharged with clarithromycin for 6 months. Streptomyces happens in immunodeficient patient. Diagnosis is based on culture and contamination was ruled out. Treatment period is longer for patients owing to slow growing nature.

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

    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

  9. A Rare Case of Functioning Adrenocortical Oncocytoma Presenting as Cushing Syndrome.

    Tartaglia, Nicola; Cianci, Pasquale; Altamura, Amedeo; Lizzi, Vincenzo; Vovola, Fernanda; Fersini, Alberto; Ambrosi, Antonio; Neri, Vincenzo

    2016-01-01

    Functioning adrenocortical oncocytoma is very rare neoplasm. It is usually nonfunctional and benign and incidentally detected. Generally, these tumors originate in the kidneys, thyroid, parathyroid, and salivary or pituitary glands; they have also been reported in other sites including choroid plexus, respiratory tract, and larynx. Histologically, they are characterized by cells with eosinophilic granular cytoplasm and numerous packed mitochondria. We reported a case of a 44-year-old female who presented with Cushing syndrome for hypersecretion of cortisol due to adrenocortical oncocytoma. Magnetic resonance of abdomen revealed a right adrenal mass. Laparoscopic adrenalectomy was performed and the tumor was pathologically confirmed as benign adrenocortical oncocytoma. After surgical treatment, Cushing's syndrome resolved. PMID:26989553

  10. Nested stromal epithelial tumor of liver presenting with Cushing syndrome: A Rare case Report

    Bita Geramizadeh

    2012-01-01

    Full Text Available Nested stromal and epithelial tumor of the liver is an extremely rare pediatric hepatic tumor. To the best of our knowledge, about 25 cases have been reported in the English literature so far, few of which accompanied with Cushing syndrome. Herein we report our experience with an 8-year-old boy presented with Cushing′s syndrome because of ectopic ACTH production by this tumor.

  11. The impact of pegvisomant treatment on substrate metabolism and insulin sensitivity in patients with acromegaly

    Lindberg-Larsen, Rune; Møller, Niels; Schmitz, Ole;

    2007-01-01

    CONTEXT: Pegvisomant is a specific GH receptor antagonist that is able to normalize serum IGF-I concentrations in most patients with acromegaly. The impact of pegvisomant on insulin sensitivity and substrate metabolism is less well described. PATIENTS AND METHODS: We assessed basal and insulin.......3 microg/liter, P = 0.02). Basal serum insulin and plasma glucose levels decreased after treatment (insulin, 54 +/- 5.9 vs. 42 +/- 5.3 pmol/liter, P = 0.001; glucose, 5.7 +/- 0.1 vs. 5.3 +/- 0.0 mmol/liter, not significant), whereas palmitate kinetics were unaltered. During the clamp, the glucose infusion...... rate increased after pegvisomant (3.1 +/- 0.5 vs. 4.4 +/- 0.6 mg/kg.min, P = 0.02), whereas the suppression of endogenous glucose production tended to increase (0.7 +/- 0.0 vs. 0.5 +/- 0.1 mg/kg.min, not significant). Total resting energy expenditure decreased after pegvisomant treatment (1703 +/- 109...

  12. Pros and cons in endocrine practice: pre-surgical treatment with somatostatin analogues in acromegaly.

    Losa, Marco; Bollerslev, Jens

    2016-06-01

    The aim of this commentary is to balance the pros and cons for pre-surgical SSA treatment in a modern perspective ending up with a pragmatic recommendation for treatment based on the current evidence and expertise of the authors. Even though prospective and randomized studies in this particular area are hampered by obvious limitations, the interpretation of the four published trials has in general been in favor of pre-treatment with SSA, showing a better outcome following surgery. However, major drawbacks of these studies, such as non-optimal diagnostic criteria for cure, potential selection bias, and timing of the postoperative evaluation in SSA pre-treated patients, limit their overall interpretation. Three matched-controlled studies showed remarkably similar results with no apparent beneficial effect of SSA pre-treatment on surgical outcome. Both prospective, randomized studies and retrospective studies did not find any significant difference in the rate of endocrine and non-endocrine complications related to surgery, despite the beneficial clinical effects of SSA treatment in most acromegalic patients. The newly diagnosed patient with acromegaly should be carefully evaluated in the trans-disciplinary neuroendocrine team and treatment individualized accordingly. The issue of SSA pre-treatment to improve surgical outcome is yet to be settled and further methodologically sound studies are probably necessary to clarify this point. PMID:26785848

  13. Patología cardiovascular en la acromegalia Cardiovascular Disease in Acromegaly

    L Fiszlejder; PM Fiszlejder

    2012-01-01

    A pesar de los modernos tratamientos de la acromegalia, esta enfermedad somete al paciente, a un riesgo cardiovascular dos a tres veces mayor que el de la población adulta normal. Ello es el resultado de una prolongada exposición de los cardiomiocitos al exceso de GH. Esto produce cambios histológicos y en la configuración geométrica de las miofibrillas, fibrosis del intersticio e hipertrofia biventricular. La historia natural de esta cardiopatía de la acromegalia presenta varios períodos evo...

  14. Place of preoperative treatment of acromegaly with somatostatin analog on surgical outcome: a systematic review and meta-analysis.

    Francisco Pita-Gutierrez

    Full Text Available CONTEXT: Transsphenoidal neurosurgery is the accepted first-line treatment of acromegaly in the majority of patients. Previous studies addressing preoperative somatostatin analog (SSA treatment and subsequent surgical cure rates are conflicting, reporting either benefits or no significant differences. OBJECTIVE: The aim of this study, based on a meta-analysis of all published reports, was to investigate whether treatment with SSA before surgery improves the surgical outcome of acromegaly. DATA SOURCES: All studies of preoperative treatment of acromegaly with SSA were systematically reviewed up to December 2011. We searched the Medline, Embase, Cochrane and Google Scholar electronic databases. STUDY SELECTION: The primary endpoint was the biochemical postoperative cure rate. We identified 286 studies, out of which 10 studies (3.49% fulfilling the eligibility criteria were selected for analysis; five retrospective studies with a control group, two prospective non-randomized trials, and three prospective controlled trials. The meta-analysis was conducted using the random-effects model. DATA EXTRACTION: Data were extracted from published reports by two independent observers. DATA SYNTHESIS: A borderline effect was detected in the analysis of all of the trials with control groups, with a pooled odds ratio (OR for biochemical cure with SSA treatment of 1.62 (95% CI, 0.93-2.82. In the analysis of the three prospective controlled trials, a statistically significant effect was identified OR: 3.62 (95% CI, 1.88-6.96. CONCLUSIONS: Preoperative treatment with SSA og GH-secreting pituitary adenomas shows a significant improvement on surgical results. This meta-analysis suggests that in centers without optimal results all patients with a GH-secreting pituitary macroadenoma should be treated with a long-acting SSA prior to surgical treatment.

  15. Selective blockade of growth-hormone by interstitial gamma-irradiation of the pituitary in acromegaly and diabetic retinopathy

    This report documents ocular and neuroendocrinological results in a series of 185 diabetic retinopathy implanted with 198Au, the activity was respectively 16.5 +- 0.5 and 13.5 +- 0.2 mCi in 2 groups of patients. In addition long-term data resulting from 198Au stereotactic implantations in a series of 10 GH hypersecreting adenomas (with or without supra-sellar expansion) are analysed in this study and compared with cases of permanent 192Iridium and 90Yttrium implantations in acromegaly. The main finding is the absence of immediate and/or late neurological, ocular and metabolic complications. (Auth.)

  16. Pulsatile growth hormone secretion in patients with acromegaly and normal men: the effects of growth hormone-releasing hormone infusion.

    Gelato, M C; Oldfield, E; Loriaux, D L; Merriam, G R

    1990-09-01

    Twenty-four GH secretory patterns were studied before and during continuous infusions of GHRH in six patients with active acromegaly and in six normal adult men. GH release was episodic in both groups. Control subjects showed a normal diurnal variation in GH release, with the majority of GH released at night (2200-0800 h); mean levels were 1.5 +/- 0.4 (SE) ng/mL (day) and 4.2 +/- 0.8 ng/mL (night). Acromegalics had no diurnal variation in GH; levels were 45.3 +/- 13.7 ng/mL (day) and 39.8 +/- 12.2 ng/mL (night). Acromegalics demonstrated an increased frequency of GH pulses compared to normals (11.8 +/- 0.8 vs. 2.2 +/- 0.3/24 h). During continuous 24-h infusions of GHRH, the normal subjects continued to show a diurnal variation in GH release, but GH pulse frequency increased to a rate (11.7 +/- 1.4 pulses/24 h) very similar to that of the patients with acromegaly. In contrast, GHRH infusion did not alter the GH pulse frequency in the acromegalics. GHRH increased the mean levels of GH in both groups (patients 80.2 +/- 20.3 vs. 41.0 +/- 12.1 ng/mL, x +/- SE. P less than 0.05; controls 10.2 +/- 2.0 vs. 3.33 +/- 0.5 ng/mL, P less than 0.01). Some of the patients with acromegaly showed a progressive decline in GH levels during the infusion period, suggesting desensitization or exhaustion of releaseable stores; however, GH levels remained above basal values in all patients. After the 24-h GHRH infusions, the GH response to a bolus of GHRH was diminished in the normal subjects (2.1 +/- 0.9 vs. 16.8 +/- 5 ng/mL, x +/- SE; P less than 0.01) but not in the acromegalic patients (30.2 +/- 8.9 vs. 35.5 +/- 12.5 ng/mL; NS). These results indicate that GH release is episodic under basal conditions and during continuous GHRH infusion in both acromegalic and normal subjects, indicating the importance of other modulators of GH release, such as somatostatin, which may remain pulsatile even in acromegaly. PMID:2118536

  17. Interferon-β treatment associated with a biochemical profile suggestive of acromegaly. A case report of a patient treated for multiple sclerosis

    Andreassen, Mikkel; Frystyk, Jan; Miller, Karen K; Kristensen, Lars

    2010-01-01

    We describe a 34-year-old female treated with IFN-β for 8 years with a biochemical profile suggestive of acromegaly. The patient presented with elevated serum insulin-like growth factor-I (IGF-I) and insufficient suppression of growth hormone (GH) during oral glucose tolerance test (OGTT). There ...

  18. Clinical profile and outcome of patients with acromegaly according to the 2014 consensus guidelines: Impact of a multi-disciplinary team

    Pinaki Dutta

    2015-01-01

    Conclusion: Acromegaly has wide-ranging manifestations from acral enlargement to altered sensorium; incidental diagnosis was not prevalent in our series. Majority of the cases were due to the presence of a pituitary macroadenoma. Better cure rate can be achieved only when a dedicated group of multi-disciplinary team is involved.

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

    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.)

  20. Unusual cause for recurrent Cushing syndrome and its diagnosis by computed tomography and NP-59 radiocholesterol scanning

    Harris, R.D.; Herwig, K.R. (Univ. of California, Irvine, Orange (USA))

    1990-09-01

    Cushing syndrome can recur following an adrenalectomy. One of the primary causes is recurrence of adrenal carcinoma either locally or from metastases. Hyperplasia and hyperfunction of adrenal remnants may also occur if there is pituitary stimulation. We have a patient in whom recurrent Cushing syndrome developed from small nonmalignant deposits of adrenal tissue in the perirenal adipose tissue following adrenalectomy of a benign adenoma. These deposits were identifiable by computed tomography. A false-negative NP-59 iodocholesterol scan was instructive in pointing out some problems in the interpretation of this type of scan for adrenal tissue.

  1. An adolescent girl referred with Cushing syndrome--does she or does she not have the syndrome?

    Kao, Kung-Ting; Zacharin, Margaret

    2016-01-01

    Cushing syndrome and lipodystrophy syndromes share similar clinical features. This report describes an adolescent girl with newly diagnosed familial partial lipodystrophy type 2 (FPLD2) who was initially referred for Cushing syndrome. The type of abnormal fat deposition syndrome can be elucidated by careful clinical examination. FPLD2 can lead to type 2 diabetes mellitus and early cardiovascular events. Partial lipodystrophy presenting for the first time in adolescence can be mistaken for corticosteroid excess. Early diagnosis and preventative management of cardiovascular risk factors are crucial. PMID:26677885

  2. Unusual cause for recurrent Cushing syndrome and its diagnosis by computed tomography and NP-59 radiocholesterol scanning

    Cushing syndrome can recur following an adrenalectomy. One of the primary causes is recurrence of adrenal carcinoma either locally or from metastases. Hyperplasia and hyperfunction of adrenal remnants may also occur if there is pituitary stimulation. We have a patient in whom recurrent Cushing syndrome developed from small nonmalignant deposits of adrenal tissue in the perirenal adipose tissue following adrenalectomy of a benign adenoma. These deposits were identifiable by computed tomography. A false-negative NP-59 iodocholesterol scan was instructive in pointing out some problems in the interpretation of this type of scan for adrenal tissue

  3. Impacto de un programa educativo para el paciente con síndrome de Cushing: Estudio Multicéntrico

    Martínez Momblán, Mª Antonia

    2013-01-01

    INTRODUCCIÓN: El síndrome de Cushing (SC) representa una de esas enfermedades endocrinas raras, está caracterizada por una hipersecreción de cortisol; está causada por un tumor en la hipófisis (enfermedad de Cushing), o más raramente por un tumor adrenal o ectópico. La principal causa de morbilidad y mortalidad en el SC es la enfermedad cardiovascular, incluso si los pacientes han sido tratados de manera eficaz (es decir, se ha controlado el hipercortisolismo). A pesar de las múltiples compl...

  4. Ectopic Cushing' syndrome caused by a neuroendocrine carcinoma of the mesentery

    Petersenn Stephan

    2006-04-01

    Full Text Available Abstract Background ACTH overproduction within the pituitary gland or ectopically leads to hypercortisolism. Here, we report the first case of Cushing' syndrome caused by an ectopic ACTH-secreting neuroendocrine carcinoma of the mesentery. Moreover, diagnostic procedures and pitfalls associated with ectopic ACTH-secreting tumors are demonstrated and discussed. Case presentation A 41 year-old man presented with clinical features and biochemical tests suggestive of ectopic Cushing's syndrome. First, subtotal thyroidectomy was performed without remission of hypercortisolism, because an octreotide scan showed increased activity in the left thyroid gland and an ultrasound revealed nodules in both thyroid lobes one of which was autonomous. In addition, the patient had a 3 mm hypoenhancing lesion of the neurohypophysis and a 1 cm large adrenal tumor. Surgical removal of the pituitary lesion within the posterior lobe did not improve hypercortisolism and we continued to treat the patient with metyrapone to block cortisol production. At 18-months follow-up from initial presentation, we detected an ACTH-producing neuroendocrine carcinoma of the mesentery by using a combination of octreotide scan, computed tomography scan, and positron emission tomography. Intraoperatively, use of a gamma probe after administration of radiolabeled 111In-pentetreotide helped identify the mesenteric neuroendocrine tumor. After removal of this carcinoma, the patient improved clinically. Laboratory testing confirmed remission of hypercortisolism. An octreotide scan 7 months after surgery showed normal results. Conclusion This case underscores the diagnostic challenge in identifying an ectopic ACTH-producing tumor and the pluripotency of cells, in this case of mesenteric cells that can start producing and secreting ACTH. It thereby helps elucidate the pathogenesis of neuroendocrine tumors. This case also suggests that patients with ectopic Cushing's syndrome and an octreotide

  5. Ectopic Cushing' syndrome caused by a neuroendocrine carcinoma of the mesentery

    ACTH overproduction within the pituitary gland or ectopically leads to hypercortisolism. Here, we report the first case of Cushing' syndrome caused by an ectopic ACTH-secreting neuroendocrine carcinoma of the mesentery. Moreover, diagnostic procedures and pitfalls associated with ectopic ACTH-secreting tumors are demonstrated and discussed. A 41 year-old man presented with clinical features and biochemical tests suggestive of ectopic Cushing's syndrome. First, subtotal thyroidectomy was performed without remission of hypercortisolism, because an octreotide scan showed increased activity in the left thyroid gland and an ultrasound revealed nodules in both thyroid lobes one of which was autonomous. In addition, the patient had a 3 mm hypoenhancing lesion of the neurohypophysis and a 1 cm large adrenal tumor. Surgical removal of the pituitary lesion within the posterior lobe did not improve hypercortisolism and we continued to treat the patient with metyrapone to block cortisol production. At 18-months follow-up from initial presentation, we detected an ACTH-producing neuroendocrine carcinoma of the mesentery by using a combination of octreotide scan, computed tomography scan, and positron emission tomography. Intraoperatively, use of a gamma probe after administration of radiolabeled 111In-pentetreotide helped identify the mesenteric neuroendocrine tumor. After removal of this carcinoma, the patient improved clinically. Laboratory testing confirmed remission of hypercortisolism. An octreotide scan 7 months after surgery showed normal results. This case underscores the diagnostic challenge in identifying an ectopic ACTH-producing tumor and the pluripotency of cells, in this case of mesenteric cells that can start producing and secreting ACTH. It thereby helps elucidate the pathogenesis of neuroendocrine tumors. This case also suggests that patients with ectopic Cushing's syndrome and an octreotide scan positive in atypical locations may benefit from

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

    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)

  7. Adrenal scintigraphy using 131I-19-iodocholesterol in the exploration of Cushing's syndrome

    On the basis of 7 cases representing the essential types of aetiology of Cushing's syndrome, it is possible to define the value, limitations and role of adrenal scintigraphy using 131I-19-iodocholesterol in the diagnostic investigation of hypercortisolism. The essential value of the investigation is morphological rather than functional. Adrenal scintigraphy has the same role in diagnostic investigation as various radiological procedures such as pneumoperitoneum and angiography. The latter, however, are technically difficult and fraught with risk, in contrast to the isotopic examination. The principle limitations of the method are not dosimetric but lie in the relative difficulty of interpretation of the documents obtained

  8. Sustained biochemical control in patients with acromegaly treated with lanreotide depot 120 mg administered every 4 weeks, or an extended dosing interval of 6 or 8 weeks: a pharmacokinetic approach

    Gomez-Panzani E; Chang S; Ramis J; Landolfi MM; Bakker B

    2012-01-01

    Edda Gomez-Panzani,1 Stephen Chang,1 Joaquim Ramis,2 Michelle M Landolfi,1 Bert Bakker11Ipsen Biopharmaceuticals, Inc, Basking Ridge, New Jersey, USA; 2Ipsen Innovation SAS, Pharmacokinetic and Drug Metabolism, Les Ulis, FranceObjective: Lanreotide depot is a long-acting somatostatin receptor ligand injected deep subcutaneously every 4 weeks for the treatment of acromegaly. The aim of the presented studies was to establish whether lanreotide depot, administered to patients with acromegaly at ...

  9. 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

    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

  10. Cushing syndrome and the anesthesiologist, two case reports

    Rudin Domi; Hektor Sula

    2011-01-01

    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 chal...

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

    A review of the physico-chemical properties of 131I-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

  12. [Subcutaneous Taenia crassiceps-cysticercosis in a dog with Cushing's syndrome].

    Nolte, A; Strube, C; Raue, K; Brämer, C; Baumgärtner, W; Wohlsein, P

    2016-01-01

    A male, 12-year-old Cairn terrier suffering from Cushing's syndrome with two therapy-resistant inflammatory subcutaneous lesions was examined pathomorphologically and parasitologically. Within the subcutaneous tissue, there was a suppurative to necrotizing and histiocytic inflammation present with the formation of caverns. Intralesional whitish-grey cysts with a diameter of 1-4 mm were detected. Molecular investigations of the cysts confirmed the preliminary morphological identification as Cysticercus longicollis. The adenohypophysis showed an infiltrative growing carcinoma. Cysticercus longicollis is the metacestode of Taenia (T.) crassiceps, a tapeworm of foxes and coyotes. Small rodents are typical intermediate hosts, in which the metacestode develops within the body cavities as well as in the subcutis. Subcutaneous cysticercosis after infection with eggs of T. crassiceps is also described in different domestic animal species and in humans, who represent aberrant intermediate hosts. Immunosuppression due to Cushing's syndrome, probably caused by the tumor of the adenohypophysis, may have played a role in the pathogenesis of the present case. PMID:26763526

  13. A Case of Cushing's Syndrome with Multiple Adrenocortical Adenomas Composed of Compact Cells and Clear Cells.

    Asakawa, Masahiro; Yoshimoto, Takanobu; Ota, Mitsutane; Numasawa, Mitsuyuki; Sasahara, Yuriko; Takeuchi, Takato; Nakano, Yujiro; Oohara, Norihiko; Murakami, Masanori; Bouchi, Ryotaro; Minami, Isao; Tsuchiya, Kyoichiro; Hashimoto, Koshi; Izumiyama, Hajime; Kawamura, Naoko; Kihara, Kazunori; Negi, Mariko; Akashi, Takumi; Eishi, Yoshinobu; Sasano, Hironobu; Ogawa, Yoshihiro

    2016-06-01

    A 58-year-old woman was referred to our hospital for Cushingoid features and diagnosed as adrenal Cushing's syndrome due to a right adrenocortical mass (60 × 55 mm). The mass was composed of three different tumors; the first one was homogeneously lipid-poor neoplasm measuring 20 × 13 mm located at the most dorsal region, the second one was heterogeneous and lipid-rich tumor containing multiple foci of calcification measuring 50 × 32 mm located at the central region, and the last one was heterogeneous harboring dilated and tortuous vessels and lipid-poor one measuring 35 × 18 mm at the most ventral region of the adrenal gland. A right adrenalectomy was subsequently performed by open surgery. Macroscopic and microscopic analyses revealed that all three tumors were adrenocortical adenomas; the first one represents a pigmented adrenocortical adenoma, the second one adrenocortical adenoma associated with degeneration, and the third one adrenocortical adenoma harboring extensive degeneration. Immunohistochemical analysis of the steroidogenic enzymes also revealed that all of the tumors had the capacity of synthesizing cortisol. This is a very rare case of Cushing's syndrome caused by multiple adrenocortical adenomas including a pigmented adenoma. Immunohistochemical analysis of steroidogenic enzymes contributed to understanding of steroidogenesis in each of these three different adrenocortical adenomas in this case. PMID:26961704

  14. Mifepristone effects on tumor somatostatin receptor expression in two patients with Cushing's syndrome due to ectopic adrenocorticotropin secretion

    C. de Bruin (Christiaan); L.J. Hofland (Leo); L.K. Nieman; P.M. van Koetsveld (Peter); A.M. Waaijers (Annet); D. Sprij-Mooij (Diana); M. van Essen (Martijn); S.W.J. Lamberts (Steven); W.W. de Herder (Wouter); R.A. Feelders (Richard)

    2012-01-01

    textabstractContext: Two patients presented with Cushing's syndrome due to ectopic ACTH secretion. Initial localization studies included computed tomography, magnetic resonance imaging, and octreoscans (111In-pentreotide scintigraphy), which were negative in both patients. They were treated with the

  15. Metabolic complications of endogenous Cushing: patient selection for screening

    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

  16. Effects of Long-term Growth Hormone Replacement in Adults With Growth Hormone Deficiency Following Cure of Acromegaly

    Tritos, Nicholas A; Johannsson, Gudmundur; Korbonits, Márta;

    2014-01-01

    /expected = 0.58 [0.48-0.70]) in comparison with the general population. There was no difference in incidence of all cancers, benign or malignant brain tumors, or diabetes mellitus between acroGHD and NFPA. CONCLUSIONS: GH replacement has comparable effects on quality of life and lipids in acroGHD and NFPA......CONTEXT: GH deficiency (GHD) may occur in adults with cured acromegaly (acroGHD). OBJECTIVE: Our objective was to examine the effectiveness and safety of GH replacement in acroGHD. DESIGN: This study was a retrospective analysis of data from KIMS (Pfizer International Metabolic Database). SETTING......: Data were extracted from a pharmaco-epidemiological survey of >16 000 GHD adults from 31 countries. PATIENTS: The effectiveness population included 115 adults with acroGHD and 142 age-, gender-, and body mass index-matched GHD adults with nonfunctioning pituitary adenoma (NFPA) followed up to 5 years...

  17. Effect of pasireotide on glucose- and growth hormone-related biomarkers in patients with inadequately controlled acromegaly.

    Schmid, Herbert A; Brue, Thierry; Colao, Annamaria; Gadelha, Mônica R; Shimon, Ilan; Kapur, Karen; Pedroncelli, Alberto M; Fleseriu, Maria

    2016-07-01

    The purpose of this study was to gain more insight into the mechanism of action of pasireotide in patients who completed the PAOLA study. PAOLA was a 24-week, Phase III, randomized, three-arm study of pasireotide LAR 40 and 60 mg versus octreotide LAR 30 mg or lanreotide Autogel 120 mg in patients with inadequately controlled acromegaly. The current work was a planned exploratory objective of the PAOLA study that evaluated changes in levels of growth hormone (GH), insulin-like growth factor 1 (IGF-1), IGF-binding proteins (IGFBP-2, IGFBP-3), glycated haemoglobin (HbA1c) and fasting plasma glucose (FPG) in each treatment arm. Responders to pasireotide LAR (mean GH levels <2.5 μg/L and normal IGF-1 levels at 24 weeks) had lower GH and IGF-1 levels at baseline (GH 5.1 ng/mL, IGF-1 519 ng/mL) than non-responders (GH 7.9 ng/mL, IGF-1 672 ng/mL). Frequency of hyperglycaemia after pasireotide treatment was similar in responders and non-responders and depended more on the baseline FPG level. 47 % of all patients treated with pasireotide LAR (40 or 60 mg) did not receive antidiabetic medication at any time during this study. This is the first study to evaluate the treatment effect of pasireotide on key hormonal and glycaemic biomarkers and to identify potential predictors of pasireotide-associated hyperglycaemia. Pre-treatment glucose status may be predictive of the development of pasireotide-associated hyperglycaemia. A large subset of patients with acromegaly does not experience major disturbances in glucose homeostasis while receiving pasireotide LAR. PMID:26906713

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

    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. PMID:15816375

  19. Management Strategies for Aggressive Cushing's Syndrome: From Macroadenomas to Ectopics

    Carlotta Pozza

    2012-01-01

    Full Text Available Cushing’s syndrome (CS is a rare but severe clinical condition represented by an excessive endogenous cortisol secretion and hence excess circulating free cortisol, characterized by loss of the normal feedback regulation and circadian rhythm of the hypothalamic-pituitary axis due to inappropriate secretion of ACTH from a pituitary tumor (Cushing’s disease, CD or an ectopic source (ectopic ACTH secretion, EAS. The remaining causes (20% are ACTH independent. As soon as the diagnosis is established, the therapeutic goal is the removal of the tumor. Whenever surgery is not curative, management of patients with CS requires a major effort to control hypercortisolemia and associated symptoms. A multidisciplinary approach that includes endocrinologists, neurosurgeons, oncologists, and radiotherapists should be adopted. This paper will focus on traditional and novel medical therapy for aggressive ACTH-dependent CS. Several drugs are able to reduce cortisol levels. Their mechanism of action involves blocking adrenal steroidogenesis (ketoconazole, metyrapone, aminoglutethimide, mitotane, etomidate or inhibiting the peripheral action of cortisol through blocking its receptors (mifepristone “RU-486”. Other drugs include centrally acting agents (dopamine agonists, somatostatin receptor agonists, retinoic acid, peroxisome proliferator-activated receptor γ “PPAR-γ” ligands and novel chemotherapeutic agents (temozolomide and tyrosine kinase inhibitors which have a significant activity against aggressive pituitary or ectopic tumors.

  20. Cushing syndrome and the anesthesiologist, two case reports

    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.

  1. A 12-month randomized crossover study on the effects of Lanreotide Autogel and Octreotide long-acting repeatable on GH and IGF-l in patients with acromegaly

    Andries, Magdalene; Glintborg, Dorte; Kvistborg, Annette;

    2007-01-01

    Background Somatostatin analogues have been used successfully for the treatment of acromegaly but no randomized studies have compared the effects of lanreotide Autogel (LAN) and octreotide acetate long-acting repeatable (OCT). Objective To compare the effect of LAN and OCT for the treatment of...... acromegaly in a randomized study design. Material and methods Twelve acromegalic patients were included and 10 patients completed treatment with LAN or OCT for 6 months and were then switched to the opposite treatment modality for 6 months without a washout period in a randomized crossover design. GH and IGF...... normalized IGF-I levels during both treatment regimes. Two patients on LAN and one on OCT had normalized IGF-I levels during one treatment and not during the other. In three patients, IGF-I levels were elevated during both therapies. Four patients developed palpable nodules, two patients on LAN and two...

  2. Serial follow-up of presurgical treatment using pasireotide long-acting release with or without octreotide long-acting release for naïve active acromegaly.

    Chang, Jan-Shun; Tseng, Ham-Min; Chang, Tien-Chun

    2016-06-01

    The aim of the present study was to evaluate the serial changes of GH and IGF-1 in seven patients with naïve, active acromegaly following presurgical treatment of the somatostatin analog pasireotide long-acting release (LAR) and octreotide LAR. The patients were treated with pasireotide LAR with or without octreotide LAR for two years and underwent transsphenoidal adenomectomy. After treatment with the somatostatin analogs, the surgical cure rate was similar to that in patients who underwent transsphenoidal surgery alone. Diabetes insipidus was not identified in any patients after the operation. Pasireotide LAR was effective on GH as well as IGF-1 suppression and tumor size decreasing when used as the primary therapy. Future large-population studies to investigate the surgical curative rate after presurgical treatment with somatostatin analogs in patients with acromegaly and macroadenomas close to the cavernous sinus are warranted. However, that hyperglycemia developed following pre-surgical treatment with pasireotide should take into consideration. PMID:27117887

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

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

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

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

    1997-03-01

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

  5. Macht das Subklinische Cushing-Syndrom krank? Untersuchung zur kardio-metabolischen Morbidität anhand des Patientenkollektivs des Universitätsklinikums Würzburg

    Klör, Miriam

    2014-01-01

    Durch zunehmenden, breiteren Einsatz bildgebender Verfahren werden immer häufiger Nebennierenraumforderungen zufällig entdeckt, sog. Inzidentalome. Ist für Tumorentitäten wie dem Conn-Adenom, adrenocorticalen Carcinom, Phäochromozytom oder manifesten Cushing-Syndrom der Krankheitswert belegt, ist dies bei den anderen Nebennierenadenomen unklarer. Diese Adenome sind in der Regel hormoninaktiv; eine Subgruppe jedoch weist ein sog. Subklinisches Cushing-Syndrom auf, beim welchem autonom Cortisol...

  6. Clinical and radiological evidence of the recurrence of reversible pegvisomant-related lipohypertrophy at the new site of injection in two women with acromegaly: a case series

    Rochira Vincenzo; Zirilli Lucia; Diazzi Chiara; Romano Stefania; Carani Cesare

    2012-01-01

    Abstract Introduction Pegvisomant-related lipohypertrophy may revert when changing the site of injection, but the lipohypertrophy may recur at the new site of injection. The strength of evidence, however, is weak and comes from information obtained from physical examination only. Case presentation We studied two Caucasian women with acromegaly, aged 51 and 71 years, with pegvisomant-related lipohypertrophy. Our two patients were evaluated at baseline, when the site of pegvisomant injection wa...

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

    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.

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

    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. The...... 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...

  9. Is it worthwhile to screen patients with type 2 diabetes mellitus for subclinical Cushing's syndrome?

    Budyal, Sweta; Jadhav, Swati Sachin; Kasaliwal, Rajeev; Patt, Hiren; Khare, Shruti; Shivane, Vyankatesh; Lila, Anurag R; Bandgar, Tushar; Shah, Nalini S

    2015-12-01

    Variable prevalence of subclinical Cushing's syndrome (SCS) has been reported in patients with type 2 diabetes mellitus (T2DM), making the need for screening in this population uncertain. It is unknown if this variability is solely due to study-related methodological differences or a reflection of true differences in ethnic predisposition. The objective of this study is to explore the prevalence of SCS in Asian Indian patients with T2DM. In this prospective single center study conducted in a tertiary care referral center, 993 T2DM outpatients without any discriminatory clinical features (easy bruising, facial plethora, proximal muscle weakness, and/or striae) of hypercortisolism underwent an overnight 1 mg dexamethasone suppression test (ODST). ODST serum cortisol ≥1.8 μg/dl was considered positive, and those with positive results were subjected to 48 h, 2 mg/day low dose DST (LDDST). A stepwise evaluation for endogenous hypercortisolism was planned for patients with LDDST serum cortisol ≥1.8 μg/dl. Patients with positive ODST and negative LDDST were followed up clinically and re-evaluated a year later for the development of clinically evident Cushing's syndrome (CS). In this largest single center study reported to date, we found 37 out of 993 (3.72%) patients had ODST serum cortisol ≥1.8 μg/dl. None of them had LDDST cortisol ≥1.8 μg/dl, nor did they develop clinically evident CS over a follow-up period of 1 year. Specificity of ODST for screening of CS was 96.3% in our cohort. None of the T2DM outpatients in our cohort had SCS, hence cautioning against routine biochemical screening for SCS in this cohort. We suggest screening be based on clinical suspicion only. PMID:26420669

  10. Regional cerebral blood flow and CSF pressures during Cushing response induced by a supratentorial expanding mass

    In order to delineate the critical blood flow pattern during the Cushing response in intracranial hypertension, regional cerebral blood flow was measured with radioactive microspheres in 12 anesthetized dogs at respiratory arrest caused either by expansion of an epidural supratentorial balloon or by cisternal infusion. Regional cerebrospinal fluid pressures were recorded and the local cerebral perfusion pressure calculated in various cerebrospinal compartments. In the 8 dogs of the balloon expansion group, the systemic arterial pressure was unmanipulated in 4, while it was kept at a constant low level (48 and 70 mm Hg) in 2 dogs and, in another 2 dogs, at a constant high level (150 and 160 mm Hg) induced by infusion of Aramine. At respiratory arrest, regional cerebral blood flow had a stereotyped pattern and was largely independent of the blood pressure level. In contrast, concomitant pressure gradients between the various cerebrospinal compartments varied markedly in the 3 animal groups increasing with higher arterial pressure. Flow decreased by 85-100% supratentorially and by 70-100% in the upper brain stem down to the level of the upper pons, while changes in the lower brain stem were minor, on the average 25%. When intracranial pressure was raised by cisternal infusion in 4 dogs, the supratentorial blood flow pattern at respiratory arrest was appriximately similar to the flow pattern in the balloon inflation group. However, blood flow decreased markedly (74-85%) also in the lower brain stem. The results constitute another argument in favour of the Cushing response in supratentorial expansion being caused by ischemia in the brain stem. The critical ischemic region seems to be located rostrally to the oblongate medulla, probably in the pons. (author)

  11. Delayed radiation necrosis of the central nervous system in patients irradiated for pituitary tumours

    Grattan-Smith, P.J.; Morris, J.G.; Langlands, A.O. (Westmead Hospital, Sydney (Australia))

    1992-10-01

    Four cases of delayed radiation necrosis involving the CNS were found in a group of 46 patients irradiated for pituitary tumours over a six year period. This occurred in three of 11 patients with Cushing's disease representing an incidence of 27% in this group. There were no cases among 11 patients with acromegaly or among seven with prolactinomas. One case (6%) was found in the 17 patients with chromophobe adenomas. Standard doses of radiation were delivered to these patients and the findings support suggestions that the metabolic disturbances of Cushing's disease may reduce tolerance to radiation. Our results and a literature review indicate that if radiotherapy is used to treat Cushing's disease, the total dose should be less than 50 Gy at 2 Gy per day fractionation. (Author).

  12. Cushing Syndrome

    ... links Share this: Page Content What is Cushing’s syndrome? Cushing’s syndrome is a condition that occurs when the body’s ... medication or as a result of a tumor, Cushing’s syndrome can develop. Many factors influence whether this happens, ...

  13. Cushing syndrome

    ... remove the tumor. Radiation after removal of a pituitary tumor in some cases. Cortisol replacement therapy after surgery ... include any of the following: Diabetes Enlargement of pituitary tumor Fractures due to osteoporosis High blood pressure Kidney ...

  14. Cushing's Syndrome

    ... If surgery fails or only produces a temporary cure, surgery can be repeated, often with good results. After curative pituitary surgery, the production of ACTH drops two levels below normal. This drop is natural and temporary, and patients are given a synthetic ...

  15. Widespread reductions of white matter integrity in patients with long-term remission of Cushing's disease

    Steven J.A. van der Werff

    2014-01-01

    Conclusion: Patients with a history of endogenous hypercortisolism in present remission show widespread changes of white matter integrity in the brain, with abnormalities in the integrity of the uncinate fasciculus being related to the severity of depressive symptoms, suggesting persistent structural effects of hypercortisolism.

  16. Antiphospholipid Syndrome: primary or secondary to Systemic Lupus Erythematosus? Description of a clinical case of avitaminosis D in premenopausal woman with pseudo-Cushing syndrome

    Mauro Turrin

    2014-06-01

    Full Text Available Low vitamin D levels have been described in obese individuals and in some autoimmune diseases, such as Systemic Lupus Erythematosus (SLE and primary antiphospholipid syndrome (pAPS. In particular, more than 50% of premenopausal women with pAPS have hypovitaminosis D. In this issue we report a case of an obese, premenopausal, and hypertensive woman with pseudo-Cushing syndrome, affected by deep venous thrombosis associated with pulmonary embolism after rib fracture who presented hypovitaminosis D. 7 years before, diagnosis of pAPS had been made after the detection of thrombocytopenia (present at a young age and arterial ischemia of a lower limb. For seven years she was treated with acetylsalicylic acid without complications. We found positive anti-dsDNA antibodies, a triple antiphospholipid antibodies (aPL positivity and levels of vitamin D < 4 µg/l. The case report arises some questions: is vitamin D deficiency due to obesity or APS? Is the positivity of anti-dsDNA indicative of progression to SLE? Is preventive therapy with hydroxychloroquine indicated? Does the high-risk aPL profile justify a high-intensity and life-long anticoagulation regimen?http://dx.doi.org/10.7175/cmi.v8i2.912

  17. Primary pigmented nodular adrenocortical disease presenting with a unilateral adrenocortical nodule treated with bilateral laparoscopic adrenalectomy: a case report

    Kaltsas Gregory

    2010-07-01

    Full Text Available Abstract Introduction Primary pigmented nodular adrenocortical disease is a rare cause of adrenocorticotropic hormone-independent Cushing's syndrome. We report an uncommon primary pigmented nodular adrenocortical disease case presenting with a unilateral adrenocortical nodule and provide a brief overview of the existing literature. Case presentation A 27-year-old Caucasian woman was admitted to our Department with adrenocorticotropic hormone-independent Cushing's syndrome. Its cause was initially considered a left adrenocortical adenoma based on computer tomography imaging. The patient underwent left laparoscopic adrenalectomy and histological examination revealed pigmented micronodular adrenal hyperplasia. Evaluation for the presence of Carney complex was negative. Six months later recurrence of hypercortisolism was documented and a right laparoscopic adrenalectomy was performed further establishing the diagnosis of primary pigmented nodular adrenocortical disease. After a nine-year follow-up there is no evidence of residual disease. Conclusions Even though primary pigmented nodular adrenocortical disease is a rare cause of Cushing's syndrome, it should be included in the differential diagnosis of adrenocorticotropic hormone-independent Cushing's syndrome, especially because adrenal imaging can be misleading mimicking other adrenocortical diseases. Bilateral laparoscopic adrenalectomy is the preferred treatment in these subjects.

  18. Alternative interpretations, history, and experiment: Reply to Cushing, Crease, Bevilacqua, and Giannetto

    Eger, Martin

    1995-04-01

    Cushing's contribution on Bohm's deterministic interpretation of quantum mechanics raises the question of whether self-understanding is at issue in physical science as it is in social science. This question is pursued further in the present paper. Bevilacqua's and Giannetto's belief in the importance of history and the lifeworld of the researcher is one I share; but their prescription of the historical context as the only context of interest leads to an extended discussion of the several types of philosophical hermeneutics now practiced, and of other existing links between science and the lifeworld. Historical studies, I argue, should be used to interpret current texts, current concerns, even current textbooks, not to replace any of these. While I do not share Robert Crease's belief in the primacy of experiment, I agree that it is fundamental in natural science, and must not be neglected in favor of theory. However, the pervasive nature of theoretical languages, their role in understanding experiment and in communicating results, still justify a special hermeneutical concern for this aspect of science, particularly in education.

  19. Cases of Psychiatric Morbidity in Pediatric Patients After Remission of Cushing Syndrome.

    Keil, Margaret F; Zametkin, Alan; Ryder, Celia; Lodish, Maya; Stratakis, Constantine A

    2016-04-01

    Endogenous Cushing syndrome (CS) may have different effects in children than what has been described in adults. Previous studies of children and adolescents with CS have identified cognitive decline despite reversal of brain atrophy after remission of CS. Although the observations of parents of children and adolescents with CS support personality changes, significant psychopathology has not been described in the literature. We report 9 children who underwent successful surgery (transsphenoidal surgery [TSS] or resection of bronchial carcinoid) for treatment of CS and subsequently developed significant affective pathology. Affective symptoms included anger-rage outbursts, suicidal ideation, irritability, anxiety, and depression. One child, who committed suicide 60 months after TSS, had recently discontinued antidepressant medication. She had a history of anxiety during active CS and was treated with an anxiolytic. The 7 patients with onset of symptoms within 7 months of TSS were on glucocorticoid replacement, and 1-year follow-up evaluation showed recovery of hypothalamic-pituitary-adrenal axis and biochemical evidence of remission. The 2 patients who presented with onset of symptoms at 48 months or later underwent endocrine evaluation that showed biochemical evidence of remission and normal anterior pituitary hormone levels. This is the first report of affective symptoms and behavioral dysregulation, including suicidal ideation, in a subgroup of children and adolescents after remission of CS. Health care providers caring for children with CS who have been cured should continue to screen for mental illness, monitor for changes in behavior, and refer as appropriate to mental health professionals. PMID:27025959

  20. 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

    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

  1. Treatment of pituitaary dependent cushing's syndrome with closed stereotactic radiosurgery by means of 60Co gamma radiation

    Four patients with pituitary dependent Cushing's syndrome treated with external irradiation to the pituitary using 60Co gamma irradiation given with a stereotactic technique. The size of the sella turcica was normal or slightly enlarged in all patients. The doses given varied between 7000 and 10000 rad, and the observation time ranged between 14 and 20 months. Three of the patients showed complete clinical remission and one marked improvement. One patient developed ACTH insufficiency, while none developed insufficient secretion of other pituitary hormones. No complications of the irradiation were observed. (author)

  2. Lupus eritematoso sistémico síndrome de cushing y psiquiatría

    Pájaro Silva, Ludwig

    2014-01-01

    Se presenta el caso de una paciente con diagnósticode lupus eritematoso sistémico y síndromede Cushing quien presentó síntomas afectivosde características depresivas e insomnio de conciliación,quien recibió múltiples tratamientosfarmacológicos con pobre respuesta inicial. Lapsiquiatría de enlace consolida una alternativade tratamiento multidisciplinario para una aproximaciónmás eficiente a las patologías médicascon síntomas psiquiátricos.

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

    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 131I-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 131I-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. 131I-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 131I-adosterol scintigraphy and without suppression of serum ACTH may not require steroid hormone

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

    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.

  5. Cushing's Syndrome caused by pigmented adrenocortical micro nodular dysplasia - A familial case

    Introduction: We present a Case of Cushing's syndrome (CS) in a 16 year old male adolescent. Adrenocortical micro nodular dysplasia is a rare cause of CS. It mostly develops in the first two decades of life. In pathogenesis a stimulatory effect of circulating Immunoglobulins on adrenal steroidogenesis has been postulated. Familial cases have been reported in relation to Carney's Syndrome. We report the clinical case at first diagnosis and preoperative follow up of 1 year prior to treatment. The leading symptoms were severe bilateral (fibrotic) gynaecomastia, weight gain and growth retardation, without hypertension,but osteoporosis, secondary hypogonadism and glucose intolerance. Laboratory findings and the results of functional tests were diagnostic for CS. In addition LDH (I-131 Isotopes), CK, Lipoproteins, GPT, Androstendion, Prolactin were elevated. MRI abdomen revealed a slight enlargement of the adrenals, and suspected a bilateral micro nodular dysplasia. Iodo-cholesterol-scan under dexamethason suppression showed a diffuse bilateral Iodo-cholesterol uptake confirming the autonomous production of cortisol bilateral in the adrenals.Whole body bone scan showed a diffuse reduced diphosphonate uptake in the skeleton and the growth plates. The bone mineral density was significantly reduced.Radiologically osteoporosis was overt. The rapid increase of free urinary cortisol excretion/24h within one year of observation led to a total bilateral adrenalectomy. Postoperative 5 year follow up examinations. Documentation of the outcome and recovery of clinical signs,symptoms and laboratory findings, discussion about the most appropriate long-term substitution therapy. Familial anamnesis:affected family member was the father (micro nodular bilateral adrenocortical dysplasia), the aunt (pararenal incidentaloma, histologically lipoma) and a cousin (micro nodular adrenocortical dysplasia). Sequential analysis of the menin gene from the patient was negative.The detection of

  6. [Other specific types of diabetes].

    Vila, Greisa; Gessl, Alois W; Riedl, Michaela; Luger, Anton

    2016-04-01

    Numerous endocrine diseases are associated with impaired glucose metabolism and can induce diabetes mellitus. With the exception of hyperthyroidism, where this is uncommon, these diseases are rare. Acromegaly and Cushing syndrome are frequently associated with impaired glucose tolerance and diabetes. In contrast, this is a rare finding in pheochromocytoma and Conn syndrome. Among the many drugs that can induce diabetes this can be observed most frequently with hormones, atypic antipsychotic drugs and immunosuppressives. In addition, diseases of the pancreas such as pancreatitis, pancreatic carcinoma, cystic fibrosis and hemochromatosis can cause diabetes as well as Down syndrome, Klinefelter syndrome, Turner syndrome and Prader Willi syndrome and rare immunmediated or genetic syndromes. PMID:27056389

  7. Circadian variation in serum free and total insulin-like growth factor (IGF)-I and IGF-II in untreated and treated acromegaly and growth hormone deficiency

    Skjaerbaek, Christian; Frystyk, Jan; Kaal, Andreas; Laursen, Torben; Møller, Jens; Weeke, Jørgen; Jørgensen, Jens Otto Lunde; Christiansen, Jens Sandahl; Ørskov, Hans

    2000-01-01

    Abstract OBJECTIVE: It is generally accepted that there is no clinically significant circadian variation in total insulin-like growth factor (IGF)-I or total IGF-II in healthy subjects. In contrast there is a significant nocturnal decrease in free IGF-I in healthy subjects, corresponding to the...... nocturnal increase in IGF binding protein-1. In this study we have investigated the circadian variation in circulating free IGF-I and IGF-II in patients with acromegaly and patients with adult onset growth hormone deficiency. PATIENTS: Seven acromegalic patients were studied with and without treatment with...... a slow-release formulation of octreotide. Seven GH-deficient patients were studied without GH replacement. In addition 5 of the GH-deficient patients were studied during GH replacement. DESIGN: Serum samples were obtained every hour for 24 h. Free IGF-I and IGF-II were measured every 2nd hour. Total...

  8. Correlations between forced oscillation technique parameters and pulmonary densitovolumetry values in patients with acromegaly.

    Camilo, G B; Carvalho, A R S; Machado, D C; Mogami, R; Kasuki, L; Gadelha, M R; Melo, P L; Lopes, A J

    2015-10-01

    The aims of this study were to evaluate the forced oscillation technique (FOT) and pulmonary densitovolumetry in acromegalic patients and to examine the correlations between these findings. In this cross-sectional study, 29 non-smoking acromegalic patients and 17 paired controls were subjected to the FOT and quantification of lung volume using multidetector computed tomography (Q-MDCT). Compared with the controls, the acromegalic patients had a higher value for resonance frequency [15.3 (10.9-19.7) vs 11.4 (9.05-17.6) Hz, P=0.023] and a lower value for mean reactance [0.32 (0.21-0.64) vs 0.49 (0.34-0.96) cm H2O/L/s2, P=0.005]. In inspiratory Q-MDCT, the acromegalic patients had higher percentages of total lung volume (TLV) for nonaerated and poorly aerated areas [0.42% (0.30-0.51%) vs 0.25% (0.20-0.32%), P=0.039 and 3.25% (2.48-3.46%) vs 1.70% (1.45-2.15%), P=0.001, respectively]. Furthermore, the acromegalic patients had higher values for total lung mass in both inspiratory and expiratory Q-MDCT [821 (635-923) vs 696 (599-769) g, P=0.021 and 844 (650-945) vs 637 (536-736) g, P=0.009, respectively]. In inspiratory Q-MDCT, TLV showed significant correlations with all FOT parameters. The TLV of hyperaerated areas showed significant correlations with intercept resistance (rs=-0.602, P<0.001) and mean resistance (rs=-0.580, P<0.001). These data showed that acromegalic patients have increased amounts of lung tissue as well as nonaerated and poorly aerated areas. Functionally, there was a loss of homogeneity of the respiratory system. Moreover, there were correlations between the structural and functional findings of the respiratory system, consistent with the pathophysiology of the disease. PMID:26445330

  9. Correlations between forced oscillation technique parameters and pulmonary densitovolumetry values in patients with acromegaly

    G.B. Camilo

    2015-01-01

    Full Text Available The aims of this study were to evaluate the forced oscillation technique (FOT and pulmonary densitovolumetry in acromegalic patients and to examine the correlations between these findings. In this cross-sectional study, 29 non-smoking acromegalic patients and 17 paired controls were subjected to the FOT and quantification of lung volume using multidetector computed tomography (Q-MDCT. Compared with the controls, the acromegalic patients had a higher value for resonance frequency [15.3 (10.9-19.7 vs 11.4 (9.05-17.6 Hz, P=0.023] and a lower value for mean reactance [0.32 (0.21-0.64 vs 0.49 (0.34-0.96 cm H2O/L/s2, P=0.005]. In inspiratory Q-MDCT, the acromegalic patients had higher percentages of total lung volume (TLV for nonaerated and poorly aerated areas [0.42% (0.30-0.51% vs 0.25% (0.20-0.32%, P=0.039 and 3.25% (2.48-3.46% vs 1.70% (1.45-2.15%, P=0.001, respectively]. Furthermore, the acromegalic patients had higher values for total lung mass in both inspiratory and expiratory Q-MDCT [821 (635-923 vs 696 (599-769 g, P=0.021 and 844 (650-945 vs 637 (536-736 g, P=0.009, respectively]. In inspiratory Q-MDCT, TLV showed significant correlations with all FOT parameters. The TLV of hyperaerated areas showed significant correlations with intercept resistance (rs=−0.602, P<0.001 and mean resistance (rs=−0.580, P<0.001. These data showed that acromegalic patients have increased amounts of lung tissue as well as nonaerated and poorly aerated areas. Functionally, there was a loss of homogeneity of the respiratory system. Moreover, there were correlations between the structural and functional findings of the respiratory system, consistent with the pathophysiology of the disease.

  10. Pamidronic acid and cabergoline as effective long-term therapy in a 12-year-old girl with extended facial polyostotic fibrous dysplasia, prolactinoma and acromegaly in McCune-Albright syndrome: a case report

    Classen Carl; Mix Monika; Kyank Ulrike; Hauenstein Christina; Haffner Dieter

    2012-01-01

    Abstract Introduction McCune-Albright syndrome is a complex inborn disorder due to early embryonal postzygotic somatic activating mutations in the GNAS1 gene. The phenotype is very heterogeneous and includes polyostotic fibrous dysplasia, typically involving the facial skull, numerous café-au-lait spots and autonomous hyperfunctions of several endocrine systems, leading to hyperthyroidism, hypercortisolism, precocious puberty and acromegaly. Case presentation Here, we describe a 12-year-old C...

  11. Análisis coste-beneficio y eficacia de las pruebas utilizadas en el diagnóstico diferencial del síndrome de Cushing

    Puig de Dou, Jaume

    2001-01-01

    Objetivo: 1) Evaluar la eficacia diagnóstica y costes de las distintas pruebas bioquímicas y radiológicas utilizadas en el diagnóstico etiológico del síndrome de Cushing. 2) Establecer, según las pruebas evaluadas, el algoritmo diagnóstico más eficaz y eficiente. MATERIAL Y MÉTODOS: Se realizaron a noventa pacientes con un síndrome de Cushing (80 mujeres) diagnosticado entre 1982 y 1999, edad media de 35.7 ± 13.1 años: 1) Pruebas bioquímicas: ACTH basal, prueba de frenación fuerte con dexamet...

  12. Acromegaly (Beyond the Basics)

    ... Mental fogginess or mood disturbances • Fatigue • Sinus congestion • Bad dreams • Constipation, which is uncommon but can be ... on UpToDate's then-current terms. UpToDate may embed technology in the Licensed Materials causing them to become ...

  13. The first description of metyrapone use in severe Cushing Syndrome due to ectopic ACTH secretion in an infant with immature sacrococcygeal teratoma. Case Report.

    Wojcik, Malgorzata; Kalicka-Kasperczyk, Anna; Luszawska-Kutrzeba, Teresa; Balwierz, Walentyna; Starzyk, Jerzy B

    2015-12-01

    Cushing syndrome due to ectopic secretion of ACTH in infants is rare. The treatment of choice is radical resection of the tumour in combination with pre-operative chemotherapy using steroidogenesis inhibitors if necessary. If radical surgery is not possible, palliative treatment of hypercortisolemia is recommended. The most frequently used drug in infants is ketoconazole. Experience with the use of metyrapone is poor. We report an 8-month-old female infant with congenital immature sacrococcygeal teratoma secreting AFP, beta hCG and ACTH who had undergone non-radical resection of the tumour mass and was receiving standard risk chemotherapy (vinblastine, bleomycin, and cisplatin). The infant initially presented at the age of 6 months with ACTH-dependent Cushing syndrome (cortisol and ACTH level 325 ng/mL, 112 pg/mL respectively). Treatment with ketoconazole was initiated with a dose of 600 mg/day. Due to its ineffectiveness metyrapne was added in increasing dosages, up to 1,500 mg/day. In addition the schema of chemotherapy was changed (adriamycin, bleomycin, carboplatin), which resulted in normalization of cortisol levels and blood pressure. There were no metyrapone side effects during the treatment period. We can conclude that treatment with metyrapone at a dose of 1500 mg/day might be effective and safe in infants with Cushing syndrome. PMID:26859587

  14. 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

    Dayse Caldas

    2001-01-01

    Full Text Available Os glicocorticóides têm sido largamente utilizados na prática clínica desde os princípios de 1950. Enquanto são bem conhecidos os efeitos adversos relacionados ao seu uso oral ou parenteral, não existem muitos relatos na literatura sobre efeitos adversos relacionados ao seu uso tópico. Relatamos o caso clínico de um rapaz de 16 anos com rinite alérgica que desenvolveu síndrome de Cushing exógena por uso abusivo de descongestionante nasal à base de dexametasona numa dose diária que variava de 0,5-0,7mg durante um período de 4 anos. O desmame do glicocorticóide foi iniciado substituindo-se o descongestionante em uso por outro sem glicocorticóide associado a doses progressivamente decrescentes de dexametasona administrada via oral, medicação esta que foi definitivamente suspensa 3 meses após o início do desmame - época na qual foi constatada recuperação do eixo hipotálamo-hipófise-adrenal. Chamamos a atenção para a importância do acompanhamento criterioso dos pacientes em uso, a longo prazo, de toda e qualquer medicação que contenha glicocorticóide, eventualmente até com monitorização mais rigorosa através da determinação do cortisol livre urinário ou do cortisol plasmático após teste de estímulo com o ACTH dose baixa (1mcg, permitindo assim o diagnóstico de insuficiência adrenal, mesmo que incipiente, mas que na presença de stress leve a moderado poderia oferecer risco de vida. Diante da possibilidade de severos efeitos colaterais sistêmicos mesmo quando utilizados esteróides nasais, devemos sempre que possível dar preferência aos glicocorticóides de menor biodisponibilidade e insistir junto ao paciente para que seja respeitada a dose assim como o tempo de uso prescrito.Glucocorticoids have been largely used in the clinical practice since the early 1950s. While unwanted side effects related to its oral or parenteral use are well known, there are not many reports about such effects when

  15. 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

    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

  16. Pregnancy and pituitary adenomas.

    Glezer, Andrea; Jallad, Raquel S; Machado, Marcio C; Fragoso, Maria C; Bronstein, Marcello D

    2016-09-01

    Infertility is frequent in patients harboring pituitary adenomas. The mechanisms involved include hypogonadism secondary to hormonal hypersecretion (prolactin, growth hormone and cortisol), stalk disconnection and pituitary damage. With the improvement of clinical and surgical treatment, pregnancy in women harboring pituitary adenomas turned into a reality. Pituitary hormonal hyper- and hyposecretion influences pregnancy outcomes, as well as pregnancy can interfere on pituitary tumors, especially in prolactinomas. We review literature about specific follow-up and management in pregnant women harboring prolactinomas, acromegaly, or Cushings disease and the impact of clinical and surgical treatment on each condition. PMID:26977888

  17. Guías para el diagnóstico y tratamiento de acromegalia, prolactinomas y enfermedad de Cushing

    Chen-Ku Chih Hao

    2004-10-01

    Full Text Available Los tumores hipofisiarios funcionantes más frecuentes son los productores de prolactina (prolactinomas, hormona de crecimiento (acromegalia y ACTH (síndrome de Cushing. Existe mucha controversia con respecto al diagnóstico y manejo de estas patologías. Este artículo presenta una guía para el diagnóstico y tratamiento de tales tumores hipofisiarios, y ha sido elaborado por la Asociación Costarricense de Endocrinología, Diabetes y Nutrición. La causa más frecuente de acromegalia son los tumores hipofisiarios productores de hormona de crecimiento. El diagnóstico usualmente se retarda varios años luego de haberse iniciado los cambios, por lo lento que se producen las manifestaciones clínicas. El tratamiento inicial de elección consiste en la cirugía, reservándose tratamiento médico con análogos de somatostatina como primera elección, para casos donde ha fallado el tratamiento quirúrgico. Otra posibilidad de tratamiento consiste en la radioterapia, cuya eficacia se ve limitada por el tiempo necesario para hacer efecto. Con respecto a las hiperprolactinemias, se deben descartar otras causas que produzcan elevación de prolactina. Si se identifica un prolactinoma, el tratamiento va a ser médico en la mayoría de los casos, reservándose la cirugía para aquellos macroprolactinomas con compromiso visual que no han reducido de tamaño con el tratamiento médico. El tratamiento de elección consistió en agonistas dopaminérgicos, y en Costa Rica la elección será bromocriptina, aunque hay otras opciones como cabergolina y quinagolide. En la enfermedad de Cushing existen diferentes pruebas de diagnóstico, tanto para tamizaje como para diagnóstico confirmatorio. El tratamiento inicial debe ser cirugía, seguida por radioterapia. En este caso el tratamiento médico es mucho menos eficaz que en las otras dos entidades.

  18. Pediatric Cushing′s disease: Management Issues

    Martin O Savage

    2012-01-01

    Full Text Available 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.

  19. Clinical and radiological evidence of the recurrence of reversible pegvisomant-related lipohypertrophy at the new site of injection in two women with acromegaly: a case series

    Rochira Vincenzo

    2012-01-01

    Full Text Available Abstract Introduction Pegvisomant-related lipohypertrophy may revert when changing the site of injection, but the lipohypertrophy may recur at the new site of injection. The strength of evidence, however, is weak and comes from information obtained from physical examination only. Case presentation We studied two Caucasian women with acromegaly, aged 51 and 71 years, with pegvisomant-related lipohypertrophy. Our two patients were evaluated at baseline, when the site of pegvisomant injection was the periumbilical abdominal region, and then four months after switching the injection site from the abdomen to both thighs. Both physical examination and radiological studies (magnetic resonance imaging and dual energy X-ray absorptiometry demonstrated that the abdominal lipohypertrophy progressively reverted in both patients after switching the site of injection to the thighs. However, lipohypertrophy reappeared at the new site of injection. The radiological outcome confirmed the reversibility of pegvisomant-related lipohypertrophy and strengthened the body of evidence on this issue. Conclusion In clinical practice, physical examination of the injection site or sites leads to an early detection of lipohypertrophy during pegvisomant treatment. Radiological procedures may be of help to confirm subcutaneous fat changes and for a precise monitoring of fat redistribution. Patients should get appropriate information about lipohypertrophy before starting pegvisomant treatment since the rotation of the site of injection may prevent lipohypertrophy.

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

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

    2015-10-01

    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

  1. Adrenal scintigraphy with 131I-19-iodocholesterol in the diagnosis of Cushing's syndrome associated with adrenal tumor

    Seven patients with Cushing's syndrome secondary to adrenocortial tumors were studied using 131I-19-iodocholesterol. The diagnoses of all cases were verified histologically. In three cases with adenoma the uptake of the tracer was in the tumor only, while the two patients with adrenocortical carcinoma failed to show adrenal accumulation of the labelled compound. In two patients there was a hyperplasia-like scintigraphic pattern, while the stimulation and suppression biochemical tests suggested adrenal tumor. One of these cases was verified as a mixed form (adenoma plus hyperplasia), and the tumor bearing gland was significantly larger on the scan which helped the preoperative localization. In the other case, verified as bilateral multiple adrenocortical adenomas, the autonomus function of both adrenals was proved by dexamethasone suppression scanning. It seems reasonable to use the latter as an adjunctive diagnostic procedure in patients where there is a discrepancy between the standard scintiscan and the biochemical indexes of adrenal hyperfunction. (orig.) 891 MG/orig. 892 MBE

  2. Bilateral symmetrical adrenal hypermetabolism on FDG PET/CT due to Cushing syndrome in well differentiated neuroendocrine carcinoma.

    Aktas, G E; Soyluoglu Demir, S; Sarikaya, A

    2016-01-01

    The (18)F-FDG PET/CT scan has been suggested for whole-body imaging to identify ectopic adrenocorticotrophic hormone secreting tumours, but there are some challenges involved. The case of a patient is presented, who was admitted with the pre-diagnosis of ectopic ACTH syndrome. On the CT, a nodular lesion was detected in the medial segment of the right lung. The FDG uptake of the lesion seemed to be increased visually, but was not pathological quantitatively (SUVmax: 1.8) on the PET/CT. There was also diffuse increased uptake (SUVmax: 14.2) in the enlarged adrenal glands. The lesion was reported as a possible malignant lesion with low FDG affinity, such as a low grade neuroendocrine tumour, while the diffuse enlarged adrenal glands with high uptake were interpreted as diffusely hyperplasic, due to Cushing's syndrome. The patient was treated with a surgical wedge resection. The histopathological diagnosis confirmed that the tumour was a grade 1 well-differentiated neuroendocrine carcinoma. PMID:26522002

  3. Cushing syndrome secondary to a medullary thyroid carcinoma: report of a case and review of the literature = Síndrome de Cushing secundario a carcinoma medular de tiroides: descripción de un caso y revisión de la literatura

    Gutiérrez Restrepo, Johnayro; Castro Martínez, Diva Cristina; Vélez Hoyos, Alejandro; Pérez Cadavid, Juan Camilo

    2014-01-01

    We report the case of a 29-year old female who was evaluated because of a thyroid tumor. The initial pathological classification was an insular thyroid carcinoma. There was strong involvement in the neck, mediastinum and lungs. Three years after receiving specific therapy for her thyroid neoplasia, she developed a Cushing syndrome and liver lesions suggestive of metastases from the primary tumor. A review of the previous pathological material revealed a medullary thyroid carcinoma producing A...

  4. Desenvolvimento de um radioimunoensaio de dexametasona no soro como subsídio para o diagnóstico da síndrome de Cushing Development of serum dexamethasone radioimmunoassay to corroborate Cushing's syndrome diagnosis

    Lilian Fukusima Hayashi

    2011-10-01

    Full Text Available INTRODUÇÃO: O teste de supressão com 1 mg de dexametasona (TSDx é amplamente empregado no rastreamento da síndrome de Cushing (SC dada sua elevada acurácia diagnóstica. A SC é um distúrbio endocrinometabólico resultante do hipercortisolismo crônico com característica de ausência de supressão do cortisol no TSDx. OBJETIVO: Desenvolver um radioimunoensaio (RIE para a dosagem de dexametasona (Dx no soro para complementar o TSDx. MÉTODOS: Foram imunizados três coelhos com o conjugado dexametasona-21-hemissuccinato-BSA para escolha do melhor anticorpo e o RIE foi desenvolvido de acordo com as recomendações da RE 899/2003 da Agência Nacional de Vigilância Sanitária (ANVISA. Analisamos 96 voluntários, sendo 67 submetidos ao TSDx e 29 não, e 12 pacientes com SC, estudados na Universidade Federal de São Paulo (UNIFESP e na Santa Casa de Misericórdia de São Paulo. RESULTADOS: O anticorpo contra Dx selecionado mostrou boa especificidade, coeficiente de variação (CV% intra e interensaio INTRODUCTION: The 1 mg dexamethasone suppression test (DxST is widely used to screen Cushing's syndrome (CS due to its high diagnostic accuracy. CS is an endocrine-metabolic disorder caused by hypercorticism, which is characterized by the absence of cortisol suppression in DxST. OBJECTIVE: To develop a radioimmunoassay (RIA for the measurement of serum dexamethasone (Dx to complement DxST. METHODS: Three rabbits were inoculated with dexamethasone-21-hemisuccinate-BSA in order to choose the best antibody. Serum Dx RIA was performed according to RE 899/2003 (Agência Nacional de Vigilância Sanitária [ANVISA] regulations. Serum samples from 96 volunteers from Universidade Federal de São Paulo (UNIFESP and Santa Casa de Misericórdia de São Paulo were analyzed, 67 of which were submitted to DxST and 29 were not. There were 12 patients with CS. RESULTS: The Dx antibody chosen showed good specificity. Intra- and interassay CV were < 20% with 93

  5. Síndrome de Cushing: veinticinco años de experiencia clínica en Navarra Cushing´s syndrome: twenty-five years of clinical experience in Navarre

    L. Pérez García

    2012-12-01

    Full Text Available Fundamento. El objetivo de este trabajo es conocer la situación del Síndrome de Cushing (CS en cuanto a etiología, diagnóstico, tratamiento y curación en los últimos 25 años en Navarra. Material y métodos. Este estudio -retrospectivo, observacional-, se ha llevado a cabo sobre 36 pacientes diagnosticados de SC entre 1985 y 2010. Resultados. El 80,5% eran mujeres y el 19,5% hombres. La edad media al diagnóstico fue 39,3±13 años. Las principales manifestaciones clínicas fueron: obesidad (85,3%, cara de luna llena (35,3% y miopatía (50%. Etiológicamente, el 58,3 % tenían origen hipofisario, el 36,1% adrenal y el 5,5% ectópico. La media de CLU fue 343,5±310,1 μg/24horas. El cortisol sérico tras el Nugent fue de 15,6±8,6 μg/dl. La media del cortisol plasmático tras el frenado débil fue 15,1±14,2 μg/dl. Existe correlación lineal directa estadísticamente significativa (pBackground. The aim of this work is to determine the situation of Cushing´s Syndrome (CS with respect to aetiology, diagnosis, treatment and cure in the last 25 years in Navarre. Methods. Retrospective, observational study on 36 patients diagnosed with CS between 1985 and 2010. Results. Eighty point five percent were women and 19.5% were men. The average age on diagnosis was 39.3±13 years. The main clinical manifestations were: obesity (85.3%, full moon face (35.3% and myopathy (50%. Aetiologically, in 58.3% the origin was hypophisiary, in 36.1% it was adrenal, and in 5.5% it was ectopic. The UFC average was 343.5±310.1 μg/24hours. Serum cortisol after Nugent test was 15.6±8.6 μg/dl. Low dose plasma cortisol average was 15.1±14.2 μg/dl. There is a statistically significant (p< 0.01 direct lineal correlation between UFC and the Nugent test, as well as between the Nugent test and low dose cortisol test. There are no statistically significant differences between adrenal and hypophisiary aetiology in either demographic factors or analytical results

  6. Despistaje rutinario de Síndrome de Cushing en pacientes con obesidad mórbida: ¿es realmente necesario?

    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...

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

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

    2001-01-01

    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...

  8. How Many People Are Affected by or at Risk for Cushing's Syndrome?

    ... diseases increases a person’s risk of developing a pituitary tumor, 4 which can lead to Cushing’s syndrome. These ... 3131. PMID 19734443 . [top] Melmed, S. (2011). Pathogenesis of pituitary tumors. Nature Reviews Endocrinology , 7 , 257-266. [top] « What ...

  9. Treatment of acromegaly patients with risk-adapted single or fractionated stereotactic high-precision radiotherapy. High local control and low toxicity in a pooled series

    The purpose of this work was to evaluate a prospectively initiated two-center protocol of risk-adapted stereotactic radiosurgery (SRS) or stereotactic radiotherapy (SRT) in patients with acromegaly. In total 35 patients (16 men/19 women, mean age 54 years) were prospectively included in a treatment protocol of SRS [planning target volume (PTV) < 4 ccm, > 2 mm to optic pathways = low risk] or SRT (PTV ≥ 4 ccm, ≤ 2 mm to optic pathways = high risk). The mean tumor volume was 3.71 ccm (range: 0.11-22.10 ccm). Based on the protocol guidelines, 21 patients were treated with SRS and 12 patients with SRT, 2 patients received both consecutively. The median follow-up (FU) reached 8 years with a 5-year overall survival (OS) of 87.3 % [confidence interval (CI): 70.8-95.6 %] and 5-year local control rate of 97.1 % (CI: 83.4-99.8 %). Almost 80 % (28/35) presented tumor shrinkage during FU. Endocrinological cure was achieved in 23 % and IGF-1 normalization with reduced medication was achieved in 40 % of all patients. An endocrinological response was generally achieved within the first 3 years, but endocrinological cure can require more than 8 years. A new adrenocorticotropic hypopituitarism occurred in 13 patients (46.4 %). A new visual field disorder and a new oculomotor palsy occurred in 1 patient, respectively. Patients with occurrence of visual/neurological impairments had a longer FU (p = 0.049). Our SRS/SRT protocol proved to be safe and successful in terms of tumor control and protection of the visual system. The timing and rate of endocrine improvements are difficult to predict. One has to accept an unavoidable rate of additional adrenocorticotropic hypopituitarism in the long term. (orig.)

  10. Pamidronic acid and cabergoline as effective long-term therapy in a 12-year-old girl with extended facial polyostotic fibrous dysplasia, prolactinoma and acromegaly in McCune-Albright syndrome: a case report

    Classen Carl

    2012-01-01

    Full Text Available Abstract Introduction McCune-Albright syndrome is a complex inborn disorder due to early embryonal postzygotic somatic activating mutations in the GNAS1 gene. The phenotype is very heterogeneous and includes polyostotic fibrous dysplasia, typically involving the facial skull, numerous café-au-lait spots and autonomous hyperfunctions of several endocrine systems, leading to hyperthyroidism, hypercortisolism, precocious puberty and acromegaly. Case presentation Here, we describe a 12-year-old Caucasian girl with severe facial involvement of fibrous dysplasia, along with massive acromegaly due to growth hormone excess and precocious puberty, with a prolactinoma. Our patient was treated with a bisphosphonate and the prolactin antagonist, cabergoline, resulting in the inhibition of fibrous dysplasia and involution of both the prolactinoma and growth hormone excess. During a follow-up of more than two years, no severe side effects were noted. Conclusion Treatment with bisphosphonates in combination with cabergoline is a suitable option in patients with McCune-Albright syndrome, especially in order to circumvent surgical interventions in patients suffering from polyostotic fibrous dysplasia involving the skull base.

  11. DISEASES

    Pletscher-Frankild, Sune; Pallejà, Albert; Tsafou, Kalliopi;

    2015-01-01

    Text mining is a flexible technology that can be applied to numerous different tasks in biology and medicine. We present a system for extracting disease-gene associations from biomedical abstracts. The system consists of a highly efficient dictionary-based tagger for named entity recognition of...... human genes and diseases, which we combine with a scoring scheme that takes into account co-occurrences both within and between sentences. We show that this approach is able to extract half of all manually curated associations with a false positive rate of only 0.16%. Nonetheless, text mining should not...... stand alone, but be combined with other types of evidence. For this reason, we have developed the DISEASES resource, which integrates the results from text mining with manually curated disease-gene associations, cancer mutation data, and genome-wide association studies from existing databases. The...

  12. 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

    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

  13. Cushing syndrome secondary to a medullary thyroid carcinoma: report of a case and review of the literature = Síndrome de Cushing secundario a carcinoma medular de tiroides: descripción de un caso y revisión de la literatura

    Gutiérrez Restrepo, Johnayro

    2014-08-01

    Full Text Available We report the case of a 29-year old female who was evaluated because of a thyroid tumor. The initial pathological classification was an insular thyroid carcinoma. There was strong involvement in the neck, mediastinum and lungs. Three years after receiving specific therapy for her thyroid neoplasia, she developed a Cushing syndrome and liver lesions suggestive of metastases from the primary tumor. A review of the previous pathological material revealed a medullary thyroid carcinoma producing ACTH, instead of the insular carcinoma. Based on this case a review of the literature is presented.

  14. Despistaje rutinario de Síndrome de Cushing en pacientes con obesidad mórbida: ¿es realmente necesario?

    María Rosa Alhambra Expósito

    2014-05-01

    Full Text Available 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 incluyeron 399 pacientes que iban a someterse a cirugía bariátrica. A todos los pacientes se les realizó una historia clínica completa, incluyendo exploración física y test de Nugent, como parte del protocolo precirugía. Resultados: 399 pacientes (308 mujeres, edad media 41,9 ± 10,5 años; IMC medio 51,5 ± 8,4 kg/m². El 10,3% de los pacientes presentaban prediabetes y el 27,8% diabetes. En 21 de los 399 pacientes, el despistaje fue anormal. En 8 pacientes, medimos cortisol libre en orina de 24 horas (CLU, siendo en todos normal al menos en dos ocasiones (CLU < 150 mcg/24 h, lo que descartaba SC. En 13 pacientes, repetimos el test de Nugent; sólo en 3, el test resultó patológico. Dos de los pacientes estaban en tratamiento con carbamacepina, lo que se consideró un falso positivo. El otro paciente se diagnosticó de Enfermedad de Cushing. Conclusión: La prevalencia de SC fue muy baja en pacientes con obesidad mórbida. Nuestros datos sugieren que no se debería realizarse un despistaje de SC de forma rutinaria.

  15. Ectopic Cushing syndrome

    ... tumors of the pancreas Medullary carcinoma of the thyroid Small cell tumors of the lung Tumors of the thymus gland ... syndrome Pancreatic islet cell tumor Pheochromocytoma Rheumatoid arthritis Thyroid cancer - medullary carcinoma Tumor Update Date 10/28/2015 Updated by: Brent ...

  16. What Causes Cushing's Syndrome?

    ... in the lungs, pancreas (pronounced PAN-kree-uhs ), thyroid, or thymus How Tumors Can Cause Cushing’s Syndrome Normally, the pituitary gland ... cancerous, are mostly found in the lungs, pancreas, thyroid, and thymus. ... are more vulnerable to tumors in one or more glands that influence cortisol ...

  17. Cushing's Syndrome in Children

    ... may detect only about half of ACTH producing pituitary tumors; however, recently, at the National Institutes of Health, ... problems, dia- betes, and increased susceptibility to infections. · Pituitary tumors (adenomas) are usually treated by surgical removal, which ...

  18. Treatment of acromegaly patients with risk-adapted single or fractionated stereotactic high-precision radiotherapy. High local control and low toxicity in a pooled series

    Bostroem, Jan Patrick [Mediclin Robert Janker Clinic and MediClin MVZ Bonn, Department of Radiosurgery and Stereotactic Radiotherapy, Bonn (Germany); University Hospital of Bonn, Department of Neurosurgery, Bonn (Germany); Kinfe, Thomas; Pintea, Bogdan [University Hospital of Bonn, Department of Neurosurgery, Bonn (Germany); Meyer, Almuth [HELIOS Klinikum Erfurt, Department of Endocrinology, Erfurt (Germany); Gerlach, Ruediger [HELIOS Klinikum Erfurt, Department of Neurosurgery, Erfurt (Germany); Surber, Gunnar; Hamm, Klaus [HELIOS Klinikum Erfurt, Department of Radiosurgery, Erfurt (Germany); Lammering, Guido [Mediclin Robert Janker Clinic and MediClin MVZ Bonn, Department of Radiosurgery and Stereotactic Radiotherapy, Bonn (Germany); Heinrich-Heine-University of Duesseldorf, Department of Radiotherapy and Radiation Oncology, Duesseldorf (Germany)

    2015-01-10

    The purpose of this work was to evaluate a prospectively initiated two-center protocol of risk-adapted stereotactic radiosurgery (SRS) or stereotactic radiotherapy (SRT) in patients with acromegaly. In total 35 patients (16 men/19 women, mean age 54 years) were prospectively included in a treatment protocol of SRS [planning target volume (PTV) < 4 ccm, > 2 mm to optic pathways = low risk] or SRT (PTV ≥ 4 ccm, ≤ 2 mm to optic pathways = high risk). The mean tumor volume was 3.71 ccm (range: 0.11-22.10 ccm). Based on the protocol guidelines, 21 patients were treated with SRS and 12 patients with SRT, 2 patients received both consecutively. The median follow-up (FU) reached 8 years with a 5-year overall survival (OS) of 87.3 % [confidence interval (CI): 70.8-95.6 %] and 5-year local control rate of 97.1 % (CI: 83.4-99.8 %). Almost 80 % (28/35) presented tumor shrinkage during FU. Endocrinological cure was achieved in 23 % and IGF-1 normalization with reduced medication was achieved in 40 % of all patients. An endocrinological response was generally achieved within the first 3 years, but endocrinological cure can require more than 8 years. A new adrenocorticotropic hypopituitarism occurred in 13 patients (46.4 %). A new visual field disorder and a new oculomotor palsy occurred in 1 patient, respectively. Patients with occurrence of visual/neurological impairments had a longer FU (p = 0.049). Our SRS/SRT protocol proved to be safe and successful in terms of tumor control and protection of the visual system. The timing and rate of endocrine improvements are difficult to predict. One has to accept an unavoidable rate of additional adrenocorticotropic hypopituitarism in the long term. (orig.) [German] Zielsetzung dieser Arbeit ist die Evaluation eines prospektiv angelegten Behandlungsprotokolls einer risikoadaptierten stereotaktischen Radiochirurgie (SRS) oder stereotaktischen Radiotherapie (SRT) von Patienten mit Akromegalie aus 2 Zentren. Insgesamt 35 Patienten (16

  19. A designated centre for people with disabilities operated by The Cheshire Foundation in Ireland, Cork

    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.

  20. Sustained biochemical control in patients with acromegaly treated with lanreotide depot 120 mg administered every 4 weeks, or an extended dosing interval of 6 or 8 weeks: a pharmacokinetic approach

    Gomez-Panzani E

    2012-12-01

    Full Text Available Edda Gomez-Panzani,1 Stephen Chang,1 Joaquim Ramis,2 Michelle M Landolfi,1 Bert Bakker11Ipsen Biopharmaceuticals, Inc, Basking Ridge, New Jersey, USA; 2Ipsen Innovation SAS, Pharmacokinetic and Drug Metabolism, Les Ulis, FranceObjective: Lanreotide depot is a long-acting somatostatin receptor ligand injected deep subcutaneously every 4 weeks for the treatment of acromegaly. The aim of the presented studies was to establish whether lanreotide depot, administered to patients with acromegaly at an extended dosing interval of 6 or 8 weeks, is effective in maintaining appropriate serum growth hormone (GH and insulin-like growth factor-1 (IGF-1 levels, with acceptable tolerability.Methods: Two studies were conducted. Study B1 compared lanreotide depot 120 mg (every 4, 6, or 8 weeks with lanreotide microparticle formulation 30 mg (every 7, 10, or 14 days in 98 patients who had a GH level of ≤2.5 ng/mL and normalized IGF-1. Study B2 evaluated lanreotide depot 120 mg administered to 64 patients every 8 weeks, after which the dosing interval was adjusted based on GH levels.Results: Mean lanreotide trough serum concentrations at steady state for all dosing intervals were >1.13 ng/mL, shown to achieve a GH level of ≤2.5 ng/mL. In Study B1, following treatment with lanreotide depot given every 6 or 8 weeks, 87.5% and 93.9% of patients, respectively, had normalized GH, whereas 83.3% and 88.5% of patients, respectively, had both normalized GH and IGF-1. In Study B2, 88.9% had normalized GH and 42.9% of patients had normalized GH and IGF-1 following lanreotide depot every 8 weeks. Gastrointestinal disorders, generally mild/moderate in severity, were the most common adverse events.Conclusion: In the studies presented, lanreotide depot 120 mg every 4, 6, or 8 weeks provided effective hormonal control with acceptable safety. An extended dosing interval is a feasible approach for patients adequately controlled with lanreotide depot 60 or 90 mg every 4 weeks

  1. Carney complex (CNC

    Bertherat Jérôme

    2006-06-01

    Full Text Available Abstract The Carney complex (CNC is a dominantly inherited syndrome characterized by spotty skin pigmentation, endocrine overactivity and myxomas. Skin pigmentation anomalies include lentigines and blue naevi. The most common endocrine gland manifestations are acromegaly, thyroid and testicular tumors, and adrenocorticotropic hormone (ACTH-independent Cushing's syndrome due to primary pigmented nodular adrenocortical disease (PPNAD. PPNAD, a rare cause of Cushing's syndrome, is due to primary bilateral adrenal defect that can be also observed in some patients without other CNC manifestations or familial history of the disease. Myxomas can be observed in the heart, skin and breast. Cardiac myxomas can develop in any cardiac chamber and may be multiple. One of the putative CNC genes located on 17q22-24, (PRKAR1A, has been identified to encode the regulatory subunit (R1A of protein kinase A. Heterozygous inactivating mutations of PRKAR1A were reported initially in 45 to 65 % of CNC index cases, and may be present in about 80 % of the CNC families presenting mainly with Cushing's syndrome. PRKAR1A is a key component of the cAMP signaling pathway that has been implicated in endocrine tumorigenesis and could, at least partly, function as a tumor suppressor gene. Genetic analysis should be proposed to all CNC index cases. Patients with CNC or with a genetic predisposition to CNC should have regular screening for manifestations of the disease. Clinical work-up for all the manifestations of CNC should be performed at least once a year in all patients and should start in infancy. Cardiac myxomas require surgical removal. Treatment of the other manifestations of CNC should be discussed and may include follow-up, surgery, or medical treatment depending on the location of the tumor, its size, the existence of clinical signs of tumor mass or hormonal excess, and the suspicion of malignancy. Bilateral adrenalectomy is the most common treatment for Cushing

  2. Effectiveness of low-dose pasireotide in a patient with Cushing's disease: antiproliferative effect and predictivity of a short pasireotide suppression test.

    Grossrubatscher, Erika; Zampetti, Benedetta; Dalino Ciaramella, Paolo; Doneda, Paola; Loli, Paola

    2015-08-01

    This case shows efficacy of low-dose pasireotide in biochemical and clinical control of severe hypercortisolism and in tumor volume reduction in a patient with an ACTH-secreting macroadenoma. The drug may be an option for long-term treatment in some patients where control of tumor mass is an important clinical endpoint. PMID:26331021

  3. Clinical results of stereotactic hellium-ion radiosurgery of the pituitary gland at Lawrence Berkeley Laboratory

    The first therapeutic clinical trial using accelerated heavy-charged particles in humans was performed at Lawrence Berkeley Laboratory (LBL) for the treatment of various endocrine and metabolic disorders of the pituitary gland, and as suppressive therapy for adenohypophyseal hormone-responsive carcinomas and diabetic retinopathy. In acromegaly, Cushing's disease, Nelson's syndrome and prolactin-secreting tumors, the therapeutic goal in the 433 patients treated has been to destroy or inhibit the growth of the pituitary tumor and control hormonal hypersecretion, while preserving a functional rim of tissue with normal hormone-secreting capacity, and minimizing neurologic injury. An additional group of 34 patients was treated for nonsecreting chromophobe adenomas. This paper discusses the methods and results of stereotactic helium-ion radiosurgery of the pituitary gland at Lawrence Berkeley Laboratory. 11 refs

  4. Clinical results of stereotactic hellium-ion radiosurgery of the pituitary gland at Lawrence Berkeley Laboratory

    Levy, R.P.; Fabrikant, J.I.; Lyman, J.T.; Frankel, K.A.; Phillips, M.H.; Lawrence, J.H.; Tobias, C.A.

    1989-12-01

    The first therapeutic clinical trial using accelerated heavy-charged particles in humans was performed at Lawrence Berkeley Laboratory (LBL) for the treatment of various endocrine and metabolic disorders of the pituitary gland, and as suppressive therapy for adenohypophyseal hormone-responsive carcinomas and diabetic retinopathy. In acromegaly, Cushing's disease, Nelson's syndrome and prolactin-secreting tumors, the therapeutic goal in the 433 patients treated has been to destroy or inhibit the growth of the pituitary tumor and control hormonal hypersecretion, while preserving a functional rim of tissue with normal hormone-secreting capacity, and minimizing neurologic injury. An additional group of 34 patients was treated for nonsecreting chromophobe adenomas. This paper discusses the methods and results of stereotactic helium-ion radiosurgery of the pituitary gland at Lawrence Berkeley Laboratory. 11 refs.

  5. Clinical results of stereotactic helium-ion radiosurgery of the pituitary gland at Lawrence Berkeley Laboratory

    Levy, R.P.; Fabrikant, J.I.; Lyman, J.T.; Frankel, K.A.; Phillips, M.H.; Lawrence, J.H.; Tobias, C.A.

    1989-12-01

    The first therapeutic clinical trial using accelerated heavy-charged particles in humans was performed for the treatment of various endocrine and metabolic disorders of the pituitary gland, and as suppressive therapy for adenohypophyseal hormone-responsive carcinomas and diabetic retinopathy. Since then, over 800 patients have received stereotactically-directed plateau-beam heavy-charged particle pituitary irradiation at this institution. In acromegaly, Cushing's disease, Nelson's syndrome and prolactin-secreting tumors, the therapeutic goal in the 433 patients treated has been to destroy or inhibit the growth of the pituitary tumor and control hormonal hypersecretion, while preserving a functional rim of tissue with normal hormone-secreting capacity, and minimizing neurologic injury. An additional group of 34 patients was treated for nonsecreting chromophobe adenomas. This paper discusses the methods and results of these treatments. 11 refs.

  6. Clinical results of stereotactic helium-ion radiosurgery of the pituitary gland at Lawrence Berkeley Laboratory

    The first therapeutic clinical trial using accelerated heavy-charged particles in humans was performed for the treatment of various endocrine and metabolic disorders of the pituitary gland, and as suppressive therapy for adenohypophyseal hormone-responsive carcinomas and diabetic retinopathy. Since then, over 800 patients have received stereotactically-directed plateau-beam heavy-charged particle pituitary irradiation at this institution. In acromegaly, Cushing's disease, Nelson's syndrome and prolactin-secreting tumors, the therapeutic goal in the 433 patients treated has been to destroy or inhibit the growth of the pituitary tumor and control hormonal hypersecretion, while preserving a functional rim of tissue with normal hormone-secreting capacity, and minimizing neurologic injury. An additional group of 34 patients was treated for nonsecreting chromophobe adenomas. This paper discusses the methods and results of these treatments. 11 refs

  7. Diagnostic use of facial image analysis software in endocrine and genetic disorders: review, current results and future perspectives.

    Kosilek, R P; Frohner, R; Würtz, R P; Berr, C M; Schopohl, J; Reincke, M; Schneider, H J

    2015-10-01

    Cushing's syndrome (CS) and acromegaly are endocrine diseases that are currently diagnosed with a delay of several years from disease onset. Novel diagnostic approaches and increased awareness among physicians are needed. Face classification technology has recently been introduced as a promising diagnostic tool for CS and acromegaly in pilot studies. It has also been used to classify various genetic syndromes using regular facial photographs. The authors provide a basic explanation of the technology, review available literature regarding its use in a medical setting, and discuss possible future developments. The method the authors have employed in previous studies uses standardized frontal and profile facial photographs for classification. Image analysis is based on applying mathematical functions evaluating geometry and image texture to a grid of nodes semi-automatically placed on relevant facial structures, yielding a binary classification result. Ongoing research focuses on improving diagnostic algorithms of this method and bringing it closer to clinical use. Regarding future perspectives, the authors propose an online interface that facilitates submission of patient data for analysis and retrieval of results as a possible model for clinical application. PMID:26162404

  8. Computed tomography in the diagnosis of adrenal disease

    From June 1977 through June 1980, sixty-one patients who were suspected to have adrenal diseases were examined with a CT scanner at Tokyo Women's Medical College. They consist of twenty five primary hyperaldosteronism, eight Cushing's syndrome, twenty pheochromocytoma and eight other adrenal masses. Ten patients were unexpectedly found to have adrenal lesion or mass simulating an adrenal tumor on CT performed for other reasons. CT findings were reviewed and correlated with surgical findings, postmortem studies and with results of other diagnostic modalities. 1. Primary hyperaldosteronism. Fifteen of twenty-five patients underwent surgery. Thirteen were pathologically proved to have aldosteronoma and two hyperplasia. Ten of thirteen patients with aldosteronoma were correctly diagnosed by CT scan. 2. Cushing's syndrome. Unilateral adenoma was correctly diagnosed preoperatively by CT scan on two surgically proved cases. CT showed marked enlargement of the adrenal gland with multiple nodules measuring less than 2 cm in diameter in the patient with nodular hyperplasia. Four patients were found to have normal-appearing adrenals with CT scan. 3. Pheochromocytoma. Three adrenal and one juxta-adrenal pheochromocytomas were detected by CT scan. Pheochromocytoma was considered as very unlikely on the basis of CT scan as well as further clinical investigation in sixteen patients. The value of CT scan for localization of extraadrenal pheochromocytoma remains established. 4. Miscellaneous adrenal disease and extra-adrenal masses simulating adrenal lesions. Two primary carcinoma, two bilateral metastasis, two adrenal neuroblastoma and a cyst were detected by CT scan. In cases with a huge mass, however, the origin and histologic diagnosis could not always be determined by CT scan. (author)

  9. CT of the normal adrenal glands and image diagnosis of the adrenal diseases

    The appearances of the adrenal glands of 200 normal subjects were evaluated using computed tomography. Comparative assessments were also made of adrenal diseases in 44 patients with using CT, ultrasound, adrenal scintigraphy and angiography. 100%(5/5) of the adenomas in Cushing's Syndrome were detected by CT, scintigraphy and venography; and 60% (3/5) of them, by ultrasonography. All(4/4) adrenal hyperplasias were diagnosed by scintigraphy; and 75% (3/4) of them by C.T. The differential diagnosis of adenomas and hyperplasia in Cushing's Syndrome can be accomplished using scintigraphy and CT. The rates for diagnosing adenomas and hyperplasia in primary aldosteronism were 91%(10/11) using CT and venography; 82%(9/11) using scintigraphy; and 20% (2/10) using ultrasonography. Small masses in the adrenal glands difficult to detect, especially in the left adrenal, using ultrasonography. All (4/4) phenochromocytomas were detected using either CT, ultrasonography of arteriography. However, considering their occaseonal ectopic origins and multiplicity, CT is regarded the examination of choice for such lesions. All 3 of the adrenal tumors in children were detected equally well by CT, ultrasonography and arteriography. Arteriography was especially valuable in determining their precise origins. All 3 nonfunctioning tumors were equally well detected by CT, ultrasonography and arteriography; however, CT and ultrasonography are regarded of special value in clinical follow up examinations. Metastatic adrenal tumors were detected at a rate of 92% (23/25) using CT. (J.P.N.)

  10. Reliability and Validity Evaluation of SF-36 among Patients with Cushing's Syndrome%健康调查简表用于库欣综合征患者生活质量评价的信效度检验

    蒋培琴; 周莹霞; 史佳; 邱娴

    2013-01-01

    目的 评价健康调查简表(36-item short form,SF-36)用于测量库欣综合征患者生活质量的信度和效度.方法 2011年1月至2012年4月便利选取上海市瑞金医院内分泌科的73例库欣综合征患者,采用SF-36对研究对象进行调查,信度检验包括内部一致性信度和折半信度,结构效度检验采用因子分析法.结果 SF-36内部一致性信度的Cronbach α系数为0.822,折半信度Spearman-Brown系数为0.771;将8个维度的得分进行因子分析,提取2个公因子,累计方差贡献率为64.587%.结论 中文版SF-36用于评价国内库欣综合征患者的生活质量具有较好的信度和效度,接受性好;在目前国内库欣综合征患者的生活质量研究尚处于初级阶段的前提下,可推广应用.%Objective Evaluation of the reliability and validity of SF-36 among patients with Cushing's syndrome. Methods Seventy-three patients with Cushing ' s syndrome from department of endocrinology of Shanghai Ruijin Hospital were included in this study according to inclusion criteria. The included subjects were measured by the SF-36. Cronbach's alpha and Spearman-Brown coefficients were used to assess reliability of SF-3 6 ; factor analysis was used to assess the construct validity; functional status score was used as the criterion to assess the criterion validity of SF-3 6. Results Cronbach' s alpha coefficient of SF-3 6 was 0. 82 2 , Split-half coefficient of SF-3 6 was 0. 7 71 , the correlation coefficient between the two parts scale was 0. 62 7. Two factors were extracted from the original eight dimensions by factor analysis. The accumulative variance contribution of the factors was 6 4. 5 87%. Conclusion With good reliability and validity, SF-3 6 scale was suitable to assess quality of life of patients with Cushing' s syndrome.

  11. Acute pancreatitis and Cushing's syndrome.

    Clague, H W; B. Warren; Krasner, N.

    1984-01-01

    A case of acute necrotizing pancreatitis in a 53-year-old man with an ectopic adrenocorticotrophin (ACTH) producing bronchial carcinoma is described. The aetiology of acute pancreatitis in relation to steroid therapy and malignancy is discussed and it is suggested that excess endogenous steroid production may also cause acute pancreatitis.

  12. 以中心性漿液性脉络膜视网膜和Cushing综合征为首发症状的肾上腺髓脂肪瘤1例报告%One case report of combined central serous chorioretinopathy and Cushing's syndrome associated with adrenal myelolipoma

    刘玲; 杨昉; 张仁良

    2011-01-01

    @@ Introduction Although adrenal myelolipoma was considered to be a non-functioning benign tumor composed of mature adipose cells and hematopoietic elements in the past, accumulating evidence suggested that adrenal myelolipoma sometimes associated with adrenocortical dysfunction, endocrine or ocular disorders. And there have been several reports that patients with adrenal myelolipoma simultaneously sufferred from Cushing ' s syndrome, Conn ’ s syndrome,pheochromocytoma, adrenogenital syndrome or virilization[1 - 2].

  13. Radioimmunoassay measurements of serum thyrotropin in patients with hypothalamic-pituitary and thyroid diseases

    Serum TSH was measured by means of double antibody radioimmunoassay using a commercial Kit Daiichi, in 21 normal subjects, 200 patients with thyroid disease and 130 patients with hypothalamic-pituitary diseases. Serum TSH concentrations in normal subjects were <2 to 8 μU/ml which rose to 8-40 μU/ml after administration of 500 μg TRH intravenously. Serum TSH was undetectable and did not respond to TRH in all untreated patients and in some euthyroid patients with Graves' disease after treatment. Undetectable TSH and no response to TRH were also observed in most patients with a hyperfunctioning thyroid nodule and those with subacute thyroiditis in the acute phase. In some patients with Hashimoto's thyroiditis and all patients with adult myxedema and cretinism, serum TSH levels were increased and showed hyperresponse to TRH. The ratio of bioassay and radioimmunoassay potency estimates for TSH in sera obtained before TRH was not statistically different from that obtained after TRH administration to patients with primary hypothyroidism. Elevated serum TSH was promptly decreased by the administration of thyroid hormone to the patients. More than 50% of patients with pituitary adenoma, acromegaly and craniopharyngioma showed normal basal TSH and no or low response of TSH to TRH. Administration of TRH failed to stimulate a rise in serum TSH in 2 sisters with isolated TSH deficiency with cretinism. Basal TSH was undetectable and showed delayed response to TRH in patients with anorexia nervosa. (auth.)

  14. Disease: H00484 [KEGG MEDICUS

    Full Text Available , Oklu R, Campbell JB Cushing proximal symphalangism and the NOG and GDF5 genes. Pediatr Radiol 38:209-15 (2...Van den Berghe H Multiple synostosis syndrome. Eur J Pediatr 134:273-5 (1980) PMID:16532400 Dawson K, Seeman

  15. Patients' experience of nursing with Acromegaly

    Jensen, Jonna Gintberg; Høi, Henriette Brahe

    for junior doctors was informative educational. Subsequently various initiatives were undertaken to optimize nursing. Among other things, hiring a specialist chief nurse, an informative theme evening for the staff, preparation of a short-term record for documentation, endocrinology training program......, and in 2011 a quantitative survey of 20 patients was completed. Patients' experiences of nursing in the control admissions were in focus. The inclusion criterion was that the patients had followed the control admissions since 2005. The results were: A greater satisfaction with call letter, receipt...... and hospitalization, a significant positive change in nursing since 2007, and the staff showed greater interest and commitment. However, there is still room for improvement as to daily discomfort....

  16. ontaneous Remission of Acromegaly Due to Apoplexy

    Cevdet Duran; Canan Ersoy; Sinem Kıyıcı; Naile Bolca; Erdinç Ertürk; Ercan Tuncel; Sazi İmamoğlu

    2008-01-01

    Pituitary apoplexy is a rare clinical syndrome characterized by sudden onset of headache and vomiting, accompanied by visual disturbance or ocular motility impairment and pituitary insufficiency. Pituitary apoplexy presenting with these symptoms is seen in approximately 3% of patients with surgically treated pituitary adenomas. In acromegalic patients, apoplexy can be related to some provocating factors and rarely may be spontaneous. We present the case of an acromegalic patient with spontane...

  17. 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

    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

  18. Dexamethasone suppression test

    ... be due to: Adrenal tumor that produces cortisol Pituitary tumor that produces ACTH Tumor in the body that ... test: no change Cushing syndrome caused by a pituitary tumor (Cushing disease) Low-dose test: no change High- ...

  19. Reasons for decision in the matter of TransCanada Keystone Pipeline GP Ltd. : facilities and toll methodology[Application dated 23 November 2007 pursuant to sections 58 and 21 of the National Energy Board Act for the Keystone Cushing Expansion Project

    NONE

    2008-07-15

    The Keystone Pipeline Project consists of a 1,235 km pipeline extending from Hardisty, Alberta to Haskett, Manitoba. It involves the construction of 2 new pipeline segments and the conversion of 1 segment of the TransCanada Pipelines Limited Mainline Line 100-1 from oil to gas service. The Canadian portion of the pipeline will connect, through a new line to be built in the United States to markets in Wood River and Patoka Illinois, providing access to western Canada crude oil producers to the southern Petroleum Administration Defence District (PADD) 2 region of the United states. In November 2007, TransCanada Keystone Pipeline GP Ltd. applied to the National Energy Board for approval of variances to some previously approved facilities which would in effect permit the construction of additional transmission facilities. Keystone planned to add 473 km of pipeline from the Nebraska/Kansas border to Cushing, Oklahoma (Cushing Extension). The company also applied for variance in the associated tolling methodologies. In particular, Keystone requested to increase the motor sizes of 20 pumping units at 7 pump stations forming part of the facilities approved in the OH-1-2007 proceedings, and requested for the relocation of 3 pumping stations. Keystone also requested that the Board exempt it from the provisions of section 30 through 32 as required of the National Energy Board Act in respect of the proposed new facilities. The company also endeavored to obtain toll methodology, pursuant to Part 4 of the Act, for the Cushing Expansion shipper. This document presented the Board's view in terms of economics of the project; traffic tolls and tariffs; engineering; land matters; public and Aboriginal consultation; and environmental and socio-economic matters. Having considered all the evidence before it, the Board was satisfied that it is in the public interest to approve the Cushing Expansion Project. The Board also considered the proposed toll methodology and found it to be

  20. Lung Disease

    ... ePublications > Our ePublications > Lung disease fact sheet ePublications Lung disease fact sheet This information in Spanish (en ... disease? More information on lung disease What is lung disease? Lung disease refers to disorders that affect ...

  1. MR imaging in adrenal diseases

    Twenty-five patients affected by adrenal glands pathology underwent CT and MRI: 6 nonfuctioning adenomas, 2 Cushing's adenomas, 2 Conn's adenomas, 6 metastases, 3 cystis, 2 carcinomas (Cushing's syndrome), 1 Lymphoma and 3 pheochromocytomas. Diagnosis was subsequently confirmed either at surgery, or autopsy, or with needle biopsy. In all cases normal adrenal glands and pathological lesions were showed by MRI. T1 signal intensity and mass diameter were compared with T2 signal intensity, represented by the intensity ratio between the adrenal mass vs normal hepatic parenchyma. MRI signal intensity, usually high in case of malignancy and low in adenomas, shows a mean value which is much wider than that referred to mass diameter evaluation (carcinoma is larger than adenoma); for this reason those findings have proved to be insufficiently accurate for adrenal tissue characterization, even for the evaluation of cysts and pheochromocytomas. In the same cases CT showed higher accuracy

  2. Reevaluation of Acromegalic Patients in Long-Term Remission according to Newly Proposed Consensus Criteria for Control of Disease

    Elisa Verrua

    2014-01-01

    Full Text Available Acromegaly guidelines updated in 2010 revisited criteria of disease control: if applied, it is likely that a percentage of patients previously considered as cured might present postglucose GH nadir levels not adequately suppressed, with potential implications on management. This study explored GH secretion, as well as hormonal, clinical, neuroradiological, metabolic, and comorbid profile in a cohort of 40 acromegalic patients considered cured on the basis of the previous guidelines after a mean follow-up period of 17.2 years from remission, in order to assess the impact of the current criteria. At the last follow-up visit, in the presence of normal IGF-I concentrations, postglucose GH nadir was over 0.4 μg/L in 11 patients (Group A and below 0.4 μg/L in 29 patients (Group B; moreover, Group A showed higher basal GH levels than Group B, whereas a significant decline of both GH and postglucose GH nadir levels during the follow-up was observed in Group B only. No differences in other evaluated parameters were found. These results seem to suggest that acromegalic patients considered cured on the basis of previous guidelines do not need a more intensive monitoring than patients who met the current criteria of disease control, supporting instead that the cut-off of 0.4 mcg/L might be too low for the currently used GH assay.

  3. Clinical evaluation of thyrotropin-releasing hormone (TRH) test with a sensitive immunoradiometric thyrotropin (TSH) assay kit

    Thyrotropin-releasing hormone (TRH) test was performed using a commercially available immunoradiometric thyrotropin (TSH) assay kit (RIA-gnost hTSH) in patients with endocrine diseases. The basal serum concentration of TSH ranged from 0.2 to 2.9 μU/ml in healthy subjects. The values for endocrine diseases, except for Graves' disease, were almost within the normal range. A significant increase in TSH values caused by TRH test was observed in females compared with males (4.4 - 24.7 μU/ml vs 4.1 - 12.3 μU/ml). In cases of Graves' disease, there was a good correlation between the basal TSH value and the response of TSH to TRH. However, in the other endocrine diseases, including acromegaly, prolactinoma, anorexia nervosa, Cushing syndrome, and hypopituitarism, the response of TSH to TRH did not necessarily correlated with the basal TSH value. TRH test would be of value in elucidating pathophysiologic features, as well as in accurately diagnosing secretion reserve of TSH. (Namekawa, K.)

  4. Clinical evaluation of thyrotropin-releasing hormone (TRH) test with a sensitive immunoradiometric thyrotropin (TSH) assay kit

    Nakamura, Saeko; Demura, Reiko; Yamanaka, Yukako; Ishiwatari, Naoko; Jibiki, Kazuko; Odagiri, Emi; Demura, Hiroshi

    1987-10-01

    Thyrotropin-releasing hormone (TRH) test was performed using a commercially available immunoradiometric thyrotropin (TSH) assay kit (RIA-gnost hTSH) in patients with endocrine diseases. The basal serum concentration of TSH ranged from 0.2 to 2.9 ..mu..U/ml in healthy subjects. The values for endocrine diseases, except for Graves' disease, were almost within the normal range. A significant increase in TSH values caused by TRH test was observed in females compared with males (4.4 - 24.7 ..mu..U/ml vs 4.1 - 12.3 ..mu..U/ml). In cases of Graves' disease, there was a good correlation between the basal TSH value and the response of TSH to TRH. However, in the other endocrine diseases, including acromegaly, prolactinoma, anorexia nervosa, Cushing syndrome, and hypopituitarism, the response of TSH to TRH did not necessarily correlated with the basal TSH value. TRH test would be of value in elucidating pathophysiologic features, as well as in accurately diagnosing secretion reserve of TSH. (Namekawa, K.).

  5. Radiotherapy for pituitary adenomas: long-term outcome and complications

    Rim, Chai Hong; Yang, Dae Sik; Park, Young Je; Yoon, Won Sup; Lee, Jung AE; Kim, Chul Yong [Korea University Medical Center, Seoul (Korea, Republic of)

    2011-09-15

    's disease). Amenorrhea was abated in 7 of 10 patients, galactorrhea in 8 of 8 patients, acromegaly in 7 of 11 patients, Cushing's disease in 4 of 4 patients. Long-term complication was observed in 4 patients; 3 patients with cerebrovascular accident, 1 patient developed dementia. Of these patients, 3 of 4 received more than 60 Gy of irradiation. EBRT is highly effective in preventing recurrence and reducing mass effect of non-secreting adenoma. Effort to improve tumor control of secreting adenoma is required. Careful long-term follow-up is required when relatively high dose is applied. Modern radiosurgery or proton RT may be options to decrease late complications.

  6. Radiotherapy for pituitary adenomas: long-term outcome and complications

    To evaluate long-term local control rate and toxicity in patients treated with external beam radiotherapy (EBRT) for pituitary adenomas. We retrospectively reviewed the medical records of 60 patients treated with EBRT for pituitary adenoma at Korea University Medical Center from 1996 and 2006. Thirty-fi ve patients had hormone secreting tumors, 25 patients had non-secreting tumors. Fifty-seven patients had received postoperative radiotherapy (RT), and 3 had received RT alone. Median total dose was 54 Gy (range, 36 to 61.2 Gy). The definition of tumor progression were as follows: evidence of tumor progression on computed tomography or magnetic resonance imaging, worsening of clinical sign requiring additional operation or others, rising serum hormone level against a previously stable or falling value, and failure of controlling serum hormone level so that the hormone level had been far from optimal range until last follow-up. Age, sex, hormone secretion, tumor extension, tumor size, and radiation dose were analyzed for prognostic significance in tumor control. Median follow-up was 5.7 years (range, 2 to 14.4 years). The 10-year actuarial local control rates for non-secreting and secreting adenomas were 96% and 66%, respectively. In univariate analysis, hormone secretion was significant prognostic factor (p = 0.042) and cavernous sinus extension was marginally significant factor (p = 0.054) for adverse local control. All other factors were not significant. In multivariate analysis, hormone secretion and gender were significant. Fifty-three patients had mass-effect symptoms (headache, dizziness, visual disturbance, hypopituitarism, loss of consciousness, and cranial nerve palsy). A total of 17 of 23 patients with headache and 27 of 34 patients with visual impairment were improved. Twenty-seven patients experienced symptoms of endocrine hypersecretion (galactorrhea, amenorrhea, irregular menstruation, decreased libido, gynecomastia, acromegaly, and Cushing's disease

  7. Preoperative normalization of cortisol levels in cushing's disease after medical treatment: Consequences for somatostatin and dopamine receptor subtype expression and in vitro response to somatostatin analogs and dopamine agonists

    R. van der Pas (Rob); R.A. Feelders (Richard); F. Gatto (Federico); C. de Bruin (Christiaan); A.M. Pereira (A.); P.M. van Koetsveld (Peter); D. Sprij-Mooij (Diana); A.M. Waaijers (Annet); F. Dogan (Fadime); S. Schulz (S.); J.M. Kros (Johan); S.W.J. Lamberts (Steven); L.J. Hofland (Leo)

    2013-01-01

    textabstractContext: Corticotroph pituitary adenomas often highly express the dopamine 2 receptor (D2R) and somatostatin receptor subtype 5 (sst5). The sst2 expression is relatively low, likely resulting from downregulating effects of high cortisol levels. This may explain why the sst2-preferring so

  8. Preoperative Normalization of Cortisol Levels in Cushing's Disease After Medical Treatment: Consequences for Somatostatin and Dopamine Receptor Subtype Expression and In Vitro Response to Somatostatin Analogs and Dopamine Agonists.

    Pas, R. van der; Feelders, R.A.; Gatto, F.; Bruin, C. de; Pereira, A.M.; Koetsveld, P.M. van; Sprij-Mooij, D.M.; Waaijers, A.M.; Dogan, F.; Schulz, S.; Kros, J.M.; Lamberts, S.W.J.; Hofland, L.J.

    2013-01-01

    Context: Corticotroph pituitary adenomas often highly express the dopamine 2 receptor (D2R) and somatostatin receptor subtype 5 (sst5). The sst2 expression is relatively low, likely resulting from downregulating effects of high cortisol levels. This may explain why the sst2-preferring somatostatin a

  9. Addison's disease presenting with idiopathic intracranial hypertension in 24-year-old woman: a case report

    Moore Peter

    2010-02-01

    Full Text Available Abstract Introduction Idiopathic intracranial hypertension can rarely be associated with an underlying endocrine disorder such as Cushing's syndrome, hyperthyroidism, or with administration of thyroxine or growth hormone. Though cases of idiopathic intracranial hypertension associated with Addison's disease in children have been reported, there is only one documented case report of this association in adults. We describe a case of an acute adrenal insufficiency precipitated by idiopathic intracranial hypertension in a Caucasian female. Case presentation A 24-year-old Caucasian woman was acutely unwell with a background of several months of generalised fatigue and intermittent headaches. She had unremarkable neurological and systemic examination with a normal computerised tomography and magnetic resonance imaging of the brain. Normal cerebrospinal fluid but increased opening pressure at lumbar puncture suggested intracranial hypertension. A flat short synacthen test and raised level of adrenocorticotrophic hormone were consistent with primary adrenal failure. Conclusion Addison's disease can remain unrecognised until precipitated by acute stress. This case suggests that idiopathic intracranial hypertension can rarely be associated with Addison's disease and present as an acute illness. Idiopathic intracranial hypertension is possibly related to an increase in the levels of arginine vasopressin peptide in serum and cerebrospinal fluid secondary to a glucocorticoid deficient state.

  10. Apoplexia pituitária seguida de remissão endócrina: relato de dois casos Pituitary apoplexy followed by endocrine remission: report of two cases

    MARCELO MIRANDA

    1998-09-01

    Full Text Available A apoplexia pituitária é evento raro e a ocorrência de remissão endócrina em pacientes portadores de tumores secretores é ainda mais incomum. O presente estudo relata os casos de dois pacientes portadores de macroadenomas (um com doença de Cushing e outro com acromegalia nos quais houve remissão endócrina após apoplexia tumoral. A primeira paciente era portadora de doença de Cushing e teve episódio ictal espontâneo de cefaléia e vômitos, após o qual iniciou remissão endócrina. Como houvesse persistência de imagem de macroadenoma à ressonância magnética, a paciente foi submetida a cirurgia transesfenoidal, sendo encontrado apenas cisto hemorrágico hipertensivo, sem sinais de tumor. O segundo paciente apresentava acromegalia e enquanto realizava um teste de LHRH teve evento agudo de cefaléia e vômitos, sem perda visual e instalação de diabetes insipidus. A tomografia computadorizada de sela túrcica mostrou sinais de sangue. Como não houve quadro visual agudo, o paciente foi seguido com exames de imagens seriadas, que demonstraram o desaparecimento completo da lesão e o aparecimento de sela vazia. A avaliação endócrina mostrou remissão da acromegalia. Tendo em vista a tendência à recidiva já documentada na literatura, esses pacientes devem continuar em seguimento a longo prazo.Pituitary apoplexy is rare and endocrine remission in patients with apopletic secreting pituitary adenomas is even rarer. This study reports on two patients with pituitary macroadenomas (one with Cushing's disease and the other with acromegaly in whom endocrine remission occurred after apoplexy. The first patient had Cushing's disease and had an ictus of headache and vomiting after which she started a progressive remission of hypercortisolism. A post-apoplexy MRI disclosed persistence of a sellar and supra-sellar mass. She was submitted to transesphenoidal surgery. An hypertensive hemorhagic cyst was found with no tumor. The second patient

  11. Glomerular Diseases

    ... Research Training & Career Development Grant programs for students, postdocs, and faculty Research at NIDDK Labs, faculty, and ... diabetes, digestive and liver diseases, kidney diseases, weight control and nutrition, urologic diseases, endocrine and metabolic diseases, ...

  12. Kawasaki Disease

    ... Content Article Body What is Kawasaki disease? Kawasaki disease is a serious and perplexing disease, the cause of which is ... influenza) with aspirin has been linked with a serious disease called Reye syndrome. Always consult your pediatrician before ...

  13. Ribbing disease

    Ribbing disease is a rare sclerosing dysplasia that involves long tubular bones, especially the tibia and femur. It occurs after puberty and is reported to be more common in women. In this article we describe how Ribbing disease can be differentiated from diseases like Engelmann-Camurati disease, van Buchem disease, Erdheim-Chester disease, osteoid osteoma, chronic osteomyelitis, stress fracture, etc

  14. Long-term results of radiotherapy in the treatment of pituitary adenomas in children and adolescents

    Grigsby, P.W.; Thomas, P.R.; Simpson, J.R.; Fineberg, B.B.

    1988-12-01

    A retrospective review was performed of 11 children and adolescents (less than 19 years of age) with diagnosed pituitary adenomas. The patients were treated with subtotal resection and postoperative irradiation (S + R) or with irradiation alone (RT) at the Radiation Oncology Center, Mallinckrodt Institute of Radiology, Washington University Medical Center, from January 1958 through December 1982. Patient conditions at diagnosis were acromegaly in one, Nelson's syndrome in one, prolactinoma in three, chromophobe adenoma in three, and Cushing's disease in three. Median follow-up was 15.6 years (range 6.3-29.5 years). Only two patients have had failure: one at 8.6 years and the other at 20.7 years following treatment. All four patients with visual field (VF) defects at diagnosis underwent S + R, with only one developing recurrent disease. The remaining seven patients, who did not have VF defects, received RT only, and there has been one failure in this group. None have suffered long-term visual complications. All have been able to continue school and/or work. Three of eight females have borne children. Hypopituitarism requiring medication occurred in all who received S + R and in four of seven who received RT only.

  15. Cirurgia endoscópica transnasal da região selar: estudo dos primeiros 100 casos Transnasal endoscopic surgery of the sellar region: study of the first 100 cases

    Jackson Gondim

    2003-09-01

    custos hospitalares. As desvantagens são a diminuição da profundidade de campo, a necessidade de constante controle do endoscópio e a necessidade de maior experiência com as técnicas de endoscopia.An endoscopic endonasal transsphenoidal approach to the sella was performed in 100 consecutive patients, with a follow up from 3 to 55 months: 57 females and 43 males, age ranging from 14 and 70 years. 76 cases pituitary adenomas: 22 were acromegaly (7 microadenomas and 15 macroadenomas; 21 null cell adenomas (3 microadenomas and 18 macroadenomas; 19 Cushing disease (11 microadenomas and 8 macroadenomas, 10 prolactinomas (6 microadenomas and 4 macroadenomas, and 4 LH adenomas (4 macroadenomas. In this serie, remission was achieved in 44.8% for macroadenomas, 60% for acromegaly, 27.7% for null cell adenoma, 50% for Cushing disease, 50% for prolactinomas and 50% for LH adenomas, and 81.4% for microadenomas 85% for acromegaly, 100% for null cell adenoma, 81.8% for Cushing disease, 66% for prolactinoma. We had also four craniopharyngiomas, four sphenoidal mucocele, three sphenoidal aspergillus, one Rathke cyst, one hypophysitis, one cavernous aneurysm, one encefalocele, one intrasellar meningioma, one intrasellar tuberculoma and a sphenoid fibrous dysplasia. In this series we also had six fistulas of the anterior base that were completely cured. We had a mortality of 2, one null cell giant adenoma in a 57 years old man and another patient, 38 years old, with a giant craniopharyngioma. The morbidity was: two cured meningitis, three cured fistulas, and two permanent diabetes insipidus. Endoscopic endonasal transsphenoidal surgery in this series resulted with comparable surgical outcomes to conventional microscopic transsphenoidal surgery. The advantages of this technique have been represented by an easier access to the lesion, better visualisation and increased illumination of the surgical sites, microdissection of the tumor with maximum preservation of the pituitary function

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

    ... Resources and Publications Is there a cure for Cushing’s syndrome? Skip sharing on social media links Share this: Page Content Untreated Cushing’s syndrome can be life-threatening—fortunately, most people with ...

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

    ... Resources and Publications What are the treatments for Cushing’s syndrome? Skip sharing on social media links Share this: Page Content Treatment for Cushing’s syndrome depends on the reason for the extra cortisol ...

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

    ... Resources and Publications What are the symptoms of Cushing’s syndrome? Skip sharing on social media links Share this: Page Content Most people with Cushing’s syndrome have a range of symptoms, 1 and one ...

  19. Early Identification of Cushing's Syndrome in Children

    ... Testing Pediatric Cushing’s Adrenal Cancer Early Identification of Cushing’s Syndrome in Children Meg Keil, MS, CNP, Dalia Batista, ... NICHD, The National Institutes of Health, Bethesda, MD Cushing’s Syndrome in Children The incidence of endogenous Cushing’s syndrome ...

  20. Cushing's Syndrome Masquerading as Treatment Resistant Depression

    Anil Kumar, B. N.; Sandeep Grover

    2016-01-01

    Treatment resistant depression (TRD) is a common clinical occurrence among patients treated for major depressive disorder. A significant proportion of patients remain significantly depressed in spite of aggressive pharmacological and psychotherapeutic approaches. Management of patient with treatment resistant depression requires thorough evaluation for physical causes. We report a case of recurrent depressive disorder, who presented with severe depressive episode without psychotic symptoms, n...

  1. Alzheimer's Disease

    Alzheimer's disease (AD) is the most common form of dementia among older people. Dementia is a brain disorder that ... higher if a family member has had the disease. No treatment can stop the disease. However, some ...

  2. Infectious Diseases

    Infectious diseases kill more people worldwide than any other single cause. Infectious diseases are caused by germs. Germs are tiny living ... to live NIH: National Institute of Allergy and Infectious Diseases

  3. Heart Disease

    ... Got Homework? Here's Help White House Lunch Recipes Heart Disease KidsHealth > For Kids > Heart Disease Print A A ... chest pain, heart attacks, and strokes . What Is Heart Disease? The heart is the center of the cardiovascular ...

  4. Lyme disease

    ... Causes Lyme disease is caused by bacteria called Borrelia burgdorferi ( B burgdorferi ). Blacklegged ticks and other species of ... Names Borreliosis; Bannwarth syndrome Images Lyme disease organism, Borrelia burgdorferi Tick, deer engorged on the skin Lyme disease - ...

  5. Huntington's Disease

    Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste ... express emotions. If one of your parents has Huntington's disease, you have a 50 percent chance of ...

  6. Krabbe disease

    ... for Krabbe disease. Some people have had a bone marrow transplant in the early stages of the disease, but ... counseling is recommended for people with a family history of Krabbe disease who are considering having children. ...

  7. Stargardt Disease

    ... Congenital Amaurosis Macular Degeneration Retinitis Pigmentosa Stargardt Disease Usher Syndrome Other Retinal Diseases Glossary News & Research News & ... for retinal degenerative diseases like retinitis pigmentosa (RP), Usher syndrome and macular degeneration . Back to top What ...

  8. Reportable diseases

    Notifiable diseases ... Centers for Disease Control and Prevention. National Notifiable Diseases Surveillance System (NNDSS). Last updated May 4, 2015. Available at: wwwn.cdc.gov/nndss . Accessed September 9, 2015.

  9. Crohn's Disease

    Crohn's disease causes inflammation of the digestive system. It is one of a group of diseases called inflammatory ... small intestine called the ileum. The cause of Crohn's disease is unknown. It may be due to an ...

  10. Wilson Disease

    ... Wilson disease. Health care providers do not use brain imaging tests to diagnose Wilson disease, though certain findings ... testing. [ Top ] Clinical Trials The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and other ...

  11. Farber's Disease

    ... Awards Enhancing Diversity Find People About NINDS NINDS Farber's Disease Information Page Synonym(s): Ceramidase Deficiency Table of Contents ( ... Trials Related NINDS Publications and Information What is Farber's Disease? Farber’s disease, also known as Farber's lipogranulomatosis, describes ...

  12. Sandhoff Disease

    ... body. Sandhoff disease is a severe form of Tay-Sachs disease, the incidence of which had been particularly ... gene therapy seen in an animal model of Tay-Sachs and Sandhoff diseases for use in humans. NIH ...

  13. Legionnaires' Disease

    ... lung disease Have a weak immune system Legionnaires' disease is serious and can be life-threatening. However, most people recover with antibiotic treatment. Centers for Disease Control and Prevention

  14. Ribbing disease

    Mukkada Philson

    2010-01-01

    Full Text Available Ribbing disease is a rare sclerosing dysplasia that involves long tubular bones, especially the tibia and femur. It occurs after puberty and is reported to be more common in women. In this article we describe how Ribbing disease can be differentiated from diseases like Engelmann-Camurati disease, van Buchem disease, Erdheim-Chester disease, osteoid osteoma, chronic osteomyelitis, stress fracture, etc.

  15. Celiac Disease

    Hero Brokalaki; Nikolaos Fotos

    2008-01-01

    Celiac disease is a small intestine disease caused by the immunological response to gluten, a component of wheat, rye and barley. The worldwide prevalence of celiac disease ranges between 0.2% and 2.2 %. The clinical features of celiac disease includes diarrhea, steatorrhea, flatulence, abdominal pain and weight loss. The asymptomatic type of celiac disease is characterized by soft or normally shaped stool, weakness, lassitude and moderate weight loss. In children, celiac disease usually aris...

  16. Parkinson's disease.

    Playfer, J R

    1997-01-01

    Parkinson's disease is a common disabling disease of old age. The diagnosis of idiopathic Parkinson's disease is based on clinical signs and has poor sensitivity, with about 25% of patients confidently diagnosed as having the disease actually having other conditions such as multi-system atrophy and other parkinsonism-plus syndromes. Benign essential tremor and arteriosclerotic pseudo-parkinsonism can easily be confused with Parkinson's disease. The cause of Parkinson's disease remains unknown...

  17. Review of 154 patients with non-functioning pituitary tumors

    A retrospective review was carried out on the charts of 154 patients with pituitary tumors that were not associated with Cushing's Disease or acromegaly. Patients were divided into three treatment groups: (1) surgery + post-operative radiation (S + R); (2) radiotherapy (R); and (3) surgery followed by radiation at the time of recurrence (S). Pretreatment evaluation revealed that patients in (S + R) had significantly larger tumors than patients in (R). However, there was no difference in the endocrinological status of patients prior to therapy in all treatment groups. The results of treatment were similar in the (S + R) and (R) treatment groups. This suggests that patients with small pituitary tumors may be treated successfully with radiation alone. Analysis of the recurrences revealed several reasons for failure initial therapy, chiefly very large or locally aggressive tumors. Patients had recurrences up to 15 years after initial therapy. Therefore, follow-up of these patients should continue over a long period of time. Complications of each form of therapy were evaluated. Only one case of vascular occlusion was felt to be radiation-related. Long term steroid replacement therapy also was associated with complications. These included avascular necrosis of the femoral heads, gram negative sepsis and Addisonian crisis. The role of surgery as a single modality could not be assessed

  18. Newcastle disease

    Newcastle disease (ND), referred to as Exotic Newcastle disease (END) in the U. S., is an acute viral disease of domestic poultry and many other bird species and a recognized worldwide problem. Occurrence of END is due to an infection with virulent strains of Newcastle disease virus (NDV) and is a ...

  19. Heart Diseases

    ... you're like most people, you think that heart disease is a problem for others. But heart disease is the number one killer in the U.S. ... of disability. There are many different forms of heart disease. The most common cause of heart disease is ...

  20. Whipple's Disease

    ... Disease Organizations​​ (PDF, 341 KB)​​​​​ Alternate Language URL Whipple Disease Page Content On this page: What is ... Nutrition Points to Remember Clinical Trials What is Whipple disease? Whipple disease is a rare bacterial infection ...

  1. [Prion diseases].

    Stoĭda, N I; Zavalishin, I A

    2012-01-01

    Prion diseases are a family of progressive neurodegenerative disorders caused by prions. There are four human prion diseases: Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, fatal insomnia and Kuru. They can arise in three different ways: acquired, familial or sporadic. We review clinical presentations, pathophysiology, morphological picture, diagnostic procedures and available treatment options of prion diseases. PMID:23235426

  2. Heart Diseases

    ... you're like most people, you think that heart disease is a problem for others. But heart disease is the number one killer in the ... of disability. There are many different forms of heart disease. The most common cause of heart disease ...

  3. Lyme Disease

    Weil, M L

    1989-01-01

    Lyme disease, caused by spirochete Borrelia burgdorferi, is the most common vector-borne disease in the United States. The clinical presentation varies depending on the stage of the illness: early disease includes erthyma migrans, early disseminated disease includes multiple erythema migrans, meningitis, cranial nerve palsies and carditis; late disease is primarily arthritis. The symptoms and signs of infection resolve in the vast majority of patients after appropriate treatment with antimicr...

  4. Measurement of bone mineral content by dual photon absorptiometry in patients with metabolic bone diseases

    Ohtani, Masami; Hino, Megumu; Ikekubo, Katsuji (Kobe City General Hospital (Japan)) (and others)

    1991-12-01

    Dual photon absorptiometry was used to measure bone mineral content in 225 patients with metabolic bone diseases (84 males and 102 females) and 186 healthy subjects (25 males and 200 females). Mineral content of the lumbar vertebrae tended to rapidly decrease after the age of 40 in healthy female subjects. For males, gradual decrease in mineral content was associated with aging. Bone mineral content showed a correlation with the severity of osteoporosis as shown on X-ray films. Mineral content tended to be decreased in the lumbar vertebrae in patients with vertebral compression fracture, and in the femur in patients with vertebral or femoral fracture. For hyperthyroidism, mineral content of the lumbar vertebrae was decreased in some females, but was within normal limit in males. Hyperparathyroidism and hypoparathyroidism tended to be associated with decrease and increase in mineral content, respectively. Two each patients with osteomalacia or Cushing syndrome had a decreased mineral content. In these patients, it was increased after the treatment. (N.K.).

  5. Complicações das cirurgias hipofisárias: análise de 120 cirurgias no HUCFF-UFRJ Complications of pituitary surgery: analysis of 120 operations at HUCFF-UFRJ

    ALICE HELENA DUTRA VIOLANTE

    1999-09-01

    Full Text Available Estudamos uma série de 108 pacientes, submetidos a 120 intervenções cirúrgicas para tratamento de tumores hipofisários realizadas no Hospital Universitário Clementino Fraga Filho (HUCFF da Universidade Federal do Rio de Janeiro (UFRJ, no período de 1979 a julho de 1998, com o objetivo de analisar a morbidade e mortalidade pós-operatória imediata. A idade dos pacientes variou entre 15 e 70 anos. Os diagnósticos etiológicos foram: adenomas não secretores, 46 (38,34%; acromegalia, 30 (25%; prolactinoma, 29 (24,16% e doença de Cushing, 15 (12,5 %. As principais complicações endocrinológicas foram: pan-hipopituitarismo,16 (13,34%; diabetes insípidus central (DIC, 15 (12,5%; hiposuprarenalismo, 4 (3,34%. Complicações neurocirúrgicas: infecção, 13 (10,84% e fístula liquórica, 6 (5 %. Ocorreram 2 óbitos (1,67%. Nossos resultados se enquadram dentro dos encontrados na literatura internacional. Quando analisamos ano a ano esta casuística identificamos queda progressiva da morbimortalidade, demonstrando a importância não só da habilidade do neurocirurgião, como da uniformidade da equipe que acompanha estes pacientes.We evaluated 120 pituitary operations performed at Hospital Universitario Clementino Fraga Filho of the Universidade Federal do Rio de Janeiro from 1979 to July 1998 with the aim of analysing the immediate post operative morbidity and mortality. The ages ranged from 15 to 70 years and the clinical diagnoses were: nonsecreting adenomas, 46 (38,34%; acromegaly, 30 (25%; prolactinomas, 29 (24,16% and Cushing`s disease 15 (12,5%. The main endocrine complications were : panhypopituitarism, 16 (13,34%; diabetes insipidus,15 (12,5% and adrenocortical insufficiency, 4 (3,34%. Neurological complications: infection, 13 (10,84%; and cerebrospinal fluid leakage, 6 (5%. Two patients died. Our results agree with those of the international literature. We identified a progressive decrease of morbidity and mortality due to surgeon

  6. Wilson Disease

    Wilson disease is a rare inherited disorder that prevents your body from getting rid of extra copper. You need ... copper into bile, a digestive fluid. With Wilson disease, the copper builds up in your liver, and ...

  7. Chagas Disease

    Chagas disease is caused by a parasite. It is common in Latin America but not in the United States. ... nose, the bite wound or a cut. The disease can also spread through contaminated food, a blood ...

  8. Legionnaires' Disease

    Legionnaires' disease is a type of pneumonia caused by bacteria. You usually get it by breathing in mist from ... spread from person to person. Symptoms of Legionnaires' disease include high fever, chills, a cough, and sometimes ...

  9. Parkinson's Disease

    Parkinson's disease (PD) is a type of movement disorder. It happens when nerve cells in the brain don't ... coordination As symptoms get worse, people with the disease may have trouble walking, talking, or doing simple ...

  10. Raynaud's Disease

    Raynaud's disease is a rare disorder of the blood vessels, usually in the fingers and toes. It causes the ... secondary Raynaud's, which is caused by injuries, other diseases, or certain medicines. People in colder climates are ...

  11. Addison Disease

    ... blood pressure and water and salt balance. Addison disease happens if the adrenal glands don't make ... problem with your immune system usually causes Addison disease. The immune system mistakenly attacks your own tissues, ...

  12. Gaucher Disease

    Gaucher disease is a rare, inherited disorder in which you do not have enough of an enzyme called glucocerebrosidase. ... It usually starts in childhood or adolescence. Gaucher disease has no cure. Treatment options for types 1 ...

  13. Fifth Disease

    Fifth disease is a viral infection caused by parvovirus B19. The virus only infects humans; it's not the same parvovirus that dogs and cats can get. Fifth disease mostly affects children. Symptoms can include a low ...

  14. Meniere's Disease

    Meniere's disease is a disorder of the inner ear. It can cause severe dizziness, a roaring sound in your ... together over several days. Some people with Meniere's disease have "drop attacks" during which the dizziness is ...

  15. Valve Disease

    ... Myocarditis Obstructive Sleep Apnea Pericarditis Peripheral Vascular Disease Rheumatic Fever Sick Sinus Syndrome Silent Ischemia Stroke Sudden Cardiac Arrest Vulnerable Plaque Valve Disease | Share Related terms: heart valves, valve insufficiency, valve regurgitation, valve stenosis, valvular ...

  16. Fifth disease

    Parvovirus B19; Erythema infectiosum; Slapped cheek rash ... Fifth disease is caused by human parvovirus B19. It often affects preschoolers or school-age children during the spring. The disease spreads through the fluids in the nose and mouth ...

  17. Endocrine Diseases

    ... Fibrosis Research Identifies Key Hurdle in Quest for Cystic Fibrosis Treatment More Research News Clinical Trials Clinical trials offer ... people and opportunities to help researchers find better treatments in the ... Fibrosis Graves' Disease Hashimoto's Disease Human Growth Hormone and ...

  18. Autoimmune Diseases

    ... Some examples of CAM are herbal products, chiropractic , acupuncture , and hypnosis . If you have an autoimmune disease, ... Toll-Free: 877-226-4267 National Institute of Diabetes and Digestive and Kidney Diseases, NIH, HHS Phone: ...

  19. Heart Disease

    ... Added Sugar Intake and Cardiovascular Diseases Mortality Among US Adults, examines the relationship between consuming too much added sugar and the risk of heart disease death. When it Comes to Blood Pressure, Make Control ...

  20. Huntington disease

    Huntington chorea ... Huntington disease is caused by a genetic defect on chromosome 4. The defect causes a part of ... 10 to 28 times. But in persons with Huntington disease, it is repeated 36 to 120 times. ...

  1. Autoinflammatory Diseases

    ... descriptions of some of the more common autoinflammatory diseases. The Immune System Familial Mediterranean Fever (FMF) Neonatal Onset Multisystem ... and monocytes to destroy harmful substances. In autoinflammatory diseases, this innate immune system causes inflammation for unknown reasons. It reacts, ...

  2. Legionnaire disease

    ... features on this page, please enable JavaScript. Legionnaire disease is an infection of the lungs and airways. It is caused by Legionella bacteria. Causes The bacteria that cause Legionnaire disease have ...

  3. Addison disease

    ... or fungal infections Hemorrhage into the adrenal glands Tumors Risk factors for the autoimmune type of Addison disease include other autoimmune diseases : Swelling (inflammation) of the thyroid gland that often results in reduced thyroid function ( chronic ...

  4. Lyme Disease

    ... enabling JavaScript. Top Banner Content Area Skip Content Marketing Share this: Main Content Area Lyme Disease Lyme ... research with the major goals of developing better means of diagnosing, treating, and preventing the disease. To accomplish ...

  5. Celiac Disease

    ... disease early before it causes damage to the intestine. But because it's easy to confuse the symptoms with other intestinal disorders, such as irritable bowel syndrome, inflammatory bowel disease , or lactose intolerance , teens with ...

  6. Parasitic Diseases

    ... water, a bug bite, or sexual contact. Some parasitic diseases are easily treated and some are not. Parasites ... can be seen with the naked eye. Some parasitic diseases occur in the United States. Contaminated water supplies ...

  7. Fifth Disease

    ... are otherwise healthy. But for some people fifth disease cause serious health complications. People with weakened immune systems caused ... transplants, or HIV infection are at risk for serious complications from fifth disease. It can cause chronic anemia that requires medical ...

  8. Menetrier's Disease

    ... with a weakened immune system, CMV can cause serious disease, such as retinitis, which can lead to blindness. ... weakened immune system in order to prevent a serious disease from developing as a result of CMV. Antiviral ...

  9. Legionnaires' Disease

    ... common name for one of the several illnesses caused by Legionnaires' disease bacteria (LDB). Legionnaires' disease is an infection of the ... Legion Convention in Philadelphia. L. pneumophila had undoubtedly caused previous ... disease bacteria (LDB). L. pneumophila is a gram-negative rod ...

  10. Autoinflammatory Diseases

    We present a review article on the autoinflammatory diseases, narrating its historical origin and describing the protein and molecular structure of the Inflammasome, the current classification of the autoinflammatory diseases and a description of the immuno genetics and clinical characteristics more important of every disease.

  11. Paddlefish Diseases

    Durborow, R.M.; Kuchta, Roman; Scholz, Tomáš

    Hoboken, New Jersey: John Wiley & Sons, Inc, 2015 - (Mims, S.; Shelton, W.), s. 227-265 ISBN 9780813810584 Institutional support: RVO:60077344 Keywords : brown blood disease * immunity * interferon regulatory factor * paddlefish diseases * Southern Regional Aquaculture Center Subject RIV: GJ - Animal Vermins ; Diseases, Veterinary Medicine

  12. Brain Diseases

    ... know what causes some brain diseases, such as Alzheimer's disease. The symptoms of brain diseases vary widely depending on the specific problem. In some cases, damage is permanent. In other cases, treatments such as surgery, medicines, or physical therapy can correct the source of the problem or ...

  13. Lyme Disease

    Ozdemir, Davut; İnce, Nevin

    2014-01-01

    Lyme disease (LD) is caused by the spirochete, Borrelia burgdorferi sensu lato complex. Humans are infected by a tick bite to the skin. This disease is a non-contagious infectious disease. It has been known since the 19th century. LD has a worldwide distribution. It is endemic in Europe, North and South America. There are case reports since 1990 in Turkey. The clinical presentation varies depending on the stage of the disease. Lyme disease is classified into three stages: early localized dise...

  14. Glomerular disease.

    Vaden, Shelly L

    2011-08-01

    Glomerular diseases are a leading cause of chronic kidney disease in dogs but seem to be less common in cats. Glomerular diseases are diverse, and a renal biopsy is needed to determine the specific glomerular disease that is present in any animal. Familial glomerulopathies occur in many breeds of dogs. However, most dogs with glomerular disease have acquired glomerular injury that is either immune-complex mediated or due to systemic factors, both of which are believed to be the result of a disease process elsewhere in the body (i.e., neoplastic, infectious, and noninfectious inflammatory disorders). A thorough clinical evaluation is indicated in all dogs suspected of having glomerular disease and should include an extensive evaluation for potential predisposing disorders. Nonspecific management of dogs with glomerular disease can be divided into 3 major categories: (1) treatment of potential predisposing disorders, (2) management of proteinuria, and (3) management of uremia and other complications of glomerular disease and chronic kidney disease. Specific management of specific glomerular diseases has not been fully studied in dogs. However, it may be reasonable to consider immunosuppressive therapy in dogs that have developed a form of glomerulonephritis secondary to a steroid-responsive disease (e.g., systemic lupus erythematosus) or have immune-mediated lesions that have been documented in renal biopsy specimens. Appropriate patient monitoring during therapy is important for maximizing patient care. The prognosis for dogs and cats with glomerular disease is variable and probably dependent on a combination of factors. The purpose of this article is to discuss the general diagnosis and management of dogs with glomerular disease. PMID:21782143

  15. Follow-up of patients with pituitary tumors before and after operation, medical treatment and radiotherapy

    From 1976 till 1981 56 patients with pituitary tumors were observed. From a total of 51 adenomas 17 prolactinomas (33.3%), 15 acromegalies (29.4%), 4 Cushing-syndromes (7.8%), 1 TSH-producing adenoma (1.9%) and 14 hormone-inactive adenomas (27.4%) were found. In addition, there were 4 craniopharyngeomas and 1 intrasellar meningeoma. For the individual types of adenomas prae- and postoperative hormonal data after transcranial resp. transsphenoidal adenomectomy are listed. Prolactinomas responded well to bromocriptine therapy in some cases, whereas acromegalies showed no remission after bromocriptine. After radiotherapy of 4 recidive-adenomas remission in one case was seen. Problems and objects of long-term-care of patients with pituitary tumors are discussed. (author)

  16. Celiac Disease

    Hero Brokalaki

    2008-07-01

    Full Text Available Celiac disease is a small intestine disease caused by the immunological response to gluten, a component of wheat, rye and barley. The worldwide prevalence of celiac disease ranges between 0.2% and 2.2 %. The clinical features of celiac disease includes diarrhea, steatorrhea, flatulence, abdominal pain and weight loss. The asymptomatic type of celiac disease is characterized by soft or normally shaped stool, weakness, lassitude and moderate weight loss. In children, celiac disease usually arises between the first and the third year of age, with diarrhea, flatulence and low weight. The malabsorption in small intestine causes many extaintestinal manifestations, such us anemia, bone abnormalities, hemorrhage and neuropathy. Celiac disease is diagnosed by histological examination of tissue samples taken by duodenum due gastroscopy and by the detection of certain antibodies in blood (anti-GL-IgG, anti-GL-IgA, ΕΜΑ-IgA και anti-tTg-IgA. The only therapeutic approach to celiac disease is a gluten-free diet and, if it is necessary, the administration of iron, folic acid, calcium and vitamins (K, B12. The prognosis of celiac disease is excellent, if there is an early diagnosis and the patient keeps for life a gluten free diet.

  17. Celiac disease

    Radlović Nedeljko

    2013-01-01

    Full Text Available Celiac disease is a multysystemic autoimmune disease induced by gluten in wheat, barley and rye. It is characterized by polygenic predisposition, high prevalence (1%, widely heterogeneous expression and frequent association with other autoimmune diseases, selective deficit of IgA and Down, Turner and Williams syndrome. The basis of the disease and the key finding in its diagnostics is symptomatic or asymptomatic inflammation of the small intestinal mucosa which resolves by gluten-free diet. Therefore, the basis of the treatment involves elimination diet, so that the disorder, if timely recognized and adequately treated, also characterizes excellent prognosis.

  18. [Moyamoya disease].

    Esin, R G; Isayeva, Yu N; Gorobets, E A; Tokareva, N V; Esin, O R

    2016-01-01

    Moya-moya is a rare cerebrovascular disease characterized by the progressive occlusion of cerebral vessels with partial switching off the circle of Willis and arteries that feed it. The article provides a review of literature, modern diagnostic criteria and a description of a single clinical case. The onset of the disease in this patient was characterized by headache and speech disorders.An analysis of speech disorders showed that they were systemic. They were registered at all language levels (phonetic, lexical,morphological, syntactic). A long diagnostic search may be explained by clinical manifestations that are atypical for other cerebrovascular diseases and by the rarity of the disease. PMID:27386589

  19. Coeliac disease.

    Green, Peter H R; Jabri, Bana

    2003-08-01

    Coeliac disease is a genetically-determined chronic inflammatory intestinal disease induced by an environmental precipitant, gluten. Patients with the disease might have mainly non-gastrointestinal symptoms, and as a result patients present to various medical practitioners. Epidemiological studies have shown that coeliac disease is very common and affects about one in 250 people. The disease is associated with an increased rate of osteoporosis, infertility, autoimmune diseases, and malignant disease, especially lymphomas. The mechanism of the intestinal immune-mediated response is not completely clear, but involves an HLA-DQ2 or HLA-DQ8 restricted T-cell immune reaction in the lamina propria as well as an immune reaction in the intestinal epithelium. An important component of the disease is the intraepithelial lymphocyte that might become clonally expanded in refractory sprue and enteropathy-associated T-cell lymphoma. Study of the mechanism of the immune response in coeliac disease could provide insight into the mechanism of inflammatory and autoimmune responses and lead to innovations in treatment. PMID:12907013

  20. Celiac disease

    Holtmeier Wolfgang

    2006-03-01

    Full Text Available Abstract Celiac disease is a chronic intestinal disease caused by intolerance to gluten. It is characterized by immune-mediated enteropathy, associated with maldigestion and malabsorption of most nutrients and vitamins. In predisposed individuals, the ingestion of gluten-containing food such as wheat and rye induces a flat jejunal mucosa with infiltration of lymphocytes. The main symptoms are: stomach pain, gas, and bloating, diarrhea, weight loss, anemia, edema, bone or joint pain. Prevalence for clinically overt celiac disease varies from 1:270 in Finland to 1:5000 in North America. Since celiac disease can be asymptomatic, most subjects are not diagnosed or they can present with atypical symptoms. Furthermore, severe inflammation of the small bowel can be present without any gastrointestinal symptoms. The diagnosis should be made early since celiac disease causes growth retardation in untreated children and atypical symptoms like infertility or neurological symptoms. Diagnosis requires endoscopy with jejunal biopsy. In addition, tissue-transglutaminase antibodies are important to confirm the diagnosis since there are other diseases which can mimic celiac disease. The exact cause of celiac disease is unknown but is thought to be primarily immune mediated (tissue-transglutaminase autoantigen; often the disease is inherited. Management consists in life long withdrawal of dietary gluten, which leads to significant clinical and histological improvement. However, complete normalization of histology can take years.

  1. McCune-Albright syndrome

    ... bones in the face Gigantism Irregular, large patchy cafe-au-lait spots , especially on the back ... Acromegaly Adrenal abnormalities Gigantism Hyperparathyroidism Hyperthyroidism Hypophosphatemia Large cafe-au-lait spots on the skin Liver disease, ...

  2. Skin morphological changes in growth hormone deficiency and acromegaly

    Lange, Merete Wolder; Thulesen, J; Feldt-Rasmussen, U;

    2001-01-01

    To evaluate the histomorphology of skin and its appendages, especially eccrine sweat glands, in patients with GH disorders, because reduced sweating ability in patients with growth hormone deficiency (GHD) is associated with increased risk of hyperthermia under stressed conditions....

  3. Beta-adrenoceptor-blocking drugs, growth hormone and acromegaly.

    Feely, J.

    1980-01-01

    Chronic treatment with oxprenolol or propranolol in active hypertensive patients was associated with elevation of serum growth hormone (GH). Propranolol, 80 mg orally, caused a marked rise in GH in 3 of 4 acromegalic patients.

  4. Hartnup disease

    Jerajani Hemangi

    1994-01-01

    Full Text Available A rare case of Hartnup disease is presented - the patient being an 11 year old school girl suffering from a typically pellagroid rash in the absence of any other signs of malnutrition. No accompanying neurological or psychiatric features are seen, but electro-encephalography revealed abnormal baseline activity. Investigations and management are detailed and the literature on Hartnup disease reviewed.

  5. Hartnup disease

    Jerajani Hemangi; Amladi Sangeeta; Kohli Malavika; Parekh Roopali

    1994-01-01

    A rare case of Hartnup disease is presented - the patient being an 11 year old school girl suffering from a typically pellagroid rash in the absence of any other signs of malnutrition. No accompanying neurological or psychiatric features are seen, but electro-encephalography revealed abnormal baseline activity. Investigations and management are detailed and the literature on Hartnup disease reviewed.

  6. Alexander Disease

    ... Alexander disease is a progressive and often fatal disease. The destruction of white matter is accompanied by the formation of Rosenthal fibers, which are abnormal clumps of protein that accumulate in non-neuronal cells ... are sometimes found in other disorders, but not in the same amount or area ...

  7. Facioscapulohumeral disease

    Padberg, George Waltherus Adrianus Maria

    1982-01-01

    The purpose of this study is to discuss several aspects of facioscapulohumeral disease, also called "autosomal dominant facioscapulohumeral muscular dystrophy" or "Landouzy-Dejerine type of muscular dystrophy" or "Landouzy-Dejerine' s disease" . We consider this disorder well defined and recognizabl

  8. Tickborne Diseases

    ... eye rash similar to that found in Lyme disease, which is caused by bacteria transmitted by the deer tick. Although researchers know that the lone star tick transmits the infectious agent that causes STARI, they ... both bacterial diseases. Babesiosis is caused by parasites carried by deer ...

  9. Whipple's disease

    ... include: Complete blood count ( CBC ) Polymerase chain reaction (PCR) test to check for the bacteria that cause the disease Small bowel biopsy Upper GI endoscopy (viewing the intestines with a flexible, lighted tube in a process called enteroscopy ) This disease may ...

  10. Lyme Disease

    ... simply be displayed as a subtle area of pink or red skin rather than the classic bull's-eye rash. Overview Lyme disease is the result of infection with the bacteria Borrelia burgdorferi. The disease is transmitted by infected ticks that also feed on mice and deer. The tick can be found attached ...

  11. Barlow's Disease

    Sozen, Yunus V.; Tozun, Remzi; Berkman, Mahmut; Akalin, Yilmaz; Kara, Ayhan Nedim

    2004-01-01

    Scurvy that is so rare nowadays is clinically and radiologically very well defined. Two cases brought to our clinic in 1979 and 1980 are presented because ths disease process was so far advanced and because the clinical ond especsally radiological manifesations of the disease were so typical. The radiological and clinical characteristics of scurvy are elucidated because of these two cases.

  12. Parasitic diseases

    Foundations of roentgenological semiotics of parasitic diseases of lungs, w hich are of the greatest practical value, are presented. Roentgenological pictu res of the following parasitic diseases: hydatid and alveolar echinococcosis, pa ragonimiasis, toxoplasmosis, ascariasis, amebiasis, bilharziasis (Schistosomias is) of lungs, are considered

  13. Whipple's Disease

    ... she sends a report to the gastroenterologist for review. Read more in Upper GI Endoscopy at www.digestive.niddk.nih.gov. How is Whipple disease treated? The health care provider prescribes antibiotics to destroy the T. whipplei bacteria and treat Whipple disease. Health care providers choose ...

  14. Pneumococcal Disease

    ... 000 adults age 65 years and older. Pneumococcal disease can cause serious illness and lifelong complications. Pneumococcal meningitis can cause hearing loss, seizures, blindness, and paralysis. Serious heart problems are ... its worst forms, pneumococcal disease kills one in every four to five people ...

  15. Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis.

    Scheinfeld, Noah

    2013-06-01

    diseases is likely underreported. Pyoderma vegetans has been noted in 2 cases of HS and 4 cases of Inflammatory Bowel Disease (IBD) and is likely a clue to the linkage of the pathology of IBD and HS. Pityriasis rubra pilaris, in particular Type VI related to HIV, has a relationship more commonly with acne conglobata, but with HS also. Single case reports of diseases associated with HS include systemic lupus erythematosus, acromegaly, Down syndrome, Bazex-Dupre´-Christol, and prurtis ani, but these might be coincidences. Pyogenic Arthritis, Pyoderma gangrenosum, and Acne (PAPA Syndrome) and Pyoderma gangrenosum, Acne, and Suppurative Hidradenitis (PASH Syndrome) are pyodermic-arthritic syndromes that are associated with HS. Erythema nodosum and granulomatous lobular mastitis have been reported with HS but the significance of these reports is uncertain. Because of scarring, HS can result in lymphedema including scrotal elephantiasis and verrucous lymphedema. HS is sometimes accompanied by obesity, hypertension, and anemia and can be considered a disease in the spectrum of metabolic syndrome, a skin disease with systemic consequences. HS, like other types of chronic inflammation when long standing in the perianal and perineal areas, can result in squamous cell cancer. A variety of drugs can induce HS. These include lithium, sirolimus, cyclosporine, vemurafenib, and oral contraceptives. Inverse psoriasis or psoriasis vulgaris as a side effect of infliximab therapy may be associated with HS. These associations aside, most cases of HS occur in isolation without coincident morbidity. PMID:24011308

  16. Celiac Disease

    Manoochehr Karjoo

    2014-08-01

    Full Text Available Celiac disease also known as gluten-sensitive enteropathy is characterized by intestinal mucosal damage and malabsorption from dietary intake of wheat, rye or barley. Symptoms may appear with introduction of cereal in the first 3 years of life. A second peak in symptoms occurs in adults during the third or forth decade and even as late as eight decade of life. The prevalence of this disease is approximately 1 in 250 adults. The disease is more prevalent in Ireland as high as 1 in 120 adults. The disorder occurs in Arab, Hispanics, Israeli Jews, Iranian and European but is rare in Chinese and African American. To have celiac disease the patient should have the celiac disease genetic markers as HLA DQ 2 and HLA DQ 8. Patient with celiac disease may have 95 per cent for DQ 2 and the rest is by DQ 8. Someone may have the genetic marker and never develops the disease. In general 50 percent with markers may develop celiac disease. To develop the disease the gene needs to become activated. This may happen with a viral or bacterial infection, a surgery, delivery, accident, or psychological stress. After activation of gene cause the tight junction to opens with the release of Zonulin This results in passage of gluten through the tight junction and formation of multiple antibodies and autoimmune disease. This also allows entrance of other proteins and development of multiple food allergies. As a result is shortening, flattening of intestinal villi resulting in food, vitamins and minerals malabsorption.

  17. Wilson's Disease

    MH Amirian

    1986-07-01

    Full Text Available The reported case is a 9-year old female who was presented with following clinical signs and symptoms: Excessive salivation, difficulty in speaking and writing, deteriorating school performance, spasticity, dysarthria, dysphagia, tremor of the wrists and shoulders. The initial impression was Wilson's disease. Increased copper in the urine after 24 hour treatment with D-penicillamine, low serum copper level and low ceruloplasmin and presence of kayser-fleischer rings in ophthalmologic slit lamp examination confirmed the diagnosis of Wilson's disease and treatment with D-penicillamine was started. It is important to keep Wilson's disease in mind in evaluating patients with presentation similar to that of this patient. Wilson's disease is a recessively inherited defect of copper metabolism. In 70-805 of cases, the presenting clinical manifestations are hepatic or neurological. kayser-fleischer ring is present in 75% of children who present with hepatic symptoms and in all children with neurological changes. The histologic appearance of liver biopsy in Wilson's disease is not pathognomonic. The early diagnosis and treatment of Wilson's disease is extremely important, as effective therapy is available. D-penicillamine is the most effective drug in treating Wilson's disease.

  18. Crohn's disease.

    Shanahan, Fergus

    2012-02-03

    Crohn\\'s disease is a disorder mediated by T lymphocytes which arises in genetically susceptible individuals as a result of a breakdown in the regulatory constraints on mucosal immune responses to enteric bacteria. Regulation of immune reactivity to enteric antigens has improved understanding of the pathophysiological mechanisms of Crohn\\'s disease, and has expanded therapeutic options for patients with this disorder. Disease heterogeneity is probable, with various underlying defects associated with a similar pathophysiological outcome. Although most conventional drug treatments are directed at modification of host response, therapeutic manipulation of the enteric flora is becoming a realistic option.

  19. Mitochondrial Diseases

    ... in your body tissues. If you have a metabolic disorder, something goes wrong with this process. Mitochondrial diseases are a group of metabolic disorders. Mitochondria are small structures that produce energy in ...

  20. Chagas disease

    Insect control with insecticides and houses that are less likely to have high insect populations will help control the spread of the disease. Blood banks in Central and South America screen donors for ...

  1. Pompe Disease

    ... weakness progressing to respiratory weakness and death from respiratory failure after a course lasting several years. The heart ... the age of one year from either cardiorespiratory failure or respiratory infection. For individuals with late onset Pompe disease, ...

  2. Alzheimer disease

    Senile dementia - Alzheimer type (SDAT); SDAT; Dementia - Alzheimer ... The exact cause of Alzheimer disease (AD) is not known. Research shows that certain changes in the brain lead to AD. You are more likely ...

  3. Menetrier's Disease

    ... campuses in Maryland and Arizona Research Resources Protocols, repositories, mouse models, plasmids, and more Technology Advancement & Transfer ... through its clearinghouses and education programs to increase knowledge and understanding about health and disease among patients, ...

  4. Gaucher disease

    Enzyme replacement therapy is available. A bone marrow transplant may be needed in severe cases. ... is recommended for prospective parents with a family history of Gaucher disease. Testing can determine if parents ...

  5. Leishmaniasis Disease

    ... please visit this page: About CDC.gov . Parasites - Leishmaniasis Parasites Home Share Compartir Disease Ulcerative skin lesion, ... border, on a Guatemalan patient who has cutaneous leishmaniasis. (Credit: B. Arana, MERTU, Guatemala) Marked splenomegaly (enlargement/ ...

  6. Legionnaires disease

    McHale, P.A.

    1988-01-01

    Describes the history of legionnaires disease, its formation and occurrence, air conditioning systems and cooling towers where legionella has been shown to exist, and precautions in the mining environment. 4 figs.

  7. Liver Diseases

    Your liver is the largest organ inside your body. It helps your body digest food, store energy, and remove poisons. There are many kinds of liver diseases. Viruses cause some of them, like hepatitis ...

  8. Sever's Disease

    ... results from physical activities and sports that involve running and jumping, especially those that take place on hard surfaces, such as track, basketball, soccer, and gymnastics. Sever's disease also can result from ...

  9. Graves' Disease

    ... thyroid disease may be sensitive to harmful side effects from iodine. Taking iodine drops or eating foods containing large amounts of iodine—such as seaweed, dulse, or kelp—may cause or worsen hyperthyroidism. ...

  10. Parkinson's Disease

    ... about 5 to 10 percent of people with Parkinson's have "early-onset" disease which begins before the age of 50. Early-onset forms of Parkinson's are often inherited, though not always, and some ...

  11. Pick disease

    ... legal advice early in the course of the disorder. Advance care directive , power of attorney, and other legal actions can make it easier to make decisions regarding the care of the person with Pick disease.

  12. Hashimoto's Disease

    ... a disease that keeps your body from absorbing vitamin B12 and making enough healthy red blood cells Lupus — ... or more tests. Sometimes, routine screening of thyroid function reveals a mildly underactive thyroid in a person ...

  13. Alzheimer disease

    Senile dementia - Alzheimer type (SDAT); SDAT; Dementia - Alzheimer ... The exact cause of Alzheimer disease (AD) is not known. Research shows that certain changes in the brain lead to AD. You are more ...

  14. Crohn's Disease

    ... most cases, people only take this medication for short periods of time since it can increase the chance of developing megacolon. Bowel Rest Sometimes Crohn's disease symptoms are severe and a person may need to ...

  15. Behcet's Disease

    ... 25 years and access publications (since 1985) and patents resulting from NIH funding. PubMed is a free ... http://www.nidcr.nih.gov National Institute of Diabetes and Digestive and Kidney Diseases Website: http://www. ...

  16. Extrapyramidal disease

    2008-01-01

    2008119 Therapeutic effect of neuropeptide PACAP27 on Parkinson′s disease in mice. WANG Gang(王刚), et al.Dept Neurol & Neurol Instit, Ruijin Hosp, Shanghai Jiaotong Univ, Med Sch, Shanghai 200025. Chin J Neurol 2007;40(12):837-841. Objective To investigate the effects of different doses of pituitary adenylate cyclase-activating polypeptide (PACAP) on the functional and morphological outcome in a mice model of Parkinson′s disease (PD) re

  17. Celiac disease

    Holtmeier, Wolfgang; Caspary, Wolfgang F

    2006-01-01

    Celiac disease is a chronic intestinal disease caused by intolerance to gluten. It is characterized by immune-mediated enteropathy, associated with maldigestion and malabsorption of most nutrients and vitamins. In predisposed individuals, the ingestion of gluten-containing food such as wheat and rye induces a flat jejunal mucosa with infiltration of lymphocytes. The main symptoms are: stomach pain, gas, and bloating, diarrhea, weight loss, anemia, edema, bone or joint pain. Prevalence for cli...

  18. Celiac disease

    Holtmeier Wolfgang; Caspary Wolfgang F

    2006-01-01

    Abstract Celiac disease is a chronic intestinal disease caused by intolerance to gluten. It is characterized by immune-mediated enteropathy, associated with maldigestion and malabsorption of most nutrients and vitamins. In predisposed individuals, the ingestion of gluten-containing food such as wheat and rye induces a flat jejunal mucosa with infiltration of lymphocytes. The main symptoms are: stomach pain, gas, and bloating, diarrhea, weight loss, anemia, edema, bone or joint pain. Prevalenc...

  19. Extrapyramidal disease

    2010-01-01

    2010380 Evaluation non-motor symptoms in Parkinson’s disease and its influence on ability of daily living. WANG Rongfei(王荣飞),et al. Dept Neurol,1st Hosp,Guangzhou Med Coll,Guangzhou 510000. Chin J Neurol 2010;43(4):273-276. Objective To evaluate the non-motor symptoms (NMS) in Parkinson’s disease (PD),and its influence on ability of daily living (ADL) in PD

  20. HIV and Rheumatic Disease

    ... A Patient / Caregiver Diseases & Conditions HIV & Rheumatic Diseases HIV and Rheumatic Disease Fast Facts Rheumatic diseases related ... knows he or she has HIV. What are HIV-associated rheumatic diseases? Some diseases of the joints ...